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Pathology of Low-grade Adenocarcinoma of Probable Endolymphatic Sac Origin (Aggressive Papillary Tumour of the Middle Ear)

Dr Sampurna Roy MD

 

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Endolymphatic sac papillary tumour (endolymphatic sac adenoma, temporal-mastoid bone adenoma or adenocarcinoma, low-grade adenocarcinoma of potential endolymphatic sac origin, aggressive papillary tumor of the temporal bone, Heffner's tumor) is a rare lesion that involves the temporal bone.

Most of the tumours seem to have arisen in the endolymphatic sac portion of the endolymphatic system.

The tumour usually appears alone, but in a number of affected patients, it is accompanied by von Hippel-Lindau disease.

Endolymphatic sac papillary tumours are destructive lesions that exhibit locally aggressive behavior. They slowly grow into the posteromedial section of petrous temporal bone. 

The main symptoms produced by these lesions include hearing loss and cranial nerve deficits.

Light microscopy reveals two chief patterns: a follicular pattern, reminiscent of thyroid parenchyma; and a papillary/solid pattern. Both patterns are often admixed in the same tumour, and the individual tumour cells are cytologically bland. 

The tumour may show numerous periodic acid-Schiff diastase resistant reactive globules.

Immunohistochemical analysis of these tumours usually reveals positivity  for cytokeratin, vimentin, epithelial membrane antigen, and (less frequently) S-100 protein and neuron-specific enolase.

Local excision is curative for endolymphatic sac papillary tumours.

The currently favored method of treatment consists of excision and long-term follow-up. The role of adjuvant radiotherapy as treatment is controversial.

 

Further reading

Endolymphatic sac papillary tumor: a case report and review.

Endolymphatic sac tumor located around semicircular canals.

Differential grading of endolymphatic sac tumor extension by virtue of von Hippel-Lindau disease status.

Endolymphatic sac tumor (aggressive papillary tumor of middle ear and temporal bone): report of two cases with analysis of the VHL gene.

Endolymphatic sac tumor (low-grade papillary adenocarcinoma) of the temporal bone.

Hearing preservation surgery for small endolymphatic sac tumors in patients with von Hippel-Lindau syndrome.

Endolymphatic sac tumor and von Hippel-Lindau disease. Review of the literature.

Endolymphatic sac tumor: a case report

Aggressive papillary tumor of the middle ear: a true entity or an endolymphatic sac neoplasm?

Aggressive papillary tumor of middle ear/temporal bone and adnexal papillary cystadenoma. Manifestations of von Hippel-Lindau disease.

Aggressive papillary tumors of the endolymphatic sac: clinical and tissue culture characteristics.

Endolymphatic sac tumors: histopathologic confirmation, clinical characterization, and implication in von Hippel-Lindau disease.

Are papillary adenomas endolymphatic sac tumors?

Low-grade papillary adenocarcinoma of the endolymphatic sac. Report of three cases with immunohistochemical study

Differential expression of transthyretin in papillary tumors of the endolymphatic sac and choroid plexus.

Endolymphatic sac tumor: a rare tumor of internal ear. Report of two cases

Cytology of endolymphatic sac tumor.

Endolymphatic sac tumor: a rare tumor of internal ear. Report of two cases.

 

 Ear Pathology Online - Home Page

 

Ear Pathology Online -Index 

 

                                                                                    

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

 

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