Publication date: Available online 12 June 2016
Source:Journal of Oral and Maxillofacial Surgery
Author(s): Amir Azari, Daniel Petrisor, John Wright, G.E. Ghali
Melanotic neuroectodermal tumors of infancy (MNTI) are pigmented, rapidly growing, and occur predominantly within the bony head and neck structures. There are fewer than 400 cases reported in the literature1 with many of these described as single case reports due to their rarity. MNTIs are derived from the neural crest, and appear in areas that develop from neuroectodermal pathways; the most common of these is the maxilla.2 Locations in other intraosseous and extraosseous structures have been characterized, including the mandible (6%), skull, brain, and epididymis.1 Infants in the first year of life are usually affected, compelling prompt diagnosis and treatment as well as close monitoring. Prognosis is generally good despite the rapidity of growth. The incidence of recurrence is about 20% overall and perhaps higher (33%) in mandibular cases.1 Given the rarity of this disease, an overestimation of recurrence or metastases may be more frequently reported.3The tumors are categorized as benign but aggressive, with rare reports of malignant features based on histology4 or metastasis. Surgical resection in the pursuit of tumor-free margins is the primary treatment modality for all tumors given its potential for extensive growth; this is especially true of malignant tumors, which comprise less than 25 cases in the literature.1,5 Chemotherapy and radiotherapy have been used as adjuncts in such cases, with varying reports of success in reducing morbidity and mortality.6-8 The management of a metatstatic mandibular tumor, in particular, has only been documented in one other case.9 The purpose of this case report is to present treatment and reconstruction considerations in a metastatic mandibular MNTI and to review the associated literature.
Source:Journal of Oral and Maxillofacial Surgery
Author(s): Amir Azari, Daniel Petrisor, John Wright, G.E. Ghali
Melanotic neuroectodermal tumors of infancy (MNTI) are pigmented, rapidly growing, and occur predominantly within the bony head and neck structures. There are fewer than 400 cases reported in the literature1 with many of these described as single case reports due to their rarity. MNTIs are derived from the neural crest, and appear in areas that develop from neuroectodermal pathways; the most common of these is the maxilla.2 Locations in other intraosseous and extraosseous structures have been characterized, including the mandible (6%), skull, brain, and epididymis.1 Infants in the first year of life are usually affected, compelling prompt diagnosis and treatment as well as close monitoring. Prognosis is generally good despite the rapidity of growth. The incidence of recurrence is about 20% overall and perhaps higher (33%) in mandibular cases.1 Given the rarity of this disease, an overestimation of recurrence or metastases may be more frequently reported.3The tumors are categorized as benign but aggressive, with rare reports of malignant features based on histology4 or metastasis. Surgical resection in the pursuit of tumor-free margins is the primary treatment modality for all tumors given its potential for extensive growth; this is especially true of malignant tumors, which comprise less than 25 cases in the literature.1,5 Chemotherapy and radiotherapy have been used as adjuncts in such cases, with varying reports of success in reducing morbidity and mortality.6-8 The management of a metatstatic mandibular tumor, in particular, has only been documented in one other case.9 The purpose of this case report is to present treatment and reconstruction considerations in a metastatic mandibular MNTI and to review the associated literature.
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