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Surgical management of malignant melanotic nerve sheath tumors: an institutional experience and systematic review of the literature

Abdul Karim Ghaith, Sarah E. Johnson, Victor Gabriel El-Hajj, Oluwaseun O. Akinduro, Marc Ghanem, Gaetano De Biase, Loizos Michaelides, Antonio Bon Nieves, W. Richard Marsh, Bradford L. Currier, John L. Atkinson, Robert J. Spinner, and Mohamad Bydon

OBJECTIVE

Malignant melanotic nerve sheath tumors are rare tumors characterized by neoplastic melanin-producing Schwann cells. In this study, the authors report their institution’s experience in treating spinal and peripheral malignant melanotic nerve sheath tumors and compare their results with the literature.

METHODS

Data were collected from 8 patients who underwent surgical treatment for malignant melanotic nerve sheath tumors between 1996 and 2023 at Mayo Clinic and 63 patients from the literature. Time-to-event analyses were performed for the combined group of 71 cases to evaluate the risk of recurrence, metastasis, and death based on tumor location and type of treatment received. Unpaired 2-sample t-tests and Fisher’s exact tests were used to determine statistical significance between groups.

RESULTS

Between 1996 and 2023, 8 patients with malignant melanotic nerve sheath tumors underwent surgery at the authors’ institution, while 63 patients were identified in the literature. The authors’ patients and those in the literature had the same mean age at diagnosis (43 years). At the authors’ institution, 5 patients (63%) experienced metastasis, 6 patients (75%) experienced long-term recurrence, and 5 patients (62.5%) died. In the literature, most patients (60.3%) were males, with a peak incidence between the 4th and 5th decades of life. Nineteen patients (31.1%) were diagnosed with Carney complex. Nerve root tumors accounted for most presentations (n = 39, 61.9%). Moreover, 24 patients (38.1%) had intradural lesions, with 54.2% (n = 13) being intramedullary and 45.8% (n = 11) extramedullary. Most patients underwent gross-total resection (GTR) (n = 41, 66.1%), followed by subtotal resection (STR) (n = 12, 19.4%), STR with radiation therapy (9.7%), and GTR with radiation therapy (4.8%). Sixteen patients (27.6%) experienced metastasis, 23 (39.7%) experienced recurrence, and 13 (22%) died. Kaplan-Meier analyses showed no significant differences among treatment approaches in terms of recurrence-free, metastasis-free, and overall survival (p > 0.05). Similar results were obtained when looking at the differences with respect to intradural versus nerve root location of the tumor (p > 0.05).

CONCLUSIONS

Malignant melanotic nerve sheath tumors are rare tumors with a high potential for malignancy. They carry a dismal prognosis, with a pooled local recurrence rate of 42%, distant metastasis rate of 27%, and mortality rate of 26%. The findings from this study suggest a trend favoring the use of GTR alone or STR with radiation therapy over STR alone. Mortality was similar regardless, which highlights the need for the development of effective treatment options to improve survival in patients with melanotic schwannomas.

In Journal of Neurosurgery: Spine Publish Before Print

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Core outcomes in nerve surgery: development of a core outcome set for ulnar neuropathy at the elbow

Thomas J. Wilson, Gavin A. Davis, Nora F. Dengler, Fernando Guedes, Marie-Noëlle Hébert-Blouin, Megan M. Jack, Line G. Jacques, Thomas Kretschmer, Mark A. Mahan, Rajiv Midha, Willem Pondaag, Ross C. Puffer, Lukas Rasulic, Wilson Z. Ray, Elias Rizk, Carlos A. Rodriguez-Aceves, Yuval Shapira, Brandon W. Smith, Mariano Socolovsky, Robert J. Spinner, and Eric L. Zager

OBJECTIVE

Ulnar neuropathy at the elbow (UNE) is common, affecting 1%–6% of the population. Despite this, there remains a lack of consensus regarding optimal treatment. This is primarily due to the difficulty one encounters when trying to assess the literature. Outcomes are inconsistently reported, which makes comparing studies or developing meta-analyses difficult or even impossible. Thus, there is a need for a core outcome set (COS) for UNE (COS-UNE) to help address this problem. The objective of this study was to utilize a modified Delphi method to develop COS-UNE.

METHODS

A 5-stage approach was utilized to develop COS-UNE: stage 1, consortium development; 2, literature review to identify potential outcome measures; 3, Delphi survey to develop consensus on outcomes for inclusion; 4, Delphi survey to develop definitions; and 5, consensus meeting to finalize the COS and definitions. The study followed the Core Outcome Set-STAndards for Development (COS-STAD) recommendations.

RESULTS

The Core Outcomes in Nerve Surgery (COINS) Consortium comprised 21 participants, all neurological surgeons representing 11 countries. The final COS-UNE consisted of 22 data points/outcomes covering the domains of demographic characteristics, diagnostics, patient-reported outcomes, motor/sensory outcomes, and complications. Appropriate instruments, methods of testing, and definitions were set. The consensus minimum duration of follow-up was 6 months, with the consensus optimal timepoints for assessment identified as preoperatively and 3, 6, and 12 months postoperatively.

CONCLUSIONS

The authors identified consensus data points/outcomes and also provided definitions and specific scales to be utilized to help ensure that clinicians are consistent in their reporting across studies on UNE. This COS should serve as a minimum set of data to be collected in all future neurosurgical studies on UNE. The authors hope that clinicians evaluating ulnar neuropathy will incorporate this COS into routine practice and that future studies will consider this COS in the design phase.

In Journal of Neurosurgery Publish Before Print

Open access

Posterior interosseous nerve paralysis secondary to an extraneural ganglion cyst from a radial neck pseudarthrosis: illustrative case

Karina A. Lenartowicz, Robert J. Spinner, Kimberly K. Amrami, and Shawn W. O’Driscoll

BACKGROUND

Many benign and malignant tissue or bony lesions have been reported as causes of extrinsic or intrinsic posterior interosseous nerve (PIN) neuropathy at the proximal forearm/elbow region. The authors describe an unusual cause of external compression of the PIN due to a ganglion cyst arising from a radial neck pseudarthrosis (a false joint).

OBSERVATIONS

Decompression of the PIN with the release of the arcade of Frohse was performed with resection of the radial head and the ganglion cyst. By 6 months postoperatively, the patient had a complete neurological recovery.

LESSONS

This case illustrates a previously unreported cause of extraneural compression of the PIN from a pseudarthrosis. The mechanism for compression in this case from the radial head pseudarthrosis is likely attributable to the sandwich effect, in which the PIN is sandwiched between the arcade of Frohse at the supinator from above and the cyst below.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 1 (Jul 2023)

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Characterizing peroneal nerve injury clinicoradiological patterns with MRI in patients with sciatic neuropathy and foot drop after total hip replacement

Kitty Y. Wu, Kimberly K. Amrami, Kirsten M. Hayford, and Robert J. Spinner

OBJECTIVE

Sciatic nerve injury following total hip arthroplasty (THA) predominantly affects the peroneal division of the sciatic nerve, often causing a foot drop. This can result from a focal etiology (hardware malposition, prominent screw, or postoperative hematoma) or nonfocal/traction injury. The objective of this study was to compare the clinicoradiological features and define the extent of nerve injury resulting from these two distinct mechanisms.

METHODS

Patients who developed a postoperative foot drop within 1 year after primary or revision THA with a confirmed proximal sciatic neuropathy based on MRI or electrodiagnostic studies were retrospectively reviewed. Patients were divided into two cohorts: group 1 (focal injury), including patients with an identifiable focal structural etiology, and group 2 (nonfocal injury), including patients with a presumed traction injury. Patient demographics, clinical examinations, subsequent surgeries, electrodiagnostic study results, and MRI abnormalities were noted. The Student t-test was used to compare time to onset of foot drop and time to secondary surgery.

RESULTS

Twenty-one patients, treated by one surgeon, met inclusion criteria (8 men and 13 women; 14 primary THAs and 7 revision THAs). Group 1 had a significantly longer time from THA to the onset of foot drop, with a mean of 2 months, compared with an immediate postoperative onset in group 2 (p = 0.02). Group 1 had a consistent pattern of localized focal nerve abnormality on imaging. In contrast, the majority of patients in group 2 (n = 11) had a long, continuous segment of abnormal size and signal intensity of the nerve, while the other 3 patients had a segment of less abnormal nerve in the midthigh on imaging. All patients with a long continuous lesion had Medical Research Council grade 0 dorsiflexion prior to secondary nerve surgeries compared with 1 of 3 patients with a more normal midsegment.

CONCLUSIONS

There are distinct clinicoradiological findings in patients with sciatic injuries resulting from a focal structural etiology versus a traction injury. While there are discrete localized changes in patients with a focal etiology, those with traction injuries demonstrate a diffuse zone of abnormality within the sciatic nerve. A proposed mechanism involves anatomical tether points of the nerve acting as points of origin and propagation for traction injuries, resulting in an immediate postoperative foot drop. In contrast, patients with a focal etiology have localized imaging findings but a highly variable time to the onset of foot drop.

In Journal of Neurosurgery Volume 139: Issue 6 (Dec 2023)

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Neuromuscular choristoma and circumferential nerve territory desmoid-type fibromatosis: imaging findings supporting a nerve-driven mechanism

Andres A. Maldonado, Stephen M. Broski, Jodi M. Carter, Tomas Marek, B. Matthew Howe, and Robert J. Spinner

OBJECTIVE

Neuromuscular choristoma (NMC) is a rare developmental malformation of peripheral nerve that is frequently associated with the development of a desmoid-type fibromatosis (DTF). Both NMC and NMC-DTF typically contain pathogenic CTNNB1 mutations and NMC-DTF develop only within the NMC-affected nerve territory. The authors aimed to determine if there is a nerve-driven mechanism involved in the formation of NMC-DTF from the underlying NMC-affected nerve.

METHODS

Retrospective review was performed for patients evaluated in the authors’ institution with a diagnosis of NMC-DTF in the sciatic nerve (or lumbosacral plexus). MRI and FDG PET/CT studies were reviewed to determine the specific relationship and configuration of NMC and DTF lesions along the sciatic nerve.

RESULTS

Ten patients were identified with sciatic nerve NMC and NMC-DTF involving the lumbosacral plexus, sciatic nerve, or sciatic nerve branches. All primary NMC-DTF lesions were located in the sciatic nerve territory. Eight cases of NMC-DTF demonstrated circumferential encasement of the sciatic nerve, and one abutted the sciatic nerve. One patient had a primary DTF remote from the sciatic nerve, but subsequently developed multifocal DTF within the NMC nerve territory, including 2 satellite DTFs that circumferentially encased the parent nerve. Five patients had a total of 8 satellite DTFs, 4 of which abutted the parent nerve and 3 that circumferentially involved the parent nerve.

CONCLUSIONS

Based on clinical and radiological data, a novel mechanism of NMC-DTF development from soft tissues innervated by NMC-affected nerve segments is proposed, reflecting their shared molecular genetic alteration. The authors believe the DTF develops outward from the NMC in a radial fashion or it arises in the NMC and wraps around it as it grows. In either scenario, NMC-DTF develops directly from the nerve, likely arising from (myo)fibroblasts within the stromal microenvironment of the NMC and grows outward into the surrounding soft tissues. Clinical implications for patient diagnosis and treatment are presented based on the proposed pathogenetic mechanism.

In Journal of Neurosurgery Publish Before Print

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.

In Journal of Neurosurgery: Case Lessons Volume 5 (2023): Issue 19 (May 2023)

Open access

Perineurioma of the sciatic nerve with reticular, microcystic, and pseudolipoblastic features: illustrative case

Karina A. Lenartowicz, Kimberly K. Amrami, Jorge Torres-Mora, and Robert J. Spinner

BACKGROUND

Perineuriomas are peripheral nerve sheath tumors that are composed of benign, localized proliferations of perineural cells and further subclassified as intraneural or extraneural (soft tissue) based on their relationship to the histological boundaries of the nerve. Multiple histological variants have been described, and herein the authors present the first known case of a pseudolipoblastic perineurioma affecting the nerve.

OBSERVATIONS

A 52-year-old woman presented with a 5-year history of progressive, severe left buttock pain radiating down to the top of her foot and ankle, without any associated weakness, with a large mass in her sciatic nerve noted on magnetic resonance imaging (MRI). She underwent resection, which demonstrated a pseudolipoblastic perineurioma of the sciatic nerve, an unusual histological subtype composed of perineurial cells with an abundant clear intracytoplasmic background. Postoperatively, her pain resolved, and follow-up MRI showed no tumor persistence or recurrence.

LESSONS

On imaging, this lesion had a benign appearance, with areas suggestive of subacute hemorrhage, and was associated with a nerve. Although the distinctive morphological features of this lesion may suggest liposarcoma, careful morphological evaluation and appropriate immunohistochemical studies allow its correct classification.

In Journal of Neurosurgery: Case Lessons Volume 5 (2023): Issue 15 (Apr 2023)

Open access

Peroneal intraneural ganglion cyst with a nearly invisible joint connection (even to advocates of the articular theory): illustrative case

Karina A. Lenartowicz, Kimberly K. Amrami, and Robert J. Spinner

BACKGROUND

The articular (synovial) theory describes the formation of intraneural ganglion cysts through defects in the capsule of synovial joints. While the articular theory is gaining significant traction in the literature, it is not universally accepted. Therefore, the authors present a case of a plainly visible peroneal intraneural cyst, although the subtle joint connection was not identified specifically at the time of surgery, with subsequent rapid extraneural cyst recurrence. The joint connection was not immediately evident on review of the magnetic resonance imaging, even to the authors who have a large experience with this clinical entity. The authors report this case to demonstrate that all intraneural ganglion cysts have joint connections, although they may be difficult to identify.

OBSERVATIONS

An occult joint connection in the intraneural ganglion poses a unique diagnostic and management dilemma. High-resolution imaging is a valuable tool used to identify the articular branch joint connection as part of the surgical planning.

LESSONS

Based on the articular theory, all intraneural ganglion cysts will have a joint connection through an articular branch, although this may be small or nearly invisible. Failure to appreciate this connection can lead to cyst recurrence. A high index of suspicion of the articular branch is needed for surgical planning.

In Journal of Neurosurgery: Case Lessons Volume 5 (2023): Issue 7 (Feb 2023)

Open access

Unrecognized neuromuscular choristoma with recurrent desmoid-type fibromatosis and Marjolin ulcer: expanding the spectrum of neuromuscular choristoma sequelae within the nerve territory? Illustrative case

Andres A. Maldonado, Stephen M. Broski, Jodi M. Carter, and Robert J. Spinner

BACKGROUND

Neuromuscular choristoma (NMC) is a rare congenital lesion in which muscle tissue is admixed with nerve fascicles within a peripheral nerve. Patients commonly present in early childhood with neuropathy, plexopathy, or chronic undergrowth in the distribution of the affected nerve.

OBSERVATIONS

The authors present the case of a 35-year-old man with unrecognized neuromuscular NMC of the sciatic nerve, which resulted in recurrent, multicentric NMC-associated desmoid-type fibromatosis (NMC-DTF) within the nerve territory in association with a Marjolin ulcer, a cutaneous malignancy.

LESSONS

Based on anatomical and pathophysiological findings described in this case report, the authors support the association between NMC-DTF and Marjolin ulcer.

In Journal of Neurosurgery: Case Lessons Volume 5 (2023): Issue 6 (Feb 2023)

Open access

Occult lipomatosis of the nerve as part of macrodystrophia lipomatosa: illustrative case

Tomas Marek, Kimberly K. Amrami, and Robert J. Spinner

BACKGROUND

Macrodystrophia lipomatosa (MDL) is characterized by progressive overgrowth affecting soft tissues and bony structures and is part of lipomatous overgrowth syndromes. MDL has been associated with lipomatosis of the nerve (LN), an adipose lesion of nerve that has a pathognomonic magnetic resonance imaging (MRI) appearance as well as a mutation in the PIK3CA gene. The authors present a case of occult LN in the setting of MDL.

OBSERVATIONS

A 2-year-old boy with progressive soft tissue overgrowth of his proximal right lower extremity was initially diagnosed with neurofibromatosis type 1 (NF1). At our institution, NF1 as well as other overgrowth syndromes including PTEN hamartoma tumor syndrome were excluded. He was diagnosed as having so-called MDL. Upon reinterpretation of the patient’s MRI studies, short-segment LN involving the proximal sciatic nerve and part of lumbosacral plexus was identified. He underwent 2 debulking/liposuction procedures for soft tissue overgrowth. Genetic testing of tissue revealed a mutation in PIK3CA.

LESSONS

Thorough clinical examination (for signs of overgrowth) as well as an MRI study of the entire neural pathway is a critical part of the diagnostic workup to evaluate for LN. The authors believe that an increasing association of LN, even when occult, will emerge that will explain many cases with marked nerve-territory overgrowth.

In Journal of Neurosurgery: Case Lessons Volume 5 (2023): Issue 2 (Jan 2023)