Abstract
Paraganglioma are rare type of neuroendocrine tumors that arise from embryonal neural crest. Paraganglioma of adrenal medulla is known as Pheochromocytoma. Extra adrenal localization has incidence of 2-8 per million people per year, which can extend from upper cervical region to pelvis related to autonomic nervous system. Even rarer is occurrence of paragangliomas outside the usual distribution of paraganglionic tissue. Mesenteric paragangliomas are extremely rare. The present case was of a non-functional extra adrenal paraganglioma occurring in a 43-year-old male presented with complain of pain in abdomen and mild discomfort. Abdominal CECT revealed well defined enhancing solid mass with central necrosis in right side of mesentry. Explorative laparotomy and surgical excision were done, on histology typical “zellballen” pattern was found. Paragangliomas are rare neuroendocrine tumor known to store and secrete catecholamines and hence are known as functional paragangliomas and may present with symptoms like headache, sweating, palpitations and hypertension. On other hand, they may remain silent and non-functional and present with vague symptoms like pain abdomen due to episodic release of catecholamines. Surgical excision is treatment of choice. Long term follow up is mandatory as tumor is prone to recur and metastasize.
Highlights
On localization of paragangliomas is the adrenal medulla and they are usuallyParaganglioma are rare type of neuroendocrine tumor that arise from embryonal neural crest [1]
Paragangliomas are tumors originating from the neuroendocrine elements of the paraganglia
Extra-adrenal localization is observed in 5-10% of all paragangliomas [2]
Summary
Paraganglioma are rare type of neuroendocrine tumor that arise from embryonal neural crest [1]. Paraganglia contain tissues such as the adrenal medulla, carotid and aortic bodies, paraganglia in sympathetic and parasympathetic nerves and organs of Zuckerkandl. Paragangliomas are tumors originating from the neuroendocrine elements of the paraganglia. Extra-adrenal localization is observed in 5-10% of all paragangliomas [2]. The most common extraadrenal paragangliomas occur as carotid bodies. Paragangliomas have been seen in the gastrointestinal tract of the duodenum. Mesenteric paragangliomas are exceedingly infrequent [3]. With an annual incidence estimated at 1/100,000, paragangliomas re-
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