Locally Invasive Pheochromocytoma Combined with Primary Malignant Adrenal Lymphoma

ABSTRACT Objective: Pheochromocytoma (PHEO) combined with primary adrenal lymphoma is extremely rare. We describe a case of locally invasive PHEO combined with primary malignant lymphoma. Methods: We provide a case description with biochemical analyses, imaging, and pathologic findings. Results: A 79-year-old male presented with a 17-cm, complex mass in the left adrenal gland with non-contrast Hounsfield units of 100 and left para-aortic enlarged lymph nodes imaged by computed tomography. Biochemical evaluation showed plasma and 24-hour urinary normetanephrine significantly elevated about 22 times (about 13.5 times above the normal upper limit) while metanephrine levels were normal. With adequate perioperative preparation, en bloc resection of the left adrenal gland was performed. The pathology revealed a tumor consisting of B-cell lymphoma, a PHEO forming a large adrenal mass with soft tissue invasion with extensive sclerosis and a separate nodule with PHEO without sclerosis. Immunohistochemistry and in situ hybridization of the lymphoma were consistent with Epstein-Barr virus-positive, diffuse large B-cell lymphoma. Immunohistochemistry of the PHEO was positive for chromogranin, synaptophysin, and S100. The Ki67 index was 8.7% and PHEO of the adrenal gland scaled score was 8 (≥4 is considered potentially malignant). Conclusion: To the best of our knowledge this is the first case demonstrating locally invasive and potentially malignant PHEO combined with primary malignant lymphoma in the same adrenal gland. Abbreviations: PAL = primary adrenal lymphoma; PASS = pheochromocytoma of the adrenal gland scaled score; PHEO = pheochromocytoma