Sarcomatoid hepatocellular carcinoma with chondroid variant: case report with immunohistochemical findings

A case of sarcomatoid hepatocellular carcinoma (HCC) with a chondroid variant in a 72-year-old woman is reported. Histologically, the tumor consisted of typical HCC of adult type, with an adenocarcinoma-like structure and a sarcomatoid component containing multinodular chondroid foci. Transition from carcinoma to sarcomatoid component was observed. The cells containing mucus were extremely few in adenocarcinoma-like structures. Immunohistochemically, other than typical HCC, some of the chondroid cells, adenocarcinoma-like cells and sarcomatoid cells were positive for alpha-fetoprotein.     

Sarcomatoid carcinoma of the prostate: a case report

Sarcomatoid carcinoma of the prostate is a rare variant of prostatic cancer, with less than 100 cases reported in the literature up to date. Tumors are most commonly composed of an admixture of both malignant glandular and spindle cell elements. The sarcomatoid component can vary from 5 to 99%. We report a case of a 76-year old Caucasian man who underwent transurethral resection of the prostate for the treatment of bladder outlet obstruction. Histopathologic examination revealed a tumor with malignant epithelial and sarcomatous elements. The malignant epithelial component consisted of poorly differentiated adenocarcinoma (Gleason score 5+4=9/10) and the sarcomatous component was mainly composed of undifferentiated spindle cells. On immunohistochemistrythe latter expressed a positive staining for vimentin. Several cells were positively stained for cytockeratin AE3 and myoD1 while all were negative for actin, desmin and myogenin. The diagnosis of sarcomatoid carcinoma was finally made. Although sarcomatoid carcinoma of the prostate is a highly aggressive neoplasm and patients have a poor prognosis, our patient is still alive one year after diagnosis.     

Sarcomatoid hepatocellular carcinoma with hepatoblastoma-like features in an adult

A mixed epithelial and mesenchymal tumor of the liver arising in an adult is rare and is mostly classified as sarcomatoid hepatocellular carcinoma (HCC). In this study, a case of sarcomatoid HCC in an adult with hepatoblastoma (HB)-like features, which produced difficulty in the differential diagnosis between sarcomatoid HCC and mixed HB, is presented. The epithelial component of the tumor composed of poorly differentiated HCC, Edmondson's grade III, and more primitive components, which were embryonal and small cell undifferentiated components of HB-like areas. The small undifferentiated cells surrounded HCC and the embryonal component of HB-like area, and revealed transition partly to areas of rhabdomyosarcoma. A small portion of chondrosarcoma was also noted. Immunohistochemical analysis showed that HCC and the embryonal component of HB-like areas expressed alpha-fetoprotein (AFP) and cytokeratin 8. The small undifferentiated cells were negative for AFP but stained with cytokeratin 8 as well as CD56, which is a marker of primitive cells in many sarcoma and HB. It is not certain whether small undifferentiated cells belong to hepatic progenitor cells or primitive mesenchymal cells. Polymerase chain reaction-single-strand conformation polymorphism analysis for beta-catenin mutation using microdissection revealed no mutation of any components. A review was undertaken of the cases previously reported as adult hepatoblastoma without detailed immunohistochemical study and consider many of them may be sarcomatoid HCC. These primitive and sarcomatoid components would be arising from the dedifferentiation process of HCC.     

Chromophobe renal cell carcinoma with liposarcomatous dedifferentiation - report of a unique case

Sarcomatous transformation of chromophobe renal cell carcinoma (CRCC) is a well recognized phenomenon. Of the published cases with sarcomatous transformation of CRCC, none have shown liposarcomatous differentiation. Out of a cohort of 250 cases of CRCC, 19 (7.6%) showed sarcomatous differentiation. In one case (female, age 46 years), the sarcomatous component of the tumor displayed histological features of a pleomorphic liposar-coma. Light microscopic examination revealed a biphasic pattern with a chromophobe renal cell carcinoma(CRCC) and a high-grade sarcomatous component containing large pleomorphic lipoblasts. In several areas both components were intermingled. The conventional CRCC component showed classic histological features with calcifications, medium-sized polygonal cells arranged in solid-alveolar structures with raisinoid nuclei, pale-eosinophilic flocculent cytoplasm with perinuclear haloes. In addition, a microcystic-adenomatous component had luminal spaces filled with erythrocytes. The CRCC was positive with Hale''s colloidal iron-stain whereas the sarcomatous component was negative. The CRCC component was diffusely positive for cytokeratin 7, parvalbumin and racemase but negative for cy-tokeratin 20, vimentin, CD10, carboanhydrase IX and S100-protein. The pleomorphic liposarcomatous component displayed immunereactivity for CD10, vimentin, racemase and focally for carboanhydrase IX. The proliferative activity (Mib-1/Ki-67) was 5% in the CRCC and 30% in the pleomorphic liposarcomatous component. No immunereactivity for MDM2 or CDK4 was detected.This is the first reported case of a sarcomatoid CRCC where the sarcomatous component displayed features of a pleomorphic liposarcoma. The patient died from widespread metastatic disease 12 months after nephrectomy.     

Sarcomatoid carcinoma of the adrenal gland: A case report and review of literature

Reports about adrenocortical carcinomas (AC) mixed with sarcomatous areas are very rare. The terminology and pathogenesis of such biphasic tumors remain controversial. Herein, we report a case of sarcomatoid carcinoma of the adrenal gland in a 75-year-old woman who presented with left abdominal pain of one month's standing. The results of abdominal ultrasonography and computed tomography (CT) showed the presence of a large heterogeneous adrenal mass. A left adrenalectomy and complete splenectomy were performed. Histologically, the neoplasm showed areas of adrenocortical carcinoma and areas of sarcomatoid spindle cell proliferation. When examined immunohistochemically, the carcinomatous cells stained positively for S-100 protein, Melan-A protein, and neuron-specific enolase (NSE), and focally for vimentin and the cytokeratin marker MNF 116. Also, the carcinomatous cells were immunoreactive to the monoclonal antibody HMB-45. The sarcomatous component expressed vimentin, as well as other smooth and skeletal muscle markers. Liver metastases appeared 3 months postoperatively. Twelve months after removal of the primary tumor, the patient died of her disease. To the best of our knowledge, only seven cases of adrenal sarcomatoid carcinoma have been reported in the medical literature. We review the reported cases according to the 2004 classification of the World Health Organization (WHO) of lung tumors, and highlight the histogenesis, diagnosis, and clinical course of this very aggressive tumor.     

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.”     

Thymic carcinosarcoma consisting of squamous cell carcinomatous and embryonal rhabdomyosarcomatous components. Report of a case and review of the literature

A case of thymic carcinosarcoma in an 83-year-old Japanese man is presented. He died of superior vena cava syndrome caused by a rapidly enlarged anterior mediastinum tumor eight months after initial symptoms. Autopsy revealed a 16 x 12 x 25 cm-sized, tan yellow, whitish tumor with a multinodular and microcystic appearance located in the left anterior mediastinum, which involved the residual thymus. The tumor had directly invaded the left pleura, and had metastasized to the right lung and spleen. Histologic examinations of the primary tumor showed a sarcomatous component consisting of racquet- or spindle-shaped cells with cross striations, and small nests of atypical squamous cells scattered throughout the tumor; neither transition between the two components nor intermediate cells with both epithelial and mesenchymal features was seen. Electron microscopic and immunohistochemical examinations confirmed the rhabdomyomatous differentiation of the sarcomatoid component. To our knowledge, there have been only two reported cases showing histologic features similar to the present tumor. For the histogenesis of thymic carcinosarcoma, we propose two hypotheses. The first is that sarcomatous cells are derived from carcinomatous cells by tumoral metaplasia. Secondly, that this type of tumor originates from thymic primitive cells with multidirectional differentiation potential. In accordance with the latter, we consider that the present tumor originated from thymic primitive cells. Thymic carcinosarcoma is a highly malignant tumor, and most patients die within a year. Appropriate therapies must be developed.     

Thymic Carcinosarcoma Consisting of Squamous Cell Carcinomatous and Embryonal Rhabdomyosarcomatous Components

A case of thymic carcinosarcoma in an 83-year-old Japanese man is presented. He died of superior vena cava syndrome caused by a rapidly enlarged anterior mediastinum tumor eight months after initial symptoms. Autopsy revealed a 16 × 12 × 25 cm-sized, tan yellow, whitish tumor with a multinodular and microcystic appearance located in the left anterior mediastinum, which involved the residual thymus. The tumor had directly invaded the left pleura, and had metastasized to the right lung and spleen. Histologic examinations of the primary tumor showed a sarcomatous component consisting of racquet- or spindle-shaped cells with cross striations, and small nests of atypical squamous cells scattered throughout the tumor; neither transition between the two components nor intermediate cells with both epithelial and mesenchymal features was seen. Electron microscopic and immunohistochemical examinations confirmed the rhabdomyomatous differentiation of the sarcomatoid component. To our knowledge, there have been only two reported cases showing histologic features similar to the present tumor. For the histogenesis of thymic carcinosarcoma, we propose two hypotheses. The first is that sarcomatous cells are derived from carcinomatous cells by tumoral metaplasia. Secondly, that this type of tumor originates from thymic primitive cells with multidirectional differentiation potential. In accordance with the latter, we consider that the present tumor originated from thymic primitive cells. Thymic carcinosarcoma is a highly malignant tumor, and most patients die within a year. Appropriate therapies must be developed.     

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 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.     

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.     

Chromophobe renal cell carcinoma with heterologous (liposarcomatous) differentiation: a case report

A 44-year-old woman presented with right flank mass of 6 months duration. A right side renal tumor was diagnosed, and a radical nephrectomy was performed. Histopathological examination showed chromophobe renal cell carcinoma (CRCC) with sarcomatoid transformation. The sarcomatous component contained large pleomorphic lipoblasts. The CRCC was positive for Hale's colloidal iron stain, whereas the sarcomatous component was negative. Based on histopathological and immunohistochemical findings, a diagnosis of sarcomatoid CRCC with heterologous liposarcomatous differentiation was made. To the authors' knowledge, this is the second reported case of a sarcomatoid CRCC where the sarcomatous component displayed features of liposarcoma. The case has been reported for its rarity.     

Expression of epithelial markers in sarcomatoid carcinoma: an immunohistochemical study

Thirty-four cases of sarcomatoid carcinoma with minimal epithelial components (SC) and six cases of sarcomatous tumour without any epithelial component (ST) in various organs were studied by the immunoperoxidase technique for the expression of epithelial markers, cytokeratins and epithelial membrane antigen (EMA). Employing antibodies against both high and low molecular weight cytokeratins, sarcomatoid components in 30 examples of SC were stained positively. Epithelial membrane antigen was demonstrated in 19 out of 34 SC. The positive cells for epithelial markers within sarcomatoid components in some cases of SC, which were regarded as originating from squamous cell carcinoma, tended to be seen less frequently than in the tumours derived from adenocarcinoma or transitional cell carcinoma. In six cases of ST, stain for EMA was negative and stain for cytokeratins was positive in three examples. The immunohistochemical examination of epithelial markers in the tumours of these types may be of value in differentiating these tumours from true sarcomas.     

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.     

The extracellular matrix in sarcomatoid carcinomas of the breast

The distribution of the main extracellular matrix components has been investigated immunohistochemically in four cases of breast sarcomatoid carcinomas. The histogenesis of these tumours is still unclear, but most evidence suggests that the sarcomatous component originates from mesenchymal conversion of carcinomatous cells. We found that carcinomatous portions of the tumours were associated with linear basement membrane-like deposition of laminin, type IV collagen and heparan sulphate proteoglycan that partially circumscribed the epithelial nests. The sarcomatous components produced an extracellular matrix immunoreactive to fibronectin, type I, III and VI collagens and tenascin. However, in two cases, in some sarcomatous areas, focal pericellular staining for antibodies to laminin and type IV collagen was seen. These results indicate that modifications observed in the double tissue component of these tumours involve not only the cell shape and the cytoskeleton, but also the components of the extracellular matrix. The significance of these findings and their relevance in the understanding of the phenotypic pattern changes of these biphasic tumours are discussed.     

Sarcomatoid carcinoma with components of small cell carcinoma and undifferentiated carcinoma of the gallbladder

We report a case of sarcomatoid carcinoma with components of small cell carcinoma and undifferentiated carcinoma of the gallbladder. An 84-year-old woman was admitted to our university hospital with right upper abdominal pain and back pain. Clinical diagnosis of a gallbladder tumor was made based on the findings of abdominal ultrasonography, computed tomography and endoscopic retrograde cholangiopancreatography, and a cholecystectomy was carried out. On gross examination a pedunculated polypoid tumor protruded into the lumen of the gallbladder. Histologically the tumor was composed of carcinomatous and sarcomatous components; the carcinomatous component consisted mainly of small cell carcinoma and undifferentiated carcinoma. In general, the carcinomatous component of sarcomatoid carcinoma of the gallbladder consists of adenocarcinoma, and there have only been two previously reported cases in which the carcinomatous component consisted of small cell carcinoma or undifferentiated carcinoma. Because the patient's prognosis may be influenced by the peculiar carcinomatous component in such cases, it is important to accumulate case reports that clarify their clinicopathological features.     

Immunohistochemistry of gliosarcoma with liposarcomatous differentiation

A case of gliosarcoma composed of glioblastoma and liposarcoma is presented. A 70-year-old Japanese man was admitted to hospital because of dysarthria and aphasia. Magnetic resonance imaging indicated a brain tumor located in the temporal–parietal area of the left hemisphere. He rejected any therapy and died of respiratory failure. At autopsy the tumor was well-demarcated with firm consistency and myxoid appearance, accompanied by necrosis and hemorrhage. Microscopically the tumor consisted of both glial and sarcomatous components, compatible with a gliosarcoma. Lipoblast-like tumor cells were identified in the sarcomatous area. Glial component was observed in the periphery and was diffusely positive for CD56 and S100 protein and focally for glial fibrillary acidic protein. Only a small number of tumor cells in the sarcomatous area expressed neurogenic markers. Lipoblast-like tumor cells were positive for S100 protein but negative for any other neurogenic markers. A significant number of tumor cells were positive for retinoblastoma protein (pRB) in the glial area, whereas only a few of them were positive in the sarcomatous area, indicating alteration of pRB in sarcomatous component. The present tumor is a rare gliosarcoma with liposarcomatous differentiation; alteration of pRB may play a role in sarcomatous transformation of glial component.     

Pulmonary carcinosarcoma: immunohistochemical and ultrastructural studies

A case of pulmonary carcinosarcoma in a 68-year-old male patient is reported. The tumor in the resected left upper lobe extended mainly endobronchially, invading the normal bronchial lumina and mucosa. The carcinomatous component consisted of poorly differentiated squamous cell carcinoma and was mainly located in the periphery of the tumor nests. The sarcomatous component consisted of chondrosarcoma and was mainly located in the center of the tumor nests. Tumor cells in the sarcomatous component reacted with anti-S-100 protein antibody and were surrounded with abundant homogeneous extracellular matrix staining positively with Alcian blue. The transition from the carcinomatous component to the sarcomatous component appeared to be very smooth. The tumor cells in both the carcinomatous and sarcomatous components reacted with anti-epithelial membrane antigen antibody. Ultrastructurally, the tumor cells with tonofibrils in the carcinomatous component were apposed and connected to each other by desmosomes. By contrast, in the sarcomatous component, the tumor cells had well-developed and dilated rough endoplasmic reticulum and were arranged loosely in a myxomatous matrix. Some tumor cells in the sarcomatous component had occasional tonofibrils, and were apposed and connected to each other by desmosome-like structures. It is shown for the first time, ultrastructurally and immunohistochemically, that the tumor cells in the sarcomatous component of pulmonary carcinosarcomas have features of both epithelial and mesenchymal cells. It is suggested that the sarcomatous component in the present case is derived from the carcinomatous component.     

Synchronous squamous cell carcinoma of the kidney,squamous cell carcinoma of the ureter,and sarcomatoid carcinoma of the urinary bladder: A case report

The author presents a unique case of a synchronous triple carcinoma of kidney, ureter, and urinary bladder. A 73-year-old man was admitted to our hospital because of hematuria and lumbago. Endoscopy and imaging modalities revealed a bladder tumor, a left ureter tumor, and a left kidney tumor. No other tumors were found in the body by imaging modalities. Cystectomy and left nephroureterectomy were performed. The bladder tumor was a large polypoid tumor consisting of pleomorphic sarcomatoid carcinoma (80%) and high-grade papillary urothelial carcinoma (20%), both invading the deep muscle layer. There were gradual merges between the two. The sarcomatous component was composed of malignant spindle, polygonal, and giant cells. Lymphovascular permeation was pronounced. Immunohistochemically, the sarcomatous element was positive for vimentin and various types of cytokeratins (CK), while the urothelial carcinoma element was positive for various types of CK and negative for vimentin. The ureter tumor was small and obstructed the ureter lumen. It was a pure squamous cell carcinoma without a urothelial component. The ureter tumor invaded the adventitia. A mild degree of lymphovascular invasion was recognized. Immunohistochemically, the tumor cells were positive for various types of CK but negative for vimentin. In the kidney, almost the entire kidney parenchyma was replaced by a tumor. The renal pelvis was broadly erosive but was free of apparent tumors. Histologically, the renal tumor was a pure squamous cell carcinoma without a urothelial component. Broad necrosis was present, and lymphovascular permeation was pronounced. The renal pelvis and calices were devoid of apparent tumor cells, but renal squamous cell carcinoma was present just beneath the pelvis and calices. Immunohistochemically, the kidney tumor was positive for various types of CK and negative for vimentin.