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)

A total of 14 well differentiated rhabdomyosarcomas were diagnosed at necropsy in 10,000 mice. Of the 14 affected mice, ten were BALB/cJ, and there was one case each of A/HeJ, BALB/cByJ, C58/J, and C.B-17-scid/scid strains. Most often (10/14) tumors originated in the quadriceps muscles and metastases occurred in six cases. When submitted, affected mice were 2 to 8 months of age, with a mean age of 4 months. Tumor frequency for BALB/cJ mice was calculated to be 2.4/100,000 mice retained as breeders. No sexual dimorphisms were determined when data were correlated to actual numbers of each sex in the colony. All 14 primary tumors and metastases were positive by immunohistochemistry for the proteins pan myosin, sarcomeric actin, desmin, actin, and myosin, but were negative for smooth muscle actin, thus confirming the diagnosis. Using cell free homogenates of primary tumors, inoculated by intraperitoneal or intramuscular injection, tumors were not induced in either BALB/cJ or C58/J mice observed over a 22-week period. Southern blot analysis of DNA prepared from tumors and hybridized with a murine leukemia virus probe that recognizes both ecotropic and dualtropic viruses did not demonstrate viral genomic fragments in addition to those known to occur in each strain.
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PMID:Skeletal muscle rhabdomyosarcomas in inbred laboratory mice. 183 Apr 33

We describe 11 patients with a distinctive salivary gland neoplasm. Most of the patients were adult women who presented with a painless mass. Nine tumors arose in minor salivary glands of the oral cavity (82%). Microscopically, they were characterized by the formation of trabeculae, cords, islands, and/or nests of monomorphic clear cells that were glycogen rich and mucin negative and were surrounded by hyalinized bands with foci of myxohyaline stroma. Cells with eosinophilic and granular cytoplasm were also noted. Both cell types showed minimal nuclear pleomorphism and a very low mitotic index. The neoplasms all had infiltrative borders. Immunohistochemically, the tumor cells expressed cytokeratins and epithelial membrane antigen, but not S-100 protein and smooth muscle actin. Ultrastructurally, the tumor cells contained abundant glycogen, desmosomes, peripheral tonofilaments, and prominent interdigitating microvilli without actin myofilaments or dense bodies. These immunohistochemical and ultrastructural findings provide evidence of epithelial differentiation without myoepithelial differentiation. For these tumors, we propose the name, hyalinizing clear cell carcinoma (HCCC). These are low-grade malignant neoplasms. Two patients had ipsilateral cervical lymph node metastases at presentation, but with surgical excision and either preoperative or post-operative radiotherapy in three cases, eight of 10 patients with clinical follow-up are alive and well without evidence of recurrence. The mean clinical follow-up is 3.6 years, with a range of 6 months to 11 years. One patient died as a result of surgery, another died of unrelated causes, and one patient was lost to follow-up.
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PMID:Hyalinizing clear cell carcinoma of salivary gland. 753 Apr 10

Inflammatory myofibroblastic tumor (IMT) or inflammatory pseudotumor is a spindle cell proliferation of disputed nosology, with a distinctive fibroinflammatory and even pseudosarcomatous appearance. Although the lung is the best known and most common site, inflammatory myofibroblastic tumor occurs in diverse extrapulmonary locations. We report our experience with 84 cases occurring in the soft tissues and viscera of 48 female patients and 36 male patients between the ages of 3 months and 46 years (mean, 9.7 years; median, 9 years). A mass, fever, weight loss, pain, and site-specific symptoms were the presenting complaints. Laboratory abnormalities included anemia, thrombocytosis, polyclonal hypergammaglobulinemia, and elevated erythrocyte sedimentation rate. Sites of involvement included abdomen, retroperitoneum, or pelvis (61 cases); head and neck, including upper respiratory tract (12 cases); trunk (8 cases); and extremities (3 cases). The lesions ranged in size from 1 to 17 cm (mean, 6.4; median, 6.0). Excision was performed in 69 cases. Eight had biopsy only. Five patients received chemotherapy or radiation in addition to undergoing biopsy or resection as initial treatment. Sixteen patients had multinodular masses involving one region. Clinical follow-up in 53 cases revealed that 44 patients were alive with no evidence of disease, four were alive with IMT, and five were dead. Thirteen patients had one or more recurrences at intervals of 1-24 months (mean, 6 months; median, 10 months). No distant metastases were documented. The five patients who died had complications either due to the location of the lesion (heart, peritoneum, retroperitoneum, or mesentery) or related to treatment (lymphoproliferative disorder following hepatic transplantation; sepsis following wound infection). The abdominal masses were the largest. All tumors were firm and white with infiltrative borders and focal myxoid change. Three basic histologic patterns were recognized: (a) myxoid, vascular, and inflammatory areas resembling nodular fasciitis; (b) compact spindle cells with intermingled inflammatory cells (lymphocytes, plasma cells, and eosinophils) resembling fibrous histiocytoma; and (c) dense plate-like collagen resembling a desmoid or scar. Immunohistochemistry demonstrated positivity for vimentin, muscle-specific actin, smooth muscle actin, and cytokeratin consistent with myofibroblasts. Based on this series, inflammatory myofibroblastic tumor is a benign, nonmetastasizing proliferation of myofibroblasts with a potential for recurrence and persistent local growth, similar in some respects to the fibromatoses.
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PMID:Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. 866 41

We present 25 cases of a primary pulmonary sarcoma bearing histological, immunohistochemical, and ultrastructural features indistinguishable from those of monophasic synovial sarcoma of soft tissue. The patients were 11 men and 14 women between the ages of 16 and 77 years. Clinically, the most common symptoms were chest pain, cough, shortness of breath, and hemoptysis. The lesions involved all lung segments. Grossly, they varied in size from 0.6 to 20 cm and were described as soft to rubbery tumors with areas of necrosis and hemorrhage, some with cystic changes. Two lesions involved the bronchial wall and in one case the tumor was described as encircling the bronchial tree. Histologically, all of the lesions were characterized by an atypical spindle cell proliferation with a solid growth pattern. Areas of myxoid, neural, hemangiopericytic, and epithelial-like growth pattern were observed. Mitoses, necrosis, and hemorrhage were seen in all lesions in varying proportions. Immunohistochemical studies for epithelial membrane antigen (EMA) and keratin showed strong focal positivity in 25 of 25 and 23 of 25 lesions, respectively. Immunohistochemical study for vimentin showed diffuse strong positivity in all lesions. Other immunostains, including desmin, smooth muscle actin, and S-100 protein, were negative. Electron microscopy in three cases showed spindle cells with elongated nuclei containing abundant cytoplasmic rough endoplasmic reticulum and well developed desmosome type intercellular junctions. Follow-up information ranging from 2 to 20 years was obtained in 18 patients. Six patients died of their tumors, whereas four patients died of unrelated causes without evidence of recurrence or metastases. Eight patients were alive with disease (recurrence and/or metastases) from 1 to 7 years after diagnosis. Four patients were alive and well without evidence of recurrence or metastases from 2 to 20 years (mean follow-up, 12.5 years). The present group of lesions appears to constitute a distinctive and as yet previously undescribed primary sarcoma of the lung, which probably represents the visceral counterpart of monophasic synovial sarcoma of soft tissue in a pulmonary location. Because of their distinctive biology these lesions should be distinguished from a variety of primary and metastatic malignancies of the lung.
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PMID:Primary pulmonary sarcomas with features of monophasic synovial sarcoma: a clinicopathological, immunohistochemical, and ultrastructural study of 25 cases. 775 Sep 31

Desmoplastic malignant melanoma (DMM) is a rare variant of spindle cell melanoma. We report a case of DMM on the forehead secondarily involving the orbit. The diagnosis was based on light microscopic features, including prominent peripheral cell nest formation and spindle cell fascicles in densely collagenous stroma. Immunohistochemical studies showed strong uniform staining for S100 antigen throughout the tumour. It was negative for HMB 45, smooth muscle actin, desmin, cytokeratins and Type IV collagen. Electron microscopy showed neither melanosomes nor myelin figures. The clinical and histological characteristics of desmoplastic malignant melanoma, and its differential diagnosis of malignant schwannoma, are discussed. DMM has a poor prognosis, since it tends to invade deeply, recur locally and metastasize readily.
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PMID:Desmoplastic malignant melanoma presenting with orbital involvement. 791 65

A surgical case of intra-abdominal desmoplastic small cell tumour with divergent differentiation (IDS-CT) in a patient with Peutz-Jeghers syndrome (PJS) is reported. The patient was a 23-year-old woman who underwent surgery for a tumour in the left paraovarian region. Widespread peritoneal metastases were noted. Histopathological examination revealed solid cell nests composed of medium-sized cells demarcated by desmoplastic stroma. Gland-like spaces were found within many cell nests. Tumour cells were positive for cytokeratin and smooth muscle actin, but negative for desmin. Both ovaries contained minute foci of a sex cord tumour with annular tubules. This is the first documentation of the association of IDSCT with PJS. Since patients with PJS have an increased risk of cancer in various organs, the occurrence of IDSCT in the present patient might not be fortuitous.
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PMID:Intra-abdominal desmoplastic small cell tumour in a patient with Peutz-Jeghers syndrome. 795 6

The clinical and pathological features of 77 cases of intraductal (intracystic) papillary carcinoma (IPC) of the breast are reported. It should be recognized as an intraductal carcinoma variant and distinguished from invasive papillary carcinoma. Intraductal papillary carcinoma remains a difficult diagnosis as there are four different epithelial growth patterns any of which may predominate. Low grade nuclear features occur in one third of cases, a so-called "stratified spindled cell" epithelial proliferation with bland morphology occurs in one quarter of cases, and a dimorphic population of malignant cells, which may in part be confused with myoepithelial cells, occurs in one quarter of cases. The 77 cases studied were from the 10-year interval 1970 to 1979. The effect on prognosis of cytoarchitectural features, duct wall and stromal invasion, and associated intraductal carcinoma were evaluated. The contribution of immunohistochemistry to the diagnosis using antibodies to smooth muscle actin, S-100 protein, and CAM 5.2 was examined. The 10-year survival rate was 100%, and the 10-year disease-free survival rate was 91%. Mastectomy had been performed in 72% of patients. Three of the patients developed metastases; two were alive with tumor and one died of other causes. Six patients had local recurrence in the chest wall; one was alive without disease, two were alive with tumor, and three died of other causes. An associated intraductal carcinoma of usual nonnecrotic or comedo type was present in 40% of all cases. When IPC recurred or metastasized, it did so as invasive papillary carcinoma in six of seven cases. Stromal invasion was found in 13 patients. Local recurrence developed in two of these. Invasion was not seen in any of the three patients who developed metastases. However, this may be a function of sampling as there was an average of 5.2 tumor sections per case. Patients with low grade tumors had no recurrence or metastasis, and in the absence of invasion may be treated by local excision. Patients with higher grade tumors have an increased risk of recurrence and metastasis.
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PMID:Intraductal (intracystic) papillary carcinoma of the breast and its variants: a clinicopathological study of 77 cases. 805 21

Malignant myoepithelioma of the salivary gland is discussed in terms of its clinical behaviour, morphological features and the frequent pre-existence of a pleomorphic adenoma. The study comprised six female and four male patients aged 14-63 years (mean age 38.9 years). Two tumours presented as intraoral lesions and eight were located in the parotid gland. Tumour cells displayed a morphological spectrum ranging from round epithelioid cells to spindle-shaped and stellate cells. Most cells displayed reactivity for high molecular weight keratins and in four tumours there was strong immunoreactivity for smooth muscle actin. Malignant myoepithelioma seems to arise in two different clinical settings: either de novo or in a recurrent pleomorphic adenoma. De novo malignant myoepitheliomas arise in normal salivary gland, tend to be more aggressive and have a short clinical history. Recurrences may not develop or may occur as a single event within a short time interval, and metastases develop in the lungs. Malignant myoepitheliomas arising in recurrent pleomorphic adenomas have a long clinical history, are characterized by multiple recurrences and have to be distinguished from aggressive carcinomas arising in these adenomas. In contrast, the tumours described in the present series arising in pleomorphic adenomas showed local aggressiveness and metastases did not occur until decades after the first treatment. The general opinion that all malignant tumours that arise from pleomorphic adenomas are highly aggressive is not confirmed by the present study.
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PMID:Malignant myoepithelioma of salivary glands: clinicopathological features of ten cases. 811 28

Alveolar soft part sarcoma (ASPS) was described as a distinct entity in 1952. ASPS mainly affects young adults, with a slight prevalence of female patients. It arises in the extremities, trunk, and head and neck region. Metastases are frequent and are mainly localized to lungs, brain, and bone. Local recurrences are rare if the primary tumor is completely excised. Adjuvant chemotherapy or radiotherapy are not useful. Death eventually supervenes, after several years, in most of the patients. Histogenesis and differentiation of ASPS, since its first description, have remained a matter of controversy. Many hypotheses have been taken into consideration, among which neural and muscular differentiation are the most plausible. The finding that intracytoplasmic crystals, present in most of the cases, are composed of actin, and the immunocytochemical localization in ASPS of several muscular markers, such as actin (skeletal and smooth muscle actin), desmin, and MyoD1 are all features that point towards skeletal muscle differentiation.
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PMID:Alveolar soft-part sarcoma: a new type of rhabdomyosarcoma? 820 47

Nine vulvar and three vaginal angiomyofibroblastomas from patients 23 to 71 years of age (mean, 46 yr) were analyzed. The tumors were well circumscribed and ranged from 0.9 to 11 cm (average, 4.7 cm) in maximal dimension. On microscopic examination, they had hypercellular and hypocellular areas. The neoplastic cells were spindle-shaped, plasmacytoid, or epithelioid; a variable number were binucleated or multinucleated cells. A focal storiform pattern was present in one tumor, and, in one tumor, the neoplastic cells formed a collar around a central area of dense collagen. There was no significant nuclear atypia, and there was less than one mitotic figure per 10 high-power fields. The tumors contained small- to medium-sized blood vessels, which were characteristically thin walled and, occasionally, ectatic and branching. The stroma was edematous, separated collagen fibers and contained a variable number of inflammatory cells, especially lymphocytes and mast cells. Three vulvar tumors contained a variable amount of fat. Ultrastructural study of three tumors showed intracytoplasmic, dilated, rough endoplasmic reticulum, moderate numbers of pinocytotic vesicles, and numerous filaments without dense bodies; rare intercellular rudimentary junctions were identified. Eleven of 11 tumors were immunoreactive for vimentin, 11 of 12 for desmin, three of 11 for muscle actin, one of 12 for smooth muscle actin, and four of 12 for CD34. There was no staining for factor XIIIa, keratin, S100 protein, Leu-7, glial fibrillary acidic protein, or CD68. Follow-up revealed no recurrences or metastases. Angiomyofibroblastoma is a distinctive benign tumor that arises most commonly in the vulva and vagina and has a diverse histologic and immunohistochemical profile.
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PMID:Angiomyofibroblastoma of the vulva and vagina. 868 29


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