Questioning the Connection Between Sarcoidosis and Gastrointestinal Carcinoid Tumors

Abstract

A 52-year-old African-American male with a past medical history of hypertension and hyperlipidemia presented to the hospital with a 7-10 day history of fatigue, malaise, fever, and cough productive of greenish sputum. His symptoms resolved with a course of Levaquin. His workup revealed lymphadenopathy on chest xray, so he underwent a CT scan and an eventual PET scan demonstrating extensive mediastinal and bilateral hilar lymphadenopathy along with multiple small lung nodules measuring up to 5 mm in the right upper lobe. There was also thickening along the mid to distal esophagus with presence of an adjacent 1.3 cm paraesophageal lymph node. Gastroenterology was consulted for endoscopic evaluation of the esophageal abnormalities. Esophagogastroduodenoscopy (EGD) was performed and revealed a normal esophagus and stomach, but a 2 cm nodule in the duodenal bulb. Biopsy was consistent with well differentiated neuroendocrine tumor (carcinoid). EUS with fine needle aspiration and biopsy was performed on the subcarinal and paraesophageal lymph nodes. Both nodes showed small compact granulomas without necrosis, consistent with sarcoidosis. Complete workup for infectious etiology, including bacterial and AFB cultures, was negative. The association between systemic sarcoidosis and malignant neoplasms has been reported before, but the relationship specifically between carcinoid tumors and sarcoidosis is yet to be detailed. Literature review did not reveal reports of concurrent sarcoidosis and gastrointestinal carcinoid tumors; however, there have been reports of sarcoidosis and pulmonary carcinoid. Levy et al 1997 report a case series of several patients who were encountered at Mayo Clinic with both sarcoidosis and carcinoid tumors. As with our patient, their series was unable to determine the chronology of events in which these two conditions occur. There remains controversy as to whether sarcoidosis predisposes patients to carcinoid tumors or whether it is the tumors that cause a sarcoid reaction. Our case provides suggestion that an association between extrapulmonary carcinoid tumor and sarcoidosis may exist.