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Open access

Brain metastasis and intracranial leptomeningeal metastasis from malignant peripheral nerve sheath tumors: illustrative cases

Masasuke Ohno, Shoichi Haimoto, Satoshi Tsukushi, Waki Hosoda, Fumiharu Ohka, and Ryuta Saito

BACKGROUND

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft-tissue tumors. Intracranial metastasis from MPNSTs is quite rare.

OBSERVATIONS

The authors report on a 73-year-old male whose MPNST metastasized to the brain and a 32-year-old male with leptomeningeal metastasis from MPNST and review 41 cases of MPNST that developed intracranial metastasis, as reported in the literature.

LESSONS

Brain metastasis and leptomeningeal metastasis of MPNSTs show different clinical courses and require pathology-specific treatment.

Open access

Melanoma metastasis to the femoral nerve: a novel case of melanoma masquerading as mononeuropathy. Illustrative case

Jaime R. Guerrero, Khaled M. Taghlabi, Sara A. Meyer, Lokeshwar S. Bhenderu, Saeed S. Sadrameli, Clive I. Shkedy, Amir H. Faraji, and Robert C. Rostomily

BACKGROUND

Metastatic cancer may involve the central and peripheral nervous system, usually in the late stages of disease. At this point, most patients have been diagnosed and treated for widespread systemic disease. Rarely is the involvement of the peripheral nervous system the presenting manifestation of malignancy. One reason for this is a proposed “blood-nerve barrier” that renders the nerve sheath a relatively privileged site for metastases.

OBSERVATIONS

The authors presented a novel case of metastatic melanoma presenting as intractable leg pain and numbness. Further workup revealed concurrent disease in the brain and breast, prompting urgent treatment with radiation and targeted immunotherapy.

LESSONS

This case highlights the rare presentation of metastatic melanoma as a mononeuropathy. Although neurological complications of metastases tend to occur in later stages of disease after initial diagnosis and treatment, one must remember to consider malignancy in the initial differential diagnosis of mononeuropathy.

Open access

Malignant intracerebral nerve sheath tumor in a patient with Noonan syndrome: illustrative case

Callum M. Allison, Syed Shumon, Abhijit Joshi, Annelies Quaegebeur, Georges Sinclair, and Surash Surash

BACKGROUND

Malignant peripheral nerve sheath tumors (MPNSTs) within the neuroaxis are rare, usually arising from peripheral and cranial nerves. Even more scarce are cranial subclassifications of MPNSTs termed “malignant intracerebral nerve sheath tumors” (MINSTs). These tumors are aggressive, with a strong tendency for metastasis. With this presentation, alongside resistance to adjunctive therapy, complete excision is the mainstay of treatment, although it is often insufficient, resulting in a high rate of mortality.

OBSERVATIONS

The authors report the case of an adult patient with a history of Noonan syndrome (NS) presenting with slowly progressive right-sided hemiparesis and right-sided focal motor seizures. Despite initial imaging and histology suggesting a left frontal lobe high-grade intrinsic tumor typical of a glioblastoma, subsequent molecular analysis confirmed a diagnosis of MINST. The patient’s neurological condition improved after gross-total resection and adjuvant chemo-radiation; he remains on follow-up.

LESSONS

MINSTs are rare neoplasms with a poor prognosis; management options are limited, with surgery being the cornerstone of treatment. Reports on rare tumors such as this will increase awareness of this particular pathology and disclose clinical experience. In this case, the authors were unable to establish a definite cause-and-effect relation between NS and MINST. Nevertheless, it remains the first reported case in the literature.

Open access

Surgical treatment of common peroneal neuropathy in schwannomatosis: illustrative cases

Robert Y. North, Rita Snyder, John M. Slopis, and Ian E. McCutcheon

BACKGROUND

Neurofibromatosis syndromes such as neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis often result in painful symptoms related to tumor burden.

OBSERVATIONS

Painful symptoms classically associated with common points of peripheral nerve entrapment, such as common peroneal neuropathy at the fibular tunnel, may present in patients both with and without focal tumor involvement.

LESSONS

Surgical decompression at the point of entrapment, with or without resection of tumor, may provide symptomatic relief. Examples of surgical decompression at the point of entrapment, both with and without resection of tumor, are presented.