Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Extramedullary hematopoiesis is extremely rare in the absence of any hematologic disorder or systemic disease, and there have been no reports of this condition in the endometrium. The authors describe the cases of four patients who complained of irregular uterine bleeding, lower abdominal pain, or both. Microscopic examination of the curetted endometria showed chronic inflammation in two patients, chronic inflammation and leiomyomas in one patient, and a well-differentiated adenosquamous adenocarcinoma with adenomyosis and leiomyomas in one patient. Light-microscopic examination of curetted sections from all four patients revealed isolated foci of extramedullary hematopoiesis composed of one or more lineages of blood cell precursors. Sufficient tissue was available from two patients to confirm hematopoietic differentiation through immunohistologic analysis, using antibodies to von Willebrand factor, ABH blood group antigens, CD34, and CD15.
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PMID:Extramedullary hematopoiesis in the endometrium. Report of four cases and review of the literature. 817 71

We describe the clinicopathological features of gastrointestinal autonomic nerve tumours in nine patients where the diagnosis was confirmed by electronmicroscopy. Most patients presented with abdominal pain. At laparotomy, large intra-abdominal tumour masses were found which tended to be cystic and haemorrhagic. The predominant histological patterns were nests, sheets and fascicles of spindle and epithelioid cells. Immunohistochemistry showed positive staining for neuron specific enolase (9/9), PGP 9.5 (9/9), NKI/C3 (7/9), vimentin (7/9), alpha-smooth muscle actin (5/9), vasoactive intestinal peptide (3/9) and CD34/QBend10 (2/9). Grimelius staining was positive in two of nine cases. All tumours were negative for CAM 5.2, chromogranin, synaptophysin, Leu 7, neurofilament protein, muscle-specific actin (HHF-35) and desmin (D33). Ultrastructural examination showed cellular processes and dense-core granules in all cases. Three tumours had microtubules and/or intermediate filaments, particularly in cell processes. Skeinoid fibres were seen in three cases. No convincing synapses or small (synaptic-type) vesicles were identified. There was no evidence of epithelial, smooth muscle or nerve sheath differentiation. Two patients died due to tumour, two died of unknown causes and the remainder are alive 2-44 months after presentation. Four of the five survivors have recurrent/residual intra-abdominal tumour. So-called gastrointestinal autonomic nerve tumours are apparently slow-growing malignant tumours showing neuronal differentiation. Four cases arose in the mesentery/retroperitoneum or omentum rather than bowel wall and therefore a more appropriate nomenclature might be intra-abdominal stromal tumour with neuronal differentiation.
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PMID:Gastrointestinal autonomic nerve tumours: a report of nine cases. 887 44

A total of 28 patients with leukemia underwent intensive therapy with megadose chemotherapy and stem cell transplantation. Of 16 patients who had busulfan therapy, 2 developed the clinical syndrome of veno-occlusive disease of the liver (VOD) characterized by progressive abnormalities in liver function, abdominal pain, and ascites. Postmortem findings showed VOD of the central and sublobular hepatic vein. Immunohistochemical analysis using anti-actin and CD34 mAb was employed in an attempt to clarify the process of fibrosis. Actin positive cells had accumulated around the central vein. Ito cell activation might be responsible for secondary fibrosis following endothelial injury. We report autopsy findings and immunohistochemical results.
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PMID:Veno-occlusive disease of the liver following bone marrow transplantation: a clinical-pathological study of autopsy cases. 889 36

The clinical, histologic, and immunohistochemical features of six cases of hemorrhagic adrenal pseudocysts are reported together, with a review of the English literature on this topic since 1950. The mean age at presentation was 57 years (range, 30-72 yr). There were four men and 2 women. The average cyst size was 9.2 cm (range, 6-16 cm). In four patients, the hemorrhagic adrenal pseudocysts were incidental findings. The remaining two patients presented with an abdominal mass and hypertension, respectively. The hemorrhagic pseudocysts were unilocular cystic masses surrounded by a fibrous capsule and containing abundant amorphous material, blood, and fibrin. Numerous dilated, thin-walled, vascular channels that stained strongly for Factor VIII-related antigen, collagen IV, laminin, Ulex europaeus agglutinin I lectin, and CD34 were present within the fibrous capsule, cyst contents, and surrounding residual adrenal gland. These findings support a vascular origin for these lesions, and they are thought, therefore, to be related to endothelial adrenal cysts. The literature review of 111 vascular adrenal cysts (85 hemorrhagic pseudocystic type and 26 endothelial type) showed similar clinical features. The mean age at presentation was 44.5 years (range, 5 d-95 yr), with a female predominance (62%). The most common clinical presentation was abdominal pain (35%), followed by incidental findings (32%). There were no significant clinical differences between hemorrhagic and endothelial type cysts. In some cases, the presence of intracystic islands of cortical cells can cause diagnostic confusion with adrenal cortical tumors. The presence, however, of a rich intracystic and capsular vascular network, normal-appearing islands of cortical cells, and abundant thrombotic fibrinous material, rather than necrotic tumor cells, should rule out the possibility of a degenerating adrenal cortical neoplasm.
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PMID:Vascular adrenal cysts: a clinicopathologic and immunohistochemical study of six cases and a review of the literature. 919 68

Angiosarcoma developed at the site of a Dacron vascular prosthesis 8 years after an aortobifemoral bypass graft insertion. The tumor was composed of epithelioid cells, which showed positive staining for cytokeratin and expression of the common endothelial markers CD31, CD34, and von Willebrand factor. Ultrastructural examination showed aggregates of large cells with intercellular lumina and focal perinuclear whorls of intermediate filaments. The patient, who had abdominal pain and weight loss, died of disseminated pelvic and abdominal disease 6 months after diagnosis. Sarcomas associated with vascular Dacron grafts and angiosarcomas associated with metal or polymer foreign bodies are rare. Their development is probably analogous to the common experimental development of foreign body-associated sarcomas in rodents. Physicians caring for patients with vascular grafts or metal foreign bodies should be aware of this complication.
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PMID:Epithelioid angiosarcoma associated with a Dacron vascular graft. 1055 12

Adrenal vascular cysts are rare lesions that might be considered in the differential diagnosis of adrenal tumors. Their origin is not clear. We report the clinicopathological findings of a large adrenal hemorrhagic pseudocyst (AHP) in a 73-yr-old man who complained of abdominal pain. An abdominal CT showed a 9 cm tumor in the left adrenal. A fine-needle aspiration biopsy (FNAB) was hemorrhagic and inconclusive. The tumor was excised and touch imprints were taken showing groups of spindled and fusiform cells with elongated nuclei, without atypia. Histologically, the tumor was well delimited by a fibrous capsule and contained numerous cystic spaces lined by endothelial cells and filled with erythrocytes, fibrin thrombus, and necrotic debris. Immunohistochemical study showed strong positivity for factor VIII-RA, CD31, and CD34. Also, the remaining adrenal showed a prominent frame of thin and medium caliber vessels, supporting a vascular origin for this entity. This case illustrates the difficulty in making a diagnosis by FNA and to keep in mind AHP when hematic aspirates are obtained from an adrenal tumor mass.
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PMID:Vascular adrenal pseudocyst: cytologic and immunohistochemical study. 1064 23

Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibroushistiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.
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PMID:Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases. 1100 38

We report two patients with gastrointestinal stromal tumors (GISTs) of the small intestine that expressed c-kit protein (CD117). One was a 68-year-old woman with epigastralgia and vomiting. A submucosal tumor of the upper jejunum was detected, and partial resection was carried out. The histology revealed a GIST negative for CD34 but positive for CD117. The other was a 42-year-old woman with progressive anemia, melena and lower abdominal pain. Intussusception was detected, and a partial resection was carried out. A submucosal tumor of the lower jejunum was noted. The histology revealed a GIST positive for both CD34 and CD117.
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PMID:Gastrointestinal stromal tumors of the small intestine that expressed c-kit protein. 1106 32

Schwannomas of the colon and rectum are uncommon and incompletely characterized tumors, and only a small number of cases have been reported. This study was undertaken to determine the clinicopathologic profile of such tumors. A total of 20 colorectal schwannomas were identified and analyzed in a review of 600 mesenchymal tumors of the colon and rectum from the files of the Armed Forces Institute of Pathology. The schwannomas occurred equally in men (n = 9) and women (n = 11) in a wide age range (18-87 years; median age 65 years). The most common location was cecum (n = 7), followed by sigmoid and rectosigmoid (n = 6), transverse colon (n = 3), descending colon (n = 2), and rectum (n = 1); the location of one tumor had not been specified. The tumors commonly presented as polypoid intraluminal lesions, often with mucosal ulceration. Rectal bleeding, colonic obstruction, and abdominal pain were the most common presenting symptoms. The most common histologic variant (n = 15) was a spindle cell schwannoma with a trabecular pattern and vague or no Verocay bodies. These tumors ranged from 0.5 to 5.5 cm in diameter. A lymphoid cuff with germinal centers typically surrounded these tumors and focal nuclear atypia was often present, but mitotic activity never exceeded 5 per 50 HPF. All four epithelioid schwannomas occurred in the descending colon or sigmoid, three of them as small submucosal tumors. There was one plexiform schwannoma in the sigmoid composed of multiple nodules of prominently palisading schwann cells similar to those seen in conventional soft tissue schwannomas. All tumors studied were strongly positive for S-100 protein and also for low affinity nerve growth factor receptor (p75), collagen IV, and GFAP. Three tumors had CD34-positive cells, but all were negative for CD117 (KIT), neurofilament proteins, smooth muscle actin, and desmin. The percentage of MIB-1-positive cells was usually less than 1% and never higher than 3%. Colorectal schwannomas behaved in a benign fashion with no evidence of aggressive behavior or connection with neurofibromatosis 1 or 2, based on follow-up information on 18 patients.
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PMID:Schwannomas in the colon and rectum: a clinicopathologic and immunohistochemical study of 20 cases. 1142 Apr 55

Malignant mesenchymal tumors of the gallbladder are exceedingly rare. We report a malignant stromal tumor of the gallbladder with a phenotype of interstitial cells of Cajal. To our knowledge, only the benign counterpart of this tumor has been described previously. A 34-year-old woman presented with right upper quadrant abdominal pain. At the time of cholecystectomy, the gallbladder was noted to have a thickened wall and a polypoid mass arising in the neck of the gallbladder. Histologic sections showed a cellular proliferation of spindle neoplastic cells that were arranged in short fascicles. Numerous mitotic figures and foci of necrosis were noted. The neoplastic cells expressed CD117 (c-Kit protein) and vimentin. They were negative for smooth muscle actin, desmin, myoglobin, cytokeratin, S100 protein, and CD34. Our case demonstrates that a malignant stromal tumor that is histologically and immunohistochemically identical to gastrointestinal stromal tumor can occur in the gallbladder, and that the expression of CD117 may be of therapeutic importance.
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PMID:Malignant stromal tumor of the gallbladder with interstitial cells of Cajal phenotype. 1190 May 79


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