This sarcoma arises from the pericytes, cells that are located around vessels. Histologically they have a vascular configuration with small hyperchromatic cells. Recurrence rate is high and frankly malignant variants may metastasize to lungs, liver, and bone.
Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/Other Diagnostic Testing Gross Appearance and Clinical Variants Histopathological Features and Variants Special Stains/
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PATHOGENESIS CHARACTERIZATION INSULIN-LIKE GROWTH FACTORS
Molecular pathology of hemangiopericytomas accompanied by severe hypoglycemia: oncogenes, tumor-suppressor genes and the insulin-like growth factor family.
Pavelic K, Cabrijan T, Hrascan R, Vrkljan M, Lipovac M, Kapitanovic S, Gall-Troselj K, Bosnar MH, Tomac A, Grskovic B, Karapandza N, Pavelic LJ, Kurslin B, Spaventi S, Pavelic J.
Division of Molecular Medicine Ruder Boskovic Institute, Zagreb, Croatia.
J Cancer Res Clin Oncol 1998;124(6):307-14 Abstract quote
Relatively little is known about molecular genetic events that participate in the genesis and progression of hemangiopericytoma.
In this study, we describe two cases of hemangiopericytoma accompanied by severe hypoglycemia. Tumor cells from patient 1 exhibited insulin-growth factor I (IGF I) and insulin-like growth factor I receptor (IGF IR) mRNA transcripts. Tumor cells from patient 2 exhibited IGF II, IGF IR and IGF binding proteins 1-3 mRNA. Serum from patient 2 contained IGF II, mostly in a large molecular form ("big" IGF II); the IGF II level did not change after the tumor removal. The presence of IGF IR in tumor cells was confirmed by immunoprecipitation with antibodies that recognize human IGF IR subunit (visualized as a 460-kDa band). The hemangiopericytoma cells derived from patient 1 expressed 210000 IGF I receptors/cell. Specific binding of IGF I to the tumor cell membrane fraction was higher in tissue from patient 1, while the tissue of patient 2 showed relatively low IGF I binding. In contrast, IGF II binding was much higher in tissue from patient 2. Both tumor tissues showed positive immunostaining for c-Jun; one tumor showed strong immunostaining for c-Myc, H-Ras and p53, while the other exhibited strong reaction with H-Ras antibodies only. No loss of the heterozygosity at the genes APC, NFI and nm23-H1 loci in tumor tissue obtained from patient 1 was found.
In effect, our results suggest multiple molecular genetic changes in hemangiopericytoma -- activation of some oncogenes and the IGF growth factor family. IGF ligands together with IGF IR could be responsible for hypoglycemia and perhaps the transformed phenotype.
Color and duplex Doppler sonography of hemangiopericytoma.
Juan C, Huang G, Chin S, Hsueh C, Wu C, Hsiao H, Jen T, Chao D, Lee S.
Department of Radiology, National Defense Medical Center and Tri-Service General Hospital, 8, Section 3, Ting-Chow Road, Taipei, Taiwan.
J Clin Ultrasound 2001 Jan;29(1):51-5 Abstract quote
We report the color Doppler sonographic features in a case of hemangiopericytoma of the thigh in a 52-year-old woman.
Color Doppler sonography demonstrated the vascularity of the tumor, and spectral analysis showed waveform changes that suggested the presence of intratumoral arteriovenous shunting. The color Doppler findings correlated well with angiographic findings.
Color Doppler sonography can demonstrate intratumoral arteriovenous shunting in hemangiopericytoma and may be used to help avoid profuse bleeding when performing a preoperative biopsy.
Magnetic resonance spectroscopy of brain hemangiopericytomas: high myoinositol concentrations and discrimination from meningiomas.
Barba I, Moreno A, Martinez-Perez I, Tate AR, Cabanas ME, Baquero M, Capdevila A, Arus C.
Departament de Bioquimica i Biologia Molecular, Universitat Autonoma de Barcelona, Cerdanyola del Valles, Spain.
J Neurosurg 2001 Jan;94(1):55-60 Abstract quote
OBJECT: Hemangiopericytomas are a rare type of brain tumor that are very similar to meningiomas in appearance and symptoms but require different treatment. It is not normally possible to distinguish between them by using magnetic resonance (MR) imaging and computerized tomography studies. However, discrimination may be possible by using in vivo MR spectroscopy (MRS) because the biochemical composition of these two lesions is different. The goal of this study was to describe the use of MRS in discriminating between these similar tumor types.
METHODS: In vivo MRS spectra were acquired in 27 patients (three with hemangiopericytomas and 24 with meningiomas) by using a single-voxel proton brain examination system at 1.5 teslas with short- (20-msec) and long- (135-msec) echo times. In addition, brain biopsy specimens obtained by open craniotomy were frozen within 5 minutes of resection and stored in liquid nitrogen until they were used. The specimens were powdered, extracted with perchloric acid, redissolved in 2H2O2 and high-resolution in vitro MRS was used at 9.4 teslas to record their spectra.
CONCLUSIONS: In this study the authors show that hemangiopericytomas could be clearly distinguished from meningiomas because they have a larger peak at 3.56 ppm. Measurements of extracts of the tumors and comparison of spectra acquired with MRS at long- (135-msec) and short- (20-msec) echo times established that this was due to the much higher levels of myoinositol in the hemangiopericytomas.
LABORATORY MARKERS INSULIN GROWTH FACTOR II
Insulin-like growth factor II-producing intra-abdominal hemangiopericytoma associated with hypoglycemia.
Matsuda S, Usui M, Sakurai H, Suzuki H, Ogura Y, Shiraishi T.
Department of Surgery, Nagai General Hospital, Tsu, Japan.
J Gastroenterol 2001 Dec;36(12):851-5 Abstract quote
We report a patient with insulin-like growth factor (IGF)-II-producing hemangiopericytoma with hypoglycemia in whom repeated intra-abdominal recurrences developed over a period of about 10 years and tumor resection was performed four times.
A 67-year-old woman was admitted to our hospital in 1995 because of hypoglycemic attacks. In 1985, partial resection of the small bowel had been performed for a 17-cm abdominal tumor of the transverse mesocolon, and the pathological diagnosis was hemangiopericytoma. In 1991, left hemicolectomy had been performed for a mesosigmoidal tumor associated with hypoglycemia. In 1994, hysterectomy, bilateral adnexectomy, and resection of an intrapelvic tumor were performed. The fourth operation was performed in 1996, about 10 years after the first operation. The spleen was removed, together with more than 1500 tumors having a total weight of 1,660 g.
The hypoglycemia was ameliorated after each operation. Before this operation, her serum IGF-I level was low, but her IGF-II level was within the normal range; however, the Western immunoblot method showed that most of the IGF-II was high-molecular-weight IGF-II. The tissue IGF-I level was also low, and the IGF-II level was high, suggesting an IGF-II-producing tumor.
We suspect that the mechanism of the hypoglycemia in this patient was related to the high-molecular-weight IGF-II produced by the tumor. The patient died in 1997 because of tumor recurrence.
CHARACTERIZATION GENERAL VARIANTS CNS
- Intraventricular hemangiopericytoma.
Al-Brahim N, Devilliers R, Provias J.
Department of Pathology, McMaster University, Hamilton, Ontario, Canada.
Ann Diagn Pathol. 2004 Dec;8(6):347-51. Abstract quote
Hemangiopericytoma is a rare tumor of the central nervous system and has seldom been reported intraventricularly. A 55-year-old woman presented with gradual onset of left side weakness, gait ataxia, and tendency to miss objects in the left visual field of uncertain duration.
Magnetic resonance imaging with contrast showed a tumor with homogenous enhancement in the right lateral ventricle. The patient underwent right temporoparietal stealth-assisted craniotomy and surgical removal of the tumor. Histopathologic examination with ancillary tests confirmed hemangiopericytoma.
Awareness that hemangiopericytoma can occur as an intraventricular tumor is important for clinicians and pathologists. Because of radiologic similarity, this tumor is not to be confused with intraventricular meningioma because the prognosis is different.
Four cases of meningeal hemangiopericytoma treated with surgery and radiotherapy.
Someya M, Sakata KI, Oouchi A, Nagakura H, Satoh M, Hareyama M.
Department of Radiology, Sapporo Medical University, School of Medicine, S1W16, Chuo-Ku, Sapporo 060-8543, Japan.
Jpn J Clin Oncol 2001 Nov;31(11):548-52 Abstract quote
We report our experiences of four cases with meningeal hemangiopericytoma treated with surgery and postoperative radiotherapy and survey the literature to elucidate the efficacy of radiotherapy.
Patients were treated with surgical resection and 46-52 Gy postoperative radiotherapy. Three patients had local control for 30, 54 and 138 months, respectively and one patient had local recurrence after 49 months. Distant metastases were observed in two patients; one had multiple bone, liver and lung metastases and the other multiple bone and brain metastases. For bone and brain metastases, better tumor control was obtained with palliative radiotherapy and stereotactic radiotherapy.
Literature analyses demonstrated that surgery and postoperative radiotherapy of 50 Gy or more resulted in significantly better local control than surgery alone (p = 0.02).
Stereotactic radiosurgery was effective for intracranial recurrence or metastasis, especially when the tumor volume was <8 cm(3) and >15 Gy at the 50% isodose line was used. Radiotherapy for bone metastases was also effective for palliation.
True hemangiopericytoma of the nasal cavity.
Watanabe K, Saito A, Suzuki M, Yamanobe S, Suzuki T.
Pathology Division, Fukushima Medical University School of Medicine Hospital, 1 Hikariga-oka, Fukushima City, 960-1295, Japan.
Arch Pathol Lab Med 2001 May;125(5):686-90 Abstract quote
Two cases of nasal tumors with pericytic myoid differentiation are reported.
The tumors occurred in a 77-year-old woman and a 60-year-old man as polypoid lesions covered by normal mucosa. Histologically, the tumors were composed of uniform short spindle or stellate cells with indistinct cell borders arranged in narrow and short fascicles. Numerous blood vessels of various sizes were common in both cases. The tumor cells of both cases stained intensely with anti-vimentin and anti-actin antibodies, but not with anti-desmin, CD34, or anti-high-molecular-weight caldesmon antibodies. Ultrastructural examination revealed well-developed actin thin filaments with dense bodies, subplasmalemmal plaques, intercellular junctions, and irregular discontinuous basement membranes.
These histopathologic features suggest true pericytic differentiation of the tumors (true hemangiopericytoma), unlike soft tissue-type hemangiopericytoma. Generally, sinonasal hemangiopericytomas are subdivided into soft tissue-type hemangiopericytomas and true hemangiopericytomas identical to the cases presented here. Soft tissue-type hemangiopericytomas are frequently highly aggressive, whereas true hemangiopericytomas show localized benign behavior.
Sinonasal true hemangiopericytomas should be strictly differentiated from soft tissue-type hemangiopericytomas.
Sinonasal-type hemangiopericytoma: a clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation.
Thompson LD, Miettinen M, Wenig BM.
Am J Surg Pathol. 2003 Jun;27(6):737-49. Abstract quote
Sinonasal-type hemangiopericytoma is an uncommon upper aerodigestive tract tumor of uncertain cellular differentiation.
We report 104 cases of sinonasal-type hemangiopericytoma diagnosed between 1970 and 1995 from the files of the Armed Forces Institute of Pathology. There were 57 females and 47 males ranging in age from 5 to 86 years (mean 62.6 years). The most common clinical presentation was airway obstruction (n = 57) and/or epistaxis (n = 54), with symptoms averaging 10 months in duration. The tumors involved the nasal cavity alone (n = 47) or also a paranasal sinus (n = 26), were polypoid, and measured an average of 3.1 cm.
Histologically, the tumors were submucosal and unencapsulated and showed a diffuse growth with fascicular (n = 37) to solid (n = 50) to focally whorled (n = 7) patterns. The tumor cells were uniform in appearance with minimal pleomorphism and had spindle-shaped (n = 82) to round/oval (n = 18) nuclei with vesicular to hyperchromatic chromatin and eosinophilic to amphophilic to clear-appearing cytoplasm with indistinct cell borders. Multinucleated (tumor) giant cells were identified in a minority of cases (n = 5). Mitotic figures were inconspicuous and necrosis was absent. The tumors were richly vascularized, including staghorn-appearing vessels that characteristically had prominent perivascular hyalinization (n = 92). An associated inflammatory cell infiltrate that included mast cells and eosinophils was noted in the majority of cases (n = 87). The immunohistochemical profile included reactivity with vimentin (98%), smooth muscle actin (92%), muscle specific actin (77%), factor XIIIa (78%), and laminin (52%).
Surgery was the treatment of choice for all of the patients; adjunctive radiotherapy was given to four patients. Recurrences developed in 18 patients within 1-12 years from diagnosis. Ninety-seven patients were either alive (n = 51, mean 16.5 years) or dead (n = 46, mean 9.6 years) but free of disease. Four patients had disease at the last follow-up: three died with disease (mean 3.6 years) and one patient is alive with disease (28.3 years). Recurrent tumor (17.8%) can be managed by additional surgery.
The majority of sinonasal-type hemangiopericytomas behave in a benign manner with excellent long-term prognosis (88% raw 5-year survival) following surgery alone. Sinonasal-type hemangiopericytomas have a characteristic light microscopic appearance with an immunophenotypic profile resembling that of glomus tumors.
Polypoid dermal hemangiopericytoma: a case report.
Pollock AM, Sweeney EC.
Histopathology Department, St. James Hospital and Trinity College, Dublin, Ireland.
Am J Dermatopathol 1998 Oct;20(5):506-8 Abstract quote
A polypoid dermal lesion with histologic, immunohistochemical, and ultrastructural features of hemangiopericytoma is described.
Such tumors, arising in the dermis, are exceptionally rare, and whereas the tumor bears some resemblance to meningioma-like tumors of the skin and the well-recognized animal counterpart, canine hemangiopericytoma, it is histologically distinct.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
Myofibromatosis in adults, glomangiopericytoma, and myopericytoma: a spectrum of tumors showing perivascular myoid differentiation.
Granter SR, Badizadegan K, Fletcher CD.
Department of Pathology, Brigham & Women's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.
Am J Surg Pathol 1998 May;22(5):513-25 Abstract quote
The clinicopathologic features of 24 tumors showing perivascular myoid differentiation are described. These included tumors with histologic features of "infantile-type" myofibromatosis occurring in adult patients (8 cases), tumors with composite features of "hemangiopericytoma" and glomus tumor (9 cases), and tumors with a distinctive concentric perivascular proliferation of spindle cells (7 cases).
Evidence of morphologic overlap among these groups suggests they are closely related neoplasms that form a single spectrum. Age of patients with lesions resembling infantile-type myofibromatosis ranged from 23 to 67 years (median, 37 years).
Clinicopathologic manifestations of this disease included multicentricity (4 cases), local recurrence (3 cases), persistence of congenital lesions into adulthood (4 cases), and tumors that were multifocal within the confines of one anatomic region (7 cases). Histologically, all cases showed a biphasic pattern that consisted of fascicles of spindle cells with abundant eosinophilic cytoplasm that resembled smooth muscle, in addition to a population of more primitive spindled cells associated with a hemangiopericytomalike vascular pattern. Six cases showed reversal of the typical zonation seen in pediatric cases in that the primitive component surrounded the more mature fascicular areas. Also described are nine tumors with features that are intermediate between glomus tumor and hemangiopericytoma, which we have designated glomangiopericytoma.
These tumors are characterized by prominent branching vessels lined by a single row of endothelial cells surrounded by epithelioid cells with a glomoid appearance. In other areas, the tumors showed typical hemangiopericytomatous foci similar to those in the myofibromatosis cases. The principal points of distinction were a lack of myoid nodules and an absence of small primitive cells with basophilic cytoplasm. Ages of these patients ranged from 17 to 78 years (median, 35 years). All tumors were located in the subcutaneous tissue and the superficial soft tissue of the extremities. Recurrence developed in one of six patients with follow-up information. The recurrent tumor had features of angiomatoid malignant fibrous histiocytoma.
Finally, we describe a subset of tumors characterized by concentric periluminal proliferation of bland, round to ovoid cells, which we have designated as myopericytoma. Patient age ranged from 10 to 66 years (median, 40 years). All were located in subcutaneous and superficial soft tissue of distal extremities. One patient had two recurrences in 3 years after initial excision.
Our study suggests that these three lesional groups comprise a histologic continuum of tumors that share clinical similarities and that, perhaps, are designated more appropriately as perivascular myomas. The relationship of this family of tumors to so-called hemangiopericytoma is discussed.
VARIANTS INTRAVASCULAR MYOPERICYTOMA
McMenamin ME, Calonje E.
Department of Dermatopathology, St. John's Institute of Dermatology, St. Thomas' Hospital, London, UK.
J Cutan Pathol 2002 Oct;29(9):557-61 Abstract quote
BACKGROUND: Myopericytoma is a benign tumor composed of cells that show apparent differentiation towards putative perivascular myoid cells called myopericytes. It arises most commonly in the dermis or subcutaneous tissue of the extremities in adults.
METHODS: We describe a myopericytoma that was unusual in its intravascular location.
RESULTS: A 54-year-old man presented with a 10-year history of a painful slowly growing 1.5-cm nodule in the subcutaneous tissue of the thigh. Histologic examination of the excised lesion showed that is was entirely contained within the lumen of a vein. It was composed of a proliferation of myoid-appearing spindle cells, which were arranged in a striking concentric pattern around numerous blood vessels, in a manner that accentuated the vessel walls. This pattern is characteristic of myopericytoma. In some areas, fascicles of spindle cells, embedded in a myxoid stroma, bulged into the lumina of lesional vessels, reminiscent of myofibroma/myofibromatosis. Lesional spindle cells were diffusely positive for smooth muscle actin, focally positive for CD34 and were negative for desmin, cytokeratin, S100 protein, HMB-45 and CD31.
CONCLUSION: This case illustrates that myopericytoma can be entirely intravascular in its location.
Lipomatous hemangiopericytoma: a morphologically distinct soft tissue tumor.
Ceballos KM, Munk PL, Masri BA, O'Connell JX.
Department of Pathology, Vancouver General Hospital, British Columbia, Canada.
Arch Pathol Lab Med 1999 Oct;123(10):941-5 Abstract quote
Hemangiopericytoma of soft tissue is a controversial pathologic entity. The relative nonspecificity of the characteristic branching capillary pattern and cytologic features of the constituent cells, in addition to the lack of a distinct immunohistochemical staining profile, has resulted in uncertainty and a lack of consensus regarding this subgroup of tumors. Notwithstanding the doubt surrounding this entity, a morphologically unique variant, designated lipomatous hemangiopericytoma, was reported in 1995. To our knowledge, there have been no further reports of these tumors since the original description.
We describe a lipomatous hemangiopericytoma that arose within the thigh of a 41-year-old woman. The tumor presented as a slowly enlarging, minimally tender, pulsatile mass. The tumor was completely excised and was found to be composed of an admixture of typical hemangiopericytoma and predominantly mature adipose tissue. Unlike previous descriptions of this entity, the current example exhibited a full range of adipocyte differentiation, including many multivacuolated adipocytes of variable size with characteristic nuclear scalloping (lipoblast-like cells). The mitotic count was less than 2 per 10 high-power fields examined. The clinical course has been benign during the short follow-up period.
We discuss the pathologic features, including the immunohistochemical staining profile and ultrastructural appearance of this distinctive tumor, and briefly discuss the relationship between hemangiopericytoma and solitary fibrous tumor of soft tissue, a neoplasm with many clinical and pathologic similarities.
Lipomatous hemangiopericytoma: a rare variant of hemangiopericytoma that may be confused with liposarcoma.
Folpe AL, Devaney K, Weiss SW.
Department of Pathology, Emory University Hospital, Atlanta, Georgia 30322, USA
Am J Surg Pathol 1999 Oct;23(10):1201-7 Abstrac quote
We describe the clinicopathologic features and biologic behavior of 16 cases of histologically benign hemangiopericytoma containing a variable amount of mature fat as an intrinsic part of the neoplasm.
These so-called lipomatous hemangiopericytomas occurred primarily in men (12 men and 4 women) with a mean age of 54 years (range, 33-74 years). All occurred in deep soft tissue and had an average size of 10 cm when first detected. All were characterized by a relatively sharp border and typical histologic features of hemangiopericytomas, including oval to round cells surrounding a sinusoidal and staghorn vasculature often with perivascular hyalinization. Mature fat varied in amount but usually occupied approximately one quarter to three quarters of the area of tumor. Mitotic activity was low, with more than half the cases having no mitotic activity. Five cases showed moderate nuclear atypia. In four cases, the pericytic regions had sclerotic zones.
In contrast to liposarcoma, neither lipoblasts nor isolated atypical hyperchromatic cells within mature fat, as are seen in well-differentiated liposarcoma, were present. Immunohistochemistry performed in four cases showed factor XIIIa in tumor cells and an intricate pattern of immunoreactivity around cells for type IV collagen. CD34 and smooth-muscle actins were identified in two of four cases. Follow-up in seven cases showed no recurrences or metastases within the follow-up period of 1 to 7 years. Because these lesions are located in deep soft tissue and contain large amounts of mature fat, they could be mistaken for well-differentiated liposarcomas in limited biopsy material, although the distinction is easily made in examining the entire specimen.
The lipomatous hemangiopericytoma represents yet another example of a bimodal mesenchymal tumor containing mature fat and raises the question of whether a common cytogenetic abnormality can explain the emergence of two clonal populations in this hybrid tumor.
Lipomatous hemangiopericytoma: a fat-containing variant of solitary fibrous tumor? Clinicopathologic, immunohistochemical, and ultrastructural analysis of a series in favor of a unifying concept.
Guillou L, Gebhard S, Coindre JM.
University Institute of Pathology, Lausanne, Switzerland.
Hum Pathol 2000 Sep;31(9):1108-15 Abstract quote
The clinicopathologic, immunohistochemical and ultrastructural features of 13 lipomatous hemangiopericytomas are presented. There were 6 male and 7 female patients whose ages at diagnosis ranged from 27 to 75 years (median 48) all presenting with a mass of variable duration. The tumor sizes ranged from 1.7 cm to 19 cm (median 5.5 cm). The locations included the orbit (1), neck (1), mediastinum (1), epicardium (1), retroperitoneum (3), right iliac fossa (1), and upper (1) and lower (4) extremity.
Histologically, the lesions were composed of a varying admixture of spindle-shaped to round cells, variably collagenous stroma, adipose tissue, and branched, often thick-walled, hemangiopericytoma-like vessels. For 11 tumors, the mitotic activity ranged from 1 to 3 mitoses per 10 high-power fields (HPF). One tumor which contained hypercellular areas showed 13 mitoses per 10 HPF, and another hypercellular lesion showed up to 43 mitoses per 10 HPF, abnormal mitoses, and necrosis. Immunohistochemically, tumor cells were invariably positive for vimentin and CD99, and mostly for CD34 but negative for desmin, keratin, CD31, CD117 (c-kit), and inhibin. About half of the tumors showed reactivity for bcl-2. Occasionally, focal reactivity was also observed for smooth muscle actin, muscle-specific actin, S100 protein, and epithelial membrane antigen.
Ultrastructural examination of seven cases showed features in keeping with fibroblastic, myofibroblastic, or pericytic differentiation.
Treatment consisted of simple tumorectomy in 10 cases and wide excision in 3. Follow-up information on 10 patients (range: 6 to 77 months; median: 18 months) showed no recurrence.
Lipomatous hemangiopericytoma which share the clinical, pathologic, immunohistochemical, and ultrastructural features of solitary fibrous tumor (SFT) is likely to represent, in most cases, a fat-containing variant of SFT.
CHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE
The histological spectrum of hemangiopericytoma: application of immunohistochemical analysis including proliferative markers to facilitate diagnosis and predict prognosis.
Middleton LP, Duray PH, Merino MJ.
Laboratory of Pathology, National Cancer Institute, Bethesda, MD 20892, USA.
Hum Pathol 1998 Jun;29(6):636-40 Abstract quote
Hemangiopericytoma (HPC) is an uncommon vascular neoplasm thought to be derived from pericytes. Prediction of patient outcome is difficult based what is currently known about these tumors and histological parameters alone.
We compiled 27 cases of HPC and evaluated the spectrum of histological features to investigate whether there was any correlation between histology, immunostaining, prognostic markers, and patient outcome. The following parameters were evaluated: vasculature, histological pattern (solid, myxoid, trabecular, alveolar), degree of cellular pleomorphism, necrosis, mitoses, and giant cell content. Immunohistochemistry was performed to determine the reactivity for CD 31, CD34, vimentin, actin, cytokeratin, S100, actin, and SMA. Proliferative rate was analyzed using antibodies to PCNA and MIB1. Patient's age ranged from 8 months to 75 years (mean, 35; median, 31). Twenty of 27 cases were located in the extremities. The tumors were grossly described as lobulated and well circumscribed (n=12) and nonencapsulated (n=15). By histology, the characteristic ramifying or staghorn vasculature pattern was seen in all cases. A solid histological pattern was mixed with an alveolar pattern in three cases, trabecular pattern in six cases, and myxoid pattern in two cases. Tumor cells were uniform, polygonal to spindle-shaped, often with vesicular nuclei. Tumor giant cells were present in 9 of 27 cases; necrosis, in 11 of 27. Mitoses ranged from 0 to 14 per 10 high-power fields (HPF). Cellular pleomorphism was 1+ in nine cases, 2+ in 12 cases, and 3+ in six cases.
Immunohistochemistry showed reactivity for CD34 and vimentin in all cases. Actin was focally positive in one case, and SMA was focally positive in another. CD 31, cytokeratin, and S100 were uniformly nonreactive. Proliferative index measured by PCNA and MIBI ranged between less than 1% and 40% of tumor cells.
Follow-up was available in 22 cases and ranged from 1 year to 15 years. Seven patients had metastases, and two recurred locally. Thirteen patients had no evidence of disease at last checkup. Parameters associated with recurrences or metastases include a trabecular pattern, the presence of necrosis, mitoses, vascular invasion, and cellular pleomorphism. Features associated with an aggressive biological behavior can be identified histologically. There was some, but not total, correlation between proliferative markers and tumor aggressiveness.
Low-affinity nerve growth factor receptor (p75) in dermatofibrosarcoma protuberans and other nonneural tumors: a study of 1,150 tumors and fetal and adult normal tissues.
Fanburg-Smith JC, Miettinen M.
Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Hum Pathol 2001 Sep;32(9):976-83 Abstract quote
Low-affinity nerve growth factor receptor (p75) is a member of the tumor necrosis factor receptor family. It may modulate the binding of nerve growth factor (NGF) to the functional high-affinity receptor tyrosine kinase (trk) A. NGF is thought to be responsible for growth, apoptosis, and function of the nervous system. The presence of this receptor (p75) was determined in a large group of neural and nonneural tumors and fetal and adult tissues.
One thousand one hundred fifty tumors were analyzed with monoclonal antibody for p75, along with selected normal fetal and adult tissues. Immunoreactivity for p75 was present in adult pericytes, perivascular fibroblasts, basal cells of several types of epithelia, perineurial cells, and dendritic reticulum cells. Additionally, a wide zone of subepithelial mesenchyme and skeletal muscle were positive in the first-trimester fetus, but were diminished or negative in the adult.
Consistently positive nonneural mesenchymal tumors included dermatofibrosarcoma protuberans (DFSP), embryonal and alveolar rhabdomyosarcoma, synovial sarcoma, and spindle cell hemangio(endotheli)oma. Schwann cell tumors, ganglioneuroma, granular cell tumor, and malignant peripheral nerve sheath tumor (MPNST) were also p75 positive.
Mesenchymal nonneural tumors that were variably positive (32% to 69%) for p75 included fibrosarcoma variants, solitary fibrous tumor, hemangiopericytoma, spindle cell lipoma, Ewing's sarcoma, mesenchymal chondrosarcoma, and malignant melanoma. Nervous system tumors such as paragangliomas, neuroblastoma, meningioma, and perineurioma and nonneural mesenchymal tumors, including extraskeletal osteosarcoma, benign fibrous histiocytomas, fibromas, alveolar soft part sarcoma, epithelioid sarcoma, smooth muscle and gastrointestinal stromal tumors, and angiosarcomas, were almost always negative for p75.
Epithelial tumors that were consistently positive included mixed tumor and adenoid cystic carcinoma, whereas mesothelioma, adenocarcinomas, and most squamous cell carcinomas were negative. p75 is not a specific marker for nerve sheath tumors. It is present in a variety of other mesenchymal tumors including synovial sarcoma and in CD34-positive tumors such as DFSP, spindle cell lipoma, and hemangiopericytoma.
The presence of p75 in nonneural tumors such as DFSP and rhabdomyosarcoma mimic its presence in early fetal mesenchyme and skeletal muscle, suggesting oncofetal expression in these tumors. p75 may be useful to distinguish DFSP from benign fibrous histiocytoma.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES JUXTAGLOMERULAR CELL TUMOR
Juxtaglomerular cell tumor: a clinicopathologic study of four cases and review of the literature.
Martin SA, Mynderse LA, Lager DJ, Cheville JC.
Department of Pathology and Laboratory Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Am J Clin Pathol 2001 Dec;116(6):854-63 Abstract quote
We studied 4 new cases of juxtaglomerular cell tumor and compared their morphologic and immunohistochemicalfeatures with 2 renal hemangiopericytomas and 5 cutaneous glomus tumors.
The juxtaglomerular tumors were resectedfrom 2 males and 2 females (mean age at diagnosis, 23 years). Three patients manifested with severe hypertension. Tumors ranged from 2.2 to 8.0 cm and were well circumscribed.
The tumors consisted of solid sheets and nodules of variably sized tumor cells with round, oval, and spindled nuclei alternating with edematous microcystic foci. Nuclear atypia, present in all tumors, was a prominent feature in 2. Mitotic activity was not identified. All cases showed hemorrhage, numerous mast cells, and thick-walled blood vessels. Unusual features included coagulative tumor necrosis, a hemangiopericytoma-like vascular pattern, and hyalinized stroma. All tumors were immunoreactive for CD34 and actin. Ultrastructural analysis revealed the presence of rhomboid-shaped renin protogranules. Patients were treated by partial or radical nephrectomy and followed up for 14 to 48 months. There were no recurrences or metastases.
The characteristic clinical and morphologic features of juxtaglomerular cell tumor permit distinction from renal hemangiopericytoma and other renal tumors.
- Myopericytoma of Skin and Soft Tissues: Clinicopathologic and Immunohistochemical Study of 54 Cases.
Mentzel T, Tos AP, Sapi Z, Kutzner H.
From *Dermatopathologische Gemeinschaftspraxis, Friedrichshafen, Germany; daggerDepartment of Pathology, Regional Hospital, Treviso, Italy; and double daggerDepartment of Pathology, St.John's Hospital, Budapest, Hungary.
Am J Surg Pathol. 2006 Jan;30(1):104-113. Abstract quote
Perivascular neoplasms comprise traditionally glomus tumor and hemangiopericytoma (HPC). Whereas glomus tumor represents a well-defined entity, the existence of HPC as a separate entity has been questioned because a number of neoplasms of different lines of differentiation are characterized by a HPC-like vascular growth pattern. Myopericytoma represents a recently delineated entity showing a HPC-like vascular pattern. A large series of myopericytoma of skin and soft tissues has been analyzed to further characterize the clinicopathologic spectrum of this entity.
Fifty-four cases of myopericytoma of skin and soft tissues were retrieved and the histology reviewed. Immunohistochemical stainings using alpha-smooth muscle actin (ASMA), desmin, and h-caldesmon antibodies were performed, and clinical data and follow-up information were obtained from referring pathologists. Thirty-four patients were male and 18 were female (gender was unknown in 2 cases). Patient age ranged from 13 to 87 years (median, 52 years). The lower extremities were most commonly affected (26 cases) followed by the upper extremities (16 cases), the head and neck region (4 cases), and the trunk (2 cases); exact location was unknown in 5 cases. In 20 cases, the neoplasms were confined to the dermis, in 6 cases an extension into the subcutis was seen, and 24 as well as 4 cases arose in subcutaneous and deep soft tissue, respectively. Two cases were multicentric; and in 1 of these patients, multiple anatomic regions were involved.
Histologically, in all cases, numerous thin-walled vessels and a concentric, perivascular arrangement of ovoid, plump spindled to round myoid tumor cells was seen. However, a broad morphologic spectrum ranging from hypocellular, fibroma-like (3 cases), myofibroma-like (2 cases), angioleiomyoma-like (12 cases), and HPC-like neoplasms (13 cases) to classic myopericytomas (14 cases) and immature, cellular lesions (2 cases) was noted. In addition, 2 neoplasms with focal glomoid features, 5 intravascular, and 1 malignant myopericytomas were found. Prominent cytologic atypia and increased proliferative activity (>3 mitoses/10 high power fields) was seen in 4 and 2 cases, respectively. Immunohistochemically, all cases tested stained positively for ASMA. In addition, 29 of 32 cases tested stained positively for h-caldesmon, whereas desmin was only focally positive in 3 of 33 cases. Follow-up information was available in 46 cases (range, 7-168 months; median, 48 months). Despite marginal or incomplete excision in 23 of 46 cases, only 2 neoplasms (1 malignant and 1 intravascular myopericytoma) recurred locally (within 1 and 4 years, respectively).
Despite overlapping morphologic features to angioleiomyoma and myofibroma, myopericytoma represents a distinct perivascular, myoid neoplasm of skin and soft tissues, characterized by a broad morphologic spectrum of concentrically, perivascularly growing myoid tumor cells that stain positively for ASMA and often for h-caldesmon, whereas desmin is usually negative. Most cases of myopericytoma behave in a benign fashion, but local recurrences and rarely metastases may occur in atypical and malignant neoplasms.
Rosette-forming epithelioid osteosarcoma: a histologic subtype with highly aggressive clinical behavior.
Okada K, Hasegawa T, Yokoyama R.
Department of Orthopaedics, Akita University School of Medicine, Akita, Japan.
Hum Pathol 2001 Jul;32(7):726-33 Abstract quote
Osteosarcoma shows a variety of histologic patterns. Rarely, this tumor may appear epithelioid, including a rosettelike configuration simulating glands.
We retrospectively reviewed 16 cases of osteosarcoma with rosettelike structures treated at the National Cancer Center and Akita University, Japan, from 1972 to 1999. The 16 patients were under 30 years of age, and males were predominant in the sex distribution. The tumors arose primarily in the metaphysis of long tubular bones, and the most common symptom was pain. Roentgenographically, the lesions showed a highly destructive appearance with varying degrees of mineralization. Twelve patients (75%) died of multiple lung metastases in spite of surgery with wide surgical margins and systemic chemotherapy. The estimated cumulative 5-year survival rate was 15%, significantly worse than the rate of 55% in 70 cases of conventional osteoblastic osteosarcoma without rosettelike structures arising in long tubular bones. All of the 16 tumors, originally classified as conventional osteoblastic osteosarcoma, predominantly displayed a small multinodular growth pattern with lacelike osteoid deposits in the center between dilated blood vessels showing a hemangiopericytoma-like appearance. Ten tumors (63%) showed immunoreactivity for epithelial membrane antigen.
We believe rosette formation in osteosarcomas of long tubular bones is an ominous sign; therefore, those tumors should be distinguished from osteosarcomas with conventional morphotypes.
SOLITARY FIBROUS TUMOR
Solitary fibrous tumor of the soft tissue. An immunohistochemical and ultrastructural study.
Hasegawa T, Hirose T, Seki K, Yang P, Sano T.
First Department of Pathology, University of Tokushima School of Medicine, Japan.
Am J Clin Pathol 1996 Sep;106(3):325-31 Abstract quote
Solitary fibrous tumor is a rare spindle cell neoplasm of adults that usually arises in the pleura, recently reported in other locations. The authors describe three cases of solitary fibrous tumors in adults that occurred as circumscribed masses in the somatic soft tissue, including the arm, back, and abdomen.
Histologically, they were characterized by a proliferation of spindle cells separated by thick bands of collagen and prominent vascularity often showing a hemangiopericytoma-like pattern. The spindle cells, having low mitotic figures and little nuclear atypicality, exhibited a variety of growth patterns, including storiform, fascicular and herringbone, and nuclear palisading. Vimentin and CD34 immunoreactivities were observed in many spindle cells of all tumors. They had ultrastructural features of fibroblast and myofibroblast in two cases examined.
Solitary fibrous tumors seem to represent distinct mesenchymal neoplasms that require us to identify their unusual location other than the pleura and be familiar with their histologic appearances for arriving at the correct diagnosis.
Myxoid synovial sarcoma: an underappreciated morphologic subset.
Krane JF, Bertoni F, Fletcher CD.
Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.
Mod Pathol 1999 May;12(5):456-62 Abstract quote
Focal myxoid change is a well-recognized feature of synovial sarcoma, but the presence of a predominantly myxoid stroma is rare.
We describe seven cases of myxoid synovial sarcoma in which marked myxoid change initially obscured the diagnosis, leading to confusion (principally with malignant peripheral nerve sheath tumor). The median age (20 yr) and tumor location (four lower extremity, two upper extremity, and one head and neck region) were similar to those found in typical synovial sarcoma.
Histologically, five cases were monophasic spindle cell lesions with a lacy appearance in areas with a prominent Alcian blue-positive myxoid stroma. Each case had foci with more typical features of synovial sarcoma, including a fascicular growth pattern with a variably collagenized stroma, stromal mast cells, a hemangiopericytoma-like vascular pattern, and calcification. Two cases showed small foci of glandular (biphasic) differentiation. Immunohistochemically, all of the seven cases were positive for epithelial membrane antigen, four of six were positive for pan-keratin, three of six were positive for S-100, two of four were positive for CD99, and six of six were negative for desmin. Clinical follow-up in six cases ranged from 8 to 48 months (median, 21 mo). Local recurrence developed in three patients at 9, 20, and 24 months, respectively. In one of these three patients, lung metastases developed at 13 months, and the patient died of disseminated disease at 23 months. In another of the three patients, lung metastases developed at 27 months. Three patients had no evidence of disease at 8, 15, and 15 months.
Our data are too limited to indicate any clinical differences between myxoid synovial sarcoma and conventional synovial sarcoma Recognition of this rare histologic variant of synovial sarcoma is important because it can easily be mistaken for other myxoid spindle cell neoplasms, potentially resulting in suboptimal therapy.
PROGNOSIS AND TREATMENT CHARACTERIZATION GENERAL
Hemangiopericytoma: a 20-year single-institution experience.
Spitz FR, Bouvet M, Pisters PW, Pollock RE, Feig BW.
Department of Surgical Oncology, The University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
Ann Surg Oncol 1998 Jun;5(4):350-5 Abstract quote
BACKGROUND: Hemangiopericytoma is an uncommon soft tissue sarcoma. We sought to evaluate the long-term outcome of a consecutively treated patient cohort with hemangiopericytoma.
METHODS: The study involved 36 adult patients (older than 16 years) with hemangiopericytoma treated at The University of Texas M. D. Anderson Cancer Center between July 1975 and July 1995. Data on clinicopathologic parameters, surgical treatment, adjuvant therapy, disease recurrence, and survival were obtained from a review of medical records.
RESULTS: The median follow-up was 57 months. Twenty-eight patients (78%) underwent complete and potentially curative resection of their primary disease. Of the nine patients (32%) who had local recurrences, four (57%) had epidural tumors and three (43%) had retroperitoneal tumors, but none had extremity tumors. Extremity tumors were associated with a significantly prolonged local recurrence-free survival compared to tumors at nonextremity anatomic sites (P <.05). Ten patients had recurrences at distant sites. Of the 13 patients who experienced any form of disease recurrence, four had recurrences after a disease-free interval of more than 5 years. The 5-year actuarial survival rate for the entire group of 36 patients was 71%. Noncurative surgical treatment (P=.007) and development of distant metastatic disease (P=.013) were associated with shortened survival.
CONCLUSION: Extended survival is common in hemangiopericytoma patients treated with curative intent. However, local and distant recurrences may occur after a prolonged disease-free interval, emphasizing the need for long-term follow-up. Retroperitoneal and meningeal tumors were associated with higher local recurrence rates; therefore, adjuvant therapies should be considered and evaluated for tumors at these sites.
Hemangiopericytoma in children and infants.
Rodriguez-Galindo C, Ramsey K, Jenkins JJ, Poquette CA, Kaste SC, Merchant TE, Rao BN, Pratt CB, Pappo AS.
Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105-2794, USA
Cancer 2000 Jan 1;88(1):198-204 Abstract quote
BACKGROUND: Hemangiopericytoma (HPC) is a soft-tissue neoplasm most commonly seen in adults; only 5-10% of cases occur in children. Childhood HPC comprises two distinct clinical entities. In children older than 1 year, it behaves in a manner similar to adult HPC. Infantile HPC, however, although histologically identical to adult HPC, has a more benign clinical course. The reasons for these differences in the natural history of HPC are not well understood.
METHODS: The authors reviewed the clinicopathologic features of HPC as well as the treatment and outcomes of the 12 children (9 males and 3 females) treated for this disease at St. Jude Children's Research Hospital over a 35-year period.
RESULTS: At diagnosis, 9 patients were older than 1 year and 3 were younger than 1 year. Among the 9 older patients, tumors were most commonly found in the lower extremities (n = 5). One patient had been treated for acute lymphoblastic leukemia 15 years earlier. One patient had metastatic disease at diagnosis, and three had unresectable tumors. Two patients experienced objective responses to chemotherapy. Three patients died of disease progression. Among the three infants, two had unresectable disease at diagnosis, and both experienced excellent responses to neoadjuvant chemotherapy. In one case, the response of the tumor to chemotherapy correlated with maturation to hemangioma. All three infants are alive without evidence of disease.
CONCLUSIONS: HPC in children older than 1 year does not differ from adult HPC, and aggressive multimodality therapy is required. Infantile HPC, on the other hand, is characterized by better clinical behavior, with documented chemoresponsiveness and spontaneous regression, and requires a more conservative surgical approach. In some cases of infantile HPC, this benign behavior correlates with maturation to hemangioma.
Proliferation index as a prognostic marker in hemangiopericytoma of the head and neck.
Kowalski PJ, Paulino AF.
Department of Pathology, University of Michigan Hospitals, Room 2G332, 1500 East Medical Center Drive, Ann Arbor, Michigan 48109-0054, USA.
Head Neck 2001 Jun;23(6):492-6 Abstract quote
BACKGROUND: Hemangiopericytoma (HPC) of the head and neck is a rare neoplasm whose biologic behavior is difficult to predict by means of conventional histologic parameters.
METHODS: H & E-stained sections from 12 cases of HPC were reviewed. Proliferation index was assessed using an immunoperoxidase stain for MIB-1 (Ki-67).
RESULTS: The study group consisted of 4 adult men, 5 adult women, and 1 infant male. Necrosis, hypercellularity, and pleomorphism were found in 1, 5, and 6 case(s), respectively. The mitotic index per 10 high power fields varied from 0-1 to 15. Proliferation indices using MIB-1 ranged from 2.6% to 52.5%. Clinical follow-up revealed 3 cases with recurrence all possessing proliferation indices of approximately 10%.
CONCLUSIONS: Standard histomorphologic features may be inadequate predictors of clinical outcome. A proliferation index of 10% or greater may indicate a more aggressive subset of HPC of the head and neck.
- Successful treatment via chemotherapy and surgical resection of a femoral hemangiopericytoma with pulmonary metastasis.
Ferigo N, etal.
Pediatric Hematology and Oncology Unit, Hopital Nord, University of Saint Etienne, Saint Etienne 42055, France.
- J Pediatr Hematol Oncol. 2006 Apr;28(4):237-40. Abstract quote
Hemangiopericytoma (HPC) is a soft-tissue neoplasm composed of proliferating capillary pericytes. It has variable and unpredictable malignancy and most commonly occurs in the fifth or sixth decade of life.
Diagnosis is based on the histological aspect. HPC is exceedingly rare in childhood. In both adults and children, curative surgery is the most important predictor of survival. The place of chemotherapy in the treatment of HPC is not well established.
We describe a case of adult-type metastatic HPC of the thigh in a 13-year-old boy. The response to neoadjuvant chemotherapy was excellent, and local control of this initially unresectable tumor was achieved without radiation therapy or mutilating surgery. The child is alive and well and has had 8 years of follow-up after treatment.
CENTRAL NERVOUS SYSTEM AND MENINGES
- Intracranial hemangiopericytomas: correlation of topoisomerase IIalpha expression with biologic behavior.
Chacko G, etal.
Division of Neuropathology, Department of Neurological Sciences, Christian Medical College and Hospital, Vellore 632004, India.
- Surg Neurol. 2006 Jan;65(1):11-7 Abstract quote
BACKGROUND: Meningeal hemangiopericytomas are aggressive tumors that have a high rate of recurrence despite gross total resection and radiation therapy. Topoisomerase, a cell proliferation marker, is also a target of certain chemotherapeutic agents, and its nuclear levels are speculated to predict efficacy of targeted therapy. The aim of this study was to correlate the topoisomerase IIalpha proliferation index (TPI) with biologic behavior in intracranial hemangiopericytomas.
METHODS: Clinical, radiological, and management data in 27 patients with intracranial hemangiopericytoma admitted between 1990 and 2003 were reviewed. Immunohistochemistry was performed on all the tumors using a monoclonal antibody to topoisomerase IIalpha, and the proliferation index was calculated. The effect of TPI on outcome was sought.
RESULTS: The male/female ratio was 15:12. The mean age at presentation was 31.33 years. A radical excision of tumor was done in 18, subtotal excision in 2, partial excision in 4, and a biopsy in 3 patients. Tumor recurrence was noted in 15 (55.6%) of the 27 patients (mean follow-up duration, 51.5 months). The time to recurrence ranged from 7 months to 8 years (mean, 49 months). The 5-year recurrence-free survival was 33.8% in patients with a TPI of 5% or greater, and 72% in patients with a TPI of less than 5%. The relative risk of recurrence was 2.9 times greater in patients with a TPI 5% or greater as compared with those a TPI of less than 5%.
CONCLUSION: Our study suggests that cases with a radical excision, radiation therapy, or a TPI index of less than 5% have a longer recurrence-free survival. A TPI of 5% or greater is a reliable predictor of recurrence.
Meningeal hemangiopericytoma: a retrospective study of 21 patients with special review of postoperative external radiotherapy.
Dufour H, Metellus P, Fuentes S, Murracciole X, Regis J, Figarella-Branger D, Grisoli F.
Department of Neurosurgery, H pital de la Timone Adultes, Marseille, France.
Neurosurgery 2001 Apr;48(4):756-62; discussion 762-3 Abstract quote
OBJECTIVE: To specify that postoperative radiotherapy is useful for preventing local recurrence and neuraxis recurrence of surgically treated meningeal hemangiopericytomas.
METHODS: We retrospectively studied 21 patients with meningeal hemangiopericytoma who were followed in our department during a 34-year period. In 17 patients, the meningeal hemangiopericytoma was intracranial, and in 4 there was an intradural extramedullary localization. These groups were studied separately.
RESULTS: Of the 17 patients with intracranial hemangiopericytoma, all underwent surgery; 8 also underwent radiotherapy (5,000-6,400 rads) (Group I), and 9 did not (Group II). The mortality rate was zero for Group I patients and 55% for Group II. The mean local recurrence rate was 52% (12.5% in Group I and 88% in Group II; P < 0.05). Neuraxis recurrences occurred in two patients in Group II, and none occurred in Group I (P = 0.4). Peripheral metastasis took place in two patients (22%) in Group II and in one patient (12.5%) in Group I (P = 0.5). Of the four patients with intradural extramedullary hemangiopericytoma, all underwent surgery. Two patients received 4000 rads of radiotherapy after intervention. No patient in this group had a recurrence.
CONCLUSION: For patients with intracranial meningeal hemangiopericytoma, surgical removal followed by external radiotherapy reduced the risk of local recurrence. It was not demonstrated that postoperative radiotherapy protected against neuraxis metastasis. Radiotherapy did not protect against peripheral metastasis, which can occur up to several years after the first operation. It appears that radiotherapy after surgery for local or neuraxis recurrence did not avoid further recurrence. Radiosurgery is indicated for recurrent tumors measuring less than 25 mm in greatest diameter. For intradural extramedullary localizations, the value of postoperative radiotherapy is more questionable.
HEAD AND NECK
Hemangiopericytoma of the head and neck.
Billings KR, Fu YS, Calcaterra TC, Sercarz JA.
Department of Otolaryngology, Southwestern Medical Center, Dallas, TX, USA.
Am J Otolaryngol 2000 Jul-Aug;21(4):238-43 Abstract quote
PURPOSE: To report a series of patients with hemangiopericytoma (HP) of the head and neck, to review pathological features of these tumors, and to discuss management options.
MATERIALS AND METHODS: A retrospective review of the medical records at the University of California, Los Angeles (UCLA) Medical Center in Los Angeles, CA, was done in order to identify those patients with primary HP of the head and neck, including soft tissue and mucosal sites.
RESULTS: Ten patients with HP of the head and neck were identified. There was an equal sex distribution and an average age of 36 (range 10-65). Seven of the tumors arose from soft tissue sites in the head and neck, and the remaining 3 arose from the mucosa. All patients underwent wide excision of the primary lesion with a local recurrence rate of 40%. Thirty percent of patients developed metastatic lung disease 0 to 8 years after initial diagnosis. Each patient who developed metastatic disease had abundant mitoses on pathological review compared with rare or absent mitoses in the lesions that took a more benign course.
CONCLUSIONS: Pathological appearance of resected HP is predictive of later metastatic potential. Long-term follow-up is necessary in patients even after radical resection because recurrence or metastasis may be delayed by many years.
Childhood splenic hemangiopericytoma: a previously unreported entity.
Ciftci AO, Gedikoglu G, Firat PA, Senocak ME, Buyukpamukcu N.
Department of Pediatric Surgery, Hacettepe University Medical Faculty, Ankara, Turkey.
J Pediatr Surg 1999 Dec;34(12):1884-6 Abstract quote
The first childhood case of splenic hemangiopericytoma in a 10-year-old boy is presented. The clinicopathologic features of this unique entity are discussed with special emphasis on differential diagnosis and treatment by comparing with the previously reported adult cases. There are no specific presumptive clinical and laboratory findings, including tumor markers and imaging techniques that distinguish hemangiopericytoma from other splenic masses.
The most important diagnostic aid is to bear this entity in mind when a child presents with an unexplained splenic mass. Splenectomy associated with chemotherapy or radiotherapy in the presence of systemic or local recurrences is mandatory for the appropriate treatment.
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
Staghorn-This describes a histologic pattern with gaping vessels resembling a staghorn. Although not pathognomonic, this pattern is frequently present with this tumor.
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