A Rare Case of Simultaneous Pheochromocytoma Accompanied with an Ileocecal Neuroendocrine Tumor

The detection rates of neuroendocrine tumors (NETs) are rising, which has led to a better understanding of their histopathologic characteristics. We present a case of adrenal pheochromocytoma with an incidental finding of a concurrent ileocecal NET detected on early post-adrenalectomy imaging. A 59-year-old male was noted to have microscopic hematuria on a routine workup. Further imaging identified a right adrenal lesion suggestive of pheochromocytoma. He was reporting intermittent headaches, and his urinary normetanephrine and norepinephrine levels were elevated. He underwent a right retroperitoneal adrenalectomy, and pathology confirmed a pheochromocytoma with no malignancy. Postoperatively, he developed a hematoma at the surgical bed that was identified on CT scan. An incidental lesion at the level of the ileocecal valve was noted on the CT scan as well. This was further investigated by colonoscopy and biopsy that confirmed a carcinoid tumor. He had no gastrointestinal symptoms, chromogranin A was normal, and 24-h urinary 5-hydroxyindolacetic acid levels were slightly elevated. He subsequently underwent a laparoscopic right hemicolectomy 3 months from his initial adrenalectomy. Final pathology confirmed a grade 2 NET with metastatic involvement of 4 regional lymph nodes. We report an extremely rare case of simultaneous pheochromocytoma accompanied with an ileocecal NET. Genetic testing for multiple endocrine neoplasia was undertaken and was found negative for 9 tested genes.