Multifocal malignant paraganglioma of the paranasal sinuses: A case report

Abstract

Paragangliomas are tumors that arise from the paraganglia that are distributed in the head and neck from skull base to aortic arch. The most commonly described sites are the aortic bodies, the temporal bone, and the vagal body. Rarely have these tumors been described in areas where there is apparently no paraganglionic tissue. Twenty four confirmed paragangliomas located in the nasal cavity and the paranasal sinus have been reported, only 4 of which were malignant. 1,2 We report the fifth case of malignant nasal paraganglioma and the first multifocal form described in this area. CASE REPORTA 72-year-old woman presented to our department with a painless mass in the right submandibular triangle that appeared 5 months earlier, progressively increasing in size. Her medical history was unremarkable except for a right nasal polypectomy performed at another institution 7 years earlier. Cytologic analysis of a fine needle puncture realized on the node revealed weakly basophil cells organized in nests, suggesting a paraganglioma.Surgical exploration, performed to make a diagnosis, found a lymph node adhered to the submandibular gland that was removed.

A case of sinonasal paraganglioma with a different morphology: nine-year follow-up

“…All previous sinonasal paragangliomas described in the literature had a typical zellballen pattern. [1][2][3][4][5][6] To our knowledge, this is the first report of a sinonasal paraganglioma with atypical lipoblast-like vacuolated cells. The extraordinary morphology described herein is very rare.…”

“…[3,4] In a typical histopathological examination of a paraganglioma, neoplastic or chief cells are arranged in well-defined nests, which have the classic alveolar or so-called zellballen pattern. [5] An immunohistochemical study highlights the presence of S-100 protein-positive sustentacular cells at the periphery of the clusters of chief cells.…”

Nasal Cavity Paraganglioma: Literature Review and Discussion of a Rare Case

“…These are all hypervascular tumors, with contrast enhancement, presenting the classic “salt-and-pepper” appearance and variable patterns of intensity on MRI [5,10,20,21,22,23,24,25,26,27,28,29,30,31,32]. …”

“…The presence of these cells has been reported in stillborn infants, and the origin of paragangliomas of this site has been suggested to derive from residual specialized neural crest cells along cranial arteries [10,11,12]. …”

Sinonasal Paraganglioma With Long-Delayed Recurrence and Metastases: Genetic and Imaging Findings

“…Unusual or exceptional locations have also been reported (eg, larynx, thyroid, sinonasal region, nasopharynx, orbit, tongue). Sinonasal PGLs (SNPGL) are exceptional tumors that can be malignant (1)(2)(3)(4). When malignant, SNPGLs are very aggressive, with rapid local spread as well as high metastatic potential, despite surgical resection.…”