Melanotic Neuroectodermal Tumor Research Articles

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.

An unusual occurrence of melanotic neuroectodermal tumor of infancy with malignant features

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, commonly benign, congenital pigmented neoplasm that originates from the neural crest. In contrast to peripheral MNTI usually found in the maxilla, cerebral MNTI has a much worse outcome. We report a case of intracranial MNTI with malignant features in a 2-year-old child. The clinical, radiological, histological, and immunohistochemical findings are described, and the published data on this rare entity are reviewed.

Melanotic neuroectodermal tumour of infancy: Does cytology give a diagnostic clue?

Melanotic neuroectodermal tumour of infancy: Does cytology give a diagnostic clue?

Melanotic Neuroectodermal Tumor of Infancy with a Negative VMA: A Case Report and Review of the Presentation, Etiology, and Management

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare rapidly growing tumor, which although benign, it is locally aggressive and destructive to the surrounding structures. The maxilla is the most commonly involved site, and MNTI is known to be locally aggressive if left untreated. Neural crest cells differentiate into sympathetic neurons that produce catecholamines. These catecholamines degrade, leaving the residual products homovanillic acid and vanillylmandelic acid (VMA). Because of this, patients with MNTI may have high levels of VMA in their urine and should be tested for this if MNTI is suspected. This is a presentation of a healthy 6-month-old girl with a 1-month history of swelling in the anterior maxilla. A computed tomography (CT) scan revealed a radiolucent lesion of the anterior maxilla, which following biopsy, was histologically diagnosed as MNTI, although the patient had normal levels of VMA. A three-dimensional model was reconstructed from the CT scan and used in planning surgical excision. A differential diagnosis of MNTI should be included in cases of infants presenting with fast growing expansile swelling in the head and neck region.

Melanotic neuroectodermal tumor of infancy of the jaw bones: Update on the factors influencing survival and recurrence.

The aim of the present study was to integrate the available data published on melanotic neuroectodermal tumor of infancy (MNTI) of the jaw bones. An electronic search was undertaken in April 2018. Hundred forty-seven publications and 371 patients were included. The lesion was more prevalent in males and in the second to sixth months of life. The lesions mostly presented as a rapidly growing bluish swelling and the most commonly involved was the anterior maxilla. The mean follow-up was 51.1 months (range 1-408 months). Age (P ≤ .0001), location (P = .007), occurrence of lymph node metastasis (P ≤ .0001), treatment (P = .001), recurrence (P ≤ .0001), and distant metastasis (P = .0001) were independently associated with survival. Recurrence was significantly correlated with age (P = .0001), distant metastasis (P = .0001), and treatment (P = .0001). Patients older than 12 months, with lesions in the mandible, positive regional lymph node metastasis, treated with chemotherapy, recurrence, or distant metastasis presented the worst prognosis.

Melanotic Neuroectodermal Tumor of Infancy.

Melanotic neuroectodermal tumor of infancy, albeit rare and generally regarded as benign, is an important tumor to recognize because of its rapid growth, potential for local recurrence, and small round blue cell morphology, which can lead to misdiagnosis of a malignant neoplasm. To review its clinical presentation and immunomorphologic findings, and discuss common entities in the differential diagnosis. The study involved PubMed searches, including multiple review articles, case studies, retrospective studies, selected book chapters, and University of Michigan cases. Melanotic neuroectodermal tumor of infancy most commonly occurs in the bones of the head and neck region during the first year of life, but it can also present in other locations, including the central nervous system, testes, ovaries, and subcutaneous soft tissues. Histologically, it is composed of a biphasic population of cells, consisting of epithelioid melanin-producing cells and primitive neurogenic cells in a fibrocollagenous stroma. These microscopic findings, especially in small biopsies, can lead to a broad differential diagnosis that includes malignant small round blue cell tumors and malignant melanoma. Melanotic neuroectodermal tumor of infancy commonly has an infiltrative growth pattern, and anatomic constraints often lead to incomplete resection and local recurrence, requiring multiple surgical operations. Because melanotic neuroectodermal tumor of infancy can mimic a more aggressive and aggressively treated malignancy, recognition of this rare tumor is very crucial for pathologists.

Melanotic Neuroectodermal Tumor of Infant: Two Case Reports from Sulaimani

Melanotic neuroectodermal tumor of infancy is a rare osteolytic-pigmented neoplasm that primarily affects the maxilla of newborn infants. Two cases of melanotic neuroectodermal tumor of infancy were presented to the department of oral and maxillofacial pathology in Sulaimani Dental College, 2015. Clinical assessment and histologic diagnosis of the classic findings supported the final diagnosis, and no recurrence was detected after one year follow up.

Pigmented lesions of the oral mucosa: A cross-sectional study of 458 histopathological specimens.

To evaluate clinical, demographic, and histopathological characteristics of pigmented lesions of the oral mucosa. A cross-sectional study was conducted over a 64-year period. Information was collected from medical charts, and all archived histopathological specimens with diagnoses of any pigmented lesion were retrieved. Statistical analysis was performed using SPSS software. A total of 34,127 archived specimens were reviewed, revealing 458 (1.34%) pigmented lesions, of which 230 were melanocytic and 228 nonmelanocytic. Most patients were females (74.2%), white-skinned (49.1%), in the third and seventh decades of life (mean of 45years). Most lesions were macular (59.8%), followed by plaques and nodules (4.8%), measuring 0-5mm (41.9%). Cheek mucosa (21.0%), alveolar mucosa (16.6%), and gingiva (11.8%) were the most commonly affected sites. Amalgam tattoo was applied in 212 cases (46.3%), followed by melanotic macule (22.9%) and nevus (20.5%). Other diagnoses included racial pigmentation, exogenous pigmentation, melanotic neuroectodermal tumor of infancy, melanoma, melanoacanthoma, smoker's melanosis, and heavy metal pigmentation. Pigmented lesions represent an uncommon diagnosis in oral pathology routines. The most frequent entities are amalgam tattoo, melanotic macule, and nevus. Patients are usually middle-aged women presenting a small, long-lasting, macular lesion on the cheek mucosa.

Melanotic Neuroectodermal Tumor of Infancy in the Maxilla

Melanotic neuroectodermal tumor of infancy is uncommon and has rapid expansile growth. Melanotic neuroectodermal tumor of infancy is a very rare and benign but locally aggressive neoplasm that originates from neural crest and is composed of relatively primitive pigment-producing cells. The lesion commonly affects the maxilla of infants during the first year of life. Common treatment methods include surgical excision and resection of the tumor. The aim of this article is to present the diagnosis and treatment of a patient with melanotic neuroectodermal tumor that occurred in the anterior maxilla of a 6-month-old male baby.

Difficult Nasal Intubation Using Airway Scope® for a Child With Large Tumor

We report a case of difficult nasal intubation utilizing a Pentax-Airway scope® AWS-100. A 4-month-old female with a rapidly growing melanotic neuroectodermal tumor was scheduled for resection under general anesthesia. The tumor was a large rubbery mass located in the middle of the mandible. For nasal intubation using the AWS, guidance of the tube toward the glottis was attempted using pediatric Magill forceps. Although we could hold the tube with the Magill forceps, it was difficult to insert the tube into the trachea due to the limited space in her hypopharynx. We then used a standard laryngoscope with a Miller straight blade for direct visual laryngoscopy and successfully intubated the patient with the aid of the pediatric Magill forceps. We often experience difficulty navigating a nasal endotracheal tube toward the glottis even when a clear glottic view is obtained with video laryngoscopes, especially in children with a small oropharyngeal space. However, some reports have been shown that video laryngoscopes are useful for intubation of the difficult airway and causes less stress to the upper airway than direct visual laryngoscopy. Video laryngoscopy can be an excellent way to provide nasal intubation in some but not all children.

Melanotic neuroectodermal tumour of infancy: A report of two cases

IntroductionMelanotic neuroectodermal tumour of infancy (MNTI) is a benign tumour of infancy, most commonly affecting the head and neck region. First described in 1918, less than 500 cases have been reported in the literature. MNTI is aggressive in nature & has a high rate of recurrence. Presentation of casesIn this retrospective case series, we report two cases of MNTI that presented at our unit; both cases were managed by wide excision and have been followed up uneventfully for over two years. DiscussionMNTI has a recurrence rate of up to 20%. Patient’s age can play a significant role in recurrence rate. Although this neural crest tumour is somewhat rare in the literature, there is a consensus with regards to surgical management; the gold standard remains to be wide excision with safety margin. Select cases may benefit from adjuvant and neoadjuvant therapy. ConclusionOwing to its locally aggressive nature and high recurrence rate, prompt diagnosis and surgical intervention is advised in cases of MNTI. Further understanding of this tumour is needed on a microscopic level in order to determine clear prognostic factors.

Melanotic neuroectodermal tumor of infancy: A rare case report

Melanotic neuroectodermal tumor of infancy (MNTI) is a very rare neoplasm of infants seen in the oral cavity at or around birth. It shows rapid expansile growth which necessitates proper diagnosis and management. A 6.5-month-old female child reported with a swelling in the maxillary anterior alveolus, which was rapidly increasing in size and was asymptomatic. Radiographic examination showed a diffuse osteolytic radiolucent lesion in the anterior maxillary region and displacement of the developing primary tooth buds. Ultrasonography report revealed well-defined cystic lesion with calcifying foci within and with no internal vascularity. Wide surgical excision under general anesthesia was performed. Histopathological report revealed a nonencapsulated mass composed of a dual population of small round blue cells and larger melanin-containing epithelial cells in a dense cellular fibrous stroma. Tumor is seen to entrap mature bony trabeculae and soft tissue on the whole favoring a diagnosis of MNTI. Early recognition and regular follow-up is the key to successful treatment.

Melanotic neuroectodermal tumor of infancy (MNTI) of the head and neck: A French multicenter study

ObjectivesMelanotic neuroectodermal tumor of infancy (MNTI) of the head and neck is a rare entity with uncertain clinical behavior. Radical surgical resection is the current recommended treatment, however this can cause severe aesthetic and functional sequelae. The aim of this study was to clinically characterize MNTIs and to stratify risk factors that may influence locoregional recurrence. MethodsA retrospective multicenter study, including 11 patients from eight centers with a confirmed diagnosis of MNTI, was conducted. Epidemiological, clinical, radiological, pathological, and immunohistochemical examinations were reviewed. A statistical analysis using a t-test was conducted to calculate parameters correlating with tumor recurrence. ResultsMNTIs mainly occurred in the maxilla, with a mean age at diagnosis of 3.18 months (range: 0–6 months). Primary surgery was performed on 10 patients, with a clear margin resection on two patients. Overall recurrence rate was 27% with a survival of 100% at time of follow-up. No statistical correlation between recurrence rate, age at diagnosis, localization, resection margins, and pathological and immunohistochemical characteristics could be established. ConclusionIn our study, locoregional tumor recurrence did not seem to correlate with resection margins, so a conservative surgical approach may need to be considered to avoid functional and aesthetic sequelae.

MELANOTIC NEUROECTODERMAL TUMOR OF INFANCY: A CASE REPORT

Melanotic neuroectodermal tumor of infancy (MNTI) is a neoplasm that primarily affects children under 1 year of age. A 4-month-old male patient was referred for evaluation of a lesion with 1 month of evolution. On intraoral examination, a submucosal nodular mass, slightly blue, with evident telangiectasia and firm on palpation on the left maxillary alveolar ridge, extending from the midline to the canine area and measuring about 1.5 cm was observed. The patient underwent an incisional biopsy where histologic and immunohistochemical analysis revealed epithelioid cells forming nests, with abundant pigment of melanin, positive for AE1/AE3. Other cell types resembling neuroblasts were present and positive for CD56, synaptophysin, chromogranin A, and enolase. The diagnosis of MNTI was established and the patient was referred for treatment. Surgical resection was performed along with 4 adjacent teeth. The patient is in follow-up for 1 year without recurrence and prosthetic rehabilitation is being considered.

Papillary cystadenoma of right testis: Case report and literature review

Testicular cystadenoma is ranked the second commonest benign neoplasm. Other benign epididymal neoplasms include adenomatoid tumor (most common), leiomyoma, serous (nonpapillary) cystadenoma, cavernous hemangioma, and melanotic neuroectodermal tumor. Adenocarcinoma, mesothelioma, and metastatic renal cell carcinoma are malignant tumors that can affect the epididymis. A 24-year-old male undergraduate with a 3-month history of mildly tender right testicular swelling histologically diagnosed as papillary cystadenoma is presented. This case is presented from our locality as the first of its’ kind; and because it can be a possible manifestation of other diseases like von Hippel- Lindau (VHL) disease.