Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P10721 (c-kit)
 6,575 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Gonadoblastoma is defined as a neoplasm containing nests of germ cells and cells resembling Sertoli cells or granulosa cells. Gonadoblastomas arise almost exclusively in dysgenetic gonads. They are associated with an increased risk of developing germ cell tumours. Testicular germ cell tumours in adults are preceded by carcinoma in situ cells, which are characterized by their morphology, by their immunohistochemical expression of placental-like alkaline phosphatase, the proto oncogene c-kit and/or epitopes for the monoclonal antibodies M2A, 43-9F and TRA-1-60, and by their aneuploid DNA content. In order to elucidate if gonadoblastomas are in situ neoplasms from the beginning, showing similarities with carcinoma in situ cells in otherwise normal testes, we investigated the germ cells in gonadoblastomas for their expression of the immunohistochemical markers of carcinoma in situ cells from six patients aged 8 1/2 months to 20 years and 4 months. In addition, the DNA content of the germ cells from five of the six patients was also determined by densitometric measurement on Feulgen stained specimens. The germ cell populations were heterogeneous both within the same patient and between the patients. Expression of the testicular carcinoma in situ markers was detected in specimens from all the patients and germ cells with an aneuploid DNA distribution pattern in accordance with testicular carcinoma in situ cells were detected. However, apparently normal immature germ cells were also present in four of the patients of whom two also had germ cells with a morphology similar to normal spermatogonia. Thus, gonadoblastoma is most likely an in situ germ cell neoplasia from the beginning. It seems probable that the germ cell tumours associated with gonadoblastomas originate from the carcinoma in situ cells inside the gonadoblastoma. Our findings of carcinoma in situ cells in gonadoblastomas from children support the theory that the cells arose prenatally.
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PMID:Heterogeneity of gonadoblastoma germ cells: similarities with immature germ cells, spermatogonia and testicular carcinoma in situ cells. 919 Mar 60

Gonadoblastomas are rare mixed germ cell neoplasms, which are frequently diagnosed in testes excised for other reasons. In human patients these tumours are usually associated with undescended testes or dysgenetic gonads. This report describes a 10-year-old male dog with mixed gonadal dysgenesis ("streak testis" on the right side and testis on the left side) and bilateral gonadoblastomas. Immunohistochemical analysis revealed an atypical and mitotically active germ cell population which co-expressed placental alkaline phosphatase and c-kit oncogene, admixed with inhibin- and S-100 protein-positive sex cord cells. The findings were generally consistent with previous reports of the dual population of neoplastic cells in gonadoblastomas affecting human patients, and closely resembled the findings in the only other case reported in dogs. Moreover, the findings further support the proposal that gonadoblastomas should be considered a special form of in-situ germ cell neoplasia.
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PMID:Bilateral gonadoblastomas in a dog with mixed gonadal dysgenesis. 1500 85

Gonadoblastomas are neoplasms of dysgenetic gonads which may undergo regression or become overgrown by malignant germ cell tumors (mGCTs). Since little is known about their relationship to normal gonadal development and mGCTs, we studied the phenotype and antigenic profile of gonadoblastomas in comparison with adjacent dysgerminomas and fetal gonads. Three cases of gonadoblastomas and fetal gonads of both sexes were analyzed using oncofetal markers to M2A-antigen (M2A), germ cell alkaline phosphatase (PLAP/GCAP), receptor tyrosine kinase c-kit (c-kit), and somatic angiotensin converting enzyme (sACE) as well as the proliferation marker MIB-1. Morphologically, microfollicular pattern of gonadoblastomas showed a fetal germ cell organization reminiscent of oocytic clusters of fetal ovaries. They contained both cell types, similar to oocytes (M2A-, GCAP-, c-kit+/-, sACE-) and oogonia (M2A+, GCAP+, c-kit+, sACE+). The percentage of germ cells immunoreactive for oncofetal markers and the proliferation index increased from microfollicular over coronary patterns to adjacent dysgerminomas. Supportive cells of gonadoblastomas showed a uniform phenotype (CK18+, vimentin+, sACE+, alpha-inhibin+, M2A-) but in contrast to fetal germ cells lacked a clear equivalence to fetal tissues. Our results show that gonadoblastomas mimic female fetal ovary and exhibit a stepwise progression from follicular pattern to coronary pattern and finally to dysgerminomas.
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PMID:Gonadoblastoma: evidence for a stepwise progression to dysgerminoma in a dysgenetic ovary. 1596 43

Testicular gonadoblastoma is an uncommon tumor in domestic animals. The current study describes a testicular gonadoblastoma in 2 pet domestic rabbits (Oryctolagus cuniculus domesticus) based on the histomorphological, immunohistochemical, and ultrastructural characteristics of the tumor. The tumor tissue consisted of discrete tubular structures separated by a fibrous stroma. The tubular structures were composed of large round cells similar in appearance to germ cells in the seminiferous tubules, and small spindle cells with oval or elongated nuclei resembling Sertoli cells. The spindle cells showed peculiar structural patterns arranged in a coronal or follicular pattern, often forming Call-Exner bodies like those in an ovarian granulosa cell tumor. One case was concomitant with seminoma. Immunohistochemistry revealed large round cells that were positive for c-kit and placental alkaline phosphatase, while spindle cells were positive for vimentin and Wilms tumor protein. Ultrastructurally, the spherical eosinophilic material (Call-Exner body) consisted of duplicated basal lamina, and sex cord components surrounded a single degenerative cell similar to a germ cell.
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PMID:Testicular gonadoblastoma in two pet domestic rabbits (Oryctolagus cuniculus domesticus). 2190 69