Giant Adrenal Myelolipoma and Congenital Adrenal Hyperplasia: a Case Report and Review of the Literature

Abstract

Introduction Adrenal myelolipomas (AMs) are rare, benign tumors without endocrine activity comprised of mature adipose and hematopoietic tissue. Surgical excision may be required for lesions which have associated symptoms, those that are growing, or have features concerning malignancy. Congenital adrenal hyperplasia (CAH) is often associated with adrenal masses such as AM. The majority of literature regarding AMs consists of case reports and case series with few describing giant AMs in the context of CAH. Case Presentation We present the case of a 50-year-old woman with CAH and surgical history relevant for left adrenalectomy who presented to the emergency department with abdominal pain. Computed tomography (CT) imaging led to a diagnosis of diverticulitis which was treated non-operatively. The scan also revealed a 20 × 13 × 23-cm right retroperitoneal mass causing mass effect on the duodenum, pancreas, right kidney, and inferior vena cava. CT-guided biopsy demonstrated adrenal and adipose tissue with hematolymphoid aggregates possibly representing myelolipoma but unable to rule out malignancy. Due to concern for retroperitoneal liposarcoma, the patient underwent an exploratory laparotomy with resection of > 40-cm right retroperitoneal mass, right nephrectomy, right adrenalectomy, and cholecystectomy. Final pathology demonstrated a myelolipoma with intra-tumoral ossification and hemorrhage as well as diffuse cortical hyperplasia with lymphoid aggregates. Due to acquired complete adrenal insufficiency, the patient required stress-dose steroids and steroid taper post-operatively. Conclusions Key points from this report include the occurrence of a giant AM in the context of CAH and special considerations in the diagnosis and operative approach of a giant retroperitoneal mass.