Cerebellopontine angle tumor in a patient with a maternally inherited SDHD gene mutation.
نویسندگان
چکیده
Acoustic neuromas are the most common tumor in the cerebellopontine angle (CPA) but are rare in the general population. Paragangliomas are rarer still and, in a minority of cases (20%), are known to be caused by errors in the SDHD gene. Mutations in this gene are highly penetrant when inherited paternally but not maternally. We present the first reported case of a patient with a CPA tumor and a maternally inherited SDHD gene mutation.
منابع مشابه
Primary Intracranial Malignant Nerve Sheath Tumor in the Cerebellopontine Angle in a Woman with Neurofibromatosis Type 2
Neurofibromatosis type 2 is an inherited disease. The main manifestation of the disease is the development of symmetric, non-malignant brain tumors in the region of the cranial nerve VIII usually as schwannoma. We report here a 20-year-old woman with primary intracranial malignant nerve sheath tumor located in the left cerebellopontine angle. Histologically, the tumor showed malignant spindle c...
متن کاملSecondary or Symptomatic Hemifacial Spasm caused by a Tumor of Cerebellopontine Angle Case Report and Review of the Literature
Background & Importance: Primary hemifacial spasm is usually caused by microvascular compression of the facial nerve at its root exit zone at brainstem without any space-occupying pathology. Secondary or so-called symptomatic hemifacial spasm has an additional underlying pathology e.g. tumors leading to the microvascular compression. We review and discuss the pathophysiology and the literature ...
متن کاملExophytic Cerebellar Glioblastoma in the Cerebellopontine Angle: Case Report and Review of the Literature
We report the unique case of a 69-year-old man with a cerebellar glioblastoma showing an exophytic growth pattern. Magnetic resonance imaging revealed a heterogeneously gadolinium-enhancing tumor in the right cerebellopontine angle. The preoperative differential diagnoses included an intraaxial tumor, such as high-grade glioma, and an extraaxial tumor, such as a meningioma or neurinoma. The tum...
متن کاملSurgical Outcomes of Cerebellopontine angle Tumors in 50 Cases
Introduction: To report our experience with a large series of surgical procedures for removal of cerebellopontine angle (CPA) tumors using different approaches. Materials and Methods: This was a retrospective analysis of 50 patients (mean age, 49 years) with CPA tumors (predominantly acoustic neuroma) who underwent surgical removal using appropriate techniques (principally a translabyrinthine...
متن کاملSomatic and occult germ-line mutations in SDHD, a mitochondrial complex II gene, in nonfamilial pheochromocytoma.
Most pheochromocytomas are sporadic but about 10% are though to be hereditary. Although the etiology of most inherited pheochromocytoma is well known, little is known about the etiology of the more common sporadic tumor. Recently, germ-line mutations of SDHD, a mitochondria complex II gene, were found in patients with hereditary paraganglioma. We sought to determine whether SDHD plays a role in...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- The international tinnitus journal
دوره 14 2 شماره
صفحات -
تاریخ انتشار 2008