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<p>Neurofibromas arising in the carotid space are extremely uncommon and often pose a diagnostic challenge due to their deep-seated location, overlap with schwannomas and close relationship with major neurovascular structures. Pre-operative identification is further complicated because most cervical nerve sheath tumors encountered radiologically are schwannomas rather than neurofibromas. A 21-year-old female presented with a left upper cervical swelling and dysphagia. There was no tonsillar enlargement, but rather a smooth left anterior pillar bulge. Cross-sectional imaging (contrast-enhanced CT and MRI) demonstrated a well-defined carotid-space lesion showing homogeneous enhancement, vessel displacement without encasement and heterogeneous T2 signal. Based on radiological characteristics, a vagal schwannoma was suggested in the differential diagnosis, along with carotid body tumor and paraganglioma. CT angiography confirmed maintained cervical and cerebral arterial flow. The mass was excised via a transcervical approach. Intra-operatively, the lesion was noted to be arising from the cervical sympathetic chain, rather than the vagus nerve as initially suspected. Histopathology revealed an unencapsulated spindle-cell neoplasm composed of interlacing fascicles of wavy spindle cells with entrapped nerve fibers. Immunohistochemistry showed diffuse S-100 positivity, confirming a solitary neurofibroma. Carotid-space neurofibromas are rare but should be included in the differential diagnosis of deep cervical masses causing vessel displacement with preserved fat planes. Radiology may closely mimic vagal schwannoma, highlighting the importance of multimodality imaging correlation with operative findings and histopathological confirmation. Awareness of this entity facilitates appropriate surgical planning and reduces the risk of intra-operative neurovascular complications.</p>

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Cervical Sympathetic Chain Neurofibroma with Skull Base Extension: A Rare Case with Diagnostic and Surgical Challenge

  • 期刊论文

  • 发表日期：

    2026年7月27日

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  • Meghana Matta Raja,
  • Chekuri Charmila,
  • Manu S. Babu,
  • G. D. Mahajan,
  • Aditi S. Moruskar,
  • Komal D. Sawaimul

摘要

Neurofibromas arising in the carotid space are extremely uncommon and often pose a diagnostic challenge due to their deep-seated location, overlap with schwannomas and close relationship with major neurovascular structures. Pre-operative identification is further complicated because most cervical nerve sheath tumors encountered radiologically are schwannomas rather than neurofibromas. A 21-year-old female presented with a left upper cervical swelling and dysphagia. There was no tonsillar enlargement, but rather a smooth left anterior pillar bulge. Cross-sectional imaging (contrast-enhanced CT and MRI) demonstrated a well-defined carotid-space lesion showing homogeneous enhancement, vessel displacement without encasement and heterogeneous T2 signal. Based on radiological characteristics, a vagal schwannoma was suggested in the differential diagnosis, along with carotid body tumor and paraganglioma. CT angiography confirmed maintained cervical and cerebral arterial flow. The mass was excised via a transcervical approach. Intra-operatively, the lesion was noted to be arising from the cervical sympathetic chain, rather than the vagus nerve as initially suspected. Histopathology revealed an unencapsulated spindle-cell neoplasm composed of interlacing fascicles of wavy spindle cells with entrapped nerve fibers. Immunohistochemistry showed diffuse S-100 positivity, confirming a solitary neurofibroma. Carotid-space neurofibromas are rare but should be included in the differential diagnosis of deep cervical masses causing vessel displacement with preserved fat planes. Radiology may closely mimic vagal schwannoma, highlighting the importance of multimodality imaging correlation with operative findings and histopathological confirmation. Awareness of this entity facilitates appropriate surgical planning and reduces the risk of intra-operative neurovascular complications.