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PubMed · 14911749

[Precocious macrogenitosomia with pseudohermaphroditism; multiple anatomical changes].

G GUILLAIN·I BERTRAND
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Adrenogenital SyndromeDiseaseDisorders of Sex DevelopmentEndocrine GlandsEndocrine System DiseasesGanglioneuromaHumansENDOCRINE GLANDS/diseasesGANGLIONEUROMAHERMAPHRODITISMJournal Article

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G GUILLAIN, I BERTRAND. 1952-02-20. [Precocious macrogenitosomia with pseudohermaphroditism; multiple anatomical changes].. https://pubmed.ncbi.nlm.nih.gov/14911749/

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PubMed2007

Clinical and pathological features associated with the testicular tumor of the adrenogenital syndrome.

PURPOSE: Testicular tumor of the adrenogenital syndrome is a rare clinical entity found in young men with endocrine disorders. Histologically it resembles Leydig cell tumor. We 1) reviewed the clinical features of testicular tumor of the adrenogenital syndrome and 2) determined if special histopathological features of the tumor and synaptophysin reactivity could distinguish testicular tumor of the adrenogenital syndrome from Leydig cell tumor. MATERIALS AND METHODS: We reviewed the medical and pathological records for all patients with testicular tumor of the adrenogenital syndrome seen at our institution from 1978 to 2004. These tumors were examined by histological and immunophenotypic methods for comparison to Leydig cell tumor. RESULTS: A total of 14 males with an endocrine disorder had pathological evidence of testicular tumor of the adrenogenital syndrome. These tumors were often bilateral (93% or 13 of 14 cases), associated with pain (92% or 12 of 13) and refractory to medical management with high dose exogenous steroids (93% or 13 of 14). Testicular tumor of the adrenogenital syndrome was managed by tumor enucleation in 7 patients (54%) and by radical orchiectomy in 6 (46%). All patients had resolution of pain at 3-month followup. Upon histological review features found to be more common to testicular tumor of the adrenogenital syndrome compared with Leydig cell tumor were nuclear pleiomorphism, low mitotic activity, extensive fibrosis, lymphoid aggregates, adipose metaplasia and prominent lipochrome pigment. Synaptophysin (ICN, Costa Mesa, California) reactivity was strong in testicular tumor of the adrenogenital syndrome but rarely observed in Leydig cell tumor. CONCLUSIONS: In our series medical treatment failed in patients with testicular tumor of the adrenogenital syndrome and conservative surgical therapy was possible in select individuals. We identified special histopathological and immunophenotypic features, including synaptophysin staining, which distinguish testicular tumor of the adrenogenital syndrome from Leydig cell tumor.

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PubMed2006

[Forgotten evidence of adrenogenital syndrome].

At present, the adrenogenital syndrome must surely be reckoned among the most widely known conditions in endocrine pathology. It may come as a surprise that no unambiguous cases of adrenogenitalism are on record before the end of the 19th century. While granting that impressive clinical descriptions may be found in isolated instances, postmortem findings are either totally missing or were not recorded in sufficient detail, thus greatly diminishing their value for retrospective study. This, however, does not apply to a case published by Luigi de Crecchio, a Naples anatomist, in 1865, of a female pseudo-hermaphrodite. De Crecchio provides, for the first time ever, a clear description of a rare pathology, underpinned by an exemplary postmortem examination, detailed clinical findings, and biographical data. Based on a complete translation of De Crecchio's article, our study comments on this almost forgotten case history, placing it, at the same time, in the context of the history of endocrine research.

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