Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report 38 cases of inflammatory fibrosarcoma occurring in 23 females and 15 males, 2 months to 74 years of age (median, 8.5 years; mean, 15 years) with symptoms of abdominal pain (17 cases), anemia (21 cases), fever (14 cases), mass (16 cases), and gastrointestinal obstruction (7 cases). Primary tumor sites included mesentery and retroperitoneum (31 cases), omentum (two cases), mediastinum (two cases), liver (one case), diaphragm (one case), and abdominal wall (one case). Sizes ranged from 2.4 cm to 20 cm (mean, 9.6 cm). Follow-up data in 27 cases revealed local recurrences in 10 patients, with multiple local recurrences in three and histologically proven distant metastases to lung (two cases) and brain (one case). Five patients died from their disease (median, 20 months). All tumors, including metastases, consisted of fibroblasts, myofibroblasts, and plasma cells, with variable degrees of fibrosis and calcification. Immunostains indicate myofibroblastic differentiation; 18 of 20 (90%) stained for actin, 15 of 18 (83%) for vimentin, and 10 of 13 (77%) for keratin (primarily in a submesothelial location). Ultrastructural studies also disclosed myofibroblastic features. The locally aggressive, recurrent nature of these neoplasms, as well as the occurrence of metastases and tumor deaths, indicate that they are potentially malignant neoplasms that we believe are better classified as inflammatory fibrosarcomas, not as cellular inflammatory pseudotumors.
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PMID:Inflammatory fibrosarcoma of the mesentery and retroperitoneum. A tumor closely simulating inflammatory pseudotumor. 174 82

Leiomyoblastomas are rare smooth muscle tumors occurring most commonly in the gastrointestinal tract of adults. We report the unusual occurrence of a gastric leiomyoblastoma in a child who presented with refractory iron deficiency anemia. The tumor had a superficial erosion which was apparently the source of intermittent hemorrhage resulting in anemia. Epithelioid cells, spindled cells, and cells arranged in a perithelial pattern were seen by microscopy, characteristic of a leiomyoblastoma. Immunohistochemical staining demonstrated muscle actin, vimentin, desmin, and alpha-1-antichymotrypsin, and electron microscopy showed focal densities alternating with thin filaments. All of these features help to differentiate the tumor from an inflammatory pseudotumor. Leiomyoblastomas are associated with paragangliomas and pulmonary chondromas in children, but these were not present in this child. The prognosis of leiomyoblastomas may be related to tumor size, mitotic rate, and cellular characteristics.
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PMID:Gastric leiomyoblastoma (epithelioid leiomyoma) occurring in a child: a case report. 271 1

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

Gastrointestinal Autonomic Nerve Tumors (GANTs) are an underrecognized group of gastrointestinal stromal tumors (GISTs) putatively arising from the neural plexuses of the bowel wall. Approximately 24 cases have been previously reported. Their histogenesis, malignant potential, morphology and phenotypic features are not well defined. We present details of 10 GANTs iterating features, predominantly ultrastructural, allowing distinction from other GISTs. Clinical details are: sex-7M, 3F; age range 31-79 yrs, mean 53; symptoms/signs--abdominal pain 3, GI bleeding 3, mass 2, anemia 2. Follow-up ranged from 1-102 mths, mean 29. Seven tumors involved the small intestine and 3 were gastric. Tumor size ranged from 30-160 mm, mean 79. They were solid and cystic, often transmural and usually involved mesentery and retroperitoneum. Spindled and epithelioid cells were "compartmentalized" by a branching microvasculature. Eosinophilic, PAS positive stromal globules were prominent. Paraffin immunostaining results were (number positive/total): vimentin (8/9), NSE (10/10), S100 protein (6/10), neurofilament protein (0/9), synaptophysin (3/9), desmin (2/9, focal), smooth-muscle actin (0/9). Ultrastructural diagnostic features were elaborate, branching cytoplasmic processes containing microtubules, intermediate filaments and varying numbers of neurosecretory granules. Characteristic features were elaborate smooth endoplasmic reticulum enmeshed with intermediate filaments, pleomorphic mitochondria with lamellar cristae, mitochondrial-RER complexes, confronting RER cisternae, and circumscribed collections of stromal "skeinoid" fibres. There were no features of smooth muscle, Schwannian or perineurial differentiation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Gastrointestinal autonomic nerve tumors: a clinicopathological, immunohistochemical and ultrastructural study of 10 cases. 789 47

We describe a rare example of inflammatory pseudotumor of the pancreas in a 42-yr-old woman, which developed following chemotherapy for lymphoma of the uterine cervix. The patient had developed fatigue, weight loss, abdominal pain, and anemia; abdominal CT scan showed a large mass in the pancreas. Examination of the resected specimen revealed a fleshy, well-circumscribed, 7-cm mass. Histologically, there was a hypocellular to moderately hypercellular, bland spindle-cell proliferation admixed with a prominent infiltrate of lymphocytes, histiocytes, and plasma cells. The spindle cells were vimentin positive but negative for muscle markers; electron microscopy revealed only fibroblastic cells. DNA analysis revealed a diploid population with low S-phase fraction. The patient was well at 6-mo follow-up. It is important for the pathologist to be aware of the existence of this entity in unusual locations such as the pancreas so as to avoid a mistaken diagnosis of malignancy.
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PMID:Inflammatory pseudotumor of the pancreas. 870 1

We report a gastrointestinal autonomic nerve tumor of the stomach with a giant abscess. The patient had fever and pain and was found to have anemia and an abdominal mass. X-ray and endoscopic examination showed a gastric submucosal tumor with a fistula to the gastric lumen. Partial gastrectomy was performed and no metastasis was found. On gross examination, the excised tumor was seen to be a submucosal solid tumor with a giant abscess. Alpha streptococci and anaerobic gram-negative rods were cultured from the pus of the abscess. The tumor resembled a gastric myogenic tumor composed of spindle cells, partly showing storiform and epithelioids. Tumor cells showed positive staining for vimentin and neuron-specific enolase but were negative for desmin, alpha-smooth muscle actin, and S-100 protein. Ultrastructural examination showed remarkable interdigitation of cytoplasmic processes with neurosecretory granules between the tumor cells. This lesion was similar to previously described gastrointestinal autonomic nerve tumors. Gastrointestinal autonomic nerve tumors are a rare, distinct subtype of gastrointestinal stromal tumors; although several cases of focally necrotic tumors have been reported, there has been only one report of the tumor with an abscess, as in our case.
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PMID:Gastrointestinal autonomic nerve tumor with giant abscess. A case report and literature review. 925 61

The clinicopathologic, immunohistochemical, and ultrastructural features of soft tissue angiosarcomas are not well defined. Eighty cases of angiosarcoma that involved the deep subcutis, skeletal muscle, retroperitoneum, mesentery, and mediastinum are reported. The lesions occurred in 50 male and 30 female patients who were 5-97 years of age; the peak incidence was in the seventh decade of life. A variety of associated conditions were documented in 20 of these cases, including a history of other neoplasms (some irradiated), synthetic vessel grafts, heritable conditions, and prior trauma or surgery. The angiosarcomas occurred in the extremities (n = 43 cases), trunk (n = 28), and the head and neck (n = 9) regions, with the thigh and the retroperitoneum being the most common sites. They often were characterized as enlarging, painful masses of several weeks' duration and were occasionally associated with acute hemorrhage, anemia, or a coagulopathy. The tumors measured 1-15 cm in diameter (median 5 cm) and frequently were hemorrhagic and multinodular. There was a wide morphologic spectrum within and between cases, including areas similar to cavernous and capillary hemangioma, Dabska tumor, spindle cell and epithelioid hemangioendothelioma, various spindle cell sarcomas, or carcinoma. Histologically, epithelioid angiosarcoma was the most frequently observed pattern; 70% of cases had epithelioid cells that were arranged in nests, clusters, papillae, and gaping vascular channels. Hemorrhage tended to obscure the diagnosis in several cases and often was associated with papillary endothelial hyperplasia-like areas. All 42 cases studied immunohistochemically stained at least focally for Factor VIII-related antigen, and nearly all stained strongly for vimentin, which accentuated the endothelial cells and vessel lumen formation. CD34 antigen was detected in 74% of cases, BNH9 in 72%, and cytokeratins in 35%. Epithelial membrane antigen, S-100 protein, and HMB45 were not detected. Fifty-five percent of the tumors had intracytoplasmic aggregates of laminin. Immunostains for alpha-smooth muscle actin demonstrated a prominent pericytic component in several tumors (24%). Ki67 immunostains with MIB1 indicated high proliferative activity (> or =10%) in 72% of cases. p53 immunoreactivity (>20% nuclear staining) was observed in 20% of cases. Ultrastructural studies performed on poorly differentiated areas of 12 cases showed groups of cells, which were frequently epithelioid, surrounded by basal lamina, and closely associated with pericytes, along with intercellular and intracellular lumina with or without red blood cells. Whorls of abundant intermediate filaments, occasional tonofilamentlike structures, and pinocytotic vesicles also were noted. In contrast to the findings of others, Weibel-Palade bodies were not seen. Follow-up in 49 cases (61%) showed that 53% of patients were dead of disease at a median interval of 11 months, whereas 31% had no evidence of disease at a median interval of 46 months. The remaining patients were either alive with disease (14%) or alive but disease status was unknown (2%). There were local recurrences in 20% of cases and distant metastases in 49%, most frequently to the lungs, followed by the lymph nodes, soft tissues, bone, liver, and other sites. These results indicate that angiosarcoma of soft tissue is a high-grade sarcoma. Older patient age, tumor location in the retroperitoneum, and larger tumor size as well as detection of MIB1 in > or =10% of the tumor cell population were all associated with a poorer prognosis.
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PMID:Angiosarcoma of soft tissue: a study of 80 cases. 963 Jan 75

A yearling Arabian filly was referred to the Veterinary Medical Teaching Hospital with a history of weight loss, profound anemia, and peritoneal effusion. At necropsy, a large, soft, mottled tan and red neoplastic mass was at the pelvic inlet replacing the left ovary. Additional tumor nodules of various sizes were disseminated throughout the mesentery, diaphragm, and serosal surfaces of the abdominal viscera. Histologically, the neoplasm had sheets of large round to polygonal cells separated into lobules by fibrous connective tissue with multifocal areas of necrosis. Tumor cells stained strongly for alkaline phosphatase. Immunohistochemically, tumor cells expressed vimentin and were negative for cytokeratin. Ultrastructurally, the neoplastic cells had a characteristic nucleolus with an elaborate reticular nucleolonema in an irregular configuration. This is the first in-depth detailed report of this very rare germ cell tumor of the ovary in horses.
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PMID:Dysgerminoma in an Arabian filly. 968 77

A 75-year-old male presented to our hospital with a complaint of macroscopic hematuria. Laboratory examinations in peripheral blood showed slight anemia and the increase of acute phase reactants (c-reactive protein, immunosuppressive acidic protein, alpha 2-globulin). Abdominal enhanced computerized tomography revealed a huge tumor with calcification at the upper pole of the right kidney. Magnetic resonance imaging (MRI) showed a low intensity mass at the upper portion of the right kidney on T2-weighted sequences. The right radical nephrectomy was performed and the histopathological diagnosis was sarcomatoid renal cell carcinoma because of positive immunohistochemical staining for vimentin and negative for cytokeratin. Because sarcomatoid type has a highly malignant behavior and poor prognosis among renal cell carcinomas, an adjuvant treatment which is effective in controlling the disease is awaited.
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PMID:[Sarcomatoid renal cell carcinoma: a case report]. 1087 54

To clarify the cellular differentiation features and facilitate diagnosis of angiomatoid (malignant) fibrous histiocytoma (AFH), four cases of AFH were examined by clinicopathologic, immunohistochemical and ultrastructural analyses. The age of the patients ranged from 10 to 24 years (mean, 17 years) and the sex distribution was equal. All cases were of subcutaneous origin: three arose in the trunk and one in the upper extremity. All patients presented with systemic symptoms, including inflammatory signs and anemia. After a mean follow up of 11 years 3 months, all patients were alive and well, although one patient twice developed local recurrence after surgery. All cases presented as multinodular, cystic and hemorrhagic tumors ranging in size from 4 to 11 cm (mean, 8 cm) and were characterized by sheets of bland spindle or round cells with oval nuclei within a circumscribed nodule often surrounded by a lymphocytic cuff. One tumor showed predominantly round cell morphology similar to Ewing's sarcoma/primitive neuroectodermal tumor. All cases (100%) exhibited immunoreactivity for vimentin, desmin, CD68 and CD57 (Leu-7). Three cases (75%) were positive for synaptophysin, and reactivity for alpha-smooth muscle actin, epithelial membrane antigen, neuron-specific enolase and CD99 (O-13) was present in two cases (50%) each. The three cases examined by electron microscopy had a mixture of fibrohistiocytic, myofibroblastic and undifferentiated cells containing cytoplasmic processes and dense-core granules. It is important for accurate diagnosis of this peculiar soft-tissue tumor to recognize that it has a variety of immunophenotypes, such as histiocytic, myofibroblastic, epithelial and neural, and may occasionally have a predominantly round cell morphology.
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PMID:Angiomatoid (malignant) fibrous histiocytoma: a peculiar low-grade tumor showing immunophenotypic heterogeneity and ultrastructural variations. 1101 87


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