A case of adrenal myelolipoma complicated with Prader-Willi syndrome

Abstract

Introduction: Prader-Willi syndrome is a congenital disorder that occurs in one in 10 000-30 000 children and is characterized by obesity, short stature, and intellectual disability.

Case presentation: A 24-year-old male patient with Prader-Willi syndrome presented with an enlarged adrenal tumor. Computed tomography detected a well-defined mass. Magnetic resonance imaging revealed an increased signal intensity predominantly in fatty areas, suggesting adrenal myelolipoma. Laparoscopic left adrenalectomy was performed. Postoperatively, the patient developed mild pulmonary atelectasis, myelolipoma was confirmed by histopathology, and there was no recurrence at approximately 2 years postoperatively.

Conclusion: This is the first report of Prader-Willi syndrome complicated with adrenal myelolipoma, which was removed laparoscopically.

Keywords: Prader‐Willi syndrome; adrenal myelolipoma; intellectual disability; laparoscopic adrenalectomy; obesity.

Fig. 1

Abdominal CT scan shows a retroperitoneal tumor of 78 × 70 mm², mostly consisting of fatty density areas (a). Changes in tumor size on CT images. (a) Current (78 × 70 mm²); (b) 10 years ago (6.5 × 6 mm²); (c) 2 years ago (57 × 45 mm²).

Fig. 2

CT findings Axial view. Subcutaneous fat is 40–50 mm thick, indicated by an orange line.

Fig. 3

(a) Macroscopic findings of the resected specimen show a large yellow solid tumor with a normal adrenal gland. (b) Histological examination shows focal hematopoietic elements in mature adipocytes (Hematoxylin and eosin staining, 200×)