Melanotic neuroectodermal tumor of infancy: a case report.

BACKGROUND: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare clinically benign, pigmented, tumor of neural crest origin which commonly occurs in the maxilla. It is a rare tumor that may pose difficulty in differentiating from other malignant round cell tumors. CASE PRESENTATION: A 5-month-old Ethiopian infant presented with a mass on his forehead. A wide excision of the lesion was done and subjected to histopathologic evaluation. The histologic and immunohistochemistry for synaptophysin studies confirmed that the infant was having MNTI. The patient was followed and there was no sign of recurrence at the 6th and 9th months of follow-up. CONCLUSION: MNTI should be considered as a differential diagnosis for tumors occurring in the head region in infants and prolonged follow-up may be needed to check for possible recurrence of the tumor.

Melanotic neuroectodermal tumor of infancy arising in the skull: a case report.

Melanotic neuroectodermal tumor of infancy is a rare and usually benign neoplasm occurring in children of young age. This pigmented tumor typically presents in the head and neck region, but other locations may be involved. We report in this article a rare case of a 3-month-old girl presenting with a slowly growing mass localized in the anterior fontanelle. The patient's magnetic resonance imaging (MRI) showed a mass extending both extracranial and intracranial, and compressing the adjacent structures. The patient underwent subtotal resection of the mass and a histological study confirmed the diagnosis of melanotic neuroectodermal tumor of infancy. The patient presented later on with a recurrence. An early diagnosis and surgical management for these tumors remain the only guarantees to limit the progression and prevent their recurrence and metastasis.

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 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.

Computed tomographic imaging features of maxillary and mandibular melanotic neuroectodermal tumor of infancy: Report of 2 cases and systematic review.

OBJECTIVE: The aim of this systematic review was to determine the computed tomographic (CT) imaging characteristics of maxillary and mandibular melanotic neuroectodermal tumor of infancy (MNTI). Two cases from our institution were also presented. STUDY DESIGN: Full-text case reports and case series of histopathologically proven gnathic MNTI with CT figures of diagnostic quality were searched in PubMed, Scopus, Web of Science, Ovid, and Google Scholar databases from July 2021 to February 2022. Descriptive statistics were used to determine the frequency of each CT feature of gnathic MNTI. RESULTS: Fifty-two published studies met the eligibility criteria, providing a total of 53 maxillary and mandibular MNTIs for analysis. In order of frequency, the CT features of gnathic MNTI that were present in over half of the study sample were bone expansion (53, 100%), a well-defined periphery (49, 92.5%), tooth displacement (45, 84.9%), and a bilocular radiolucent internal pattern (32, 60.4%). CONCLUSIONS: The bilocular radiolucent internal pattern has not been recognized as a common CT feature of gnathic MNTI. When associated with a well-defined, expansile mass in the infantile maxilla or mandible, this imaging characteristic can support a radiologic interpretation of MNTI.

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.

Clinical and imaging features of melanotic neuro-ectodermal tumor of infancy of the maxillary bone: report of four cases and review of the literature.

Melanotic neuro-ectodermal tumor of infancy (MNTI) is an extremely rare tumor. The purpose of this study was to describe the imaging features of maxillary bone MNTIs and introduce the key points for clinical diagnosis of MNTI. We retrospectively reviewed four patients with histology-proven MNTIs arising from the maxillary bone. All patients underwent ultrasonic inspections, CT and/or MR scanning. Combined with previously literature, the imaging features were comprehensively evaluated and analyzed. All MNTIs showed a firm, non-ulcerated rapidly-growing soft-tissue swelling with pigmented (blue-colored or black-colored or gray-colored) mucosa. The onset ages were younger than 6 month-old. CT images showed osteolytic or expansive bone destruction of the involved maxillae, fragmentary cortical bone, "free-floating" tooth germs, with or without spiculated/sunburst periosteal reaction. The tumor appeared lightly hyper-intense on T2-weighted sequences, while isointense or lightly hypo-intense or lightly hyper-intense signal on T1-weighted sequences. Enhanced images all displayed heterogeneous enhancement. No metastasis features of lymph nodes or abdominal organs were demonstrated by cervical and abdominal ultrasonic inspections. As a conclusion, accurate recognition of the imaging features of MNTI combined with history and clinical manifestations (early infancy, painless, firm, pigmented mucosa, non-ulcerating lesion) can provide clues for diagnosis of this rare entity.

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.

Pineal anlage tumor: A case report and clinico-pathological review.

Pineal anlage tumor (PAT) is an extremely rare tumor of paediatric population. It is considered as a subtype of pineoblastoma having ectomesenchymal/ rhabdomyoblastic and cartilaginous differentiation. PAT is presumed to have an aggressive behaviour with propensity for craniospinal fluid (CSF) spread due to histologically resemblance with pineoblastoma, thus requiring intensive multimodality treatment with craniospinal irradiation and chemotherapy. Here we report a case of PAT in a 35 years old lady along with clinical and pathological review. To the best of our knowledge only less than ten cases of pineal anlage tumors have been reported in the literature and index case is only second in the adult age group (rare histology in rare age group).

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.

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.

Pigmented Lesions.

Oral pigmented lesions have a wide range of clinical presentations, some of which correlate with cutaneous pigmented lesions. This article highlights these correlates and underscores important differences that can potentially have clinical impact. Moreover, given a nonspecific presentation of an oral pigmented lesion, the article provides a reference to aid clinicians with differential diagnoses based on clinical features. This article is an overview of pigmented lesions of the oral cavity, including localized reactive pigmented lesions, neoplastic pigmented lesions, and pigmented lesions as sequelae of a systemic disease.