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Open access

Adult neuromuscular choristoma, a rare peripheral nerve pathology: illustrative case

Ashley Ricciardelli, Alex Flores, Hsiang-Chih Lu, Ekin Guney, Arie Perry, and Joey Grochmal

BACKGROUND

Neuromuscular choristomas (NMCs) are rare tumors involving aberrant intercalation of muscle fibers into peripheral nerves, most commonly the sciatic nerve. Although benign, these lesions risk developing into NMCs with desmoid-type fibrosis (NMC-DTFs), aggressive lesions potentially requiring amputation. Currently, information on NMCs and the link between NMCs and NMC-DTFs is limited in adults, with the majority of cases reported in children. We present the case of a 66-year-old male with a sciatic NMC alongside a Preferred Reporting Items for Systematic Reviews and Meta-Analyses–based systematic review of similar cases to better characterize this lesion in the adult population.

OBSERVATIONS

A male presented with 10 years of progressive left lower-extremity weakness and paresthesia, and a mildly enlarged proximal sciatic nerve was discovered on magnetic resonance imaging. He underwent left sciatic fascicular nerve biopsy, with histopathological examination identifying the lesion as an NMC. The literature review revealed that our case, alongside other cases of adults with NMCs, shared characteristics similar to NMCs in the pediatric population.

LESSONS

More comprehensive studies of adults with NMCs are needed, as the current literature contains limited information concerning disease course, diagnostic characteristics, and treatment. Furthermore, NMCs in adults should be handled with care because of the increased likelihood of transformation to NMC-DTF after surgical intervention.

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

Internal iliac artery aneurysm masquerading as a sciatic nerve schwannoma: illustrative case

Lokeshwar S. Bhenderu, Khaled M. Taghlabi, Taimur Hassan, Jaime R. Guerrero, Jesus G. Cruz-Garza, Rachel L. Goldstein, Shashank Sharma, Linda V. Le, Tue A. Dinh, and Amir H. Faraji

BACKGROUND

Schwannomas are common peripheral nerve sheath tumors. Imaging techniques such as magnetic resonance imaging (MRI) and computed tomography (CT) can help to distinguish schwannomas from other types of lesions. However, there have been several reported cases describing the misdiagnosis of aneurysms as schwannomas.

OBSERVATIONS

A 70-year-old male with ongoing pain despite spinal fusion surgery underwent MRI. A lesion was noted along the left sciatic nerve, which was believed to be a sciatic nerve schwannoma. During the surgery for planned neurolysis and tumor resection, the lesion was noted to be pulsatile. Electromyography mapping and intraoperative ultrasound confirmed vascular pulsations and turbulent flow within the aneurysm, so the surgery was aborted. A formal CT angiogram revealed the lesion to be an internal iliac artery (IIA) branch aneurysm. The patient underwent coil embolization with complete obliteration of the aneurysm.

LESSONS

The authors report the first case of an IIA aneurysm misdiagnosed as a sciatic nerve schwannoma. Surgeons should be aware of this potential misdiagnosis and potentially use other imaging modalities to confirm the lesion before proceeding with surgery.

Open access

Etiology of spastic foot drop among 16 patients undergoing electrodiagnostic studies: patient series

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

BACKGROUND

Differentiating foot drop due to upper motor neuron (UMN) lesions from that due to lower motor neuron lesions is crucial to avoid unnecessary surgery or surgery at the wrong location. Electrodiagnostic (EDX) studies are useful in evaluating patients with spastic foot drop (SFD).

OBSERVATIONS

Among 16 patients with SFD, the cause was cervical myelopathy in 5 patients (31%), cerebrovascular accident in 3 (18%), hereditary spastic paraplegia in 2 (12%), multiple sclerosis in 2 (12%), chronic cerebral small vessel disease in 2 (12%), intracranial meningioma in 1 (6%), and diffuse brain injury in 1 (6%). Twelve patients (75%) had weakness of a single leg, whereas 2 others (12%) had bilateral weakness. Eleven patients (69%) had difficulty walking. The deep tendon reflexes of the legs were hyperactive in 15 patients (94%), with an extensor plantar response in 9 patients (56%). Twelve patients (75%) had normal motor and sensory conduction, 11 of whom had no denervation changes of the legs.

LESSONS

This study is intended to raise awareness among surgeons about the clinical features of SFD. EDX studies are valuable in ruling out peripheral causes of foot drop, which encourages diagnostic investigation into a UMN source for the foot drop.

Open access

Radial nerve myofibroma: a rare benign tumor with perineural infiltration. Illustrative case

Kitty Y. Wu, David J. Cook, Kimberly K. Amrami, and Robert J. Spinner

BACKGROUND

Myofibromas are benign mesenchymal tumors, classically presenting in infants and young children in the head and neck region. Perineural involvement, especially in peripheral nerves within the upper extremity, is extremely rare in myofibromas.

OBSERVATIONS

The authors present the case of a 16-year-old male with a 4-month history of an enlarging forearm mass and rapidly progressive dense motor weakness in wrist, finger, and thumb extension. Preoperative imaging and fine needle biopsy confirmed the diagnosis of a benign isolated myofibroma. Given the dense paralysis, operative management was indicated, and intraoperative exploration showed extensive involvement of tumor within the radial nerve. The infiltrated nerve segment was excised along with the tumor, and the resulting 5-cm nerve gap was reconstructed using autologous cabled grafts.

LESSONS

Perineural pseudoinvasion can be an extremely rare and atypical feature of nonmalignancies, resulting in dense motor weakness. Extensive nerve involvement may still necessitate nerve resection and reconstruction, despite the benign etiology of the lesion.

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.