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. 2025 Jan;47(1):386-393.
doi: 10.1002/hed.27923. Epub 2024 Aug 21.

Management of bilateral head and neck paragangliomas at a single-institution across four decades

Nikhil Bellamkonda  1 , Evan L Tooker  2 , Anne Naumer  3 , Luke O Buchmann  1 , Wendy Kohlmann  3 , Hilary C McCrary  1 , Neil S Patel  1 , Mana Espahbodi  1
Affiliations

Management of bilateral head and neck paragangliomas at a single-institution across four decades

Nikhil Bellamkonda et al. Head Neck. 2025 Jan.

Abstract

Background: Bilateral head and neck paragangliomas (HNPGLs) require nuanced management to balance tumor control with functional preservation.

Methods: All patients seen at a single-institution for bilateral paraganglioma between 1983 and 2023 were retrospectively reviewed. Demographics, genetic testing results, and tumor characteristics were analyzed and compared to treatment modality and cranial nerve outcomes.

Results: There were 49 patients with 116 tumors (90 carotid body tumors [CBTs], 15 vagal paragangliomas [VPs], and 11 jugular paragangliomas [JPs]). Twenty-six patients had SDH pathologic variants (PV). Surgical management was more commonly utilized in younger patients (OR: 0.97, 95% CI: 0.950-0.992) and for JPs (OR: 9, 95% CI: 1.386-58.443). In surgical cases, CBTs had a lower risk of postoperative cranial nerve deficits compared to JPs and VPs (OR: 0.095, 95% CI: 0.013-0.692).

Conclusions: Younger patients with bilateral HNPGLs, especially those with JP and CBT, are more often treated with surgery. CBTs have lowest risk of cranial nerve deficits after surgery.

Keywords: SDH; bilateral paraganglioma; carotid body tumor; jugular paraganglioma; vagal paraganglioma.

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Conflict of interest statement

Neil S. Patel, MD, serves as a consultant for Viridian Therapeutics, has received research support from Cochlear Corp., and has functioned as a speaker on behalf of IotaMotion and Zeiss. All other others have no financial relationships to disclose.

Figures

FIGURE 1
FIGURE 1
Relationship between (A) surgical treatment and number of tumors and (B) surgical treatment and tumor location. Number of tumors was not associated with surgical treatment. JPs were most frequently treated with surgery. [Color figure can be viewed at wileyonlinelibrary.com]
FIGURE 2
FIGURE 2
Relationship between (A) radiation treatment and number of tumors and (B) radiation treatment and tumor location. Radiation was more commonly utilized in patients who had a greater number of tumors, and less frequently in the management of CBTs. [Color figure can be viewed at wileyonlinelibrary.com]
FIGURE 3
FIGURE 3
Relationship between cranial nerve deficits and treatment strategy. Cranial deficits were more common in those treated with surgery, less common with observation, and not correlated to radiation therapy. (A) Of 62 tumors undergoing surgery only, 11 (18%) had cranial nerve deficits postoperatively, compared to one (2%) with cranial nerve deficits preoperatively. (B) Of the eight tumors treated with radiation only, two (25%) had cranial nerve deficits after radiation, compared to zero with cranial nerve deficits prior to radiation. (C) Of the 46 tumors undergoing observation, only one (2%) had cranial nerve deficits. Of the 70 tumors undergoing treatment, 14 (20%) had cranial nerve deficits. (D) Among tumors undergoing surgery, CBTs were the least likely to develop cranial nerve deficits after surgery. [Color figure can be viewed at wileyonlinelibrary.com]
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