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Temporal bone findings in multiple endocrine neoplasia type 2B.
1Department of Otolaryngology, Fukushima Medical University School of Medicine, Japan.
Summary
This case report details the first known temporal bone findings in multiple endocrine neoplasia type 2B (MEN-2B). Autopsy revealed metastatic medullary thyroid carcinoma (MTC) and neuromas in the temporal bone.
Area of Science:
- Oto-neurology
- Endocrinology
- Pathology
Background:
- Multiple Endocrine Neoplasia type 2B (MEN-2B) is a rare genetic disorder.
- It is characterized by medullary thyroid carcinoma (MTC), pheochromocytoma, and mucosal neuromas.
- Temporal bone involvement in MEN-2B has not been previously documented.
Observation:
- A 43-year-old Japanese male with MEN-2B presented with MTC, pheochromocytoma, and Marfanoid habitus.
- Despite surgical intervention for MTC and pheochromocytoma, recurrence occurred 14 years later, leading to death from hypertensive intracranial hemorrhage.
- Autopsy revealed widespread metastases, including in the temporal bones.
Findings:
- Histopathological examination of the temporal bones showed metastatic MTC and neuromas.
- The cochlear aqueduct was noted to be widely patent.
- Other autopsy findings included metastatic MTC in multiple organs and recurrent pheochromocytoma.
Implications:
- This report highlights the potential for temporal bone infiltration in MEN-2B.
- It underscores the importance of comprehensive histopathological examination in rare genetic syndromes.
- Further research may elucidate the specific mechanisms and clinical significance of temporal bone findings in MEN-2B.