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[A case of neurinoma combined with testicular feminization syndrome].
Nihon Kyobu Shikkan Gakkai Zasshi. 1993 Jan; 31(1):94-8.NK

Abstract

A 23-year-old female was admitted to our hospital because of a mediastinal tumor. She complained of primary amenorrhea, and abdominal echosonography was therefore performed, revealing the absence of a uterus. Chromosome analysis revealed a 46 XY genotype, indicating the patient to be a male, not a female. Left renal venography demonstrated residual blood vessels originating in the Mullerian ducts. Thoracotomy and laparotomy were performed, and the mediastinal tumor and bilateral testes were resected. Postoperative pathology revealed that the mediastinal tumor was a neurinoma. There was no spermatogenesis, but hyperplasia of Sertoli cells in the testes was seen. Neither the neurinoma nor the testes were malignant. Bioassay of the testes showed no androgen receptors, leading to the diagnosis of testicular feminization syndrome with the complete form of androgen insensitivity. There have been many reports of an association between testicular feminization syndrome and malignant tumors of the testes, but few reports of an association between testicular feminization syndrome and benign tumors such as neurinoma. Accordingly, the present case was considered to be unusual and worth reporting.

Authors+Show Affiliations

Respiratory Division, Kyoto-Katsura Hospital, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

jpn

PubMed ID

8468828

Citation

Chiba, W, et al. "[A Case of Neurinoma Combined With Testicular Feminization Syndrome]." Nihon Kyobu Shikkan Gakkai Zasshi, vol. 31, no. 1, 1993, pp. 94-8.
Chiba W, Matsubara Y, Sawai S, et al. [A case of neurinoma combined with testicular feminization syndrome]. Nihon Kyobu Shikkan Gakkai Zasshi. 1993;31(1):94-8.
Chiba, W., Matsubara, Y., Sawai, S., Konishi, T., Ishida, H., Hanawa, T., Kosaba, S., & Ikeda, S. (1993). [A case of neurinoma combined with testicular feminization syndrome]. Nihon Kyobu Shikkan Gakkai Zasshi, 31(1), 94-8.
Chiba W, et al. [A Case of Neurinoma Combined With Testicular Feminization Syndrome]. Nihon Kyobu Shikkan Gakkai Zasshi. 1993;31(1):94-8. PubMed PMID: 8468828.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [A case of neurinoma combined with testicular feminization syndrome]. AU - Chiba,W, AU - Matsubara,Y, AU - Sawai,S, AU - Konishi,T, AU - Ishida,H, AU - Hanawa,T, AU - Kosaba,S, AU - Ikeda,S, PY - 1993/1/1/pubmed PY - 1993/1/1/medline PY - 1993/1/1/entrez SP - 94 EP - 8 JF - Nihon Kyobu Shikkan Gakkai zasshi JO - Nihon Kyobu Shikkan Gakkai Zasshi VL - 31 IS - 1 N2 - A 23-year-old female was admitted to our hospital because of a mediastinal tumor. She complained of primary amenorrhea, and abdominal echosonography was therefore performed, revealing the absence of a uterus. Chromosome analysis revealed a 46 XY genotype, indicating the patient to be a male, not a female. Left renal venography demonstrated residual blood vessels originating in the Mullerian ducts. Thoracotomy and laparotomy were performed, and the mediastinal tumor and bilateral testes were resected. Postoperative pathology revealed that the mediastinal tumor was a neurinoma. There was no spermatogenesis, but hyperplasia of Sertoli cells in the testes was seen. Neither the neurinoma nor the testes were malignant. Bioassay of the testes showed no androgen receptors, leading to the diagnosis of testicular feminization syndrome with the complete form of androgen insensitivity. There have been many reports of an association between testicular feminization syndrome and malignant tumors of the testes, but few reports of an association between testicular feminization syndrome and benign tumors such as neurinoma. Accordingly, the present case was considered to be unusual and worth reporting. SN - 0301-1542 UR - https://www.unboundmedicine.com/medline/citation/8468828/[A_case_of_neurinoma_combined_with_testicular_feminization_syndrome]_ L2 - http://www.diseaseinfosearch.org/result/7031 DB - PRIME DP - Unbound Medicine ER -