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Mark A. Mahan, Kimberly K. Amrami, and Robert J. Spinner

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Marie-Noëlle Hébert-Blouin, Kimberly K. Amrami, and Robert J. Spinner

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Stepan Capek, Benjamin M. Howe, Kimberly K. Amrami, and Robert J. Spinner

OBJECT

Perineural spread along pelvic autonomie nerves has emerged as a logical, anatomical explanation for selected cases of neoplastic lumbosacral plexopathy (LSP) in patients with prostate, bladder, rectal, and cervical cancer. The authors wondered whether common radiological and clinical patterns shared by various types of pelvic cancer exist.

METHODS

The authors retrospectively reviewed their institutional series of 17 cases concluded as perineural tumor spread. All available history, physical examination, electrodiagnostic studies, biopsy data and imaging studies, evidence of other metastatic disease, and follow-up were recorded in detail. The series was divided into 2 groups: cases with neoplastic lumbosacral plexopathy confirmed by biopsy (Group A) and cases included based on imaging characteristics despite the lack of biopsy or negative biopsy results (Group B).

RESULTS

Group A comprised 10 patients (mean age 69 years); 9 patients were symptomatic and 1 was asymptomatic. The L5–S1 spinal nerves and sciatic nerve were most frequently involved. Three patients had intradural extension. Seven patients were alive at last follow-up. Group B consisted of 7 patients (mean age 64 years); 4 patients were symptomatic, 2 were asymptomatic, and 1 had only imaging available. The L5–S1 spinal nerves and the sciatic nerve were most frequently involved. No patients had intradural extension. Four patients were alive at last follow-up.

CONCLUSIONS

The authors provide a unifying theory to explain lumbosacral plexopathy in select cases of various pelvic neoplasms. The tumor cells can use splanchnic nerves as conduits and spread from the end organ to the lumbosacral plexus. Tumor can continue to spread along osseous and muscle nerve branches, resulting in muscle and bone “metastases.” Radiological studies show a reproducible, although nonspecific pattern, and the same applies to clinical presentation.

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Ziv Williams, Kimberly K. Amrami, and Robert J. Spinner

✓Tumoral calcinosis is a rare disorder that leads to diffuse calcium phosphate deposition into soft tissue and may be seen in the setting of uremia, hyperparathyroidism, or vitamin D intoxication. This lesion can produce significant local pain and can limit mobility in large joints where it tends to occur. Less commonly, it may produce neurological symptoms by compressing or encompassing adjacent neurovascular structures. Tumoral calcinosis involving nerve structures is challenging to treat, primarily because of its extensive size and propensity to infiltrate. Although surgical intervention can often provide symptomatic improvement, this lesion tends to recur in the presence of elevated calcium phosphate levels, and its management therefore requires a combined multidisciplinary surgical and medical approach. The authors describe two cases in which patients developed tumoral calcinosis producing peripheral nerve compression and discuss their respective surgical and medical management.

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Robert J. Spinner, Bernd W. Scheithauer, and Kimberly K. Amrami

✓ The authors describe the case of a patient with a novel cause of medial plantar symptoms due to extrinsic compression by a schwannoma arising within the adventitia of the tibial artery in the ankle region. Additionally they provide the operative, histological, and imaging findings.

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Nikhil K. Prasad, Mark A. Mahan, Benjamin M. Howe, Kimberly K. Amrami, and Robert J. Spinner

Lipomatosis of nerve (LN) is a rare disorder of peripheral nerves that produces proliferation of interfascicular adipose tissue. It may be associated with soft-tissue and bony overgrowth within the affected nerve territory. LN has been almost exclusively reported in appendicular peripheral nerves; the median nerve at the wrist and palm is among the most common locations. The authors present a new pattern of LN that shows circumferential proliferation of fat around the epineurium of the nerve. They believe that this case and the two other documented examples in the literature (also affecting cervical and thoracic spinal nerves) share the same new pattern of LN. Defining the full spectrum of adipose lesions of the nerve and establishing a cause-effect relationship with nerve-territory overgrowth disorders may offer options for future management through targeted nerve lesioning.

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Nikhil K. Murthy, Kimberly K. Amrami, Stephen M. Broski, Patrick B. Johnston, and Robert J. Spinner

OBJECTIVE

Neurolymphomatosis (NL) is a rare manifestation of lymphoma confined to the peripheral nervous system that is poorly understood. It can be found in the cauda equina, but extraspinal disease can be underappreciated. The authors describe how extraspinal NL progresses to the cauda equina by perineural spread and the implications of this on timely and safe diagnostic options.

METHODS

The authors used the Mayo Clinic medical records database to find cases of cauda equina NL with sufficient imaging to characterize the lumbosacral plexus diagnosed from tissue biopsy. Demographics (sex, age), clinical data (initial symptoms, cerebrospinal fluid, evidence of CNS involvement, biopsy location, primary or secondary disease), and imaging findings were reviewed.

RESULTS

Ten patients met inclusion and exclusion criteria, and only 2 of 10 patients presented with cauda equina symptoms at the time of biopsy, with 1 patient undergoing a cauda equina biopsy. Eight patients were diagnosed with diffuse large B-cell lymphoma, 1 with low-grade B-cell lymphoma, and 1 with mantle cell lymphoma. Isolated spinal nerve involvement was identified in 5 of 10 cases, providing compelling evidence regarding the pathophysiology of NL. The conus medullaris was not radiologically involved in any case. Lumbosacral plexus MRI was able to identify extraspinal disease and offered diagnostically useful biopsy targets. FDG PET/CT was relatively insensitive for detecting disease in the cauda equina but was helpful in identifying extraspinal NL.

CONCLUSIONS

The authors propose that perineural spread of extraspinal NL to infiltrate the cauda equina occurs in two phases. 1) There is proximal and distal spread along a peripheral nerve, with eventual spread to anatomically connected nerves via junction and branch points. 2) The tumor cells enter the spinal canal through corresponding neural foramina and propagate along the spinal nerves composing the cauda equina. To diffusely infiltrate the cauda equina, a third phase occurs in which tumor cells can spread circumdurally to the opposite side of the spinal canal and enter contralateral nerve roots extending proximally and distally. This spread of disease can lead to diffuse bilateral spinal nerve disease without diffuse leptomeningeal spread. Recognition of this phasic mechanism can lead to identification of safer extraspinal biopsy targets that could allow for greater functional recovery after appropriate treatment.

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Marie-Noëlle Hébert-Blouin, Kimberly K. Amrami, Bernd W. Scheithauer, and Robert J. Spinner

Object

In clinical practice, schwannomas are among the most common types of nerve sheath tumors. Their clinical presentation, imaging characteristics, and operative features are well known. Over the past 20 years, clinical outcomes have improved due to resection of these tumors at a fascicular level. Despite these advances, a subgroup of patients with schwannomas is associated with a disappointing neurological outcome following resection. The purpose of this study was to correlate the imaging and histological features in this group of patients with more anatomically complex forms of schwannomas.

Methods

In a retrospective review performed at their institution over a 10-year period, the authors found a subgroup of patients with complex multinodular/plexiform schwannomas affecting major peripheral nerves. Eleven patients were identified, and the clinical, imaging, and pathological features of their disease were reviewed.

Results

The clinical presentation of multinodular/plexiform schwannomas of major peripheral nerves may be similar to that of conventional schwannomas, but their imaging features, operative appearance, and outcomes differ.

Conclusions

Preoperatively and intraoperatively, the distinguishing features of multinodular/plexiform schwannomas of major peripheral nerves may be subtle and can easily go unrecognized, thus explaining the often suboptimal surgical results. Familiarity with the imaging and operative features of multinodular/plexiform schwannomas will no doubt alter treatment approaches and improve neurological function in this subgroup of patients.

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Robert J. Spinner, Bernd W. Scheithauer, Kimberly K. Amrami, Doris E. Wenger, and Marie-Noëlle Hébert-Blouin

Object

Adipose lesions of nerve are rare and poorly understood. Their current classification, although not universally accepted, generally includes lipomatosis of nerve with or without localized macrodactyly, and intra- as well as extraneural lipoma. The authors believe that the spectrum of these lesions and their interrelationships are not currently appreciated. They propose an adaptation to the existing framework to illustrate the expanding spectrum of adipose lesions of nerve by considering lipomatosis and lipoma singly or in combination.

Methods

Fourteen representative cases are presented to demonstrate not only the intraneural and extraneural examples of lipomatosis and lipoma, but also their anatomical combinations.

Results

Based on the cases presented and a careful literature review, a conceptual approach to the classification of adipose lesions of nerve is generated. This approach incorporates the 2 essential lesions, lipomatosis of nerve and lipoma, in both their intra- and extraneural forms. This permits expansion to encompass combinations.

Conclusions

To press the concept that adipose tumors of nerve are a broad but interrelated spectrum of lesions, the authors propose modification of the present classification system. This approach provides an orderly platform for progress, reflects understanding of these interrelated lesions, and facilitates optimal treatment by distinguishing resectable from nonresectable components.

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Chandan G. Reddy, Kimberly K. Amrami, Benjamin M. Howe, and Robert J. Spinner

OBJECT

Knee dislocations are often accompanied by stretch injuries to the common peroneal nerve (CPN). A small subset of these injuries also affect the tibial nerve. The mechanism of this combined pattern could be a single longitudinal stretch injury of the CPN extending to the sciatic bifurcation (and tibial division) or separate injuries of both the CPN and tibial nerve, either at the level of the tibiofemoral joint or distally at the soleal sling and fibular neck. The authors reviewed cases involving patients with knee dislocations with CPN and tibial nerve injuries to determine the localization of the combined injury and correlation between degree of MRI appearance and clinical severity of nerve injury.

METHODS

Three groups of cases were reviewed. Group 1 consisted of knee dislocations with clinical evidence of nerve injury (n = 28, including 19 cases of complete CPN injury); Group 2 consisted of knee dislocations without clinical evidence of nerve injury (n = 19); and Group 3 consisted of cases of minor knee trauma but without knee dislocation (n = 14). All patients had an MRI study of the knee performed within 3 months of injury. MRI appearance of tibial and common peroneal nerve injury was scored by 2 independent radiologists in 3 zones (Zone I, sciatic bifurcation; Zone II, knee joint; and Zone III, soleal sling and fibular neck) on a severity scale of 1–4. Injury signal was scored as diffuse or focal for each nerve in each of the 3 zones. A clinical score was also calculated based on Medical Research Council scores for strength in the tibial and peroneal nerve distributions, combined with electrophysiological data, when available, and correlated with the MRI injury score.

RESULTS

Nearly all of the nerve segments visualized in Groups 1 and 2 demonstrated some degree of injury on MRI (95%), compared with 12% of nerve segments in Group 3. MRI nerve injury scores were significantly more severe in Group 1 relative to Group 2 (2.06 vs 1.24, p < 0.001) and Group 2 relative to Group 3 (1.24 vs 0.13, p < 0.001). In both groups of patients with knee dislocations (Groups 1 and 2), the MRI nerve injury score was significantly higher for CPN than tibial nerve (2.72 vs 1.40 for Group 1, p < 0.001; 1.39 vs 1.09 for Group 2, p < 0.05). The clinical injury score had a significantly strong correlation with the MRI injury score for the CPN (r = 0.75, p < 0.001), but not for the tibial nerve (r = 0.07, p = 0.83).

CONCLUSIONS

MRI is highly sensitive in detecting subclinical nerve injury. In knee dislocation, clinical tibial nerve injury is always associated with simultaneous CPN injury, but tibial nerve function is never worse than peroneal nerve function. The point of maximum injury can occur in any of 3 zones.