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PubMed2001

Melanotic neuroectodermal tumor of infancy involving the mandible: 7-year follow-up after hemimandibulectomy and costochondral graft reconstruction.

Oral and maxillofacial tumors occur rarely in the pediatric population compared with the adult population. We report a case of a 6-months old female infant suffering from a melanotic neuroectodermal tumor of infancy involving the mandible. Tumor resection was performed using a submandibular approach; the mandibular defect was reconstructed primarily with autogenous costochondral grafts. During a 7-year follow-up period, there has been no tumor recurrence. The costochondral graft healed well; tracing of panoramic radiographs at 2, 3, and 6 years documented some vertical overgrowth and growth retardation in the transversal dimension. The authors conclude that the use of costochondral grafts despite its controversial role for mandibular reconstruction can be recommended in particular after continuity resections in newborn infants. However, long-term follow-up is necessary as well as secondary corrective surgery at early skeletal maturity.

Bone Transplantation↗
PubMedDate not supplied

Melanotic neuroectodermal tumors of the brain and skull.

A case of melanotic neuroectodermal tumor in the cerebellum of a 21-year-old man is presented. Melanin was found mainly in small neoplastic cells rather than in large epithelioid cells. The tumor also contained neurons, as well as neoplastic tissue of ependyma, choroid plexus; and astrocytes. We propose that this neuroectodermal tumor resulted from a combination of cells originating in the neural crest and in the neural tube. The small cells have been described as medulloblasts, but they may be poorly differentiated ependymal cells, or, sympathicoblasts. The presence of pigment in these cells is compatible with an origin in the neural crest. The so-called melanotic progonoma, occurring most often in the maxilla of infants, is reviewed in relation to the melanotic neuroectodermal tumor of the brain. The tumor in facial bone occurs in adults as well as infants, in locations other than the maxilla, and has malignant forms. Melanotic neuroectodermal tumors, whether in brain or bone, are therefore similar in behavior as well as histologic appearance. The finding of similar tumors in these different locations is readily explained embryologically.

Adult↗
PubMedDate not supplied

[Melanotic neuroectodermal tumor in a newborn].

The tumor consists of two types of cells: small with abundant processes cells capable of dividing and probably being neuroblasts, and the big melanocytes forming bands and glandular-like structures. Tumor cells (mainly melanocytes) express pancytokeratins, S-100 protein, neuron-specific enolase, synaptophysin and melanin antigen. These data are the additional indication that melanotic neuroectodermal tumor is the derivative of the neural crest.

Antigens, Neoplasm↗
PubMed1990

Melanotic neuroectodermal tumor of infancy: a case report.

The melanotic neuroectodermal tumor of infancy is a rare benign neoplasm that appears as a rapidly enlarging mass, most commonly in the anterior part of the palate. The tumor is believed to be of neurocrestal origin. The case report of a 3-month-old infant with a rapidly growing melanotic neuroectodermal tumor of infancy is presented. The tumor was surgically excised, and no evidence of recurrence has been found.

Biopsy↗
PubMedDate not supplied

Melanotic neuroectodermal tumor of infancy: clinical, radiologic, and pathologic findings in five cases.

Five pathologically proved melanotic neuroectodermal tumors of infancy are reported. These rare neoplasms of infancy exhibit a distinct predilection for the maxillary bone. Three tumors originated in the maxilla, one in the calvaria, and one in the cerebellar vermis. Those occurring in bone did not metastasize but were locally invasive, as reflected in their radiologic appearance. Bone erosion, expansion, hyperostosis, and osteogenesis can occur in the same neoplasm and were appreciated best on CT. MR imaging showed the soft-tissue component and extent of the neoplasm better than CT did. The pathologic findings from all five cases (and one possibly related melanotic tumor of the face) revealed abundant melanin. MR imaging of two melanotic tumors showed isointense T1-weighted and slightly hyperintense T2-weighted signals. This appearance is contrary to that of most melanin-containing tumors, which exhibit enhanced T1 and T2 relaxation, and indicates that variables other than the absolute amount of melanin may determine the MR signal. Clinically, rapid neoplastic growth and excessive melanin production by the tumor cells caused facial disfigurement and visible blue black discoloration. All five melanotic neuroectodermal tumors were resected and the vermian tumor was also irradiated. Four of five children were well and free from disease 1 month to 7 years after resection. The calvarial tumor was incompletely resected and involved the underlying brain, eventually causing death. The clinical, radiologic, and pathologic features of melanotic neuroectodermal tumors of infancy are reviewed. Melanotic neuroectodermal tumors of infancy that involve bone can be diagnosed from the clinical and radiologic findings. Prompt diagnosis and surgical resection are essential for cure.

Cerebellar Neoplasms↗
PubMed2002

Melanotic neuroectodermal tumor of infancy discovered after head trauma.

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.

Brain Neoplasms↗
PubMed2000

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

A case of melanotic neuroectodermal tumor of infancy occurring in the mandible is described. The patient was a 1-month-old boy with a rapidly growing tumor of the mandible. Computed tomography showed 2 well-defined osteolytic lesions in the right mandible. Histopathologic diagnosis of a biopsy specimen was melanotic neuroectodermal tumor of infancy. The tumor was excised with removal of the surrounding bone, but 1(1/2) months later it recurred, and segmental mandibulectomy and reconstruction of the defect with a titanium miniplate was performed. Retrospectively, evidence of recurrence was noted on computed tomography taken on the tenth postoperative day. The recurrence was caused by incomplete removal of the tumor. Histopathologically, the tumor cells of the recurrent lesion were dispersed extensively in the bone marrow, and bone remodeling was active. The surgical procedure may have stimulated tumor cell proliferation and reactive bone formation. The patient was followed for 2 years with no evidence of recurrence or metastasis.

Humans↗
PubMed1999

Role of radical surgery for intracranial melanotic neuroectodermal tumor of infancy: case report.

OBJECTIVE AND IMPORTANCE: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, locally aggressive tumor that arises most commonly from the maxilla or mandible. Infrequently, it originates from the cranial vault, and recent reports have described a favorable outcome after radical surgery. Some lesions are particularly problematic, such as those located along the cranial midline or cranial base and those with significant intracranial extension. Currently, there is no effective adjuvant therapy for MNTI; radiation is precluded by the patients' young age, and chemotherapy trials have not demonstrated long-term efficacy. CLINICAL PRESENTATION: A 2-month-old infant boy presented with a firm, immobile subcutaneous mass behind the right ear. The mass had been present at birth and enlarged with time. INTERVENTION: Initial resective surgery down to the dura resulted in massive tumor recurrence within weeks. Successful management required repeat surgery including excision of the dura and dural venous sinuses. CONCLUSION: This patient's large MNTI of the cranial base was successfully managed by radical surgery. Although MNTI is a rapidly growing tumor that is locally highly invasive, radical surgery may be associated with a favorable outcome and offers the potential for long-term cure.

Humans↗
PubMed1996

Melanotic neuroectodermal tumor of infancy: report of a case.

The melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon benign neoplasm frequently detected in young infants, which has a predilection for the head and neck region, particularly maxilla. The purpose of this article is to present a documented case of MNTI and discuss clinical, radiological and histopathological features of the disease. The biological behavior of the tumor is emphasized.

Alveolar Process↗
PubMed1992

Heterologous tissue elements in melanotic neuroectodermal tumor of infancy.

Two cases of melanotic neuroectodermal tumor of infancy (MNTI) contained highly cellular stromal areas consisting of spindle cells exhibiting mitotic activity. In one case, single spindle cells exhibited the same immunohistochemical profile as tumor cells forming part of the epithelial component. In the other case woven bone was formed in a dense fibroblastic stroma. These tumor parts were judged to be heterologous tissue elements that also may be observed in other kinds of neuroectodermal tumors and that reflect the potential of the neural crest to differentiate into various mesenchymal tissue types.

Cell Nucleolus↗
PubMedDate not supplied

Melanotic neuroectodermal tumor of the neurocranium in infancy.

Melanotic neuroectodermal tumors of the neurocranium are a rare but life-threatening disorder of infancy. 11 previously reported cases are reviewed in terms of clinical presentation, radiological diagnosis, and management. A twelfth case, a 4-month-old infant who developed three discrete sites of tumor unilaterally in the neurocranium is presented. Several hypotheses for the mechanism of formation of these tumors are reviewed. The authors propose that the mechanism of formation involves a dysontogenesis of neural crest tissue and that these tumors form, at least in part, from fragments of melanin-containing arachnoid villi which are displaced during embryonic development.

Craniotomy↗
PubMed1987

Melanotic neuroectodermal tumor of infancy.

A case of melanotic neuroectodermal tumor of infancy has been presented, and the etiology, clinical presentation, and management of this rare tumor have been discussed. The tumor may not be familiar to most otolaryngologists and should be included in the differential diagnosis of pediatric head and neck neoplasms.

Female↗
PubMed1986

Melanotic neuroectodermal tumor of infancy.

A case of melanotic neuroectodermal tumor of infancy was presented. This tumor occurred in the right maxillary alveolar ridge of 3-month-old female infant, showing bluish enlargement of alveolar mucosa with the displacement of central deciduous incisor. We described the gross, microscopic, and ultrastructural findings of this tumor. This case appears to be the first case of MNTI, reported in a Korean.

Female↗
PubMed1975

Melanotic neuroectodermal tumor of infancy.

Recurrence of a melanotic neuroectodermal tumor of infancy was clinically evident eight weeks after the original lesion had been surgically removed. Concentrations of urinary vanilmandelic acid were elevated immediately after surgery but returned to normal a month after removal of the tumor. The location of the recurrent tumor, the rapidity of recurrence, and the concentrations of urinary VMA favor a theory of neural crest origin.

Diagnosis, Differential↗
PubMed1995

Melanotic neuroectodermal tumor of infancy: a molecular approach to diagnosis. Case report.

A case of melanotic neuroectodermal tumor of infancy arising from the transverse sinus is presented. The tumor was located on the outer surface of the dura and extended extracranially through the occipitomastoid suture. Two cell populations were observed: pigmented melanocyte-like cells and small neuroblast-like cells. Ultrastructural analysis revealed epithelial tumor cells and melanosomes at various stages. Expression of melanotransferrin messenger RNA transcripts within the tumor tissue was observed using a reverse transcriptase-polymerase chain reaction method in addition to immunohistological studies. The positive expression of melanotransferrin confirmed that this melanotic neuroectodermal tumor was derived from neural crest cells.

Base Sequence↗
PubMed1985

Melanotic neuroectodermal tumor of infancy (retinal anlage tumor).

Six cases of the melanotic neuroectodermal tumor of infancy, including light and electron microscopic findings, are presented. The tumor, now believed to originate from neural crest, is usually cured by wide excision; however, malignant cases have been reported. Histopathologically, this lesion resembles neural retina and retinal pigment epithelium. It may involve the orbit but most frequently occurs in the maxilla. One case presented involved the orbit, another the anterior fontanelle, and four presented in the maxilla.

Facial Neoplasms↗
PubMedDate not supplied

Melanotic neuroectodermal tumor of infancy.

We present a case of malignant melanotic neuroectodermal tumor of infancy arising in the skull and secondarily invading brain. The central tumor was hyperintense to brain on T1-weighted images and hypointense to brain on T2-weighted images. This appearance corresponded to the surgical and histologic findings of melanin-containing tumor.

Brain Neoplasms↗