ESPE Abstracts

Objectives: We describe an unusual presentation of Histiocytosis in a 17 year old female with a large lung mass, and bilateral adrenal masses.

Methods: Case report

Results: A 17-year-old female presented with chest pain, as well as several weeks history of fatigue, decreased appetite, a dry cough and unintentional weight loss. No history of fevers or night sweats. Her vital signs and physical exam were normal; no virilization or cushingoid features. CBC showed normocytic anemia. She had normal electrolytes. CT-A Chest revealed a 9.2 x 8 x 7.4 cm heterogeneous hypervascular mass in the right lower lung; a similar-appearing 7.1 x 5.4 x 6.9 cm heterogeneous mass in the left adrenal gland and a 3.4 cm hypodense mass in the right adrenal gland. Tumor markers showed normal CA 19-9, AFP, Beta-Hcg, CA 125, and CEA. Dopamine, Epinephrine and Norepinephrine levels were normal. Urine metanephrine to creatinine ratio was normal at 226 mg /g. HVA and VMA were in normal ranges at 3.9 and 3.6 mg/g respectively. Free metanephrines and normetanephrines were both normal at less than 0.2 nmol/L. Testing for echinococcus, tuberculosis and coccidioides was negative. Endocrine workup was normal with 17-OHP at 81 ng/ml, Aldosterone 2.0 ng/dl, Renin 1.5 ng/mL/h, DHEAS 37 mg /dl, Testosterone 14 ng/dl, Progesterone less than 10 ng/dl, ACTH 29 pg/ml and AM Cortisol 18.2 mg/dL. She underwent IR-guided biopsy of the left adrenal mass and was discharged. She was readmitted for chemotherapy after preliminary pathology from the adrenal mass returned ALK-positive. MRI spine showed evidence of spinal metastasis, so she received a short course of palliative radiation therapy. She was started on Crizotinib, an ALK/ROS1 inhibitor. Final pathology report returned as ALK-positive Histiocytosis. Follow up CT after starting Crizotinib showed decrease in size of lung and adrenal masses.

Conclusions: The differential diagnosis for bilateral adrenal masses includes metastatic diseases (renal, lung cancer, melanoma), lymphoma, pheochromocytoma, ACTH–dependent Cushing's, congenital adrenal hyperplasia, primary aldosteronism, bilateral macronodular adrenal hyperplasia and infections such as tuberculosis or fungal. This case was unique in that Histiocytosis presented as bilateral adrenal masses along with a large lung mass. We hope that by reporting this interesting case we can increase physician awareness of this condition, and help expand the differential diagnosis for bilateral adrenal masses.