Adrenal Leiomyoma: A Rare Cause of Adrenal Incidentaloma

Presentation Number: SAT 360
Date of Presentation: April 1st, 2017

Saleha Babli*1, Eyad Al-Kharashi2, Khalid Alhajri2 and Mohammed Aldawish1
1Prince Sultan Military Medical City, Riyadh, Saudi Arabia, 2Prince Sultan Military Medical City

Abstract

Background: Leiomyoma arising from adrenals are extremely rare, with less than 20 cases reported in medical literature. Liomyomas though benign lesions, may frequently confuse with malignancy on Imaging studies. The average age of presentation of adrenal lieomyoma is (2-72 years) with female preponderance. They are usually unilateral and hormonally inactive. Human immunodeficiency virus and Ebestein –Barr virus infections have been observed in 44.4 % and 16.6% of cases respectively. However, direct link between immunodeficiency and adrenal lieomyoma has not been established. Adrenal lieomyoma can present as huge abdominal masses mimicking malignancy.

Clinical Case:  A 33 years old lady, referred to endocrinology in the context of left adrenal incidentaloma detected in CT chest performed to evaluate chronic cough. she had no history of fever, night sweat or weight loss, no history of abdominal pain, patient is not hypertensive and no symptoms to suggest Cushing's Syndrome, pheochromacytoma or hyperandrogenism. Family history was irrelevant and physical examination showed normal blood pressure, BMI of 18 and no sign suggestive of adrenal hyperfunctioning. CT scan showed, two left adrenal masses, the larger one is heterogeneous with thick peripheral calcification, mild peripheral enhancement and large central non enhancing component likely representing necrosis measured 7.7x6.2x 7.3 cm, there was no invasion to the adjacent structures. The other lesion was located inferior and medial to the former one and showed mild peripheral enhancement with central area of necrosis measured 3.5x3.3x3.3 cm. Scan through both lungs show multiple branching nodular densities representing tree in bud appearance. Laboratory investigations showed normal dexamethasone suppression test and plasma metanephrins, serologic test for Epstein- barr virus and HIV were negative. regarding her chronic cough patient was evaluated by pulmonologist and was diagnosed as hypersensitivity pneumonitis. she underwent uneventful left adrenalectomy. Gross pathologic evaluation indicated a grey lobulated mass weighed 262 grams and measured 15x9x5 cm. with pale tan whorly cut surface and focal classification. Microscopic evaluation showed a well –circumscribed and encapsulated spindle cells arranged in fascicles and whorls, no features of mitosis or pleomorphism , residual normal adrenal tissue was noted at the periphery. The immunohistochemical (IHC) studies were positive for smooth muscle actin (SMA) and desmin which support the diagnosis of lieomyoma. Additional IHC studies included Melan-A, human melanocyte black 45 ( HMB45) , and Ebestien –Barr virus latent membrane protein (EBV-LMP ) were all negative.

 

Conclusion: Adrenal liomyoma is very rare and should be considered in the differential diagnosis of huge unilateral non-functioning incidentally detected adrenal lesions.

 

Nothing to Disclose: SB, EA, KA, MA