A rare case of endolymphatic sac tumour: clinicopathologic study and surgical management.

[von hippel-lindau disease]

Objective . Endolymphatic sac tumor (ELST ) is a rare neoplasm arising from the intrapetrous portion of the endolymphatic sac , either isolated or in association with the von Hippel-Lindau disease . We report a sporadic case of ELST with an overview of the literature and a discussion of clinic-radiological , histopathologic , and surgical findings . Case Report . A young woman presented with a progressive hearing loss in the left ear . Otoscopy showed a reddish , bleeding hypotympanic mass . CT demonstrated an expansile lytic mastoid lesion extending to the middle ear , with bone erosion . MRI confirmed a lesion of increased signal on T 1 -weighted sequences . The patient underwent a canal wall-down tympanoplasty with complete removal of the tumor . Histopathology was consistent with a papillary ELST . Immunohistochemistry was positive for cytokeratin and chromogranin A . Conclusion . This paper highlights the rarity of ELST , the need for an accurate neuroradiological and immunohistochemical study at the early stages , and the timeliness of surgical treatment .

Diseases
Validation

Diseases presenting "neoplasm" symptom

adrenal incidentaloma

alexander disease

aromatase deficiency

carcinoma of the gallbladder

cholangiocarcinoma

cowden syndrome

cushing syndrome

cutaneous mastocytosis

dedifferentiated liposarcoma

dentin dysplasia

erdheim-chester disease

esophageal adenocarcinoma

esophageal carcinoma

familial hypocalciuric hypercalcemia

focal myositis

hodgkin lymphoma, classical

hydrocephalus with stenosis of the aqueduct of sylvius

junctional epidermolysis bullosa

kabuki syndrome

liposarcoma

lymphangioleiomyomatosis

pleomorphic liposarcoma

primary effusion lymphoma

proteus syndrome

pyomyositis

von hippel-lindau disease

waldenström macroglobulinemia

well-differentiated liposarcoma

werner syndrome

wolf-hirschhorn syndrome

This symptom has already been validated