Abnormal growth rate of a benign cervical sympathetic chain schwannoma





Abstract


Background


Schwannomas are rare benign tumors that arise from the Schwann cells of the nerve sheath. Schwannomas are characteristically indolent, growing approximately 1–3 mm a year. We report a case of cervical sympathetic chain schwannoma (CSCS) with a rapid rate of growth while being regularly imaged for another disease process.


Methods


A 57-year-old female with a history of papillary thyroid cancer (PTC) presented with a left level IV neck mass discovered on a routine ultrasound. The mass was not visualized on her most recent ultrasound 11 months prior.


Results


The patient underwent extracapsular enucleation of the schwannoma, which arose from the cervical sympathetic chain. Final pathology confirmed the diagnosis of a benign schwannoma.


Conclusions


This case serves as a reminder that not all benign CSCSs are indolent and also highlights the fact that the rapid rate of growth of these lesions is not necessarily indicative of a malignant transformation.


Highlights





  • Not all cervical sympathetic chain schwannomas (CSCS) are slow growing.



  • Rapid growth rate of a schwannoma is not necessarily indicative of a malignant transformation.



  • This is the first reported case of the emergence and accelerated growth rate of a benign CSCS under observation.



Introduction


Schwannomas are rare benign tumors that arise from the Schwann cells of the nerve sheath. Approximately 25–45% of all schwannomas present in the extracranial head and neck region [ ]. Cervical sympathetic chain schwannomas (CSCS) most commonly present in middle aged patients as an asymptomatic neck mass [ ]. Schwannomas are characteristically indolent, growing approximately 1–3 mm a year [ ].


Here, we report a case of CSCS with a rapid rate of growth while being regularly imaged for another disease process. This rate of growth was atypical of schwannomas and raised concerns for an underlying malignant process. Our case demonstrates that benign CSCSs can grow at an abnormally rapid pace, which should be considered while deciding upon a plan of care.


Case presentation


A 57-year-old female with a history of papillary thyroid cancer (PTC) presented with a left level IV neck mass that extended into level VI. This was identified on a routine follow-up physical exam and surveillance ultrasound. The patient’s original thyroid disease was treated at an outside institution 12 years prior with a total thyroidectomy, central compartment neck dissection, and RAI ablation (129 mCi). Final pathology showed a multifocal PTC with the largest nodule measuring 7 mm with extrathyroidal extension and no vascular invasion. The margins were involved by the tumor. Three out of five central compartment nodes were positive for metastatic disease. One year postoperatively, a PET/CT identified a suspicious lung nodule (SUV max 3.9) and hypermetabolic cervical lymph nodes (SUV max 2.8). A post-treatment whole-body scan four days after repeat RAI treatment (150 mCi) demonstrated no iodine uptake. Fine needle aspiration of the cervical lymph nodes was positive for metastatic PTC. The patient underwent a right neck dissection, and was consistently monitored by an experienced radiologist with annual neck ultrasounds and thyroglobulin levels for ten years ( Fig. 1 a). During that interval and prior to the current presentation, there was no evidence of recurrent disease and thyroglobulin levels were persistently low.




Fig. 1


A. Neck ultrasound of the left thyroid bed from December 2018 shows a mildly echogenic well-defined lesion posterior to the left common carotid artery with punctate echogenic foci measuring 9 × 10 mm which had been stable since ultrasound examinations of 2013. B. Neck ultrasound from November 2019 shows a well-defined lobulated hypoechoic mass measuring 6.0 × 2.4 × 2.1 cm. The lesion is lateral to the left common carotid artery.

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Jul 5, 2021 | Posted by in OTOLARYNGOLOGY | Comments Off on Abnormal growth rate of a benign cervical sympathetic chain schwannoma
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