Melanotic neuroectodermal tumor of the brain recurring 12 years after complete remission: case report

Abstract

We describe a rare case of melanotic neuroectodermal tumor (MNT) of the brain recurring 12 years after complete remission. An 11-year-old girl initially presented with exotropia and bilateral papilledema. Magnetic resonance (MR) imaging revealed an intracranial extraaxial large tumor at the midfrontal region. T(1)-weighted MR imaging showed the tumor to be well delineated with homogeneous enhancement by gadolinium. The tumor was subtotally removed, and the histological diagnosis was MNT. The residual tumor became enlarged, so gamma knife radiosurgery was performed 5 months after initial surgery. The enhanced lesion disappeared, but another lesion emerged 3 years later. A second gamma knife radiosurgery was performed for this local recurrence. The enhanced lesion disappeared once again. Twelve years after the second gamma knife radiosurgery, another local recurrence was detected. This tumor was subtotally removed. Histological examination confirmed the same diagnosis of MNT. This case suggests that MNTs not completely resected need long-term follow up, even if complete remission was achieved after adjuvant therapy.

Calvarial malignant melanotic neuroectodermal tumour of infancy presenting with widespread intracranial metastasis

“…One had exclusive subarachnoid metastasis and the other had both, subarachnoid and intra-parenchymal metastasis (Lacy and Kuhar, 2010;Omodaka et al, 2010). Our patient had neither lymph node or liver metastasis, nor did she have unusually high serum levels of catecholamines, routinely assayed in patients with malignant MNTI (Butt et al, 2009;Omodaka et al, 2010;Scheller et al, 2010). The histological appearance of the lesion did not necessitate the performance of immunological tests such as S-100, HMB 45 and Ki-67 as an index for proliferation (Butt et al, 2009;Scheller et al, 2010).…”

“…Incompletely resected tumours need long-term follow up, even if complete remission was achieved after adjuvant therapy. This is evidenced by the recurrence reported 12 years after subtotal tumour excision and 2 sessions of gammaknife radiosurgery in a 11 year-old girl (Butt et al, 2009;Omodaka et al, 2010,). The recurrence and malignancy rates have been reported to be 10%e15% and 1.9%e6.6% respectively (Neven et al, 2008).…”

“…There are 11 previous reports of malignant MNTI, of which only 2 had intracranial metastatic lesions. One had exclusive subarachnoid metastasis and the other had both, subarachnoid and intra-parenchymal metastasis (Lacy and Kuhar, 2010;Omodaka et al, 2010). Our patient had neither lymph node or liver metastasis, nor did she have unusually high serum levels of catecholamines, routinely assayed in patients with malignant MNTI (Butt et al, 2009;Omodaka et al, 2010;Scheller et al, 2010).…”

“…Small round cell neoplasms in children like neuroblastoma, Ewing's sarcoma, malignant melanoma and lymphoma are important differential diagnoses (George et al, 1995;Jeon et al, 2008;Neven et al, 2008). About 40% of tumour recurrences are documented within 4 weeks after surgery (Omodaka et al, 2010) and may be as high as 50% in cases without wide resection (Butt et al, 2009).…”

Giant melanotic neuroectodermal tumor of infancy (melanotic progonoma) of the head and neck: report of a malignant case

“…Melanotic neuroectodermal tumor of infancy is a rare, mainly benign, pigmented lesion of neural crest origin, usually affecting infants in the 1st year of life. 1,2,7,15,18 Only 9% of cases are diagnosed after the age of 12 months. The tumor usually arises in the anterior maxillary alveolar ridge.…”