Significantly Elevated FDG Activity in Neurothekeoma.

ABSTRACT: Neurothekeoma is a very rare cutaneous lesion affecting mainly young patients. The lesion is generally benign. We report here that intense FDG activity on PET/CT was noted at the site of primary neurothekeoma of the right shoulder in a 16-year-old boy. Abnormal FDG activity was also seen in the metastatic neurothekeoma in the lung in the same patient 2 years later.

Nerve Sheath Myxoma in the Lower Extremity: A Rare Case with Description of Magnetic Resonance Imaging and Sonographic Findings.

BACKGROUND This report is of a nerve sheath myxoma presenting as a slow-growing mass in the back of the left ankle of a 36-year-old man that was investigated by ultrasound and magnetic resonance imaging (MRI) before the diagnosis was confirmed by histopathology. CASE REPORT We report a nerve sheath myxoma of the ankle in a 36-year-old man. The palpable abnormality was falsely assumed to be a ganglion cyst prior to advanced imaging. Magnetic resonance imaging demonstrated a lobular mass with high T2 and intermediate T1 signal as well as moderate enhancement. T2 sequences also demonstrated distinctive internal septae. These internal septae were also noted on sonographic evaluation prior to biopsy. The patient was treated with surgical excision, and pathologic analysis showed myxoid nodules with loose arrangements of spindled cells separated by fibrous septae. S-100 protein and glial fibrillary acidic protein positivity by immunohistochemistry staining was demonstrated. Follow-up imaging at 12 months showed no evidence of tumor recurrence. CONCLUSIONS This case highlights that while nerve sheath myxomas are rare tumors, they should be considered in cases of cutaneous soft-tissue masses with myxoid imaging features. Ultrasound and magnetic resonance imaging features of thin internal septae may be present and correspond well with the unique histopathological characteristics of these lesions. This report shows the importance of imaging of peripheral soft-tissue masses, including ultrasound and MRI, which can identify localized and benign features and the solid, cystic, and myxoid areas, which were characteristic in this case of benign nerve sheath myxoma.

A rare localization of neurothekeomas of radial nerve: A case report.

Neurothekeoma is a very rare benign connective tissue tumour that presumably derived from nerve sheath cells. We described the case of a rare localization of neurothekeoma in the upper limb with a strange presentation. A 49 years-old woman presented to the Physical Medicine and Rehabilitation Division of the Umberto I Hospital referring an intensive pain associated to paresthesias at the left forearm lasting from six months. The patient had a history of epicondylitis confirmed with an elbow RMN showing an increased thickness of the tendon insertions on the epicondiloidea region of the elbow. Rehabilitative and physical therapy has been done without symptoms remission. An ultrasound evaluation showed an oval formation well circumscribed in the context of the radial nerve. It was easy to demonstrate the relevance of the radial nerve, following it from the arch of Frohse until the humeral sulcus of the radial nerve. A MRI that showed a mass, mildly hypointense on T1- weighted sequences and hyperintense on T2-weighted images, with nonhomogeneous enhancement post-contrast, attributable to expansionary pathology of the radial nerve. A biopsy was done and the lesion was described as a benign tumor of nerve sheath, i.e., a Neurothekeoma of the radial nerve. Patients was surgically treated, the tumor has been removed and she referred the resolution of symptomatology.

Nerve sheath myxoma (neurothekeoma) of cerebellopontine angle: case report of a rare tumor with brief review of literature.

Nerve sheath myxoma (neurothekeoma) are rare benign nerve sheath tumors, usually arising in the skin of the head and neck region and upper extremities in young females. To the best of author's knowledge only two cases of intracranial neurothekeoma have been published in the English literature. These tumors were located in the parasellar area and in middle cranial fossa. This is the first case report of cerebellopontine angle neurothekeoma and third case report of intracranial neurothekeoma. This patient, a 45-year-old female, presented to us with complaints of right side progressive hearing loss for 12 months and swaying during walking for 8 months. Histologically the tumor had lobular appearance with spindle or stellate cells embedded in abundant myxoid background. The tumor cells were diffusely positive for S100. The patient was symptom free at eight month follow up.

[Neurothekeoma of the orbit]. / Przypadek neurothekeoma oczodolu.

AIM: The aim of this study was to present a rare benign tumour of neural origin located in the orbit. MATERIAL AND METHODS: Authors describe case of six-year-old boy with tumour of the right orbit, upper eyelid and right zygomatic fossa. Medical history revealed gradually increasing in size, painless tumour. Before admission to the hospital computed tomography was taken to assess the size, localization of the tumour and to plan the proper treatment. RESULTS: The patient was operated on using Krönlein approach. Tumour was removed in macroscopically radical limits without need to incise the muscles responsible for eye movement. Histopathological examination revealed: Neurothekeoma, classic type. During one year follow-up there was no recurrence. CONCLUSION: Neurothekeoma may occur in young boys in eyelid and orbit. A unique localization of neurothekeoma in the orbit, close to the lacrimal gland shows that this kind of histopatological tumours may infiltrate deeper tissues. Surgical treatment of neurothekeoma is the same as in other benign tumours.

Cellular neurothekeoma of the maxilla.

Neurothekeomas are uncommon benign soft tissue tumors of nerve sheath origin. They occur predominately in the head and neck or upper trunk of children and young adults. A 15-month-old boy presented with an enlarging mass of the right maxilla. Radiologic imaging demonstrated an expansile lesion of the nasomalar region. An incisional biopsy resulted in the diagnosis of neurothekeoma. This lesion should be considered as part of the differential diagnosis of pediatric soft tissue head and neck masses. We discuss the presentation, evaluation, and treatment of these rare benign lesions.

Right ventricular nerve sheath tumour and patent foramen ovale presenting with transient ischemic attack.

A 66-year-old man presented with dyspnea and a transient ischemic attack. Echocardiography revealed a right ventricular mass and patent foramen ovale. During surgery the mass was found to be a malignant nerve sheath tumour. Several weeks later, the patient developed small bowel obstruction, and laparotomy disclosed multiple metastases involving the small and large bowel. The paper describes the clinical course and management of this patient and reviews the pertinent literature.