Congenital salivary gland anlage tumor - in utero and postnatal imaging.

We present a case of an infant with congenital salivary gland anlage tumor, with fetal and postnatal imaging. To the best of our knowledge, this is the first case describing the in utero imaging findings of salivary gland anlage tumor. A fetal MRI was performed secondary to the clinical finding of polyhydramnios, which identified a nasopharyngeal mass. Because findings were concerning for airway obstruction, the fetus was delivered by ex utero intrapartum treatment (EXIT) to airway procedure. A postnatal CT confirmed the findings of the fetal MRI. The lesion was resected when the baby was 4 days old and recovery was uneventful.

Urgent maxillectomy in infants for rare infantile maxillary tumors.

OBJECTIVE: The purpose of this study was to present our experience with definitive surgical management of infants with Melanotic neuroectodermal tumor of infant (MNTI) and epithelioid hemangioendothelioma (EHE). PATIENTS AND METHODS: This study included four male infants with histologically proven MNTI (three infants) and EHE (one infant). CT scan and MRI were for taken for the patients preoperatively. Surgical intervention was used in all infants. RESULTS: This study is a retrospective study that reflects our experience in the last 20 years in maxillectomy in infants. All infants were male with mean age 6 months (2-9 months). Complete resection of the tumor was achieved all infants with no residual or recurrence. Neither chemotherapy nor radiation was used in this study. CONCLUSIONS: MNTI and EHE are rare tumor of infant. They present as a slow painless hard swelling of the maxilla. Imaging is an essential before surgical treatment. Complete excision is curative.

Melanotic neuroectodermal tumour of infancy: a case report.

A case of melanotic neuroectodermal tumor of infancy occurring in the maxilla in a 13 day old neonate is described. Computed tomography and histopathology confirmed the diagnosis and a submucosal excision was carried out when the infant was 30 days old. But three weeks later the patient reported back with a recurrence and a wide surgical excision was performed. The recurrence may have been caused by incomplete removal of the tumor cells and the initial surgical procedure may have stimulated tumour cell proliferation. Fortunately, 6 month follow up of the patient showed no recurrence.

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.

Melanotic neuroectodermal tumor of infancy.

A 6-month-old female presented with a swelling of the left superior maxillary bone. A radiograph revealed a radiolucent lesion in the left anterior maxilla, and CT, showed a solid mass. At surgery, the lesion was a cystic dental tumor of ectopic location which was treated by excision. The pathological diagnosis was melanotic neurorectodermal tumor. This is an uncommon benign tumor of the neural crest origin that occurs mainly in the maxilla (70% of cases) but can occur in other areas such as the skull and the mandible. CT reveals a hyperdense mass and MR shows a hypointense mass on T1-weighted images and an iso-intense mass on T2-weighted images. The evolution is usually benign after surgical removal.

Congenital melanotic neuroectodermal tumour of infancy: report of a case.

A case of a congenital melanotic neuroectodermal tumour in a male neonate is described. Management consisted of wide excision of the tumour as opposed to simple enucleation.