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". 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. This case report aims to raise awareness of the problem of spinal cord MMNST and contribute to greater knowledge of this rare tumor.

Blaschkoid melanotic cutaneous lupus erythematosus with "melanocytic nests".

Melanotic cutaneous lupus erythematosus (LE) is a newly described clinical variant of chronic cutaneous LE, presenting with localized or diffuse brownish or grayish macular and reticulated pigmentation in the absence of erythema, scaling, atrophy, scarring, or telangiectasia. The diagnosis is based upon histopathology, which demonstrates the characteristic features of LE with an interface vacuolar dermatitis with melanophages, and a superficial and deep, perivascular and periadnexal lymphocytic infiltrate with mucin deposition. Herein, we describe a case of a 61-year-old White male presenting with melanotic cutaneous LE with a blaschkoid distribution on his face in which the histopathological phenomenon of "true melanocytic nests" in the setting of a lichenoid pattern was seen. We want to highlight how nests of cellular aggregates at the dermoepidermal junction labeling with melanocytic markers may occur in the setting of an interface tissue reaction. This benign reactional pattern may mimic atypical melanocytic proliferations, especially on sun-damaged skin. Clinicopathological correlation and careful microscopic examination using a panel of multiple melanocytic markers is crucial for making an accurate final diagnosis. All the cases of melanotic cutaneous LE reported in the literature are also reviewed.

Cis-acting mutation affecting GJA5 transcription is underlying the Melanotic within-feather pigmentation pattern in chickens.

Melanotic (Ml) is a mutation in chickens that extends black (eumelanin) pigmentation in normally brown or red (pheomelanin) areas, thus affecting multiple within-feather patterns [J. W. Moore, J. R. Smyth Jr, J. Hered. 62, 215-219 (1971)]. In the present study, linkage mapping using a back-cross between Dark Cornish (Ml/Ml) and Partridge Plymouth Rock (ml+/ml+ ) chickens assigned Ml to an 820-kb region on chromosome 1. Identity-by-descent mapping, via whole-genome sequencing and diagnostic tests using a diverse set of chickens, refined the localization to the genomic region harboring GJA5 encoding gap-junction protein 5 (alias connexin 40) previously associated with pigmentation patterns in zebrafish. An insertion/deletion polymorphism located in the vicinity of the GJA5 promoter region was identified as the candidate causal mutation. Four different GJA5 transcripts were found to be expressed in feather follicles and at least two showed differential expression between genotypes. The results showed that Melanotic constitutes a cis-acting regulatory mutation affecting GJA5 expression. A recent study established the melanocortin-1 receptor (MC1R) locus and the interaction between the MC1R receptor and its antagonist agouti-signaling protein as the primary mechanism underlying variation in within-feather pigmentation patterns in chickens. The present study advances understanding the mechanisms underlying variation in plumage color in birds because it demonstrates that the activity of connexin 40/GJA5 can modulate the periodic pigmentation patterns within individual feathers.

PRAME immunohistochemistry is useful in differentiating oral melanomas from nevi and melanotic macules.

BACKGROUND: Oral melanocytic neoplasms pose a diagnostic challenge to pathologists owing to their rarity relative to those in the skin. The utility of PRAME in distinguishing nevi from melanomas has been established in the skin, but limited information exists regarding its usefulness in the oral cavity. METHODS: Thirty-five previously diagnosed pigmented oral lesions were retrospectively evaluated with PRAME. The lesions consisted of 16 oral nevi, 10 melanomas, and 10 melanotic macules. RESULTS: Strong and diffuse nuclear PRAME staining was observed in all but one of the oral melanomas, which showed no staining. No nuclear PRAME staining was observed in any of the oral nevi or melanotic macules. CONCLUSIONS: PRAME is a useful tool in the evaluation of oral melanocytic neoplasms. Our data indicate that PRAME is a highly specific but incompletely sensitive marker of oral melanoma. Larger studies could further illuminate the diagnostic value of PRAME in oral lesions.

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

OBJECTIVE: 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. MATERIALS AND METHODS: 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 22 years. 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. RESULTS: 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 4.5 cm (2.3-13.0 cm). 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 4.8 years (range 1.3-10.2 years), six were disease-free, while two had recurrence or metastases, and four had died of metastases. CONCLUSION: 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.

Peutz-Jeghers Syndrome: Lessons to be Learned in the Clinical Diagnosis.

Introduction: Peutz-Jeghers Syndrome (PJS) is an autosomal dominant disease presenting with hamartomatous polyps in the gastrointestinal tract and hyperpigmented macules on lips and oral mucosa. The incidence of this syndrome is approximately 1 in 1,20,000 births. Materials and Methods: In this article, we are presenting 11 cases of PJS which were misdiagnosed and patients were compelled to visit hospital repeatedly. All these cases were diagnosed based on clinical suspicion, family history, and histopathological examination of specimens. Most of the cases presented with intussusception and required emergency surgical management. Results: PJS can be diagnosed by the presence of microscopically confirmed hamartomatous polyps and a minimum of two of the following clinical criteria: Family history, mucocutaneous melanotic spots, and small bowel polyps with bleeding per rectally. The diagnosis can be missed if the melanotic spots on the face are missed. Routine investigations, imaging, and endoscopy were done in all cases. PJS patients need regular follow-up due to chance of recurrence of symptoms and susceptibility to cancer. Conclusion: PJS needs a high index of suspicion for diagnosis in cases of recurrent abdominal pain with bleeding per rectum. Proper family history and meticulous clinical examination for melanosis are very important to prevent the misdiagnosis of these cases.

Nevus, melanoma, or something else? Mesenchymal neoplasms with melanocytic differentiation.

The overwhelming majority of cutaneous neoplasms with melanocytic differentiation are nevi, melanomas, or less commonly melanocytomas. Nevertheless, there is also a group of mesenchymal neoplasms with genuine melanocytic differentiation which can create diagnostic difficulties with significant repercussions. These can rarely present as primary or metastatic cutaneous lesions. The ones that are relevant to a dermatopathologist include malignant melanotic nerve sheath tumor, perivascular epithelioid cell neoplasm, and clear cell sarcoma. This work will provide a thorough review of clinical presentation, morphologic and immunohistochemical features as well as molecular pathogenesis of these tumors. We hope to familiarize the general dermatopathology readership with a group of neoplasms of mesenchymal lineage exhibiting melanocytic differentiation and ultimately avoid diagnostic misadventures.

Rare malignant melanotic nerve sheath tumors of the upper limb nerves: utility of high-frequency ultrasonography in preoperative imaging.

Melanotic nerve sheath tumor (MNST) is a rare variant of schwannoma. Here, we report an unusual case of multiple MNST lesions located in the upper limb nerves. The patient presented with a mass on the left wrist in 2016 and another mass on the left thumb in 2017. In both instances, magnetic resonance imaging scans confirmed multiple giant-cell tumors of the tendon sheath. Persistent pain in the left upper limb and numbness in the ring finger and little finger recurred in 2021. High-frequency ultrasound (HFUS) showed that the left brachial plexus nerves (C5-8) were widened compared with those on the contralateral side; the neuroma formed at the lateral cord, and the median nerve was markedly thickened. The surgical findings were consistent with the ultrasound results. Pathology confirmed that the tumors were malignant MNSTs. HFUS is important for preoperative diagnosis and lesion localization, even identifying some lesions that are unrecognized on magnetic resonance imaging; thus, HFUS is crucial for improving surgical strategy and decision-making.

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

BACKGROUND: Malignant 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 PRESENTATION: A 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. CONCLUSIONS: Our 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.

[Melanotic neuroectodermal tumor of infancy. Clinical case of large fontanel tumor and literature review]. / Melanoticheskaya neiroektodermal'naya opukhol' mladentsev. Klinicheskii sluchai opukholi v oblasti bol'shogo rodnichka i obzor literatury.

Melanotic neuroectodermal tumor of infancy (MNTI) is a neonatal tumor with progressive growth and high recurrence rate. Aggressive growth and localization of tumor often lead to significant cosmetic defects of cranial and facial bones. The authors report MNTI in a 6-month-old boy with lesion of the large fontanel. Total resection was followed by recurrence after 3 weeks. Repeated resection with subsequent radiotherapy was performed. The follow-up period was 6 months after repeated resection. There was no tumor growth throughout this period. Considering this case and world experience, we can conclude that treatment strategy for MNTI is still unclear.

Malignant melanotic Xp11 neoplasms exhibit a clinicopathologic spectrum and gene expression profiling akin to alveolar soft part sarcoma: a proposal for reclassification.

The classification of the distinct group of mesenchymal neoplasms, first described as 'Xp11 translocation perivascular epithelioid cell tumor (PEComa)' and for which the term 'melanotic Xp11 neoplasm' or 'Xp11 neoplasm with melanocytic differentiation' has recently been proposed, remains challenging and controversial. We collected 27 melanotic Xp11 neoplasms, the largest series to date, for a comprehensive evaluation. Fourteen of the cases, together with eight alveolar soft part sarcomas (ASPS), nine conventional PEComas and a control group of seven normal tissues were submitted to RNA sequencing. Follow-up available in 22 patients showed 5-year overall survival and 5-year disease-free survival of 47.6 and 35.7%, respectively, which were similar to ASPS and significantly worse than conventional PEComa. Univariate analysis of location (occurring in the kidney versus not kidney), infiltrative growth pattern, nuclear pleomorphism, mitotic activity ≥2/50 high-power fields (HPF), necrosis and lymphovascular invasion were found to be associated with overall survival and/or disease-free survival. Multivariate analysis identified that location was the only factor found to independently correlate with disease-free survival. More importantly, RNA sequencing-based clustering analysis segregated melanotic Xp11 neoplasm and ASPS from other tumors, including conventional PEComa and Xp11 translocation renal cell carcinoma, and formed a compact cluster representative of the largely similar expression signature. Here we clearly define the true biologic nature of melanotic Xp11 neoplasms which are distinctive malignant mesenchymal tumors, rather than simply PEComa variants with occasionally unpredictable behavior. Meanwhile, melanotic Xp11 neoplasm and ASPS more likely represent phenotypic variants of the same entity, which is distinct from conventional PEComa and Xp11 translocation renal cell carcinoma. Based on these important findings, melanotic Xp11 neoplasm might be reclassified into a distinctive entity together with ASPS, independent from PEComa, in future revisions of the current WHO categories of tumors of soft tissue and bone for the improved reclassification. © 2020 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Diagnostic accuracy of pigmented labial macules by in vivo reflectance confocal microscopy and correlation among techniques.

BACKGROUND: Pigmented labial macules (PLMs) are clinical, dermoscopic, and histopathologic challenges. OBJECTIVE: To describe and evaluate the utility of reflectance confocal microscopy (RCM) in PLMs and to establish a correlation between dermoscopy, RCM, histopathology, and immunohistochemistry. METHODS: Prospective study of PLMs from 4 tertiary referral dermatology centers. The study included 51 biopsy specimen-proven PLMs. Dermoscopic, RCM images, and histopathologic preparations were evaluated for malignant criteria. Diagnostic accuracy of RCM for melanoma diagnosis, RCM Lip Score previously reported, and κ values between techniques were calculated. RESULTS: Included were 5 melanomas and 46 benign PLMs. Dermoscopically, melanomas exhibited more frequently ≥3 colors and ≥3 structures. With RCM, pagetoid spreading, epithelial disarray, continuous proliferation of atypical cells around papillae, nonhomogeneously distributed papillae, marked cellular atypia, and a higher number of dendritic cells per papillae were more frequent in melanomas. The RCM Lip Score was significantly higher in malignant lesions. Good κ values were observed in most of the evaluated features. A perfect sensitivity and specificity was obtained combining dermoscopy and RCM. LIMITATIONS: A low number of melanomas were obtained. CONCLUSIONS: RCM improves lip melanoma diagnosis, and the RCM Lip Score represents a useful tool for the evaluation of a PLM.

A practical guide on the use of imiquimod cream to treat lentigo maligna.

Lentigo maligna (LM) is a common in situ melanoma subtype arising on chronically sun-damaged skin and mostly affects the head and neck region. Localisation in cosmetically sensitive areas, difficulty to obtain wide resection margins and advanced patient age/comorbidities have encouraged investigation of less invasive therapeutic strategies than surgery in managing complex cases of LM. Radiotherapy and imiquimod have emerged as alternative treatment options in this context. The treatment of LM with imiquimod cream can be challenging due to the nature of the disease including its often large size, variegated appearance, involvement of adnexal structures, poorly defined peripheral edge and frequent localisation close to sensitive structures such as the eyes and lips, and elderly patients with multiple comorbidities. Prolonged and unpredictable inflammatory reaction and side effects and compliance with a patient-delivered therapy can also be challenging. In the literature to date, studies evaluating the use of imiquimod to treat LM have utilised varying methodologies and provided short follow-up and these limitations have impaired the development of clear guidelines for dosage and management of side effects. Based on our multidisciplinary experience and review of the literature, we propose practical clinical strategies for the use of imiquimod for treating LM, detailing optimal administration procedures in various clinical scenarios and long-term management, with the aim of facilitating optimal patient outcomes.

Melanotic Neuroectodermal Tumor of Infancy at Skull: Rare and Rapid-Growing Tumor but Histologically Benign.

INTRODUCTION: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare and rapid-growing tumor. However, a neurosurgeon should not overlook this entity when differential diagnosing rapid-growing skull tumor because its histology nature is just benign, and the prognosis is much better than other malignant tumors. CASE PRESENTATION: We reported the case of a 5-month old male presenting with progressive rapid-growing skull tumor which became 10 cm in diameter in only 5 months compared to the normal head circumference at birth. At first, we thought of malignant skull tumor and performed only biopsy to establish diagnosis. But, when the pathology revealed benign MNTI, we performed preoperative tumor embolization and then radical surgery. Good result was observed. DISCUSSION: Skull MNTI is the second most common location after the maxilla. Even advanced imaging nowadays cannot distinguish MNTI from other malignant tumors definitely. Urgent biopsy is recommended to establish diagnosis of this benign tumor first. Preoperative angiography with tumor embolization is recommended when feasible, followed by craniotomy with radical resection.

Ketoprofen Combined with UVA Irradiation Exerts Higher Selectivity in the Mode of Action against Melanotic Melanoma Cells than against Normal Human Melanocytes.

Malignant melanoma is responsible for the majority of skin cancer-related deaths. The methods of cancer treatment include surgical removal, chemotherapy, immunotherapy, and targeted therapy. However, neither of these methods gives satisfactory results. Therefore, the development of new anticancer therapeutic strategies is very important and may extend the life span of people suffering from melanoma. The aim of this study was to examine the effect of ketoprofen (KTP) and UVA radiation (UVAR) therapy on cell proliferation, apoptosis, and cell cycle distribution in both melanotic melanoma cells (COLO829) and human melanocytes (HEMn-DP) in relation to its supportive effect in the treatment of melanoma. The therapy combining the use of pre-incubation with KTP and UVAR causes a significant increase in the anti-proliferative properties of ketoprofen towards melanoma cells and the co-exposure of melanotic melanoma cells induced apoptosis shown as the mitochondrial membrane breakdown, cell-cycle deregulation, and DNA fragmentation. Moreover, co-treatment led to GSH depletion showing its pro-apoptotic effect dependent on ROS overproduction. The treatment did not show a significant effect on normal cells-melanocytes-which indicates its high selectivity. The results suggest a possible benefit from the use of the ketoprofen and ultraviolet A irradiation as a new concept of melanotic melanoma therapy.

Melanotic Neuroectodermal Tumor of Infancy: Does Enucleation Alone Suffice?

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare melanin-containing mesenchymal tumor of neural crest origin. We present a case of MNTI in a 1-year-old girl. It was managed successfully with conservative excision (enucleation).

Necrotic cell death induces melanotic mass formation in Drosophila.

The immune system protects its host from not only invading parasites and parasitoids, but also altered self tissue, including dying cells. Necrotic cells are strongly immunogenic, but in Drosophila this has not been directly addressed, due partially to the fact that knowledge about necrosis in Drosophila currently lags behind that for other models. Upon the loss of cell matrix attachment, endocycling polyploid tissues of the Drosophila larva undergo autophagy instead of apoptosis; we employed this system as a model to examine cell death modalities and immunity. Here, we report that larval fat body cells depleted of integrin undergo not only autophagy, but also necrotic cell death, and that a blockade of reaper, grim, hid, or the downstream caspases enhances necrosis. These cells elicit melanotic mass formation, an autoimmune-like response. We also show that necrosis is the main cause of melanotic mass formation in these anchorage-depleted polyploid cells.

Melanotic neuroectodermal tumor of infancy to the skull: case-based review.

BACKGROUND: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare tumor, which usually occurs in infants under the age of one. Early diagnosis and radical surgery seem to be critical for long-term cure. CASE PRESENTATION: We describe a case of a 4-month-old boy with a MNTI to the skull. The mass was first noticed at 4 month of age and grew very rapidly over a time of 2 weeks. Initially, a fine needle biopsy ruled out a sarcoma and led to the diagnosis. The tumor originated from the sphenoid wing and infiltrated the frontotemporal bone, the lateral wall of the right orbit, and the underlying dura mater. A total excision of the tumor, including the adjacent bone and dura, was achieved. Reconstruction of the bone was performed using absorbable plates and Tutobone. Histology confirmed the initial diagnosis, while molecular diagnosis showed high conformity of the MNTI with medulloblastoma group 3. The patient recovered well, while the reconstruction led to a good cosmetic result. A local recurrence occurred leading to a single-dose chemotherapy with Vincristine and a second surgery after 15 weeks. Thereafter, the patient developed recurrent large pseudomeningocele, which was treated by multiple shunt procedures and finally reconstruction of the bone using Palacos. Radiological follow-up 3 months after the second resection showed no tumor recurrence. CONCLUSION: Radical surgery for MNTI is to date the gold standard since it seems to minimize recurrence rates. Because of the rapid and destructive growth within the bone, reconstruction is necessary, which can be very challenging in infants.

Retrospective single-center study evaluating clinical and dermoscopic features of longitudinal melanonychia, ABCDEF criteria, and risk of malignancy.

BACKGROUND: Longitudinal melanonychia (LM) is a common finding in clinical practice; however, it has a broad differential diagnosis, including subungual melanoma (SUM), which can be difficult to distinguish clinically from benign conditions. OBJECTIVE: To identify clinical and dermoscopic features that distinguish histopathologically diagnosed SUM from benign LM and to evaluate the validity of the ABCDEF criteria among patients on whom a biopsy was performed. METHODS: Retrospective cohort study of consecutive patients who underwent nail matrix biopsy for LM at a single center from January 2011 to November 2017. RESULTS: A total of 84 cases in which biopsy was performed (8 cases of SUM and 76 benign) were included in the analysis. The patients with SUM were younger (P = .011), had their melanonychia longer (P = .017), and presented with a wider band (P = .002) and greater width percentage (P < .001) than patients with benign LM did. The number of ABCDEF criteria met did not differ between the groups. LIMITATIONS: Retrospective single-center study; patients who did not undergo biopsy could not be studied. CONCLUSIONS: In the cases of LM in which biopsy was performed, SUM usually presented with a wider band and greater width percentage than benign LM did. The number of ABCDEF criteria met was not different between the groups. Because many of the clinical and dermoscopic signs were less consistent, biopsy should be performed in cases with any concerning band, especially in those with width percentage higher than 40%.

Intracranial melanotic schwannomas: a rare variant with unusual adherent features.

Intracranial melanotic schwannomas (IMSch) are extremely rare nerve sheath tumors with features of Schwann cells that produce melanin. After a thorough review of the available literature since 1967, we report not only the 20th case of IMSch but a comprehensive modern-era analysis of radiographic and histological key-points to be considered when diagnosing and treating patients with this rare known entity. This is the case of a 43 years-old woman who presented with severe headaches 9 years ago (2008). At that time, MRI of the brain showed a 1.5 × 1.4 cm lesion at the level of the left cerebellar peduncle without any evidence of edema, mass effect or hydrocephalus. Given that the patient was neurologically intact, a conservative management with serial MRIs was recommended. Patient stopped following up due to the absence of symptoms. Over the course of the past year, patient noted mild left sided hearing loss and facial weakness, as well as some balance instability that progressed over the last 3 months. Given the presentation and progression of these signs and symptoms, a new MRI was performed in which considerable growth of the lesion was identified, measuring 2.5 × 2.8 × 2.6 cm with mass effect on the pons and the inferior fourth ventricle. She underwent a far lateral approach without a C1 hemilaminectomy for the resection of this lesion. Final pathology was consistent with a non-psammomatous melanotic schwannoma (NPMS) with areas of necrosis. Besides this case, only two other cases of IMSch with findings of necrosis have been reported in the literature, all of them reporting a subtotal resection. Evaluation of all previously reported cases of IMSch shows a male prevalence with a 1.6:1 male to female ratio. IMSch is radiographically T2 hypointense and can be differentiated from Schwannomas that are classically T2 hyperintense. In this case, only a subtotal resection was feasible due to the tumor's overwhelming inherent attachment to vital structures such as cranial nerves (CN), brainstem, and vasculature. While MSch is considered histologically benign, several factors including localization, surrounding structures, the rate of growth, tumor volume resection and histological necrosis should be considered in determining prognosis and further adjuvant treatment planning.