Melanotic neuroectodermal tumor of infancy successfully treated with metformin

Abstract

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.

Pigmented Neuroectodermal Tumor in Maxilla of an Infant : Report of a Rare Neoplasm in Bandung, Indonesia

“…12 The origin of this tumor was a controversy in the early time, hence the many names suggested, such as pigmented epulis, congenital melanocarcinoma, pigmented adamantinoma, retinal anlage tumor, and pigmented tumor of the jaw of infants. 3,5,12 In 1966, Borello and Gorlin reported a melanotic tumor in an infant and found an elevated level of urinary vanillylmandelic acid (VMA), the VMA level was returned to normal after the removal of the tumor. High level of VMA is useful for diagnosing a tumor of neural crest origin.…”

“…1,4 Borello and Gorlin (1966) found that the tumor was associated with an increased levels of vanillylmandelic acid (VMA) and suggested that the tumor is of neural origin. 4,5 There was approximately 500 cases reported worldwide between 1918 until now. 3,5 In Indonesia, there were only one case that has been reported in literature.…”

“…4,5 There was approximately 500 cases reported worldwide between 1918 until now. 3,5 In Indonesia, there were only one case that has been reported in literature. 6 Head and neck region have a great predilection of this tumor, about 67% of all reported cases, maxilla is the most common site affected.…”

“…Recurrent MNTI is reported to have an excellent result with radiotherapy and chemotherapy. 4,5,11 We hereby present a case of MNTI treated with surgical resection without the use of any graft.…”

Inflammatory myofibroblastic tumor successfully treated with metformin: A case report and review of literature

“…Several preclinical, epidemiological, and clinical studies have demonstrated that metformin, a first-line antidiabetic drug, can reduce the overall risk and mortality of neoplasms[ 12 - 23 ]. Metformin is capable of inhibiting protein and lipid synthesis of tumor cells via the activation of adenosine phosphate protein kinase (AMPK) activity, which in turn downregulates the mechanical target of rapamycin complex 1 (mTORC1) signaling pathway, thereby limiting tumor growth and proliferation[ 24 ].…”