Sporadic primary malignant intracerebral nerve sheath tumors: case report and literature review

Abstract

We report the case of a seventy-five year old woman with a sporadic primary malignant intracerebral nerve sheath tumor (MINST). These tumors fall within the spectrum of malignant peripheral nerve sheath tumors (MPNST) but confirming the diagnosis of a MINST can be difficult due to its rarity and unusual intraparenchymal location. Radiographically, MINST's mimic malignant glioma and should be considered in the differential diagnosis of enhancing cerebral lesions. In this report, we present a comprehensive panel of histological, immunohistochemical, ultrastructural, and genetic considerations that may be used to diagnosis MINST based on their similarities to MPNSTs and brain parenchyma location.

Supratentorial Intracerebral Schwannoma : Its Fate and Proper Management

“…Excluding 9 malignant cases1,10,12), we found 59 published case reports and series in the English-language literature. These tumors were reported within various age groups, but most commonly are reported in pediatric and young adult populations.…”

Malignant Intracerebral Nerve Sheath Tumors: A Case Report with Review of the Literature

“…A comprehensive histopathological analysis is warranted and includes detailed observation of cellular structure, immunostaining, and electron microscopy. Fortunately, as suggested by Barnard et al [9], recognizing the similarity of MINST with the more common MPNST can help guide diagnosis and treatment. MPNST generally have poor outcomes because of their invasive nature and ability to metastasize to various organs [1].…”

“…For almost all cases, surgery with the goal of GTR was first-line treatment. As with MPNST, achieving complete resection can improve long-term outcomes [1, 9, 20]. This can be complemented with radiotherapy and/or chemotherapy, but their effect on prognosis is inconclusive.…”

“…The majority of MINST cases are sporadic and have afflicted patients as young as 1 year and as old as 75 years. Not surprisingly, the diagnosis of these tumors may be problematic, and the obscurity of their cellular origin further complicates matters [2, 3, 9]. Treatment almost always involves surgery.…”

Imaging Diagnosis—malignant Peripheral Nerve Sheath Tumor Presenting as an Intra‐axial Brain Mass in a Young Dog

“…The origin of intracranial malignant peripheral nerve sheath tumors not associated with the 12 cranial nerves remains controversial. Developmental hypotheses for the histiogenesis have included displaced neural crest cells and abnormal differentiation of multipotential mesenchymal elements . Nondevelopmental theories consider the cell of origin for these tumors to be the Schwann and other mesenchymal cells present in the meningeal branches of trigeminal nerves .…”