Session: SUN 29-49-Congenital Adrenal Hyperplasia & Ectopic Cushing's
Poster Board SUN-34
Case:29 y/o Female was referred for evaluation of Left (L) adrenal mass and chronic abdominal pain.
Past history: Ambiguous genitalia at birth, hirsutism, primary amenorrhea and recurrent UTI’s. She received prednisone from age 6-16 for bone age advancement but was unaware of her diagnosis. Medication: Vicodin q6hrs for pain. Family history: non-contributory, mid-parental height 160 cm.
Physical examination: Height 134 cm(<3%ile),Weight 68.5 kg, BMI 38 kg/m2, BP 143/94mm Hg. Generalized obesity. Abdomen: soft, tenderness in L lower quadrant and flanks. Tanner II breasts, Tanner V pubic hair. Clitoromegaly (clitoral index 160mm2), Ferriman-Gallawey score 13.
Laboratory tests: 6AM: Cortisol 3.8 mcg/dL(5-25), ACTH 458 pg/mL (0-46), testosterone 260 ng/dL(8-60), 17-hydroxyprogesterone 17,900 ng/dL (<285), plasma renin activity:3.9 ng/mL/hr(0.6-4.3), Na: 139 mg/dL(135-144) and K: 3.9 mg/dL(3.3- 5.1).
Pelvic ultrasound: prepubertal uterus and ovaries.
Adrenal CT: Lobulated, enhanced L adrenal mass of 10x7cm compressing the L kidney.Upper portion with lower density suggestive of fat (-64 Hounsfield units (HU)). Right(R) adrenal: enlarged with a 4x5 cm low density mass.
18 Fluorodeoxyglucose(FDG) Positron Emission Tomography (PET)-CT: L adrenal:Large bilobed mass. Upper portion lipid containing(-35HU), solid lower portion hypermetabolic, maximal standardized uptake value (SUVmax) 28.7. R adrenal:Enlarged, hypermetabolic, SUV max 8.74 with a lipid centered nodule (-81 HU).
Pathology post L adrenalectomy: Well circumscribed lesion composed of mature adipose tissue and hematopoietic precursor cells consistent with a myelolipoma, with areas of myxoid degeneration. Diffusely hyperplastic adrenal cortex with nodules of heavily pigmented adrenal cortical cells.
Conclusions: Untreated simple virilizing CAH results in multiple adverse outcomes including lack of pubertal development, short stature and tumor formation. Although myelolipomas are benign hormonally inactive tumors, they can cause chronic abdominal pain and, unlike other benign tumors, can demonstrate increased uptake with 18FDG corresponding to the adenomatous and hematopoietic components.
Disclosure: DPM: Clinical Researcher, Diurnal. Nothing to Disclose: AM, AE, CV, CMM, NAA, DP, MQ, EK
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