A Rare Presentation of Primary Cutaneous Low-Grade Neuroendocrine Tumor of the External Auditory Canal.

OBJECTIVE

To describe the first case of a primary cutaneous low-grade neuroendocrine tumor (cLGNET) originating from the external auditory canal as well as our team's surgical management.

PATIENT: A healthy 34-year-old female presented with a low-grade neuroendocrine tumor of her right external auditory canal (EAC) which extended from the posterior-superior aspect of the EAC into the middle ear.

INTERVENTION: A complete otological examination was performed in addition to CT and MRI imaging. The low-grade neuroendocrine tumor was surgically biopsied and further surgery was recommended for complete resection.

RESULTS

Audiogram revealed profound right sensorineural hearing loss. CT scan demonstrated complete opacification of the right EAC, middle ear, and mastoid air cells, dystrophic calcification in the mesotympanum overlying the cochlear promontory, and no associated osseous erosion. MRI revealed abnormal FLAIR hyperintensity and enhancement of the labyrinthine segment of the right facial nerve, cochlea, and horizontal and posterior semicircular canals. An enhancing mass opacifying the right EAC demonstrating restricted diffusion on diffusion-weighted image was also evident. Pathologic examination and immunohistochemical staining confirmed a diagnosis of primary cLGNET of the EAC.

CONCLUSION

Primary cLGNETs of the external ear are exceedingly rare but should be considered if an adult patient presents with a mass in the EAC. Management should include early biopsy and surgical excision followed by histological and immunohistochemical confirmation.