Endocrine Abstracts

Introduction

Oncocytic neoplasms arising in adrenal tissue are extremely rare with nearly 150 cases being reported in literature. They are mostly nonfunctioning benign tumors, incidentally discovered, but 20% of them demonstrate elements of malignancy and up to 30% appear to affect hormone production.

Case report

A 35 years old female with primary amenorrhea, diagnosed with Congenital Adrenal Hyperplasia due to 21-hydroxylase deficiency at age of 2, who underwent feminizing genitoplasty 5 years after diagnosis, presented in our clinic complaining of abdominal pain, nausea, vomiting and hypotension. She was on treatment with 1 mg Dexamethasone daily with poor medication adherence. Clinical findings included: altered general condition, dry skin and mucosae, hirsutism, BP = 70/50 mmHg with orthostatic hypotension, HR = 100 bpm, intense spontaneous and on palpation pain located in the left iliac fossa and moderate abdominal dystension. Lab tests revealed an important inflammatory syndrome, normocytic normochromic anemia, hypoglycemia (65 mg/dl), high-normal potassium level (5 mmol/l), hyponatremia (131 mmol/l), hypocalcemia (8.3 mg/dl). She started IV hydrocortisone and oral fludrocortisone. Hormonal workup showed high 17-OH progesterone (> 20 ng/ml), low 0800 h cortisol (1.58 µg/dl), high ACTH (1362 pg/ml), high renin (170 pg/ml), normal aldosterone (243 pg/ml). Abdominal and pelvic MRI revealed on the superior pole of left adrenal gland a 55/60 mm tumoral mass with polycyclic contour and at the level of the left anexa another tumoral mass with cystic components, thick walls, with maximum diameters of 47/75 mm. After the acute episode remission, the patient was transferred to surgery unit where was performed left adrenalectomy with left anexectomy. Hystopatological exam established the diagnosis of adrenocortical oncocytic neoplasm with uncertain malignant potential and immunochemestry was positive in tumoral cells for inhibin A, Melan A, Calretinin, Synapto and negative for Chromogranin A, Ki67~ 2%.

Conclusion

Oncocytic adrenocortical neoplasm is a rare tumor of the adrenal gland which origin, biological behavior, diagnostic criteria and prognosis are controversial. To our knowledge, only one case of adrenocortical oncocytoma in a patient with congenital adrenal hyperplasia was previously reported in the literature. More data are needed for better management of these patients.