31 year old with heairng loss&tinnitus

 

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Doctor's Information

Name : Hamidreza
Family : Haghighatkhah
Affiliation :Radiology department,ShohadaTajrish Hospital,SBMU
Academic Degree : Associate professor of Radiology
Email : This email address is being protected from spambots. You need JavaScript enabled to view it.
Resident : Leila Ahmadian Mehrgoo

 

Case Section

Head & Neck Imaging

 

Patient's Information

Gender : Female
Age : 31

 

Clinical Summary

Hearing Loss and tinnitus

 

Imaging Findings

 Axial T2-weighted, MR image acquired through the internal auqitory canals (lACs). Filling defects are seen in both lACs. The mass on the left is larger and extends out into thecerebellopontine angle. Bilateral acoustic tumors suggest neuronbromatosis type 2 . Axial Tl-weighted, three-dimensional Fourier transform, high-resolution, gradient echo MR image acquired after contrast through the lACs. Both tumors of the lACs are seen as enhancing small masses. Multiple Meningioma arising from the posterior aspect of the left temporal bone and right cerebellar hemisphere. Axial T2-weighted MR image demonstrates a rounded mass is hyperintense . axial Tl-weighted image demonstrates intense homogeneous contrast enhancement of the dural-based tumor (duraltail). Conus myxopapillary ependymoma and torasic ependymoma is seen. A, Tl-weighted sagital MR image shows a hypointense expansion of the conus., T2-weighted sagital MR image demonstrates an exophytic cystic lesion of the conus that contains a fluid-fluid level.,A postcontrast Tl-weighted sagital MR image shows the sharp rim enhancement of the tumor.,A postcontrast Tl-weighted axial section demonstrates compression of the conus by the enhancing cystic mass

 

Differential Diagnosis

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Final Diagnosis

Bilatral acoustic Schwannnoma tumor and multiple Meningioma & Ependymoma in neurofibromatosis type 2 (NF-2)

 

Discussion (Related Text)

Schwan noma is a benign (WHO grade I) encapsulated tumor that arises from the Schwann cells of the nerve sheaths of cranial and spinal nerves. The clinical presentation varies according to the site of origin Vestibular schwan noma (also called acoustic neuroma or neurinoma) typically presents with symptoms of a mass in the cerebellopontine angle, including tinnitus, sensorineural hearing loss, and facial paresthesias.Despite the tumor's origin from the vestibular nerve, symptoms and signs of vestibular dysfunction do not become manifest until relatively late. These tumors have a distinct propensity to involve the sensory nerve roots, and motor symptoms are uncommon. Multiplicity of intracranial schwannomas strongly suggests the presence of neurofibromatosis type 2 (NF-2), a congenital inherited disorder associated with a mutation on the long arm of chromosome 22.The occurrence of bilateral vestibular schwannomas (is pathognomonic MISME (multiple inherited schwannomas, meningiomas, of this disorder, which has also been termed and ependymomas). The diagnosis of vestibular schwan noma on CT rests on the demonstration of a well-circumscribed, globular or ovoid, hypodense to isodense (with respect to the adjacent pons and cerebellum) extra-axial mass in the cerebellopontine angle cistern with its base on the posterior aspect of the petrous temporal bone in the region of the internal auditory meatus. Meningiomas are the most common primary extracerebral tumors of the CNS, accounting for approximately 20% of primary brain tumors.They occur mainly in middle and old age with a peak incidence in the fifth through seventh decades of life. these tumors can, however, be found in all age groups. Meningiomas exhibit a strong sex predilection, occurring preponderantly in females, with a female-to-male ratio of at least 2: 1. However, meningiomas associated with hereditary tumor syndromes such as NF-2 generally occur in younger patients and do not demonstrate a gender predilection. Common sites of origin are in the frontal and parietal convexities and the parasagittal region (in close association with the falx cerebri); tumors in these locations constitute about 50% of all intracranial meningiomas. Meningiomas arising in relation to the sphenoid wings, olfactory grooves, sylvian fissures, and parasellar regions represent about 35%. Less than 10% arise below the level of the tentorium, mainly from the meninges overlying the clivus and petrous pyramid and the leaves and free edge of the ten torium. Multiple meningiomas are reported in 6% to 9% of cases, occurring as a component of NF_2 and less commonly sporadically. Meningioma typically has a broad base on a dural surface. The invaginating mass displaces and compresses the underlying brain and causes flattening of the adjacent cerebral cortex. Ependymomas represent 60% of spinal cord neoplasms. They are found more commonly in men and usually present between 20 and 60

 

References

Haaga 2009

 

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