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 Tumour of Infancy.

Melanotic neuroectodermal tumour of infancy (MNTI) is a locally aggressive neoplasm of neural crest origin. It is primarily a paediatric tumour, and 95% of the cases occur in children below 1 year. The tumour mainly affects the head and neck region. It shows a predilection for the craniofacial sites and the most common site affected is anterior maxilla. Microscopically, it is characterized by a biphasic population of neuroblastic cells and pigmented epithelial cells. Although generally considered as a benign tumour, it can invade the adjacent muscle and bone, causing destruction of the involved site. The rapidity in growth and the aggressive clinical behaviour of the tumour can be deceptive and hence lack of familiarity with the clinical characteristics of the tumour may often lead to an erroneous diagnosis of malignancy. The treatment of choice for MNTI is excision, and it is usually curative. Extensive surgery in a child may interfere with the normal growth and development of the facial structures and reconstruction can be very challenging in infants. Hence, early diagnosis is critical for the effective management. Clinical findings, histopathological features, and differential diagnosis of a classic case of melanotic neuroectodermal tumour of infancy are discussed.

[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.

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 in the mandible: A case report.

RATIONALE: Melanocytic neuroectodermal tumor of infancy (MNTI) is a rare benign pigmented neoplasm that arises from the neural crest and has an aggressive growth pattern. It is predominantly seen in infants under 1 year of age, and the most common site of involvement is the maxilla. The currently accepted treatment is removal by surgical resection. Herein, we report a case of MNTI that involved the anterior alveolar ridge of the mandible in a 6-month-old infant. PATIENT CONCERNS: A case of a 6-month-old male child with a huge mass in the anterior alveolar ridge of the mandible. DIAGNOSIS: The tumor was diagnosed using histopathological and immunohistochemical techniques on the biopsy specimen obtained following incisional biopsy. Based on the findings, a final diagnosis of MNTI was established. INTERVENTIONS: Radical resection of the tumor was performed, after determining the extent of resection by referring to the mandibular 3D model created using the pre-operative CT data. OUTCOMES: The postoperative course was uneventful, and no recurrence has been observed to date for more than 4 years after surgery. LESSONS: This case emphasizes that early diagnosis and radical surgery are critical to the effective treatment, as MNTI exhibits rapid and destructive growth. It also requires careful and close follow-up because of high recurrence rates.

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.

[Orbital melanotic neuroectodermal tumor in an infant].

A 3-month-old infant patient presented with a 1-week history of a quickly progressive mass in the left eye. A hard, irregularly shaped tumor was found in the superolateral orbit. B-scan showed an uneven hypoecho and an undefined lesion. On the orbital CT, a soft tissue mass was located around the left zygomatic tubercle, involving part of the bone and lacrimal gland. The left orbital mass was removed under general anesthesia after admission. The histological and immunohistochemical examination revealed it was a melanotic neuroectodermal tumor. The infant patient was followed up for 6 months after the operation, and no recurrence was found. (Chin J Ophthalmol, 2021, 57: 372-374).

Melanotic Neuroectodermal Tumor of Infancy in the Ulna of an Infant: A Very Rare Tumor: A Case Report.

CASE: A 4-month-old boy presented with a nontender swelling of the right proximal forearm of 2 months' duration. Radiological evaluation showed lytic lesion surrounded by sclerotic bone in the ulna with soft-tissue extension. Histopathological examination showed tumor of round and epithelioid cells containing melanin, interpreted as melanotic neuroectodermal tumor. The patient underwent a wide excision of the shaft of the ulna with creation of radioulnar synostosis. There is no recurrence 6 years after surgery. CONCLUSION: Melanotic neuroectodermal tumor is rare in the appendicular skeleton and has to be differentiated from other round cell tumors and osteomyelitis. As far as we know, this is the first reported case in the ulna.

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.

Melanotic neuroectodermal tumor of infancy successfully treated with metformin: A case report.

RATIONALE: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare tumor originated from neural crest cells with the potential for recurrence and metastasis. The peak age for the disease is during the first year after birth. The current therapy is primarily surgery. The patient reported here is the first case of MNTI treated with metformin. PATIENT CONCERNS: A case of a 4-month-old infant with a history of swelling in the mouth for 1 month. DIAGNOSIS: The tumor was diagnosed using radiology, pathology, and immunohistochemistry, and it was performed with complete surgical resection. Unfortunately, the tumor recurred 3 months after surgery. INTERVENTIONS: We prescribed metformin for the infant. OUTCOMES: Currently, after 9 months of treatment, the tumor is well controlled without apparent side effects. LESSONS: The case presented suggested that metformin may be an underlying therapy for MNTI.

Oral melanotic neuroectodermal tumor of infancy: Management of a case affecting the maxilla.

Melanotic neuroectodermal tumor is a rare benign congenital neoplasm involving the head-and-neck region found in infants <1 year of age. The lesion most commonly affects the maxilla. We report the case of a 4-month-old male child with a tumor involving the anterior region of the maxilla along with a brief review of literature, differential diagnosis, and treatment.

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 from the skull: report of an unusual case, review of the literature, and a diagnostic approach.

INTRODUCTION: Melanotic neuroectodermal tumor of infancy is a rare neoplasm mainly seen in children under 1 year of life. The most common location of the tumor is the maxilla followed by the cranial vault. Surgery is the treatment of choice and outcome mainly depends on extent of resection. OBJECTIVES: To report an atypical case of an 8-year-old patient with a melanotic neuroectodermal tumor of infancy, to review the cases with melanotic neuroectodermal tumor of infancy arising from the skull published over the last 13 years, and to provide a diagnostic approach that may allow recognition of a pattern in these rare neoplastic lesions. METHODS: A case is reported with a description of the clinical, radiological, surgical, and histopathological features. Additionally, the literature was reviewed to identify reports of patients with melanotic neuroectodermal tumor of infancy arising from the cranial vault and all cases published in PubMed over the last 13 years were included. Only studies that evaluated clinical, radiological, surgical, and histopathological findings were included. CONCLUSION: Melanotic neuroectodermal tumor of infancy is a rare entity that may present with unusual features, but nevertheless has an identifiable pattern that allows the tumor to be considered in the differential diagnosis of intracranial space-occupying lesions in children.

Melanotic neuroectodermal tumor of infancy in ovary: A rare case report.

RATIONALE: Melanotic neuroectodermal tumor of infancy (MNTI) is an extremely rare benign pigmented neoplasm of neural crest origin with rapid expansile growth and a high recurrence rate. It is predominantly found in infants of <1 year of age, involvement of the head-and-neck region is the most common presentation though it is reported at other sites including mediastinum, shoulder, thigh, foot, epididymis, uterus and ovary. The patient reported here is the third case of MNTI presenting in an ovary, and the first reported in the infant ovary. PATIENT CONCERNS: A 33-month-old girl was presented to our unit for a huge abdominal mass. DIAGNOSIS: MNTI was eventually diagnosed by histological manifestations supplemented with immunohistochemical findings. INTERVENTIONS: Exploratory laparotomy and complete resection were conducted successfully. OUTCOMES: Postoperative course was uneventful and no recurrence was displayed in the 6-month follow-up. LESSONS: This case emphasizes that pediatric surgeons and pathologists must always consider the possibility of MNTI while dealing with ovarian neoplasms in infants. Although considered to be a benign tumor, proper treatment and close clinicoradiological follow-up of this tumor are of great importance.

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 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.

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.

Melanotic neuroectodermal tumor of infancy: A rare pediatric head and neck lesion.

Melanotic neuroectodermal tumors of infancy are rare tumors arising from the neural crest and typically present during the first 12 months of life. The majority involve the facial bones, although melanotic neuroectodermal tumors of infancy of the skull and extremities have been observed with less frequency, as in the present case. This entity may initially be presented to the dermatologist as a scalp mass and should be considered in the differential diagnosis of infants with rapidly growing head and neck lesions.