Oncocytic Adrenocortical Carcinoma in an Infant: Clinical Findings and Imaging Features

Patel Meet1, Maneesh Kumar Vijay2, Tarun Kumar Jain1

  • 1Department of Nuclear Medicine.

Clinical Nuclear Medicine
|November 25, 2025
PubMed

Insights

This study reports an exceptionally rare case of oncocytic adrenocortical carcinoma in an infant. Early diagnosis and surgical removal were key to successful treatment of this pediatric malignancy.

Area of Science:

  • Pediatric Oncology
  • Endocrinology
  • Pathology

Background:

  • Pediatric adrenocortical carcinoma (ACC) is a rare malignancy.
  • The oncocytic variant of ACC is exceptionally rare, particularly in infants.
  • ACC can present with hormonal overactivity, such as elevated androgens.

Purpose of the Study:

  • To describe a rare case of oncocytic adrenocortical carcinoma in an infant.
  • To highlight diagnostic and management strategies for this rare pediatric tumor.

Main Methods:

  • Case report of a 1-year-old girl with symptoms of excessive hair growth and hoarseness.
  • Hormonal evaluation revealing elevated androgens.
  • Diagnostic imaging using whole-body 18F FDG PET-CT.
  • Surgical excision of the adrenal mass.
  • Histopathological confirmation and grading (Wieneke score).

Main Results:

  • The patient presented with clinical signs of androgen excess.
  • Imaging identified a large left adrenal mass.
  • Histopathology confirmed oncocytic adrenocortical carcinoma with a high malignancy score (Wieneke score 5).
  • The patient had an uneventful postoperative recovery.

Conclusions:

  • Oncocytic adrenocortical carcinoma is an extremely rare pediatric malignancy.
  • Multimodality diagnostic approaches are crucial for timely diagnosis.
  • Surgical resection is the primary treatment modality.
  • Postoperative management includes hormonal replacement and monitoring.

Related Concept Videos

Anatomy of the Adrenal Glands01:17

Anatomy of the Adrenal Glands

The adrenal or supra-renal glands, situated above the kidneys and aligned with the twelfth rib, are paired pyramid-shaped structures crucial for the body's stress response. During stress, these glands secrete hormones vital for adaptive physiological reactions.
These glands possess a distinctive yellow tinge due to the stored cholesterol and fatty acids required for hormone synthesis. They are encased in a fibrous capsule and cushioned by fat.
The adrenal gland comprises two distinct...
4.2K
Aneurysm II: Clinical Manifestations and Diagnostic Studies01:21

Aneurysm II: Clinical Manifestations and Diagnostic Studies

Thoracic, aortic arch and abdominal aneurysms are significant vascular conditions that can present with various clinical manifestations and lead to serious complications. Understanding these manifestations and the appropriate diagnostic studies is essential for effective management and treatment.Thoracic Aortic AneurysmsThoracic aortic aneurysms often remain asymptomatic until they reach a size that impinges on adjacent structures. They typically cause deep, diffuse chest pain that radiates to...
251
Imaging Studies I: CT and MRI01:14

Imaging Studies I: CT and MRI

Introduction: MRI and CT scans are crucial advancements in medical imaging techniques, playing a vital role in diagnosing conditions related to the gastrointestinal (GI) system. Each scan serves distinct purposes, targets specific areas, and requires unique nursing duties.
Description of the Procedures
Computed Tomography (CT) scan:
Computed Tomography (CT) scans use X-ray technology to generate detailed images of bones, organs, and tissues. During the scan, the patient lies on a moving table...
764
Imaging Studies I: Kidney, Ureter, and Bladder Studies01:28

Imaging Studies I: Kidney, Ureter, and Bladder Studies

Kidney, Ureter, and Bladder (KUB) StudiesKidney, Ureter, and Bladder (KUB) studies are standard diagnostic imaging procedures used to assess the anatomy of the urinary system. They are commonly utilized for patients experiencing abdominal pain or urinary symptoms. By using a simple X-ray of the abdomen, KUB studies can reveal structural and pathological abnormalities within the kidneys, ureters, and bladder. These studies are particularly valuable in diagnosing kidney stones, urinary...
246
The Retinoblastoma Gene01:20

The Retinoblastoma Gene

Tumor suppressor genes are normal genes that can slow down cell division, repair DNA mistakes, or program the cells for apoptosis in case of irreparable damage. Hence, they play an essential role in preventing the proliferation of damaged cells.
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
4.6K
Radiological Investigation III: Pulmonary Angiogram and PET Scan01:13

Radiological Investigation III: Pulmonary Angiogram and PET Scan

Radiological investigations are paramount in the diagnosis and management of various pulmonary diseases. Two essential investigations are the Pulmonary Angiogram and the Positron Emission Tomography (PET) Scan.
Pulmonary Angiogram
A Pulmonary Angiogram is an invasive procedure involving injecting a contrast medium through a catheter threaded into the pulmonary artery or the right side of the heart to visualize the pulmonary vasculature. Computed Tomography (CT) scans have mainly replaced this...
390