Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Six patients with ovarian sex cord stromal tumor with annular tubules (SCTAT) were diagnosed and treated from 1968 through 1988, accounting for 6% of a total of 99 cases of ovarian sex cord stromal tumors admitted during the same period. Of these, 3 were operated on for the first time and 3 were recurrent cases referred from other hospitals. The menstrual disturbance was characterized by menometrorrhagia followed by sustained amenorrhea. Glandular atrophy and decidual change of stromal were observed in 3 of the 4 endometrial samples available for histologic examination. Serum hormone assays carried out in 2 cases revealed elevated levels of both estradiol and progesterone. The primary tumor was unilateral in 5 cases and bilateral in one the recurrent tumors were exclusively retroperitoneal and ipsilateral to the primary ovarian tumors. In these cases, lymphatic metastasis was also observed in the ipsilateral pelvic lymph nodes. Complete and sustained remission has been achieved in all 3 patients receiving primary surgery by unilateral salpingo-oophorectomy. After removal of the metastatic tumors in 3 recurrent cases, 2 have survived with residual diseases for 2 and 9 years respectively and one died 4 years after the secondary surgery.
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PMID:[Ovarian sex cord stromal tumors with annular tubules: a report of 6 cases]. 220 22

Twenty-one cases in which sarcomas metastasized to the ovaries are reported. The patients ranged from 18 to 79 (average 42) years of age; only five of them were over 50 years old. Eleven tumors were primary in the uterus and 10 outside the genital tract. Three uterine tumors were leiomyosarcomas, and eight, endometrial stromal sarcomas. The extragenital primary tumors were leiomyosarcoma of the stomach (1) and small intestine (2), retrovesical leiomyosarcoma (1), fibrosarcoma of the anterior abdominal wall (1), sarcoma of the mesentery of smooth muscle or neural type (1), hemangiosarcoma probably primary in the heart (1), osteosarcoma of the maxilla (1), chondrosarcoma of the rib (1), and Ewing's sarcoma of the pubic bone (1). The ovarian tumors, most of which were large, were discovered at the same time as the primary tumors in 11 cases; in seven cases, the ovarian tumor was discovered 7 months to 9 years after diagnosis of the primary tumor. In three cases, the ovarian tumors were discovered 4, 7, and 10 months before detection of the primary neoplasm. Two of these tumors were endometrial stromal sarcomas, and one, an epithelioid leiomyosarcoma of the stomach. Eleven ovarian metastases were bilateral. On microscopic examination, the greatest difficulty in pathologic interpretation was posed by the metastatic endometrial stromal sarcomas because of their simulation of sex cord-stromal tumors. Features helpful in their distinction from these tumors included the frequent presence of extra-ovarian disease, bilaterality, and a characteristic content of small arteries resembling the spiral arteries of the late secretory endometrium. The other tumor that caused major diagnostic difficulty was the metastatic epithelioid leiomyosarcoma from the stomach, which had a pattern that initially suggested the solid-tubular pattern of a Sertoli cell tumor.
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PMID:Sarcomas metastatic to the ovary: a report of 21 cases. 237 88

Hepatic metastases occur rarely in epithelial ovarian carcinoma and also appear to be unusual in malignant stromal tumors of the ovary (granulosa cell tumors). Recently two patients with extensive hepatic metastasis from this primary tumor, were treated. Review of the experience at the City of Hope National Medical Center provided three additional patients with a confirmed diagnosis of granulosa cell tumor of the ovary, one of whom is alive and disease-free. A review of clinical and pathologic data revealed that both of the other patients died of their disease and had hepatic metastases proven at autopsy. Hemorrhagic events complicating the clinical course of these patients were frequent. It is believed that the frequency of hepatic metastasis in granulosa cell tumor of the ovary may be higher than has been appreciated in the past, and that the cystic-hemorrhagic nature of these lesions contributes to the morbidity and mortality associated with granulosa cell tumors of the ovary.
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PMID:Hepatic metastasis in granulosa cell tumor of the ovary. 389 Oct 73

Certain tumors of the female genital tract have a very probability of expressing CEA activity. These include endocervical carcinomas and ovarian Brenner tumors. This information may be helpful in allowing one to distinguish among several diagnostic possibilities on a given biopsy. CEA detection apparently fails to distinguish between endometrial hyperplasias versus endometrial carcinomas. Gonadal stromal tumors of the ovary are generally negative, whereas the common epithelial tumors (depending on the histological type) have a reasonable probability of having detectable antigen. Since plasma CEA levels have had a demonstrated usefulness in following patients who were known to have elevated CEA levels prior to the removal of their primary tumor in the endometrium, ovary, and cervix, it is valuable to have an estimate of probability that a tumor will express CEA in evaluating the results of plasma radioimmunoassays for this antigen.
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PMID:Carcinoembryonic antigen in gynecologic patients. II. Immunohistological expression. 733 57

Endometrial stromal tumors may pose a problem in diagnosis when they appear as metastatic lesions without a known primary tumor. To determine the usefulness of electron microscopy in identifying them in these situations, optimally fixed low-grade stromal sarcomas (five), normal endometrial specimens (six), and malignant mesodermal mixed tumors (four) were studied. The endometrial stromal sarcomas had a general resemblance to normal proliferative endometrial stroma, being composed of undifferentiated cells, fibroblasts, and myofibroblasts. One stromal tumor showed evidence of partial epithelial differentiation. One of the four malignant mesodermal mixed tumors had a fibrosarcomalike component, but there was insufficient resemblance to normal endometrial stroma to indicate a relationship between the two. Together with a review of the literature, this study indicates that electron microscopy is useful in the diagnosis of low-grade endometrial stromal tumors by demonstrating characteristic cellular findings as well as a lack of features specific for other round cell and spindle cell neoplasms.
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PMID:Role of electron microscopy in metastatic endometrial stromal tumors. 826 99

The first published chromosomal pattern of the retroperitoneal lymph node metastasis of a malignant gonadal stroma cell tumor of the adult testis is presented. Karyotyping showed structural chromosomal abnormalities and loss of the Y-chromosome. This loss was confirmed in primary tumor and metastasis using fluorescence in situ hybridization (FISH). The characteristic chromosomal abnormality of adult testicular germ cell tumors, an i(12p), was not present. The results are compared with other data of testicular and ovarian sex cord stromal tumors. From the comparison of the male tumors, it is concluded that loss of the Y-chromosome might have a pathogenetic significance.
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PMID:Loss of the Y-chromosome in the primary metastasis of a male sex cord stromal tumor: pathogenetic implications. 1043 30

Gastrointestinal stromal tumors (GIST), which form a rare group of neoplasias of the gastrointestinal tract, have not yet been fully investigated. Although good progress has been made in their diagnosis, classification of these lesions with regard to their histogenesis and biological behavior remains problematic. Between 1994 and 1998, 18 GIST patients underwere operation in the Department of Surgery. Twelve of these patients (67%) had stromal tumors in the stomach, and six (33%) had intestinal stromal tumors. The primary tumor could be removed in all patients with R0 resection. Six patients developed hematogenous liver metastasis, with the size of their primary tumor exceeding 10 cm. Extrahepatic distant metastases were not found in any case. Lymphadenectomy showed that lymph node metastases did not occur. Histological evaluation was made according to the guidelines of Lewin, Weinstein and Riddell. Currently established therapy is limited to complete surgical resection of the primary tumor and its metastases. Adjuvant or neoadjuvant chemotherapy approaches have failed.
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PMID:[Gastrointestinal stromal tumors--problems in diagnosis and therapy]. 1044 91

Gastrointestinal stromal tumors (GISTs), the most common mesenchymal tumors of the gastrointestinal tract, typically express the KIT protein. Activating mutations in the juxtamembrane domain (exon 11) of the c-kit gene have been shown in a subset of GISTs. These mutations lead into ligand-independent activation of the tyrosine kinase of c-kit, and have a transforming effect in vitro. Several groups have studied the clinical implication of the c-kit mutation status of exon 11 in GISTs and a possible relationship between c-kit mutations and malignant behavior has been established. Recently, a 1530ins6 mutation in exon 9 and missense mutations, 1945A>G in exon 13 of the c-kit gene were reported. The frequency and clinical importance of these findings are unknown. In this study we evaluated 200 GISTs for the presence of mutations in exons 9 and 13 of c-kit. Six cases revealed 1530ins6 mutation in exon 9 and two cases 1945A>G mutation in exon 13. All tumors with mutations in exon 9 and 13 lacked mutations in exon 11 of c-kit. None of the analyzed tumors had more than one type of c-kit mutation. All but one of the eight tumors with mutations in exon 9 or 13 of the c-kit gene were histologically and clinically malignant. All four of six cases with exon 9 mutation of which location of primary tumor was known, were small intestinal, suggesting that this type of mutation could preferentially occur in small intestinal tumors. Exon 9 and 13 mutations seem to be rare, and they cover only a small portion (8%) of the balance of GISTs that do not have mutations in exon 11 of c-kit. This finding indicates that other genetic alterations may activate c-kit in GISTs, or that KIT is not activated by mutations in all cases.
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PMID:Mutations in exons 9 and 13 of KIT gene are rare events in gastrointestinal stromal tumors. A study of 200 cases. 1102 12

Three endometrial and one extrauterine endometrioid stromal tumors (three sarcomas and one stromal nodule) with a prominent component of epithelioid cells with abundant eosinophilic cytoplasm are described. The patients were 39, 48, 56 and 86 years of age. The endometrial sarcomas were described grossly as an ill-defined tan nodule and "ragged and papillary," respectively, and had the typical infiltrative pattern of low-grade endometrial stromal sarcoma. The stromal nodule was a 13-cm, well circumscribed, yellow, fleshy mass. The extrauterine tumor was probably primary in the sigmoid colon. Oval to polygonal epithelioid cells with abundant eosinophilic cytoplasm accounted for 50% to 90% of the tumor cells. The cytoplasm was granular in one case. None of the tumors contained cells with a rhabdoid appearance. Nuclear and other features did not differ from those of usual endometrial-endometrioid stromal tumors except in one case in which there was greater nuclear pleomorphism. There was strong diffuse cytoplasmic immunoreactivity of all four tumors for vimentin and for CD10 in three of three tumors tested, as well as extensive and moderate reactivity for NK1/C3 and focal weak reactivity for CD68 in two of three tumors tested. Muscle actin positivity was very focal to extensive and weak to strong in all three tumors tested, mainly in the epithelioid areas; alpha-smooth muscle actin was focally to extensively positive in the epithelioid areas of two of three tumors tested; and focal strong desmin positivity (interpreted as indicating smooth muscle metaplasia) was found in the epithelioid areas of one of four tumors. A vaginal recurrence in one case had similar cytologic features to the primary tumor but when examined initially in the absence of adequate history posed diagnostic difficulty, as did evaluation of the uterine tumor in two other cases and the extrauterine tumor in the final case. The differential in these cases is primarily with an epithelioid smooth muscle tumor when they are uterine primaries. The typical infiltration facilitates this distinction in the cases of endometrial stromal sarcomas, but this feature is usually only evident in hysterectomy specimens. In limited samples such as biopsy or curettage specimens, and in some cases of recurrent tumor, awareness that endometrial-endometrioid stromal tumors can have epithelioid cells is crucial in the formulation of the differential diagnosis. Diverse oxyphilic tumors, including deciduoid malignant mesothelioma, can potentially be in the differential diagnosis with extrauterine (endometrioid) stromal sarcomas with epithelioid cells. Immunohistochemical evaluation may potentially provide major aid in diagnosis.
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PMID:Epithelioid endometrial and endometrioid stromal tumors: a report of four cases emphasizing their distinction from epithelioid smooth muscle tumors and other oxyphilic uterine and extrauterine tumors. 1178 23

Primary malignant tumors of the small intestine are rare. Malignant gastrointestinal stromal tumors are the third most common neoplasm among primary malignant small bowel tumors. A 56-year-old woman was admitted to our hospital because of appetite loss and dyspnea with movement. On admission, physical examination revealed severe anemia in her conjunctiva and a tumor in her left abdomen. Her hemoglobin level was 6.2 g/dL and other laboratory data were normal. Abdominal ultrasonograms and computed tomograms revealed a 55 x 70-mm heterogeneous mass and multiple low-density masses in the liver. Superior mesenteric arteriograms revealed a hypervascular tumor fed by the jejunal arteries. A malignant gastrointestinal stromal tumor arising from the jejunum with liver metastases was suspected. Partial resection of the affected jejunum and left trisegmentectomy of the liver were performed. The resected primary tumor was 120 x 45 x 65 mm. The tumor was mainly submucosal, but extended outside the jejunum; it was elastically firm and multiloculated. A small ulcer was seen on the mucosal side. The metastatic liver tumors were solid or cystic with diameters of 20 to 40 mm. Histopathological examination revealed that the tumors were characterized by fascicular proliferation of spindle-shaped cells. Immunohistochemical staining was positive for CD34 and c-kit, and negative for S-100 protein and smooth muscle actin. This case was a malignant gastrointestinal stromal tumor originating in the jejunum with liver metastases. The primary tumor and liver metastases were successfully resected simultaneously.
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PMID:Malignant gastrointestinal stromal tumor of the jejunum with liver metastasis. 1223 33


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