Endolymphatic sac tumor with intralabyrinthine hemorrhage: a case report / 临床耳鼻咽喉头颈外科杂志

A 27-year-old female patient suffering endolymphatic sac tumor with intralabyrinthine hemorrhage was reported. The patient had hearing loss in the left ear with continuous tinnitus, and MRI showed the soft tissue shadow of endolymphatic sac. Considering that the tumor involved semicircular canal and vestibule,endolymphatic cyst tumor resection was performed by labyrinth route. After surgery, there was no cerebrospinal fluid leakage and facial nerve function was normal. More importantly, enhanced MRI of temporal bone showed no tumor recurrence 1 year after surgery.

Endolymphatic Sac Tumour: A Case Report and Review of the Literature

Introduction Endolymphatic sac tumor (ELST) is a slow-growing, low-grade, locallyinfiltrative tumor arising from the endolymphatic sac/duct, which is located in the posterior part of the petrous temporal bone. It may be sporadic in origin, or may be associated with Von-Hippel Lindau (VHL) syndrome. Case description A 40-year-old female patient with an ELST without VHL syndrome who was treated successfully by microsurgical extirpation of the tumor. Discussion We discuss the radiological features and the histopathology of this rare tumor and review the relevant literature. Conclusion The case herein reported adds to the previously-reported cases of this rare tumor.

Hemangioma of Endolymphatic Sac in Von Hippel-Lindau Patient / 대한이비인후과학회지

The present study reports a rare case of capillary hemangioma of endolymphatic sac. A 23-year-old male who underwent von Hippel-Lindau disease presented with recurrent sudden sensorineural hearing loss. Magnetic resonance imaging revealed a heterogenous enhanced mass in the right endolymphatic sac, which was hyperintense on the enhanced T1-weighted images and inhomogenous on the T2-weighted images. Pre-operatively, this tumor was believed to be an endolymphatic sac tumor because of the history of von Hippel-Lindau disease. During the surgery, vascular tumor was removed by transmastoid approach. A histopathological examination indicated that the tumor was a capillary hemangioma. To the best of our knowledge, the present study is the second case of hemangioma in the endolymphatic sac and first case of von Hippel-Lindau disease.

A Case of Endolymphatic Sac Tumor Removal with Modified Translabyrinthine Approach / 대한이비인후과학회지

Endolymphatic sac tumor (ELST) is a very rare tumor arising from the epithelium of the endolymphatic sac. Pathologically, ELST shows papillary growth patterns with extensive bony and soft tissue destruction. ELST commonly shows symptoms of endolymphatic hydrops such as hearing loss, vertigo and tinnitus by blocking the endolymphatic duct. As the tumor enlarges, patients experience various symptoms that include multiple cranial nerve palsy. Ten to thirty percents of ELST are related to Von Hippel Lindau (VHL) diseases. Also, bilateral tumors can develop when a patient has VHL-related ELST. The goal of treatment for ELST is total tumor eradication. The type of surgical approach is chosen by the surgeon in consideration of the tumor grade and the hearing status of the patient. In this report, we present a patient with ELST who was successfully treated with the modified translabyrinthine approach. We have also reviewed relevant literatures.

Endolymphatic Sac Tumors : Report of Four Cases

Endolymphatic sac tumor is rare, locally aggressive hypervascular tumor of papillary structure, arising from the endolymphatic duct or sac in the posterior petrous bone. We present four cases with this tumor. Two patients were male and the other two were female. Age of each patient was 15, 52, 58, and 67 years. Three patients presented with progressive hearing loss and sustained vertigo for months to years and another one was referred for the tumor detected in routine medical check-up. Preoperative embolization was performed in 3 patients. Complete excision of the tumor was achieved in all patients using translabyrinthine or retrosigmoid approach. Herein, we describe the clinical and radiographic features, surgical treatment and pathologic findings with a review of the literature.

A Case of Endolymphatic Sac Tumor / 대한이비인후과학회지

Endolymphatic sac tumor (ELST) is very rare but its aggressive papillary neoplasm destructs the temporal bone widely and often involves the intracranial structures. The most effective treatment is complete surgical removal, but it is very difficult because the character of tumor is very aggressive and extensive to the intracranial region. This tumor is not yet reported in Korea but we have recently experienced one case of ELST which we treated surgically by the traslabyrinthine/petrosal combined approach. We report the clinical and histological aspects of this rare tumor with a review of the literatures.

Clinicopathological and imaging feature of endolymphatic sac tumor / 解放军医学杂志

Objective To study the clinicopathological and imaging features,key points of diagnosis and prognosis of endolymphatic sac tumor.Methods Seven cases of endolymphatic sac tumor were studied with routine histopathological,immunohisochemical staining and CT,MRI scanning.Patients' prognosis and life quality were aesessed by follow-up.Results The clinical symptoms of patients were progressive sensorineural deafness,tinnitus,vertigo and affection of cranial nerves.CT revealed reticular of moth-eaten bony destruction of the petrosal region,and MRI revealed a region of high-signal intensity void of vascular flowing signals.The histopathologic examination of the tumor presented a papillary-cystic pattern.The tumor cells were round or cubaid in shapen,with nuclei in similar shape and size.There were cyst-like structares filled mith pinkish gelatinous.Tumor cells were roundor cuboid in shape,and some of them contained clear cytofrlasm.There was materials,which were stained strongly with PAS.Hemorrhage,cholesterol crystal and foreign body giant cells were found in the tumor stroma with abundant small blood vessels and extravasation of blood,cells with intracellular hematin,and giant cells collagen.There was also proliferation of fibrous connective tissues.Necrosis and bone destruction were also found in some cases.Immunohistochemical results showed that CK and NSE were positive in all the tumors,and CD56 and S-100 were positive in part of cases,GFAP,EMA,CgA,TG and Syn were negative in all the specimens.One patient had received radiotherapy after partial resection.Relapse occurred only in one patient two years after the first surgery,and no metastasis or relapse occurred in the rest of patients.Conclusions Endolymphatic sac tumor is a rare neoplasm.The diagnosis relies mainly on histopathological and immunohisochemical findings.The patients often have good prognosis.