Melanotic Neuroectodermal Tumor of infancy Symptoms, Causes, Treatment

Melanotic Neuroectodermal Tumor of infancy Symptoms, Causes, Treatment

Tumors that appear during the early or infant stage of life are known as childhood tumors or tumors of infancy. Melanotic neuroectodermal tumor of infancy refers to a rare oral cavity tumor. It is observed in patients usually at the time of birth. It is a rare, neuroblastic, and biphasic tumor. It is a pigmented epithelial neoplasm of the neural crest origin or craniofacial sites involving gums or oral cavities. This tumor arises in the maxilla of infants and neonates and the simple cure is none other than the removal. It occurs during the first year of life. Melanotic neuroectodermal tumor of infancy (MNTI) is a rapidly growing tumor. Despite its local destruction and rapid growth, it is considered a benign tumor rather than malignant ones. The treatment of choice is usually considered; as surgical excision. The recurrence rate may vary between 10 to 15%. However, the report of malignant behavior is 6.5%.

The recurrence rate varies between 10% and 15%, and malignant behavior is reported in 6.5% of the cases. 

Melanotic Neuroectodermal Tumor Symptoms

The case of a 5-month-old male child with a Melanotic Neuroectodermal Tumor of infancy showed the development of a mass in the anterior portion of the maxilla growing for two months. The first symptom of this infancy tumor was swelling associated with an erupting tooth. For the erupted tooth, the child’s parents reported a private practitioner who earlier extracted the tooth of the child. The increased swelling obstructed the oral cavity causing complications in feeding. A provisional diagnosis of MNTI of the patient was done at the Department of Oral and Maxillofacial Surgery.

Melanotic Neuroectodermal Tumor of infancy Symptoms, Causes, Treatment 

The physical examination of the tumor showed clear and prominent facial deformity in the affected infants. A dome-shaped sessile mass in the maxillary alveolar region and hard palate region. 

Most patients develop an unusual mass with a bluish appearance in the mouth. This bluish appearance is due to the pigmentation in the cells that appear blue over the overlying mucosa or epithelium. The imaging studies show a radiolucent mass without any well-defined borders. Calcifications are visible within the unusual mass. More than 70% of the patients involve the maxilla; the skull and mandible are affected less often. There may be an elevated Vanillylmandelic acid level. 

Melanotic Neuroectodermal Tumor Causes

The causes of infancy tumors are usually inherited DNA changes or mutations from the parents that increase their risk of certain types of cancer. These changes are present and associated with every cell of the body. Some changes are linked only with an increased risk of the tumor; the other changes can cause syndromes with developmental or health problems. 

However, most childhood cancers or tumors are not due to inherited DNA changes. 

These tumors may sometimes arise in the child’s early phase and are called an acquired mutation. Acquired mutations cannot be passed to the children of the affected person. Sometimes the causes of gene changes in certain adult cancers are known. However, in most childhood tumors, the causes of DNA changes are not known. A few individuals may get affected due to outside factors like radiation exposure. There are several other unknown causes associated with infancy tumors. However, many are most likely due to the random events inside the cell.

Melanotic Neuroectodermal Tumor Treatment

A benign course followed after the complete local excision with clear surgical margins. Neoadjuvant therapy is considered for tumors that are difficult to treat. This kind of therapy is also associated with CNS and other vital structures. Neoadjuvant therapy also assists in treatment when clear surgical margins are not achievable. 

According to one recent unpublished case study at the University of Michigan, the chemotherapeutic treatment was used successfully after three failed attempts in the complete resection of the tumor with dural involvement. The patient with chemotherapy was followed with imaging and physical exams and had no proof of recurrence of the tumor after 30 months of follow-up. Very little is known about the effects of chemotherapy on the growth and morphology of tumors. In rare cases, chemotherapy is used for residual or recurrent tumors. The results are fair enough. However, it is unpredictable with approximately 30% of patients having a recurrence. In around 10% of the cases, metastasis may be seen involving the liver, bone, lymph nodes, and soft tissue. It seems to cause maturation of the primitive kind of tumor cells and reduction of the neuroblast-like component and predominance of the epithelial melanogenic component. Due to the rarity of this condition, it is not easy to conduct prospective studies on a large scale. 

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