High Resolution MRI Confirms Torsional Injury as Mechanism for Combined Ankle and Common Fibular (Peroneal) Nerve Injuries.

Common fibular nerve (CFN) injury due to ankle fracture is an underreported complication. The authors have proposed that torsional injury to the ankle can be translated along the interosseous membrane (IOM), producing tension on the CFN at the fibular neck. A 23-year-old woman presented to our clinic for left foot drop. Three months prior, the patient sustained a fall with left ankle inversion injury while running. She was diagnosed with a minor ankle fracture and placed in an orthopaedic boot. Unfortunately, her swelling worsened and one week later the patient was diagnosed with foot drop, which was further corroborated with EMG studies showing severe CFN injury localizing to the fibular neck. Because of the lack of recovery, she underwent decompression of the CFN. She experienced immediate symptomatic relief. High resolution imaging in this case supports our previous mechanism for indirect trauma to the ankle resulting in CFN injury. (Journal of Surgical Orthopaedic Advances 33(1):053-055, 2024).

Plexal intraneural perineurioma: an analysis of the clinicoradiologic presentation of this rare variant.

PURPOSE: Intraneural (IN) perineurioma is a rare benign peripheral nerve sheath tumor, typically presenting as a painless, progressive mononeuropathy in adolescents. A rare plexal variant has been described, although there are little data describing its clinicoradiologic features. Herein, we present the largest case series of plexal IN perineuriomas reported in the literature. METHODS: Electronic medical records (EMR) from 1990 to 2022 from a single academic institution were reviewed for a diagnosis of IN perineurioma involving the brachial or lumbosacral plexus. This identified 18 patients, of which 17 had available MR imaging. We reviewed the EMR for demographics, clinical presentation, imaging characteristics, and surgical outcomes. RESULTS: Eighteen patients were identified. Most patients were male (11/18, 61%) and first developed symptoms at the age of 9.6 years (range 7 months to 55 years). Diagnosis occurred on average at the age of 22 years (4-57 years), which is significantly earlier than distal IN perineurioma (p = 0.0096). All patients (100%, 17/17) presented with motor polyneuropathy and muscular atrophy in multiple nerve distributions, with associated sensory loss (12/17, 71%). Most plexal lesions occurred in the brachial plexus (66%, 12/18). Five (29%, 5/17) patients presented with a hand/foot discrepancy, and 5 patients (29%) had a limb length discrepancy. Five patients underwent tendon transfer, of which two had failure of tendon transfer at most recent follow-up (50%, 2/4) due to progression of neurologic loss affecting the donors. Of patients managed nonoperatively, 87% of patients (6/7) with follow-up information demonstrated disease progression with worsening motor function or sensory loss, and 2 patients demonstrated progression on imaging at most recent follow-up. CONCLUSIONS: Plexal perineurioma represents an uncommon variant of IN perineurioma that presents as a progressive motor and sensory polyneuropathy in childhood or early adolescence. Surgical management remains controversial, and tendon transfer tends to result in poor long-term surgical outcomes.

Hybrid tumors with perineurioma components: a systematic review of the literature and illustrative case.

PURPOSE: Hybrid peripheral nerve sheath tumors (HPNST) are a newly recognized class of peripheral nerve sheath tumor, composed of at least two areas characteristic of perineurioma, schwannoma, or neurofibroma. The literature consists only of case reports and small series; therefore, we present an illustrative case and an analysis of all reported cases of HPNST with a perineurioma component in the literature. METHODS: A systematic search of the literature was performed to identify all reported cases of hybrid perineurioma-schwannoma or perineurioma-neurofibroma in the world's literature. Individual cases were analyzed for demographics, clinical features, imaging, and outcomes. RESULTS: A total of 159 cases were identified across 41 studies. Hybrid tumors tended to present in mid-adulthood (median 38.5 years), predominantly affected females (57%, 89/156), as a painless (63%, 63/100) mass, or swelling. Ten patients (10/74, 14%) had a history of neurofibromatosis 1, and 2 patients a history of neurofibromatosis 2 (2/74, 3%). The majority (78%, 122/157) of cases occurred superficially, most commonly in the lower extremity (25%, 39/157). Perineurioma-schwannoma was the most reported (86%, 137/159) pathologic diagnosis, with 3 cases presenting with malignant features. Two cases reocurred after resection. CONCLUSION: HPNST tend to occur in mid-adulthood and present as slowly progressive, painless, superficial masses, with a heterogeneous appearance on imaging. These entities pose a unique diagnostic challenge and likely remain under-recognized in the literature and current clinical practice. They pose low risk of recurrence or malignant transformation, and future work regarding the association with neurofibromatosis and genetic profiles is needed.

PET imaging characteristics of neuromuscular choristoma and associated desmoid-type fibromatosis.

BACKGROUND: Neuromuscular choristoma (NMC) is a rare peripheral nerve lesion characterized by abnormal presence of muscle within nerve. Associated desmoid-type fibromatosis (NMC-DTF) often develops. We report 18F-fluorodeoxyglucose positron emission tomography (FDG PET) characteristics of NMC and NMC-DTF and propose that increased FDG activity within NMCs may be associated with subclinical NMC-DTF or NMC-DTF "precursor" tissue. METHODS: Our institutional database was searched for all NMC cases. Inclusion criteria were 1) confirmed diagnosis of NMC with or without biopsy, and 2) available PET and MRI studies. PET data included SUVmax and SUVmean of NMCs, contralateral limb normal skeletal muscle and unaffected nerves, and SUVmax of NMC-DTF if present. SUV values were compared using paired t-test. A p value of < 0.05 was considered statistically significant. RESULTS: Our cohort consisted of 9 patients with NMC, 8 cases involving sciatic nerve and 1 of brachial plexus. On PET imaging, all NMC-affected nerve segments showed significantly higher FDG uptake (SUVmax/mean) compared to both contralateral normal nerve and normal skeletal muscle (all P < 0.05). Similar to sporadic DTF, NMC-DTF was highly FDG-avid (average SUVmax of 4.2). SUVmax in NMC with or without concurrent NMC-DTF did not differ (p = 0.76). Within NMC-affected nerve segment, FDG activity was relatively higher in areas with low T1/T2 MR signal. CONCLUSION: All NMCs were more FDG avid compared to both normal skeletal muscle and contralateral unaffected nerve, arguing against the presence of heterotopic muscle in NMC as the source of FDG avidity. FDG avidity within NMC may reflect subclinical NMC-DTF or a precursor lesion, as NMC-DTF are highly FDG-avid, and the highest regions of FDG avidity in NMC occurred in regions with MR characteristics associated with NMC-DTF (i.e., lower T1/T2 signal). We believe that the integration of FDG PET with serial MR imaging in patient follow up will clarify its utility in both detection and surveillance of NMC-DTF.

Tibial intraneural ganglion cysts at the superior tibiofibular joint treated with joint resection alone: a proof of concept.

BACKGROUND: Intraneural ganglion cysts involving the tibial nerve are rare. Recent evidence has supported an articular (synovial) theory to explain the joint-related origin of these cysts; however, optimal operative treatment for cysts originating from the STFJ remains poorly understood. Therefore, we present a novel strategy: addressing the joint itself without addressing the articular branch and/or the cyst. METHODS: Records of patients with tibial intraneural ganglion cysts with a connection to the STFJ who were treated with a joint resection alone at a single academic institution were reviewed. The clinicoradiographic features, operative intervention, and postoperative course were recorded. RESULTS: We identified a consecutive series of 7 patients. These patients (4/7 male, 57%) were 43 (range 34-61) years of age and all presented with symptoms of neuropathy. The patients underwent resection of the synovial surfaces of the STFJ without disconnection of the articular branch or decompression of the cyst. Postoperatively, three patients regained partial motor function (43%, n=7), although four patients noted continued sensory abnormality (57%, 4/7). All six patients with postoperative MRIs had some evidence of regression of the cyst. CONCLUSIONS: This novel surgical technique serves as a proof of concept-highlighting the fact that treating the primary source (the joint origin) can be effective in eliminating the secondary problem (the cyst itself). While this study shows that this simplified approach can be employed in select cases, we believe that superior results (faster, fuller recovery) can be achieved with combinations of disconnecting the articular branch, decompressing the cyst, and/or resecting the joint.

What is new in intraneural perineurioma?

Since the initial description of intraneural (IN) perineurioma in 1964, advances in the understanding of the clinical presentation, diagnostic imaging, pathologic features, and genetic underpinnings have changed how this pathology is managed. IN perineuriomas are rare, benign peripheral nerve sheath tumors, most frequently coming to clinical attention when patients present with painless, progressive weakness or sensory loss in adolescence or young adulthood. The gold standard of diagnosis has traditionally been with targeted tissue biopsy demonstrating "pseudo-onion bulb" formation with positive epithelial membrane antigen (EMA) staining. However, modern magnetic resonance imaging is allowing some patients to forgo biopsy. Recent genetic studies of IN perineuriomas have demonstrated common TRAF7 point mutations and rare NF2 mutations, which may present targets for diagnosis or therapy in the future. Current advances have allowed for us to provide improved patient counseling with informed understanding for various clinical scenarios. With the workup and diagnosis now clearly defined, the next frontier is for improving the lives of patients with IN perineuriomas through the interaction between restoration of functional deficits and advances in our understanding of the genetics of this entity.

7T for clinical imaging of benign peripheral nerve tumors: preliminary results.

PURPOSE: MRI has become an essential diagnostic imaging modality for peripheral nerve pathology. Early MR imaging for peripheral nerve depended on inferred nerve involvement by visualizing downstream effects such as denervation muscular atrophy; improvements in MRI technology have made possible direct visualization of the nerves. In this paper, we share our early clinical experience with 7T for benign neurogenic tumors. MATERIALS: Patients with benign neurogenic tumors and 7T MRI examinations available were reviewed. Cases of individual benign peripheral nerve tumors were included to demonstrate 7T MRI imaging characteristics. All exams were performed on a 7T MRI MAGNETOM Terra using a 28-channel receive, single-channel transmit knee coil. RESULTS: Five cases of four pathologies were selected from 38 patients to depict characteristic imaging features in different benign nerve tumors and lesions using 7T MRI. CONCLUSION: The primary advantage of 7T over 3T is an increase in signal-to-noise ratio which allows higher in plane resolution so that the smallest neural structures can be seen and characterized. This improvement in MR imaging provides the opportunity for more accurate diagnosis and surgical planning in selected cases. As this technology continues to evolve for clinical purposes, we anticipate increasing applications and improved patient care using 7T MRI for the diagnosis of peripheral nerve masses.

Nerve relationships to the sacrospinous ligament: application to suspension procedures and transgluteal approaches for nerve repair and tumor removal with three case illustrations.

BACKGROUND: Transvaginal suspension procedures often use the sacrospinous ligament (SSL), which attaches onto the ischial spine (IS). However, nerve-related sequelae (e.g., sciatic nerve injury) following such procedures have been reported. Therefore, the current anatomical study was performed to better understand these relationships. Additionally, three case illustrations of patients with injury to the sciatic nerve following sacrospinous ligament suspension procedures are included to exemplify the significance of a thorough knowledge of this anatomy. METHODS: In 20 human adult cadavers (40 sides), a gluteal dissection was performed to expose the IS and SSL and regional nerves near the greater sciatic foramen. Measurements between the IS and SSL were made between these structures and surrounding nerves. RESULTS: The average distance between the IS and sciatic nerve was 1.4 cm. From this bony part, the average distance to the S1 and S2 ventral rami was 3.1 cm and 1.9 cm, respectively. From the IS to the lumbosacral trunk, pudendal nerve, nerve to obturator internus, and superior gluteal nerve, the mean distance was 4 cm, 0.5 cm, 0.7 cm, and 4.5 cm, respectively. From the SSL to the lumbosacral trunk, S1 ventral ramus, and S2 ventral ramus, there was an average distance of 4.2 cm, 1.6 cm, and 0.8 cm, respectively. Statistically, in females, the distances from the IS and SSL to the sciatic nerve, lumbosacral trunk, superior gluteal nerve, and S1 and S2 ventral rami were shorter when compared to males. CONCLUSION: An improved understanding of the relationship between the SSL and IS and nerves near the greater sciatic foramen can lead to fewer intraoperative complications during approaches to various peripheral nerves in this region. Lastly, these relationships might help better understand the nerve injuries following pelvic suspension procedures that use the SSL.

Does complete regression of intraneural ganglion cysts occur without surgery?

PURPOSE: The dynamic nature of intraneural ganglion cysts, including spontaneous expansion and regression, has been described. However, whether these cysts can regress completely in the absence of surgical management has important therapeutic implications. Therefore, we aim to review the literature for cyst regression without surgical intervention. METHODS: We reviewed our database of 970 intraneural ganglion cysts in the literature to search for evidence of complete regression based on strict radiologic confirmation, either spontaneously, or after percutaneous cyst aspiration or steroid injection. RESULTS: We did not find any examples of complete regression without surgical treatment that met inclusion criteria. Spontaneous regression was reported in four cases; however, only two cases had follow-up imaging, both of which demonstrated residual cysts. Nineteen cases of percutaneous intervention were found in the literature, 13 of which reported clinical improvement following aspiration/steroid injection; however, only seven cases had available imaging. Only two cases reported complete resolution of cyst on MR imaging at follow-up, but reinterpretation found residual intraneural cyst in both cases. CONCLUSION: We believe that pathology (structural abnormalities and/or increased joint fluid) always exists at the joint origin of intraneural ganglion cysts which persist even with regression of the cyst. The persistence of a capsular abnormality or defect can lead to recurrence of the cyst in the future, and while imaging may show dramatic decreases in cyst size, truly focused assessment of images will show a tiny focus of persistent intraneural cyst at the joint origin. Thus, expectant management or percutaneous intervention may lead to regression, but not complete resolution, of intraneural ganglion cysts.

Radiological Prevalence of Popliteal Artery Entrapment in Individuals With Anterior Leg Compartment Chronic Exertional Compartment Syndrome.

OBJECTIVE: Determine the radiological prevalence of popliteal artery entrapment (PAE) in subjects with anterior leg compartment chronic exertional compartment syndrome (CECS). DESIGN: Retrospective review. SETTING: Tertiary care center. PATIENTS: Of 71 patients diagnosed with anterior leg compartment CECS using an in-scanner exercise-based magnetic resonance imaging (MRI), 64 also completed Fast Imaging Employing Steady-State Acquisition (FIESTA) imaging. INTERVENTIONS: Electronic health records of patients diagnosed with anterior leg compartment CECS using an in-scanner exercise-based MRI between 2009 and 2018 were reviewed. MAIN OUTCOME MEASURES: Demographics, symptom laterality, and results of vascular work-up. RESULTS: Magnetic resonance imaging was positive for PAE in 33 of 64 (51.6%). Vascular evaluation was performed in 30 of 33 (90.9%). Of these 30, ankle-brachial indices (ABIs) with PAE maneuvers were performed in 29 (96.7%) and positive in 25 (86.2%). Pre-exercise and post-exercise ABIs were performed in 29 (96.7%) and abnormal in 20 (69.0%). Thirteen arterial duplex ultrasounds were performed; 10 were consistent with PAE (76.9%). An MR angiogram was performed in 8 (26.7%) and consistent with PAE in all. One computed tomography angiogram (3.3%) was completed and was normal. Overall, one or more tests were positive for PAE in all 30 with vascular evaluation. CONCLUSIONS: The radiological prevalence of PAE and anterior leg CECS was 51.6%. All subjects with vascular studies (90.9%) had one or more tests confirming radiological PAE. These findings suggest that the coexistence of PAE and CECS is common, and the PAE protocol used has a high correlation with vascular studies.

What's known and what's new in adipose lesions of peripheral nerves?

BACKGROUND: Adipose lesions of nerve primarily include intra- and extraneural lipomas and lipomatosis of nerve (LN). This paper will summarize the advances that have been made in the past decade, particularly related to LN and nerve territory overgrowth that have improved our understanding of the natural history, genetic background, diagnosis, imaging features, and clinical management. METHODS AND MATERIALS: Articles about adipose lesions of nerve were reviewed from 2011, when the last comprehensive review on this topic was published. Papers reporting advances on natural history, genetic background, diagnosis, imaging features, and clinical management were screened using PubMed and Google Scholar databases and then analyzed. Case reports and small case series were included only if they reported model examples of discussed pathologies, as these types of articles were summarized in recent systematic reviews on intraneural lipomas and LN. All eligible papers were assessed by the authors, who are subject matter experts. RESULTS: The first screen revealed 404 articles. After careful evaluation, a total of 53 articles were analyzed which includes advances in diagnosis (especially imaging), classification of the lesions, the role of somatic mutations in PIK3CA in LN, and treatment approaches for all adipose lesions of the peripheral nerve. CONCLUSION: Many advances have been made in the understanding of adipose lesions of nerve in the past decade. These pathologic entities are more readily recognized as a spectrum of lesions that share common phenotypic features.

Bilateral Nerve Involvement in Lipomatosis of Nerve.

Lipomatosis of nerve (LN) is an intriguing pathological entity defined by the abundance of fibro-adipose tissue within the epineurium, a pathognomonic magnetic resonance imaging (MRI), and frequently associated nerve-territory overgrowth. A recent systematic review showed that the majority of cases are unilateral and predominately involve the median nerve. We reviewed bilateral cases of LN to further understand this pathology. We conducted a review of the literature and our institutional databases. The cases from the literature were sorted into three groups - bilaterally confirmed LN (n = 9), unilaterally confirmed, opposite side probable LN (n = 4), and probable bilateral LN (n = 10). Review of our institutional databases identified one case: a 47-year-old man. MRI revealed LN of the brachial plexus bilaterally. To our knowledge, this is the first reported case of brachial plexus LN occurring bilaterally. More research is necessary on this topic to further understand the genetic background of this entity, particularly in relationship to the overgrowth. (Journal of Surgical Orthopaedic Advances 30(1):044-049, 2021).

Perineural spread to the brachial plexus: a focused review of proposed mechanisms and described pathologies.

BACKGROUND: Perineural spread (PNS) is an emerging mechanism for progressive, non-traumatic brachial plexopathy. We aim to summarize the pathologies (tumor and infection) shown to have spread along or to the brachial plexus, and identify the proposed mechanisms of perineural spread. METHODS: A focused review of the literature was performed pertaining to pathologies with identified perineural spread to the brachial plexus. RESULTS: We summarized pathologies currently reported to have PNS in the brachial plexus and offer a structure for understanding and describing these pathologies with respect to their interaction with the peripheral nervous system. CONCLUSIONS: Perineural spread is an underrepresented entity in the literature, especially regarding the brachial plexus. It can occur via a primary or secondary mechanism based on the anatomy, and understanding this mechanism helps to support biopsies of sacrificial nerve contributions, leading to more effective and timely treatment plans for patients.

Circumdural extension of perineural spread leading to bilateral disease in neurolymphomatosis.

INTRODUCTION: Patients with neurolymphomatosis (NL) often present with one primarily symptomatic limb but can be found to have bilateral upper or bilateral lower limb disease during workup. We sought to explain the finding of bilateral disease and understand if there was a connection to the initial, symptomatic side of disease. METHODS: We reviewed imaging studies of patients with bilateral upper or bilateral lower limb disease from a previously published cohort from our institution, as well as more recent patients seen at our institution. We reviewed demographics (sex and age), clinical data (primary or secondary disease and biopsy-proven diagnosis), and imaging findings (primary involved nerve, contralateral nerve(s) affected, and location of circumdural extension). RESULTS: We identified 8 cases with evidence of bilateral disease out of 22 cases of tumefactive NL. All eight cases were found to have circumdural extension of disease to the corresponding contralateral nerve. CONCLUSION: We describe the pathomechanism of spread in our cases of bilateral upper or bilateral lower limb disease, where NL spreads along a dominant nerve toward the spinal canal and moves circumdurally to affect the corresponding contralateral nerve. We believe this information is useful to further understand the spread of NL, as well as offering important diagnostic and prognostic information for patients.

Uniform combined reconstruction of multichannel 7T knee MRI receive coil data without the use of a reference scan.

BACKGROUND: MR image intensity nonuniformity is often observed at 7T. Reference scans from the body coil used for uniformity correction at lower field strengths are typically not available at 7T. PURPOSE: To evaluate the efficacy of a novel algorithm, Uniform Combined Reconstruction (UNICORN), to correct receive coil-induced nonuniformity in musculoskeletal 7T MRI without the use of a reference scan. STUDY TYPE: Retrospective image analysis study. SUBJECTS: MRI data of 20 subjects was retrospectively processed offline. Field Strength/Sequence: Knees of 20 subjects were imaged at 7T with a single-channel transmit, 28-channel phased-array receive knee coil. A turbo-spin-echo sequence was used to acquire 33 series of images. ASSESSMENT: Three fellowship-trained musculoskeletal radiologists with cumulative experience of 42 years reviewed the images. The uniformity, contrast, signal-to-noise ratio (SNR), and overall image quality were evaluated for images with no postprocessing, images processed with N4 bias field correction algorithm, and the UNICORN algorithm. STATISTICAL TESTS: Intraclass correlation coefficient (ICC) was used for measuring the interrater reliability. ICC and 95% confidence intervals (CIs) were calculated using the R statistical package employing a two-way mixed-effects model based on a mean rating (k = 3) for absolute agreement. The Wilcoxon signed-rank test with continuity correction was used for analyzing the overall image quality scores. RESULTS: UNICORN was preferred among the three methods evaluated for uniformity in 97.9% of the pooled ratings, with excellent interrater agreement (ICC of 0.98, CI 0.97-0.99). UNICORN was also rated better than N4 for contrast and equivalent to N4 in SNR with ICCs of 0.80 (CI 0.72-0.86) and 0.67 (CI 0.54-0.77), respectively. The overall image quality scores for UNICORN were significantly higher than N4 (P < 6 × 10-13 ), with good to excellent interrater agreement (ICC 0.90, CI 0.86-0.93). DATA CONCLUSION: Without the use of a reference scan, UNICORN provides better image uniformity, contrast, and overall image quality at 7T compared with the N4 bias field-correction algorithm. LEVEL OF EVIDENCE: 4 Technical Efficacy: Stage 1 J. Magn. Reson. Imaging 2019;50:1534-1544.

Lipomatosis of nerve and overgrowth: is there a preference for motor (mixed) vs. sensory nerve involvement?

BACKGROUND: Lipomatosis of nerve (LN) is a peripheral nerve disorder characterized by fibroadipose proliferation within the epineurium. It has been associated with nerve-territory overgrowth affecting soft tissue and/or bony structures. We sought to understand if there is an anatomical relationship associated with nerve-territory overgrowth. METHODS: A review of the literature and our institutional LN cases was performed to determine the prevalence of nerve-territory overgrowth. Only cases with sufficient clinical and/or imaging data were selected. The cases were then subdivided into two groups and analyzed: (1) motor (mixed) nerve and (2) predominant sensory nerve, based on the anatomical location of the LN lesion. Subgroup analysis was performed on median nerves affected by LN, for a more homogenous population. RESULTS: We identified 329 LN cases with sufficient information for analysis. Motor (mixed) nerve group (M) consisted of 287 cases (155 with overgrowth and 132 without overgrowth). Sensory nerve group (S) revealed group of 42 cases (4 cases with overgrowth and 38 without overgrowth). Statistical analysis comparing overgrowth status in the M and S nerve groups showed a statistically significant difference in overgrowth, favoring the M group for overgrowth (p < 0.0001). The analysis of median nerve group consisted of 225 cases in the M group (106 with overgrowth and 119 without overgrowth) and 20 cases in the S group (3 with overgrowth and 17 cases without overgrowth). A statistically significant difference in nerve-territory overgrowth status was present in the M vs. the S group, again favoring the M group for overgrowth. (p = 0.0083). Cases from our institution included 44 cases for this analysis. Forty-two cases in the M group (28 with overgrowth and 14 without overgrowth) and 2 cases in the S group (all 2 without overgrowth). CONCLUSION: We believe the association of LN and nerve-territory overgrowth might be explained by involvement of mixed motor nerves; however, the exact underlying mechanism is not known.

Adventitial cysts of the radial artery are joint connected.

Volar radial wrist masses are common. Adventitial cysts of the radial artery are rarely reported and poorly understood. We describe a case series of adventitial cysts in association with the radial artery and detail their pathophysiology and treatment. We conducted an Institutional Review Board-approved retrospective review of patients treated at our institution from 1997 to 2018. Twelve patients were identified. Presenting symptoms typically included pain and swelling over the volar radial wrist. High-resolution magnetic resonance imaging (MRI) demonstrated tubular, cystic lesions within the adventitia of the radial artery with connections to the wrist joint confirmed on multiplanar imaging: (radiocarpal joint = 10; scaphotrapeziotrapezoidal joint = 1; and intercarpal joint = 1). Seven patients underwent operation, at which time the cyst was resected and the articular branch disconnected. These patients reported resolution of their symptoms without clinical recurrence. The consistent finding of a joint connection in these cases of adventitial cysts associated with the radial artery has important clinical implications. The joint connection needs to be disconnected. Level of evidence: Level IV, case series. Clin. Anat. 32:201-205, 2019. © 2018 Wiley Periodicals, Inc.

Innervation of the anconeus epitrochlearis muscle: MRI and cadaveric studies.

Ulnar neuropathy at the cubital tunnel is common. However, a rare form of ulnar neuropathy here is due to compression from an accessory muscle, the anconeus epitrochlearis. Reports in the literature regarding the details of this muscle's innervation are vague, so the aim of the present study was to characterize this anatomy more clearly. This was a combined review of magnetic resonance imaging (MRI) from patients with an anconeus epitrochlearis and ulnar neuropathy and cadaveric dissections to characterize the innervation of this variant muscle. A review of 11 patients and three reports of ulnar neuropathy and an anconeus epitrochlearis in the literature revealed no MRI changes consistent with acute denervation of this muscle. However, in two cases, there were signs of chronic denervation of the muscle. Dissection of five cadavers revealed that the nerve supply to the anconeus epitrochlearis originated proximal to the medial epicondyle, traveled parallel to the ulnar nerve, terminated on the deep aspect of this muscle, and had a mean length of 60 mm. This clinicoanatomical study provides evidence that the innervation of the anconeus epitrochlearis is proximal to the muscle and on its deep aspect. Clin. Anat. 32:218-223, 2019. © 2018 Wiley Periodicals, Inc.

Cystic Adventitial Disease of the Tibial Vein Arising From the Subtalar Joint: A Case Report.

Soft tissue ganglion cysts are a well-known cause of tibial nerve compression in the tarsal tunnel. We describe a patient who presented with tibial nerve symptoms and was found to have an adventitial cyst of the tibial vein arising from the subtalar joint, with the joint connection confirmed both on imaging and at surgery. Surgical decompression of the cyst with transection of the vascular pedicle arising from the subtalar joint improved her symptoms at 6 months, and postoperative magnetic resonance imaging showed resolution of the cyst. Cystic adventitial disease is a rare, poorly understood condition in which a cyst is identified in the adventitia of a vessel, usually an artery. Only 3 cases of adventitial cysts have been reported in the foot and ankle region, 2 in the lesser and 1 in the greater saphenous vein. None of the previous cases have been recognized to be joint connected. This case provides additional evidence for an articular origin for adventitial cysts and helps guide management strategies for these joint-connected cysts.

Low-grade fibromyxoid sarcoma arising within the median nerve.

We report a case of low-grade fibromyxoid sarcoma arising within the median nerve. A 31-year-old woman presented with symptoms of carpal tunnel syndrome and an enlarging mass in her right palm over 1 year. MRI demonstrated a mass associated with the right median nerve with solid and cystic components. At surgery, the mass was located within the epineurium, could be bluntly dissected from the nerve fascicles, and was suspected to be a schwannoma. A 3.4 cm, tan-pink, glistening, smooth, homogenous mass was submitted to pathology. Microscopically, the tumor was a solid and cystic circumscribed nodule with a dense fibrous pseudocapsule. The tumor cells were uniformly bland and spindle-shaped, with small, hyperchromatic oval nuclei and were embedded in an alternating fibrous and myxoid stroma with a prominent curvilinear vasculature and perivascular sclerosis. The differential diagnosis for this lesion included myxoid neurofibroma, schwannoma, soft tissue perineurioma, low-grade malignant peripheral nerve sheath tumor and low-grade fibromyxoid sarcoma. The tumor cells expressed MUC4, GLUT-1, and vimentin and were negative for S-100 protein, epithelial membrane antigen, smooth muscle actin, desmin, claudin-1, neurofilament and SOX10. Fluorescence in situ hybridization, with a break-apart probe strategy, demonstrated FUS rearrangement, consistent in this morphological context with the low-grade fibromyxoid sarcoma-associated FUS-CREB3L2 or FUS-CREB3L1 fusions. Low-grade fibromyxoid sarcoma is exceptionally rare in the peripheral nerve, with only a single previously reported case. Nonetheless, as our case illustrates, this entity must be included in the differential diagnosis of unusual intraneural mesenchymal tumors. As in all other locations, intraneural low-grade fibromyxoid sarcomas should be excised with negative margins. Patients with this disease require long-term clinical follow-up, given this tumor's propensity for very late distant metastases to the lungs and other sites.