Melanotic Neuroectodermal Tumor of Infancy: A Case Image.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare benign neoplasm that can be mistaken for malignancies due to its unfamiliarity among clinicians and aggressive clinical appearance. We herein contributed by reporting an additional case of MNTI characterized by an extensive extraoral protrusion in a 2-month-old infant. The lesion involved the anterior maxilla, cheek, and infraorbital region, resulting the displacement of the nose to the contralateral side, and measuring approximately 10 cm in size. Surgical resection of the lesion was performed. After a 6-month follow-up, the patient has shown no evidence of recurrence. The rapid growth and aggressive behavior of MNTI emphasize the importance of an early diagnosis and prompt intervention in order to achieve favorable outcomes.

Melanotic Neuroectodermal Tumor of Infancy in the Epididymis: A Case Report.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign neoplasm of neural crest origin that predominantly involves the craniofacial region, involvement of the epididymis being extremely rare, with about 30 cases reported. We report an unusual case of a 5-month-old male with MNTI in the epididymis. The patient underwent orchiectomy. Half a year later, there was no sign of recurrence. Whether preoperative examination or intraoperative frozen examination, the tumor may easily be misdiagnosed as malignancy. Melanotic neuroectodermal tumor of infancy should be included in differential diagnosis in infants presenting with fast-growing scrotal swelling.

[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.

A case report of melanotic neuroectodermal tumor of infancy complicated with congenital heart disease and hypothyroidism.

To the best of our knowledge, thus far there are no reported cases of melanotic neuroectodermal tumor of infancy (MNTI) with multiple complications. In this case report, we describe the clinical phenotype of MNTI in a 9-month-old female infant associated with tetralogy of Fallot (TOF), a congenital heart defect, and congenital hypothyroidism (CH). Our study showed that the growth of MNTI was delayed by a lower dosage of levothyroxine (L-T4) that was prescribed to treat CH because of the presence of TOF, a severe congenital heart disease. However, the standardized dosage of L-T4 improved thyroid function but stimulated the rapid growth of MNTI. Our report demonstrated that treatment with L-T4 affects the progression of MNTI. Our findings demonstrated the role of thyroid hormone in MNTI growth and progression. Furthermore, our study suggested that the treatment of co-morbidities in children with MNTI requires careful consideration of their effects on the growth and progression of MNTI.

Melanotic neuroectodermal tumor of infancy: Case report and literature review.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare benign tumor. Here, we report the diagnosis and treatment of 1 case of MNTI in the maxilla and discuss its clinical and pathological features, imaging features, treatment, and prognosis.

Melanotic neuroectodermal tumor of infancy (MNTI) - A rare entity.

Melanotic neuroectodermal tumor of infancy is a rare pigmented pediatric tumor seen at craniofacial sites with the most common site being maxilla. This tumor arises from neural crest origin with a polyphenotypic expression of epithelial, neuroblastic, and melanotic markers. It is a locally aggressive tumor with rapid, expansile, and destructive growth. The tumor has fairly high chances of recurrence and malignant transformation, if not diagnosed and treated with time. There is no standard protocol for management owing to its rarity. Hereby, we present one such case of a 2-month-old male child with rapidly enlarging upper jaw swelling. The patient was treated with wide local excision, followed by two cycles of chemotherapy. The patient is in follow-up and doing well with no evidence of any local recurrence or metastasis till date.

Melanotic Neuroectodermal Tumor of Infancy: A Rare Case Report.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, pigmented tumor. It is a locally aggressive neoplasm of neural crest origin with a high recurrence rate. It occurs in craniofacial sites in more than 90% of cases and most commonly in the maxilla. It may also occur in epididymis, testis, ovaries, soft tissue, and bones of the extremities. It occurs in infants younger than 1 year of age with a slight male preponderance. We report a rare case of a child presenting with midline frontal pigmented MNTI.

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

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 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.

Calvarial Melanotic Neuroectodermal Tumor of Infancy with Rhabdomyosarcomatous differentiation-A Rare Case.

BACKGROUND: Malignant neuroectodermal tumor of infancy is a rare neural crest cell-derived neoplasm of infants. Histologically, melanotic neuroectodermal tumor of infancy usually consists of 2 types of cells: neuroblast-like and melanocyte-like cells. Here we present a rare case of melanotic neuroectodermal tumor of infancy containing a third type of cell population, that is, rhabdomyoblasts in addition to the above two. CASE DESCRIPTION: We report a case of a 10-month-old female child who was brought to us with complaints of swelling over the right forehead for the last 9 months, which started increasing in size rapidly 3 months before presenting to us. Noncontrast computed tomography scan showed a large well-defined extra-axial lesion in the right frontotemporal region. The child underwent an open biopsy under general anesthesia. Histopathological sections showed a malignant small round cell tumor consisting of hyperchromatic cells lying in sheets and lobules separated by fibrous septae. The patient underwent 7 cycles of neoadjuvant chemotherapy over a period of 2 months. The patient underwent right frontotemporal craniotomy and gross total excision of the lesion as a definitive surgery. Postoperatively, the patient was stable, and there was no new deficit. Histopathology revealed neuroblast-like and melanocyte-like cells with rhabdomyosarcomatous differentiation. The patient received chemotherapy in the postoperative period. The patient had recurrence of the tumor and died 8 months after the surgery. CONCLUSIONS: Calvarial malignant neuroectodermal tumor of infancy with rhabdomyosarcomatous differentiation is a rare entity with no cases being reported before. Neoadjuvant chemotherapy with surgical excision can be a promising modality of treatment.

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 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.

Melanotic Neuroectodermal Tumor of Infancy Arising in the Skull and Brain: A Systematic Review.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare pigmented craniofacial tumor of infants. This study aimed to analyze the literature on MNTI arising from the skull and brain. A systematic literature review was performed and 91 patients reported in 78 articles were identified from 1918 to 2019. MNTI of the skull and brain was slightly more prevalent among boys. The age at diagnosis of patients with intracranial MNTI was mainly >1 year, and most patients with skull MNTI were diagnosed within the first year of life (P < 0.001). MNTI of the skull mostly presented as a rapidly growing mass, whereas intracranial MNTI presented with increasing intracranial pressure and neurologic dysfunction. Surgical resection was the primary treatment, with an average follow-up of 25.6 months. Overall survival was related to age at diagnosis within 1 year (P = 0.001), tumor location (P < 0.001), tumor size (P = 0.010), treatment (P < 0.001), and metastasis (P < 0.001) and malignancy (P < 0.001), whereas recurrence rate was significantly associated with age (P < 0.001), tumor size (P = 0.010), complete surgical resection (P = 0.011), metastasis (P = 0.003), and malignant behavior (P = 0.001).

Melanotic neuroectodermal tumor of infancy: A rare entity.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm with a predilection for the head-and-neck region. Approximately 472 cases reported in literature till date. We report an unusual case of MNTI in a 1-month old child. A 10 cm × 5 cm × 5 cm nontender, firm, deep-seated swelling was seen involving the right zygomatic and maxillary region. Magnetic resonance imaging was suggestive of rhabdomyosarcoma and immunomorphological assessment of the excision specimen revealed the final diagnosis of MNTI. On follow-up, the patient developed recurrence 4 months after surgery and was reoperated. The present case emphasizes that albeit rare, pediatric surgeons and pathologists must always keep in mind the possibility of MNTI while dealing with maxillofacial tumors in infants. Owing to rather nonspecific clinicoradiological features, histopathology supplemented with immunohistochemistry remains the gold standard for diagnosis. Although considered to be a benign tumor, close clinicoradiological follow-up is strongly recommended given the significant risk of recurrence as highlighted by our case.

Melanotic Neuroectodermal Tumor of Infancy: A Rare Case Report.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare pigmented neoplasm of neural crest origin that most commonly arises in the maxilla of neonates and infants during the first year of life. We report the case of a three-month-old female child who presented with a 15-day history of intraoral swelling. Radiologically, the tumor was detected in the right maxilla, which was removed by wide local excision. Histopathological examination revealed the biphasic population of cells with melanin pigment deposition. Immunohistochemistry study was done confirming the diagnosis of MNTI.

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: a clinicopathological case report

Abstract Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that affects mainly children under 1 year of age. A 4-month-old boy was referred for evaluation of a lesion with 1 month of evolution. Intra-oral examination detected a firm upon palpation submucosal nodular mass, measuring 1.5 cm in diameter, affecting the anterior maxillary alveolar ridge and covered by a slightly blue mucosa with evident telangiectasia. The patient underwent an incisional biopsy and histological and immunohistochemical analyses revealed nests of AE1/AE3 positive epithelioid cells with abundant melanin pigmentation. Other cell types, resembling neuroblasts, were also present and positive for CD56, synaptophysin and enolase. The diagnosis of MNTI was established and the patient was referred for treatment. Conservative surgical resection was performed along with 3 adjacent teeth under general anesthesia. The patient is in follow-up for 1,5 year without recurrence. Conservative surgical management of MNTI may be an alternative to maxillectomy, contributing to the patient´s quality of life.

Resumo Tumor neuroectodérmico melanótico da infância (TNMI) é um neoplasma raro que afeta principalmente crianças com idade abaixo de 1 ano. Um menino de 4 meses foi referenciado para avaliação de uma lesão com 1 mês de evolução. O exame intra-oral detectou uma massa nodular submucosa firme à palpação, medindo 1,5 cm de diâmetro, afetando rebordo alveolar anterior da maxila e recoberta por mucosa de coloração levemente azulada com telangiectasia evidente. O paciente foi submetido à biopsia incisional e as análises histológica e imunohistoquímica revelaram ninhos compostos por células com abundante pigmento de melanina, positivas para AE1/AE3. Outro tipo celular, semelhante à neuroblastos, também estava presente e foram positivas para CD56, sinaptofisina e enolase. O diagnóstico de TNMI foi estabelecido e o paciente encaminhado para tratamento. Ressecção cirúrgica conservadora sob anestesia geral ao longo de 3 dentes adjacentes foi realizada. O paciente está em acompanhamento há 1 ano e meio sem sinais de recorrência. O tratamento cirúrgico conservador do TNMI pode ser uma alternativa à maxilectomia, contribuindo para a qualidade de vida do paciente.

Use of Technological Aids in the Resection of a Rare Maxillofacial Tumor of Infancy.

Melanotic neuroectodermal tumor of infancy is a rare maxillofacial tumor, usually presenting within the first year of life, often a benign locally aggressive lesion, with wide local excision providing the best opportunity of cure. In past reports, direct tissue visualization guided resection, with occasional need for reoperation to clear margins. In this case report, a method is presented to achieve clear margins in surgery using virtual surgical planning and surgical stealth navigation.

Melanotic neuroectodermal tumor of infancy: A rare case report.

Melanotic neuroectodermal tumor of infancy is a rare benign but locally aggressive neoplasm of neural crest origin with a high recurrence rate. It usually affects infants of < 1 year of age. Involvement of maxilla of an infant is the most common presentation although cases outside this setting have been reported. We report the case of a 6-month-old male child presenting with intraoral swelling. Radiologically, a tumor was detected in the right maxilla, and wide local excision was done. Histological examination revealed typical biphasic population of cells with deposits of melanin pigment. Immunohistochemistry study was done confirming the diagnosis.

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

OBJECTIVES: Melanotic 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. METHODS: A 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. RESULTS: MNTIs 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. CONCLUSION: In 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.