Testosterone-Secreting Adrenocortical Tumor in a Pubertal Girl

Sorgo, W.; Meyer, Dirk; Rodens, Klaus; Homoki, J.; Heinze, E.; B, Heymer; Siebenmann, R

doi:10.1159/000181067

Cited by 8 publications

(3 citation statements)

References 21 publications

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“…Al though nonsecreting tumors have been reported, the ma jority of children manifest clinical symptoms of hormone excess such as hypercortisolemia, virilization, feminiza tion, hyperaldosteronism or combinations of these fea tures [1][2][3][4][5][6][7][8]. Both types of adrenocortical tumors (carcino mas and adenomas) produce a wide variety of steroid hor mones.…”

Section: Introductionmentioning

confidence: 99%

Heterogeneity of Urinary Steroid Profiles in Children with Adrenocortical Tumors

Małunowicz¹,

Ginalska-Malinowska²,

Romer

et al. 1995

Horm Res

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The excretory patterns of urinary steroids determined by capillary gas chromatography in 11 children (aged 0.8-16.5 years) with adrenocortical tumors were established. In 8 patients the predominant clinical feature was virilization, in 3 others, Cushing’s syndrome. In 5 patients (3 carcinoma, 2 adenoma) very high excretion of 3β-hydroxy-5-ene steroids was observed. In 2 others (adenomas) only moderately elevated excretion of 11β-hydroxyandrosterone was found. In 1 patient (adenoma) pregnanediol dominated in the steroid profile, accompanied by moderately elevated 3β-hydroxy-5-ene steroids. Out of 3 Cushingoid patients (1 carcinoma, 2 adenomas), 1 presented an atypical urinary steroid pattern for hypercortisolemia, without 5α-reductase and 11β-hydroxysteroid dehydrogenase deficiencies. Neither the urinary steroid pattern nor tumor size alone were reliable indicators of tumor malignancy, as evaluated by a pathological examination and subsequent metastasis-free survival.

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Section: Introductionmentioning

confidence: 99%

Heterogeneity of Urinary Steroid Profiles in Children with Adrenocortical Tumors

Małunowicz¹,

Ginalska-Malinowska²,

Romer

et al. 1995

Horm Res

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“…The malignant adrenal tumor size was significantly larger than that of benign tumors, consistent with various studies on adrenal tumors. Testosterone is the most sensitive marker in the hormone evaluation of PASAT, as it is elevated in almost all PASAT patients and is the only increased androgen in certain patients ( 41 , 62 , 63 ). Some studies ( 64 , 65 ) have demonstrated that increased DS (produced uniquely by the adrenal zona reticularis) and DHEA (produced mainly by the adrenal zona reticularis) are indicators of adrenal etiology, especially DS.…”

Section: Discussionmentioning

confidence: 99%

Pure androgen-secreting adrenal tumor (PASAT): A rare case report of bilateral PASATs and a systematic review

et al. 2023

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BackgroundAdult pure androgen–secreting adrenal tumors (PASATs) are extremely rare, and their characteristics are largely unknown.MethodsA rare case of adult bilateral PASATs was reported, and a systematic literature review of adult PASATs was conducted to summarize the characteristics of PASATs.ResultsIn total, 48 studies, including 40 case reports and 8 articles, were identified in this review. Analysis based on data of 42 patients (including current case and 41 patients from 40 case reports) showed that average age was 40.48 ± 15.80 years (range of 18-76). The incidence of adult PASAT peaked at 21-30 years old, while that of malignant PASAT peaked at 41-50 years old. Most PASAT patients were female (40/42, 95.23%), and hirsutism was the most common symptom (37/39, 94.87%). Testosterone (T) was the most commonly elevated androgen (36/42, 85.71%), and 26 of 32 tested patients presented increased dehydroepiandrosterone sulfate (DS) levels. In malignancy cases, disease duration was significantly decreased (1.96 vs. 4.51 years, P=0.025), and tumor diameter was significantly increased (8.9 vs. 4.9 cm, p=0.011). Moreover, the androgen levels, namely, T/upper normal range limit (UNRL) (11.94 vs. 4.943, P=0.770) and DS/UNRL (16.5 vs. 5.28, P=0.625), were higher in patients with malignancy. In total, 5 out of 7 patients showed an increase in DS or T in the human chorionic gonadotropin (HCG) stimulation test. Overall, 41 out of 42 patients (including current case) underwent adrenal surgery, and recurrence, metastasis, or death was reported in 5 out of 11 malignant patients even with adjuvant or rescue mitotane chemotherapy.ConclusionAdult PASAT, which is predominant in women, is characterized by virilism and menstrual dysfunction, especially hirsutism. Elevated T and DS may contribute to the diagnosis of adult PASAT, and HCG stimulation test might also be of help in diagnosis. Patients with malignant PASAT have a shorter disease duration, larger tumor sizes and relatively higher androgen levels. Surgery is recommended for all local PASATs, and Malignancy of PASAT should be fully considered due to the high risk of malignancy, poor prognosis and limited effective approaches.

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“…In a minority of patients elevated excretion of 16c~-hydroxy DHA [6] and ll~3-hydroxy DHA [27] has been found. Testosterone producing adrenal tumours occur only rarely [20].…”

Section: Introductionmentioning

confidence: 99%

Virilizing adrenal tumour mimicking congenital adrenal hyperplasia with P450c11 (11β-hydroxylase) deficiency

Werder

Voutilainen

Zachmann³

1994

Eur J Pediatr

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In a girl aged 5 years with a virilizing adrenal adenoma the urinary and plasma steroid findings suggested the diagnosis of congenital adrenal hyperplasia due to P450c11 (11 beta-hydroxylase) deficiency. After removal of the tumour clinical signs receded and the hormonal values normalized. RNA analysis of the tumour tissue revealed low amounts of P450c11 mRNA which indicates that P450c11 deficiency of the adenoma caused the steroid abnormalities in this girl.

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Testosterone-Secreting Adrenocortical Tumor in a Pubertal Girl

Cited by 8 publications

References 21 publications

Heterogeneity of Urinary Steroid Profiles in Children with Adrenocortical Tumors

Heterogeneity of Urinary Steroid Profiles in Children with Adrenocortical Tumors

Pure androgen-secreting adrenal tumor (PASAT): A rare case report of bilateral PASATs and a systematic review

Virilizing adrenal tumour mimicking congenital adrenal hyperplasia with P450c11 (11β-hydroxylase) deficiency

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