Adrenal carcinosarcoma--a case report.

Carcinosarcoma of the adrenal gland is an extremely rare variant of adrenocortical carcinoma, characterized by a biphasic pattern of carcinoma and sarcoma-like components. We report a case of adrenal carcinosarcoma occurring in a 61-year-old Korean man who presented with right flank pain of one month duration radiating to the back and right iliac crest. The tumor measured 12 x 12 x 7 cm and adhered to the liver and right upper pole of the kidney. The carcinomatous component of the tumor showed polygonal cells in a broad anastomosing trabecular pattern with delicate slit-like vascular channels; the sarcomatous component showed uniform spindled cells in a fascicular pattern. Both the carcinomatous and sarcomatous portions of the tumor were positive for pan-cytokeratin, and vimentin stained only the sarcomatous areas.     

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.     

Fine-needle aspiration cytology of carcinosarcoma of the parotid gland: Cytohistological and immunohistochemical findings

Carcinosarcoma or true malignant mixed tumor of salivary glands is a very rare neoplasm which shows malignant features of the epithelial and mesenchymal components. We hereby present cytological, pathological, and immunohistochemical findings of one such case, first examined by fine-needle aspiration cytology. The aspirate was cell-rich and consisted of large cell clusters which at low magnification showed an arrangement reminiscent of a jigsaw puzzle. An amorphous substance was observed in the middle of some of these clusters. There were also numerous dissociated cells which often displayed marked atypia. Histology showed a tumor with malignant epithelial and mesenchymal components. the carcinomatous areas consisted of cells arranged in solid nests or glandular structures. the sarcomatous areas showed osteosarcoma, low-grade chondrosarcoma, and undifferentiated components. On immunohistochemistry, the tumor coexpressed epithelial and mesenchymal markers. Cytokeratin, keratin, and epithelial membrane antigen were mainly localized to the carcinomatous portion, whereas vimentin and neuron-specific enolase were restricted to the sarcomatous areas. S-100 protein was positive in both. Our findings support previous views that this tumor may be related to pleomorphic adenoma. © 1995 Willey- Liss, Inc.     

Malignant mesothelioma of the tunica vaginalis testis: a case with a predominant sarcomatous component

We present a case of malignant mesothelioma (MM) of the tunica vaginalis testis. A 64-year-old man was referred for an operation on a right hydrocele that later proved to be a tumor during surgery. The tumor was malignant with a biphasic pattern of epithelial and sarcomatous components. The latter component was predominant. Cuboidal or columnar cells formed irregular tubular structures in the epithelial component. In contrast, spindle-shaped or polygonal cells formed intricate structures with stromal connective tissues in the sarcomatous component. Immunohistochemical staining revealed that the tumor was mesothelial in origin and positive for cytokeratin, vimentin, HBME-1 antigen and calretinin. In general, MM occur in the pleura or peritoneum; those originating in the tunica vaginalis testis are very rare and represent less than 5% of all MM. In addition, MM in the tissues usually consist primarily of an epithelial component. According to previous reports tumors with a predominant sarcomatous component are extremely rare. In general, a sarcomatous component predicts poor prognosis and our case does, in fact, deteriorate over time. Our case suggests that despite its low incidence, MM must be considered when a case is diagnosed as hydrocele testicle.     

Biphasic intra-abdominal desmoplastic small cell tumor in a patient with proximal spinal muscular atrophy

A case is reported of intra-abdominal desmoplastic small cell tumor (IDSCT) with biphasic histologic features in a patient with proximal spinal muscular atrophy. The tumor was composed of small epithelial cell nests with spindle cell sarcomatous areas. Both areas were surrounded by a desmoplastic stroma. Immunohistochemical studies revealed reactivity for low molecular weight cytokeratin, epithelial membrane antigen, vimentin, desmin and Leu-7 in both areas. Electron microscopic examination demonstrated paranuclear aggregates of intermediate filaments, zonula adherens and basement membrane-like material in the epithelial cells, while spindle cells in the tumor had fewer intracytoplasmic organelles. However, intermediate or transitional forms of both types of tumor cells were frequently observed. Although IDSCT are known to express multi-phenotypes immunohistochemically, attention should be paid to the broad spectrum of cell morphology in these tumors.     

Malignant mixed müllerian tumors: an immunohistochemical study of 47 cases, with histogenetic considerations and clinical correlation.

Forty-seven cases of malignant mixed müllerian tumors were reviewed histologically and studied immunohistochemically with three major objectives: to analyze the histogenetic relationship between the carcinomatous and sarcomatous components of these neoplasms, to ascertain the practical role of immunohistochemical studies in diagnosis and classification, and to determine the prognostic significance of immunohistochemically verified rhabdomyoblastic and neuroendocrine differentiation. Epithelial differentiation (cytokeratin and/or epithelial membrane antigen expression) was confirmed in all carcinomatous components; within the sarcomatous areas, it was identified among individual cells (60% of cases) and within poorly formed clusters of cells (57% of cases). There was a statistically significant tendency for concordant expression of alpha-1-antichymotrypsin, Leu-M1, S-100, Leu-7, and neuron-specific enolase between the carcinomatous and sarcomatous components of individual cases. These two findings provide evidence of common origin for the sarcomatous and carcinomatous components of these neoplasms. Histologic review of metastases in 21 cases revealed a biphasic composition in the majority of metastatic lesions (62%), another feature that further supports a common origin for the two components. From a practical standpoint, immunohistochemistry may be helpful in accentuating the biphasic pattern of these neoplasms and in verifying the presence of rhabdomyoblastic differentiation. In most cases, however, careful morphologic examination and thorough sampling will suffice for correct diagnosis and subclassification. The presence of heterologous, rhabdomyoblastic, or neuroendocrine differentiation did not have a statistically significant influence on survival; the last of these was associated with a tendency for a more rapidly fatal course.     

Synovial sarcoma of the abdominal wall

Summary A synovial sarcoma of the abdominal wall in a 56-year old woman showed the typical features of this tumor type. Histologically a characteristic biphasic cellular pattern with epithelium-like cell complexes and sarcomatous spindle cell areas was found. The histochemical examination revealed that tumor cells synthesize glycoproteins and weakly acid glycosaminoglycans (mainly hyaluronic acid). Electron microscopically the tumor cells in epithelium-like cell islets were sometimes arranged in gland-like formations with microvilli at the luminal side, specialized intercellular junctions and a peripheral basement membrane-like condensation of the ground substance. There was no fundamental cytological difference between cells of epithelium-like and spindle cell areas. Generally the tumor cells imitated cells of the synovial membrane and we found no evidence for origin from cells of the nerve sheath. Because of the submicroscopic relationship and histochemical similarities of synovial sarcomas and mesotheliomas we suggest that they should be united in a group of sarcomas with possible biphasic cellular pattern, while preserving their clinicopathologic definition.     

Carcinosarcoma of the urinary bladder: A distinct variant characterized by small cell undifferentiated carcinoma with neuroendocrine features

Summary The clinicopathological features of two carcinosarcomas of the urinary bladder are reported. The tumours occurred in a 64- and a 66-year-old patient presenting with haematuria and both were polypoid. The epithelial component was consistent with small cell undifferentiated carcinoma with neuroendocrine differentiation, whereas the sarcomatous component did not display specific features. The carcinomatous component showed immunohistochemical reactivity for different epithelial markers as well as for chromogranin and neuron specific enolase. Conversely, the sarcomatous cells stained strongly for vimentin and in one case for muscle actin and smooth muscle actin. The differential diagnosis of biphasic tumours of the bladder is discussed and the literature reviewed.     

Spindle cell carcinoma of the uterus

A case of spindle cell carcinoma of the uterus in a 56-year-old woman is reported. Microscopically, it showed an intimate admixture of epithelial and sarcomatous elements. The epithelial nature, especially the squamous cell nature, in sarcomatous areas was indicated by immunoreactivity for keratin and ultrastructural characters, such as bundles of tonofilaments and some cell junctions, while the tumor cells in these areas were also vimentin-positive. Furthermore, ultrastructural study and immunostaining for actin demonstrated myofilaments in tumor cells in both tumor nests and sarcomatous areas. This may impart the high degree of invasiveness of spindle cell carcinoma.     

SPINDLE CELL CARCINOMA OF THE UTERUS

A case of spindle cell carcioma of the uterus in a 56-year-old woman is reported. Microscopically, it showed an intimate admixture of epithelial and sarcomatous elements. The epithelial nature, especially the squamous cell nature, in sarcomatous areas was indicated by immunoreactivity for keratin and ultrastructural characters, such as bundles of tonofilaments and some cell junctions, while the tumor cells in these areas were also vimentin-positive. Furthermore, ultrastructural study and immunostaining for actin demonstrated myofilaments in tumor cells in both tumor nests and sarcomatous areas. This may impart the high degree of invasiveness of spindle cell carcinoma.     

Uterine adenosarcoma detected by Papanicolaou smear: a case report

Adenosarcoma is a rare uterine biphasic tumor composed of benign epithelial elements and a sarcomatous stroma. Although it is well described histologically, its cytological features are rarely mentioned in the literature. We describe a case of uterine adenosarcoma that was first detected by Papanicolau (Pap) smear. Numerous crowded clusters of spindle cells were present within a bloody background, as well as a few smaller, dyscohesive groups with cells showing high N:C ratio and oval to round nuclei with coarse chromatin and small nucleoli. A few nuclear grooves were identified. Adenosarcomas are rare lesions but should be considered in the differential diagnosis when spindled cells are noted in a pap smear.     

Gliosarcoma with liposarcomatous differentiation: the new member of the lipid-containing brain tumors family

Gliosarcoma is a rare malignant, biphasic brain tumor composed of glioblastoma multiforme and sarcomatous components. Various types of sarcomatous differentiation are described in this tumor: fibrosarcomatous, malignant fibrous histiocytoma-like, chondrosarcomatous and osteosarcomatous types. We report an extremely unusual variant of liposarcomatous differentiation in gliosarcoma in 72-year-old woman. Fat cells were presented by atypical multivacuolar and monovacuolar lipoblasts, stained positive for S100. p53 that was positive in both glial and mesenchymal cells of the tumor were negative in the lipoblasts. To the best of our knowledge, this is the first report in the literature of liposarcomatous differentiation in gliosarcoma.     

Cytopathological and immunocytochemical findings of pancreatic anaplastic carcinoma with ZEB1 expression by means of touch imprint cytology

Pancreatic anaplastic carcinoma (PAC) is rare and has an aggressive clinical course. We report an autopsy case of PAC focusing on the cytopathological characteristics of the tumor and immunocytochemical staining for vimentin, E‐cadherin, and zinc finger E‐box binding homeobox 1 (ZEB1), which markers are associated with epithelial markers of epithelial‐mesenchymal transition (EMT). A 50‐year‐old woman presented to our hospital with a chief complaint of jaundice. A pancreatic head tumor and multiple liver nodules were detected on abdominal computed tomography. Biliary cytology under endoscopic retrograde cholangiopancreatography suggested ductal adenocarcinoma. Three months after admission, she died of multiorgan failure. At autopsy, touch imprint cytology using squash preparation of the pancreatic tumor identified two different cell types; numerous isolated malignant cells with large and pleomorphic nuclei and a few clusters showing irregularly overlapped nuclei and irregular contours within the necrotic background. Immunocytochemically, isolated cells were positive for vimentin and ZEB1, and negative for E‐cadherin. Conversely, clusters were negative for vimentin and ZEB1, and positive for E‐cadherin. Histologically, the tumor was composed of sarcomatous cells with small foci of adenocarcinoma, which were consistent with a diagnosis of PAC. Immunohistochemical staining of the adenocarcinoma and sarcomatous cells corresponded to those of the clusters and isolated malignant cells, respectively. Immunostaining of these EMT markers is useful to distinguish sarcomatous cells from adenocarcinoma and can contribute to the accurate diagnosis of pancreatic tumors with EMT.     

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.     

Carcinosarcoma of the esophagus with osseous and cartilagenous production. A combined study of keratin immunohistochemistry and electron microscopy

A case of polypoid carcinosarcoma of the esophagus is presented. Histologically the bulk of the tumor consisted of a sarcomatous tissue having large foci of osseous and cartilagenous differentiation and infiltrating deeply the wall, whereas a superficially, invasive squamous cell carcinoma associated with in-situ carcinoma was located at the base and luminal surface of the polypoid tumor. Intermingling of the carcinomatous and sarcomatous elements was found only in areas where they appeared to be collided. Ultrastructurally the sarcomatous portion contained cells with fibroblastic features but with no typical epithelial characteristics. Immunoperoxidase staining of the paraffin-embedded histologic sections for keratin proteins revealed, however, some positive spindle cells indicative of epithelial nature in the sarcomatous area, but the great majority of the sarcoma cells were devoid of keratin. These combined findings strongly suggest that the sarcomatous component in our case of true carcinosarcoma is derived from mesenchymal transformation (metaplasia) of the squamous carcinoma cells. The findings were discussed in light of the previous pertinent literature.     

Carcinosarcoma of the stomach. A case report with light microscopic, immunohistochemical, and electron microscopic study

A carcinosarcoma of the stomach in a 66-year-old man is reported. The tumor which had a diameter of 4 cm was an ulcerofungating mass in the posterior wall of the antrum. Histologically it was composed of well-differentiated adenocarcinoma admixed with sarcomatous elements which showed chondrocytic differentiation on light and electron microscopy. The sarcomatous areas were multifocally scattered in the central portion of the tumor and intimately blended with adenocarcinoma cells. CEA immunoreactivity was found not only in the adenocarcinoma but also in the transitional sarcomatous cells, supporting the hypothesis that this tumor might derive from mesenchymal metaplasia of a primarily epithelial neoplasm.     

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.     

Malignant fibrous histiocytoma within a recurrent chordoma. A light microscopic, electron microscopic, and immunohistochemical study

A histologically typical chordoma and its four recurrences (3.5, 4, 7, and 7.5 years later) were operated from the spinal canal and I-III lumbar vertebrae of a 49-year-old woman. In addition to the typical areas of chordoma, pleomorphic areas resembling malignant fibrous histiocytoma also were seen in the fourth recurrence. The chordoma-like areas of the fourth recurrence displayed typical epithelial features: clusters of epithelial-like cells, electron microscopically demonstrable vacuoles lined by microvilli and desmosomes, and cytokeratin positivity in immunostaining. The areas in the fourth recurrence that resembled malignant fibrous histiocytoma lacked epithelial features as judged by electron microscopy. They also were negative for cytokeratin but contained, instead, vimentin type of intermediate filaments. The results show that the sarcomatous areas as seen in the recurrent chordoma lack epithelial cell features of chordoma and suggest the possibility of altered differentiation pathway of the tumor stem cell or emergence of a new malignant cell population within the recurrent tumor.     

Immunohistochemical characterization of thyroid gland angiomatoid tumors

The histogenesis of thyroid gland angiomatoid tumors, probably as a primary angiosarcoma, has been a controversy for many years. These tumors may be variants of undifferentiated (anaplastic) carcinomas. We report a thyroid gland angiomatoid carcinoma in a 61-year-old African American male. The tumor had a heterogeneous pattern with both sarcomatous and epithelioid areas. Tumor cells lined some vascular-like spaces and others had intracytoplasmic lumens containing red blood cells. The tumor cells were found to express multiple endothelial (factor VIII-related antigen, CD31, CD34, and Ulex europaeus I lectin) and epithelial (cytokeratin and epithelial membrane antigen) markers as well as thyroglobulin by immunohistochemistry. This rare presentation demonstrates the heterogeneous nature of thyroid gland angiomatoid carcinoma with both epithelial and endothelial differentiation.     

Cytogenetic analysis shows that carcinosarcomas of the breast are of monoclonal origin

Carcinosarcoma of the breast is a rare biphasic neoplasm composed of a carcinomatous component contiguous or admixed with a pleomorphic spindle cell component. The issues of the histogenesis and clonal composition of carcinosarcomas have long been debated. We present the first cytogenetic characterization of mammary carcinosarcomas by analysis of eight tumor samples from two patients with this disease. In the first case, the same karyotypically complex clone, as well as evidence of clonal evolution, was found in samples from three separate areas of the primary tumor. The analysis of one intramammary and one axillary lymph node metastasis from the same patient, both showing only the sarcomatous tumor component, also revealed the common complex stemline and one of the two sidelines found in the primary tumor. The carcinosarcoma of the second patient contained six complex but karyotypically related clones unevenly distributed among the three samples examined. From this case, cells belonging to the carcinomatous and sarcomatous tumor components were separated by differential sedimentation and culturing in specific growth media. Analysis of both fractions showed largely the same karyotype, although one of the subclones was restricted to the epithelial component. Our findings indicate that the epithelial and mesenchymal components of mammary carcinosarcomas are both part of the neoplastic parenchyma and that they have evolved from a single common stem cell, in agreement with the hypothesis that the tumors are of monoclonal origin. Genes Chromosomes Cancer 22:145–151, 1998. © 1998 Wiley-Liss, Inc.