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Open access

Angiosarcoma arising in a schwannoma of the peripheral nervous system: illustrative case

Nikita Lakomkin, Jorge Torres-Mora, Eric J. Dozois, and Robert J. Spinner

BACKGROUND

Schwannomas of the peripheral nerves are benign tumors that can very rarely undergo malignant transformation. These lesions are particularly challenging to diagnose via noninvasive techniques but can have significant implications for treatment.

OBSERVATIONS

This is a case of a 70-year-old female with a prior history of a right sciatic notch tumor that was diagnosed as a conventional schwannoma via histology from an initial biopsy and subsequent surgical debulking. Unfortunately, she experienced significant worsening of her motor deficit, whereby her postoperative foot weakness progressed to complete foot drop in less than 2 years. In addition, she demonstrated significant radiological progression, with more than 1 to 2 cm of growth in each dimension at her subsequent evaluation, along with intractable right leg pain. An additional operation was performed to completely remove the 7 × 8 cm tumor, and histology demonstrated angiosarcoma within a schwannoma. There was no evidence of recurrence at 15 months, and the patient had significant improvement in her pain.

LESSONS

Rapidly worsening function and radiological progression are not typically seen with conventional benign nerve sheath tumors and should prompt consideration of other lesions. Angiosarcoma within schwannoma is a rare pathology and optimal therapies for these tumors in terms of surgical timing and adjuvant therapy are still unknown.

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

Nerve transfers in a patient with asymmetrical neurological deficit following traumatic cervical spinal cord injury: simultaneous bilateral restoration of pinch grip and elbow extension. Illustrative case

Alexander A. Gatskiy, Ihor B. Tretyak, Vitaliy I. Tsymbaliuk, and Yaroslav V. Tsymbaliuk

BACKGROUND

Cervical spinal cord injury (CSCI) causes severe motor deficit in upper extremities. The mixed segmental CSCI pattern is reflected in the combination of time-sensitive (TS) and non-TS myotomes in the upper extremities. Nerve transfers (NTs) restore upper extremity function yet remain TS procedures. A combination of neurological, magnetic resonance imaging (MRI), and electromyography (EMG) studies allows the identification of TS and non-TS myotomes in the upper extremities.

OBSERVATIONS

Nineteen months after NTs, flexor pollicis longus (FPL) and deep flexor of the index finger (FDP2) recovered to M4 (right UE), FPL recovered to M3 and FDP2 to M2 (left EU). The long head of the triceps brachii muscle recovered to M4 bilaterally. The Capabilities of Upper Extremity Questionnaire (CUE-Q) score for unilateral arm functionality increased by 44% (right) and 112.5% (left) and for bilateral arm functionality by 400%; the CUE-Q score for unilateral hand and finger function increased by 283% (right) and 166% (left).

LESSONS

The combination of neurological, MRI, and EMG studies before surgery and data obtained during surgery provides reliable information on the CSCI pattern, specifically the availability of motor donor nerves. Simultaneous bilateral restoration is required in the event of CSCI and significantly improves the unilateral and bilateral function of the UEs.

Open access

The value of high-resolution imaging in an occult peroneal intraneural ganglion cyst: illustrative case

Karina A. Lenartowicz, Kimberly K. Amrami, Jeffrey A. Strakowski, B. Matthew Howe, and Robert J. Spinner

BACKGROUND

Foot drop is a common complaint with a broad differential diagnosis making imaging a key part of the diagnostic workup. The authors present a patient with an occult peroneal intraneural ganglion cyst who underwent imaging with high-frequency ultrasound (US) and high-resolution magnetic resonance imaging (MRI) to highlight the role of such techniques in cases of peroneal neuropathy.

OBSERVATIONS

Intraneural ganglion cysts are emerging as a common cause of common peroneal neuropathy. Imaging with US and MRI is a valuable tool used to illustrate the pertinent anatomy and identify the articular branch joint connection and cyst as part of the surgical planning and definitive management.

LESSONS

Intraneural ganglion cysts can be small or nearly invisible and failure to appreciate the intraneural cyst can lead to symptom or cyst persistence or recurrence. High-resolution modalities can be useful in the diagnosis and surgical planning of difficult cases.

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.

Open access

Iatrogenic neurological injury after radiofrequency ablation and epidural steroid injections: illustrative cases

Lisa B. E. Shields, Vasudeva G. Iyer, Yi Ping Zhang, and Christopher B. Shields

BACKGROUND

Neck pain is often chronic and disabling. Cervical facet joint injections and epidural steroid injections are frequently used to manage chronic neck pain and cervicogenic headaches. While minimal side effects are commonly associated with these treatments, severe complications are exceedingly rare.

OBSERVATIONS

The authors report 4 cases of iatrogenic neurological injury after radiofrequency ablation (RFA) and epidural steroid injections. One patient experienced left shoulder, scapular, and arm pain with left arm and hand weakness that developed immediately after RFA for chronic neck pain. Electromyography/nerve conduction velocity (EMG/NCV) studies confirmed denervation changes in the left C8–T1 distribution. Three patients complained of numbness and weakness of the hands immediately after an interlaminar cervical epidural block. One of these patients underwent EMG/NCV that confirmed denervation changes occurring in the left C8–T1 distribution.

LESSONS

Spine surgeons and pain management specialists should be aware of neurological injuries that may occur after cervical RFA and epidural steroid injections, especially after a multilevel cervical procedure and with severe cervical spinal stenosis. EMG/NCV studies plays an important role in detecting and localizing neurological injury and in differentiating from conditions that mimic cervical root injuries, including brachial plexus trauma due to positioning and Parsonage-Turner syndrome.