Spinal Melanocytoma Research Articles

INNV-01. ASSESSMENT OF SURVIVAL AND SAFETY FOLLOWING INTRATHECAL CHEMOTHERAPY VIA OMMAYA RESERVOIR FOR PATIENTS WITH LEPTOMENINGEAL DISEASE

Abstract BACKGROUND Leptomeningeal disease (LMD) carries poor prognosis with an average survival of 3-6 months after diagnosis. Ommaya reservoirs (OR) are an accessible alternative to serial lumbar punctures for delivery of intrathecal chemotherapy (IC) and disease monitoring but are not widely used. Intraventricular administration of chemotherapy may be associated with superior efficacy and improved patient comfort. The purpose of this study was to assess safety and efficacy of IC and disease monitoring via OR in routine clinical practice. METHODS Patients who received at least one administration of IC via OR for LMD between 2017 and 2022 were included. Demographics, treatment type, complications and outcomes were recorded. RESULTS We identified 22 patients (17 females, 5 males) with mean age 50.9 ± 14.8 years. The primary tumors were: breast (12), leukemia (3), ovarian carcinoma (3), CNS lymphoma (1), urothelial carcinoma (1), spinal melanocytoma (1), and high-grade glioma (1). A total of 208 OR injections were performed [median was 9 OR injections per patient, interquartile range (IQR) (5, 13)]. Five patients (23%) experienced mild adverse events of grade 2 or lower by Common Terminology Criteria for Adverse Events. Overall risk of adverse event from injection was 3.4% (7/208). Eight patients (36.3%) converted into negative CSF cytology (median 1 month following first injection). Eighteen patients (82%) had clinical or radiological progression of their LMD (median 2 months following first injection). Eleven patients (50%) died of their LMD during follow-up. Median OS and PFS from the first injection were 5.3 months [95% CI 4.8-NE (not estimable)] and 4.3 months [95% CI 1.8-16.0], respectively. CONCLUSION Use of OR in routine clinical practice is a safe and feasible option and should be strongly considered for treatment and monitoring of patients with LMD. The median OS in our cohort is comparable to prior published series.

Intradural extramidullary dorsal melanocytoma in the adult: Case report and review of the literature

Intradural Extramedullary Spinal Melanocytoma (IESM) is an extremely uncommon tumour arising from the spinal leptomeninges; both from a gross pathology and microscopic point of view it can mimick its malignant counterpart, the Melanoma. Such tumours are usually solitary, with a lower proliferating rate and without obvious SC invasion. Their common differential diagnoses include Spinal Schwannoma and Spinal Meningiomas since they share a significant amount of radiological similarities. It is a relatively benign condition which is, to date, with no more than 24 previously reported cases, yet widely unexplored and poorly understood. We report the detailed clinical history of a 60 years old individual suffering from IESM and, by means of a thorough Literature review, the most relevant features concerning the epidemiological issues, the clinical course, the radiological appearance, the surgical results and the typical gross and microscopic pathology of a cohort of previously reported cases of IESM are extensively discussed and systematically investigated through statistical analyses in order to add to the relevant Literature a dedicated work concerning this rare and enigmatic condition.

Spinal meningeal melanocytoma in a 5-year-old child: a case report and review of literature

BackgroundMeningeal melanocytoma is considered a rare lesion arising from leptomeningeal melanocytes. Nearly two thirds of meningeal melanocytomas were reported in the intracranial compartment and the remaining one third in the spine. Spinal melanocytomas can be extradural or intradural, with extradural variant being more common, and the majority of cases have been single reports.MethodsA 5-year-old male presented with a 4-month history of non-radiating low back pain persistent at rest, with otherwise non-remarkable medical history. The patient was neurologically intact with no deficits. Preoperatively, routine laboratory investigations were non-remarkable. MRI imaging was done and showed a lesion at the level of T11 to L4, hyperintense on T1 and hypointense on T2 with homogenous contrast enhancement. Intraoperatively, the lesion was hemorrhagic, brownish, and rubbery in consistency attached to the ventral dura. Microscopic picture revealed dense cytoplasmic brown melanin pigments, with no significant mitoses or nuclear atypia. What is unique about our case is the age of the patient (5 years).ResultsTo the best of our knowledge, after reviewing the literature, this is the youngest case to be reported.ConclusionsSMM is an extremely rare tumor with a benign course. Complete surgical excision should be attempted. Age of presentation may be as young as in our case and the diagnosis of such a tumor should never be excluded in this early age group with persistent low back ache.

Spinal Melanocytoma : A Case Report

Spinal Melanocytoma : A Case Report Spinal melanocytoma, a rare tumor of melanocytic origin, usually arises from intracranial structures but may also arise from the spinal cord. They are solitary low-grade tumors and do not invade the peripheral structures. They can occur in all the age groups and are more frequent in the fifth decade with a slightly female predominance. They localize frequently intradural extramedullary especially at the cervical or thoracal region. Most authors agree that complete surgical excision is the best treatment. The role of radiotherapy and chemotherapy still remain controversial. Here, we report a patient with a melanocytoma in the right cervicothorocal medullary region which was resected gross totally.

Bifocal extra- and intradural melanocytoma of the spine: case report and literature review

Spinal melanocytoma is one of the most infrequent space-occupying lesions of the central nervous system. To the best of our knowledge, this is the first report of primary bifocal intradural melanocytoma of heterogeneous pathological grade to date. We report the case of a 43-year old patient with primary bifocal melanocytoma, clinically and radiologically resembling benign schwannoma. The patient presented with myeloradiculopathy of the left C3 dermatome. Magnetic resonance imaging of the upper spine revealed two space-occupying lesions with paraspinal extension, initially diagnosed as neurofibroma. Definitive histopathological classification of both lesions was melanocytoma. Both tumours were only partially removed due to adherence to surrounding structures. The patient underwent stereotactic external beam irradiation (EBR). Follow-up at 1 year after surgery revealed no recurrence and the patient remained free of symptoms. The clinical, radiological and pathological features of this rare tumour entity are presented and the available literature is reviewed. Intradural melanocytoma, although exceedingly rare, requires a thorough work-up to exclude malignant melanoma. With only two previous reports of multifocal melanocytoma published in the literature, standard therapy has not yet been established and complete surgical removal remains the modality of choice. Patients should be closely monitored to detect local recurrence or malignant degeneration. EBR may be considered in cases where total excision is not achievable and reduces risk of local recurrences.

SU-E-T-488: Treatment of a Unique CNS Patient with Tomotherapy

Purpose: We describe a whole spinal cord, cauda equine, and brainstem radiation treatment using an in‐house developed tomotherapy approach for a unique patient diagnosed with an extramedullary spinal melanocytoma with leptomeningeal seeding, treated with 48.6 Gy in 28 fractions. Methods and Materials: Given that the prescribed dose is within the range of tolerance to the spinal cord, tomotherapy was chosen to take advantage of the superior dose uniformity achievable with this technology and ability to deliver modulated radiotherapy in a single treatment to a long volume. The patient was treated supine and immobilized with a thermoplastic mask for the head and shoulders and a long Vac‐Lok bag for the body. The CTV consisted of the entire spinal cord, thecal sac to the level of S2 and brainstem. A 1 cm margin was applied to create the PTV. Jaws, pitch and the modulation factor were set to 5 cm, 0.43 and 2.5, respectively. Before each treatment, the treated volume was imaged for setup verification using the integrated megavoltage CT (MVCT). Weekly the patient was also imaged post‐ treatment to confirm setup stability. Results: The patient is finishing treatment at the time of abstract submission. A highly conformal dose distribution was created with doses to the organs at risk within their tolerance limits. The beam on time was 17 minutes. Patient setup proved to be trouble‐free and reproducible. The total patient‐on‐the‐bed time was approximately one hour. The 1 cm PTV margin was adequate according to pre‐ and post‐treatment MVCT image analysis. Conclusions: Tomotherapy is a safe and effective tool for treating long CNS volumes to high dose. It allows avoiding junctions and sparing healthy CNStissue and other organs at risk. A relatively simple immobilization technique used for this patient proved to be stable and reproducible with a 1 cm PTV margin.

Primary Meningeal Melanocytoma in the Thoracic Spine: A Case Report

Meningeal melanocytoma is a rare benign melanotic tumor arising from melanocytic cells in the leptomeninges. Preoperative differential diagnosis of meningeal melanocytoma from other melanotic tumors is difficult based on magnetic resonance imaging (MRI). Definitive diagnosis of meningeal melanocytoma from other melanotic tumors is done on the basis of histopathological and immunohistochemical analyses. The prognosis of this tumor is not always favorable with occasional local recurrence, especially in cases of subtotal gross resection. The authors report on a case of a 58-year-old man who presented with a melanocytoma located at the T7-8 level. The patient underwent on a total surgical excision for successful control of the tumor. During a follow-up period of 2 years, the patient presented no signs of recurrence. We emphasize the importance of discrimination of meningeal melanocytoma from other melanotic tumors to facilitate appropriate treatment. Complete resection is the treatment of choice for spinal melanocytoma. Radiation therapy should be reserved for those cases in which complete resection is not possible or in which there is recurrence.

Surgical treatment for intramedullary spinal cord melanocytomas

Spinal meningeal melanocytomas are rare lesions that are histologically benign and can behave aggressively, with local infiltration. The authors present their experience with intramedullary spinal cord melanocytomas consisting of 3 cases, which represents the second largest series in the literature. A retrospective chart review was performed following identification of all spinal melanocytomas treated at the author's institution, based on information obtained from a neuropathology database. The charts were reviewed for patient demographics, surgical procedure, clinical outcome, and long-term tumor progression. Three patients were identified in whom spinal melanocytoma had been diagnosed between 1989 and 2006. The patients' ages were 37, 37, and 48 years, and the location of their tumor was C1-3, T9-10, and T-12, respectively. All 3 had complete resection with no adjuvant radiotherapy during follow-up periods of 16, 38, and 185 months, respectively. One patient demonstrated a recurrence 29 months after resection and the other 2 patients have demonstrated asymptomatic recurrences on imaging studies obtained at 16 and 38 months following resection. With these cases added to the available literature, the evidence strongly suggests that complete resection is the treatment of choice for spinal melanocytomas. Even with complete resection, recurrences are common and close follow-up is needed for the long term in these patients. Radiation therapy should be reserved for those cases in which complete resection is not possible or in which there is recurrence.

Dose–response relationship for fractionated irradiation in the treatment of spinal meningeal melanocytomas: a review of the literature

Complete resection (CTR) of meningeal melanocytomas results in better outcome than incomplete resection (ITR). After ITR, outcome is improved by radiotherapy. CTR is less frequent with spinal melanocytomas than with cerebral melanocytomas. This study of 49 spinal melanocytoma patients was performed to define an appropriate radiation dose after ITR. All reported spinal melanocytoma cases were reviewed for extent of surgery, radiotherapy (total dose, dose per fraction), and outcome. CTR was compared to ITR for local control (LC) and overall survival (OS). ITR alone was compared to ITR plus radiotherapy (ITR + RT). In the ITR + RT group, doses of 50-52.2 Gy (1.8-2.0 Gy per fraction) were compared to doses < 50 Gy (30-45 Gy). The 5-year LC was 78% after CTR alone vs. 22% after ITR alone (P < 0.001). Five year OS was 83 and 40% (P = 0.011) respectively. ITR + RT was superior to ITR for LC (59% vs. 22%, P = 0.029) and OS (85% vs. 40%, P = 0.10). In the ITR + RT group, 50-52.2 Gy resulted in better LC than 30-45 Gy (100% vs. 33%; P = 0.042), but not in significantly better OS (100% vs. 77%, P = 0.33). CTR was associated with better significantly outcome than ITR. After ITR, the outcome was significantly improved by radiotherapy, 50-52.2 Gy was significantly superior to 30-45 Gy. To reduce the risk of radiation myelopathy, 50.4 Gy (1.8 Gy per fraction) appears appropriate for most patients. The recurrence rates after CTR would mandate the use of post-operative radiotherapy in all spinal meningeal melanocytoma cases. Our results should be confirmed in a larger series of patients when available.