Pediatric Adrenocortical Oncocytoma presenting as Cushing’s Syndrome and Peripheral Precocious Puberty: A Case Report and Review of Literature

Narayanan, Niya; Kamalanathan, Sadishkumar; Sahoo, Jayaprakash; Naik, Dukhabandhu; Kumaravel, S; Ganesh, Rajesh Nachiappa

doi:10.15605/jafes.036.02.17

Cited by 3 publications

(2 citation statements)

References 14 publications

(27 reference statements)

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“…Hormone secretion is usually mixed; androgen, cortisol, aldosterone, and rarely estrogen secretion may be excessive. [3][4][5] Both androgen and cortisol hypersecretion are associated with half of the functional tumors. Pure androgen-secreting adrenocortical tumors (PASATs) are rare.…”

Section: Discussionmentioning

confidence: 99%

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: 99%

Macropenis in a Toddler

Demiral

Çelik

Saraydaroğlu

et al. 2023

Clin Pediatr (Phila)

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“…Adrenal cortical oncocytoma is usually described as non-functional adrenal tumor and may lead to the development of Cushing's syndrome and complex clinical diagnosis. In recent years, with the progress of hospital pathological detection technology, the reporting of this rare disease is on the rise due to the decrease in missed detection and false detection rate (4,5).…”

Section: Introductionmentioning

confidence: 99%

Cushing's syndrome caused by adrenocortical oncocytoma: A case report

Gou,

Jian,

Tian

et al. 2024

Oncol Lett

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Adrenal cortical eosinophilic adenoma usually presents as non-functional adrenal tumor but may lead to Cushing's syndrome in patients. The present article reports a patient with Cushing's syndrome caused by right adrenocortical oncocytoma. The patient was treated in Urology Department of Wuchuan People's Hospital (Zunyi, China) in November 2022 because of hirsutism, weight gain and hypertension. A laparoscopic right adrenal tumor resection was performed using an abdominal approach. Following surgery, blood pressure and heart rate of the patient fluctuated within a healthy range and menstruation returned to normal. Laparoscopic adrenalectomy has obvious advantages over open adrenalectomy, such as less trauma, shorter recovery time and fewer complications. Thus, this treatment for this rare disease is safe and feasible.

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Oncocytic adrenocortical neoplasm with undetermined malignant potential and autonomous cortisol secretion

et al. 2022

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Increasing incidence of adrenal incidentalomas with hypercortisolemia not associated with overt features of Cushing’s syndrome has led to the evolution of the term autonomous cortisol secretion. Oncocytic adrenocortical neoplasms (OANs) are rare adrenal cortex neoplasms with only 250 reported cases worldwide. We present a woman in her 30s with menometrorrhagia, weight gain and increasing abdominal girth who was found to have a large right adrenal mass on abdominal CT scan. Serum cortisol was not suppressed after 1 mg dexamethasone (31.5 µg/dL) and adrenocorticotrophic hormone was not detectable (<1.0 pg/mL). She underwent right adrenalectomy under glucocorticoid coverage. Hypertension, weight, visceral adiposity and menometrorrhagia improved postoperatively. Histopathology with immunohistochemistry showed OAN with undetermined malignant potential. These kinds of tumours can only be definitively diagnosed postsurgically using the Lin-Weiss-Bisceglia system. Although they have better prognosis compared with adrenocortical carcinomas and do not require chemotherapy, patients should be closely monitored to identify recurrence promptly.

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Pediatric Adrenocortical Oncocytoma presenting as Cushing’s Syndrome and Peripheral Precocious Puberty: A Case Report and Review of Literature

Cited by 3 publications

References 14 publications

Macropenis in a Toddler

Macropenis in a Toddler

Cushing's syndrome caused by adrenocortical oncocytoma: A case report

Oncocytic adrenocortical neoplasm with undetermined malignant potential and autonomous cortisol secretion

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