Melanotic Schwannoma Research Articles

Malignant melanotic nerve sheath tumor

Malignant melanotic nerve sheath tumor, previously known as melanotic schwannoma, is a rare variant of peripheral nerve sheath tumor composed of melanin-producing Schwann cells and clinically misdiagnosed as melanoma. Malignant melanotic nerve sheath tumor is a rare tumor with uncertain biological behavior and variable treatment recommendations. Despite the histological features that seem benign, with a low proliferation index, the clinical course of this tumor often becomes malignant, with a high frequency of local relapses and the presence of tumor metastasis signs. Distinctive features of malignant melanotic tumor of the nerve sheath are a relatively young age of patients, the presence of the Carney complex signs and psammomatous bodies in the tumor. The article presents a case of malignant melanotic tumor of the nerve sheath localized in the neck with a recurrent course. In the described clinical observation, the patient underwent surgical treatment twice. Microscopic and immunohistochemical analysis of postoperative materials gave a positive result for melanoma markers and showed the absence of psammoma bodies and low mitotic activity. The clinical course of the disease was malignant, with the appearance of a relapse in the postoperative scar. Diagnosis of malignant melanotic tumor of the nerve sheath is a complex task, both from the radiological and pathological points of view. Communication between the surgeon, radiologist and pathologist, understanding the anatomical relationships of the lesion and careful attention to the histological findings are critical to making the correct diagnosis.

Intradural Melanotic Schwannoma of the Sacral Spine: An Illustrated Case Report of Diagnostic Conundrum

Schwannomas are benign and slow-growing peripheral nerve sheath neoplasms of Schwann cells. These are generally encountered in the neck, head, and flexor areas of the extremities. Even though many schwannomas are easily diagnosable, their variable morphology can occasionally create difficulty in diagnosis. In this study, we present a rare case of melanotic schwannoma of the sacrum, emphasizing the need for routine biopsy to understand the etiology. A 46-year-old man presented to the Department of Neurosurgery, Taipei Medical University Hospital, with buttock pain in the sacrum area for 1 year, which worsened in the last 1–2 months. The patient had no known history of trauma or malignancy. We evidenced an intradural extramedullary neurogenic tumor at the caudal end from S1 to S3. Histologic analysis revealed melanin deposition in the tumor cells. Round to oval tumor cells were positive for HMB-45 and S-100 proteins, suggestive of melanotic Schwannoma, which were removed by laminectomy. After 1 month, the tumor recurred and was further removed surgically. Conclusively, we observed the sacrum as an unusual anatomic site for the possible occurrence of melanotic schwannoma, especially in patients with no known history of trauma and malignancy. The possibility of melanotic schwannoma is very high. We hypothesize that melanotic schwannoma was possible because it occurred in the intradural and extramedullary regions of the spine. Hence, a routine biopsy should be performed to corroborate the exact cause and prevent incorrect presumptions.

Malignant melanotic schwannoma of the cervical spinal cord: a case report

Spinal cord malignant melanotic schwannoma (MMNST) is a rare central nervous system tumor that originates from the spinal cord or spinal myelin sheath cells and can produce melanin. This type of tumor is usually highly aggressive and malignant, with a poor prognosis. The clinical manifestations of spinal cord MMNST are mainly pain, paresthesia, muscle weakness, muscle atrophy, etc., and symptoms of spinal cord compression, such as intestinal and bladder dysfunction, paraplegia, etc. Early detection of tumor lesions can facilitate tumor removal, improve patients’ quality of life, and prolong patients’ survival. In this case report, a 27-year-old young woman was diagnosed with MMNST of the cervical spinal cord due to weakness of her limbs in our hospital, and underwent surgical resection. The patient’s limbs returned to normal after surgery. It is worth mentioning that the patient visited our hospital 7 months ago for “right upper limb pain for 3 days” and was diagnosed with a cervical spine space-occupying lesion at the same position this time, but the pathology report was “hemosiderosis”. This case report aims to raise awareness of the problem of spinal cord MMNST and contribute to greater knowledge of this rare tumor.

Chapter 15 - Peripheral nerve tumors

The chapter is focused on the neoplastic peripheral nerve lesions, which primarily involve "cranial and paraspinal nerves," as outlined in the CNS volume (WHO_Classification_of_Tumours_Editorial_Board, 2021). These include classic peripheral nerve sheath tumors such as schwannoma, neurofibroma, intraneural perineurioma, and malignant peripheral nerve sheath tumors, with their variants as well as new and more precisely defined entities, including hybrid nerve sheath tumors and malignant melanotic nerve sheath tumor (previously melanotic schwannoma).

Abstract 16777: Do Not Clown Around With Intracardiac Myxomas: A Clinical Case of Carney Complex

Introduction: Intracardiac myxomas are associated with cardiovascular mortality most frequently due to embolism and obstruction leading to heart failure. One of the most feared complications of intracardiac myxomas are sudden cardiac death, as these tumors are often identified incidentally. Carney Complex (CNC) is a rare disorder characterized by cardiac myxomas, psammomatous melanotic schwannomas, abnormal skin pigmentation, and endocrine disorders. Clinical Case: A 55 year old female with a past medical history of hypertension presented to the hospital with syncopal episodes and intermittent vision loss. Head computed tomography scan noted a large extra-axial mass present within the left cerebellopontine angle. A transthoracic echocardiogram showed a severely dilated left atria containing a large atrial mass (7.9 x 5.0 cm) that was causing obstruction of mitral inflow resulting in peak velocity 246 cm/S with a mean gradient of 12 mmHg. The patient was also noted to have dispersed epithelioid blue nevi present on skin examination. The patient met major criteria for diagnosis of Carney Complex. Conclusion: Carney complex (CNC) is an extremely rare autosomal dominant syndrome with less than 1,000 cases reported worldwide. Patients with familial history of intracardiac myxomas, multiple myxomas, characteristic skin findings, or endocrinopathies should be evaluated for CNC. Patients with cardiac myxoma secondary to CNC typically have a recurrence risk of roughly 30%. Patients with CNC should be screened with biannual transthoracic echocardiograms after resection and patients with suspected CNC should be worked up for additional tumor burden and endocrinopathies.

Peripheral nerve tumors in adults and children: clinical and diagnostic characteristics

Aim. To describe clinical and instrumental characteristics of patients with primary solitary and multiple tumors of the peripheral nerves.Materials and methods. Data from 45 patients were analyzed. In accordance with the examination results, the patients were retrospectively divided into 2 groups: 1) with solitary tumors: schwannomas (n = 22), neurofibromas (n = 10), perineuriomas (n = 5), neurofibrosarcomas (n = 2); 2) with multiple tumors: schwannomatosis (n = 3) and neurofibromatosis (n = 3).Results. The study did not show differences in the severity of motor deficit and the results of neurophysiological exams between solitary and multiple tumors; however, differences between pathomorphological types were observed. The most benign disease course was observed in patients with schwannomas, including multiple schwannomas; severe motor deficit was observed in patients with neurofibrosarcomas and crossover neurofibromas. Pain component was characteristic of neurofibrosarcomas, neurofibromas and melanotic schwannoma; perineuriomas were characterized by painless progression.Conclusion. Compulsory scan along the nerve allows to find tumors in clinically intact areas and other nerves.

Intramedullary melanotic schwannoma: a rare presentation of a rare tumor. Illustrative case.

Intramedullary melanotic schwannomas (IMSs) are rare spinal cord tumors, and a hemorrhagic IMS is an even rarer presentation of this uncommon lesion. The authors describe the second known case of a hemorrhagic IMS and briefly review the characteristics of IMSs. The patient's initial presentation and imaging indicated an intramedullary thoracic spinal cord tumor impairing lower extremity function. Intraoperatively, the lesion appeared pigmented and hemorrhagic. Pathologic analysis determined the tumor to be an IMS. Melanotic schwannomas vary in presentation and can resemble malignant melanoma but are differentiated by pathologic markers. Lesions typically present as extramedullary masses in the thoracic cord. Intramedullary presentation is rare but should be considered for pigmented tumors.

Primary Malignant Melanotic Nerve Sheath Tumour of the Neck: A Diagnostic Predicament.

Primary malignant melanotic nerve sheath tumours, previously called Melanotic schwannomas are rare pigmented tumours of nerve sheath origin, with unspecified biologic behaviour and uncommon presentation in the head and neck region. A primary melanotic schwannoma of the cervical sympathetic plexus, mimicking a cervical lymphadenopathy requires careful evaluation and planning for an optimal outcome. The management of these neoplasms is still controversial, and hence we present a case report with insight into the diagnostic dilemma in work-up of a neck mass that turned out to be a Primary Malignant Melanotic Schwannoma arising from the cervical sympathetic plexus along with a review of literature.

Magnetic resonance imaging features and clinical course of malignant melanotic nerve sheath tumors: single institution experience over two decades.

To evaluate MR features and clinical course of malignant melanotic nerve sheath tumor (MMNST), previously known as melanotic schwannoma and considered indolent and rarely metastasizing. This IRB-approved retrospective study searched 31 patients (20 male: 11 female, mean age 48; range 15-76) with histologically confirmed MMNST in a single tertiary cancer center over 22years. Pre-treatment MR was available in 12 patients and evaluated by two radiologists in consensus regarding lesion location, size, morphology, signal characteristics, contrast enhancement, local invasion, and presence of classic signs of peripheral nerve sheath tumors. Clinical outcomes, including local recurrence, metastasis, and survival, were examined in 12 patients for whom follow-up was available. The spine was the most frequent site (13/31) among all identified cases. In 12 cases with MR, lesions were well-circumscribed in 11/12 cases, with a mean size of 4.5cm (2.3-13.0cm). Ten of 12 cases showed T1 hyperintensity. In 5/9 spinal MRI, tumor involved multiple levels. All lesions showed contrast enhancement, and local bone invasion in > 50%. A dumb-bell shape was common to all spinal lesions. Classical signs of nerve sheath tumors were uncommon. Among 12 patients with a mean follow-up of 4.8years (range 1.3-10.2years), six were disease-free, while two had recurrence or metastases, and four had died of metastases. MMNST usually presents as a T1 hyperintense enhancing dumb-bell shaped mass in the spine. Multi-level involvement and bone invasion are common. MMNST is clinically aggressive with high rates of metastases and death.

Intradural and Intramedullary Melanotic Schwannoma of the Lumbar Spine, After Spinal Anesthesia - A Case Report

Spinal anesthesia is one of the most used techniques in elderly patients, with doses varying according to the proposed surgery and the surgeons [1]. Schwannomas are benign, encapsulated, slow-growing neoplasms. made up of neoplastic schwann cells differentiated [2]. The most used diagnosis is Magnetic Resonance Imaging (MRI) and confirmation is obtained through histological and immunohistochemical studies [2]. In order to study the reclassification of Melanotic Schwannomas (MS), a study was carried out in 40 clinicopathologic, immunohistochemical, and gene expression cases [3]. The purpose of this case report was diagnosed after the appearance of paresis in a patient undergoing urologic treatment under spinal anesthesia, two days after the procedure requiring hospitalization, and explain the possibility of performing spinal anesthesia in a patient with xanthochromic puncture.

A para-aortic malignant melanotic nerve sheath tumor mimicking a gastrointestinal stromal tumor: a rare case report and review of literature

BackgroundMalignant melanotic nerve sheath tumor (MMNST), formerly called melanotic schwannoma, is a rare tumor of neural crest derivation which most frequently arises from the region of spinal or autonomic nerves near the midline. Recent studies have reported malignant behavior of MMNST, and there still has no standard management guidelines. Intra-abdominal MMNST, which has never been reviewed as an entity, is even rarer. In this study, we present a rare case of a cystic MMNST arising from the para-aortic region and mimicking an intra-abdominal gastrointestinal stromal tumor (GIST), and review the literature regarding MMNSTs located in the abdominal cavity.Case presentationA 59-year-old female was incidentally found a tumor located in the left para-aortic area by non-contrast computed tomography. A Magnetic Resonance Imaging showed a cystic mass originated from the inferior mesenteric artery (IMA) territory. A GIST was initially diagnosed. The tumor was resected en bloc by laparoscopic surgery and was found between mesocolon and Gerota’s fascia with blood supply of IMA. Grossly, dark brown materials were noted at the inner surface of the cystic wall. Microscopically, the tumor cells were melanin-containing, and no psammomatous bodies were present. Immunohistochemically, the tumor showed positivity for MART1, HMB45, collagen IV, and SOX10, and negativity for AE1/AE3. MMNST was favored over malignant melanoma, since the tumor was located near ganglia and had cells with less atypical cytology and a low mitotic rate, and subsequent adjuvant radiotherapy was performed. The patient was alive with no evidence of recurrent or metastatic disease 11 months after radiotherapy.ConclusionsOur review of abdominal MMNST cases showed a female predominance, with an average age of 54.8 years, and a trend toward being a larger tumor showing cystic or necrotic changes. Local recurrence and metastasis rate were reviewed, and both showed a low rate. Diagnosis of MMNST should combine all the available findings, and complete excision of the tumor should be performed, followed by long-term patient monitoring.

Malignant psammomatous melanotic schwannoma mimicking adrenal cyst: case report

BackgroundMelanotic schwannoma is a melanin producing nerve sheath tumors. Rarely, it can be associated with psammoma bodies, called psammomatous melanotic schwannoma. Psammomatous melanotic schwannomas are associated in up to 10% of the cases with Carney’s syndrome. The rarity of the lesion, which may present at different localizations create difficulty in placing a correct initial diagnosis. Definitive diagnosis is made after complete tumor excision and pathomorphological evaluation. The prognosis depends on the anatomical localization, local invasion and presence of a high mitotic index. The main pathomorphological differential diagnosis includes schwannomas and other melanin producing tumors as melanoma.Case presentationWe present a case of an 11-year-old female with cystic lesion adjacent to right adrenal gland, mimicking adrenal cyst. Ultrasound guided biopsy was undertaken due to the cystic appearance of the formation and the lack of certain diagnosis from the non-invasive diagnostic tests. No signs of cellular and nuclear atypism were observed. The diagnosis of benign endothelial cyst with spontaneous hemorrhage was suggested. The patient underwent transabdominal laparoscopic adrenalectomy en-bloc with the cyst to prevent spillage of the cyst content due to the intimate adhesion of the lesion to the adrenal gland and vena cava inferior. Pathomorphological examination revealed malignant psammomatous melanotic schwannoma. The adrenal gland was intact with no tumor infiltration. The patient was followed up on the 1st and 2nd month afterwards the surgery by MRI with no signs of local recurrence and postoperative complications.ConclusionPsammomatous melanotic schwannoma near adrenal gland are rare and present difficulty with exact preoperative diagnosis. Complete resection should always be provided. Laparoscopic surgery is feasible if radical excision is not compromised. Long-term follow-up and Carney’s syndrome surveillance after complete excision are recommended especially in young patients.

Gastrointestinal: Melanotic schwannoma of the pancreas associated with Carney complex: A cause of acute neoplastic symptom.

Gastrointestinal: Melanotic schwannoma of the pancreas associated with Carney complex: A cause of acute neoplastic symptom.

Sporadic superficial angiomyxomas demonstrate loss of PRKAR1A expression.

Superficial angiomyxomas are cutaneous mesenchymal tumours that typically present clinically as slow-growing, solitary, asymptomatic nodules that can occur at any age. Histopathologically, these dermal and subcutaneous tumours are characterized by abundant myxoid stroma, numerous thin-walled and often arbourising blood vessels, and spindled to stellate fibroblast-like cells. While usually sporadic, superficial angiomyxomas can occasionally be associated with Carney complex (CNC), an autosomal dominant disorder characterized by inactivating germline mutations in the 1-alpha regulatory subunit of protein kinase A (PRKAR1A) and various clinical manifestations, including cardiac myxomas, facial lentigines, epithelioid blue naevi, endocrinopathies and psammomatous melanotic schwannomas. In this study, we sought to characterize the presence or absence of PRKAR1A expression by immunohistochemistry (IHC) in sporadic superficial angiomyxomas based on our observations in an index case. In total, PRKAR1A immunohistochemical expression was determined in 15 sporadic superficial angiomyxoma cases retrieved from the surgical pathology archives. IHC demonstrated that the lesional cells in 12 cases (80%) were non-reactive to antibodies against PRKAR1A. This study provides evidence in support of a role for PRKAR1A in the development of clinically non-syndromic superficial angiomyxomas. Together with previous studies, this report demonstrates that PRKAR1A may play an important role in the development of a variety of myxomatous mesenchymal tumours.

Dermatological and endocrine elements in Carney complex (Review).

Carney complex (CΝC) is a very rare, autosomal dominant, hereditary syndrome. Seventy percent of individuals with CNC have germline inactivating or deleting mutations of the CNC1 gene [currently known as protein kinase cAMP-dependent type I regulatory subunit α (PRKAR1A), located at the 17q22-24 chromosome level], with 30% of cases presenting with phosphodiesterase gene mutations. A member of the lentiginosis family, dermatological features include: skin pigmentation, cutaneous/mucosal myxomas, usually diagnosed by the age of 20 years (neonatal presentation is exceptional, requiring a meticulous differential diagnosis). Melanocyte-derived tumors such as epithelioid blue nevi (with different levels of pigmentation) and pigmented epithelioid melanocytoma (previously 'animal-type melanoma') are often found. Myxomas, mesenchymal tumors with mostly a benign pattern, may be recurrent. Primary cutaneous melanotic schwannoma are atypical, while non-skin sites are frequent. Corticotropinomas or somatotropinomas are part of the hereditary syndrome-related pituitary adenomas (representing 5% of all). Primary pigmented nodular adrenocortical disease involves bilateral cortical hyperplasia causing Cushing syndrome (CS) at an earlier age than non-CNC cases; osteoporotic fractures seem more prevalent compare to CS of other etiologies. Typically benign, a few cases of adrenocortical carcinoma have been identified. A total of 5% of familial non-medullary thyroid cancer is syndromic, also including CNC. CNC-related thyroid frame includes: hyperthyroidism, follicular hyperplasia/adenomas, follicular carcinoma (usually aggressive, bilateral or multifocal). Large cell calcifying Sertoli cell tumors of the testes have malignant behavior in adults; in children these may induce precocious puberty. Two particular mammary tumors are found: myxoid fibroadenomas and breast myxomatosis. Cutaneous/subcutaneous lesions, pigmented or not, or any focal swelling of non-identified cause needs careful examination, since dermatological elements are among the earliest and most discernable by which to detect lesions in CNC, a systemic condition with multi-level endocrine involvement.

A challenging case of sporadic melanocytoma of the jugular foramen

BackgroundThe jugular foramen (JF) can be the site of several tumours. Paragangliomas, schwannomas and meningiomas are the most commonly reported. We describe a case of melanocytoma originating from the JF and presenting with an accessory nerve palsy. Illustrative caseA 48-year-old woman presented with a 6-month history of cervical and left shoulder pain with wasting and weakness of the left trapezius. A Magnetic Resonance Imaging (MRI) showed a T1-hyperintense, T2-isointense, heterogeneously enhancing lesion involving the left JF and extending into the cerebello-medullary and cerebello-pontine cisterns. A retrosigmoid craniotomy was performed and a near-total removal achieved. The accessory nerve was involved by tumour and could not be preserved. Given the diagnostic uncertainty between melanotic schwannoma, metastatic melanoma and meningeal melanocytoma, next generation sequencing and genome-wide DNA methylation arrays were performed, documenting a mutation in GNA11 (c.6226A>T, p. Gln209Leu) and a methylation profile consistent with melanocytoma. The patient underwent adjuvant fractionated radiotherapy of the tumour remnant. A follow-up MRI 4 years after surgery did not show any tumour recurrence. ConclusionsThe differential diagnosis of skull base pigmented tumours can be challenging, particularly when they occur in unusual locations such as the JF. They can be misdiagnosed given their similar clinical, neuroradiological and pathological features if anatomy of the site of origin is not carefully considered and molecular tests are not performed, leading to erroneous treatment and follow-up planning.

Diagnostic Pathology of Tumors of Peripheral Nerve.

Neoplasms of the peripheral nervous system represent a heterogenous group with a wide spectrum of morphological features and biological potential. They range from benign and curable by complete excision (schwannoma and soft tissue perineurioma) to benign but potentially aggressive at the local level (plexiform neurofibroma) to the highly malignant (malignant peripheral nerve sheath tumors [MPNST]). In this review, we discuss the diagnostic and pathologic features of common peripheral nerve sheath tumors, particularly those that may be encountered in the intracranial compartment or in the spine and paraspinal region. The discussion will cover schwannoma, neurofibroma, atypical neurofibromatous neoplasms of uncertain biological potential, intraneural and soft tissue perineurioma, hybrid nerve sheath tumors, MPNST, and the recently renamed enigmatic tumor, malignant melanotic nerve sheath tumor, formerly referred to as melanotic schwannoma. We also discuss the diagnostic relevance of these neoplasms to specific genetic and familial syndromes of nerve, including neurofibromatosis 1, neurofibromatosis 2, and schwannomatosis. In addition, we discuss updates in our understanding of the molecular alterations that represent key drivers of these neoplasms, including neurofibromatosis type 1 and type 2, SMARCB1, LZTR1, and PRKAR1A loss, as well as the acquisition of CDKN2A/B mutations and alterations in the polycomb repressor complex members (SUZ12 and EED) in the malignant progression to MPNST. In summary, this review covers practical aspects of pathologic diagnosis with updates relevant to neurosurgical practice.

MANAGEMENT OF ENDOCRINE DISEASE: Carney complex: clinical and genetic update 20 years after the identification of the CNC1 (PRKAR1A) gene.

Described for the first time in 1985, Carney complex (CNC) is a rare dominantly inherited multiple neoplasia syndrome with almost full penetrance and characterized by both endocrine - primary pigmented nodular adrenocortical disease with Cushing's syndrome, acromegaly and thyroid tumors - and non-endocrine manifestations such as cardiac, cutaneous and mucosal myxomas, pigmented cutaneous lesions, psammomatous melanotic schwannoma, osteochondromyxoma and a wide range of other tumours with potential malignancy. The pathophysiology of CNC is a model of dysregulation of the cAMP/PKA signalling in human diseases. As described 20 years ago, inactivating heterozygous mutations of PRKAR1A formerly known as CNC1, encoding the regulatory subunit 1α of protein kinase A, are identified in more than 70% of the index cases, while inactivating mutations of genes encoding phosphodiesterases are found in rare and particular forms of the complex. There is at present no medical specific treatment for CNC, every confirmed or suspected CNC patient should be managed by a multi-disciplinary team according to each manifestation of the disease and offered a long-term follow-up and genetic counselling. The better knowledge that we have now of this fascinating rare disease and its genetics will help to improve patients outcome.

Novel response to neoadjuvant anti-PD1 therapy for a patient with retrocaval melanotic schwannoma.

Melanotic schwannoma is a rare nerve sheath tumor composed of melanin-producing Schwann cells with the potential for metastasis. These tumors can be associated with familial tumor syndromes and can cause significant symptoms related to nerve compression and mass effect. Due to the rarity of these lesions, they can be initially misidentified as melanocytomas, pigmented dermatofibrosarcoma protuberans, neurofibromas or malignant melanomas. Surgical excision is the mainstay of treatment with limited benefit from adjuvant systemic chemotherapy or radiation. Modern treatments with immune checkpoint blockade have demonstrated significant improvements in progression-free and overall survival for a variety of cancer histologies; however, anti-PD1 therapy has yet to be evaluated in patients with melanotic schwannoma. This report demonstrates a significant improvement in symptomatology and tumor stability with neoadjuvant anti-PD1 therapy for a retrocaval melanotic schwannoma initially masquerading as malignant melanoma. This report demonstrates the potential benefit of a novel therapeutic option for patients with melanotic schwannoma.

Peripheral Nerve Sheath Tumors of Head and Neck: Imaging-Based Review of World Health Organization Classification.

We aim to review the imaging appearance of peripheral nerve sheath tumors (PNSTs) of head and neck according to updated fourth edition of World Health Organization classification. Peripheral nerve sheath tumor can be sporadic or associated with neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis. Schwannoma is the most common benign PNST that can be intracranial or extracranial and appears heterogeneous reflecting its histologic composition. Melanotic schwannoma is a different entity with high prediction of malignancy; it shows hypointense signal on T2-weighted image. Neurofibroma can present by localized, plexiform, or diffuse lesion. It usually appears homogeneous or shows a characteristic target sign. Perineurioma can be intraneural seen with the nerve fiber or extraneural appearing as a mass. Solitary circumscribed neuroma and neurothekeoma commonly present as dermal lesions. Nerve sheath myxoma may exhibit high signal on T1 weighted image. Benign triton tumors can be central, aggressive lesion, or peripheral nonaggressive lesion. Granular cell tumor shows hypointense signal on T2 weighted image. Neuroglial heterotopia most commonly occurs in the nasal cavity. Ectopic meningioma arises from ectopic arachnoid cells in the neck. In hybrid PNST, combined histological features of benign PNST occur in the same lesion. Malignant PNSTs are rare with an aggressive pattern. Computed tomography and magnetic resonance imaging are complementary studies to determine the location and extent of the tumor. Advanced magnetic resonance sequences, namely, diffusion-weighted imaging and dynamic contrast enhancement, can help in differentiation of benign from malignant PNST.