title: Clinicopathological features of melanotic neuroectodermal tumor of infancy: Report of two cases.
abstract: Melanotic neuroectodermal tumor of infancy (MNTI) is an extremely rare, pigmented neoplastic entity of neural crest origin. Histological and immunohistochemical profiles indicate the presence of two components, small rounded neuroblast-like cellular areas and areas with large melanin-containing cells which consist of combination of neural, melanocytic, and epithelial cell types. Here we present two interesting cases of infants with MNTI which have different clinicopathological features. The first case is a 3-month-old female with rapidly growing MNTI involving the lacrimal sac and inferior wall of the orbital cavity, treated with total maxillectomy without orbital exenteration followed by chemotherapy. The second case is a 7-month-old male with slow-growing maxillary MNTI treated with complete surgical excision. In the female patient, histological findings revealed a predominance of neuroblast-like cellular areas and a high Ki67 index indicating rapid cellular proliferation. In the male patient however, large melanin-containing cells were dominant in this slow-growing tumor. These findings support the presence of two different types of MNTI, rapid-growing and slow-growing types, determined by the component of neuroblast-like cellular areas.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8575, Japan.
authors.forename: Kenjiro
authors.initials: K
authors.lastname: Higashi
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8575, Japan. Electronic address: ogawa@orl.med.tohoku.ac.jp.
authors.forename: Takenori
authors.initials: T
authors.lastname: Ogawa
authors.affiliation: Department of Pediatrics, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8575, Japan.
authors.forename: Masaei
authors.initials: M
authors.lastname: Onuma
authors.affiliation: Department of Pathology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8574, Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Usubuchi
authors.affiliation: Department of Plastic and Reconstructive Surgery, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8575, Japan.
authors.forename: Yoshimichi
authors.initials: Y
authors.lastname: Imai
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8575, Japan.
authors.forename: Iori
authors.initials: I
authors.lastname: Takata
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8575, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hidaka
authors.affiliation: Department of Pathology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8574, Japan.
authors.forename: Mika
authors.initials: M
authors.lastname: Watanabe
authors.affiliation: Department of Pediatrics, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8575, Japan.
authors.forename: Yoji
authors.initials: Y
authors.lastname: Sasahara
authors.affiliation: Department of Maxillofacial Prosthetics Clinic, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8574, Japan.
authors.forename: Shigeto
authors.initials: S
authors.lastname: Koyama
authors.affiliation: Department of Pediatrics, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8575, Japan.
authors.forename: Shigeo
authors.initials: S
authors.lastname: Kure
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8575, Japan.
authors.forename: Yukio
authors.initials: Y
authors.lastname: Katori
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D005134:Eye Neoplasms / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D019394:Ki-67 Antigen / Q000378:metabolism; D007766:Lacrimal Apparatus Diseases / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D008437:Maxilla / Q000601:surgery; D008441:Maxillary Neoplasms / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D009301:Nasolacrimal Duct / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D017600:Neuroectodermal Tumor, Melanotic / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D017233:Orbit Evisceration; D014057:Tomography, X-Ray Computed
chemical_list: D000970:Antineoplastic Agents; D019394:Ki-67 Antigen
keywords: Chemotherapy; Melanotic neurodermal tumor of infancy; Pathology; Surgical excision