PMON85 Co-occurrence of Carotid Body Tumor, Meningioma and Schwannoma in a Symptomatic Patient, Incidental Findings Vs Genetic Syndrome; a case report.
Authors: Arshad Iqra , Jerez Julia-Vergas , Zahra Tasneem 
Published: November 2022

Carotid body tumors, also known as chemodectomas/paraganglionomas; are rare neuroendocrine tumors that arise from parasympathetic ganglionic cells. They are usually benign with incidence of malignancy is &lt;10%. In majority of cases, tumors are asymptomatic and found incidentally on clinical exam or radiological imagining. Their association with other non-neuroendocrine tumors as seen in present case; has not been well documented in literature.

Case presentation

43 y/o female with PMH of GERD, obesity, asthma and anxiety disorder who came to endocrine clinic for left middle thyroid lobe nodule(0.9×0.6×0.6cm) evaluation; incidentally noted to have a multilobulated heterogeneous hypo echoic and iso-echoic mildly vascularized solid nodular mass (3.46×1.83×1.93 cm) in the left carotid bifurcation area; splaying the common and the internal carotid arteries on US. Review of systems was positive for left sided neck pain, intermittent palpitations, headaches, anxiety, and sleep disturbances. Clinical exam was remarkable for thyromegaly and a palpable non-tender 3cm left neck mass with no cervical lymphadenopathy. Initial labs were notable for mild elevation of chromogranin A (108.4 ng/ml) with normal levels of TSH, free T3, free T4, plasma metanephrines and 24-hour urinary metanephrines. Considering patient's persistent symptoms; plasma catecholamines levels were done and were negative. MRI soft tissue neck confirmed 2.8 cm lobular mass suggestive of paraganglioma. Metastatic work up was negative. Patient was referred for genetic testing considering young age and to rule out hereditary disorders. Interim, patient presented to the ED with complain of sudden onset lumbar backache without sphincteric involvement; neurological exam was unrevealing but on MRI lumbar spine found to have a 5.7×4.8×4cm lobulated well-circumscribed homogeneous soft tissue mass suggestive of schwannoma. Cervical and thoracic spine imaging was unremarkable. MRI brain showed a 1.1cm enhancing mass in right Meckel cave suggestive of meningioma.PET-DOTATATE scan was performed that revealed left neck mass as intensely avid, Right petrous apex mass as moderately avid and lumbar mass as not avid at all, consistent with the diagnosis of paraganglionoma, meningioma and schwannoma respectively. No evidence of DOTATATE avid nodal/ distant metastatic disease was found. Initially a hereditary etiology was suspected but genetic testing including (VHL, NF1/2, SDHD, SDHB) came back negative. Case was discussed with multidisciplinary teams; Neurosurgery service recommends conservative management of both meningioma and schwannoma at the moment considering benign course. Regarding paraganglioma, as tumor is non-secretory on repeated work up; sympathetic blockade is not warranted and patient is scheduled for pre-op embolization and surgical resection by Head and Neck surgery.

Conclusion

Paraganglionomas are generally benign non-secretory tumors that usually occur with other neuroendocrine tumors both as hereditary syndromes and sporadic cases. But their association with non-neuroendocrine tumors is mere incidental or has a genetic causation; is still unclear and warrants further research.

Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.