Intrasellar malignant peripheral nerve sheath tumor (MPNST).
ABSTRACT Intracranial malignant peripheral nerve sheath tumors (MPNST) and intrasellar schwannomas are rare tumors. We describe a case of an intrasellar schwannoma with progression to a MPNST, a finding that, although very rare, extends the differential diagnosis of intrasellar lesions.
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ABSTRACT: The concomitant presence of a pituitary adenoma with a second sellar lesion in patients operated upon for pituitary adenoma is an uncommon entity. Although rare, quite a great variety of lesions have been indentified coexisting with pituitary adenomas. In fact, most combinations have been described before, but an overview with information on the frequency of combined pathologies in a large series has not been published. We present a series of eight collision sellar lesions indentified among 548 transsphenoidally resected pituitary adenomas in two Neurosurgical Departments. The histological studies confirmed a case of sarcoidosis within a non-functioning pituitary adenoma, a case of intrasellar schwannoma coexisting with growth hormone (GH) secreting adenoma, two Rathke's cleft cysts combined with pituitary adenomas, three gangliocytomas associated with GH-secreting adenomas, and a case of a double pituitary adenoma. The pertinent literature is discussed with emphasis on pathogenetic theories of dual sellar lesions. Although there is no direct evidence to confirm the pathogenetic relationship of collision sellar lesions, the number of cases presented in literature makes the theory of an incidental occurrence rather doubtful. Suggested hypotheses about a common embryonic origin or a potential interaction between pituitary adenomas and the immune system are presented.Pituitary 07/2009; 13(1):8-17. · 1.83 Impact Factor
Article: Factors of influence upon overall survival in the treatment of intracranial MPNSTs. Review of the literature and report of a case.[show abstract] [hide abstract]
ABSTRACT: Intracranial malignant peripheral nerve sheath tumors are rare entities that carry a poor prognosis. To date, there are no established therapeutic strategies for these tumors. We review the present treatment modalities and present the current therapeutic dilemmas. We perform a statistical analysis to evaluate the prognostic factors for Overall Survival of these patients. Additionally, we present our experience with a 64-year-old man with a MPNST of the left cerebellopontine angle. To our best knowledge, forty three patients with intracranial MPNSTs, including our case, have been published in the international literature. Our analysis showed gross total resection, radiotherapy and female gender to be beneficial prognostic factors of survival in the univariate analysis. Gross total resection was recognized as the only independent predictor of prolonged Overall Survival. In our case, we performed a gross total resection followed for the first time by stereotactically guided radiotherapy. Considering the results of the statistical analysis and the known advantages of the stereotaxy, we suggest aggressive surgery followed by stereotactically guided radiotherapy as therapy of choice.Radiation Oncology 01/2010; 5:114. · 2.32 Impact Factor
Article: Recurring intracranial malignant peripheral nerve sheath tumor: case report and systematic review of the literature.[show abstract] [hide abstract]
ABSTRACT: To report the clinical presentation and management of an intracranial frontoparietal malignant peripheral nerve sheath tumor (MPNST) and its recurrence in a 6-year-old girl, along with a systematic review of the literature. A previously healthy 6-year-old girl presented with severe signs of increased intracranial pressure. Magnetic resonance imaging (MRI) revealed a large heterogeneously enhancing mass at the right frontoparietal convexity. The tumor was completely removed, the histological diagnosis was MPNST, and the patient underwent adjuvant radio- and chemotherapy. Fifteen months later, MRI revealed a small local recurrence. After upfront chemotherapy, the recurrence was removed in a radical, ie, true oncological, neuronavigationally guided, en bloc resection, including approximately 1 cm surrounding gray and white matter and overlying dura mater. Neuropathological examination revealed tumor-free margins. The patient again received adjuvant chemotherapy. Four years after diagnosis and 20 months after cessation of adjuvant therapy, there are no signs of tumor recurrence. The literature search resulted in 17 cases of intracranial MPNSTs not associated with cranial nerves. Despite macroscopically complete resection in many cases and adjuvant radio- and chemotherapy, overall survival was poor, with only 5 patients still alive upon publication (including the current patient). Intracranial MPNSTs not associated with cranial nerves are extremely rare and highly malignant tumors with poor overall survival, probably because of infiltrating growth into surrounding brain tissue. We therefore advocate consideration of nonconventional true oncological en bloc resection when approaching this rare tumor or its recurrence.Neurosurgery 01/2011; 68(4):E1152-8; discussion E1159. · 2.79 Impact Factor