Virilizing Adrenal Tumor in a 3-Year-Old Girl: Diagnosis by Computed Tomographic Scan and Ultrasound

Kaplowitz, Paul B.; Mandell, James

doi:10.1001/archpedi.1983.02140300084027

Cited by 7 publications

(13 citation statements)

References 10 publications

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“…of the virilizing adrenal tumors in three pediatric series were carcinomas [7], The present report is the first on a pubertal girl pre senting with deepening of the voice as the only clinical sign of androgenization. A small testosterone-secreting adrenal tumor with borderline increased excretion of urinary 17-ketosteroids (KS) was localized by computed axial tomographic (CAT) scanning and removed by sur gery.…”

Section: Introductionmentioning

confidence: 55%

“…As far as we know, 15 females with exclusively testosterone-secreting adrenal tumors and normal 17-K.S excretion had been recently reviewed from the literature [18,19], The youngest patient was 1 year and 8 months, 2 others below 20, and the oldest 76 years of age. If one considers cases in which elevated serum testosterone levels were associated with a slightly increased urinary 17-KS fraction (up to 70 pmol/d), another 5 patients (including the current case) may be added [1,7,17,20], In the synopsis of table 2, the patients are grouped according to their endocrine find ings. There seems to be no relation between the endo crine findings and the histopathology in these patients, although it is interesting that the 2 cases with adrenocor tical carcinoma belonged to group II and were below 20 years of age.…”

Section: Discussionmentioning

confidence: 99%

“…From this and the clinical fact that the girl had normal monthly menses, it can be confirmed that increased testosterone levels do not necessarily in fluence the secretion of gonadotropins [27], Furthermore, one wonders how testosterone as the most potent endogenous androgen should be responsible for the deepening of the voice and probably also for the bone age acceleration, without having effected other con comitant signs of androgenization. In contrast, 5 other cases below 20 years of age (table 2) presented with vir ilization and acne at first examination [1,7,18,32,33]. Additional findings were a deep voice in 4 [ 1,18,32,33], amenorrhea in 2 pubertal [18,33] and growth accelera tion in 2 prepubertal patients [7,32], Therefore, it must be suggested that the sensitivity and response of andro gen-dependent tissues to testosterone differ.…”

Section: Discussionmentioning

confidence: 99%

“…In contrast, 5 other cases below 20 years of age (table 2) presented with vir ilization and acne at first examination [1,7,18,32,33]. Additional findings were a deep voice in 4 [ 1,18,32,33], amenorrhea in 2 pubertal [18,33] and growth accelera tion in 2 prepubertal patients [7,32], Therefore, it must be suggested that the sensitivity and response of andro gen-dependent tissues to testosterone differ. It may also be possible that the unusual onset of androgenization is caused by a temporal dissociation of the testosterone effect.…”

Section: Discussionmentioning

confidence: 99%

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Testosterone-Secreting Adrenocortical Tumor in a Pubertal Girl

et al. 1988

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A girl aged 12 years and 10 months presented with deepening of the voice first noted 7 months earlier. Pubertal development was almost completed. The girl had regular monthly menses and no signs of hirsutism, clitoris enlargement or Cushing’s disease. Serum testosterone was about threefold above normal, whereas dehydroepiandrosterone was in the upper normal range. The 17-ketosteroids as well as the gas-chromatographically analyzed 5α and 5β derivatives of testosterone from urine were slightly increased. Other serum and urinary steroids were normal. Dynamic tests of the endocrine function exhibited inconclusive results. Ultrasonography revealed no ovarian cysts. A small, left-sided adrenal mass was identified by computed axial tomographic scan and removed by surgery. There were no signs of local metastasis nor of vascular extension. The histopathological diagnosis was adrenocortical carcinoma. 5 months after surgery, the preoperatively elevated steroid levels had returned to normal.

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Section: Introductionmentioning

confidence: 55%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Testosterone-Secreting Adrenocortical Tumor in a Pubertal Girl

et al. 1988

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“…Recently, abdominal ultrasonography and CT scan have proved reliable in detecting adrenal tumors as small as 1 cm in diameter. [7][8][9] They replace the less accurate intravenous urography and the more invasive adrenal arteriography. Their value was evident in our patient, in diagnosing both the original tumor and the recurrent malignancy with metastases.…”

Section: Discussionmentioning

confidence: 99%

Virilizing Adrenocortical Carcinoma in a 4-Year-Old Girl with Fatal Recurrence

et al. 1989

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Adrenocortical tumors are rare in children. 1 The great majority are hormone secreting, and they produce androgens, glucocorticoids, a mixture of both, or rarely, estrogen or aldosterone. 2 In this report, we describe a young girl who had virilization due to adrenocortical carcinoma that recurred after surgical removal. Case ReportA 4-year-old girl was referred with a 6-month history of increasing pubic and facial hair and clitoral enlargement. She was the product of an uncomplicated pregnancy, labor, and delivery. The mother did not take any hormonal therapy during pregnancy. The patient had a healthy 10-year-old brother; her parents were not related. There was no family history of abnormal sexual development or deaths in early infancy suggestive of adrenal crisis.At presentation, her weight was 112 cm and weight was 20 kg, both more than 2SD for her age. Blood pressure was 98/65 mm Hg. There was fine hair over the upper lip and dark coarse pubic hair. The clitoris measured 2.5 × 1 cm. Vaginal and urethral orifices were separate, with no labial fusion. There was no breast development, axillary hair, or abdominal masses. No signs of Cushing's syndrome were noted. There were no congenital anomalies.Investigations showed normal results for complete hemogram, serum electrolytes, urea, creatinine, morning and evening Cortisol and adrenocorticotropic hormone (ACTH), 17-hydroxyprogestrone, 11-desoxycorticosterone, 17-hydroxypregnenolone, aldosterone, and urinary 17-hydroxycorticosteroids. The karyotype was 46,××.Abnormal laboratory results included serum testosterone of 12.3 nmol/L (reference interval, 0.7 to 3.5 nmol/L); dehydroepiandrosterone sulfate (DHAS), 25.7 μmol/L (reference interval, 0.3 to 1.6 μmol/L); and total urinary 17-keto-steroids, 110 μmol/d (reference value, < 6.93 μmol/d). The radiologic studies showed a bone age of 9 years; the plain film of the abdomen showed calcification in the region of the left suprarenal gland; the skull and chest x-ray appearances were normal. Ultrasonography of the adrenals showed a left suprarenal mass about 5 × 6 cm. It had a hyperechoic center suggestive of calcification (Figure 1), and the ovaries were normal. The intravenous urogram demonstrated a left suprarenal mass with calcification, displacing the left kidney downward. Computerized tomography (CT) of the abdomen showed a left suprarenal mass with normal liver and spleen (Figure 2).Left adrenalectomy was performed, and a tumor 7 cm in diameter was removed. Local extension of the tumor to the diaphragm and perirenal fat were noted. On microscopic examination, the tumor cells demonstrated considerable pleomorphism, relatively high mitotic index (5 to 20 mitoses per 10 high-power fields), with areas of hemorrhage and necrosis. The pathologic diagnosis was adrenocortical carcinoma. Postoperative DHAS and testosterone levels initially fell to normal (Table 1), and pubic and facial hair ceased to grow. She was observed in the outpatient clinic at regular intervals to assess her clinical, hormonal, and radiologic conditio...

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Current uroradiologic literature

Pollack¹

1982

Urol Radiol

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Virilizing Adrenal Tumor in a 3-Year-Old Girl: Diagnosis by Computed Tomographic Scan and Ultrasound

Cited by 7 publications

References 10 publications

Testosterone-Secreting Adrenocortical Tumor in a Pubertal Girl

Testosterone-Secreting Adrenocortical Tumor in a Pubertal Girl

Virilizing Adrenocortical Carcinoma in a 4-Year-Old Girl with Fatal Recurrence

Current uroradiologic literature

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