Primary myxoid temporal bone tumor: A rare neurosurgical manifestation of Carney complex?

Background: Carney complex (CNC) is a rare autosomal dominant syndrome, manifesting mainly with cardiac, cutaneous, and mucosal myxomas. Osteochondromyxoma is known as an extremely rare bone lesion of CNC which usually appears early in life; however, there were no reports of primary bone myxoma of the skull in the patients with CNC. We present the first case of primary myxoid skull tumor in the patient with CNC. Case Description: We report the left temporal bone tumor with significant intracranial mass effect in a 58-year- old woman already diagnosed with CNC. Complete resection of the tumor with skull bone reconstruction was carried out. Pathological diagnosis was labeled the lesion as an atypical myxoid spindle cell neoplasm. The features were different from atrial myxoma and osteochondromyxoma which has been described in CNC. There have been no signs of recurrence in 9 years follow-up. Conclusion: To the best of our knowledge, there have been no reports of the primary myxoid tumors in the skull in the patients with CNC. This paper highlighted a possible important association between CNC and primary intracranial myxoid tumors.

CT and MRI imaging of glomus tumors of the temporal bone

Glomus tumor is one of the most common temporal bone tumors. Most of them are benign and locally invasive, some are occasionally able to metastasize and have signs of malignancy. Diagnostic imaging is necessary before treatment. Computer tomography (CT) is traditionally used as a primary method of diagnosis, to recognize changes in the temporal bone. Role of magnetic resonance imaging (MRI) in temporal bone tumor diagnosis is not definitively determined.Purpose. To assess the possibilities of computer and magnetic resonance tomography, to develop an algorithm for the application of diagnostic imaging methods in the diagnosis of glomus tumors of the temporal bone.Material and methods. The article presents the experience of diagnosing 30 patients with glomus tumors.Results. The tympanic form of the glomus tumor was observed in 11 cases (37%), tympano-yugular in 19 cases (63%). CT and MRI data totally coincided in cases of small tumors (type A and B). In the presence of extended forms CT ability of assessing bone invasion, involvement of the internal carotid artery, internal jugular vein, and dural sinuses was lower than the MRI.

Endolymphaticsac tumor – case report

The aim of this paper is to present the case of a 70-year-old women with endolymphatic sac tumor and temporal bone destruction treated at Otolaryngology Department of MUG. The patient was admitted to our Department due to a 3-year history of hearing loss, dizziness and ear pain. The first diagnosis was temporal bone tumor connected with von Hippel-Lindau syndrome (VHL). The patient was surgically treated. During intraoperative examination, a neoplasm was determined. The tumor was excised via transmastoid approach with sigmoid sinus skeletonization. After treatment, her pains disappeared. Histopathological and immunohistochemical examination revealed endolymphatic sac tumor. Follow-up CT showed no tumor remission.

Extradural neurenteric cyst of the cerebellopontine angle

✓ Neurenteric cyst is a developmental malformation found mainly in the spinal canal. The authors report on a 47-year-old man with a neurenteric cyst of the cerebellopontine angle (CPA) who presented with progressive hearing disturbance and facial palsy. The tumor was located extradurally with marked destruction of the petrous bone around the internal auditory canal and demonstrated irregular and heterogeneous high-intensity signals on T1- and T2-weighted on MR images, which is atypical for neurenteric cysts. The pathological findings in samples obtained after resection disclosed a single epithelial layer (a feature of neurenteric cyst), which was accompanied by marked xanthogranulomatous changes. Although several neurenteric cysts have been reported in the CPA, extradural lesions with unusual imaging features and marked bone destruction have not been reported previously. This benign developmental lesion should be considered, although it is extremely rare, in patients harboring an extradural temporal bone tumor around the CPA.

Middle ear adenocarcinoma with intracranial extension

✓ Middle ear adenocarcinoma is a very rare, locally invasive neoplasm assumed to arise from the middle ear mucosa. Although endolymphatic sac tumor (aggressive papillary middle ear tumor) and jugulotympanic paraganglioma may show brain invasion, intracranial extension of histologically confirmed middle ear adenocarcinoma has not been previously reported. The authors describe a 53-year-old man who suffered from otalgia and tinnitus for more than 10 years and from neurological deficits for 1 year due to a large temporal bone tumor that invaded the temporal lobe. A combined neurosurgical and otolaryngological resection was performed. Pathological analysis revealed a low-grade adenocarcinoma of a mixed epithelial—neuroendocrine phenotype, which showed a close histological similarity to, and topographical relationship with, middle ear epithelium. The authors conclude that middle ear adenocarcinoma belongs to the spectrum of extracranial tumors that have possible local extension to the brain.

Papillary adenoma of endolymphatic sac origin: a temporal bone tumor in von Hippel—Lindau disease

✓ This report describes a patient with von Hippel—Lindau disease who presented with an 8-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by computerized tomography scanning and magnetic resonance imaging techniques, was partly cystic in appearance. After removal of the mass, pathological studies confirmed a papillary cystic tumor with characteristics that have been described in tumors with an endolymphatic sac origin. These rare neoplasms constitute a distinct pathological entity and deserve wider recognition.