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Journal ArticleDOI

Carcinosarcomas of the lung: a clinicopathologic study of 66 patients.

01 Dec 1999-The American Journal of Surgical Pathology (Am J Surg Pathol)-Vol. 23, Iss: 12, pp 1514-1526
TL;DR: The histologic differences between these two types of tumor suggest that they may be different entities with similar behavior, but additional studies are warranted to investigate this hypothesis.
Abstract: Carcinosarcoma is a malignant tumor having a mixture of carcinoma and sarcoma containing differentiated mesenchymal elements, such as malignant cartilage, bone, and skeletal muscle. These tumors have been linked histogenetically to pleomorphic carcinomas; it is unclear whether their clinical behavior is significantly different. To investigate this issue, we studied 66 cases of carcinosarcomas of the lung and compared them with cases from a previously published series of pleomorphic carcinomas. Carcinosarcomas show a male-to-female ratio of 7.25:1, with a mean and median age of 65 years. They most often present as solitary masses in the upper lobes and average 7 cm in diameter. Most (62%) were endobronchial or central tumors, whereas 38% were described as peripheral. The most frequent epithelial component was squamous cell carcinoma (46%), followed by adenocarcinoma (31%) and adenosquamous carcinoma (19%), whereas sarcomatous elements most frequently included rhabdomyosarcoma, chondrosarcoma, osteosarcoma, or combinations of these elements. Survival of patients with carcinosarcomas of lung was poor, with a 5-year survival rate of 21.3%. Of several clinical and pathologic parameters, only increased tumor size (with 6 cm as the optimal cutoff point) appeared to be related to reduced survival (p = 0.0195). In comparison with patients with pleomorphic carcinoma, patients with carcinosarcomas had no significant difference in the size of their tumors (p = 1.0), stage at presentation (p = 0.883), location in the lung (p = 0.073), or their overall survival (21.3% vs 15.0%) (p = 0.1038). A significantly greater proportion of patients with carcinosarcoma had squamous cell (p = 0.004) or adenosquamous (p = 0.016) carcinoma, whereas patients who had pleomorphic carcinoma showed a significantly greater frequency of adenocarcinoma (p = 0.029) and large cell carcinoma. The histologic differences between these two types of tumor suggest that they may be different entities with similar behavior, but additional studies are warranted to investigate this hypothesis.
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Book
20 Sep 2004
TL;DR: This book will not become a unity of the way for you to get amazing benefits at all, but, it will serve something that will let you get the best time and moment to spend for reading the book.
Abstract: It sounds good when knowing the pathology and genetics of tumours of the lung pleura thymus and heart in this website. This is one of the books that many people looking for. In the past, many people ask about this book as their favourite book to read and collect. And now, we present hat you need quickly. It seems to be so happy to offer you this famous book. It will not become a unity of the way for you to get amazing benefits at all. But, it will serve something that will let you get the best time and moment to spend for reading the book.

1,858 citations

Journal ArticleDOI
TL;DR: It is shown that cytokeratin 7 and TTF-1, but not surfactant protein-A, are useful immunohistochemical markers in this setting, and shows that this group of tumors has a worse prognosis than conventional non-small cell lung carcinoma at surgically curable stages I.
Abstract: We collected 75 primary pulmonary carcinomas with pleomorphic, sarcomatoid, or sarcomatous elements to better define their clinical, histologic, and immunohistochemical profile. The patient's age ranged from 42 to 81 years (mean 65 years), and the male-to-female ratio was 9.7:1. Sixty-nine patients (92%) were smokers. Cough and hemoptysis were the most frequent presenting symptoms. Fifty-nine patients (65%) died of disease: only stage significantly predicts overall survival (p = 0.0273). Microscopically, based on the WHO criteria, 58 cases were classified as pleomorphic carcinoma (51 with an epithelial component, 7 composed exclusively of spindle and giant cells), 10 as spindle cell carcinoma, 3 as giant cell carcinoma, 3 as carcinosarcoma, and 1 as pulmonary blastoma. Immunohistochemically, in the tumors composed exclusively of spindle and/or giant cells, thyroid transcription factor-1 (TTF-1) and cytokeratin 7 were positive in 55% and 70% of the cases, respectively, whereas surfactant protein-A was always negative. In pleomorphic carcinomas with an epithelial component, cytokeratin 7, TTF-1, and surfactant protein-A were positive in the sarcomatoid component in 62.7%, 43.1%, and 5.9% of the cases, respectively, whereas they were always negative in the sarcomatous part of carcinosarcomas and blastoma. In the epithelial component of pleomorphic carcinomas, cytokeratin 7, TTF-1, and surfactant protein-A were positive in 76.4%, 58.8%, and 39.2% of the cases, respectively, whereas the same antibodies did not react with the epithelial component of carcinosarcomas; in the case of blastoma, the epithelial part of the tumor was positive for cytokeratin 7 and TTF-1, whereas it was negative for surfactant protein-A. Cytokeratin 20 was always negative. In our opinion, this study: 1) supports the metaplastic histogenetic theory for this group of tumors; 2) shows that cytokeratin 7 and TTF-1, but not surfactant protein-A, are useful immunohistochemical markers in this setting; 3) confirms that stage is at the moment the only significant prognostic parameter, as in conventional non-small cell lung carcinomas; and 4) shows that this group of tumors has a worse prognosis than conventional non-small cell lung carcinoma at surgically curable stages I, justifying their segregation as an independent histologic type in the WHO classification.

339 citations

Journal ArticleDOI
TL;DR: Targeting the epithelial—mesenchymal transition program could become a valid therapeutic strategy for these life-threatening tumors, whose sensitivity to current medical manipulation is disappointing.
Abstract: Pulmonary sarcomatoid carcinomas (PSCs) are currently defined as poorly differentiated non-small-cell carcinomas containing a component with sarcoma or sarcoma-like (spindle and/or giant cell) features. They consist of 5 major histological variants, namely pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma. The segregation of PSCs into a distinct clinicopathologic entity seems justified on the basis of morphologic, behavioral, and genotypic/phenotypic attributes. As a group, PSCs generally run an aggressive clinical course and may cause major difficulties in the differential diagnosis with other primary and secondary malignancies of the lung. At present, PSCs are believed to represent a family of carcinomas "in transition," in which diverse pathways of clonal evolution account for histological differences of a common ancestor lesion. The sarcomatous or sarcomatoid component of these tumors is thought to derive from carcinoma cells during the progression of carcinogenesis through the activation of an epithelial-mesenchymal transition program leading to sarcomatous transformation or metaplasia (conversion paradigm). Conceivably, targeting the epithelial-mesenchymal transition program could become a valid therapeutic strategy for these life-threatening tumors, whose sensitivity to current medical manipulation is disappointing.

142 citations


Cites background from "Carcinosarcomas of the lung: a clin..."

  • ...PSC presents as either a central or peripheral lesion, often in the upper lobes.(8,9,13,19,26,27,29,30) It grows by invading the bronchial tree, the pulmonary parenchyma, and the adjacent anatomical structures (mediastinum and chest wall) in the form of widely necrotic and hemorrhagic, round to bosselated large masses, soft to variably firm,(3,5,8-10,13,25,27,38,46,60) sometimes rubbery to hard....

    [...]

  • ...currently discouraged,(3) it is nonetheless worth mentioning, because it has been at the basis of the unifying concept of a pathologic continuum having overt “pure” carcinomas and “pure” pulmonary sarcomas at their extremes and including intermediate forms of carcinomas with variable amounts of sarcomatous and/or sarcomatoid elements (the so-called carcinomas “in transition”).(9,25) A substantial improvement in this still confusing nomenclature was provided by the 1999 WHO classification on lung cancers, with the statement that there existed “a group of poorly differentiated NSCLC containing a component of sarcoma or sarcoma-like elements” encompassing 5 different histological subtypes organized into 3 separate diagnostic categories: carcinomas with spindle and/or giant cells (pleomorphic carcinoma, spindle cell carcinoma, and giant cell carcinoma), carcinosarcomas, and pulmonary blastomas....

    [...]

  • ...Although combinations with angiosarcoma or malignant peripheral nerve sheath tumor are theoretically possible in pulmonary carcinosarcomas, they have not been recorded in any of the major published series of these tumors.(9) Rather, sarcomatoid carcinomas resembling angiosarcomas because of the presence of freely anastomosing channels containing erythrocytes or forming blood lakes covered by anaplastic, plump epithelioid cells, or dissecting alveolar septa in a hemorrhagic background are best viewed as pseudoangiosarcomatous carcinomas, because they lack true endothelial differentiation....

    [...]

Journal ArticleDOI
TL;DR: Clinical and radiologic correlation is needed to separate some pleomorphic carcinomas with pleural involvement from sarcomatoid malignant mesothelioma, since these poorly differentiated tumors may not express the usual immunohistochemical markers for carcinoma or mesothelialioma.
Abstract: Sarcomatoid neoplasms of the lung and pleura are rare tumors that present a complex differential diagnosis, making them challenging for surgical pathologists. In the lung, the main tumors are the sarcomatoid carcinomas, including pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma. They are characterized by histologic heterogeneity; molecular data support their origin from a pluripotent stem cell that undergoes neoplastic transformation with divergent epithelial and sarcomatous differentiation. Diagnosis is difficult in small biopsy specimens and typically requires a resection specimen. Despite the presence of sarcomatoid features, these tumors are classified as lung carcinomas. Pulmonary blastomas must be distinguished from pleuropulmonary blastomas, which are a unique type of thoracic sarcoma typically occurring in young children. In the pleura, the main tumors to consider are the sarcomatoid and desmoplastic types of malignant mesothelioma, solitary fibrous tumor, and desmoid tumor. While light microscopy is sufficient to diagnose most of these tumors, immunohistochemistry can be useful in selected settings. In particular, it can aid to confirm epithelial differentiation in spindle cell carcinomas and the presence of rhabdomyosarcoma in sarcomatoid carcinomas, mesotheliomas, or pleuropulmonary blastomas. For sarcomatoid and desmoplastic mesothelioma, keratin is the most useful stain because it can highlight invasive growth and mesothelial markers are positive in only the minority of cases. Clinical and radiologic correlation is needed to separate some pleomorphic carcinomas with pleural involvement from sarcomatoid malignant mesothelioma, since these poorly differentiated tumors may not express the usual immunohistochemical markers for carcinoma or mesothelioma.

119 citations


Cites background from "Carcinosarcomas of the lung: a clin..."

  • ...Metastases can contain both histologic components of the primary tumor; carcinoma is the most common type encountered if only 1 component is found.(4,24)...

    [...]

  • ...Squamous cell carcinoma is the most common histologic type of carcinoma (Figure 9, A), followed by adenocarcinoma (Figure 9, B) and adenosquamous carcinoma.(4,24) The sarcomatous component can consist of various patterns including pure components or mixtures of rhabdomyosarcoma, chondrosarcoma, or osteosarcoma....

    [...]

  • ...S100 will stain the chondrosarcoma component.(7,24) Dacic et al(16) analyzed loss of heterozygosity with 12 polymorphic microsatellite markers and found extensive allelic loss in both the epithelial and mesenchymal components, especially for 3p, 5q, and 17p....

    [...]

  • ...Keratin may be helpful in confirming the carcinoma component.(7,24) Adenocarcinoma components may be positive for TTF-1, and squamous cell carcinoma components may express p63....

    [...]

  • ...A central location, sometimes with endobronchial involvement, can be seen in all sarcomatoid carcinomas, but it is seen more often in carcinosarcomas and least often in pleomorphic carcinomas.(4,8,26,19,21,24) They are circumscribed and unencapsulated, with a variegated, white, tan, or gray, or hemorrhagic cut surface....

    [...]

References
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Journal ArticleDOI
15 Jun 1994-Cancer
TL;DR: The authors undertook this study to define the clinical and histologic characteristics of spindle and giant cell carcinomas of the lung and the survival and prognostic features of these tumors.
Abstract: Background. The authors undertook this study to define the clinical and histologic characteristics of spindle and giant cell carcinomas of the lung and the survival and prognostic features of these tumors. Methods. Seventy-eight cases of pleomorphic (spindle and/or giant cell) carcinoma of the lung were studied by light microscopy and immunohistochemistry to establish clinical, gross, and histologic parameters. Follow-up information was obtained from contributing physicians and analyzed by statistical means to determine prognostically significant parameters. Results. The patient population consisted of 57 men and 21 women (male to female ratio, 2.7 to 1) between the ages of 35 and 83 years (mean, 62 years). Clinically, 58 patients (80%) presented with symptoms including thoracic pain, cough, and hemoptysis, whereas 14 (18%) were asymptomatic. At the time of diagnosis, 41% of the patients had clinical Stage I lesions, 6% Stage II lesions, 39% Stage III lesions, and 12% Stage IV lesions. Histologically, foci of squamous cell carcinoma were present in 8% of the tumors, large cell carcinoma in 25%, and adenocarcinoma in 45%. The remaining 22% of neoplasms were completely spindle and/or giant cell carcinomas. Spindle and giant cell carcinomas were found together in 38% of the patients. In the 69 patients for whom follow-up information was obtained, 53 (77%) died within 7 days to 6 years after diagnosis, with a 23-month mean survival (median, 10 months) (Kaplan-Meier method). There was a significant shortening of survival for patients with tumor size greater than 5 cm, clinical stage greater than 1, and lymph node involvement. The presence of nodal metastases was the most significant single prognostic factor, whereas the presence of squamous or adenocarcinomatous differentiation did not have an impact on length of survival. Conclusions. The frequency with which spindle and giant cell carcinomas are found together, their frequent association with other histologic subtypes of lung carcinoma, and the similar clinicopathologic features of these tumors suggest that they are best regarded as one type of lung cancer called pleomorphic carcinoma. Cancer 1994; 73:2936–45.

344 citations

Journal ArticleDOI
TL;DR: 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.
Abstract: 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.

123 citations

Journal ArticleDOI
TL;DR: It is proposed that pulmonary carcinomas exhibiting evidence of epithelial differentiation in a sarcomatoid component be termed spindle-cell carcinomas and that those biphasic tumors exhibiting mesenchymal differentiation into specific tissues, such as neoplastic bone, cartilage, or striated muscle, or lacking epithelial differentiate by light microscopy, immunocytochemistry, and electron microscopy be classified as carcinosarcomas.

116 citations

Journal ArticleDOI
TL;DR: Pulmonary resection with curative intent was performed in 15 of 17 patients; however, only 4 patients were alive at 6, 8, 28, and 39 months, respectively, postoperatively, and the median survival was 1 year.

108 citations

Journal ArticleDOI
TL;DR: Sarcomatoid carcinomas of the lung are the most common pulmonary neoplasms that exhibit a composition by spindled or pleomorphic tumor cells, and the clinical course of patients with this neoplasm is aggressive, with an overall 5-year survival rate approximating 20%.
Abstract: Sarcomatoid carcinomas (SC) of the lung are the most common pulmonary neoplasms that exhibit a composition by spindled or pleomorphic tumor cells As such, many of them may be confused easily with true sarcomas diagnostically unless special immunohistological or ultrastructural analyses are performed Reactivity is expected for keratin, epithelial membrane antigen, or collagen type IV in the sarcomalike elements in SC, although it may be focal Electron microscopy often shows the presence of junctional complexes between tumor cells, with or without pericellular basal lamina and cytoplasmic skeins of intermediate filaments Current terminological preferences are such that several formerly used terms--including "spindle-cell carcinoma," "pulmonary blastoma," "squamous cell carcinoma with pseudosarcomatous stroma," "pseudosarcoma," and "carcinosarcoma"--are now encompassed by the more generic designation of "sarcomatoid carcinoma" The clinical course of patients with this neoplasm is aggressive, with an overall 5-year survival rate approximating 20%

107 citations