Anaplastic and sarcomatoid carcinoma of the small intestine: a clinicopathologic study

Carcinomas involving the jejunum and ileum are rare tumors. During a review of small intestinal neoplasms, six primary carcinomas of jejunum or ileum with an anaplastic and sarcomatoid histology were identified. At presentation, three of the patients had symptoms related to metastatic disease and three had symptoms referable to the local tumor. The tumors were large (greater than 4.5 cm in diameter), usually endophytic masses composed of large cells with eosinophilic cytoplasm, anaplastic nuclei, and prominent nucleoli. In many areas, the cells had a spindled configuration. Mucin positivity was identified in all six cases. Electron microscopic findings in two cases were indicative of epithelial differentiation. The tumors behaved aggressively; all five patients for whom there was clinical follow-up died of metastases within 40 months. The six anaplastic and sarcomatoid carcinomas were compared with 29 typical adenocarcinomas arising in the jejunum or ileum. Only two of the latter group had symptoms referable to distant metastases at presentation. These tumors also tended to be smaller at presentation (11 tumors were less than 4 cm in greatest dimension). Of 25 patients with typical adenocarcinomas who had acceptable follow-up, 18 (72%) died of disease and five (20%) were alive with no evidence of disease after 5 years. We conclude that anaplastic and sarcomatoid carcinoma is a rare variant of small intestinal carcinoma with an aggressive clinical course.     

Transitional carcinoma of the ureter with osseous metaplasia of the stroma: a case report

Transitional cell carcinoma with osseous metaplasia of the stroma is a rare variant of urothelial carcinoma which must be distinguished from sarcomatoid carcinoma. We report here a further observation of this tumor variant, in a very unusual location, the ureter, in order to underline the radiological and pathological characteristics useful for the correct diagnosis, and to point out the problems of differential diagnosis. The diagnosis was made in an 85-year-old patient, presenting with chronic right lombalgias. Imaging studies showed a calcified thickening of the right ureter, associated with a major dilatation of the right excretory cavities and a parenchymal atrophy of the right kidney. A right nephro-ureterectomy was performed. Macroscopical examination showed an ossified, ill-limited tumor, measuring 4 cm in diameter, infiltrating the right ureteral wall. At histological examination, the lesion was identified as a high grade transitional cell carcinoma with extensive osseous metaplasia of the stroma. There was no evidence for a sarcomatous component (absence of mesenchymal cell proliferation, absence of mitosis in stromal cells). An early locoregional recurrence was observed three months after surgery. Our case report underlines the radiological and histological features of a rare variant of transitional cell carcinoma, which may be diagnosed by pre-operative imaging studies and which must be distinguished from a highly aggressive sarcomatoid carcinoma.     

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.     

UNUSUAL RENAL PELVIC TUMOR CONTAINING TRANSITIONAL CELL CARCINOMA, ADENOCARCINOMA AND SARCOMATOID ELEMENTS (SO-CALLED SARCOMATOID CARCINOMA OF THE RENAL PELVIS)

A case of sarcomatoid carcinoma of the renal pelvis in a 66-year-old male is reported. The patient underwent left nephroureterectomy because of renal pelvic tumor and hydronephrosis. Left renal calcification and atrophy had been diagnosed in the patient about thirty years previously. The tumor showed a polypoid configuration and occupied the renal calyces. Histologically, not only solid nests of transitional cell carcinoma (TCC) and adenocar-clnomatous glands but also large spindle-shaped cells with bizarre nuclei simulating sarcoma were identified. Immunoreactlve keratin and epithelial-membranous antigen (EMA) were demonstrated in the sarcomatoid cells, indicating that they were of epithelial origin. So far, only 10 cases of so-called sarcomatoid carcinoma or carcinosarcoma of the renal pelvis have been reported in the world. In this report, we summarize the pathological findings of previously reported cases and discuss the histogenesis of this rare tumor.     

Unusual renal pelvic tumor containing transitional cell carcinoma, adenocarcinoma and sarcomatoid elements (so-called sarcomatoid carcinoma of the renal pelvis). A case report and review of the literature

A case of sarcomatoid carcinoma of the renal pelvis in a 66-year-old male is reported. The patient underwent left nephroureterectomy because of renal pelvic tumor and hydronephrosis. Left renal calcification and atrophy had been diagnosed in the patient about thirty years previously. The tumor showed a polypoid configuration and occupied the renal calyces. Histologically, not only solid nests of transitional cell carcinoma (TCC) and adenocarcinomatous glands but also large spindle-shaped cells with bizarre nuclei simulating sarcoma were identified. Immunoreactive keratin and epithelial-membranous antigen (EMA) were demonstrated in the sarcomatoid cells, indicating that they were of epithelial origin. So far, only 10 cases of so-called sarcomatoid carcinoma or carcinosarcoma of the renal pelvis have been reported in the world. In this report, we summarize the pathological findings of previously reported cases and discuss the histogenesis of this rare tumor.     

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.     

Primary undifferentiated carcinoma of the small intestine: an immunohistochemical study and review of the literature

Primary undifferentiated carcinoma of the small intestine is an extremely rare neoplasm. Here, we report a case of primary undifferentiated carcinoma that arose from the ileum in a 65-year-old woman. Laboratory data revealed anemia and slightly elevated inflammatory parameters. Computed tomography showed a heterogeneous mass in the pelvic cavity, and magnetic resonance imaging revealed that the margin of the tumor mass was clear. Positron emission tomography using ¹⁸F-fluoro-2-deoxy-d-glucose (FDG) showed accumulation of FDG on the tumor mass with a standardized uptake value of 8.3. Partial resection of the ileum to remove the tumor was performed under a clinical diagnosis of small intestinal carcinoma. The tumor was nodulated and had a circumscribed margin 6.5 × 5.5 × 4 cm in diameter. Microscopically, the tumor was composed of giant polygonal cells with cellular atypia. Immunohistochemical examination revealed that the tumor cells expressed epithelial markers including AE1/AE3, CAM5.2, and EMA; however, lymphocytic, mesenchymal, and gastrointestinal stromal tumor markers were not expressed. We made a final diagnosis of primary undifferentiated carcinoma of the small intestine. The prognosis of patients with undifferentiated carcinoma of the small intestine is very poor. To improve the outcome of treatment, early and accurate diagnosis is essential, and additional therapy, including multimodality adjuvant therapy or the administration of novel molecular targeted drugs, should be considered.     

Inflammatory sarcomatoid carcinoma: a case report and discussion of a malignant tumor with benign appearance

Inflammatory sarcomatoid carcinoma is an aggressive tumor with an unusually benign appearance. We report the case of a 65-year-old man with a history of inoperable poorly differentiated carcinoma of the right lung, for which he had received chemoradiotherapy. A new solitary mass was discovered 4 years later in the left lung on surveillance computed tomography. The patient underwent thoracotomy with a wedge biopsy on which frozen section was performed. The nodule was vaguely granulomatous and associated with a mixed inflammatory infiltrate and a deceptively bland spindle cell proliferation. Results of immunoperoxidase studies, however, showed that the nodule contained neoplastic cells with an epithelial phenotype that were invading the pulmonary vessels. These are features of the rare inflammatory sarcomatoid carcinoma. In contrast to sarcomatoid carcinomas, this case highlights the deceptively benign appearance of inflammatory sarcomatoid carcinoma. This leads us to concur with the recommendation to exercise caution when attempting the diagnosis of apparently benign lesions on intraoperative frozen section in patients with high clinical suspicion of malignancy.     

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.     

Fine-needle aspiration cytology of sarcomatoid carcinoma of the breast: A cytologically overlooked neoplasm

Sarcomatoid carcinoma of the breast is a very uncommon neoplasm. Fine-needle aspiration findings have been rarely reported. We report a case of sarcomatoid carcinoma of the breast that was diagnosed as a typical ductal carcinoma cytologically. The patient was a 45-year-old female who had a right breast mass for a short duration. Fine-needle aspiration (FNA) showed smears predominantly composed of single cells exhibiting plasmacytoid features with moderate degree of pleomorphism. A granular bluish background was noted on the Papanicolaou-stained smears. This background was thought to represent tumor diathesis, and the diagnosis of grade II ductal carcinoma of the breast was rendered. The patient underwent a subsequent lumpectomy with axillary node dissection. Histological examination of the lumpectomy revealed a neoplasm predominantly composed of a grade II chondrosarcoma with increased cellularity. A small (10%) component showed grade II invasive ductal carcinoma of the breast. No in situ component was seen. The sarcomatoid component was positive for S-100 protein and vimentin and negative for keratin, whereas the carcinomatous component was positive for vimentin, epithelial membrane antigen, and low molecular weight keratin. Ultrastructurally, the tumor showed features of a poorly differentiated epithelial neoplasm without evidence of chondrosarcoma. Metastases were seen in seven of ten axillary lymph nodes with exclusive epithelial component. Retrospective evaluation of the FNA smears revealed an extensive metachromatic stroma on the DIFF QUIK™-stained smears. Our findings and review of the literature suggest that sarcomatoid carcinoma of the breast will be very frequently overlooked or misdiagnosed either because of the subtlety of the stroma or the predominance of the mesenchymal component. Our findings also provide supportive evidence that the use of the DIFF QUIK™ stain can be very helpful in the identification of the stroma in this neoplasm. The predominance of epithelium in the metastases in our case is further proof that tumors with dual differentiation such as sarcomatoid carcinomas and mixed muellerian tumors are truly carcinomas with metaplastic features. Diagn. Cytopathol. 16:242–246, 1997. © 1997 Wiley-Liss, Inc.     

Carcinoma of the bladder with pseudosarcomatous stroma. Presentation of a case

Carcinoma with pseudosarcomatous stroma of the bladder. Case report. We report a case of transitional cells carcinoma of urinary bladder with pseudosarcomatous stroma in a 54 years old male. The patient was admitted in hospital owing to recidivous haematuria events for about 3 months. Three urine samples were sent for cytological studies that showed the presence of transitional neoplastic cells. Subjected to a cystoscopic examination the patient revealed a roundish peduncled bladder formation of about 3.5 cm. in size. Hence three bladder lesion biopsies were done. The histological sections obtained pointed out the existence of two cellular populations: the first one presented roundish or lengthened, pluristratified cells arranged in solid nests, with transitional morfology and high atypias; the other population had sarcomatoid features characterized by lenghtened cells with hyperchromic nuclei. The two populations were mixed up and there were sometimes carcinomatous areas that merged into sarcomatoid areas. Chondroid areas were also observed. We diagnosed a G3 transitional cells carcinoma with pseudosarcomatous stroma. The lesion, after literature review, results to be somewhat rare and not easily interpretable since it may be confused with carcinosarcoma. The rarity of this case and the problems of differential diagnosis connected with it urged us to this pubblication along with a short review of literature.     

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 hepatic lymphoma with spindle cell components: a case report

Diffuse large B-cell lymphoma (DLBCL) with spindle cell components is extremely rare and often misdiagnosed as carcinoma or sarcoma. Here, we present a case of primary DLBCL with spindle cell components arising in the liver, for which a preoperative diagnosis by needle biopsies was unsuccessful. The patient was a 70-year-old man with a continuous cough. Thoracic computed tomography incidentally detected a mass of 5 cm in diameter in his liver. The initial and second needle biopsies from the liver mass were pathologically diagnosed as suspicious for sarcomatoid hepatocellular carcinoma. He underwent an extended left hepatectomy. Histological examination revealed a diffuse or epithelioid arrangement of round and polygonal cells, mixed with the fascicles of spindle-shaped cells. Immunohistochemically, all the morphological types of tumor cells showed positive reactions for a lymphocytic marker (CD45RB) and B-cell markers (CD20 and CD79a). Double-immunostaining revealed that the spindle-shaped tumor cells expressed CD20, but never expressed alpha-smooth muscle actin. Malignant lymphoma with a spindle cell morphology is quite uncommon, and this variant can be a diagnostic pitfall, especially in tiny biopsy specimens. We emphasize that pathologists should be reminded of lymphoma as a differential diagnosis of spindle cell tumors.     

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.     

Acquired cystic disease‐associated renal cell carcinoma with a focal sarcomatoid component: Report of a case showing more pronounced polysomy of chromosomes 3 and 16 in the sarcomatoid component

Acquired cystic disease (ACD)‐associated renal cell carcinoma (RCC) has recently been established. Herein we report the sixth case of ACD‐associated RCC with a sarcomatoid change. The patient was a 77‐year‐old man who regularly underwent hemodialysis for 14 years due to chronic renal failure resulting from IgA nephropathy. On computed tomography, a large right RCC was observed with contrast enhancement in the arterial phase. A nodular protrusion into the perirenal fat was detected. Right nephrectomy was performed under laparoscopy. Surgically resected specimens revealed a tan‐to‐yellow tumor (95 × 75 × 55 mm) with a whitish nodule (20 × 15 × 15 mm) invading into the perirenal fat. Histopathologically, the large carcinoma component of the tumor displayed a cribriform or microcystic growth pattern with deposition of oxalate crystals. The whitish nodule corresponded to the sarcomatoid component, and the spindled and pleomorphic tumor cells showed diffuse positivity of p53 on immunohistochemistry. Fluorescence in situ hybridization revealed trisomy of chromosomes 3 and 16 in the carcinoma component, as was expected from the literature. In addition, increased polysomy of these chromosomes was also observed in the sarcomatoid component. This finding may be related to the development of the sarcomatoid component along with the TP53 mutation.     

Peritoneal washing cytology findings of disseminated myxoid leiomyosarcoma of uterus: report of a case with emphasis on possible differential diagnosis

We report the cytology findings of a rare case of myxoid leiomyosarcoma of the uterus with intraabdominal dissemination. The cytology showed uniformly dispersed spindly to polygonal "epithelioid" tumor cells focally linked by background matrix. Spindly tumor cells attaching to and radiating from branching capillary structures were also present. Occasionally, scattered "signet-ring" cells were found, mimicking metastatic poorly differentiated adenocarcinoma. The background mucoid substance was more conspicuous in the cell block sections. Gross and histologic examination of the surgical specimen revealed a large uterine leiomyosarcoma with prominent myxoid change. Ultrastructural study showed that the "signet-ring" appearance was mainly due to degenerative cytoplasmic change with ballooning of mitochondria, dilatation of endoplasmic reticulum, and elevation of outer nuclear membrane. In contrast to other spindle cell malignancies such as sarcomatoid mesothelioma, sarcomatoid carcinoma, or malignant melanoma, true sarcoma cells in general carry a distinctive cytologic appearance in washing/effusion fluid. In a correct clinical setting, the peculiar association with mucoid matrix and absence of classic lipoblasts should also raise the suspicion of metastatic myxoid leiomyosarcoma.     

肾肉瘤样癌5例临床病理分析

目的：观察肾肉瘤样癌形态特征。方法：５例肾肉瘤样癌进行光镜观察及免疫组化染色。结果：以肉瘤样成分为主３例，血管外皮瘤样为主１例及破骨细胞瘤样巨细胞瘤１例，５例肉瘤样成分角蛋白阳性，４例波形蛋白阳性。结论：肾肉瘤样癌的形态特征为癌细胞具有双重分化，具有向间叶性肿瘤转分化的过渡方式。     

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.     

Renal cell carcinoma,chromophobe type,with collecting duct carcinoma and sarcomatoid components

We report a case of a 72-year-old man with a chromophobe renal cell carcinoma that had both sarcomatoid and collecting duct carcinoma components. The 7-cm tumor occupied the entire lower pole of the kidney and infiltrated the renal parenchyma and the pelvic-calyceal system. Histologically, it had an area of classic chromophobe renal cell carcinoma that merged into a sarcomatoid component. Closely intermixed with the sarcomatoid component was a collecting duct carcinoma component characterized by highly pleomorphic, epithelioid cells arranged in cords, nests, and tubulomicrocystic structures. The cords, nests, and tubules were associated with a florid desmoplastic stromal response and numerous inflammatory cells. In addition, dysplastic changes were noted in adjacent nonneoplastic collecting duct epithelium. Immunohistochemical studies confirmed the presence of 3 distinct components in this patient's tumor. To the best of our knowledge, this is the first reported case of a chromophobe renal cell carcinoma with sarcomatoid and collecting duct carcinoma components.