Transcanal endoscopic assisted skull base endolymphatic sac tumor resection: A rare disease with advanced technology

Endolymphatic sac tumors(ELSTs) are rare, papillary adenomatous tumors that arise from the endothelium of the endolymphatic sac. We demonstrate a difficult case of endolymphatic sac tumor and how it is managed via transcanal endoscopic assisted technique, with discussion of feasibility of transcanal approach to lateral skull base tumor.

MEFV,IRF8,ADA,PEPD,and NBAS gene variants and elevated serum cytokines in a patient with unilateral sporadic Meniere's disease and vascular congestion over the endolymphatic sac

The etiology and underlying mechanism of Meniere's disease(MD)development are still unknown,although inflammation and autoimmunity have been implicated as underlying mechanisms.The human endolymphatic sac(ES)has been reported to have innate and adaptive immune capacity in local immune reactions.In vivo demonstration of inflammation of the ES in patients with MD is missing in the literature.We report the case of a 47-year-old female patient diagnosed with unilateral MD with genetic variants and cytokine markers indicating inflammation and vascular congestion of the ES.Endolymphatic hydrops in the right cochlea(grade 2)and vestibulum(grade 3)were detected using MRI.She carried heterozygous variants in MEFV(c.442G>C),IRF8(c.1157G>T),ADA(c.445C>T),PEPD(c.151G>A),NBAS(c.4049T>C),CSF2RB(c.2222C>T),HPS6(c.277G>T),IL2RB(c.1109C>T),IL12RB1(c.1384G>T),IL17RC(c.260_271del GCAAGAGC TGGG),LIG1(c.746G>A),RAG1(c.650C>A),and SLX4(c.1258G>C,c.5072A>G).In the serum,the levels of granulocyte colony-stimulating factor(G-CSF),macrophage inflammatory protein 1a,and IL7 were significantly elevated,and the level of IL2Ra was reduced.Intratympanic administration of dexamethasone temporarily alleviated her hearing loss.Her vertigo was significantly relieved but remained slight after ES administration of corticosteroids.

Cell proliferation in the endolymphatic sac in situ during the immune response of inner ear

Background Normally, few immunocompetent cell are present in the endolymphatic sac (ES). During an active immune response in the inner ear, large amount of inflammatory cells, including immunocompetent cells, are seen in the ES. The current study aimed at assessing cellular proliferation within the ES during induced immune response in the inner ear. Methods Fifteen healthy, female SD rats were sensitized systemically with keyhole limpet hemocyanin (KLH), followed by local inoculation in the cochlea through basal turn fenestration with the same antigen. On Days 3, 7 and 14 following inoculation, the animal was sacrificed after intraperitoneal administration of 5-bromo-2’-deoxyuridine (BrdUrd), and the temporal bone harvested. Following decalcification, infiltration by BrdUrd- and IgG-positive cells in the ES was studied on frozen sections with H & E and immunohistochemical staining. Results During the secondary immune response in the inner ear against T-dependent antigens, there is increased cellular proliferation in the ES. The proliferated cells may differentiate into immunocompetent cells at the same location. Conclusions These findings indicate that the ES plays an important role in immune response of inner ear.

Management of endolymphatic sac tumors:a case series report

Objective To study clinical characteristics of endolymphatic sac tumor (ELST) and its diagnosis and treatment. Methods ELST was diagnosed in 6 cases based on surgical and histological findings. These cases were reviewed for their clinical manifestations, differential diagnosis and surgical treatment techniques. Results There were 1 male and 5 females in this group, aged from 28 to 59 years (mean age=38.7 years). The tumor was in left ear in 4 cases and in right ear in the other 2 cases. Disease courses ranged from 5 to 30 years (mean duration=12.6 years). Clinical presentations included sensorineural hearing loss (n=4), otorrhea and tinnitus (n=2), tinnitus and facial spasm(n=1), otorrhea with facial paralysis(n=1), and hearing loss with tinnitus (n=2). None of the cases was diagnosed as ELST preoperatively. Two cases were misdiagnosed as glomus jugulare tumor, 2 as chronic suppurative otitis media, 1 as sweat gland adenoma on biopsy and 1 as temporal bone tumor. Tumors were surgically resected in all 6 cases via the mastoidectomy (n=2) or combined oto-cervical or cranio-oto-cervical approaches(n=4). Postoperative cerebrospinal fluid otorrhea occurred in 1 case. The tumors were confirmed on histological examination to be a low -grade adenocarcinoma. All patients have survived at the time of this paper. Conclusion ELST is rare and commonly misdiagnosed and inadequately treated. Its prognosis is relatively favorable because of its slow growth rate.

Endolymphatic sac tumor: case report and literature review

Background:Endolymphatic sac tumor(ELST)is one of neuroectodermal tumor which arising from endolymphatic sac and duct.It is actually quite rare,with less than 200 cases reported.Although ELST presents benign appearance in histopathology,it can present aggressive destructive behavior in clinical.The cornerstone of treatment for ELST is complete surgical excision.However,it is almost impossible to completely resect the advanced stage tumor.There is still controversy about other treatments,such as radiotherapy and gamma knife surgery.Case presentation:A 47-year-old man was admitted in The First Affiliated Hospital of Fujian Medical University with a 7-year history of progressive hearing loss and near 6-month repeated attacks of headache.Preoperative CT revealed a massive intracranial lesion and associated hydrocephalus.MR scanning demonstrated a 7.2 cm×4.6 cm×4.2 cm bulky mass located in left-sided posterior cranial fossa and temporo-occipital region which showed hyperintensity on T1-weighted images and mixed signal intensity on T2-weighted images.There was no neither clinical manifestation nor family history of Von Hippel–Lindau syndrome(VHL).Due to the mass that was large and invading the bone of skull base,it was difficult to extirpate surgically,so the ventriculoperitoneal shunt combined with local biopsy was performed.The postoperative pathology and immunohistochemical findings confirmed the lesion was an endolymphatic sac tumor.After operation,the patient regularly received radiotherapy.Conclusion:The widely accepted management of ELST is complete surgical resection.However,it is difficult for surgeons to achieve radical resection with late-stage ELST.Currently,there is much dispute about the role of radiotherapy for the management of ELST in academic circles.In this case where the mass cannot be surgical removed,radiotherapy has the curative effect for ELST in terms of disease control and quality of life.

Endolymphatic sac papillary tumor： a case report

Endolymphatic sac papillary tumor （ELST） is an extremely rare and aggressive tumor characterized by hearing loss and temporal bone destruction. A case with clinical, imaging, pathologic and treatment data is reported and relevant literature is reviewed. A 25-year-old woman, with ELST underwent craniotomy for tumor subtotal resection, and the diagnosis was confirmed by pathologic examination. Postoperative radiotherapy consisted of 50.4 Gy/28 f was given accordingly. The patient is currently alive with no signs of tumor recurrence locally and no radiation side-effects observed after one year follow-up. Complete resection is impossible in most cases, local resection, adjuvant radiotherapy may provide favored local control. A long-term follow-up is highly advocated in consideration of its slow development course.