Adrenal (Excluding Mineralocorticoids)

SUN-426 - Giant Myelolipomas in Patients with Congenital Adrenal Hyperplasia: A Report of Two Cases Treated with Bilateral Adrenalectomy.

Sunday, June 14, 2026

9:00 AM - 4:00 PM CT

Location: ENDOExpo; Poster Floor

Abstract Poster Presenter(s)

Maria Laura Eugenio Russmann, MD

Endocrinology and Metabolism MD
Ramos Mejia Hospital, Argentina
Buenos Aires, Argentina

Senior Author/Primary Investigator(s)

Maria Laura Eugenio Russmann, MD

Endocrinology and Metabolism MD
Ramos Mejia Hospital, Argentina
Buenos Aires, Argentina

Co-Author(s)

GI

Gissela Inga, MD

Hospital JM Ramos Mejia
Buenos Aires, Argentina
LG

Lucas Gutnisky, MD

Hospital JM Ramos Mejia
Buenos Aires, Argentina
PS

Patricia San Martin, MD

Hospital JM Ramos Mejia
Buenos Aires, Argentina

Disclosure(s):

Maria Laura Eugenio Russmann, MD: No financial relationships to disclose

Introduction: In patients with Classic Congenital Adrenal Hyperplasia (CAH) inadequately treated, adrenal tumors are frequent (prevalence: 82% in homozygotes, 45% in heterozygotes). Generally unilateral and adenomas, though myelolipomas are described. We present two patients with Simple Virilizing (SV) CAH and suboptimal control, presenting large bilateral myelolipomas requiring surgical management.
CASE 1: Female, 44, diagnosed with SV-CAH at 1 month, treated with hydrocortisone. Presented in 2009 (age 28) with primary amenorrhea. History of genital reconstruction at 19. PE: BMI 27.3 kg/m², Tanner III, infundibuliform vagina, no clitoromegaly, acne, or hirsutism. Marked hyperandrogenemia noted; switched to dexamethasone 0.5mg, achieving menarche. Transitioned to meprednisone 4mg. CYP21A2: Mutation In2/R356. Abdominal CT: bilateral myelolipomas (left 9cm, right 5cm). Left laparoscopic adrenalectomy performed (10x15cm mass). Histopathology: myelolipoma. Lost to follow-up 2019-2022. In 2023, CT revealed voluminous mass (13.2x9.6x11cm) in Right Adrenal Gland (RAG). Right adrenalectomy performed. Histopathology: Myelolipoma and adenoma.

CASE 2: Male, 51, diagnosed SV-CAH at 20 months. Presented in 2016 (age 42) reporting erectile dysfunction, asthenia, decreased libido. Proven fertility (2 children). PE: BMI 31 kg/m², testes 12mL bilaterally, penis 8x3x3cm. Prior hydrocortisone 30mg discontinued 2 years prior. Restarted meprednisone 4mg. CYP21A2: p.Ile172Asn and DEL/CONV (heterozygous). CT: bilateral myelolipomas, predominantly left. Lost to follow-up 2016-2023. New CT: RAG mass 5.6x5.4x3.2cm (-69 HU). Left Adrenal Gland (LAG): two masses (-17 HU), 6.8x7.3x7.6cm and 12.7x10.4x9.7cm displacing structures. Left adrenalectomy performed; histology: myelolipoma. Five months later, elective right adrenalectomy. Histopathology: myelolipoma.
COMMENTS: Adrenal masses (AM) in CAH are described since 1981 (prevalence 29.3%; 7.4% myelolipomas, 30x general population). Related to late diagnosis/suboptimal control. Bilaterality reported in 22-40%. LAG is affected in 63% of unilateral cases. Generally, myelolipomas < 10cm don't require surgery, but giant ones (as in our cases) require removal due to mass effect/hemorrhage risk. Coexistence of CAH and giant myelolipomas is infrequent, linked to poor adherence. We suggest adrenal imaging in follow-up of all Classic CAH patients, especially those with inadequate control.

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