Malignant Vagal Paraganglioma

Abstract

Paragangliomas are rare, typically benign neuroendocrine tumors that represent a small portion of head and neck tumors. A small percentage of these are known to have malignant potential. They arise from the carotid body, jugular bulb or vagus nerves. There is limited literature discussing the management of malignant vagal paragangliomas. We present a case of a 25 year old female with a left malignant vagal paraganglioma. The following case presentation will describe the presentation, classic radiologic findings, and management of a malignant vagal paraganglioma along with a review of the literature.Keywords Paraganglioma Á Carotid body tumor Á Malignant paraganglioma HistoryA 25 year-old female reported to the emergency department for evaluation of a severe headache. An avid singer, she incidentally complained of difficulty with vocal range and stamina over several months. Family history was negative for head and neck cancer. Physical exam revealed fullness with mild generalized erythema of both the left posterior nasopharynx and the oropharynx. On palpation of the left oropharynx there was no discrete mass, however, the patient endorsed mild tenderness on palpation. Flexible fiberoptic laryngoscopic exam revealed normal bilateral true vocal fold motion and appearance. No cervical lymphadenopathy was appreciated and cranial nerves II-XII were intact. Radiographic FeaturesComputed tomography (CT) identified a 6 9 4 9 3 cm left post-styloid parapharyngeal space avidly enhancing mass (Fig. 1). Magnetic resonance imaging (MRI) confirmed a mass within the left parapharyngeal space that was isointense on T1, slightly hyperintense on T2, and showed multiple flow voids with gadolinium enhancement. The mass was posterior to the carotid bifurcation and anterolateral to the internal jugular vein. Radiographic findings were highly suspicious for a vagal paraganglioma or nerve sheath tumor. Urine catecholamines, vanillylmandelic acid (VMA) panel and thyroid function studies were all within normal limits. Succinyl dehydrogenase (SDH) gene testing was negative.Disclaimer: The opinions and assertions expressed herein are those of the authors and are not to be construed as official or representing the views of the Department of the Navy or the Department of Defense. I certify that all individuals who qualify as authors have been listed; each has participated in the conception and design of this work, the writing of the document, and the approval of the submission of this version; that the document represents valid work; that if we used information derived from another source, we obtained all necessary approvals to use it and made appropriate acknowledgements in the document; and that each takes public responsibility for it. We are military service members.

Paragangliomas of the Head and Neck: Epidemiology, Radiologic Diagnosis, Treatment

“…ВПГ локализуются в передне-или заднебоковом окологлоточном пространстве, верхний полюс опухоли доходит до основания черепа, а нижний может достигать бифуркации ОСА, что объясняет их интимное прилегание к блуждающим, подъязычным и языкоглоточным нервам, ВСА [16]. Дифференциально-диагностическим признаком ВПГ в отличие от КХД является переднемедиальное смещение ВСА с оттеснением кзади и латерально внутренней яремной вены без вовлечения каротидной бифуркации [42].…”

“…ВПГ представлены образованием овоидной формы, расположенным в области сонных артерий, причем ВСА смещается опухолью переднемедиально (рис. 7) (отличительный признак при сравнении с КХД), а внутренняя яремная вена -заднелатерально без вовле чения каротидной бифуркации [42].…”

“…КТ -наиболее информативный метод при малых размерах опухоли. Характеристики аналогичны КХД [42]. При больших размерах опухоли, достигающей основания черепа, отсутствие деструкции и расширения яремного отверстия служит дифференциально-диагностическим признаком в отличие от ЯПГ [15].…”

Mutation profiling in eight cases of vagal paragangliomas

“…VPGL accounts for approximately 13% of all head and neck paragangliomas (HNPGLs) and occurs more frequently in women [ 6 , 7 ]. Surgical resection is a primary treatment for VPGL, whereas radiation therapy is used in case of malignant and unresectable tumors [ 8 , 9 ]. VPGL has a lower risk for metastasis compared with carotid paraganglioma (CPGL) [ 10 ].…”

Vagal Paraganglioma Presenting as a Neck Mass Associated with Cough on Palpation

“…Paragangliomas (PGs) have classically been said to account for 0.6% of head and neck tumors, and, first described in 1935, VPs are the most rare subtype of head and neck PGs, contributing less than 5% of their total [ 1 ]. VPs typically present as a neck mass associated with hoarseness or pulsatile tinnitus [ 2 ]. PGs of the head and neck may undergo malignant transformation in 6% to 19% of cases; VPs are the most likely to become malignant and to metastasize [ 3 ].…”