Endocrine Abstracts

Introduction: Paragangliomas are neuroendocrine tumors that are derived from the embryonic neural crest cells. Because the paraganglia are widespread through the body, paragangliomas can be found in unusual anatomic locations and the diagnosis can be hard to establish. Case presentationWe present the case of an 72 year-old, hypertensive woman who arrived at the hospital for pain in the right hypochondrium. General examination revealed an abdomen without signs of peritoneal irritation, high blood pressure (170/80 mmHg) and tachycardia (110/bpm). Blood tests showed minimum leukocytosis and slightly elevated liver enzymes. Routine abdominal ultrasound revealed a mass measuring 34/32 mm in the caudate lobe of the liver. Consequent computed tomography described a well-defined liver mass measuring 40/34 mm, adjacent to caudate lobe, para-aortic. The patient underwent endoscopic ultrasound-guided fine needle aspiration, without complications, to establish the tumor type. The histopatology examination revealed compact cell nests with vesicular nuclei, absent mitoses and amphophilic cytoplasm, without well defined boundaries. Imunohistochemical testing found negative results for keratins and positive staining for S-100 in sustentacular cells and positive results for cromogranin A and synaptopysin in chief cells, with a Ki 67 of 1-2%, being established the diagnosis of paraganglioma. The patient was trasferred to our department, where she underwent further biochemical testing to establish the functional status of the tumor. Blood tests showed high values of plasma normetanephrines (1127.99 ng/l), slightly elevated values for metanephrines (124.92 ng/l) and normal values for methoxytyramine. Chromogranin A couldn’t be measured because the patient was receiving proton pump inhibitors. After establishing the diagnosis of secretory paraganglioma, the patient started the treatment with alfa and beta blockers and was sent to a surgery department.

Conclusion: The reported case is interesting because of the age of the patient and the way the diagnosis was established. Most paragangliomas are diagnosed in younger people, mostly in their third to fifth decade. Even if the majority of sympathetic paragangliomas are located in the abdomen, given the rarity of the tumor, the diagnosis can be missed without a degree of suspicion and further investigations. In our case the diagnosis was established after biopsy, which is contraindicated in a patient suspected of having paraganglioma because of the associated risks like severe hypertension, hemorrhage or fibrosis at the intervention site and because the aspirates can be difficult to distinguish from other neoplasms.