Neck paraganglioma and follicular lymphoma: a case report

• Published in: Journal of medical case reports Vol. 13; no. 1; p. 376
• Main Authors: Marchetti, Lara, Perrucci, Luca, D'Ercole, Francesca, Zatelli, Maria Chiara, Ambrosio, Maria Rosaria, Giganti, Melchiore, Carnevale, Aldo
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 20-12-2019; BioMed Central; BMC
• Subjects: Abdomen; Adrenal Gland Neoplasms - diagnostic imaging; Adrenal Gland Neoplasms - genetics; Adrenal Gland Neoplasms - pathology; Case Report; Case reports; CAT scans; Dehydrogenases; Development and progression; Diagnostic imaging; Follicular lymphoma; Gene mutation; Genes; Genetic aspects; Germ-Line Mutation - genetics; Head and Neck Neoplasms - diagnostic imaging; Head and Neck Neoplasms - genetics; Head and Neck Neoplasms - pathology; Head and neck paraganglioma; Histology; Humans; Hypertension; Imaging; Lymphatic system; Lymphoma; Lymphoma, Follicular - diagnostic imaging; Lymphoma, Follicular - genetics; Lymphoma, Follicular - pathology; Lymphomas; Magnetic Resonance Imaging; Male; Middle Aged; Mutation; Neck; Neuroendocrine tumors; Paraganglioma - diagnostic imaging; Paraganglioma - genetics; Paraganglioma - pathology; Patients; Positron emission tomography; Proteins; SDH; Succinate Dehydrogenase; Surgery; Thyroid gland; Tomography; Treatment Outcome; Tumors; Ultrasonic imaging; Ultrasonography; United States > US; Follicular lymphoma; Head and neck paraganglioma; Imaging; SDH
• ISSN: 1752-1947; 1752-1947
• DOI: 10.1186/s13256-019-2323-1

Paragangliomas and pheochromocytomas are sympathetic or parasympathetic tumors derived from the paraganglia and the adrenal medulla, respectively. Paragangliomas and pheochromocytomas can be sporadic or familial, the latter frequently being multifocal and possibly due to succinate dehydrogenase complex genes mutations. In addition, 12% of sporadic paragangliomas are related to covered succinate dehydrogenase complex mutations. The importance of identifying succinate dehydrogenase complex mutations is related to the risk for these patients of developing multiple tumors, including non-endocrine ones, showing an aggressive clinical presentation.
We report the case of a 45-year-old Caucasian man with an indolent mass in his neck. Ultrasound of his neck, magnetic resonance imaging, and 1,4,7,10-tetraazacyclododecane-N(I),N(II),N(III),N(IIII)-tetraacetic acid(D)-Phe(1)-thy(3)-octreotide (
Ga-DOTATOC) positron emission tomography-computed tomography and endocrine work-up were consistent with a carotid body paraganglioma with concomitant nodal enlargement in several body regions, which turned out to be a follicular lymphoma at histology. He was found to carry a germline Succinate dehydrogenase subunit B gene (SDHB) mutation.
It is crucial to look for a second malignancy in the case of a paraganglioma demonstrating succinate dehydrogenase complex germline mutations.