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Recurrent Primary Intrasellar Paraganglioma.

Elizabeth A Schueth1, Daniel C Martinez2, Charles G Kulwin3

  • 1Indiana University School of Medicine, 1120 W. Michigan Street, Suite 200, Indianapolis, IN 46202, USA.

Case Reports in Otolaryngology
|July 21, 2020
PubMed
Summary
This summary is machine-generated.

This case report details a rare sellar region paraganglioma (PGL) in an elderly male. Surgical intervention effectively treated the recurrent tumor, highlighting the importance of early diagnosis and resection.

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Area of Science:

  • Neuro-oncology
  • Endocrinology
  • Ophthalmology

Background:

  • Paragangliomas (PGLs) are rare neuroendocrine tumors originating from extra-adrenal chromaffin cells.
  • Sellar and suprasellar paragangliomas are exceptionally rare, with fewer than 20 cases reported.
  • These tumors can cause significant visual disturbances and hormonal imbalances due to their location.

Observation:

  • An 81-year-old male presented with bitemporal visual field defects and decreased visual acuity.
  • Imaging revealed a recurrent paraganglioma in the sellar/suprasellar region.
  • Histopathology confirmed the diagnosis with characteristic zellballen morphology and immunohistochemical markers (chromogranin, synaptophysin, S-100).

Findings:

  • The patient underwent a repeat transsphenoidal endoscopic resection for the recurrent sellar paraganglioma.
  • Immunohistochemistry confirmed the tumor as a paraganglioma.
  • Surgical debulking was effective in managing the compressive effects of the tumor.

Implications:

  • Early diagnosis and surgical resection are crucial for managing locally aggressive sellar paragangliomas.
  • Surgical intervention is effective in treating recurrent sellar PGLs and preventing morbidity.
  • Further research is needed to establish optimal adjuvant therapies, such as radiation, for sellar paragangliomas.