Primary peripheral nerve lymphoma: a case report and literature review.

Neurolymphomatosis (NL) is an uncommon malignant lymphoma characterized by selective infiltration of the central and peripheral nervous system. In this case report, we present a patient diagnosed with diffuse large B-cell lymphoma who initially manifested with peripheral neuropathy, primarily characterized by weakness of the left lower limb. By exploring its clinical manifestations, ancillary tests, and reviewing the relevant literature, we aim to deepen our understanding, diagnosis, and treatment of this disease. A 48-year-old male patient presented to the Department of Neurology, Hematology, and Neurosurgery with complaint of left lower limb weakness that had persisted for over 11 months. Initial laboratory tests and cerebrospinal fluid analysis yielded negative results. Electromyography examination indicated damage to the left lumbar plexus and iliac plexus nerves raising suspicions of nerve root involvement. Enhanced MRI of the lumbosacral plexus nerves revealed thickening and enhanced signals in left nerve roots at T12-L1, L1-2, and L3-4 levels. Additionally, local thickening and enhancement of signals were observed in the left erector spine muscle, psoas major, and iliopsoas muscles compared to the contralateral side. PEC/CT imaging displayed multiple soft tissue density shadows in the left foraminal area at the T12-1 and L1-2 levels. Bone marrow examination excluded hematological disease. Subsequent biopsy of the left foraminal nerve root at T12-L1 and the vertebral muscle at L3 level confirmed a diagnosis of diffuse large B-cell malignant lymphoma, indicating PNSL due to the involvement of multiple nerve roots. Following diagnosis, the patient underwent chemotherapy, resulting in the alleviation of his symptoms. Diagnosing PNSL can be challenging due to the nonspecific clinical manifestations and often inconclusive laboratory test results. Misdiagnosis and delayed diagnosis are common pitfalls. Electromyography may reveal damage to the affected peripheral nerves, while MR imaging might show nerve root thickening, and PET/CT can demonstrate increased lesion uptake. However, the definitive diagnosis relies on a biopsy of the lesion. Treatment for PNSL typically involves chemotherapy.

Rapidly Growing Leiomyoma Mimicking Schwannoma of the Saphenous Nerve in the Lower Extremity: An Unusual Case Report.

Leiomyomas and schwannomas are both types of rare benign soft tissue tumours. Leiomyomas are more commonly found in the lower limbs than in the upper extremities, while schwannomas are rare peripheral nerve sheath tumours that can occur in different anatomical regions. However, they rarely occur in the saphenous nerve. This case study presents a 41-year-old female patient with a solitary mass lesion located deep in the soft tissue of the anteromedial lower extremity. The physical examination revealed a palpable, elastic-hard, mobile and non-tender mass. Magnetic resonance imaging (MRI) showed an oval-shaped subcutaneous mass on contrast-enhanced T1-weighted sections. The initial MRI images suggested a schwannoma, but the tumour was later confirmed to be a leiomyoma after total enucleation. An immunohistochemical study was performed for differential diagnosis. Solitary mass lesions in the lower extremities can be mistaken for various types of tumours and misdiagnosed and require histopathological examination and good radiological imaging for differential diagnosis. Complete surgical excision is usually a safe and effective treatment for leiomyomas.

Pediatric fibromyxoid brachial plexus tumor with YWHAZ::PLAG1 gene fusion: a case report.

Pediatric fibromyxoid soft tissue tumors may be associated with gene fusions such as YHWAZ::PLAG1, with only three reported cases in the literature. We present the fourth case, a 13-year-old male with a pediatric fibromyxoid brachial plexus tumor with YWHAZ::PLAG1 gene fusion. This is also the first case to be reported in an adolescent, in the brachial plexus, and in the Philippines. The patient presented with a 10-year history of a slowly growing left supraclavicular mass and a 1-year history of intermittent dysesthesia in the left upper extremity. Neurologic examination was unremarkable. Imaging revealed a large left supraclavicular lesion with intrathoracic extension. Surgical excision was performed, and histopathology revealed a fibromyxoid tumor with YWHAZ::PLAG1 gene fusion. Although previous examples of this gene fusion pointed toward lipoblastoma as their primary pathology, our tumor does not completely fulfill the current diagnostic criteria for a lipoblastoma and may represent an intermediate form of the disease. Our case is unique not only because it is the first reported adolescent patient harboring such a lesion but also because of the relatively lengthy natural history exhibited by the tumor prior to its resection. This provided us with valuable information about its behavior, which suggests a more indolent growth pattern. This case also highlights the clinical importance of molecular testing of tumors, where recognition of disease entities can assist clinicians in deciding and advocating for the proper management.

MRI features of benign peripheral nerve sheath tumors: how do sporadic and syndromic tumors differ?

OBJECTIVES: To compare MRI features of sporadic and neurofibromatosis syndrome-related localized schwannomas and neurofibromas. METHODS: In this retrospective study, our pathology database was searched for "neurofibroma" or "schwannoma" from 2014 to 2019. Exclusion criteria were lack of available MRI and intradural or plexiform tumors. Qualitative and quantitative anatomic (location, size, relationship to nerve, signal, muscle denervation) and functional (arterial enhancement, apparent diffusion-weighted coefficient) MRI features of sporadic and syndrome-related tumors were compared. Statistical significance was assumed for p < 0.05. RESULTS: A total of 80 patients with 64 schwannomas (sporadic: 42 (65.6%) v. syndrome-related: 22 (34.4%)) and 19 neurofibromas (sporadic: 7 (36.8%) v. syndrome-related: 12 (41.7%)) were included. Only signal heterogeneity (T2W p=0.001, post-contrast p=0.03) and a diffused-weighted imaging target sign (p=0.04) were more frequent with schwannomas than neurofibromas. Sporadic schwannomas were similar in size to syndrome-related schwannomas (2.9±1.2cm vs. 3.7±3.2 cm, p = 0.6), but with greater heterogeneity (T2W p = 0.02, post-contrast p = 0.01). Sporadic neurofibromas were larger (4.6±1.5cm vs. 3.4±2.4 cm, p = 0.03) than syndrome-related neurofibromas, also with greater heterogeneity (T2W p=0.03, post-contrast p=0.04). Additional tumors along an affected nerve were only observed with syndrome-related tumors). There was no difference in apparent diffusion coefficient values or presence of early perfusion between sporadic and syndrome-related tumors (p > 0.05). CONCLUSIONS: Although syndrome-related and sporadic schwannomas and neurofibromas overlap in their anatomic, diffusion and perfusion features, signal heterogeneity and presence of multiple lesions along a nerve are differentiating characteristics of syndrome-related tumors.

Excision of large median and ulnar nerve schwannomas: a case series.

OBJECTIVE: Schwannomas are benign, slow-growing tumors originating from Schwann cells in peripheral nerves, commonly affecting the median and ulnar nerves in the forearm and wrist. Surgical excision is the gold standard treatment. This study presents our treatment strategies and outcomes for large-sized ulnar and median nerve schwannomas at the forearm and wrist level. METHODS: From 2012 to 2023, we enrolled 15 patients with schwannomas over 2 cm in size in the median or ulnar nerve at the forearm and wrist. The study included 12 patients with median nerve schwannomas (mean age: 61 years) and 3 with ulnar nerve schwannomas (mean age: 68 years), with a mean follow-up of 26.9 months. RESULTS: After surgery, all patients with median nerve schwannomas experienced mild, transient numbness affecting fewer than two digits, resolving within six months without motor deficits. Ulnar nerve schwannoma excision caused mild numbness in two patients, also resolving within six months, but all three developed ulnar claw hand deformity, which persisted but improved at the last follow-up. Despite this, patients were satisfied with the surgery due to relief from severe tingling pain. CONCLUSIONS: Schwannomas of the median, ulnar, and other peripheral nerves should be removed by carefully dissecting the connecting nerve fascicles to avoid injury to healthy ones. Sensory deficits may occur but are unlikely to significantly impact quality of life. However, in motor-dominant nerves like the ulnar nerve, there is a risk of significant motor deficits that could affect hand function, though not completely. Therefore, thorough preoperative discussion and consideration of interfascicular nerve grafting are essential.

Myxofibrosarcoma involving the sciatic nerve: From ultrasonography to histopathology.

Myxofibrosarcoma (MFS) is a unique soft tissue (fibroblastic) sarcoma, characterized by malignant with a locally infiltrative behavior. In this case image, we aimed to report an uncommon/challenging scenario of MFS involving the scatic nerve in which the potential contribution of ultrasound examination has been touched upon.

Management of an Early-Onset, Painful Tibial Nerve Neuroma Using an Autologous Nerve Graft.

This case report describes the treatment of a postoperative painful neuroma of the tibial nerve using an autologous nerve graft in a dog. The patient presented with sudden non-weight-bearing lameness 10 days after iatrogenic tibial nerve injury during preparation of a reverse saphenous conduit flap. The dog showed severe pain at the surgical site without nerve deficits. A magnetic resonance imaging examination revealed an enlarged tibial nerve at the injury site, consistent with a neuroma. Analgesics were administered over 11 days, but the patient remained in severe pain and non-weight-bearing. Therefore, surgical resection was recommended. The fusiform neuroma was resected microsurgically, and a saphenous nerve graft was transplanted using an epineural nerve repair technique. Histopathological examination was consistent with a neuroma. The dog showed immediate pain relief and weight-bearing the day after surgery with normal motor function. The dog made a full recovery by the last follow-up 6 mo after surgery. If patients develop pain and lameness following surgery or nerve injury, neuroma formation must be considered, even shortly after surgery. Microsurgical resection and autologous nerve transplantation using an epineural nerve repair technique is a viable method to treat painful neuromas and minimize the risk for recurrence in dogs.

[Schwannoma of Intercostal Nerve Resulting in Significant Pain Relief Following Excision:Report of a Case].

An 80-year-old male underwent chest computed tomography (CT) due to complaints of right-sided chest pain. A chest wall tumor was identified in the right eighth intercostal space, corresponding to the location of his pain. The patient was subsequently referred to our department for further evaluation and treatment. Utilizing single-port thoracoscopic surgery, the tumor was successfully excised. Intraoperatively, the tumor was found beneath the parietal pleura, was contiguous with to the intercostal nerve. Histopathological analysis confirmed the diagnosis of schwannoma originating from the intercostal nerve. The right-sided chest pain was reduced after operation significantly. No recurrence or relapse of symptoms was observed during follow-up. Although schwannoma of the chest wall in asymptomatic in many cases, in this case, localized pain corresponding to the tumor site was evident. This emphasizes the importance of considering schwannoma in the differential diagnosis of chest pain.

Giant Common Peroneal Nerve Schwannoma Mimicking Synovial Sarcoma: An Unusual Case Report.

Schwannoma, also known as neurilemmoma or Schwann cell tumor, is one of the most common neoplasms of the nerve sheath which usually appears at the head, neck, or upper extremity. Schwannoma occurrence in the lower extremity originating from the common peroneal nerve is rarely reported according to literary findings. We report a case of a 32-year-old man who presented with a 6-month history of a growing lump in the left knee. MRT revealed a well-defined 9.6 cm × 7.8 cm × 6.5 cm multilobular mass of heterogeneous consistency with areas of necroses with a likely diagnosis of synovial sarcoma. After surgery, a final histopathological assessment of the tumor demonstrated Antoni A and B patterns with nuclear palisading, hallmarks of a schwannoma. Postoperatively the patient suffered a neurological complication-impaired dorsiflexion of the left foot. The patient started immediate physiotherapy in the Department of Rehabilitation. Three weeks after the operation, gradual improvement in neurological function was observed. To date, complete tumor excision combined with microscopic analysis and immunohistochemical staining remains the gold standard in diagnosing and treating a peripheral nerve schwannoma. Moreover, the use of additional nerve monitoring tools during surgery could help to prevent complications.

Advances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.

Adrenal paraganglioma (or "pheochromocytoma") and extra-adrenal paraganglioma, collectively abbreviated PPGL, are rare but spectacular nonepithelial neuroendocrine neoplasms. These are the most inheritable neoplasia of all, with a metastatic potential in a varying degree. As of such, these lesions demand careful histologic, immunohistochemical, and genetic characterization to provide the clinical team with a detailed report taking into account the anticipated prognosis and risk of syndromic/inherited disease. While no histologic algorithm, immunohistochemical biomarker, or molecular aberration single-handedly can identify potentially lethal cases upfront, the combined analysis of various risk parameters may stratify PPGL patients more stringently than previously. Moreover, the novel 2022 WHO Classification of Endocrine and Neuroendocrine Tumors also brings some new concepts into play, not least the reclassification of special neuroendocrine neoplasms (cauda equina neuroendocrine tumor and composite gangliocytoma/neuroma-neuroendocrine tumor) previously thought to belong to the spectrum of PPGL. This review focuses on updated key diagnostic and prognostic concepts that will aid when facing this rather enigmatic tumor entity in clinical practice.

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.

Neurofibromatosis type I (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis represent a diverse group of genetic tumor predisposition syndromes with a shared feature of tumors affecting the peripheral nerve sheaths. PURPOSE OF REVIEW: Many advancements have been made in understanding the biologic underpinnings of these conditions, and in 2016 the first drug was approved by the FDA to treat pediatric symptomatic unresectable plexiform neurofibromas. RECENT FINDINGS: Mek inhibitors have provided a much-needed therapeutic avenue for NF1 patients with unresectable plexiform neurofibromas (PN), both for reduction of tumor bulk and for improvement in symptoms. Selumetinib is the first FDA approved drug for PN, but is only approved for children. Some research suggests that alternative Mek inhibitors and other mixed tyrosine kinase inhibitors may have better efficacy in adults. Vascular endothelial growth factor (VEGF) inhibitor bevacizumab can prolong hearing and delay the need for surgery in NF2 patients with bilateral vestibular schwannomas. This article provides an update regarding considerations and approaches when treating the tumors associated with the neurofibromatoses (NF), including risk and prognosis metrics, clinical trial results, surgical techniques, and radiation therapy recommendations.

Aplasia Cutis Congenita With Cutaneous Meningioma: A Rare Case.

ABSTRACT: Cutaneous meningioma, characterized by ectopic meningothelial cells in the dermis or subcutis, is a rare neoplasm. Generally, the most common location for cutaneous meningioma is the scalp. We report a case of cutaneous meningioma presenting as soft, light red, atrophic, and smooth patches with central blue spots. On histological examination, the tumor consisted largely of epithelioid cells, whorls, nests, and syncytial sheets of meningothelial cells. HMB-45, Melan-A, and S100 were negative; epithelial membrane antigen and somatostatin receptor 2 were positive. Ultimately, histopathologic examination and immunohistochemistry results supported a diagnosis of cutaneous meningioma. In addition, dermal dysplasia was observed above the tumor, manifested by thinning of the dermis and loss of appendages. No abnormalities were found on brain magnetic resonance imaging. Cutaneous meningioma is an extremely rare tumor, and its manifestation as an atrophic patch is even rarer. There have been mainly clinical reports of cutaneous meningioma. This was a rare case of focal aplasia cutis congenita with cutaneous meningioma. For cutaneous meningioma to be diagnosed earlier, there needs to be greater public awareness about the condition.

Surgery of Schwannoma in the upper limb - sensitivity and specificity of preoperative magnetic resonance imaging and relation between tumour size and symptoms.

BACKGROUND: Benign peripheral nerve tumours consist of different types, most commonly Schwannomas. Preoperative Magnetic Resonance Imaging (MRI) is commonly performed before surgery and Pathoanatomical Diagnosis (PAD) confirms the diagnosis. Our aims were to study the utility of MRI and the relation between tumour size and symptoms. METHODS: Retrospectively, patients, surgically treated for benign nerve tumours between 2008 and 2019, were identified and preoperative MRI, with measurement of tumour size, PAD, symptoms, peroperative details, and symptomatic outcomes of surgery, were analysed. RESULTS: The sensitivity and specificity to correctly identify Schwannomas with preoperative MRI were 85% and 50%, respectively, based on 30 Schwannomas and nine neurofibromas that were identified. Tumour size did not affect the presence of preoperative symptoms, but patients with sensory dysfunction at last follow-up had larger Schwannomas (p < 0.05). Symptoms as a palpable tumour, paraesthesia and pain improved by surgical excision (p < 0.001, p < 0.001 and p < 0.012, respectively), but sensory and motor dysfunction were common postoperatively. No malignant peripheral nerve sheath tumours (MPNST) were found. Using a surgical microscope, instead of only loop magnification, lowered the risk of perioperative nerve injuries (p < 0.05), but did not further diminish postoperative symptoms. CONCLUSIONS: Early and accurate diagnosis of Schwannomas is valuable for adequate presurgical preparation and prompt surgical intervention. Preoperative examination with MRI has a high sensitivity, but low specificity; although recent advancement in MRI technology indicates improvement in diagnostic precision. Surgical excision is preferably performed early in conjunction with symptomatic debut to improve outcome.

Ultrasound and MR imaging of peripheral nerve tumors: the state of the art.

Peripheral nerve sheath tumors are a heterogeneous subgroup of soft tissue tumors that either arise from a peripheral nerve or show nerve sheath differentiation. On imaging, direct continuity with a neural structure or location along a typical nerve distribution represents the most important signs to suggest the diagnosis. Ultrasound and magnetic resonance imaging are the best modalities to evaluate these lesions. First, it is necessary to differentiate between a true tumor and a non-neoplastic nerve condition such as a neuroma, peripheral nerve ganglion, intraneural venous malformation, lipomatosis of nerve, or nerve focal hypertrophy. Then, with a combination of clinical features, conventional and advanced imaging appearances, it is usually possible to characterize neurogenic tumors confidently. This article reviews the features of benign and malignant peripheral nerve sheath tumors, including the rare and recently described tumor types. Furthermore, other malignant neoplasms of peripheral nerves as well as non-neoplastic conditions than can mimick neurogenic tumor are herein discussed.

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.

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.

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.

Updates in the Management of Central and Peripheral Nervous System Tumors among Patients with Neurofibromatosis Type 1 and Neurofibromatosis Type 2.

BACKGROUND: Neurofibromatosis type 1 and neurofibromatosis type 2 are unrelated, distinct genetic disorders characterized by the development of central and peripheral nervous system tumors. SUMMARY: Neurofibromatosis type 1 is the most common inherited tumor predisposition syndrome with a lifelong increased risk of benign and malignant tumor development, such as glioma and nerve sheath tumors. Neurofibromatosis type 2 classically presents with bilateral vestibular schwannoma, yet it is also associated with non-vestibular schwannoma, meningioma, and ependymoma. Historically, the number of effective therapies for neurofibromatosis-related neoplasms has been limited. KEY MESSAGE: In the past decade, there have been significant advances in the development of precision-based therapies for NF-associated tumors with an increased emphasis on functional outcomes in addition to tumor response. Continued scientific discovery and advancement of targeted therapies for NF-associated neoplasms are necessary to continue to improve outcomes for patients with NF.

Primary and metastatic paraganglioma of the cranial vault.

AIM: Primary paragangliomas (PGs) are extra-adrenal neuroendocrine tumours that are extremely rare. Multiple lesions in the central nervous system raise suspicion of a metastatic process. Lack of consensus on their management warrants the categorization of existing literature to evaluate management options. METHODS: A systematic review of the medical literature on paraganglioma within the cranial vault was completed in accordance with PRISMA guidelines using the Medline database. Tumour physical measures, management parameters, and immunohistochemistry of all documented cases of primary and secondary paraganglioma within the cranial vault were descriptively compared. This review was augmented by comparison with our centre's case of a 48-year-old man diagnosed with metastatic PG originating in the cauda equina and seeding in the cerebellum. Histological parameters within the literature was also established. RESULTS: The systematic literature review yielded published 52 papers. Most prevalent primary intracranial PGs (n = 37) were in the sellar region (78%, n = 23) and the cerebellum (21%, n = 6). The highest progression free survival was seen in primary sellar PGs (87.5% by 34.5 months) and cerebellar PGs (100% by 35.7 months) when treated with adjuvant radiotherapy with subtotal resection or gross total resection, respectively. Contrasting, the most frequent intracranial PGs metastases (n = 15) occurred in the cerebellum (36%, n = 6), and the cerebral parenchyma (29%, n = 4). Their recurrence rate was between 4 and 10% and these metastasized PG in the cerebellum are slow growing (8.9 years, range: 3-22 years). Adjuvant radiotherapy with Gross Total Resection resulted in the optimum progression-free survival (100% up to 48 months) for the patient with PGs metastasis to the cerebellum. CONCLUSION: Metastatic PGs tend to be slow-growing and are clinically silent tumours. Diagnosed patients should undergo regular surveillance neuroradiological assessment, regardless of symptomatology, for metastases along the complete neural axis. We recommend operative management with GTR and adjunct RT in these patients.

Mobile cauda equina ependymoma, case report and literature review.

The aim of this study was to describe a very rare presentation of ependymoma that can derive from intraoperative complications for surgeons and increased morbidity for the patients. Multiple cases of mobile cauda equina tumors leading to inadequate approach have been reported in the literature, however, the reports of ependymoma histology are very rare. This report describes the third adult case in recent years and a retrospective review of cases reported in the literature until today. We show the clinical and radiographic presentation of a 79 years old patient that presented a mobile cauda equina tumor in the preoperative image, who went to surgery for resection and arthrodesis, obtaining in post-operative histology the diagnostic of ependymoma. The preoperative image shows the migration of the tumor avoiding the wrong approach. The patient went to surgery for laminectomy, pedicular screw arthrodesis L1-S1, and tumor removal, obtaining a complete resection and confirmed histology diagnostic of ependymoma, unfortunately, the patient died five days after by a cardiac arrest. The recent literature reports only four cases of mobile ependymoma, two of them in pediatrics patients and just only two adult cases, which means this is the third adult case of an ependymoma. Mobile cauda equina ependymoma is a very rare neoplasm presentation but is an entity that can lead to mistakes in the approach and may be useful for the neurosurgeons who day per day treat this pathology beware of this possibility.