Adrenocortical adenoma manifesting as Cushing’s syndrome 
and pseudo-precocious puberty in a toddler

Boro, Hiya; Kubihal, Suraj; Dutta, Rimlee; Kubihal, Vijay; Alam, Sarah; Tandon, Nikhil

doi:10.5114/pedm.2021.109122

Cited by 1 publication

(2 citation statements)

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“…Congenital adrenal hyperplasia may be misdiagnosed in patients with high levels of 17-OHP. 11,12 In our patient, testosterone was very high, FSH and LH were suppressed, and androstenedione and DHEA-S were high, while 17-OHP was normal.…”

Section: Discussionmentioning

confidence: 53%

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Macropenis in a Toddler

Demiral

Çelik

Saraydaroğlu

et al. 2023

Clin Pediatr (Phila)

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A 21-month-old male was admitted to the endocrine clinic with the complaint of penis enlargement. He was born to nonconsanguineous parents after 38 weeks of uneventful gestation. His birth weight was 3600 g (0.4 standard deviation score [SDS]/67 th percentile). The remainder of his medical history was unremarkable. He had 1 healthy brother. On physical examination, his body weight was 16.5 kg (2.4 SDS/99.2 th percentile), height 97 cm (2.9 SDS/99.8 th percentile), and body mass index 17.5 (0.58 SDS/71 th percentile). His blood pressure was normal. The penile length was 7 cm, pubic hair was at Tanner stage 2, and testicular volume was 1 mL bilaterally. He had android fat distribution, increased muscle mass, facial acne, and deepening of the voice (Figure 1).Laboratory examination revealed markedly increased testosterone levels (>35 nmol/L) with suppressed follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels (0.08 and 0.06 mIU/mL, respectively). Dehydroepiandrostenedione sulfate (DHEA-S) and androstenedione were found to be increased (105 µg/dL and 1.72 µg/L, respectively). Blood count and other biochemistry were normal, except for serum alkaline phosphatase (557 U/L). Thyroid function tests were normal. His morning (7.30 am) serum cortisol level was 7.4 μg/ dL, and midnight (11 pm) serum cortisol level was 1.1 μg/dL. The circadian rhythm of cortisol was normal. His plasma adrenocorticotropic hormone (ACTH) was not suppressed (35 pg/mL). There was no hypercortisolism clinical symptoms. The bone age was 3.5 years according to the Greulich and Pyle method. Laboratory findings are shown in Table 1. Abdominal and scrotal ultrasound (US) were normal. Abdominal magnetic resonance image (MRI) showed a nodular lesion in the left adrenal gland (Figure 2). Final DiagnosisThe initial differential diagnoses list included precocious puberty, congenital adrenal hyperplasia, familial testotoxicosis, adrenocortical tumor (ACT), and testicular tumor. A final diagnosis of ACT and peripheral precocious puberty was made.

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Section: Discussionmentioning

confidence: 53%

“…Some scoring systems based on histopathologic examination of the mass are used to diagnose adenoma or carcinoma. 1,11 First, the Weiss criteria were reported in 1984. This scoring system was modified because 3 patients who were accepted as having adenoma according to the Weiss criteria developed relapse during follow-up.…”

Section: Discussionmentioning

confidence: 99%