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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., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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., (© 2022. The Author(s).)

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) .

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., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/B377)., (Copyright © 2021 by The Journal of Bone and Joint Surgery, Incorporated.)

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., (© 2021 S. Karger AG, Basel.)

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.

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., Competing Interests: None

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.

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 (MNTI) is a rare, benign neoplasm of neural crest origin more commonly seen in the craniofacial region. We report a case of MNTI of the epididymis in a 6-month-old male child with emphasis on the sonographic appearance which has not been previously described. In this case, the mass was inseparable from the testicle and therefore the differential diagnosis considered both extratesticular and intratesticular masses. MNTI should be added to the differential diagnosis of scrotal masses, particularly if they present in a child younger than 12 months of age., (© 2018 Wiley Periodicals, Inc.)

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 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 tumour of infancy is an uncommon pigmented neoplasm of neural crest origin. It was first described in 1918 by Krompecher, known as congenital melanocarcinoma at that time. Although it is generally agreed upon that it is a benign entity, it is locally aggressive and has a significant recurrent risk, reported to be between 10-15%. There have also been prior reports of malignant behaviour in these tumours, although extremely rare. The majority of cases of this tumour (about 70%) arise from the maxilla and its occurrence in the cranial vault represents approximately 15.6% of cases. We describe a rare case of melanotic neuroectodermal tumour of infancy, with simultaneous involvement of the cranial vault and petrous temporal bone, in a four-month-old child, complicated by post-surgical pseudo-meningocele. This case illustrates the diagnostic dilemma in differentiating reactive osseous sclerosis from direct tumour infiltration, both of which can occur in the context of melanotic neuroectodermal tumour of infancy. The discussion places emphasis on differential diagnoses and useful radiological features to assist in clinching the diagnosis of melanotic neuroectodermal tumour of infancy.

We present the case of a woman referred to our department at 34 weeks of pregnancy with a fetal ultrasonographic scan showing a mass that had developed within the right maxilla with invasion of the orbit. A retrospective examination showed that this tumor had been present since the 12th week of pregnancy. At 39+4 weeks of gestation, a boy was born. He presented a black firm aspect in the maxilla. A computed tomographic scan and magnetic resonance imaging revealed a soft tissue swelling over the right maxilla, extending into the orbit but without invasion of the globe. Surgical biopsy confirmed a melanotic neuroectodermal tumor of infancy. The pathologic examination did not show any neuroblast-like component on the hematoxylin eosin saffron staining. Because of the extension and the size of the lesion, neoadjuvant chemotherapy was carried out. At day 21, the patient received 1 cycle of low-dose cyclophosphamide and vincristine, 2 cycles of etoposide and carboplatin, and thereafter 1 cycle of cyclophosphamide, adriamycin, and vincristin because the lesion kept growing. After stabilization of the size of the tumor, at 4 months, a maxillectomy and partial resection of the orbital floor and lateral orbital wall was performed on the patient. As a complete resection would have required orbital exenteration, surgery was performed deliberately incomplete leaving a macroscopic residue (R2). At 2.5 years of follow-up, the patient showed complete remission with no lesions evident on magnetic resonance imaging.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare lesion that typically manifests in the first year of life, most commonly involving the facial bones. We present 2 infants with MNTI involving the posterior skull with associated compression of the superior sagittal sinus (SSS). A review of the anatomical locations of MNTI is offered, and the implications of SSS involvement are described. This represents the first known description of MNTI with involvement of the posterior SSS., (© 2016 S. Karger AG, Basel.)

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare pigmented craniofacial tumor of newborns and infants. We report the imaging findings of a 3-month old male patient with a maxillary MNTI. Detailed discussion on imaging features on various magnetic resonance sequences and CT scan are included. Characteristic radiographic appearance is also described. MNTI, of neural crest origin, display a biphasic population of melanin containing cells and neuroblastic cells, within a moderately vascularized fibrous stroma. The child underwent complete surgical excision with no evidence of recurrence at one year follow up. MNTI is an unusual tumor occurring in early childhood with a predilection for the maxilla. Clinical findings, CT scan and MRI may allow a preoperative diagnosis., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Melanotic neuroectodermal tumor of infancy (MNTI) is an extremely rare, pigmented neoplastic entity of neural crest origin. Histological and immunohistochemical profiles indicate the presence of two components, small rounded neuroblast-like cellular areas and areas with large melanin-containing cells which consist of combination of neural, melanocytic, and epithelial cell types. Here we present two interesting cases of infants with MNTI which have different clinicopathological features. The first case is a 3-month-old female with rapidly growing MNTI involving the lacrimal sac and inferior wall of the orbital cavity, treated with total maxillectomy without orbital exenteration followed by chemotherapy. The second case is a 7-month-old male with slow-growing maxillary MNTI treated with complete surgical excision. In the female patient, histological findings revealed a predominance of neuroblast-like cellular areas and a high Ki67 index indicating rapid cellular proliferation. In the male patient however, large melanin-containing cells were dominant in this slow-growing tumor. These findings support the presence of two different types of MNTI, rapid-growing and slow-growing types, determined by the component of neuroblast-like cellular areas., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare tumor, usually diagnosed within the first year of age, with a predilection for the maxilla. Although the tumor is usually benign, its rapidly growing nature and ability to cause major deformities in surrounding structures necessitate early diagnosis and intervention. It is important that medical and dental specialists are prepared to make the diagnosis and proceed with appropriate intervention. The authors performed a systematic review of the 472 reported cases from 1918 through 2013 and provided a comprehensive update on this rare entity that can have devastating effects on young patients. This investigation uncovered age at diagnosis as an important prognostic indicator, because younger age correlated with a higher recurrence rate. The authors also present a case report of a 5-month-old girl diagnosed with MNTI and review her clinical presentation and imaging and histopathologic findings., (Copyright © 2015 American Association of Oral and Maxillofacial Surgeons. All rights reserved.)

Melanotic neuroectodermal tumor of infancy is a rare melanin-containing neoplasm with locally aggressive and rapid expansile growth, usually involving the maxilla, skull, and mandible of early infancy. Radical surgery is critical for a long-term outcome. We present a case of 14-month-old girl with rapid-growing subcutaneous mass arising in the right temporal bone and extending intracranially on computed tomographic scan. Radical surgery was performed. A brownish-black tumor composed of large pigmented epithelioid cells, positive for cytokeratins and HMB-45, and nests of small neuroblast-like cells positive for neuron-specific enolase and synaptophysin, was diagnosed as melanotic neuroectodermal tumor of infancy. The patient remained well without evidence of recurrence for 1 year after surgery. Clinicopathological features, management alternatives and outcome were discussed., (© The Author(s) 2013.)

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare condition that occurs normally in the anterior maxilla of infants aged <1 year. The use of clinical and imaging tools for MNTI is extremely important to prevent problems with function as well as the aesthetic features in a breastfeeding child. Brazil is a multiethnic country with a poor epidemiological policy and little data to track the incidence of certain diseases, including MNTI. It is important to study this pathology with ethnicity as a factor to improve the current epidemiological programs and establish better post-treatment management. This paper describes a case of a 2-month-old male of African-indigenous descent and Brazilian Amazon residency, who presented to our unit in 2009 with a history of an expanding mass involving the anterior maxilla. Clinical and computerized tomography scans were used to diagnose the mass as MNTI, which was removed by total excision. A biopsy later confirmed the MNTI diagnosis.

Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon lesion with remarkably consistent histopathologic features that arises primarily in the pediatric population. We describe a MNTI arising in the anterior maxilla of a 6-month-old boy., (© 2011 Wiley Periodicals, Inc.)

Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon tumor of neural crest origin, almost invariably identified in the head and neck region. It is a relatively benign tumor but can recur and rarely metastasize. Herein, a rare case of MNTI in the thigh of an infant is documented. Radiographic imaging showed a sclerotic and lytic lesion in the metadiaphyseal region of the right femur with a soft tissue component. The clinicoradiological impression was of a malignant round cell tumor and osteomyelitis. The patient's urinary vanil mandelic acid (VMA) level was elevated. FNAC smears revealed a tumor with round to polygonal cells, focally containing black melanin pigment. Biopsy and tumor excision revealed a tumor with cells in cords and nests embedded in a fibrocollagenous stroma and showed bone and soft tissue infiltration. On immunohistochemistry, tumor cells displayed immunoreactivity to vimentin, cytokeratin (CK), CK7, CK19, and MIC2, and focal reactivity for synaptophysin and HMB45. Diagnosis of an MNTI was made. The patient underwent induction chemotherapy with extracorporeal radiotherapy, followed by a soft tissue excision that revealed residual tumor. Surgically, free marrow margins were accomplished, and the patient is currently being followed-up. To the best of our knowledge this case is the fifth documented case in the English literature of MNTI in the thigh. The diagnostic and therapeutic implications are discussed.

Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon melanin-containing mesenchymal tumor of neural crest origin. What make this tumor unique and interesting is its characteristic predilection for anterior maxilla (premaxilla) and the presence of pigment melanin which gives the tumor distinct clinicopathological, immunohistochemical, ultrastructural and imaging features. Although first described almost a century ago, to the authors' knowledge, only a few hundred cases of MNTI have been reported worldwide in the English medical literature. The pool of documented radiological findings is even more sparse as not more than a dozen cases could be abstracted from an Internet search of the radiology literature. We document a case of MNTI and describe the imaging findings with intent to contribute to its small but accruing radiological data.

Introduction: Melanotic progonoma or melanotic neuroectodermal tumor is a rare tumor in infancy. This lesion has to be considered in the differential diagnosis of benign or malignant lesions of calvarium., Case Report: The authors present a case of a 4-month-old infant with left retroauricular mass. The patient had a subcutaneous mass that is fixed to the underlying skull. CT and MRI scans showed left occipitotemporal expansile mass. The tumor was removed by surgery. A tumor, brownish-black in color, was diagnosed as melanotic progonoma. The patient remained symptom-free for the last 2 years after complete surgery., Discussion: Extracranial subcutaneous masses involving the skull are uncommon in infants. Benign or malignant lesions may occur as lumps on calvarium. Physical examination and some laboratory findings are helpful in the assessment of patient. Benign or malignant lesions can be differentiated by craniography, CT, or MRI scans, but exact diagnosis of melanotic progonoma is made by histopathology and immunostaining, as was in the presented case. Cranial vault progonomas have a better outcome by complete surgery. The tumors usually do not recur in long-term period.

An 8-month-old boy presented with a 6-week history of a skull mass of the anterior fontanelle. The mass was excised, and the histopathologic features were diagnostic for melanotic neuroectodermal tumor of infancy. The tumor showed focal myogenin positivity, which has not been previously reported in this tumor. The patient has no evidence of recurrent tumor 10 months after the excision. No adjuvant therapy was given. In addition, we stained a case of melanotic neuroectodermal tumor of infancy obtained from Columbus Children's Hospital; focal myogenin positivity was present.

We present a case of a 4-month-old female infant with a maxillary melanotic neuroectodermal tumor of infancy (MNTI) and review the pooled data from previous publications on this entity. The literature to date comprises 378 reported cases from 1918 to the present, from which data on the presence or absence of metastatic disease was available in 311, and on the presence or absence of local recurrence in 165. These pooled data suggest a local recurrence rate of 36% with metastasis occurring in 7% of cases. At present, the optimal management includes complete surgical excision with clear margins, but there are no reliable histopathological or molecular features to predict the biological behavior in individual cases.

Background: Reconstruction of mandibular defects following tumor resection in infants is a particular challenge. Although autologous rib grafts have no relevance nowadays for restoration of mandibular bone defects following ablative tumor surgery due to limited bone stock and the availability of other donor areas, they are a useful surgical alternative following tumor surgery in infants., Patients and Method: We here report on the 5- and 10-year follow-up of two children who were diagnosed with benign tumors of the mandible with osseous destruction at the age of 4 and 6 months, respectively. Histological diagnoses were melanotic neuroectodermal tumor and hemangioendothelioma of the mandible. Following continuity resection of the mandible lateral mandibular bone defects were restored using autologous rib grafts. Yearly clinical and radiological follow-up visits in both children were performed to assess growth of the facial skeleton as well as mandibular growth., Result: Cephalometric measurements on panorex films as well as 3D CT scans revealed slight vertical growth excess and transversal growth inhibition of the reconstructed mandible in comparison with the non-operated side., Discussion: Although further growth of rib grafts is difficult to predict and occlusal disharmony may occur due to physiologic maxillary growth and growth of the unaffected mandible, we believe that autologous rib grafts can be ideally used for restoration of mandibular continuity defects in newborns. Clinical follow-up visits on a yearly basis and orthodontic controls are useful for early orthodontic treatment of growth deficits. Further corrective surgery with bone augmentation or osseous distraction is required following completion of growth of the facial skeleton.

Background: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that develops during the first year of life and grows rapidly. Early diagnosis and radical surgery are critical for a long-term cure. We report a rare case of MNTI in the skull and discuss the importance of the radical surgery and the long-term follow-up results., Case Presentation: We describe a case of a 4-month-old girl with an MNTI in the skull who underwent the operation 11 years ago. The mass in the frontotemporosphenoid region grew rapidly after birth. The patient underwent a craniotomy. By referring to the histological findings of frozen section during surgery, a total excision of the tumor including its adjacent hypertrophic bone was performed. The patient has remained well without evidence of recurrence or neurological abnormality for 11 years., Conclusion: Radical surgery for MNTI provides complete cure. According to the literature including our case, there should be follow-up for at least 2 years after surgery. Especially in cases in which tumors recur, follow-up should be for longer periods because of the possibility of its malignant change.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that generally arises in the maxilla during the first year of life. Involvement of bones of the cranial vault or brain is extremely rare. We describe a 7-month-old black female who presented after falling out of bed onto a concrete floor. Subsequently, she developed an anterior frontal mass that enlarged over several days. Radiographs of the skull at her local hospital showed a depressed right frontal skull fracture. However, computerized tomography of the head (reviewed at our institution) revealed a slightly hyperdense extra-axial mass which crossed the anterior frontal midline, widening the metopic suture and extending into the anterior subgaleal scalp. Hyperostosis of the adjacent frontal calvarium was also present. A craniotomy revealed a dark, 1.5-cm calcified epidural lesion with some features of an unusual hematoma. Microscopic evaluation revealed a chronic hematoma and MNTI. The tumor recurred within a year. MNTI should be included in the differential diagnosis of epidural and skull lesions in infants., (Copyright 2002 S. Karger AG, Basel)

We describe CT and MR findings in a 23-month-old infant with a melanotic neuroectodermal tumour of the pineal gland. The tumour has been stereotactically biopsied and surgically resected. The pathological diagnosis was made on the resected piece. Embryology of the pineal gland and the histology of melanotic neuroectodermal tumour of infancy are discussed.

Melanotic neuroectodermal tumor of infancy is a rare pigmented neoplasm occurring in infants before 1 year of age. It is a rapidly growing tumor that most frequently affects the craniofacial skeleton. Although melanotic neuroectodermal tumor of infancy is benign in the vast majority of cases, inadequate excision, occasional multicentricity, and a small malignant potential result in a fairly high recurrence rate. On the basis of data obtained from the literature and our clinical experience, we advocate an aggressive surgical approach consisting of complete surgical excision when vital structures are not involved. Histopathologic confirmation of complete excision is mandatory to minimize the risk of recurrence and provide the patient with curative treatment and minimal morbidity.

Melanotic neuroectodermal tumour of infancy is an uncommon neoplasm that usually occurs in children aged one year or less. Difficulty in deciding the cellular origin of this tumour has led to numerous names, including congenital melanocarcinoma, melanotic epithelial odontoma, melanotic ameloblastoma, and retinal anlage tumour. Electron microscopy and histochemical studies, however, have now established the neural crest origin. The most frequent site of occurrence is the maxilla followed by the skull, the brain and the mandible. The genital organs are the most frequent extracranial site. We present two cases of melanotic neuroectodermal tumour of infancy arising in the maxilla.

The melanotic neuro-ectodermal tumour of infancy is an uncommon benign tumour of neural crest origin occurring in infants with a predilection for the anterior maxilla. This lesion shows a good response to conservative surgical excision with few recurrences but it should be followed up for long periods of time because of the rare malignant change possible in the tumour.