Hermaphroditismus verus: Klinik, Diagnose und Therapie im Erwachsenenalter

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This case report describes a 20-year-old woman with true hermaphroditism who presented with an endometriosis cyst, highlighting the diagnostic and therapeutic management of ovarian tumors in intersex patients.

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This case report describes a 20-year-old female with true hermaphroditism who presented for evaluation of suspected gonadal abnormalities following prior intersex surgery in infancy. Clinical examination revealed an eight-centimeter left ovarian mass, which was identified intraoperatively as an endometriosis cyst and excised while preserving ovarian tissue, alongside a right ovotestis from which the testicular portion was removed due to malignancy risk. The authors detail the various combinations of ovarian and testicular tissues found in such cases and emphasize that early diagnosis and therapy are crucial for maintaining gonadal function and supporting psychosexual development. Relevance to endometriosis: The paper documents a coincidental finding of an endometriosis cyst within the context of managing true hermaphroditism, but it is not centrally about endometriosis or adenomyosis.

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Abstract

A 20-year old woman, who underwent an intersex operation during infancy, was presented for evaluation of the gonads because of suspected true hermaphroditism. The phenotype was female with regular menstruations. Examination revealed an ovarian tumour of 8 cm on the left side and a normal right ovary. During the operation, the tumour was revealed as an endometriosis cyst, which could be excised whilst preserving the ovarian tissue. The right gonad was an ovotestis. The testicular part was removed because of a 4% risk of malignancy. The different combinations of ovary/testis and ovotestis are described. Diagnosis and therapy of true hermaphroditism should be performed as early as possible to obtain an adequate gonadal function and an undisturbed psychosexual development.
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Subscribe to RSS DOI: 10.1055/s-2007-1022897 © Georg Thieme Verlag Stuttgart · New York Hermaphroditismus verus: Klinik, Diagnose und Therapie im Erwachsenenalter True Hermaphroditism: Clinical Picture, Diagnosis and Therapy in AdulthoodPublication History Publication Date: 16 June 2008 (online) Zusammenfassung Eine 20jährige Frau, die im Kindesalter wegen eines intersexuellen Genitale operiert worden war, wurde bei V. a. Hermaphroditismus verus zur Abklärung der Adnexe vorgestellt. Die Entwicklung war weiblich, Menstruationen regelmäßig. Bei der gynäkologischen Untersuchung fand sich ein 8 cm großer linksseitiger Ovarialtumor bei unauffälligem rechtsseitigem Adnex. Intraoperativ stellte sich der Tumor als Endometriosezyste dar, die unter Erhalt des Restovars ausgeschält wurde. Auf der rechten Seite bestand ein Ovotestis, dessen Testisanteil bei einem Entartungsrisiko von 4% entfernt wurde. Die verschiedenen Kombinationen von Ovar/Hoden und Ovotestis werden beschrieben. Diagnose und Therapie des Hermaphroditismus versus sollten so früh wie möglich erfolgen, um eine adäquate Gonadenfunktion zu erhalten und eine ungestörte psychosexuelle Entwicklung zu ermöglichen. Abstract A 20-year old woman, who underwent an intersex operation during infancy, was presented for evaluation of the gonads because of suspected true hermaphroditism. The phenotype was female with regulär menstruations. Examination revealed an ovarian tumour of 8 cm on the left side and a normal right ovary. During the operation, the tumour was revealed as an endometriosis cyst, which could be excised whilst preserving the ovarian tissue. The right gonad was an ovotestis. The testicular part was removed because of a 4% risk of malignancy. The different combinations of ovary/testis and ovotestis are described. Diagnosis and therapy of true hermaphroditism should be performed as early as possible to obtain an adequate gonadal function and an undisturbed psychosexual development.

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endometriosis

MeSH descriptors

Disorders of Sex Development Adult Choristoma Choristoma Choristoma Choristoma Disorders of Sex Development Disorders of Sex Development Disorders of Sex Development Endometrial Neoplasms Endometrial Neoplasms Endometrial Neoplasms Endometrial Neoplasms Endometriosis Endometriosis Endometriosis Endometriosis Female Gonadal Steroid Hormones Gonadal Steroid Hormones

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