This rare tumor of the lung primarily occurs in childhood. It usually appears as a pulmonary and or pleural-based mass and is characterized histologically by a primitive, variably mixed blastematous and sarcomatous appearance. It generally has a poor prognosis but a combination of surgical removal and chemotherapy may lead to cures in select cases.
Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/
Other Diagnostic Testing
and Clinical Variants
Differential Diagnosis Prognosis Treatment Commonly Used Terms Internet Links
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Pleuropulmomary blastoma INCIDENCE Rare AGE RANGE-MEDIAN Childhood usually GEOGRAPHY
Pleuropulmonary blastoma: a marker for familial disease.
Priest JR, Watterson J, Strong L, Huff V, Woods WG, Byrd RL, Friend SH, Newsham I, Amylon MD, Pappo A, Mahoney DH, Langston C, Heyn R, Kohut G, Freyer DR, Bostrom B, Richardson MS, Barredo J, Dehner LP.
Department of Hematology/Oncology, Children's Health Care, St. Paul, Minnesota 55102, USA.
J Pediatr 1996 Feb;128(2):220-4 Abstract quote
OBJECTIVE: To catalog and evaluate patterns of disease in families of children with pleuropulmonary blastoma (PPB).
METHODS: Data have been collected since 1988 on 45 children with PPB and their families. All pathologic materials were centrally reviewed. Preliminary molecular genetic analyses were performed when possible.
RESULTS: In 12 of 45 patients, an association was found between PPB and other dysplasias, neoplasias, or malignancies in the patients with or in their young relatives. The diseases found to be associated with PPB include other cases of PPB, pulmonary cysts, cystic nephromas, sarcomas, medulloblastomas, thyroid dysplasias and neoplasias, malignant germ cell tumors, Hodgkin disease, leukemia, and Langerhans cell histiocytosis. Abnormalities of the p53 tumor suppressor gene, Wilms tumor suppressor gene (WT1), and the putative second genetic locus for Wilms tumor (WT2) were not found in preliminary investigations.
CONCLUSIONS: The occurrence of PPB appears to herald a constitutional and heritable predisposition to dysplastic or neoplastic disease in approximately 25% of cases. All patients with PPB and their families should be investigated carefully. Further research of this new family cancer syndrome may provide insight into the genetic basis of these diseases.
DISEASE ASSOCIATIONS CHARACTERIZATION CONGENITAL CYSTIC ADENOMATOID MALFORMATION
Acta Radiol. 2004 May;45(3):289-92. Abstract quote
Pleuropulmonary blastoma (PPB) is a rare primary malignant pulmonary tumor in pediatric patients.
We report the development of PPB in the area of a previous pulmonary cyst in two children, one boy and one girl 5 and 12 years old, respectively.
We present the clinical and radiological findings. A short review of the literature is included.
Pleuropulmonary blastoma in congenital cystic adenomatoid malformation: report of a case.
Federici S, Domenichelli V, Tani G, Sciutti R, Burnelli R, Zanetti G, Domini R.
Department of Pediatric Surgery, University of Bologna, Italy
Eur J Pediatr Surg 2001 Jun;11(3):196-9 Abstract quote
Pulmonary blastoma is a rare malignant tumor seen in both adults and children. Approximately only 25% of cases occur in pediatric patients, many of whom affected by a congenital pulmonary cystic lesion.
The clinical features, radiological findings and management of a 3-year-old boy affected by a pulmonary blastoma which arose in a congenital cystic adenomatoid malformation are reported, and an extensive review of the literature is also made.
Because of the well-known tendency of cystic pulmonary diseases to develop malignancies, authors recommend the surgical excision of these kind of lesion or at least their close radiological follow-up.
Pulmonary blastoma after liver transplant: a case report.
Tartarone A, Romano G, Galasso R, Coccaro M, Cammarota A, Sgambato A, Bochicchio A.
Department of Medical Oncology, Centro di Riferimento Oncologico della Basilicata, Rionero in Vulture (PZ), Italy.
Tumori 2002 Mar-Apr;88(2):173-5 Abstract quote
There is an increased risk of cancer after organ transplantation mainly due to the immunosuppressive therapy required in these patients.
We report a case of biphasic pulmonary blastoma in an adult male who underwent liver transplant for hepatocellular carcinoma in March 1999, followed by immunosuppressive treatment and adjuvant chemotherapy with epirubicin. Disease-free survival lasted 18 months, then a diagnosis of biphasic pulmonary blastoma was made and the patient underwent a lung lobectomy. Five months after surgical resection a recurrence of this rare tumor was recorded and two cycles of cisplatin + etoposide and ifosfamide + etoposide and one cycle of second-line chemotherapy with vinorelbine were administered. The tolerability and the efficacy of this treatment were poor. The patient died less than one year after diagnosis. To our knowledge this is the first reported case of pulmonary blastoma in a transplant patient.
Our findings confirm that organ transplant recipients deserve long-term medical surveillance also in the absence of graft complications, and that pulmonary blastoma is an aggressive tumor with a poor prognosis.
PATHOGENESIS CHARACTERIZATION CHROMOSOMAL ABNORMALITIES
- Hidden chromosomal abnormalities in pleuropulmonary blastomas identified by multiplex FISH.
Quilichini B, Andre N, Bouvier C, Chrestian MA, Rome A, Intagliata D, Coze C, Lena G, Zattara H.
Departement de Genetique Medicale, CHU-Hopital d'Enfants La Timone, Bd Jean Moulin, 13385 Marseille Cedex 5, France.
BMC Cancer. 2006 Jan 5;6:4. Abstract quote
BACKGROUND: Pleuropulmonary blastoma (PPB) is a rare childhood dysontogenetic intrathoracic neoplasm associated with an unfavourable clinical behaviour.
CASES PRESENTATION: We report pathological and cytogenetic findings in two cases of PPB at initial diagnosis and recurrence. Both tumors were classified as type III pneumoblastoma and histological findings were similar at diagnosis and relapse. In both cases, conventional cytogenetic techniques revealed complex numerical and structural chromosomal abnormalities. Molecular cytogenetic analysis (interphase/metaphase FISH and multicolor FISH) identified accurately chromosomal aberrations. In one case, TP53 gene deletion was detected on metaphase FISH. To date, only few cytogenetic data have been published about PPB.
CONCLUSION: The PPB genetic profile remains to be established and compared to others embryonal neoplasia. Our cytogenetic data are discussed reviewing cytogenetics PPBs published cases, illustrating the contribution of multicolor FISH in order to identify pathogenetically important recurrent aberrations in PPB.
- Comparative genomic hybridization analysis of a pleuropulmonary blastoma.
Roque L, Rodrigues R, Martins C, Ribeiro C, Ribeiro MJ, Martins AG, Oliveira P, Fonseca I.
Cytometry and Cytogenetic Laboratory, Center for Immunology and Molecular Pathology, Portuguese Cancer Institute, R. Prof. Lima Basto 1099-023, Lisbon, Portugal.
Cancer Genet Cytogenet. 2004 Feb;149(1):58-62. Abstract quote
Pleuropulmonary blastoma (PPB) is a rare, aggressive dysontogenetic tumor of childhood.
We report the comparative genomic hybridization (CGH) study performed on a case of PPB in a 3-year-old-boy. The tumor was characterized by several chromosomal imbalances. Gains observed affected regions: 1q12-q23, 3q23-qter, 8pter-q24.1, 9p13-q21, 17p12-p11, 17q11-q22, 17q23-q25, 19pter-p11, and 19q11-q13.3. Whole chromosome gains were detected at 2 and 7. Loss of genetic material was found at regions: 6q13-qter, 10pter-p13, 10q22-qter, and 20p13. To our knowledge, there have been no CGH reports on PPB, but it is interesting to note that 1) the alterations found confirm previous cytogenetic reports describing gains of chromosomes 2 and 8 as recurrent abnormalities in this type of tumor, suggesting that a gene or genes of putative relevance in PPB pathogenesis are mapped at 8p11-p12, and 2) the CGH profile of this case is very similar to those observed in embryonal rhabdomyosarcomas, in which gains of 2 or 2q, 7 or 7q, and 8 or 8p and loss of 10q22-qter are consistently found.
This finding supports the hypothesis that PPB may be tumorigenetically related with embryonal rhabdomyosarcoma.
Use of multicolor spectral karyotyping in genetic analysis of pleuropulmonary blastoma.
Barnard M, Bayani J, Grant R, Teshima I, Thorner P, Squire J.
Department of Pediatric Laboratory Medicine, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8.
Pediatr Dev Pathol 2000 Sep-Oct;3(5):479-86 Abstract quote
Pleuropulmonary blastoma (PPB) is a rare, malignant intrathoracic pediatric tumor. It arises from the lung, pleura, or mediastinum and its pathogenesis and relationship to other pediatric solid tumors is not well understood.
In this study, a case of PPB in a 3-year-old girl was studied using a combination of molecular genetic methods and cytogenetics. Molecular analysis of the commonly encountered fusion translocation gene products of pediatric solid tumors failed to detect a rearrangement. Cytogenetic analysis, supplemented by multicolor spectral karyotyping (SKY), identified an unbalanced translocation between chromosomes 1 and X, resulting in additional copies of 1q, an extra copy of Xq, and loss of part of Xp. In addition, trisomy 8 was detected.
The identification of new chromosomal alterations and confirmation of previously reported ones in this rare neoplasm helps to improve our understanding of its pathogenesis and association with other pediatric tumors.
Gains of chromosome 8 are confined to mesenchymal components in pleuropulmonary blastoma.
Vargas SO, Nose V, Fletcher JA, Perez-Atayde AR.
Department of Pathology, Harvard Medical School, Boston, MA 0211 5, USA.
Pediatr Dev Pathol 2001 Sep-Oct;4(5):434-45 Abstract quote
Pleuropulmonary blastoma, an aggressive tumor that is emerging as a distinct entity of childhood, is characterized by mesenchymal elements (including undifferentiated blastema and often cartilaginous, rhabdomyoblastic, or fibroblastic differentiation) and epithelium-lined spaces.
We investigated two patients with pleuropulmonary blastoma, a 3-year-old boy and an 11-year-old girl, both with large cystic masses replacing one lung. In both children, the post-chemotherapy resection specimens showed more maturation of rhabdomyoblasts and more nuclear pleomorphism in all mesenchymal cell lines, compared with biopsies sampled before treatment. Karyotypic analysis demonstrated gains in chromosome 8 in both cases and 17p deletion in one case. Fluorescent in situ hybridization analysis demonstrated that the chromosome 8 gains were present in all mesenchymal elements, including undifferentiated blastematous, rhabdomyoblastic, fibroblastic, and chondroblastic areas. Epithelial cells showed no chromosome 8 gains. The chromosome 8 aberrations were not appreciably different in pre- versus post-chemotherapy tissue.
Our findings substantiate previous reports that polysomy of chromosome 8 is a consistent feature of pleuropulmonary blastoma. Further, they indicate that clonal proliferation in pleuropulmonary blastoma is restricted to the malignant mesenchymal elements, supporting the notion that the epithelial components of this tumor are non-neoplastic.
p53 and K-ras mutational genotyping in pulmonary carcinosarcoma, spindle cell carcinoma, and pulmonary blastoma: implications for histogenesis.
Holst VA, Finkelstein S, Colby TV, Myers JL, Yousem SA.
Department of Pathology, University of Pittsburgh Medical Center, PA 15213-2582, USA.
Am J Surg Pathol 1997 Jul;21(7):801-11 Abstract quote
In an attempt to understand the molecular pathogenesis of biphasic pulmonary neoplasms, the authors studied 25 cases of carcinosarcoma, spindle cell carcinoma, and pulmonary blastoma using a combined immunohistochemical and topographic genotyping approach for the presence of p53 abnormalities within the different epithelial and mesenchymal components of these tumors.
Genotyping involved a search for point mutational damage in p53 exons 5-8, which was correlated with p53 immunoreactivity. This analytical approach demonstrated p53 missense point mutations in four of nine cases of spindle cell carcinoma with a 100% concordance rate between p53 immunopositivity and the presence of DNA mutational damage. One of six carcinosarcomas, heterologous in type, exhibited a p53 mutation. The concordance rate among carcinosarcomas was also 100%. However, the concordance rate among classic biphasic pulmonary blastomas was only 43%, with one of seven cases demonstrating a p53 mutation by DNA genotyping. The lack of concordance in pulmonary blastomas was possibly due to the existence of genotypically distinct subsets of tumor cells likely bearing mutations among largely nonmutated cells. In a similar fashion, among three well-differentiated fetal type adenocarcinomas, no p53 mutations were detected despite the presence of focal p53 immunopositivity in one of the cases. No K-ras mutations were detected in any of the 25 tumors examined.
Monoclonal histogenesis from a single totipotential cell in a subset of these neoplasms (six of 22 cases) was supported by the finding of p53 overexpression and identical p53 mutational genotype in both the epithelial and spindle elements of the tumors. Furthermore, the finding of a small percentage of p53-positive tumor cells within one or both components suggests late acquisition of p53 mutational change in a subset of pulmonary blastomas.
Pediatr Radiol. 2005 Apr;35(4):387-91. Epub 2005 Jan 19. Abstract quote
BACKGROUND: Pleuropulmonary blastoma (PPB) is a rare tumor of the chest seen in young children and recently recognized as distinct from the pulmonary blastoma typically encountered in adults.
OBJECTIVE: The purpose of this study is to review and describe the findings of PPB on radiography and CT in four patients.
METHODS: Radiographs and CT findings were reviewed in four patients with pathologically proven PPB.
RESULTS: All four cases demonstrated large masses in the right hemithorax with heterogeneous low attenuation, pleural effusion, contralateral mediastinal shift, and lack of chest wall invasion.
CONCLUSION: When a large pleural-based mass is identified in a young child, PPB should be considered. Suggestive findings include absence of chest wall invasion, presence of pleural fluid, right-sided location, and heterogeneous low attenuation.
Ga-67 scintigraphy in pulmonary blastoma in a child.
Howman-Giles R, Dalla Pozza L, Uren R.
Department of Nuclear Medicine, Children's Hospital, Sydney, New South Wales, Australia.
Clin Nucl Med 1993 Feb;18(2):120-2 Abstract quote
Pulmonary blastoma is a rare primary malignancy of the lung with a small number of cases reported in children. The primary treatment is surgery, however radiotherapy and chemotherapy are also used. The ability to determine whether the tumor has been fully removed and when recurrence occurs is important for appropriate treatment and prognosis.
A case of a 7-year-old girl with pulmonary blastoma is reported in which the primary tumor is extremely Ga-67-avid and in which the gallium scans were helpful in determining early recurrence and metastatic disease to the brain.
LABORATORY MARKERS HYPERCALCEMIA
Pulmonary blastoma with fatal hypercalcemia.
Stancic V, Grbac I, Ferencic Z, Pejsa V, Knezevic F.
Department of Medicine, University Hospital Sestre milosrdnice, Zagreb, Croatia.
Respiration 1997;64(3):247-50 Abstract quote
We describe a 50-year-old, previously healthy male with metastatic pulmonary blastoma associated with hypercalcemic and hyperosmolar complications which caused his death after 5 days.
The primary tumor consisted of epithelial [cytokeratin, beta-hCG CEA, neuron-specific enolase (NSE)-positive] and mesenchymal components (beta-hCG, vimentin, NSE-positive, while the metastases had only a mesenchymal component.
Pleuropulmonary blastoma is THE pulmonary blastoma of childhood.
Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes and St Louis Children's Hospital, Washington University Medical Center, St Louis, MO 63110.
Semin Diagn Pathol 1994 May;11(2):144-51 Abstract quote
Pulmonary blastoma was defined by Spencer and others as a neoplasm with histopathological features thought to be reminiscent of Wilms' tumor. Unlike the other embryonal-fetal neoplasms that typically occur in early childhood, the majority of pulmonary blastomas have been reported in adults. One explanation offered by Spencer for the delayed clinical presentation of pulmonary blastoma is the continued development of lung parenchyma well past the postnatal period. It has been proposed that the pulmonary blastoma is a variant of carcinosarcoma, which is seen almost exclusively in adults.
The classic pulmonary blastoma is described as a neoplasm with a mixture of primitive tubular profiles, immature blastema, and a spindle cell stroma. More recently, some have included the well-differentiated adenocarcinoma of fetal type as a pure epithelial expression of pulmonary blastoma.
By contrast, most cases of pulmonary blastoma in children have been described as having an exclusive mesenchymal composition, either embryonal rhabdomyosarcoma or a complex, mixed blastematous and sarcomatous neoplasm. Some pulmonary blastomas in children, particularly those with only embryonal rhabdomyosarcoma, have presented as a peripheral multicystic lesion that has been interpreted as a developmental cyst, either cystic adenomatoid malformation or bronchogenic cyst. Other tumors have been described as intrathoracic and anatomically separate from the lung, like some extralobar sequestrations.
We have proposed the designation pleuropulmonary blastoma for these pulmonary or extrapulmonary neoplasms of childhood. We propose that this neoplasm is the rightful pulmonary blastoma rather than the pulmonary blastoma of Spencer, which has been the long-time pretender to the title.
Pleuropulmonary blastoma in an adult: an initial case report.
Hill DA, Sadeghi S, Schultz MZ, Burr JS, Dehner LP.
Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes-Jewish Hospital, Washington University Medical Center, St. Louis, Missouri 63110, USA.
Cancer 1999 Jun 1;85(11):2368-74 Abstract quote
BACKGROUND: Pleuropulmonary blastoma (PPB) is a unique dysontogenetic neoplasm of childhood. Its primitive, sarcomatous features are analogous to those of other dysembryonic or dysontogenetic tumors, such as Wilms tumor, hepatoblastoma, neuroblastoma, and embryonal rhabdomyosarcoma. PPB typically presents in young children, most younger than 5 years, as a pulmonary and/or pleural-based tumor with cystic, solid, or combined cystic and solid features. These neoplasms are characterized histologically by primitive mesenchymal or a mixture of primitive and sarcomatous components and generally have an unfavorable clinical outcome: death occurs within 1-2 years after diagnosis.
METHODS: Clinicopathologic and radiographic findings of a man age 36 years with a cystic and solid mass in the left hemithorax were reviewed and compared with previously studied cases of PPB.
RESULTS: Pathologic examination of the mass revealed a cystic and solid neoplasm composed of malignant mesenchymal cells that were immunoreactive for vimentin and muscle specific actin and focally for desmin. The architectural and cytologic appearances as well as the immunohistochemical profile were those of type II PPB.
CONCLUSIONS: To the authors' knowledge, all previously reported cases of PPB occurred in children age 12 years or younger. They believe that this case represents the first occurrence of PPB in an adult and documents the finding that, although it is uncommon, adults can develop primitive neoplasms that are usually associated with the pediatric population. In addition, the clinicopathologic features observed in the authors' adult patient were consistent with their experience with this tumor type in children. The patient died less than 1 year after diagnosis.
Bilateral cystic pleuropulmonary blastoma in early infancy.
Picaud JC, Levrey H, Bouvier R, Chappuis JP, Louis D, Frappaz D, Claris O, Bellon G.
Departments of Neonatology, Pathology, and Pediatric Surgery at the Edouard Herriot Hospital, Lyon, France.
J Pediatr 2000 Jun;136(6):834-6 Abstract quote
We report 2 cases of multifocal cystic (type 1) pleuropulmonary blastoma, diagnosed during the first 6 months of life.
This rare entity must be recognized before evolution into the prognostically unfavorable type 2 or type 3 pleuropulmonary blastoma.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood.
Manivel JC, Priest JR, Watterson J, Steiner M, Woods WG, Wick MR, Dehner LP.
Department of Laboratory Medicine, University of Minnesota Medical School and Hospital, Minneapolis.
Cancer 1988 Oct 15;62(8):1516-26 Abstract quote
The authors studied 11 pediatric intrathoracic neoplasms that share clinicopathologic features and constitute a specific tumor in children. These neoplasms were intrapulmonary, mediastinal, or pleural-based masses.
A common histologic feature was the presence of small, primitive cells with blastematous qualities separated by an uncommitted stroma. Focal rhabdomyosarcomatous, chondrosarcomatous, and liposarcomatous differentiation was observed. Epithelial components had bland cytologic features and probably represented entrapped benign epithelium and/or mesothelium.
The prognosis for these patients was grave; seven patients died of their disease 5 months to 2 years after diagnosis. Two patients have survived disease-free for 10 and 12 years after diagnosis. Two recent cases are alive 14 and 32 months after diagnosis. This neoplasm constitutes a distinct entity which has been reported in the literature as pulmonary blastoma in children. It differs from pulmonary blastoma in adults because of its variable anatomic location, primitive embryonic-like blastema and stroma, absence of a carcinomatous component, and potential for sarcomatous differentiation. The designation of pleuropulmonary blastoma is suggested by the authors for these intrathoracic neoplasms of childhood rather than pulmonary blastoma for histogenetic and anatomic reasons.
The clinicopathologic features, immunophenotypic and ultrastructural characteristics, possible histogenesis, and differential diagnosis of these neoplasms from other thoracopulmonary tumors in children serve as the basis for this report.
Pleuropulmonary blastoma in childhood. A tumor of divergent differentiation.
Hachitanda Y, Aoyama C, Sato JK, Shimada H.
Department of Pathology, Childrens Hospital Los Angeles, California 90027.
Am J Surg Pathol 1993 Apr;17(4):382-91 Abstract quote
Pleuropulmonary blastoma (PPB) is a rare and highly aggressive tumor in children and is distinct from ordinary pulmonary blastoma or carcinosarcoma of the lung.
This report describes the phenotypical characteristics of seven PPB tumors. The patients were five females and two males, all diagnosed in their third year of life. Five tumors were located within the pulmonary parenchyma, one in the mediastinum, and one involved both lung and mediastinum. Two children are alive and five have died of disease.
Histologically, PPB showed a diffuse proliferation of undifferentiated blastemal cells with additional areas of chondroblastic foci (six tumors), storiform pattern (three tumors), alveolar pattern (one tumor), and lipoblastic differentiation (one tumor). Immunohistochemically, tumor cells were positive for vimentin in five of five tumors, histiocytic markers (A1AT and A1ACT, four of six cases; lysozyme, two of six; KP1, three of five) and myogenic markers (desmin, four of six; HHF35, five of six). S-100 was expressed in the chondroblastic areas (in three of four cases). Epithelial membrane antigen and cytokeratin were positive only in epithelial or mesothelial cells entrapped in the tumor.
Ultrastructural examination demonstrated a similarity between the proliferating cells in PPB and those seen in malignant fibrous histiocytoma; that is, PPB tumor was mainly composed of a mixture of primitive, fibroblastic, myofibroblastic, histiocytoid, and fibrohistiocytoid cells. Also identified were cells with rhabdomyoblastic differentiation (two tumors) showing thick and thin filaments with Z-discs.
In conclusion, PPB showed phenotypical diversity and was composed of MFH-type cells and cells with more specific mesenchymal differentiation.
Pleuropulmonary blastoma: a clinicopathologic study of 50 cases.
Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP.
Department of Hematology/Oncology, Children's Health Care, St. Paul, Minnesota, USA.
Cancer 1997 Jul 1;80(1):147-61 Abstract quote
BACKGROUND: Pleuropulmonary blastoma (PPB) is a unique dysontogenetic neoplasm of childhood that appears as a pulmonary and/or pleural-based mass and is characterized histologically by a primitive, variably mixed blastematous and sarcomatous appearance.
METHODS: Histologic material from all cases was reviewed and the tumors subclassified as type I (purely cystic), type II (cystic and solid), or type III (purely solid). Data regarding presenting symptoms, family history, operative findings, pathologic subtypes, therapeutic interventions, and outcome were correlated with survival by standard statistical methods.
RESULTS: The series was comprised of 24 males and 26 females. Respiratory difficulty with or without fever was the most common clinical symptom reported. Cyst formation in the affected lung was identified radiographically in 19 children (38%) at or before the definitive pathologic diagnosis. The ages at presentation of the 7 type I, 24 type II, and 19 type III PPBs were significantly different: 10, 34, and 44 months, respectively (P < 0.001). Local recurrence developed in 1 of 7 type I PPBs (14%) and in 18 of 43 type II and III PPBs (46%); distant metastasis occurred in 13 patients, chiefly to the brain/spinal cord or bone, and was observed only in those with type II or type III PPB. Patients with pleural or mediastinal involvement fared significantly worse than those without such involvement. Five-year survival was 83% for type I and 42% for types II and III. Survival differences on the basis of pathologic subtype did not reach statistical significance.
CONCLUSIONS: PPB is an aggressive, intrathoracic neoplasm of early childhood with an unfavorable outcome. Although survival differences among patients with different histologic subtypes of disease did not reach statistical significance, the apparently better outcome for patients with purely cystic type I tumors may be borne out in a large series. These observations support the premise that type I and III PPB are bridged morphologically by type II PPB with its combined cystic and solid features. The PPB should be regarded as the pulmonary dysontogenetic analogue to Wilms' tumor in the kidney, neuroblastoma in the adrenal gland, and hepatoblastoma in the liver. Molecular genetic investigations, especially in constitutional PPB, should be revealing. In view of the poor outcomes for patients with types II and III, new and aggressive therapies must be developed.
Pleuropulmonary blastoma: A case report documenting transition from type I (cystic) to type III (solid).
Wright JR Jr.
Departments of Pathology, Surgery, and Bio-Medical Engineering, Dalhousie University Faculty of Medicine, Halifax, Nova Scotia, Canada.
Cancer 2000 Jun 15;88(12):2853-8 Abstract quote
BACKGROUND: Pleuropulmonary blastoma is a rare, aggressive neoplasm that typically occurs in young children. It has been classified as type I, II, or III on the basis of the cystic versus solid nature of the lesion as well as the histologic appearance. Although it has been speculated that type I lesions may have a tendency to progress into type III lesions, no such case has been reported to date.
METHODS: A case of type I pleuropulmonary blastoma in a girl age 2 years 9 months was found in our departmental archive. This case, originally diagnosed as a hamartoma over 20 years ago, predated the description of this pathologic entity. Over a 3-year period, the patient underwent excisions of a primary tumor and 3 subsequent recurrences, thus allowing us to follow the natural history of this neoplasm.
RESULTS: The primary tumor was a large, multicystic mass (roughly 90% cystic by volume) with benign histologic appearance except for occasional foci with bland, embryonal rhabdomyosarcomatous features. In subsequent recurrences, the resected specimens became increasingly solid and had an anaplastic, multiphasic mesenchymal pattern.
CONCLUSIONS: The course of the patient described here represents the first case in which transition from type I (cystic) to type III (solid) was documented.
Pulmonary blastoma with rhabdomyosarcomatous differentiation: an electron microscopic and immunohistochemical study.
Heckman CJ, Truong LD, Cagle PT, Font RL.
Department of Pathology, Baylor College of Medicine, Houston, TX 77030.
Am J Surg Pathol 1988 Jan;12(1):35-40 Abstract quote
Pulmonary blastoma is a rare lung tumor composed of epithelial and mesenchymal elements; the latter element may show various patterns of differentiation toward mature tissue, such as cartilage, smooth muscle, and bone. Rhabdomyoblastic differentiation in pulmonary blastoma is quite rare; only five such cases have been reported.
We report two cases of pulmonary blastoma with rhabdomyoblastic differentiation documented for the first time by electron microscopy and immunohistochemistry including documentation for myoglobin, actin, vimentin and desmin. The diffuse and prominent rhabdomyoblastic differentiation in one case is most unusual.
CHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE GENERAL
Pulmonary blastoma: an ultrastructural and immunohistochemical study with special reference to nuclear filament aggregation.
Yang P, Morizumi H, Sano T, Hirose T, Hasegawa T, Seki K, Hizawa K.
First Department of Pathology, University of Tokushima School of Medicine, Japan.
Ultrastruct Pathol 1995 Nov-Dec;19(6):501-9 Abstract quote
A case is presented of pulmonary blastoma occurring in the right upper lobe of a 25-year-old man without distinct clinical features and laboratory abnormality. Light microscopic analysis revealed that the tumor was composed of branching glands and morulae embedded in a primitive but bland mesenchyme.
Immunohistochemically the epithelial cells were immunoreactive for cytokeratins, S-100 protein, protein gene product 9.5, chromogranin A, calcitonin, and Ki-67 (MIB-1); the mesenchymal cells were immunoreactive for vimentin, actin, cytokeratins, and Ki-67; and all the tumor cells were negative for p53, estrogen receptor protein, and human chorionic gonadotropin beta. Characteristically, many epithelial cells contained optically clear nuclei which were immunoreactive for biotin (M743). Electron microscopic analysis revealed that the optically clearing change was due to replacement of the central area of the nuclei by a mass of parallel-arranged 7- to 10-nm filaments, and biotin-immunoreactive products were mainly localized in the nuclear matrix. Additionally, spherical bodies were identified in the cytoplasm of the nuclear filament-aggregated cells, suggestive of an intimate pathogenetic association of the two morphological abnormalities.
The similarity of the aggregated nuclear filaments to those observed in gestational endometrium and ovarian endometrioid carcinoma implies that a similar mechanism plays a role in the pathogenesis of these abnormalities.
Aberrant Nuclear/Cytoplasmic Localization and Gene Mutation of beta-Catenin in Classic Pulmonary Blastoma: beta-Catenin Immunostaining Is Useful for Distinguishing Between Classic Pulmonary Blastoma and a Blastomatoid Variant of Carcinosarcoma.
Nakatani Y, Miyagi Y, Takemura T, Oka T, Yokoi T, Takagi M, Yokoyama S, Kashima K, Hara K, Yamada T, Nozawa A, Inayama Y, Sakamoto K, Ogawa N, Kitamura H, Resl M, Cho SH, Koss MN, Mark EJ.
*Department of Pathology, Yokohama City University Medical Center, daggerDivision of Anatomic and Surgical Pathology, Hospital of
Yokohama City University, Departments of double daggerSurgery and section signPathology, Yokohama City University Graduate School of Medicine and School of Medicine, paragraph sign
Clinical Research Laboratory, Kanagawa Prefectural Cancer Center, and Department of Surgery, Kanagawa Prefectural Cardiovascular and Respiratory Center, Yokohama, Japan; #Department of Pathology, Japanese Red Cross Medical Center, and **Department of Pathology, Kanto Central Hospital, Tokyo, Japan; daggerdaggerDepartment of Pathology, St. Marianna University, Kawasaki, Japan; double daggerdouble daggerDepartment of Pathology, Oita Medical University, Oita, Japan; section sign section signDepartment of Medical Technology, Nagoya University School of Health Sciences, Nagoya, Japan; paragraph sign paragraph signDepartment of Pathology, Aichi Medical University, Aichi, Japan; Department of Pathology, Gifu Municipal Hospital, Gifu, Japan; ##Department of Pathology, Charles University Medical School, Hradec Kralove, Czech Republic; ***Department of Pathology, Yonsei University College of Medicine, Seoul, Korea; daggerdaggerdagger
Department of Pathology, Keck School of Medicine at University of Southern California, Los Angeles, CA; and double daggerdouble daggerdouble daggerDepartment of Pathology, Harvard Medical School and Massachusetts General Hospital, Boston, MA.
Am J Surg Pathol. 2004 Jul;28(7):921-927. Abstract quote
It is now known that gene mutation of beta-catenin with subsequent nuclear/cytoplasmic (N/C) overaccumulation of the protein plays an important role in tumorigenesis of various organs.
We recently demonstrated that low-grade adenocarcinoma of the fetal lung type (L-FLAC)/well-differentiated fetal adenocarcinoma (WDFA), the epithelial prototype of classic pulmonary blastoma (CPB), shows N/C localization of beta-catenin with genetic mutation. This prompted us to further investigate the state of beta-catenin abnormality in CPB and related neoplasms.
We studied 9 lung tumors previously diagnosed as biphasic pulmonary blastoma (PB). Histologically, 4 cases (median age 34 years) were CPB with l-FLAC/WDFA as the epithelial component, whereas 5 cases (median age 65 years) were a variant of carcinosarcoma with high-grade FLAC/clear cell adenocarcinoma with fetal lung features as the epithelial component, which we term the blastomatoid variant of carcinosarcoma (BCS).
Immunohistochemically, all 4 CPBs showed aberrant N/C localization of beta-catenin both in the epithelial and mesenchymal components, with especially high staining intensity in the budding glands and morules. In contrast, all 5 BCSs showed preserved or diminished membranous expression and no significant N/C expression of beta-catenin in the epithelial component, and absent or focal N/C expression of beta-catenin in the mesenchymal component. Mutational analysis of exon 3 of the beta-catenin gene revealed that 3 CPBs harbored missense mutations (S29F, S37F, and S37F), whereas none of the 5 BCSs had this mutation. This study suggests that beta-catenin gene mutations may play a role in the tumorigenesis of CPB. Although CPB and BCS have often been grouped together as biphasic PB, they are different entities based on immunohistochemical and molecular analysis of beta-catenin. Immunostaining for beta-catenin is useful for the discrimination.
Mutations in the p53 gene in pulmonary blastomas: immunohistochemical and molecular studies.
Bodner SM, Koss MN.
Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA.
Hum Pathol 1996 Nov;27(11):1117-23 Abstract quote
Well-differentiated fetal adenocarcinomas and biphasic blastomas are types of lung cancer that contain glands that mimic the appearance of fetal lung.
Biphasic blastomas also show a primitive embryonic stroma. Despite histological similarities leading these two tumors to be classified as pulmonary blastomas, they have distinct clinical and prognostic features. Little information is available on genetic changes in these tumors because they are rare; therefore, the authors studied nine biphasic blastomas and 12 well-differentiated fetal adenocarcinomas for the presence of mutations in the p53 gene. Mutations in the p53 gene are common in other lung cancers, and the type of mutation in the p53 gene can provide information about the original or inciting mutagens.
The authors found five biphasic blastomas (42%) had mutations in the p53 gene by immunohistochemical and molecular analysis, whereas none of the well-differentiated fetal adenocarcinomas contained mutations. These results provide molecular support for the significance of distinguishing between well-differentiated fetal adenocarcinoma and biphasic blastoma histologically and identify several types of p53 gene mutations that occur in these tumors.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES ADENOCARCINOMA, FETAL TYPE
Pulmonary adenocarcinomas of the fetal lung type: a clinicopathologic study indicating differences in histology, epidemiology, and natural history of low-grade and high-grade forms.
Nakatani Y, Kitamura H, Inayama Y, Kamijo S, Nagashima Y, Shimoyama K, Nakamura N, Sano J, Ogawa N, Shibagaki T, Resl M, Mark EJ.
Division of Anatomic and Surgical Pathology, Hospital of Yokohama City University, Yokohama, Japan.
Am J Surg Pathol 1998 Apr;22(4):399-411 Abstract quote
Seven cases of high-grade adenocarcinoma of fetal lung type (H-FLAC) are compared with nine cases of pulmonary endodermal tumor resembling fetal lung or low-grade adenocarcinoma of fetal lung type (L-FLAC). Of the seven patients with of H-FLAC, four were men and three were women. All of the patients but one were in their 60s or 70s. Five patients were smokers.
After resection of the tumor, three patients died of metastases, two patients are alive with no evidence of disease, and two patients died of a postoperative complication. Histologically, H-FLAC and L-FLAC have both complex glandular structures resembling fetal lung and neuroendocrine differentiation. Two cases of H-FLAC had stromal proliferation typical of biphasic pulmonary blastoma. The H-FLAC was distinguished from L-FLAC by the presence of disorganized glands, large vesicular nuclei, prominent nucleoli, pronounced anisonucleosis, absence of morules, transition to conventional adenocarcinoma, broad areas of necrosis, desmoplastic stroma, overexpression of p53 protein, and production of alpha-fetoprotein. High and low grades of FLAC explain discrepancies in previously reported clinicopathologic features of FLAC. The H-FLAC needs to be distinguished from L-FLAC. Both forms may have stromal components, so both have been referred to as blastomas. The H-FLAC represents the prototype of so-called pulmonary blastoma predominantly seen in the elderly, whereas L-FLAC and its biphasic form predominate in the middle-aged population.
Pulmonary lipoblastoma in an 18-month-old child: a unique tumor in children.
Kanu A, Oermann CM, Malicki D, Wagner M, Langston C.
Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA.
Pediatr Pulmonol 2002 Aug;34(2):150-4 Abstract quote
Lipoblastoma, a rare benign tumor of adipose tissue, typically occurs in the superficial or deep layers of soft tissue on the trunk or extremities.
Other sites of occurrence include the head, neck, and retroperitoneum. Lipoblastoma of the chest wall and parietal pleural have been reported, but occurrence within the lung has not been previously described.
We report a case of pulmonary lipoblastoma in a young child presenting with complete opacification of the left hemithorax and mediastinal shift on chest radiograph. A lobectomy was performed, and the diagnosis was made by histological examination.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSTIC FACTORS
Pediatr Blood Cancer. 2006 Apr 17; [Epub ahead of print] Abstract quote
PURPOSE: To evaluate the prognostic factors in a series of children affected by pleuropulmonary blastoma (PPB).
PATIENTS AND METHODS: Clinicopathological findings, treatment, and outcome of 22 PPB cases observed in 13 Italian Associations for Pediatric Hematology and Oncology centers are reported. Clinical data, surgical notes, pathologic findings, and summaries of treatment were taken from the charts and correlated with outcome by standard statistical methods.
RESULTS: The series included 22 patients (14 males) with a median age of 30.5 months followed up for a median of 22 months (range 2-176 months). In nine patients the PPB developed with lung involvement only. Congenital lung cysts were recorded in five cases. Nine patients had recurrences. Gender, side, tumor size, pre-existing lung cysts, and extent of surgical resection at diagnosis did not significantly affect survival by univariate analysis. Achieving total resection of the tumor at any time of treatment resulted in a significantly better prognosis (P = 0.01), whereas extrapulmonary involvement at diagnosis resulted in a significantly worse prognosis (P = 0.01). Estimated 15-year event-free and overall survival rates were 44 and 49% for all patients, respectively.
CONCLUSIONS: PPB is an aggressive neoplasm. Total resection of PPB performed at any time of treatment appears to provide a better outcome, whereas extrapulmonary involvement at diagnosis worsens the prognosis.
J Pediatr Surg. 2006 Jan;41(1):66-71. Abstract quote
BACKGROUND/PURPOSE: Pleuropulmonary blastoma (PPB) is a rare primary neoplasm of pleuropulmonary mesenchyme. Fewer than 170 children have been reported, and few single institutions have reported more than several cases. Treatment for this condition is primarily surgical resection; however, increasing experience suggests that adjuvant chemotherapy may decrease recurrence and improve outcome.
METHODS: We reviewed the charts of all children with PPB treated at our institution since 1960. We reviewed the prenatal features, clinical presentation, operation, pathological findings, adjuvant treatment, and outcome.
RESULTS: Ten children (6 boys and 4 girls) were treated for PPB at a mean age of 3.2 +/- 4.3 years. In 2, a cystic lung mass was diagnosed prenatally, and in 8, a cystic or solid and cystic lung mass was diagnosed postnatally (right lung, 3; left lung, 4; and bilateral, 3). In no patient was PPB considered preoperatively. Surgical resection was performed at 1 day to 11 years (median, 23 months) of age. Seven children had complete resection; 1 had microscopic residual disease, and 2 had gross residual disease. Pathology showed type I PPB in 7, type II in 1, and type III in 2. Five patients received adjuvant chemotherapy with vincristine, actinomycin, and cyclophosphamide-based regimens. At follow-up (mean, 7.7 +/- 11.5 years; range, 1-456 months), children with type I PPB have no evidence of disease (n = 6) or are lost to follow-up (n = 1), whereas all those with type II/III PPB have died of the disease.
CONCLUSIONS: PPB must be included in the differential diagnosis of a fetus, neonate, or child with a cystic lung mass. This finding supports early resection of these lesions rather than observation or treatment with nonoperative strategies.
Pleuropulmonary blastoma: long-term survival and literature review.
Romeo C, Impellizzeri P, Grosso M, Vitarelli E, Gentile C.
Department of Clinical and Experimental Medicine, University of Catanzaro, Catanzaro, Italy.
Med Pediatr Oncol 1999 Oct;33(4):372-6 Abstract quote
BACKGROUND AND PROCEDURE: Pleuropulmonary blastoma (PPB) identifies different types of pulmonary tumors in the pediatric age. It is extremely uncommon and is known to have a very poor prognosis, with only few cases surviving after 10 years follow-up. Our experience with such a patient and long-term follow-up (12 years) is therefore of interest. She was a 2-year-old girl admitted with a history of respiratory distress and underwent a left thoracotomy. The tumor was removed and after 1 month a polychemotherapy course was started using dactinomycin and vincristine for 6 months. The patient remains disease-free 12 years after the operation.
CONCLUSIONS: The biologic behavior of the tumor is considered unpredictable, and for this reason much effort has been expended in trying to identify prognostic factors. The preoperative size of the mass (size <5 cm) in our patient, its complete excision, and the histologic aspects might be helpful in identifying favorable prognostic factors.
Pediatr Hematol Oncol. 2005 Jan-Feb;22(1):71-6. Abstract quote
Pleuropulmonary blastoma is a rare and aggressive primary intrathoracic neoplasm of children. T'he prognosis is extremely poor with frequent metastasis to the brain and bone.
A 4-year-old boy successfully treated with multimodal therapy despite unfavorable prognostic factors is presented.
The authors support the use of aggressive treatment of pleuropulmonary blastoma with surgery, chemotherapy, and radiotherapy.
Pleuropulmonary blastoma: management and prognosis of 11 cases.
Indolfi P, Casale F, Carli M, Bisogno G, Ninfo V, Cecchetto G, Bagnulo S, Santoro N, Giuliano M, Di Tullio MT.
Pediatric Oncology Service-Pediatric Department II, University of Napoli, Naples, Italy.
Cancer 2000 Sep 15;89(6):1396-401 Abstract quote
BACKGROUND: Pleuropulmonary blastoma (PPB) is a rare and aggressive malignant tumor that affects children and adults. This neoplasm is histologically characterized by primitive blastema and a malignant mesenchymal stroma that often demonstrates multidirectional differentiation. Despite the introduction of multimodal therapy, the prognosis of patients with PPB remains poor.
METHODS: In the current study the authors reported on PPB cases from a national retrospective search performed in 18 Italian Associations for Pediatric Hematology and Oncology centers. Clinical data, surgical notes, pathologic findings, and summaries of chemotherapy and radiotherapy were obtained from reports and correlated with outcome by standard statistical methods.
RESULTS: The series included 11 patients (7 boys and 4 girls) with a median age of 32 months. Respiratory distress was the most common clinical symptom. In three patients the PPB developed from other primary dysplastic diseases: cystic adenomatoid malformation in one case and congenital lung cysts in the other two cases. Five patients experienced disease recurrences (local recurrence in three patients and distant metastasis in two patients, within the central nervous system and an intraocular location, respectively). Patients with a type 2 histologic pattern and/or pleural involvement were found to have a worse outcome compared with patients without such features. Event free survival at 2 years from the time of diagnosis was 45% for all patients. Overall survival at 2 years was 72% for all patients.
CONCLUSIONS: PPB is an aggressive neoplasm of early childhood and to the authors' knowledge no adequate therapy has been defined to date for patients with PPB. After making the diagnosis, the main goal of therapy should be radical surgery, even in patients with microscopic residual disease. Because the response to chemotherapy is poor, the authors' experience suggests that chemotherapy should be given with local radiotherapy in the majority of patients.
Granata C, Gambini C, Carlini C, Repetto P, Torre M, Mazzola C, Mattioli G, Jasonni V.
Department of Paediatric Surgery, Giannina Gaslini Institute, Genova, Italy.
Eur J Pediatr Surg 2001 Aug;11(4):271-3 Abstract quote
We report a rare case of pleuropulmonary blastoma arising in a 3-year-old boy and involving the middle and upper lobes of the right lung. Radical resection of the mass was achieved by a bilobectomy.
Microscopic examination of the histologic sections showed the typical findings of blastemal and mesenchymal areas with focal zones of rhabdomyoblastic and liposarcomatous differentiation. Monthly cycles of chemotherapy consisting of ifosfamide, vincristine and actinomycin D were given for 10 months after surgical resection. Our patient is presently alive and disease-free two years after diagnosis.
As complete tumour ablation is essential to prevent local recurrence and allow any chance of survival, the favourable outcome of our patient is probably due to the radical resection of the neoplasm.
Aggressive multimodal treatment of pleuropulmonary blastoma.
Parsons SK, Fishman SJ, Hoorntje LE, Jaramillo D, Marcus KC, Perez-Atayde AR, Kozakewich HP, Grier HE, Shamberger RC.
Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts 02115, USA.
Ann Thorac Surg 2001 Sep;72(3):939-42 Abstract quote
Pleuropulmonary blastoma is a rare intrathoracic neoplasm almost solely confined to childhood. Survival is poor.
The authors report 2 children with extensive intrathoracic disease who are long term survivors after multimodal therapy. Both children received multiagent neoadjuvant chemotherapy, followed by surgical resection to remove all gross tumor.
Postoperative chemotherapy was given to both children; radiotherapy was also given in the second case because of a question of positive tumor margins. Experience supports the use of multimodal therapy, including an aggressive surgical approach in the potentially curative treatment of this tumor.
Unexpected response of a pulmonary blastoma on radiotherapy: a case report and review of the literature.
Surmont VF, van Klaveren RJ, Nowak PJ, Zondervan PE, Hoogsteden HC, van Meerbeeck JP.
Department of Pulmonology, Respiratory Oncology Unit, University Hospital Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium.
Lung Cancer 2002 May;36(2):207-11 Abstract quote
Pulmonary blastoma (PB) is a rare malignant tumor of the lung. Treatment is primarily surgical, although, combination chemotherapy has been reported to result in objective responses in inoperable tumors or after incomplete resections.
To our knowledge, this is the first report of a very radiosensitive PB, which showed major tumor reduction after several fractions of radiotherapy without further tumor regression after additional chemotherapy with cisplatin and etoposide. The literature on the treatment of PB is reviewed.
Pulmonary blastoma: medium-term results from a regional center.
Zaidi A, Zamvar V, Macbeth F, Gibbs AR, Kulatilake N, Butchart EG.
Department of Cardiothoracic Surgery, University Hospital of Wales, Cardiff, United Kingdom.
Ann Thorac Surg 2002 May;73(5):1572-5 Abstract quote
BACKGROUND: Pulmonary blastomas are rare lung tumors that morphologically resemble fetal pulmonary structure and can exist in two forms, biphasic and monophasic. We reviewed our experience over a 12-year period with emphasis on the clinical features, management, and outcome.
METHODS: Patients with a diagnosis of pulmonary blastoma from January 1988 to July 1999 were identified from the database of the Department of Histopathology, Llandough Hospital, Cardiff. Specimens had been obtained from bronchoscopy, fine-needle aspiration, trucut biopsy, and thoracotomy.
RESULTS: Six patients were identified from 2,720 histologically proven lung cancers (0.2%). Median age was 35.5 years and sex ratio was equal. Overall, 4 patients underwent resection and are all alive (median, 43.5 months). Three of these had advanced tumors at presentation (stage IIIb or IV), two of which were successfully downstaged with neoadjuvant chemotherapy, and the third treated with postoperative radiotherapy. Nonresected cases succumbed at a median of 5.5 months.
CONCLUSIONS: Although pulmonary blastomas are rare, those affected represent a group of patients with advanced tumors for whom a coordinated approach from both oncologists and surgeons can achieve excellent medium-term results.
Pulmonary blastoma: report of five cases and identification of clinical features suggestive of the disease.
Robert J, Pache JC, Seium Y, de Perrot M, Spiliopoulos A.
Clinic of Thoracic Surgery, University Hospital, Geneva, Switzerland
Eur J Cardiothorac Surg 2002 Nov;22(5):708-11 Abstract quote
OBJECTIVE: Identification of clinical features suggestive of pulmonary blastoma (PB) through a retrospective comparison with cases of non-small cell lung cancer (NSCLC) operated during the same period.
METHODS: Between 1977 and 1999, five patients were operated for PB at Geneva University Hospital (four women and one man, aged 32-46 years - mean 36.8) versus 1913 consecutive patients (1558 men and 355 women, mean age 61.2) for primary NSCLC. In the PB subgroup (0.3%), the pulmonary tumor was single, located in an upper lobe in all but one instance, and measured between 5 and 13 cm (mean 9.6), whereas in the total NSCLC group, 27% of patients had tumors <3 cm (T1), evenly distributed in both lungs. All but one PB patients were symptomatic, compared to 45% in the NSCLC group.
RESULTS: The five patients with PB underwent curative pulmonary excisions (lobectomy in three and pneumonectomy in two) with mediastinal lymph node sampling. Pathological examination revealed extensive tumor necrosis in four, and N2 lymph node metastases in four (in the total NSCLC group, N2 disease was diagnosed in 21%). Postoperatively, three PB patients received radio- and/or chemotherapy. Four patients died between six and 30 months after the operation (mean 15), whereas 5-year survival in the NSCLC group was 32%, with a median survival of 3.7 years; the fifth patient is alive 28 months later, without any sign of recurrence.
CONCLUSIONS: Compared to operated NSCLC, PB are rare, large, and symptomatic tumors; they affect younger patients and carry a worse prognosis.
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