Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rhabdoid tumor is a well-accepted clincopathologic entity among childhood renal neoplasms; similar tumors have been described in extrarenal locations. We present the clinicopathologic profile and the immunohistochemical features of a series of soft tissue rhabdoid tumors. Twenty-eight cases coded as extrarenal rhabdoid tumor (ERRT), RT, possible ERRT, and "large cell sarcoma" were retrieved from the Armed Forces Institute of Pathology soft tissue registry. The tumors were reclassified according to strict criteria by light microscopy, clinical information, immunohistochemistry, and, in some cases, electron microscopy. Soft tissue rhabdoid tumor (STRT) was defined as (1) a tumor composed of noncohesive single cells, clusters, or sheets of large tumor cells with abundant glassy eosinophilic cytoplasm, an eccentric vesicular nucleus, and an extremely large nucleolus; (2) positivity for vimentin and/or cytokeratin or other epithelial markers by immunostaining; and (3) exclusion of other tumor types with rhabdoid inclusions (melanoma, other sarcomas, carcinoma). Eighteen cases met our criteria for soft tissue rhabdoid tumors. The median patient age was 13 years (range, 6 months to 56 years). Ninety-four percent of STRT cases were positive for vimentin and 59% for pan-cytokeratin. Sixty-three percent and 60% were positive for CAM 5.2 and EMA, respectively. Seventy-nine percent stained for at least one epithelial marker; 76% stained for both vimentin and epithelial markers simultaneously. Forty-two percent stained for MSA, and 14% for CEA and SMA. CD99, synaptophysin, CD57 (Leu-7), NSE, and focal S100 protein were identified in 75%, 66%, 56%, 54%, and 31% of the STRT cases, respectively. All STRT cases examined were negative for HMB-45, chromogranin, BER-EP4, desmin, myoglobin, CD34, and GFAP. Follow-up examination in 61% of the STRT patients revealed that 64% of patients died of disease within a median follow-up interval of 19 months (range, 4 months to 5 years); 82% had metastases to lung, lymph nodes, or liver; 22% had local recurrences before metastasis; and 18% were alive without known disease status (median, 5.5 years). Soft tissue rhabdoid tumor is a highly aggressive sarcoma, predominantly of childhood. Besides having nearly consistent coexpression of vimentin and epithelial markers, STRTs show positivity for multiple neural/neuroectodermal markers that overlap with those of primitive neuroectodermal tumor.
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PMID:Extrarenal rhabdoid tumors of soft tissue: a clinicopathologic and immunohistochemical study of 18 cases. 993 May 72

In a 5-year-old Holstein cow, a neoplasm composed of a large intramuscular mass and multiple metastases in the lungs and lymph nodes was diagnosed as a pleomorphic rhabdomyosarcoma. This neoplasm was characterized by marked variation in tumor cell size and giant cells with single bizarre nuclei. Although the presence of cross striations and myoglobin could be confirmed, expression of alpha-smooth muscle actin (SMA) was also recognized in a few cells. Neoplastic cells showing intense staining for desmin, vimentin and proliferating cell nuclear antigen irrespective of their size differed from those in an embryonal rhabdomyosarcoma that exhibited a wide spectrum of differentiation, reminiscent of normal skeletal myogenesis. The cellular pleomorphism and SMA expression seemed to be characteristic of deviation from normal muscle cells or satellite cells in adult muscle.
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PMID:Pleomorphic rhabdomyosarcoma in a cow. 1121 54

Metastases from prostatic adenocarcinoma (prostate cancer) are characterized by their predilection for bone and typical osteoblastic features. An in vitro model of bone metastases from prostate cancer was developed using a bicompartment coculture system of mouse osteoblasts and human prostate cancer cells. In this model, the bone-derived prostate cancer cell lines MDA PCa 2a and MDA PCa 2b induced a specific and reproducible increase in osteoblast proliferation. Moreover, these cells were able to induce osteoblast differentiation, as assessed by increased alkaline phosphatase activity, Osteocalcin expression, and calcified matrix formation. This osteoblastic reaction was confirmed in vivo by intrafemoral injection of MDA PCa 2b cells into severe combined immunodeficiency disease mice. In contrast, the highly undifferentiated, bone-derived human prostate cancer cell line PC3 did not produce an osteoblastic reaction in vitro and induced osteolytic lesions in vivo. The osteoblast differentiation induced by MDA PCa 2b cells was associated with up-regulation of the osteoblast-specific transcriptor factor Cbfa1. Moreover, treatment of osteoblasts with conditioned medium obtained from MDA PCa 2b cells resulted in up-regulation of Cbfa1 and Osteocalcin expression. In support of the differentiation studies, a microarray analysis showed that primary mouse osteoblasts grown in the presence of MDA PCa 2b cells showed a shift in the pattern of gene expression with an increase in mRNA-encoding Procollagen type I and Osteopontin and a decrease in mRNA-encoding proteins associated with myoblast differentiation, namely myoglobin and myosin light-chain 2. Taken together, these findings suggest that the bone-derived prostate cancer cells MDA PCa 2a and MDA PCa 2b promote differentiation of osteoblast precursors to an osteoblastic phenotype through a Cbfa1-dependent pathway. These results also established that soluble factors produced by prostate cancer cells can induce expression of osteoblast-specific genes. This in vitro model provides a valuable system to isolate molecules secreted by prostate cancer cells that favor osteoblast differentiation. Moreover, it allows to screen for therapeutic agents blocking the osteoblast response to prostate cancer.
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PMID:Prostate cancer cells induce osteoblast differentiation through a Cbfa1-dependent pathway. 1145 20

A variant of large cell carcinoma showing a rhabdoid phenotype, which is rare among primary lung cancers, is presented. A 59-year-old man was admitted to hospital for an operation. Computed tomography scans showed a mass with a smooth border, invading the thoracic wall. A right upper lobe lobectomy was carried out with resection of a part of the thoracic wall. Pathological examination showed that the tumor was mostly composed of cells with prominent eosinophilic cytoplasmic globules and giant cells, which did not adhere to each other. Cytologically, the tumor cells contained nuclei with a reticular chromatin pattern and one to two prominent nucleoli, and hyaline-like and reticular inclusion bodies, which were immunohistochemically positive for vimentin, but not for alpha-smooth muscle actin, myoglobin or pan-actin. Radiological and laboratory examinations did not detect the presence of the tumor in other organs, indicating that the primary lesion was not situated elsewhere. Metastasis to the right adrenal gland was observed 1 year and 4 months after the operation; however, the patient has been free of the disease 3 years and 11 months after the second operation of an adrenalectomy. This case showed a relatively good prognosis, which is rare among rhabdoid tumors of various organs that generally have poor prognoses with rapid, fatal progression.
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PMID:Large cell carcinoma of the lung with a rhabdoid phenotype. 1244 36

An unusual myometrial tumor was encountered in a 70-year-old female who presented with lower abdominal fullness and symptoms related to pulmonary metastases. Laparotomy revealed a uterine mass that was removed by total abdominal hysterectomy and bilateral salpingo-oophorectomy. Multiple liver metastases also were noted. Pathologic examination of the hysterectomy specimen revealed a 25-cm, well-circumscribed myometrial mass that had a firm, white-to-yellow, focally whorled, sectioned surface with focal hemorrhage and necrosis. Within the main mass was a discrete, 7-cm, soft, gelatinous, reddish-yellow nodule. The main tumor was a well-to-moderately differentiated leiomyosarcoma, whereas the gelatinous nodule was rhabdomyosarcomatous and contained rhabdomyoblasts that exhibited cytoplasmic cross striations and immunoreactivity for myoglobin. No epithelial elements were detected in the neoplasm. At the time of last follow-up, the patient was undergoing chemotherapy. This is only the second well-documented case of a hybrid leiomyosarcoma-rhabdomyosarcoma of the uterus.
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PMID:Leiomyosarcoma of the uterus with focal rhabdomyosarcomatous differentiation. 1508 50

The spindle cell variant of rhabdomyosarcoma (RMS) is uncommon and is most often encountered in the paratesticular region of children in whom it has a good prognosis. Only isolated cases in adulthood have been described. Sixteen cases of spindle cell RMS occurring in adults were retrieved from our files. Eleven patients were male and 5 were female. Patient age ranged from 18 to 79 years (median, 32 years). Tumor size varied from 1.5 to 35 cm (median, 6 cm). The head and neck region, including the oral cavity, parotid gland, nasopharynx, and nasal cavity, was the commonest affected area, accounting for >50% of the cases, followed by retroperitoneum, thigh, leg, subscapular area, hand, vulva, and paratesticular region (1 case each). Follow-up was available in 12 cases, ranging from 1 to 102 months (median, 16.5 months). Treatment modalities included surgery, chemotherapy, and radiation. Two patients died of uncontrolled local disease 13 and 27 months after diagnosis; 4 were alive without disease at 12, 17, 24, and 102 months, including 1 patient with metastasis to 10 of 50 pelvic lymph nodes at presentation; 3 are alive with localized disease at 16, 17, and 19 months; and 1 was followed for 6 months and showed persistent local disease. One patient is alive at 10 months after diagnosis with evidence of metastatic disease to bone, lungs, and breast. All the tumors showed long fascicles of spindle cells with elongated, vesicular nuclei and pale indistinct cytoplasm. Scattered spindled or polygonal rhabdomyoblasts with abundant brightly eosinophilic cytoplasm were present in all cases. In 3 cases, focal areas showed pseudovascular, sclerosing features. There were no round cell or pleomorphic areas. Positive immunohistochemical results were as follows: desmin (15 of 15 cases), myf-4 (12 of 12), fast myosin (7 of 9), myoglobin (2 of 3), HHF-35 (9 of 9), and SMA (11 of 14). One tumor was focally positive for keratins and EMA. All tumors were negative for caldesmon, S-100 protein, and GFAP. Spindle cell RMS is a rare neoplasm in adults and appears to have distinct clinicopathologic features when compared with cases occurring in the pediatric population. Specifically, it appears to be most common in the head and neck region, and although only limited follow-up is available so far, these lesions appear to have a more aggressive clinical course in adults.
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PMID:Spindle cell rhabdomyosarcoma in adults. 1600 7

Herein is presented the case of an esophageal pleomorphic giant cell carcinoma combined with small cell carcinoma (SCC). The patient, a 77-year-old man, initially presented with dysphagia and hoarseness, and endoscopy indicated a large esophageal tumor. Despite chemoradiation therapy, the patient died from widespread local extension of the tumor and distant metastases approximately 8 months after onset of the symptoms. Histologically, the primary tumor was composed of pleomorphic tumor components, SCC components, and a tiny focus of squamous cell carcinoma. The pleomorphic tumor cells, consisting of solid sheets of poorly cohesive epithelioid cells and numerous multinucleated giant cells with abundant eosinophilic cytoplasm, were immunohistochemically positive for vimentin and desmin, with scattered positivity for epithelial membrane antigen (EMA) and neuron-specific enolase (NSE), but negative for myoglobin. These findings were histopathologically compatible with pleomorphic giant cell carcinoma occurring at other sites such as the lung. SCC cells, morphologically similar to their pulmonary counterpart, were positive for EMA and some neuroendocrine markers such as chromogranin A and NSE, and occasionally positive for vimentin and desmin. Esophageal pleomorphic giant cell carcinoma can occur in close association with SCC, and should be included in the differential diagnosis of esophageal tumors showing pleomorphism.
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PMID:Esophageal pleomorphic giant cell carcinoma combined with small cell carcinoma. 1761 Apr 78

Cases of leiomyosarcoma in the head and neck are very rare with only four reports of a leiomyosarcoma originating in the hypopharynx. A rare case of leiomyosarcoma of the hypopharynx is described. A 62-year-old woman, who had experienced slight difficulty of swallowing and hoarseness, visited our hospital. A smooth-surfaced tumor was found extending from the postcricoid to an area in the left pyriform sinus, and the vocal cord on the left side was fixed. Imaging studies, including FDG-PET CT imaging showed the tumor in the postcricoid, but no distinguishable distant metastases. A total laryngectomy with a left-lobe thyroidectomy was performed. Since no metastases of the regional lymph nodes were found by imaging, a neck dissection was not performed. Dense infiltration by spindle-shaped and/or round-like cells was observed histologically as the predominant growth pattern. In immunohistochemical staining, the tumor was positive for desmin and muscle actin, while it was negative for myoglobin, myogenin, cytokeratin, and S-100 protein. Electron microscopic examination did not show Z banding and sarcomeres. Those features lead to the diagnosis of a leiomyosarcoma. No recurrence has been found at 1 year after the surgery. Immunohistochemical staining and electron microscopic examination are essential for a definite diagnosis of leiomyosarcoma.
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PMID:Leiomyosarcoma of the hypopharynx: a case report. 1825 99

Alveolar soft part sarcoma (ASPS) is a malignancy with low incidence, but with poor prognosis if misdiagnosed. Immunohistochemical assay using TFE3 antibody has been shown to be a sensitive technique for ASPS diagnosis. A specific chromosomal translocation, t(X;17)(p11.2;q25), results in the ASPL-TFE3 fusion gene: it is detectable using reverse-transcription polymerase chain reaction (RT-PCR) in frozen tumor tissues of ASPS. However, the diagnostic usefulness of these markers has not been investigated in Chinese ASPS patients. Here, we report the first systematic study applying TFE3 immunoassay and ASPL-TFE3 fusion transcript detection to archival paraffin-embedded tissues in a large Chinese ASPS patient population. Sixteen patients had been diagnosed with ASPS (age, 3 to 58 y; 3 male patients and 13 female patients). Their tumors presented predominantly in the extremities (8/16), and were often located in the region of the orbit when affecting infants and children (3/16). Others had tumors in the chest wall, breast, and right pubis, respectively. One patient exhibited a tumor in the renal hilum, a location that had not been previously reported. Two patients had tumor metastases in the lung and the brain. ASPS tumors showed the best immunoreactivity to the TFE3 antibody (16/16). However, their immunoreactivity to other antibodies, including myoglobin (13/16), actin (10/16), desmin (2/16), and vimentin (2/16), was of various degrees. Positive staining was observed for the neural markers, NSE (9/16) and CgA (7/16), respectively. Using a strategy of RT-PCR, followed by a nested PCR with a different primer set, we were able to detect the expression of the chimeric ASPL-TFE3 mRNA in 11 of the 16 ASPS tumors. Of these 11, 7 were type 1 ASPL-TFE3 and 4 were type 2 ASPL-TFE3, including the tumor located in the renal hilum. No expression of ASPL-TFE3 fusion transcripts was detectable in all 38 control tumors. Our results demonstrate that the "bimarker strategy," a combination of TFE3 immunostaining and ASPL-TFE3 chimeric transcript detection, might have sufficient sensitivity and specificity in diagnosing most of the ASPSs. As both diagnostic techniques can be applied to widely available archival paraffin-embedded tissues, the usefulness of the strategy is largely implicated in routine pathology laboratories.
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PMID:Alveolar soft part sarcoma: a bimarker diagnostic strategy using TFE3 immunoassay and ASPL-TFE3 fusion transcripts in paraffin-embedded tumor tissues. 1838 56

An adult free-living European robin (Erithacus rubecula) with a large, firm, subcutaneous mass on the pectoral muscle was examined. The bird was unable to fly and died spontaneously. Necropsy revealed a yellowish, bilobate mass almost completely replacing the pectoral muscles with extensive osteolysis of the keel bone. Histopathology revealed a poorly demarcated, highly cellular sarcomatous tumour with metastases to the lungs, pulmonary blood vessels and heart. Immunohistochemistry was negative for neuron-specific enolase, S-100 protein and the p-27 major capsid protein of avian leukosis viruses. The homogeneously positive immunolabelling for vimentin and scattered positivity for myoglobin and desmin suggested a diagnosis of rhabdomyosarcoma. A retrospective examination of the records for 194 birds of the thrush family, including 64 robins submitted over a 20-year period, showed no diagnoses of neoplasia.
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PMID:Rhabdomyosarcoma of the pectoral muscles of a free-living European robin (Erithacus rubecula). 1856 58


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