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This has become the most common malignant sarcoma of older adults. It tends to occur in the deep soft tissue of the extremities and the retroperitoneum. However, it has been described in nearly every organ of the body. Signs and symptoms relate directly to the location in which it arises. It is a diagnosis of exclusion; the burden of proof is upon the pathologist to do a battery of immunohistochemical studies to exclude other sarcomas such as rhabdomyosarcoma and liposarcoma. Several histologic variants are described (storiform-pleomorphic, myxoid, inflammatory, giant cell, and angiomatoid) In general, all of these tumors follow an aggressive course and will recur unless widely excised. Angiomatoid variants appear to be a distinct disease occurring in younger patients and having a more indolent course. Recently, many investigators have questioned the uniqueness of this tumor, indicating that many are actually pleomorphic tumor derived from muscle cells or even fat cells. The outline below highlights some of the current controversies.


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INCIDENCE Most common malignant sarcoma of older adults


Post radiation May occur as a complication for a previous malignancy

Radiation-induced malignant fibrous histiocytoma of the neck in a patient with laryngeal carcinoma.

Haberal I, Samim E, Astarci M, Ozeri C.

ENT Clinic, Health Ministry Ankara Education and Training Hospital, Ankara, Turkey.

Am J Otolaryngol 2001 Mar-Apr;22(2):146-9 Abstract quote

Fibrohistiocytomas are soft tissue tumors of histiocytic origin that have a variety of histological patterns. Although cases of malignant fibrous histiocytoma (MFH) of the head and neck have been reported with increasing frequency in recent years, they are considered rare.

We report a case of the giant cell variant of MFH of the neck in which the patient had been given radiotherapy for T1 glottic cancer. Prognosis of MFH, the use of radiation as primary treatment, and its role in the development of secondary primary tumors in the head and neck region are reviewed.



Apoptosis in atypical fibroxanthoma and pleomorphic malignant fibrous histiocytoma.

Westermann FN, Langlois NE, Simpson JG.

Southend Hospital, Essex, UK.

Am J Dermatopathol 1997 Jun;19(3):228-31 Abstract quote

Explanations for the disparate behavior of atypical fibroxanthoma (AFX) as compared with pleomorphic malignant fibrous histiocytoma (MFH) have included the proposition that the former is a pseudosarcoma. Nonetheless, these tumors are now widely regarded as the same process, but with AFX behaving benignly by virtue of its superficial location. However, a neoplasm's metastastatic potential has been proposed to be related to apoptosis.

Therefore, the aim of the present study was to examine apoptotic counts, in conjunction with two important regulators of apoptosis: p53 and bcl-2, to determine if a distinction exists that may account for the different outcomes of these lesions. There was no significant statistical difference between eight AFX and nine pleomorphic MFH in terms of apoptotic behavior, proliferative indexes, p53 protein expression, or presence of bcl-2 product.

Therefore, our results further support the contention that AFX should be regarded as a form of pleomorphic MFH, which demonstrates low malignant potential by virtue of its location in readily accessible sites.


beta-Catenin Accumulation and Gene Mutation in Exon 3 in Dedifferentiated Liposarcoma and Malignant Fibrous Histiocytoma.

Sakamoto A, Oda Y, Adachi T, Saito T, Tamiya S, Iwamoto Y, Tsuneyoshi M.

Departments of Anatomic Pathology, Pathological Sciences (Drs Sakamoto, Oda, Adachi, Saito, Tamiya, and Tsuneyoshi), and Orthopaedic Surgery (Dr Iwamoto), Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.


Arch Pathol Lab Med 2002 Sep;126(9):1071-8 Abstract quote

Context.-beta-Catenin is an adhesion molecule that also plays a role in the Wnt signaling pathway.

Objective.-To analyze beta-catenin mutation and accumulation in a series of liposarcomas and malignant fibrous histiocytomas.

Design.-beta-Catenin mutation in exon 3 was studied using polymerase chain reaction-single-strand conformation polymorphism (PCR-SSCP) and direct sequencing analysis in 30 formalin-fixed, paraffin-embedded liposarcomas. The tumors included 12 dedifferentiated liposarcomas, characterized by both high-grade anaplastic components and well-differentiated liposarcoma components, plus 18 well-differentiated liposarcomas (10 lipoma-like and 8 sclerosing-type cases). The 2 components of dedifferentiated liposarcomas were analyzed independently. beta-Catenin accumulation in the nuclei or cytoplasm and Ki-67 expression (cell-proliferation marker, MIB-1 labeling index) were examined immunohistochemically. Nine storiform-pleomorphic-type malignant fibrous histiocytomas were also studied.

Results.-Dedifferentiated liposarcomas showed mutation in 2 cases (17%) and accumulation in 5 cases (42%). One of the 2 cases that showed mutations had a mutation in the well-differentiated component; this mutation was silent. The other case had mutations that differed between the 2 components. In well-differentiated liposarcomas, mutation was not seen in any of the cases (0/18; 0%); however, accumulation was seen frequently in the sclerosing-type cases (5/8; 63%), but not in the lipoma-like cases (0/10; 0%). Malignant fibrous histiocytomas showed mutation and accumulation in 5 (56%) and 4 (44%) cases, respectively, without any exact correlation between the cases. Cases with accumulation had a higher MIB-1 labeling index than those without, among both the sclerosing-type well-differentiated liposarcomas (P <.05) and the malignant fibrous histiocytomas.

Conclusions.-Our results suggest the possible involvement of beta-catenin activation caused by beta-catenin mutation in liposarcoma and malignant fibrous histiocytoma, but the contribution would seem to be different, depending on the tumor type. beta-Catenin accumulation is also thought to be related to cell proliferation in some of the cases.


Loss of chromosome 13 is the most frequent genomic imbalance in malignant fibrous histiocytomas. A comparative genomic hybridization analysis of a series of 30 cases.

Mairal A, Terrier P, Chibon F, Sastre X, Lecesne A, Aurias A.

Laboratoire de Pathologie Moleculaire des Cancers, Institut Curie, Paris, France.

Cancer Genet Cytogenet 1999 Jun;111(2):134-8 Abstract quote

Regional chromosome localizations of DNA copy number imbalances were studied by comparative genomic hybridization in 30 malignant fibrous histiocytomas: 13 primary tumors (2 myxoid, 9 storiform pleomorphic, and 2 with more undifferentiated phenotype) and 17 local recurrences (2 myxoid, 11 storiform pleomorphic, and 4 with more undifferentiated phenotype).

Abnormal comparative genomic hybridization (CGH) profiles were observed in 25 tumors (83%). The most frequent gains (ratio > 1.2) corresponded, by order of frequency, to entire Xp, and bands 1q21, 19q13.1, 19p13, 5p13-p14, 1p31, 17p, 18p, 20q, 1p35, 17q23, and 22q12. High levels of gains (ratio > 1.5) were recurrently detected for Xp (10 cases), and in bands 1q21-q22 (8 cases), 3q27 (4 cases), 5p13-p14 (3 cases), 13q32-q34 (3 cases), 15q22-q26 (3 cases), and 17p11-p12 (3 cases). Losses of 13q12-q14 or 13q21 were observed in a large proportion of tumors (17 cases), suggesting that a gene localized in this region could act as a tumor suppressor gene. Losses of 11q23, 2q32, 11p13, 10p, 1q4, 9p2, 16q12, 4q3, 10q25, 3p23, 2p24, and 12p were also recurrently observed.

Taken together, these results provide an overview of chromosome imbalances present in MFH, which could be of use for diagnostic purposes. They point to various chromosome regions which may harbor genes important for malignant fibrous histiocytomas (MFH) oncogenesis and progression.

Leiomyosarcomas and most malignant fibrous histiocytomas share very similar comparative genomic hybridization imbalances: an analysis of a series of 27 leiomyosarcomas.

Derre J, Lagace R, Nicolas A, Mairal A, Chibon F, Coindre JM, Terrier P, Sastre X, Aurias A.

Laboratoire de Pathologie Moleculaire des Cancers, INSERM U509, Institut Curie, Paris, France.


Lab Invest 2001 Feb;81(3):211-5 Abstract quote

Twenty-seven tumor samples with a diagnosis of leiomyosarcomas (LMS) were characterized by comparative genomic hybridization. The results were compared with immunohistochemical analysis of the smooth muscle profile of the tumors and expression of the RB1 gene protein. The comparative genomic hybridization profiles suggested that 7 of the 27 tumors might have been misclassified. High levels of DNA amplification were detected in 20 different small regions and recurrently involved bands 1p34, 1q21, 12q13-15, 17p, and 22q. Most recurrent simple gains were noted at sites such as 1p3, 1q21, 15q12-15, 16p, 17p and 17q, 19, 20q, 22q, and Xp. Significant losses of chromosome 13 were detected in 19 of the 27 tumors with a putative common region of loss in bands 13q14-21. Losses of chromosomes 1q, 2p and 2q, 4q, 9p, 10p and 10q, 11p and 11q23, and 16q were also highly recurrent. A comparative analysis between the most frequent genomic imbalances observed in this study of LMS and the genomic imbalances observed in a large proportion of malignant fibrous histiocytomas (MFH) from a previous study demonstrated that both types of tumors had similar recurrent imbalances.

Although MFH were once thought to be a separate member of the soft tissue sarcoma family, our observations support the hypothesis that MFH are a morphologic modulation in the tumoral progression of other sarcomas, particularly LMS.

H-, K-, and N-ras gene mutation in atypical fibroxanthoma and malignant fibrous histiocytoma

Akio Sakamoto, MD
Yoshinao Oda, MD
Eijun Itakura, MD
Yumi Oshiro, MD
Sadafumi Tamiya, MD
Yumi Honda, MD
Akira Ishihara, MD
Yukihide Iwamoto, MD
Masazumi Tsuneyoshi, MD

Hum Pathol 2001;32:1125-1231. Abstract quote

Atypical fibroxanthoma (AFX) which is histologically similar to malignant fibrous histiocytoma (MFH), occurs in the sun-exposed skin.

The presence of mutations at codons 12 and 13 of the H- and K-ras genes and in exons 1 and 2, which include codons 12, 13, and 61, of the N-ras gene was studied in 8 cases of AFX and 8 cases of storiform-pleomorphic–type MFH using polymerase chain reaction (PCR)–restriction fragment length polymorphism and PCR–single-conformation polymorphism. Two of the 8 cases of MFH showed ras mutations in the H-ras gene at codon 12 (GGC-AGC) and in the K-ras gene at codon 13 (GGC-GAC). H- and K-ras gene mutations were not seen in any of the cases of AFX (0 of 8). N-ras gene mutation was not detected in either the AFX (0 of 8) or MFH (0 of 8) cases.

In conclusion, although the number of cases in this study was small, H- and K-ras genes were present in some of the MFH cases and accordingly may play an important role in the pathogenesis of MFH. In addition, the finding that H-, K-, and N-ras gene mutations are not present in AFX may indicate why AFX has a more favorable behavior than MFH.


Immunoexpression of Ultraviolet Photoproducts and p53 Mutation Analysis in Atypical Fibroxanthoma and Superficial Malignant Fibrous Histiocytoma

Akio Sakamoto, M.D., Yoshinao Oda, M.D., Eijun Itakura, M.D., Yumi Oshiro, M.D., Osamu Nikaido, M.D., Yukihide Iwamoto, M.D. and Masazumi Tsuneyoshi, M.D.

Department of Anatomic Pathology, Pathological Sciences (AS, YoO, EI, MT), and Department of Orthopaedic Surgery (YI), Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan; Department of Pathology, Matsuyama Red Cross Hospital (YuO), Matsuyama, Japan; and Division of Radiation Biology, Faculty of Pharmaceutical Sciences (ON), Kanazawa University, Ishikawa, Japan

Mod Pathol 2001;14:581-588 Abstract quote

p53 mutation is one of the major results of ultraviolet (UV) radiation. UV photoproducts of cyclobutane pyrimidine dimers (CPDs) and pyrimidine-pyrimidone (6–4) photoproducts (64PPs) also play an important role in skin cancer development. Atypical fibroxanthoma (AFX), which mimics malignant fibrous histiocytoma (MFH) histologically, occurs in the sun-exposed skin of the elderly, and therefore, an association with UV has long been suspected.

Eighteen fibrohistiocytic skin lesions comprising AFX (n = 7), storiform-pleomorphic type MFH centered in the subcutis (superficial MFH; S-MFH; n = 4) and benign fibrous histiocytoma (BFH; n = 7) were used for immunohistochemical and molecular analysis. Eight cases of deep MFH (D-MFH) were also analyzed for UV photoproduct expression for the purposes of comparison.

Immunohistochemically, the CPD scores of AFX (3.6 ± 0.4) were significantly higher than those of S-MFH (1.3 ± 0.8), D-MFH (0.8 ± 0.5), or BHF (1.4 ± 0.7); however, the 64PP scores were extremely low in all these tumors (AFX, 0.1 ± 0.1; S-MFH, 0.0 ± 0.0; D-MFH, 0.0 ± 0.0; and BHF, 0.0 ± 0.0). AFX, S-MFH, and BFH showed immunoexpression for p53 (2/7, 2/4, and 0/7), respectively. p53 mutations were detected in AFX (4/6; 67%) and S-MFH (1/4; 25%), but not in BFH (0/5; 0%) using polymerase chain reaction–single-strand conformation polymorphism, and all of the mutations in AFX were either C-T transitions or at dipyrimidine sites.

In conclusion, AFX and S-MFH are both similar fibrohistocytic lesions; however, AFX has high immunoreactivity for CPDs compared with S-MFH, D-MFH, or BFH. These data suggest that CPDs may play an important role in the pathogenesis of AFX.



CT scan and MRI  

MRI of malignant fibrous histiocytoma of soft tissue: analysis of 13 cases with pathologic correlation.

Miller TT, Hermann G, Abdelwahab IF, Klein MJ, Kenan S, Lewis MM.

Department of Radiology, Mount Sinai Medical Center of the City University New York, New York.

Skeletal Radiol 1994 May;23(4):271-5 Abstract quote

We reviewed the magnetic resonance (MR) appearances of 13 malignant fibrous histiocytomas (MFH) of soft tissue and correlated each with the respective lesion's histopathology. The MR images were evaluated for signal intensity on T1- and T2-weighted spin echo sequences, homogeneity of the lesion, presence of internal low signal septations, and margin definition. Histologic subtypes of MFH included storiform-pleomorphic, giant cell, myxoid, and inflammatory.

We could not establish a correlation between MR appearance and histopathology. Instead, our series exhibited general features suggestive of malignant soft tissue neoplasms, namely poor margin definition, internal low signal septation, and heterogeneous high signal intensity on T2-weighted images.

Laboratory Markers  



Malignant fibrous histiocytoma of bone: a retrospective EMSOS study of 125 cases. European Musculo-Skeletal Oncology Society.

Bielack SS, Schroeders A, Fuchs N, Bacci G, Bauer HC, Mapeli S, Tomeno B, Winkler K.

Abteilung fur padiatrische Hamatologie und Onkologie, Universitats-Klinderklinik Hamburg-Eppendorf, Germany.

Acta Orthop Scand 1999 Aug;70(4):353-60 Abstract quote

In an effort to learn more about malignant fibrous histiocytoma (MFH) of bone and its prognosis with different treatment approaches, the European Musculo-Skeletal Oncology Society (EMSOS) initiated a retrospective survey among its members. Data requested included patient and treatment variables and outcome.

The information on all patients with histologically proven, primary, localized osseous extremity MFH was analyzed if surgical tumor removal was performed and disease status was documented for at least one follow-up date. 125 such patients were evaluable (74 male, 51 female; median age 34 years; tumor site femur 81, tibia 26, humerus 12, other 6). Local treatment was surgery only (110) or surgery plus radiotherapy (15). Chemotherapy was used in 97/125. On last follow-up, 85 patients remained in remission, 33 had developed metastases, 6 a local recurrence, and 1 a combined relapse. With a median follow-up of 3.9 years for patients at risk, actuarial 5-year disease-free survival was 59%.

In univariate analyses, younger age and the use of chemotherapy were associated with a more favorable outcome, as was limb-salvage surgery. 23 of 66 tumors with information on response to preoperative chemotherapy responded well (> 90% necrosis). Among these 23, only one relapsed.


Malignant fibrous histiocytoma in children.

Corpron CA, Black CT, Raney RB, Pollock RE, Lally KP, Andrassy RJ.

Department of Surgical Oncology, University of Texas M.D. Anderson Cancer Center, Houston, USA.

J Pediatr Surg 1996 Aug;31(8):1080-3 Abstract quote

Because malignant fibrous histiocytoma (MFH) rarely occurs in children, the natural history of this tumor and prognostic factors predictive of outcome have not been well described. The charts of all pediatric patients with MFH seen at M.D. Anderson Cancer Center were reviewed with respect presentation, treatment, and outcome, in an attempt to determine prognostic factors that are predictive of survival.

Forty-four pediatric patients were identified. Extremities were the most common tumor site (31 of 44 patients). Five patients presented with angiomatoid histology subtype; all subsequently survived. The estimated 5-year survival rate was 85% for clinical group I patients, 87% for clinical group II, 53% for clinical group III, and 0% for clinical group IV. The estimated 5-year survival rate was 95% for patients with tumors of less than 5 cm in diameter and 45% for those with larger tumors. Overall, the estimated 5-year survival rate was 71%.

Significant prognostic factors found to affect survival (by univariate analysis) were clinical group, tumor size, and recurrence. Gender and race were not significant predictors. The use of chemotherapy and radiation was not found to improve the chance of survival, but this most likely reflected the more frequent use of adjuvant therapy in patients with unresectable or high-grade tumors. Although adequate surgical resection continues to be the most effective treatment, investigation of adjuvant chemotherapy and radiation therapy on protocol is warranted.


Pediatric cutaneous malignant fibrous histiocytoma.

Rothman AE, Lowitt MH, Pfau RG.

Department of Dermatology, University of Maryland School of Medicine, Baltimore 21201, USA.

J Am Acad Dermatol 2000 Feb;42(2 Pt 2):371-3 Abstract quote

Malignant fibrous histiocytoma (MFH) is an aggressive soft-tissue sarcoma that most commonly occurs in the skeletal muscle of the extremities or retroperitoneum of adults. Although the majority of MFH is located beneath the fascia, the tumor occasionally occurs in the subcutaneous tissue. MFH rarely occurs in children and the disease course, prognosis, and outcome in younger patients has not been well described. We report a case of cutaneous MFH presenting on the thigh of a 12-year-old boy.


Malignant Fibrous Histiocytoma of the Spermatic Cord: Report of Two Cases and Review of the Literature

Bryan Tzy-Young Lin, M.D., Ph.D., Dean A. Harvey, M.D. and L. Jeffrey Medeiros, M.D.

Department of Surgical Pathology (BT-YL), Tarzana Regional Medical Center, Tarzana, California; Department of Pathology (DAH), Santa Monica–UCLA Medical Center, Santa Monica, California; and Division of Pathology and Laboratory Medicine (LJM), University of Texas MD Anderson Cancer Center, Houston, Texas

Mod Pathol 2002;15:59-65 Abstract quote

Malignant fibrous histiocytoma (MFH) of the spermatic cord is rare, and most published cases are single case reports that emphasize clinical presentation and management.

We describe in detail the histopathologic features of 2 cases of high-grade storiform-pleomorphic MFH arising in the spermatic cord. Both tumors occurred in elderly men, 65 years and 70 years, and were 4 cm (Case 1) and 5 cm (Case 2) in greatest dimension. The tumor mass in Case 1 was associated with satellite tumor nodules. At last follow-up, in Case 1 the patient died of metastasis, and in Case 2, the patient is alive and well 46 months after diagnosis. Review of the literature reveals 33 additional cases published in English (17 cases) or Japanese (16 cases) that include histologic description. Including the 2 cases in this report, most of the tumors occurred in older (than 50 years) patients (28 of 35 cases, 80%) and occurred as solitary masses that ranged in diameter from less than 1 cm to more than 20 cm. Nine patients presented with satellite tumor nodules. Twenty-nine (83%) tumors were of the storiform-pleomorphic type, with 3 giant cell type, 2 inflammatory type, and 1 myxoid type. These features do not differ significantly from MFH in other anatomic sites. Clinical follow-up is available in 33 cases (3–174 months; mean, 31.5 months). Twelve patients developed recurrence and metastasis; at least 4 patients died of the disease.

Tumor size does not predict the clinical progression; however, patients with progressive tumors were commonly associated with satellite nodules at time of presentation, an indication of early local metastasis.


Malignant Fibrous Histiocytoma of the Skin With Marked Inflammatory Infiltrate:: A Sarcoma Mimicking Malignant Lymphoma

Carlos Diaz-Cascajo, M.D.; Ronald Bernd, M.D.; Maria Teresa, M.D.; Fernandez-Figueras, M.D.; Susanna Borghi, M.D.
Am J Dermatopathol 2002; 24(3):251-256 Abstract quote

Three cases of malignant fibrous histiocytoma of the skin with a marked inflammatory infiltrate in the stroma are reported. The inflammatory infiltrate, composed mainly of T-lymphocytes, obscured the nature of the neoplasms, and immunohistochemical studies were required to establish the diagnosis.
Two tumors arose in the sun-damaged skin of the face, and one tumor arose in the chest wall. One patient developed a local recurrence with histopathologic findings similar to those observed in the original lesion, including the inflammatory infiltrate.

Possible differential diagnoses include large cell lymphoma, inflammatory pseudotumor, inflammatory leiomyosarcoma, and spindle cell squamous cell carcinoma. The presumed rarity of inflammatory changes in malignant fibrous histiocytoma of the skin is supported by the absence of reported cases.

Urachal malignant fibrous histiocytoma: a case report and review of the literature.

Wang BY, Boag AH, Idrees M, Young ID, Unger PD.

Department of Pathology, The Mount Sinai School of Medicine, New York, NY 10029, USA
Arch Pathol Lab Med. 2004 Apr;128(4):456-9. Abstract quote

Pathologic processes involving the urachus are usually related to inflammatory or sinofistular conditions. Neoplasms rarely arise within this structure, and when they do occur, they are typically epithelial, with mucinous adenocarcinoma being the most common. Mesenchymal lesions, both benign and malignant, have rarely been described in this location.

We report the case of a 66-year-old white man who presented with a primary urachal malignant fibrous histiocytoma and died of metastatic disease 20 months after the initial diagnosis. This is an unusual case of malignant fibrous histiocytoma arising in a urachal remnant.



Most malignant fibrous histiocytomas developed in the retroperitoneum are dedifferentiated liposarcomas: a review of 25 cases initially diagnosed as malignant fibrous histiocytoma.

Coindre JM, Mariani O, Chibon F, Mairal A, De Saint Aubain Somerhausen N, Favre-Guillevin E, Bui NB, Stoeckle E, Hostein I, Aurias A.

Department of Pathology (JMC, IH), Medical Oncology (EFG, NBB) and Surgery (ES), Institut Bergonie, Bordeaux, France.


Mod Pathol 2003 Mar;16(3):256-62 Abstract quote

Forty-four samples from 25 cases of retroperitoneal sarcoma initially diagnosed as malignant fibrous histiocytoma were histologically reviewed. Immunohistochemistry for mdm2 and cdk4 was performed on 20 cases. Comparative genomic hybridization was performed on 18 samples from 13 patients.

Seventeen cases were reclassified as dedifferentiated liposarcoma. Twenty-one of 32 samples from these patients showed areas of well-differentiated liposarcoma, allowing the diagnosis of dedifferentiated liposarcoma. Immunohistochemistry performed in 15 of these cases showed positivity for mdm2 and cdk4. Comparative genomic hybridization analysis performed on 15 samples from 11 of these patients showed an amplification of the 12q13-15 region.

Eight cases were reclassified as poorly differentiated sarcoma. Twelve samples from these patients showed no area of well-differentiated liposarcoma. Immunohistochemistry showed positivity for mdm2 and cdk4 in one of six of these patients and showed positivity for CD34 in another one. Comparative genomic hybridization analysis performed on three samples from two of these patients showed no amplification of the 12q13-15 region but showed complex profiles.

This study shows that most so-called malignant fibrous histiocytomas developed in the retroperitoneum are dedifferentiated liposarcoma and that a poorly differentiated sarcoma in this area should prompt extensive sampling to demonstrate a well-differentiated liposarcoma component, immunohistochemistry for mdm2 and cdk4, and if possible, a cytogenetic or a molecular biology analysis.

Pleomorphic malignant fibrous histiocytoma: fact or fiction? A critical reappraisal based on 159 tumors diagnosed as pleomorphic sarcoma.

Fletcher CD.

Department of Histopathology, St. Thomas's Hospital (U.M.D.S.), London, England.

Am J Surg Pathol 1992 Mar;16(3):213-28 Abstract quote

Pleomorphic malignant fibrous histiocytoma (MFH) is regarded as the most common soft tissue sarcoma of adulthood, but no definable criteria exist for its diagnosis. Possibly its only distinctive feature is its apparent lack of specific differentiation.

To determine the validity of pleomorphic MFH, 159 tumors diagnosed as pleomorphic sarcomas have been reassessed morphologically, immunohistochemically, and ultrastructurally, where possible.

Of these 97 cases (63%) proved to be specific sarcomas other than MFH, 20 proved to be nonmesenchymal neoplasms, and 42 were unclassifiable (of which 21 were either small biopsies or subtotally necrotic). Only 13% of these cases were eligible for consideration as MFH, but these showed no reproducible histological differences from the other tumors studied, nor was this group morphologically consistent. These tumors showed no evidence of true monocyte/macrophage differentiation. It is postulated that pleomorphic MFH is a noncohesive heterogeneous group of poorly differentiated neoplasms, a term that has become a meaningless diagnosis of convenience.

With sufficient effort, a specific line of differentiation can be identified in the majority of pleomorphic malignant soft tissue tumors; with advances in investigative technology, the proportion that remain unclassifiable is very likely to diminish further in the future.

Malignant fibrous histiocytoma: morphologic pattern or pathologic entity?

Hollowood K, Fletcher CD.

Department of Histopathology, St Thomas's Hospital (U.M.D.S.), London, England.

Semin Diagn Pathol 1995 Aug;12(3):210-20 Abstract quote

Since the concept of malignant fibrous histiocytoma (MFH) was introduced and subsequently popularized in the 1960's and 1970's, it has become widely regarded as the commonest soft-tissue sarcoma of adulthood. Although the initial notion that MFH was a true histiocytic tumor showing faculative fibroblastic differentiation has been disproved, and despite the lack of definable, reproducible diagnostic criteria and considerable immunophenotypic, ultrastructural and karyotypic heterogeneity, MFH is still accepted widely as a discrete clinicopathologic entity. On the other hand several recent studies have expressed considerable doubts about MFH, or at least pleomorphic MFH, as an "entity" and have suggested that it represents a common morphologic manifestation of a host of poorly differentiated sarcomas and, more rarely, other neoplasms.

This article reviews the clinicopathologic features of MFH and its established variants in the context of this debate and considers the evidence for and against their continued acceptance as distinct entities or as a cohesive group.

We conclude that the pleomorphic, giant cell and inflammatory variants each represent heterogeneous diagnostic groups which are hard to defend as cohesive entities, while the myxoid ("myxofibrosarcoma") and angiomatoid types are distinct, reproducible tumor types.

Giant cell

Retroperitoneal liposarcoma with combined well-differentiated and myxoid malignant fibrous histiocytoma-like myxoid areas.

Hisaoka M, Morimitsu Y, Hashimoto H, Ishida T, Mukai H, Satoh H, Motoi T, Machinami R.

Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.

Am J Surg Pathol 1999 Dec;23(12):1480-92 Abstract quote

To broaden the knowledge of myxoid morphology in liposarcoma, eight cases of unusual liposarcoma with combined well-differentiated and myxoid malignant fibrous histiocytoma (MFH)-like myxoid areas are reported.

The tumors arose as huge retroperitoneal masses in elderly patients, except for one that occurred in the spermatic cord. Three cases had local recurrences, and one of the seven patients who were followed up had died of the tumor.

Grossly, the tumors were mostly confluent and multinodular and showed a glistening myxoid appearance in variable proportions, which merged gradually into or were juxtaposed to yellow fatty or sclerotic whitish areas.

Microscopically, in addition to areas of well-differentiated lipoma-like or sclerosing liposarcoma, all the tumors contained myxoid portions characterized by scattered multinucleated or bizarre giant cells and a prominent plexiform vascular pattern that resembled myxoid MFH or myxofibrosarcoma. The myxoid areas were associated with discernible lipogenesis. High-grade dedifferentiation was present in one tumor.

Cytogenetically, in one case, the myxoid lesion had nonrandom chromosomal aberrations, such as ring and marker chromosomes, characteristic of a well-differentiated variant of liposarcoma. In a nested reverse transcription-polymerase chain reaction analysis using archival paraffin-embedded tissue, it was seen that none of the eight tumors with myxoid MFH-like features had TLS/FUS-CHOP fusion transcripts characteristic of myxoid and round cell liposarcomas.

These clinicopathologic and molecular features suggest that the current myxoid tumors are more closely related to well-differentiated liposarcoma rather than to ordinary myxoid liposarcoma despite their unequivocal myxoid morphology. Missense point mutations of the p53 gene were detected in two (25%) cases by single-strand conformation polymorphism and sequence analyses. Immunohistochemical expressions of p53 and mdm2 were observed in 75% of the cases, in which immunoreactive tumor cells were seen more often in the myxoid MFH-like areas.

Thus, altered p53 pathways, such as p53 gene mutation and mdm2-mediated inactivation of p53, may play a pathogenetic role in this form of tumor progression showing myxoid MFH-like morphology in liposarcoma, as has been suggested in dedifferentiated liposarcoma.


Infiltrative subcutaneous malignant fibrous histiocytoma: a comparative study with deep malignant fibrous histiocytoma and an observation of biologic behavior.

Fanburg-Smith JC, Spiro IJ, Katapuram SV, Mankin HJ, Rosenberg AE.

Soft Tissue Pathology Department, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.

Ann Diagn Pathol 1999 Feb;3(1):1-10 Abstract quote

Malignant fibrous histiocytoma (MFH) is one of the most common soft tissue sarcomas of adulthood. Although it is usually intramuscular and pseudocapsulated, we have recently observed MFHs with extremely infiltrative growth margins, which are predominantly located in the subcutis. These lesions are often associated with incomplete primary surgical excision, the subsequent need for additional surgery or adjuvant therapy, and an increased risk for local recurrence.

To further analyze the growth pattern and clinical implication of the subcutaneous infiltrative MFHs, we reviewed a series of 24 subcutis and 21 intramuscular MFHs of the extremities. Morphologically, we defined "infiltrative" as tumor extension along normal tissue planes for a minimum measurable distance of 2 mm from the edge of the main mass. Radiographic findings were correlated with pathologic findings. Of the 24 subcutis MFHs, 83% showed an infiltrative growth pattern, involving 5% to 90% (mean, 51%) of the evaluable margin. Fifty percent of patients with subcutaneous MFH had both an infiltrative growth pattern and positive surgical resection margin on initial resection. Only 25% noninfiltrative subcutaneous MFHs had a positive initial surgical resection margin. Of the 21 intramuscular MFHs, only 5 (24%) had an infiltrative growth pattern that involved 5% to 90% (mean, 40%) of the evaluable margin. One of the five tumors had 90% margin infiltration with multiple positive surgical resection margins. Of 16 noninfiltrative intramuscular MFHs, 3 (19%) had positive resection margins. Magnetic resonance imaging (MRI) and/or computed tomography (CT) scan correctly identified the growth pattern in 87% of subcutaneous and 88% of intramuscular MFHs.

Patient follow-up evaluation showed that four (17%) patients with subcutaneous MFHs had resection-proven recurrences, 6 to 57 months after diagnosis. All four of these tumors had infiltrative growth patterns and positive margins on the original surgical resection. There were no local recurrences of the intramuscular MFHs. Two patients of 20 in the infiltrative subcutaneous MFH group and two patients of four in the well-circumscribed subcutaneous MFH group had biopsy-proven metastases, which developed within 5 years after diagnosis. Six patients had metastases in the intramuscular MFH group. A group of MFHs, predominantly subcutaneous, have an extremely infiltrative growth pattern.

This growth pattern, documented by radiographic methods and/or light microscopic examination of biopsy specimens, should indicate that a wider margin on initial resection is necessary to entirely excise the lesion. The presence or absence of an infiltrative growth pattern is not predictive of the tumor's metastatic potential.



Myofibroblastic differentiation in malignant fibrous histiocytoma (pleomorphic myofibrosarcoma): a clinicopathological study.

Montgomery E, Fisher C.

The Royal Marsden NHS Trust, London, UK.

Histopathology 2001 Jun;38(6):499-509 Abstract quote

We compared the clinical and pathological features of pleomorphic malignant fibrous histiocytoma (MFH)-like soft tissue sarcomas with and without myofibroblastic differentiation on electron microscopy.

METHODS AND RESULTS: Fifty-three soft tissue tumours designated as MFH by light and electron microscopy were reassessed. Eighteen were specifically diagnosed and excluded, and follow-up (FU) information obtained for 24 of the other 35 cases. Myofibroblastic ultrastructure was seen in 7/24 (29%). Seventeen of 24 (71%) lacked myofibroblasts on electron microscopy, which showed fibroblastic or undifferentiated cells. Histologically, all tumours but one had storiform-pleomorphic areas; one myofibroblastic neoplasm was fascicular and myxoid. No other morphological differences were seen. In seven myofibroblastic cases, smooth muscle in four cases and muscle-specific actin in two cases, desmin in three cases and S100 in one case were present. In 15 other tumours, smooth muscle in five cases and muscle-specific actin in one case, and desmin in one case were present; none of these cases expressed S100. CD34 was found in the myxoid areas of one myofibrosarcoma and 3/15 other tumours. Positivity for bcl-2 was seen only in non-myofibroblastic sarcomas (4/14). On follow-up (median 41 months), 2/7 (29%) myofibroblastic tumours recurred, 5/7 (71%) metastasized, and 3/7 (43%) patients died of disease. Among the non-myofibroblastic sarcomas, with a median follow-up of 47 months, 6/17 cases (35%) recurred, 10/17 (59%) metastasized, and 7/17 patients (41%) died of disease.

CONCLUSIONS: Pleomorphic sarcomas with and without myofibroblastic differentiation on electron microscopy are clinically and histologically similar. The former display myoid immunohistochemical markers more frequently.

Electron microscopy (EM)  

Malignant fibrous histiocytoma: an ultrastructural perspective.

Suh CH, Ordonez NG, Mackay B.

University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.

Ultrastruct Pathol 2000 Jul-Aug;24(4):243-50 Abstract quote

Malignant fibrous histiocytoma is a frequent diagnosis, but the relationship of the tumors to histologically similar soft tissue neoplasms is controversial.

In this study, 157 examples representing the 4 main subtypes were reviewed by light microscopy and each tumor was studied with the electron microscope. Immunohistochemical stains were performed on 77 tumors. Electron micrographs on 100 fibrosarcomas were reviewed for comparison. Malignant fibrous histiocytomas often closely resemble fibrosarcomas at the ultrastructural level and differences between the two are generally of degree only.

Evidence for true histiocytic differentiation was not found. The immunohistochemical results did not contradict the authors' impression from electron microscopy that malignant fibrous histiocytoma forms part of the histologic spectrum of tumors of fibroblasts.


Poorly differentiated tumors

Sarcoma, melanoma, lymphoma, carcinoma

Immunoperoxidase or electron microscopy may help to delineate the diagnosis

Atypical fibroxanthoma versus benign and malignant fibrous histiocytoma. A comparative study of their proliferative activity using MIB-1, DNA flow cytometry, and p53 immunostaining.

Oshiro Y, Fukuda T, Tsuneyoshi M.

Second Department of Pathology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.

Cancer 1995 Mar 1;75(5):1128-34 Abstract quote

BACKGROUND. MIB-1 was found to be detectable in formalin fixed, paraffin embedded materials with microwave treatment using Ki-67 monoclonal antibody, and immunostaining has been widely documented as a useful marker of proliferation. Because atypical fibroxanthoma (AFX) is regarded as a fibrohistiocytic tumor with an intermediate potential, the proliferative activity of AFX was compared with that of benign and malignant fibrous histiocytomas.

METHODS. Thirty-eight soft tissue tumors including atypical fibroxanthoma (n = 5), benign fibrous histiocytoma (FH) (n = 17) and malignant fibrous histiocytoma (MFH) (n = 16) were examined using immunohistochemistry to determine MIB-1, DNA flow cytometry, and p53 (PAb 1801) immunostaining.

RESULTS. The mean of the MIB-1 labeling index (MIB-1 LI), defined as the percentage of positive cells for more than 500 cells, was determined in an increasing order as follows: FH, 3.3 +/- 1.8; AFX, 12.2 +/- 6.3; and MFH, 21.5 +/- 10.2. However, the MIB-1 LI of each case in AFX was considerably scattered, and the MIB-1 LI of AFX and MFH overlapped each other. A DNA analysis revealed that the proliferative index (S+G2+M fraction) showed no significant correlation with the MIB-1 LI, and an aneuploid pattern was present in only five (42%) of 12 cases of MFH. p53 positivity was detected in 2 (40%) of 5 cases of AFX and 6 (38%) of 16 cases of MFH.

CONCLUSIONS. Although AFX shows a lower degree in the MIB-1 LI than MFH, the MIB-1 LI shows a limited value in relation to the biologic activity of fibrohistiocytic tumors. Aneuploidy demonstrates a malignant potential in fibrohistiocytic tumors.



Prognostic factors in 227 patients with malignant fibrous histiocytoma.

Pezzi CM, Rawlings MS Jr, Esgro JJ, Pollock RE, Romsdahl MM.

Department of General Surgery, University of Texas M. D. Anderson Cancer Center, Houston 77030.

Cancer 1992 Apr 15;69(8):2098-103 Abstract quote

alignant fibrous histiocytoma, the major subset of soft tissue sarcomas, was examined for prognostic factors that could influence clinical management and research.

Two hundred twenty-seven patients with localized disease, having surgery as the principal modality, were reviewed retrospectively to identify clinical outcomes. The mean age of the patients was 54 years. Extremities were the primary tumor site in 157 patients (62.2%). Overall survival rate was 50%, including 38 patients who died of other causes. Distant metastases were most common to the lung (90%). Local recurrence alone occurred in 37 patients (16%), distant metastases alone in 52 (23%), and distant metastases with local recurrence in 25 (11%).

The primary tumor size indicated the 5-year survival rate: tumors smaller than 5 cm had a survival rate of 82%; 5 to 10 cm, 68%; and larger than 10 cm, 51%. Intermediate-grade tumors yielded a 5-year survival rate of 80%, and the 5-year survival rate for high-grade tumors was 60%. Survival rates for both grades were affected by size: tumors of high grade and smaller than 5 cm in diameter had a survival rate of 79%; 5 to 10 cm, 63%; and more than 10 cm, 41%. Grade and size emerge as significant prognostic indicators. These variables will prove helpful in treatment decisions and design of clinical studies.

Malignant fibrous histiocytoma: outcome and prognostic factors following conservation surgery and radiotherapy.

Zagars GK, Mullen JR, Pollack A.

Department of Radiotherapy, The University of Texas, M.D. Anderson Cancer Center, Houston, USA.

Int J Radiat Oncol Biol Phys 1996 Mar 15;34(5):983-94 Abstract quote

PURPOSE: Malignant fibrous histiocytoma is the most common type of soft tissue sarcoma. This communication presents an analysis of outcome and prognostic factors based on a retrospective review of patients with this disease treated by conservation surgery and radiotherapy.

METHODS AND MATERIALS: From 1966 to 1991, 271 consecutive patients with malignant fibrous histiocytoma were treated with conservation surgery and radiotherapy. The outcome with local control, metastatic relapse, and survival as end points was evaluated by univariate and multivariate statistics to delineate independently significant prognostic factors.

RESULTS: Postoperative radiation at a mean dose of 62.8 Gy was used in 195 patients and preoperative radiation at a mean dose of 50 Gy was used in 76 patients. At a median follow-up of 7.3 years, 123 patients (45%) developed disease relapse at some site. Fifty-seven (21%) developed local recurrence leading to an actuarial local relapse rate of 26% at 10 years, 83 (31%) developed metastatic relapse for a 10-year actuarial metastatic rate of 33%, and the 5-, 10-, and 15-year survival rates were 68, 60, and 46%, respectively. For local control, prior local recurrence (in 53 patients) was identified as an adverse factor, yielding a 10-year recurrence rate of 42% compared to 22% for 218 patients without prior disease (p < 0.01). Also, a positive surgical margin (in 46 patients) was adverse with a 10-year local recurrence rate of 39% compared to a recurrence rate of 17% with negative margins (167) (p=0.01). Patients with pathologically undocumented resection margins (58) had a local recurrence rate similar to those with positive margins (41% at 10 years). Tumor site (extremity vs. nonextremity), location (proximal vs. distal), size (< or = 5 cm vs. > 5 cm), and histology (myxoid vs. nonmyxoid) were not significant determinants of local outcome. For metastatic relapse, the major determinants of outcome were histology (myxoid vs. nonmyxoid) and tumor size. Myxoid tumors (59 patients) had a low metastatic propensity (13% 10-year metastatic rate) compared to nonmyxoid tumors (212 patients) (40% 10-year metastatic rate) (p < 0.01). Size was an important covariate for metastases for both myxoid and nonmyxoid tumors. For nonmyxoid tumors the 10-year metastatic rates were 23 and 51% for lesions less than or greater than 5 cm. For myxoid tumors a significant metastatic rate appeared only for tumors exceeding 10 cm (10-year metastatic rates of 8% vs. 44% for tumors less than vs. greater than 10 cm). In this retrospective review we found no evidence that adjuvant chemotherapy decreased the metastatic rate. In multivariate analysis for metastatic relapse and survival, tumor histology (nonmyxoid vs. myxoid) and size (< 5 cm vs. > 5 cm) were the only independent determinants of outcome.

CONCLUSION: Malignant fibrous histiocytoma is a heterogeneous disease and its myxoid variant must be recognized as a distinct entity. Both variants are locally aggressive and require equally aggressive local therapy. Conservation surgery striving for negative margins with radiation therapy provides acceptable local control and is the treatment of choice for this disease. Patients with myxoid tumors do not require systemic therapy; patients with nonmyxoid disease exceeding 5 cm are at significant risk for metastases and the development of effective adjuvant treatment is an important research tool.

Prognostic factors for patients with localized primary malignant fibrous histiocytoma: a multicenter study of 216 patients with multivariate analysis.

Le Doussal V, Coindre JM, Leroux A, Hacene K, Terrier P, Bui NB, Bonichon F, Collin F, Mandard AM, Contesso G.

French Federation of Cancer Centers (FNCLCC) Sarcoma Group, Paris, France.

Cancer 1996 May 1;77(9):1823-30 Abstract quote

BACKGROUND: The purpose of this study was to determine the independent prognostic variables in a well documented subset of 216 patients with localized primary malignant fibrous histiocytomas (MFH).

METHODS: Between the years 1980 and 1989, 216 patients with localized, primary (International Union Against Cancer [UICC]/American Joint Committee on Cancer [AJCC] Stage I-IVA) MFH were evaluated and treated in 10 participating centers of the sarcoma group of the French Federation of Cancer Centers (FNCLCC). Clinicopathologic factors were collected retrospectively and entered into a cooperative database. Tissue slides of all cases were jointly reviewed microscopically by the pathology subcommittee. Surgical treatment was performed on all but 6 (3%) patients. One hundred ninety-five patients (90%) were free of gross disease, with complete local control at the end of the initial treatment. The adjuvant treatment was radiotherapy in 78 patients (36%), chemotherapy in 19 patients (9%), and both in 61 patients (28%).

RESULTS: The median follow-up was 3.5 years (range, 45 days to 12 years). Five-year actuarial rates of disease specific (DSS), metastasis free (MFS), and local recurrence free (LRFS) survival were 70%, 63.3%, and 62.7%, respectively. Multivariate analyses showed that the adverse prognostic factors independently associated with decreased disease specific survival were UICC/AJC Stage III + IVA (P < 0.00001; relative risk [RR], 3.27; 95% confidence interval [CI], 1.6-6.58), residual macroscopic disease following primary local therapy (P = 0.00024; RR, 3.99, CI, 2.04-7.82), deep tumor location (P = 0.0045; RR, 3.37; CI, 1.21-9.38), non-myxoid histology (P = 0.0056; RR, 9.28; CI, 1.03-83.41), and age older than 50 years (P = 0.037; RR, 2.19; CI, 1.04-4.61). Two factors were significantly related to MFS in the patients with the poorest prognosis: histopathologic Grade 3 (P < 0.0001, RR, 3.46; CI, 2.02-5.91) and tumor size greater than 8 cm in largest dimension (P = 0.0012; RR, 2.78; CI, 1.36-3.66). With regard to LRFS, patients who did not undergo radiotherapy had reduced local control (P = 0.0043; RR, 2.36; CI, 1.46-3.83).

CONCLUSIONS: Resection of all macroscopic disease was independently associated with improved disease specific survival and adjuvant radiotherapy significantly decreased the local relapse risk. Histopathologic grade was the most important prognostic factor for DSS and MFS.

Clinicopathologic re-evaluation of 100 malignant fibrous histiocytomas: prognostic relevance of subclassification.

Fletcher CD, Gustafson P, Rydholm A, Willen H, Akerman M.

Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.

J Clin Oncol 2001 Jun 15;19(12):3045-50 Abstract quote

PURPOSE: Malignant fibrous histiocytoma (MFH) has been regarded as the most common soft tissue sarcoma (STS) in adults. Yet its true nature and the validity of this diagnostic concept have increasingly been questioned. Available data suggest that most patients with MFH can be subclassified into specific STS types, but the clinical relevance of such categorization has been argued. In a retrospective study, we reclassified 100 tumors of the extremity and trunk wall primarily diagnosed as MFH and analyzed the outcome.

PATIENTS AND METHODS: Patients were adults (median age, 70 years; range, 32 to 94 years). The median tumor size was 8 cm (range, 1 to 30 cm), and the thigh was the most common tumor location (n = 31). Median follow-up was 8 years (range, 3 to 16 years). The overall 5-year metastasis-free survival rate was 0.64. The tumors were reanalyzed histologically, immunohistochemically, and, where available, ultrastructurally, and were classified according to strict diagnostic criteria. Patients were staged according to the American Joint Committee on Cancer system, and prognoses were compared among different groups of the reclassified diagnoses, paying special attention to myogenic tumors.

RESULTS: In 84 of 100 tumors, a specific line of differentiation was either proved or strongly suggested. The most common diagnoses were myxofibrosarcoma (n = 22) and leiomyosarcoma (n = 20). Overall, 30 tumors could be grouped as some form of myogenic sarcoma. These tumors had a worse prognosis, even within the same American Joint Committee on Cancer stage, and a shorter time to metastasis than nonmyogenic tumors.

CONCLUSION: This retrospective study confirms that most so-called MFH can be subclassified by defined criteria; it provides evidence that such classification is clinically important. Specifically, pleomorphic STS showing myogenic differentiation are significantly more aggressive, a finding that allows planning future therapeutic trials.

Discriminant analysis of prognostic factors for malignant fibrous histiocytoma in soft tissue.

Shinozaki T, Kato K, Watanabe H, Yanagawa T, Ahmed AR, Takagishi K.

Department of Orthopedic Surgery, Gunma University Faculty of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan.

J Orthop Sci 2001;6(4):339-42 Abstracy quote

We prospectively followed 32 patients with soft-tissue malignant fibrous histiocytoma (MFH).

Parameters were age; sex; tumor size, location, and depth; operative method; chemotherapy; radiotherapy; and histology. Patients with recurrence or metastases due to MFH within 6 months after the initial operation were separated from those without these characteristics by discriminant analysis with statistically significant difference. The order of influential functions was histology, depth of tumor, operative method, and sex. Male patients with deep-seated storiform-pleomorphic type MFH, receiving less comprehensive surgery, had the greatest risk of local recurrence or early metastases. We have to pay particular attention to patients with these factors and perform adequate surgery, because local recurrence and metastases were found to be closely related, and to have a great influence on the prognosis of this disease.

Discriminant analysis to separate patients with MFH recurrence or metastases within 6 months after the initial operation from those without these characteristics is worthwhile for prognostic assessment.


19p+ marker chromosome correlates with relapse in malignant fibrous histiocytoma.

Choong PF, Mandahl N, Mertens F, Willen H, Alvegard T, Kreicbergs A, Mitelman F, Rydholm A.

Department of Orthopedics, University Hospital, Lund, Sweden.

Genes Chromosomes Cancer 1996 Jun;16(2):88-93 Abstract quote

In this study, we examined the relationship of 19p13 aberrations, usually leading to addition of unknown material (19p+), and ring chromosomes to clinical outcome in patients with malignant fibrous histiocytoma (MFH). A

nalysis of 69 MFHs revealed 19 tumors with 19p+ and 24 tumors with ring chromosomes. After a median follow-up period of 36 months, 24 patients developed metastases, and 27 patients developed local recurrences. Ten patients had both local recurrences and metastases. Local recurrence was more common in association with 19p+ than without. Metastasis was more common with 19p+ tumors in high-risk patients (tumor size > 5 cm and grade III-IV; n = 48) than those without 19p+.

There was a trend suggesting fewer relapses after tumors with ring chromosomes. 19p+ may be an independent marker of unfavorable outcome in MFH.

TREATMENT Wide excision if feasible
Adjuvant radiation therapy may be beneficial in improving local control

Neoadjuvant chemotherapy in malignant fibrous histiocytoma of the limbs: 10 years of experience (1983-1992) at the Rizzoli Orthopedic Institute].

[Article in Italian] Bacci G, Ferrari S, Picci P, Forni C, Donati D, Manfrini M, Baldini N, Iantorno D, Campanacci M.

Sezione di Chemioterapia, Istituto Ortopedico Rizzoli, Bologna.

Minerva Med 1996 Apr;87(4):135-46 Abstract quote

During the period September 1983 to December 1991, 47 patients with nonmetastatic malignant fibrous histiocytoma (MFH) of the limbs were treated using 3 different protocols of neoadjuvant chemotherapy activated at successive intervals.

Surgery consisted of limb salvage in 41 cases and amputation in 6. After a mean follow-up of 6.5 years 33 patients (70%) had been continuously disease-free and 14 had undergone relapses. In the latter group the first sign of recurrence was metastasis in 12 cases and local recurrence in 2 cases. These results are distinctly better than those obtained in 20 patients treated during the same period using surgery alone (24% of disease-free survival and 30% local recurrence), and compared to those obtained in an earlier study in which surgery was associated with postoperative chemotherapy alone (59% of disease-free survival and 25% of local recidivation).

The authors conclude that, as already observed in the case of osteosarcoma, neoadjuvant chemotherapy can significantly improve prognosis even in patients with bone MFH localised in the limbs. Moreover, given that, contrary to adjuvant chemotherapy, associated chemotherapy is effective not only in controlling the microscopic disease but also reducing the incidence of local recurrence, it enables amputation to be avoided in the majority of patients.

Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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