Abstract: Pediatric adrenocortical carcinoma (ACC) is rare malig-
nancy, and its oncocytic variant is exceptionally rare in infants. A 1-
year-old girl presented with excessive hair growth and hoarseness of
voice for 8 months of age. Investigations revealed elevated androgen
levels, and whole-body 18F FDG PET-CT imaging showed an
isolated large left adrenal mass. The mass was surgically excised,
and histopathology confirmed oncocytic adrenocortical carcinoma
with a Wieneke score of 5, indicating malignancy. The post-
operative course was uneventful with stabilization on anti-
hypertensive and corticosteroid therapy.
Received for publication June 30, 2025; accepted October 2, 2025.
From the *Department of Nuclear Medicine; }Department of Histo-
pathology, Mahatma Gandhi Medical College and Hospital;
¿Department of Pediatrics; and §Department of Pediatric Oncology,
Mahatma Gandhi Medical College and Hospital, Jaipur, Rajasthan,
India.
Written informed consent was taken from the patient.
Data will be available with corresponding author.
Conflicts of interest and sources of funding: none declared.
Correspondence to: Tarun Kumar Jain, MD, Department of Nuclear
Medicine and PET, Mahatma Gandhi Medical College and Hos-
pital, Jaipur, Rajasthan 302022, India. E-mail: tarun4891@gmail.
com.
Copyright @ 2025 Wolters Kluwer Health, Inc. All rights reserved.
DOI: 10.1097/RLU.0000000000006208
ACKNOWLEDGMENTS
The authors thank all technologists of the PET-CT
department for acquiring the data.
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FIGURE 1. A 1-year-old girl presented to our center with progressive excessive hair growth over the entire body and hoarseness of voice for 8 months of age. There was no family history of malignancy. The mother had hypothyroidism and managed with levothyroxine. On clinical examination, the pulse rate in both upper limbs was symmetrical and 120/min. She was hypertensive (136/84 mm Hg) and relatively equal blood pressure in both upper limbs. Systemic examinations were unremarkable. Laboratory and hematological investigations revealed markedly elevated serum DHEAS 7295 (0.47-19.4 mcg/dL) and testosterone 645.9 (0-10 ng/dL), with normal plasma metanephrines and normetanephrines. Serum cortisol levels were 33.5 mcg/dL (2.8-23mcg/dL), mildly elevated. Ultrasonography abdomen reveal large left suprarenal mass. In view of suspicious malignancy, 18F FDG PET-CT was performed. 18F FDG PET-CT maximum intensity projection image reveals focal hot spot in left mid abdomen region (A). Transaxial and coronal CECT and PET- CT images (B-E) reveal FDG avid (SUVmax: 15.6) heterogeneously enhancing lobulated well-defined soft tissue density mass (~6.9×5.8×5.3 cm) arising from the left suprarenal adrenal gland. There is no definitive scan evidence of the distant metastasis disease. The patient was managed initially with labetalol infusion, later escalated to nitroglycerin and oral antihypertensives. Surgical excision of the mass was performed under general anesthesia. Intraoperative findings confirmed a well-encapsulated adrenal mass, which was completely excised.
A
B
C
D
E
FIGURE 2. Histopathologic analysis revealed an encapsulated tumor composed of cells with eosinophilic cytoplasm, nuclear pleomorphism, atypical mitoses, and capsular invasion (A, B). The Wieneke score was 5, consistent with malignancy and poor prognosis. Immunohistochemistry was positive for CK, inhibin (C), SF-1 (D), synaptophysin (E, F), vimentin and negative for chromogranin and S-100. Final diagnosis was high-grade oncolytic adrenal cortical carcinoma. The postoperative period was uneventful. The patient was extubated after 12 hours, initiated on hydrocortisone supplementation, and maintained blood pressure with oral antihypertensives. She was discharged in stable condition with close outpatient follow-up was scheduled. Adrenocortical tumors (ACTs) are rare in infant populations, accounting for <0.2% of childhood cancers.1 Pediatric ACTs often present with signs of hormonal hyperfunction, most commonly virilization.2 ACC is characterized histologically by oncocytic cells and typically shows aggressive behavior.3 Distinction from benign lesions is crucial and relies on criteria such as the Wieneke scoring system, which evaluates features like tumor weight, size, mitotic rate, necrosis, vascular, and capsular invasion.4,5 Complete surgical excision remains the treatment of choice.6 Prognosis largely depends on tumor stage, histologic grade, and surgical margins. Adjuvant therapies are considered in cases of incomplete resection or metastasis.7 These ACC are often associated with syndromes so whole-body imaging is necessary to rule out other component of the syndrome and metastatic disease so we performed the 18F FDG PET-CT in present case.8,9 Our case underscores the importance of a multidisciplinary approach involving pediatric surgeons, oncologists, endocrinologists, nuclear medicine physicians, and intensivists for optimal management and follow-up.