Melanotic Neuroectodermal Tumor Of Infancy Research Articles

The Role of Chemotherapy in Management of Inoperable, Metastatic and/or Recurrent Melanotic Neuroectodermal Tumor of Infancy-Own Experience and Systematic Review.

Simple SummaryMelanotic Neuroectodermal Tumor of Infancy (MNTI) is a very rare neoplasm that most commonly develops within maxilla in infants. It usually has a benign clinical course and is treated with only surgery. However, patients with large, inoperable, metastatic or multiply recurring MNTI may require systemic treatment. The role of pre- and post-surgery chemotherapy (CHT) in the management of MNTI is unclear. Here, we have presented the disease courses and outcomes of four infants treated with multidrug CHT due to inoperable/recurrent MNTI. Additionally, a systematic literature review was performed which revealed 38 similar cases in the last 42 years. Most children with primarily inoperable MNTI responded to CHT, which allowed physicians to perform complete, non-mutilating delayed surgery. However, it is still uncertain whether CHT administered after incomplete resection of MNTI prevents recurrence. This study aimed to contribute to the establishment of standards of management in patients with inoperable, metastatic or persistently recurring MNTIs, which are currently lacking.Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a very rare pediatric neoplasm of neural crest origin. In most cases, it develops in infants as a localized tumor of the maxilla, and surgery is usually curative. In less than 10% of patients with inoperable, metastatic or persistently recurring MNTI, chemotherapy (CHT) may be considered; however, its role is still unclear. The aim of our study was to assess the efficacy of CHT in children with large, inoperable, metastatic and/or recurrent MNTI. Four such infants, treated with CHT in Polish and German centers of pediatric oncology, were presented. Additionally, a systematic literature search of the PubMed/MEDLINE, Scopus and Web of Science databases was performed, yielding 38 similar cases within the last 42 years. Neoadjuvant CHT, based mainly on the protocols for neuroblastoma, was often effective, allowing for complete delayed surgery in most cases. However, the role of adjuvant CHT in preventing recurrences after incomplete resection of MNTI remains unclear. Disseminated inoperable MNTI was almost universally associated with poor response to CHT and unfavorable outcome. Further investigations to elaborate standards of management in patients with inoperable, metastatic or persistently recurring MNTIs are necessary to improve outcomes.

Melanotic Neuroectodermal Tumor of Infancy: A Clinicopathological and BRAF V600E Mutation Study of 11 Cases.

PurposeTo investigate the clinicopathological features and BRAF V600E mutation of melanotic neuroectodermal tumor of infancy (MNTI).Materials and MethodsEleven cases of MNTI diagnosed at the Department of Oral Pathology were collected. Clinicopathological characteristics were obtained from the medical records. Immunostaining was performed by immunohistochemistry (IHC). Amplification-Refractory Mutation System-qPCR (ARMS-qPCR) and Sanger Sequencing were used to detect BRAF V600E mutation.ResultsOf the 11 cases, 3 cases were female and 8 cases were male. The mean age of the first symptoms was 3.2 months (range: 1 to 6 months). Ten cases (90.9%) located in maxilla but only one (9.1%) in mandible. Most of the cases demonstrated well-defined mass with lytic bone destruction and tooth germ affecting radiologically. Histologically, MNTI was consisted of large polygonal melanin-producing epithelioid cells and small round neuroblast-like cells which arranged in irregular alveolar, tubuloglandular and fissured architecture. The epithelioid cells expressed Vim, Pan-CK, NSE and HMB45, while the smalls cells expressed Syn, NSE and scattered Vim. Most cases showed low Ki-67 index (range: <1% to 50%). None of the MNTI cases showed BRAF V600E mutation. Most cases were treated with enucleation (45.4%) or curettage (36.4%). Among the 11 cases, 6 cases had follow-up information, and 2 cases had recurrence lesions after surgery.ConclusionMNTI, an extremely rare tumor, mainly affects male infants with strong preference for maxilla. Distinct histopathological features and immunohistochemical profile are helpful to distinguish from other melanin-containing tumors and small round cell tumors. No BRAF V600E mutation in MNTI is detected in the present study and needs further investigations. The factors that contribute to the local recurrence of MNTI are controversial, but the close follow-up for the patients is recommended.

Melanotic Neuroectodermal Tumor of Infancy in the Epididymis: A Rare and Considerable Cause of Scrotal Mass

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign neoplasm with a predilection for children that predominantly involves the craniofacial region. Here we report 2 cases of MNTI involving epididymis, placing emphasis on the sonographic features. Both appeared to be hypoechoic, regular shaped masses with abundant blood supplies. The unique sonographic features and age of predilection make it possible to diagnose MNTI within the scrotum by ultrasonography.

Rehabilitation following surgical management of MNETI: A splint in time saves nine!

IntroductionMelanotic neuroectodermal tumour of infancy (MNTI) is a rare, rapidly growing pigmented neoplasm of neural crest origin generally arising in infants during the first year of life. The rehabilitation of the anterior defect is necessary to circumvent feeding problems. The area is also a site for primary growth of maxilla and placing a hard splint would restrict the same. We have used a novel way to use a soft splint to overcome this issue with follow up and re-fabrication. CaseWe report a 7 months old male who presented with a rapidly growing mass history of a rapidly growing mass in the upper front part of the jaw A biopsy showed melanoticneuroectodermal tumour, and complete resection with negative margins was subsequently achieved. The patient was reviewed at follow up with a magnetic resonance imaging, which showed no residual remanants. Rehabilitation was done with a soft splint to avoid restriction of maxillary growth. ConclusionDue to its rapid growth potential and locally destructive behaviour, early diagnosis is extremely important to limit local expansion. The treatment of choice for melanotic neuroectodermal tumour of infancy (MNTI) is surgical excision. Rehabilitation is also important with due attention to avoiding restriction maxillary growth, this was overcome by use of soft splint which has scantly been reported in literature.

Rehabilitation following surgical management of mneti: a splint in time saves nine!

Introduction: Melanotic neuroectodermal tumour of infancy (MNTI) is a rare, rapidly growing pigmented neoplasm of neural crest origin generally arising in infants during the first year of life. The rehabilitation of the anterior defect is necessary to circumvent feeding problems. The area is also a site for primary growth of maxilla and placing a hard splint would restrict the same. We have used a novel way to use a soft splint to overcome this issue with follow up and re-fabrication. Case report: We report a 7 months old male who presented with a rapidly growing mass history of a rapidly growing mass in the upper front part of the jaw a biopsy showed melanotic neuroectodermal tumour, and complete resection with negative margins was subsequently achieved. The patient was reviewed at follow up with a magnetic resonance imaging, which showed no residual remnants. Rehabilitation was done with a soft splint to avoid restriction of maxillary growth. Conclusion: Due to its rapid growth potential and locally destructive behaviour, early diagnosis is extremely important to limit local expansion. The treatment of choice for melanotic neuroectodermal tumour of infancy (MNTI) is surgical excision. Rehabilitation is also important with due attention to avoiding restriction maxillary growth, this was overcome by use of soft splint which has scantly been reported in literature.

Melanotic neuroectodermal tumor of infancy: a clinicopathological study of three cases

Melanotic neuroectodermal tumor of infancy: a clinicopathological study of three cases

Melanotic neuroectodermal tumor of infancy: An immunohistochemical study and presentation of two cases

Melanotic neuroectodermal tumor of infancy: An immunohistochemical study and presentation of two cases

Oral pigmented lesions: a retrospective analysis from Brazil.

Background Pigmented lesions are uncommon in the oral mucosa, and studies investigating the incidence and types of these lesions are desired to improve the diagnostic knowledge of clinicians. The aim of this study was to analyze the distribution of oral pigmented lesions in a Brazilian population. Material and Methods A retrospective descriptive cross-sectional study was performed. Oral pigmented lesions were retrieved from the files of two oral and maxillofacial pathology services from Brazil over a 45-year period (1974-2019). The clinical data and the diagnoses of each case were retrieved and included in a Microsoft Excel® database. Results From 77.074 lesions diagnosed in this period, 761 (0.99%) represented pigmented lesions of the oral mucosa, including 351 (46.1%) melanocytic and 410 (53.9%) non-melanocytic lesions, with a higher incidence in females (73.2%) between the fourth and seventh decades of life. Amalgam tattoo (53.6%) represented the most common lesion, followed by melanotic macule (18.3%) and racial pigmentation (10.8%). Other pigmented lesions included nevus (9.9%), post-inflammatory pigmentation (3%), melanoma (2.1%), melanoacanthoma (1.4%), smoker's melanosis (0.4%), drug-induced pigmentation (0.3%), and melanotic neuroectodermal tumor of infancy (0.1%). The buccal mucosa was the most commonly affected site (25.2%), followed by the alveolar ridge (14.5%), and gingiva (11.8%). Conclusions The current findings were similar to previous studies with minor differences due methodology and characteristics of the services from where lesions were retrieved. The knowledge of these data may contribute to a better understanding of oral pigmented lesions and assist clinicians to better recognize and manage them. Key words:Pigmented lesions, pigmentation, melanin, amalgam, oral cavity.

A Case Report on Melanotic Neuroectodermal Tumor of Infancy and Review of the Literature

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign neoplasm of neural crest origin that most commonly occurs in the first year of life. The craniofacial area is affected 90% of the time with the maxilla being the affected site in the majority of cases. It has a recurrence rate of up to 27% and is associated with malignant transformation in up to 6.5% of patients. The current case describes the clinical presentation and management of a 3-month old male infant with MNTI of the maxilla. Following initial presentation, the tumor grew rapidly to compromise the aerodigestive tract. Surgical resection was performed and the adjacent bone was burred. There has been no clinical recurrence over a 4-year period of follow up.

Ototoxicity after platinum-based chemotherapy in the treatment of melanotic neuroectodermal tumour of infancy.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare infantile tumor that originates from mesenchymal-neuroectodermal cells, the treatment of which uses platinum derivatives that can affect hearing loss. The present study evaluated the long-term effects of ototoxicity following chemotherapy with cisplatin, vincristine, cyclophosphamide, teniposide and adriamycin in a 10-year-old patient after surgical removal of a MNTI tumor at the age of 8 months. Audiometric tests (high-frequency tonal audiometry, speech audiometry, speech acoustics, tympanometry and absorbance measurements) were performed during a 10-year follow-up after receiving chemotherapy. Hearing disorders in the high-frequency range (6,000 to 16,000 Hz range) were demonstrated for both ears, indicating that these may be the long-term effects of chemotherapy with use of platinum compounds during the treatment of infants.

Melanotic Neuroectodermal Tumour of Infancy: Clinical Courses and Therapeutic Options-A Review of Three Cases.

Melanotic neuroectodermal tumour of infancy (MNTI) is a rare benign neoplasm. MNTI appears most often during the first year of life, arises predominantly in the maxilla and tends to recur. We discuss possible therapeutic options given in the literature and within our experience in three cases. In our recent case, we used an intraoral approach to perform resection of the right-sided maxilla. Despite tumour-positive margins, there was no recurrence over the course of one year. In a previous case of MNTI, two recurrences occurred and 6months after last resection patient received a rib graft for maxillary reconstruction. However, at the age of 7years, the infant displayed severe maxillary hypoplasia. In a third case of MNTI, the patient was followed up after initial therapy for two decades and underwent multiple reconstruction procedures to achieve successful rehabilitation. Surgical treatment of MNTI should respect vital anatomic structures to avoid gross mutilation. The need for extended and repetitive tumour resection in early childhood can lead to growth disturbances and to further multiple reconstruction procedures in adulthood. Because of the rarity of MNTI, an international database is warranted to evaluate therapies and clinical courses over decades.

Melanotic neuroectodermal tumour of infancy with unexpected peri-operative complications

Melanotic neuroectodermal tumour of infancy with unexpected peri-operative complications

Melanotic Neuroectodermal Tumor of Infancy: Presentation of a Case Affecting the Mandible

Abstract- Melanotic neuroectodermal tumor of infancy (MNTI) is a rare and distinctive neoplasm of early infancy with rapid expansile growth and a high rate of recurrences. Most commonly the lesion affects the maxilla of infants during the first year of life, but it may also occur in the mandible, skull, brain, epididymis, and other rare locations. Common treatment methods’ include surgical excision and resection of the tumor. The aim of this article was to show the diagnosis and treatment of a 7-month-old patient with melanotic neuroectodermal tumor occurred in the anterior mandible and to demonstrate the effectiveness of the neoadjuvant chemotherapy.

Melanotic neuroectodermal tumor of infancy: a description of the experience of Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology and literature review

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare type of tumor with uncertain clinical behavior. The tumor commonly occurred in the head and neck region. Radical surgical resection is the standard of treatment, which is often associated with high risks of severe postoperative complications due to tumor localization. Alternative methods of treatment including chemotherapy/radiotherapy are used in metastatic, locally advanced and relapsed cases of MNTI. The aim of this study was to analyze clinical and morphological characteristics of the tumor, evaluate the effectiveness of various treatment options, and to describe two clinical cases of MNTI.

Rare Testicular Neoplasm: Melanotic Neuroectodermal Tumor of Infancy

Abstract We report the case of an 18-month-old boy who presented with a several-week history of left testicular swelling. On physical examination, the testicle was firm, enlarged, and nontender to palpation. The patient’s urinary habits and family history were unremarkable. An ultrasound was performed that revealed an enlarged left testis (4.7 cm) with a hypervascular heterogeneous appearance and scattered echogenic foci that raised concern for a neoplastic process. Microlithiasis was identified in the right testis (1.6 cm). AFP and hCG were within normal limits, and LDH was mildly elevated. An orchiectomy was performed and gross dissection revealed a 4.5 × 3.5 × 3.0-cm mass. The tumor was biphasic and composed of a population of poorly differentiated small round blue cells (neuroblastoma-like) and a separate component of nested epithelioid cells that had abundant cytoplasm with melanotic granules. Mitotic and karyorrhectic figures were scattered throughout. Epididymis infiltration was present. The epithelioid cells were pan-cytokeratin, EMA, and HMB-45 positive. The small round blue cells were positive for synaptophysin, vimentin, and CD117. A subpopulation was also moderately positive for CD99. A Phox2B and TDT were negative in the tumor cells, which helped to exclude neuroblastoma and lymphoma. The combined features of this case were diagnostic for melanotic neuroectodermal tumor of infancy (MNTI). This is a rare tumor with a strong predilection for the head and neck region with testicular/paratesticular localization accounting for less than 5% of MNTI cases. MNTIs are classically seen in infants under 1 year of age and typically behave in a benign fashion, although metastases to retroperitoneal lymph nodes and lymphatics have been documented. It is important to remember the differential of pediatric testicular neoplasms as it can mimic other small round blue cell tumors.

An Alternate Method of Fabrication of an Obturator in a Patient Diagnosed with Melanotic Neuroectodermal Tumor of Infancy

AbstractThe quality of treatment and rehabilitation for the pediatric head and neck cancer patient has greatly improved due to interdisciplinary management. Surgical resection of maxilla leads to functional impairment which can be adequately restored with an obturator. However, its fabrication can be challenging in a pediatric patient. A simple, practical, and time-saving procedural modification in the fabrication of obturator in a patient diagnosed with rare melanotic neuroectodermal tumor of infancy has been presented.

Melanotic neuroectodermal tumour of infancy: A clinicopathological study with emphasis on histopathology and IHC

Melanotic neuroectodermal tumour of infancy: A clinicopathological study with emphasis on histopathology and IHC - IJMPO- Print ISSN No: - 2581-4699 Online ISSN No:- 2581-4702 Article DOI No:- 10.18231/j.ijmpo.2019.005, IP International Journal of Medical Paediatrics and Oncology-IP Int J Med Paediatr Oncol

Melanotic neuroectodermal tumor of infancy

Abstract Introduction Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, rapidly growing pigmented neoplasm of neural crest origin generally arising in infants during the first year of life. Case We report a 15-month old male who presented with a 2-month history of a rapidly growing mass in the anterior. A biopsy showed melanotic neuroectodermal tumor, and complete resection with negative margins was subsequently achieved. The patient is in remission at 11 months from surgery. Conclusion Due to its rapid growth potential and locally destructive behaviour, early diagnosis is extremely important to limit local expansion. The treatment of choice for melanotic neuroectodermal tumor of infancy (MNTI) is surgical excision.

Congenital Granular Cell Epulis: Classic Presentation and Its Differential Diagnosis

Congenital granular cell epulis (CGCE) is an uncommon lesion of unclear pathogenesis. It occurs predominantly in female newborns with a predilection site of the maxillary alveolar ridge. The mass enlarges prenatally but the growth arrests after birth. Histologically, CGCE is characterized by a proliferation of polygonal cells with eosinophilic, granular cytoplasm and eccentric, bland-appearing nuclei. It closely resembles adult granular cell tumor (GCT) microscopically and S-100 immunostain is often helpful in distinguishing the two (S-100-positive in GCT and S-100-negative in CGCE). Clinically, the lesion should also be distinguished from entities such as infantile myofibroma, rhabdomyoma, melanotic neuroectodermal tumor of infancy, peripheral odontogenic fibroma, and neurofibroma. CGCE demonstrates an excellent prognosis and has not been associated with any syndromes/genetic defects or malignant transformation. Clinicians and pathologists should be familiar with this rare entity and its differential diagnosis for accurate diagnosis and management.

Melanotic neuroectodermal tumour of infancy: Refining the surgical approach

Melanotic neuroectodermal tumours of infancy (MNTI) are particularly rare and although predominantly benign, are infiltrative and locally aggressive. Presenting in the first year of life, prompt diagnosis and effective management are critical in minimizing morbidity and the risk of recurrence.A retrospective review of 11 MNTI managed at Great Ormond Street Hospital (GOSH) from 2000 to 2017 was undertaken. Eight tumours presented in the maxilla, two in the skull and one in the mandible. The primary modality of treatment was surgery in 10 cases with one patient receiving neoadjuvant chemotherapy. In spite of microscopically incomplete resection in seven cases, only three recurred. Overall, there was a local recurrence rate of 27% with no distant metastases noted.Disease-free survival was 100% with a follow-up ranging from 0.75 to 17 years (median 5 years). Taking our results in conjunction with the available literature, there is a role for conservative initial surgery of MNTI and this should be coupled with delayed reconstruction and intensive short-term follow-up. We propose an adapted treatment algorithm that aims to balance the risk of recurrence and malignant change with surgical morbidity in an infant population.