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Are papillary adenomas endolymphatic sac tumors?
A Pollak1, A Böhmer, M Spycher
1Department of Otorhinolaryngology-Head and Neck Surgery, University Hospital, Zürich, Switzerland.
The Annals of Otology, Rhinology, and Laryngology
|August 1, 1995
Summary
Papillary adenomas in the temporal bone may not originate from the endolymphatic sac. This study suggests they arise from middle ear mucosa near the jugular bulb, challenging existing theories.
Area of Science:
- Otolaryngology
- Pathology
- Oncology
Background:
- Papillary adenomas of the temporal bone are often theorized to originate from the endolymphatic sac.
- Von Hippel-Lindau disease is a rare genetic disorder associated with various tumors.
Observation:
- A patient with von Hippel-Lindau disease presented with bilateral temporal bone papillary adenomas.
- Radiological imaging indicated tumor growth centered at the jugular bulb.
- Intraoperative findings showed tumors reaching the endolymphatic sac but not invading its lumen.
Findings:
- Tumor cells exhibited ultrastructural resemblance to normal human mastoid epithelial lining.
- Immunohistochemistry revealed keratin positivity and negative reactions for vimentin, GFAP, and S-100 protein, consistent with middle ear mucosa.
- These findings suggest the papillary adenoma originated from the mucosa of pneumatic spaces adjacent to the jugular bulb.
Implications:
- The study questions the established theory that the endolymphatic sac is the sole origin of papillary-cystic tumors in the temporal bone.
- Findings support an alternative origin from the surrounding middle ear mucosa.
- This necessitates a re-evaluation of the histogenesis of temporal bone papillary adenomas, particularly in the context of genetic syndromes like von Hippel-Lindau disease.