Functional Ulnar Nerve Paraganglioma: First Documented Occurrence in the Extremity With Hitherto Undescribed Associated Extensive Glomus Cell Hyperplasia and Tumorlet Formation

Abstract

Extra-adrenal paraganglioma has never been described in the extremities. A 34-year-old woman complained of an enlarging mass in the right forearm for 18 months. Imaging showed a circumscribed vascular tumor attached to the ulnar nerve; biopsy revealed features of paraganglioma. The resected tumor consisted of zellballen pattern of chief cells staining positively for chromogranin with surrounding S100-positive sustentacular cells. The chief cells contained many neurosecretory granules and mitochondria, whereas the sustentacular cells contained a large amount of rough endoplasmic reticulum and some microfilaments. There was adjacent extensive glomus cell hyperplasia and tumorlet formation. The intraoperative blood pressure dropped abruptly on tumor removal. The serum normetanephrine level decreased from a preoperative level of 1987 pg/mL (normal < 149 pg/mL) to normal after operation. The patient admitted on questioning to a history of paroxysmal attacks of transient palpitation, hand tremors, and sweating; imaging showed no evidence of tumor in other parts of the body, and there was no family history of similar tumor; she remained well 33 months after the operation. This occurrence of functional ulnar nerve paraganglioma with the hitherto undescribed associated glomus cell hyperplasia and tumorlet formation attests to the probable existence of normal sympathetic paraganglia in the extremity and their intimate functional relationship with glomus bodies.

The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

“…Because of this widespread distribution of paraganglia, paragangliomas can occur at virtually all locations in the body except within the brain and in bone, and although they have been reported in the extremities [ 10 ], these are exceptional curiosities. Given the normal distribution of paraganglia associated with components of the autonomous nervous system, it should be no surprise that paragangliomas can occur in the gallbladder [ 11 , 12 ], and the liver [ 8 , 13 , 14 ], where they are likely to arise from small abdominal vagus nerve branches.…”

Functional Mediastinal Paraganglioma Irrigated by Coronary Artery Collaterals Associated With Bilateral Renal Artery Fibro-Dysplasia in a Hypertensive Patient

“…They can be found in all normal anatomical sites of paraganglia where chromaffin tissue is located (para-aortic sympathetic chain, sympathetic chain in the neck and mediastinum, within organs of Zuckerkandl at the origin of the inferior mesenteric artery and the wall of the urinary bladder). It can arise from all locations in the body except within the brain and in bone [ 2 ]. PGLs can be classified as functional or non-functional PGLs based on the ability of synthesizing and releasing catecholamines.…”

Excision of A Rare Bilateral Malignant Carotid Paraganglioma: What We Have Done and What’s to Do

“…They can arise in any location where paraganglia normally reside [4]. Because of widespread distribution of paraganglia, paragangliomas can occur at virtually all locations in the body except within the brain and in bone although they have been reported in the extremities [5].…”