Sarcomatoid carcinoma of the colon: a case report

Sarcomatoid carcinoma is a rare biphasic tumor characterized by a combination of malignant epithelial and mesenchymal cells. We report a rare case of sarcomatoid carcinoma of the colon. A 41-yr-old woman was hospitalized with a history of melena. Total colectomy was performed under the impression of colonic carcinoma. Histologically, the tumor was composed of differentiated adenocarcinoma in superficial portion and sarcomatoid spindle cells in deeper portion with a transitional area between the two portions. The sarcomatous areas revealed polygonal and spindle-shaped anaplastic malignant cells arranged in sheet, short fascicular or haphazard pattern. Immunohistochemically, tumor cells showed a positive immunoreaction for cytokeratin, epithelial membrane antigen, and vimentin. The histopathological and immunohistochemical transitions between the adenocarcinoma area and the spindle cell area suggested that the sarcomatous elements originated from the adenocarcinoma during tumor progression.     

Sarcomatoid carcinoma of the urinary bladder: a clinicopathologic and immunohistochemical analysis of 14 patients

Sarcomatoid carcinoma of the urinary bladder is a rare entity, in which both the histogenesis and biological behavior remain controversial. We herein describe the clinicopathologic and immunohistochemical profiles of sarcomatoid carcinomas and discuss the significance of cell adhesion molecules in the development of this peculiar neoplasm. The authors examined formalin-fixed and paraffin-embedded tissue samples from 14 patients with sarcomatoid carcinoma of the urinary bladder. An immunohistochemical analysis was performed by using antibodies against epithelial and mesenchymal antigens as well as adhesion molecules. Most patients suffered from an advanced stage of the tumor, extending to the muscular layer (7 cases) or to the perivesical tissues (5 cases). Microscopically, all 14 tumors were composed predominantly of a carcomatoid component and an obviously carcinomatous component. The sarcomatoid component was composed of a mixture of spindle cells, round cells, and pleomorphic giant cells. The carcinomatous components consisted of papillary or nonpapillary high-grade transitional cell carcinoma (TCC). The zones of gradual transition between the carcinomatous and the sarcomatous elements were focally apparent in each tumor. The findings of an immunohistochemical examination indicated that both carcinomatous and sarcomatoid components expressed epithelial antigens (pankeratin or EMA), even though the staining pattern varied from case to case. As for cell adhesion molecules, the carcinomatous components were positive for E-cadherin (8 of 12), CD44s (8 of 12), and CD44v6 (6 of 12). Although the sarcomatoid components were also positive for E-cadherin (5 of 12), CD44s (4 of 12), and CD44v6 (3 of 12), these rates were lower than those in the carcinomatous components. Six patients died of their disease between 5 and 36 months after the diagnosis was made. The recognition of sarcomatoid carcinomas has important therapeutic and prognostic implications. It seems appropriate to treat these neoplasms in the same manner as conventional high-grade TCCs with similar degrees of invasion. We consider that sarcomatoid carcinomas should be regarded as a high-grade carcinoma that shows a prominent pseudosarcomatous dedifferentiation. The sarcomatoid component of sarcomatoid carcinomas may result from either anaplastic changes or dedifferentiation related to the process of losing cell adhesion molecules.     

An autopsy case of hepatic sarcomatoid tumor: Immunohistochemical comparison with a sarcomatous component of hepatocellular carcinoma

A case of primary hepatic tumor exclusively composed of malignant cells with sarcomatous features is described and compared immunohistochemically with two cases of hepatocellular carcinoma (HCC) with a sarcomatous component. More than 30% of HCC cells were positively stained with anti-cytokeratin (CAM5.2), anti-albumin, anti-fibrinogen and anti-α₁-antitrypsin antibodies, and some with anti-epithelial membrane antigen. The present sarcomatoid tumor and the sarcomatous component with HCC showed similar immuno-histochemistry; many tumor cells were strongly immuno-reactive for vimentin and some positive for cytokeratin, albumin, fibrinogen and u,-antitrypsin. Other immunohistochemical markers, indicating specific differentiations to lineage of macrophages, muscle cells, glial cells, endothelial cells and so forth, were not detected in sarcomatous tumor cells of all cases. These findings suggest that the present sarcomatoid tumor would belong to an anaplastic sarcomatous variant of HCC.     

Sarcomatoid carcinoma (so-called pseudosarcoma) of the larynx simulating malignant giant cell tumor of soft parts. A case report

A tumor of the larynx composed of a small infiltrating and well-differentiated squamous cell carcinoma and a large polypoid sarcomatous mass showing the histologic features of the malignant giant cell tumor of soft parts (MGCT) is reported. Other types of sarcomatous components have been described with squamous cell carcinoma (SCC) of the larynx and names such as pseudosarcoma, carcinosarcoma, and spindle cell carcinoma have been used for these peculiar tumors. Because most data support that the sarcomatous component is an unusual carcinoma transformation due to unknown factors the authors prefer the term sarcomatoid carcinoma. Sarcomatoid carcinomas having the features of MGCT have been described in multiple epithelial organs. Therefore, an extensive search for a carcinoma component always should be carried out in tumors arising in any organ, including the larynx, and showing the typical histologic features of MGCT.     

Sarcomatoid carcinoma with small cell carcinoma component of the urinary bladder: a case report with review of the literature

Sarcomatoid carcinoma of the urinary bladder is an uncommon neoplasm characterized histopathologically by the presence of malignant spindle cell and epithelial components. Albeit extremely rare, sarcomatoid carcinoma with small cell carcinoma has been reported. Herein, we describe an additional case of sarcomatoid carcinoma with small cell carcinoma and squamous cell carcinoma of the urinary bladder and review the clinicopathological features of this type of tumor. An 82-year-old Japanese male presented with hematuria. Computed tomography demonstrated a large tumor in the urinary bladder. Histopathological study of the resected urinary bladder tumor showed that approximately 80% of the tumor was comprised of small cell carcinoma, and the remaining components were spindle cell proliferation (approximately 15%) and squamous cell carcinoma (5%). Both the spindle cell and squamous cell carcinoma components were intermingled with nests of the small cell carcinoma. This is the fifth documented case of sarcomatoid carcinoma with small cell carcinoma of the urinary bladder. Our review of the clinicopathological features of this type of tumor revealed that: i) elderly males are mainly affected, ii) the most common chief complaint is hematuria, iii) the epithelial component may include urothelial carcinoma, adenocarcinoma, and/or squamous cell carcinoma, and iv) the sarcomatous component is composed of spindle cell proliferation. The histogenesis of this type of tumor remains a matter of controversy. However, recent molecular analyses demonstrated a monoclonal origin of both components. This theory can account for the various types of carcinomatous components in this tumor as seen in the present case.     

Primary adrenocortical sarcomatoid carcinoma: case report and review of literature

Adrenocortical carcinoma (AC) mixed with a sarcoma or sarcoma-like component is exceptional, and only six cases have been detailed in the literature, three including osteo-, chondro-, or rhabdomyosarcoma components, and three others only showing a malignant spindle cell component. These histological subtypes, respectively called adrenal carcinosarcomas and sarcomatoid AC, represent poorly differentiated and extremely aggressive forms of carcinoma, with locoregional recurrence and metastases rapidly arising from the sarcomatous or sarcomatoid component, and death occurring in a few months. We report a case of AC in a 31-year-old man presenting as a nonfunctional tumor, with a histological biphasic pattern combining few areas of differentiated AC and extensive areas of sarcomatoid spindle cell proliferation. The patient died 3 months of locoregional and distant recurrences after surgery despite apparently total tumor resection and VP16-cisplatinum chemotherapy. This case underlines the necessity to identify and isolate these carcinoma’s subtypes with worse prognosis and the difficulties to distinguish them from metastatic carcinomas and retroperitoneal sarcomas, in relation to the particular adrenal cortex immunoprofile. According to the World Health Organization principles of terminology, we suggest these tumors be collectively classified as “adrenal sarcomatoid carcinomas,” a designation that tends to unify all carcinomas with “pleomorphic, sarcomatoid, or sarcomatous elements.”     

Pleomorphic carcinoma of the lung expressing podoplanin and calretinin

Pleomorphic carcinoma (PC) of the lung is classified as a subtype of sarcomatoid carcinoma of the lung, and peripheral PC is sometimes difficult to differentiate from the sarcomatoid component of mesothelioma. An 80‐year‐old man was referred to National Cancer Center Hospital East because a chest X‐ray showed an abnormal shadow. CT scans of the chest indicated two solid masses located in the right lower lobe, and CT‐guided needle biopsy yielded spindle‐shaped tumor cells that were immunoreactive for both podoplanin and calretinin. Mesothelioma could not be ruled out, and the tumors were surgically resected to facilitate definitive pathological diagnosis. Both tumors were composed of undifferentiated carcinoma, bronchioloalveolar carcinoma and spindle cell carcinoma, and spindle cell component was immunoreactive for podoplanin and calretinin. Ten other tumors diagnosed as peripheral PC were also tested for podoplanin and calretinin expression. The sarcomatoid component in four of the 11 cases (36%) was immunoreactive with podoplanin, and it was calretinin positive in nine of the 11 cases (82%). When making the differential diagnosis between PC and the sarcomatoid component of mesothelioma, care is required in diagnosing biopsy specimens of peripheral lung spindle‐cell tumors that are positive for both podoplanin and calretinin.     

Sarcomatoid collecting duct carcinoma.

BACKGROUND: Collecting duct carcinoma has recently been recognized as a distinct entity among renal cell carcinomas and shows aggressive clinical behavior. DESIGN: A fatal case of collecting duct carcinoma with sarcomatoid features in an 80-year-old man is described, and the results of immunohistochemistry and flow cytometric analysis are presented. RESULTS: The tumor, which was white and had infiltrating margins in the cortex and medulla, consisted of adenocarcinomatous and sarcomatous components. The adenocarcinomatous component was characterized by tubular arrangement of atypical cells with high-grade nuclear features and desmoplastic stroma. The sarcomatous component had a malignant fibrous histocytoma-like appearance. The tumor cells in the adenocarcinomatous area were positive for cytokeratin 19, Ulex europaeus agglutinin I, peanut agglutinin, and soybean agglutinin. The tumor was DNA diploid. The patient died of disease 3 months after diagnosis. CONCLUSION: This study suggests that sarcomatoid collecting duct carcinoma may be one of the most aggressive tumors of the kidney.     

Sarcomatoid carcinoma (carcinosarcoma) of the esophagus with extensive areas of osseous differentiation: a case report

A 46 year old male presented with progressively increasing dysphagia and weight loss. Esophagoscopy showed a large polypoidal growth involving the middle segment of esophagus. Histologically bulk of the tumour had a sarcoma-like appearance composed of spindly pleomorphic cells along with extensive areas of bone formation. The epithelial element was represented by a small area of squamous cell carcinoma. One of the draining lymph nodes also showed small islands of squamous cell carcinoma. A diagnosis of sarcomatoid carcinoma was made. Sarcomatoid carcinoma of the esophagus, also termed carcinosarcoma, pseudosarcoma, and spindle cell carcinoma is an unusual malignant tumour of the esophagus. The proportion of carcinomatous and sarcomatous component may vary from case to case. Rarely, the sarcomatous component may exhibit osseous differentiation as in our case.     

Pure sarcomatoid carcinoma of maxillary sinus and nasal cavity simulating malignant fibrous histiocytoma

Although a few cases of sinonasal carcinoma with focal sarcomatous differentiation have been reported, pure sarcomatoid carcinoma has not been reported in the English literature. Imaging studies and gross inspection in a 60-year-old man with left-sided face pain revealed a mass in the left maxillary sinus and nasal cavity. A large incisional biopsy specimen from the nasal cavity revealed proliferation of malignant spindle and round cells with a malignant fibrous histiocytoma (MFH) pattern. Tumor giant cells were scattered, and there were areas of a vague storiform pattern. Mitotic figures were numerous. Carcinomatous component was not recognized. The histologic diagnosis was storiform-pleomorphic MFH. Tumor cells were positive for pancytokeratins AE1/3, KL-1, and CAM5.2 and cytokeratin (CK) 18, vimentin, CD68, p53, Ki-67 (labeling, 90%), α?-antitrypsin, and α?-antichymotrypsin and negative for pancytokeratin WSS, CK 34βE14, CK7, CK8, CK14, CK19, CK20, epithelial membrane antigen, S-100 protein, desmin, α-smooth muscle actin, CD34, HMB45, chromogranin, synaptophysin, myoglobin, CD45, CD30, and CD15. Because keratins were positive in tumor cells, a diagnosis of sarcomatoid carcinoma simulating MFH was made. The patient was treated with chemoradiation without significant effect and died 9 months after initial examination.     

Spindle basaloid squamous carcinoma of the upper aerodigestive tract: immunohistochemical and clinicopathological study of three cases

We describe the clinicopathological and immunohistochemical features of three spindle (sarcomatoid) basaloid squamous carcinomas in three men aged 73, 69, and 59 years with a history of tobacco and alcohol abuse. Two tumors were located in the hypopharynx and one was located in the nasal cavity. The three tumors have a pedunculated polypoid appearance. Histologically, they were composed of conventional basaloid squamous carcinomas with extensive malignant spindle cell proliferation, comprising more than 50% of the tumor. The sarcomatoid component demonstrated immunoreactivity with one or more epithelial markers. One case in addition expressed CD99 and Bcl-2 and was originally diagnosed as monophasic synovial sarcoma; however, a subsequent biopsy disclosed basaloid squamous cell carcinoma with sarcomatoid stroma. Two patients were treated with surgery and radiation whereas one refused therapy. The patients were alive 14 (case patient 1), 10 (case patient 2), and 8 (case patient 3) months after diagnosis. In the absence of evidence from immunohistochemical or electron microscopy studies, a polypoid malignant spindle cell tumor of a mucosal surface of the upper aerodigestive tract should be considered a sarcomatoid carcinoma until proven otherwise. The type of epithelial component would determine the subtype of sarcomatoid carcinoma.     

Fine-needle aspiration biopsy of metaplastic carcinoma of the breast: Report of a case with abundant myxoid ground substance

Metaplastic carcinoma (MC) is an uncommon neoplasm of the breast. There are several variants of MC depending on the dominant histologic pattern. The components include overt infiltrating ductal carcinoma, extensive squamous differentiation and spindle cell proliferation with or without chondroid or osseous heterologous elements. In FNA smears, only 57% of cases show both ductal carcinoma and metaplastic component. Thus, in almost one half of the cases, the diagnosis is not possible by FNA. Often it is difficult to define the epithelial or sarcomatous character of malignant cells. We describe a case of metaplastic carcinoma of the breast studied by fine-needle aspiration cytology in which myxoid ground substance was the dominant feature in the cytology smears. The rest of the material was composed of scanty isolated atypical cells with large and irregular nuclei. It is important to bear in mind the diagnosis of MC and make a careful search for atypical cells when the cytological smears are mainly composed of myxoid ground substance. Diagn Cytopathol 1996;14:325–327. © 1996 Wiley-Liss, Inc.     

A Case of Pulmonary Adenocarcinoma with Sarcomatous Elements Initially Manifested as a Submucosal Tumor of the Stomach

A case of pulmonary adenocarcinoma, which was initially manifested as a gastric submucosal tumor, is presented. Endoscopy showed a submucosal tumor in the fundic region in a 79-year-old Japanese woman. Initial biopsy specimens of the stomach revealed atypical spindle cell proliferation, suggesting primary leiomyosarcoma of the stomach. However, biopsy specimens obtained one year later were diagnosed as malignant lymphoma or malignant histiocytosis of the stomach. Autopsy revealed a large necrotic lesion in the right S₈ region with metastases in multiple organs. Microscopy demonstrated well to moderately differentiated adenocarcinoma containing spindle or pleomorphic sarcomatous elements. Metastatic nodules including the gastric tumors all showed sarcomatous elements with no epithelial component. Immunohistochemistry showed positive reactions for keratin, epithelial membrane antigen, and carcinoembryonic antigen in areas of carcinoma, whereas most of the sarcomatous elements revealed no positivity for any of the antibodies used, except for focal keratin and EMA positivity in the primary site. This is a rare case of pulmonary adenocarcinoma with sarcomatous elements discovered as a gastric tumor at initial diagnosis, resulting from metastasis of the sarcomatous element in the submucosa.     

A case of pulmonary adenocarcinoma with sarcomatous elements initially manifested as a submucosal tumor of the stomach.

A case of pulmonary adenocarcinoma, which was initially manifested as a gastric submucosal tumor, is presented. Endoscopy showed a submucosal tumor in the fundic region in a 79-year-old Japanese woman. Initial biopsy specimens of the stomach revealed atypical spindle cell proliferation, suggesting primary leiomyosarcoma of the stomach. However, biopsy specimens obtained one year later were diagnosed as malignant lymphoma or malignant histiocytosis of the stomach. Autopsy revealed a large necrotic lesion in the right S8 region with metastases in multiple organs. Microscopy demonstrated well to moderately differentiated adenocarcinoma containing spindle or pleomorphic sarcomatous elements. Metastatic nodules including the gastric tumors all showed sarcomatous elements with no epithelial component. Immunohistochemistry showed positive reactions for keratin, epithelial membrane antigen, and carcinoembryonic antigen in areas of carcinoma, whereas most of the sarcomatous elements revealed no positivity for any of the antibodies used, except for focal keratin and EMA positivity in the primary site. This is a rare case of pulmonary adenocarcinoma with sarcomatous elements discovered as a gastric tumor at initial diagnosis, resulting from metastasis of the sarcomatous element in the submucosa.     

Sarcomatoid carcinoma involving the nasal cavity and paranasal sinus: a rare and highly progressive tumor

Sarcomatoid carcinoma of nasal cavity and paranasal sinuses is an extremely rare malignant neoplasm. We report a case of sarcomatoid carcinoma arising in the left-side nasal cavity and paranasal sinuses in a 65-year-old man who was hospitalized for left-side nasal epistaxis, odor sensation. Histopathologic examination revealed the tumor was composed of pleomorphic spindle and round cells with frequent mitosis, and no carcinomatous component was recognized. Immunohistochemistry revealed coexpression of cytokeratin and vimentin by the tumor cells, whereas expressions of EMA, S-100, HMB-45, Melan-A, LCA, MyoD1, CD34, CK7, F8 and desmin were negative. The diagnosis was sarcomatoid carcinoma of nasal cavity and paranasal sinuses. The neoplasm was partial resected by nasal endoscopic surgery following chemoradiation therapy immediately. But it was still progressing rapidly, and had a poor prognosis.     

A case report of gastric carcinosarcoma with rhabdomyosarcomatous and neuroendocrinal differentiation

We report herein an unusual gastric carcinosarcoma with rhabdomyosarcomatous and neuroendocrinal differentiation in a 63-year-old Japanese male. The tumor was a pedunculated large polypoid tumor (7 x 6.5 x 3.5 cm) located in the pylorus. Histologically, it invaded to the subserosa and was composed of both adenocarcinomatous and sarcomatous components. Adenocarcinomatous foci generally showed tubular to solid patterns and occupied the parts facing the gastric lumen, while the sarcomatous components showed a generally irregular and solid arrangement. There were transitions between the sarcomatous and carcinoma elements. In addition, carcinoma cells with a cord-like or trabecular arrangement similar to that seen in endocrine carcinoma expressed chromogranin A, and were mainly observed in an intermediate area between the adenocarcinomatous and sarcomatous foci. The sarcomatous areas were mainly composed of spindle cells and occasionally contained a sarcomatous component showing rhabdomyosarcomatous differentiation. This is an interesting case to consider how the variety of cell type appeared in such a type of tumor in the stomach.     

Yolk sac differentiation in urothelial carcinoma - A rare variant originating from aberrant differentiation of sarcomatoid components

Yolk sac differentiation occurs in somatic neoplasms of the gastrointestinal and gynecologic tracts; it has rarely been reported in urothelial carcinoma. Here, we report three cases of yolk sac differentiation in sarcomatoid urothelial carcinoma. The epithelioid component of the sarcomatoid urothelial carcinoma showed divergent differentiation, including squamous, conventional glandular, small cell carcinoma, and yolk sac components. The sarcomatoid component showed malignant spindle cells admixed with focal chondroid and rhabdoid elements. In all three cases, the yolk sac areas were admixed with the sarcomatoid component and showed a glandular pattern, with vacuolated, eosinophilic cytoplasm. These areas were positive for SALL4, variably positive for glypican 3 and AFP, and negative for the conventional urothelial markers GATA3, p63, and 34βE12. Yolk sac differentiation is an extremely rare occurrence in sarcomatoid urothelial carcinoma.     

Sarcomatoid carcinoma of the urinary tract

Sarcomatoid carcinoma is a rare variant of malignant tumor arising from the urinary tract. This tumor had been termed carcinosarcoma because of its carcinomatous and sarcomatous components. There is still some confusion in the terminology between true carcinosarcoma and sarcomatoid carcinoma; however, the latter is now regarded as primarily a malignant epithelial tumor with pseudosarcomatous transformation.
Four cases of sarcomatoid carcinoma arising from the urinary tract are reported. The patients were a 77 year old female, and three males aged 62, 69 and 80 years. All but the eldest patient complained of gross hematurla. Surgical removal was performed in the younger three cases, and an autopsy was done in the remaining case. All the tumors were macroscopically polypoid. Histopathologic examination revealed fasciculated spindle-cell tumors with myxold stroma or malignant fibrous histiocytoma-like spindle cell tumors. The epithelial nature was proven in these sarcomatous cells by immunohistochemical andlor electron-microscopic examinations. Only a small amount of squamous cell carcinoma components was also evident in the latter three cases. Although the younger three patients were alive at 44, 23 and 39 months'follow-up, respectively, constant careful monitoring Is recommended.     

Myogenic expression in esophageal polypoid tumors

Four cases of esophageal polypoid tumors composed of squamous cell carcinoma and spindle cell sarcomatous components were investigated. Squamous cell carcinoma was consistently present in the base of the polypoid lesions in all four cases and was also intermingled with spindle-shaped sarcomatous cells in two cases. Metastases in the lymph nodes were observed in two cases: one was squamous cell carcinoma with a sarcomatous component and the other consisted of a pure sarcomatous component. All tumors involved at least the muscularis mucosae. In the sarcomatous region, the tumor was composed of highly anaplastic cells with or without forming interlacing fascicles. Pleomorphism was marked and bizarre giant cell forms were frequent. Mitoses were frequently present. Immunohistochemical study revealed that the anaplastic cells in the sarcomatous component in all cases were immunoreactive to desmin, muscle actin, vimentin, and alpha 1-antichymotrypsin, but were negative for cytokeratin, even in the metastatic tumors of the lymph nodes. The immunohistochemical results favor myogenic differentiation of the anaplastic cells, and these tumors were considered to be true carcinosarcomas composed of squamous cell carcinoma and leiomyosarcoma.     

Spindle cell squamous carcinoma of the oral region

Summary Six cases of spindle cell squamous carcinoma (SCSC) of the oral cavity were studied clinicopathologically, immunohistochemically and ultrastructurally to summarize the clinicopathological features of this rare neoplasm and to discuss the debatable histogenesis of the sarcomatoid component and the differential diagnosis of SCSC. The mean age of the patients was 72 years and the female to male ratio was 1:2. Four of them had a history of irradiation for pre-existing squamous cell carcinoma. One patient died of SCSC. While clinical and histological prognostic factors of SCSC could not be determined, it was shown that radical surgery resulted in good prognosis. The epithelial nature of the sarcomatoid component of SCSC was clearly revealed by a combination of immunohistochemical staining for keratins and electron microscopic demonstration of tonofilament-like filaments and/or desmosome-like structures. Together with electron microscopic evaluation of the tumour cells, immunohistochemical characterization of tumour cells using antibodies to keratin, vimentin, glial fibrillary acidic protein and S-100 protein is very helpful in differentiating SCSC from true spindle cell sarcoma, melanoma and malignant myoepithelioma. In the immunohistochemical differential diagnosis of SCSC, it is important to remember that SCSC should not be ruled out of the differential diagnosis by a positive reaction for vimentin in sarcomatoid tumour cells. Absence of staining for keratin in the sarcomatoid tumour cells does not always exclude SCSC, because some SCSCs show immunoreactivity of keratin in their sarcomatoid components only with some anti-keratin antibodies. Different kinds of anti-keratin antibodies should be applied in the differential diagnosis of SCSC.