Inflammatory Myofibroblastic Adrenal Tumor: An Extreme Rare Cause of Adrenal Incidentaloma

Abstract

Introduction: An inflammatory myofibroblastic tumor (IMT) is considered a complex disease with a suggested neoplastic nature. It has a variable histological and clinical presentation. Up to now, the exact etiology is still unknown. Various sites such as the lungs (first site described in 1939), the heart, the gastrointestinal tract, and the genitourinary tract have been reported in the literature. The adrenal glands are considered an extremely rare location with no recurrence reported after surgical management. Up to our knowledge, only 9 cases have been described in the literature. The age of presentation was variable (from newborn to 57 years old). Case Report: A 70 years old female with a past medical history of well-controlled diabetes type 2, and hypertension presented with a progressively increasing chronic right flank pain for the past couple of months, associated with 10 lb weight loss. Workup was done and an incidental right adrenal mass was found on CT scan. The mass was 3.2cm x 3.3 cm partially necrotic with adjacent stranding abutting the right adrenal gland and the liver. The opposite gland was normal and there was no evidence of any other mass lesion in the abdomen. MRI adrenal showed 3.2x2.5x3.6 cm solid heterogeneous mass in the right adrenal gland (Hypointense on T1 and T2 weighted images. She had no sign or symptoms suggestive of Cushing and/or pheochromocytoma. Her hypertension was well controlled on lisinopril 10 mg. Hormonal workup was within normal range except for a mild elevation of DHEAS. Given the size of the mass, the worrisome radiological characteristics, and a family history of pheochromocytoma in her daughter, surgical options were discussed with the patient. Subsequently, the patient underwent the right robotic adrenalectomy. The postoperative course was uneventful. Pathology revealed a 6.5x6x 5 cm IMT of the adrenal. The CT scan done 6 months postoperatively didn’t show any recurrence. Discussion: and conclusion: Adrenal IMTs are extremely rare but they should be considered in the differential diagnosis of adrenal masses. Follow-up is warranted since its behavior still remains uncertain. More cases are needed to unmask the true biological behavior and pathogenesis of adrenal IMTs.