✓ 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.
Robert J. Spinner, Bernd W. Scheithauer and Kimberly K. Amrami
A report of two cases
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.
Marie-Noëlle Hébert-Blouin, Kimberly K. Amrami and Robert J. Spinner
Mark A. Mahan, Kimberly K. Amrami and Robert J. Spinner
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.
Marie-Noëlle Hébert-Blouin, Kimberly K. Amrami, Bernd W. Scheithauer and Robert J. Spinner
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.
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.
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.
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.
Robert J. Spinner, Bernd W. Scheithauer, Arie Perry, Kimberly K. Amrami, Ryan Emnett and David H. Gutmann
✓ The authors report on a patient without neurofibromatosis Type 1 or 2 (NF1 or NF2) and without evidence of schwannomatosis, who was found to have an unusual combination of nerve sheath tumors—a large cellular schwannoma and multifascicular involvement of a plexiform neurofibroma arising from the same site within the radial nerve and posterior cord of the infraclavicular brachial plexus. This case broadens the spectrum of combined pathological features of nerve sheath tumors. Genetic studies revealed a combined loss of neurofibromin and merlin in both regions and chromosome arm 22q deletion within the neurofibroma component only. The latter finding supports the suggestion that these were two different clonal neoplasms, and is consistent with a collision tumor pattern.
J. Bradley White, Bernd W. Scheithauer, Kimberly K. Amrami, Dusica Babovic-Vuksanovic and Robert J. Spinner
✓The authors present clinical, imaging, and histological features of two adult patients found to harbor a conventional schwannoma contiguous with a deep plexiform schwannoma. One patient had neurofibromatosis (NF) Type 2 (NF2), and both intracranial (bilateral oculomotor, trigeminal, acoustic, and hypoglossal schwannomas as well as meningiomas) and intraspinal (schwannomas and meningiomas) lesions. The proximal forearm lesions consisted of a conventional schwannoma and an underlying plexiform component. The second patient, who did not have NF2, presented with a similar enlarging mass in the distal arm; two contiguous lesions were resected. Both patients exhibited distinct, readily identifiable, magnetic resonance imaging features. Plexiform schwannomas are rare lesions that occur sporadically or, on occasion, in association with NF2 or meningiomas with or without multiple schwannomas. The authors believe that a more careful examination of patients with NF2 may show that these people have a higher incidence of plexiform schwannoma than previously thought. Pathological confirmation is crucial in distinguishing plexiform schwannomas from plexiform neurofibromas, because the latter are directly associated with NF1 and have a significant tendency to undergo malignant transformation. To the best of the authors’ knowledge, this is the first report of a conventional schwannoma contiguous with a deep plexiform schwannoma.
Thomas J. Wilson, B. Matthew Howe, Shelby A. Stewart, Robert J. Spinner and Kimberly K. Amrami
This study aimed to define a set of clinicoradiological parameters with a high specificity for the diagnosis of intraneural perineurioma, obviating the need for operative tissue diagnosis.
The authors retrospectively reviewed MR images obtained in a large cohort of patients who underwent targeted fascicular biopsy and included only those patients for whom the biopsy yielded a diagnosis. Clinical and radiological findings were then tested for their ability to predict a tissue diagnosis of intraneural perineurioma. The authors propose a new set of diagnostic criteria, referred to as the Perineurioma Diagnostic Criteria. The sensitivity, specificity, positive predictive value, and negative predictive value of several clinicoradiological methods of diagnosis were compared.
A total of 195 patients who underwent targeted fascicular biopsy were included in the cohort, of whom 51 had a tissue diagnosis of intraneural perineurioma. When the clinicoradiological methods used in this study were compared, the highest sensitivity (0.86), negative predictive value (0.95), and F1 score (0.88) were observed for the decision trees generated in C5.0 and rPart, whereas the highest specificity (1.0) and positive predictive value (1.0) were observed for the Perineurioma Diagnostic Criteria.
This study identified clinical and radiological features that are associated with a diagnosis of perineurioma. The Perineurioma Diagnostic Criteria were determined to be the following: 1) no cancer history, 2) unifocal disease, 3) moderate to severe hyperintensity on T2-weighted MR images, 4) moderate to severe contrast enhancement, 5) homogeneous contrast enhancement, 6) fusiform shape, 7) enlargement of the involved nerves, and 8) age ≤ 40 years. Use of the Perineurioma Diagnostic Criteria obviates the need for tissue diagnosis when all of the criteria are satisfied.
Robert J. Spinner, Kimberly K. Amrami, Michel Kliot, Shawn P. Johnston and Joaquim Casañas
Unlike the more commonly noted paralabral cysts (extraneural ganglia), which are well known to result in suprascapular nerve compression, only four cases of suprascapular intraneural ganglia have been reported. Because of their rarity, the pathogenesis of suprascapular intraneural ganglia has been poorly understood and a pathoanatomical explanation has not been provided. In view of the growing literature demonstrating strong associations between paralabral cysts and labral (capsular) pathology, joint connections, and joint communications, the authors retrospectively reviewed the magnetic resonance (MR) imaging studies and postoperative results in the two featured patients to test a hypothesis that suprascapular intraneural ganglia would have analogous findings.
Two patients who presented with suprascapular neuropathy were found to have intraneural ganglia. Connections to the glenohumeral joint could be established in both patients through posterior labrocapsular complex tears. In neither patient was the joint connection identified preoperatively or intraoperatively, and cyst decompression was performed by itself without attention to the labral tear. The suprascapular intraneural ganglia extended from the glenohumeral joint as far proximally as the level of the nerves’ origin from the upper trunk in the supraclavicular fossa. Although both patients experienced symptomatic improvement after surgery, neurological recovery was incomplete. In both cases, postoperative MR images revealed cyst persistence. In addition, previously unrecognized superior labral anteroposterior (SLAP) Type II lesions (tears of the superior labrum extending anteroposterior and involving the biceps anchor at the labrum without actual extension into the tendon) were visualized. In one patient with a persistent cyst, an MR arthrogram was obtained and demonstrated a communication between the joint and the cyst.
The findings in these two patients support the synovial theory for intraneural ganglia. Based on their experience with intraneural ganglia at other sites, the authors believe that suprascapular intraneural ganglia arise from the glenohumeral joint, egress through a superior (posterior) labral tear, and dissect within the epineurium along an articular branch into the main nerve, following the path of least resistance. Furthermore, these two cases of intraneural ganglia with SLAP lesions are directly analogous to the many cases of paralabral cysts associated with these types of labral tears. By better understanding the origin of this unusual type of ganglia and drawing analogies to the more common extraneural cysts, surgical strategies can be formulated to address the underlying pathoanatomy, improve operative outcomes, and prevent recurrences.