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Case Reports: Rare disease
Oncocytic adrenocortical neoplasm with undetermined malignant potential and autonomous cortisol secretion
  1. Mari Des San Juan1,
  2. Dian Lagamayo2,
  3. Jose Carnate2 and
  4. Mark Henry Joven1,3
  1. 1Section of Endocrinology, Diabetes, and Metabolism, The Medical City, Pasig City, Philippines
  2. 2Department of Laboratory Medicine and Pathology, The Medical City, Pasig City, Philippines
  3. 3Faculty of Medicine and Surgery, University of Santo Tomas, Manila, Philippines
  1. Correspondence to Dr Mari Des San Juan; maridessan1{at}gmail.com

Abstract

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.

  • Adrenal disorders
  • Endocrine cancer

https://doi.org/10.1136/bcr-2021-248525

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    Footnotes

    • Contributors MDSJ and MHJ provided and cared for the study patient. JC and DL performed histopathological analysis. All authors contributed to the writing of the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.