Isosexual precocious pseudopuberty secondary to a feminizing adrenal tumor

F. Comite, R. J. Schiebinger, B. D. Albertson, F. G. Cassorla, K. Vander Ven, T. F. Cullen, Donald (Lynn) Loriaux, G. B. Cutler

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

We report a 2 10/12-yr-old girl with precocious pseudopuberty due to a feminizing adrenal carcinoma without Cushing's syndrome. The patient had marked elevation of plasma concentrations of the Δ5 adrenal steroids dehydroepiandosterone and dehydroepiandrosterone sulfate and increased levels of androstenedione, estrone, estradiol, and testosterone. Adrenal microsomal 3β-hydroxysteroid dehydrogenase-isomerase 17-hydroxylase, 17,20-desmolase and 21-hydroxylase activities in the tumor and adjacent normal adrenal gland were measured. The tumor had approximately normal levels of 17-hydroxylase and 17,20-desmolase activity, with low levels of 21-hydroxylase and 3β-hydroxysteroid dehydrogenase-isomerase activities. This combination of enzyme activity may explain the absence of Cushing's syndrome and the high levels of Δ5 adrenal steroids. This patient demonstrates that adrenal neoplasms arising in girls may mimic isosexual true precocious puberty and should be included in the differential diagnosis of precocious puberty.

Original languageEnglish (US)
Pages (from-to)435-440
Number of pages6
JournalJournal of Clinical Endocrinology and Metabolism
Volume58
Issue number3
StatePublished - 1984
Externally publishedYes

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3-Hydroxysteroid Dehydrogenases
Steroid 17-alpha-Hydroxylase
Steroid 21-Hydroxylase
Precocious Puberty
Isomerases
Glandular and Epithelial Neoplasms
Cushing Syndrome
Mixed Function Oxygenases
Tumors
Adrenal Gland Neoplasms
Steroids
Dehydroepiandrosterone Sulfate
Androstenedione
Estrone
Enzyme activity
Adrenal Glands
Testosterone
Estradiol
Neoplasms
Differential Diagnosis

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology, Diabetes and Metabolism

Cite this

Comite, F., Schiebinger, R. J., Albertson, B. D., Cassorla, F. G., Vander Ven, K., Cullen, T. F., ... Cutler, G. B. (1984). Isosexual precocious pseudopuberty secondary to a feminizing adrenal tumor. Journal of Clinical Endocrinology and Metabolism, 58(3), 435-440.

Isosexual precocious pseudopuberty secondary to a feminizing adrenal tumor. / Comite, F.; Schiebinger, R. J.; Albertson, B. D.; Cassorla, F. G.; Vander Ven, K.; Cullen, T. F.; Loriaux, Donald (Lynn); Cutler, G. B.

In: Journal of Clinical Endocrinology and Metabolism, Vol. 58, No. 3, 1984, p. 435-440.

Research output: Contribution to journalArticle

Comite, F, Schiebinger, RJ, Albertson, BD, Cassorla, FG, Vander Ven, K, Cullen, TF, Loriaux, DL & Cutler, GB 1984, 'Isosexual precocious pseudopuberty secondary to a feminizing adrenal tumor', Journal of Clinical Endocrinology and Metabolism, vol. 58, no. 3, pp. 435-440.
Comite F, Schiebinger RJ, Albertson BD, Cassorla FG, Vander Ven K, Cullen TF et al. Isosexual precocious pseudopuberty secondary to a feminizing adrenal tumor. Journal of Clinical Endocrinology and Metabolism. 1984;58(3):435-440.
Comite, F. ; Schiebinger, R. J. ; Albertson, B. D. ; Cassorla, F. G. ; Vander Ven, K. ; Cullen, T. F. ; Loriaux, Donald (Lynn) ; Cutler, G. B. / Isosexual precocious pseudopuberty secondary to a feminizing adrenal tumor. In: Journal of Clinical Endocrinology and Metabolism. 1984 ; Vol. 58, No. 3. pp. 435-440.
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