PMID- 29653868 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20181211 IS - 1399-0020 (Electronic) IS - 0901-5027 (Linking) VI - 47 IP - 7 DP - 2018 Jul TI - Clinicopathological investigation of odontogenic fibroma in tuberous sclerosis complex. PG - 918-922 LID - S0901-5027(18)30112-7 [pii] LID - 10.1016/j.ijom.2018.03.025 [doi] AB - Tuberous sclerosis complex (TSC) is an autosomal dominant inherited disease characterized by systemic hamartoma and diverse systemic features. TSC1 and TSC2 are the causative genes, and mental retardation, epileptic seizures, and facial angiofibroma develop in many patients with the disease. The case of a patient with TSC who developed a central odontogenic fibroma of the mandible is reported here. The patient was a 21-year-old woman who was referred with a swelling of the labial gingiva in the region of the right lower lateral incisor and canine. Dental radiography revealed a multilocular radiolucent region with a clear boundary. The right lower lateral incisor and canine were continuous with the lesion and thus were excised en bloc. The lesion was encapsulated and easily dissected. The diagnosis on immunohistological staining was odontogenic fibroma without an epithelial component. TSC1/2 gene mutation causes abnormal activation of mammalian target of rapamycin (mTOR) downstream of the PI3K-AKT pathway. The odontogenic fibroma in this patient was positive for mTOR, suggesting that the development of the odontogenic fibroma was the result of abnormal activation of mTOR, as in angiofibroma. The clinical course of this patient is presented and the developmental mechanism of central odontogenic fibroma is discussed. CI - Copyright (c) 2018 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. FAU - Musha, A AU - Musha A AD - Department of Oral and Maxillofacial Surgery/Plastic Surgery, Gunma University Graduate School of Medicine, Gunma, Japan; Department of Radiation Oncology, Gunma University Graduate School of Medicine, Gunma, Japan. Electronic address: musha@gunma-u.ac.jp. FAU - Yokoo, S AU - Yokoo S AD - Department of Oral and Maxillofacial Surgery/Plastic Surgery, Gunma University Graduate School of Medicine, Gunma, Japan. FAU - Takayama, Y AU - Takayama Y AD - Department of Oral and Maxillofacial Surgery/Plastic Surgery, Gunma University Graduate School of Medicine, Gunma, Japan. FAU - Sato, H AU - Sato H AD - Department of Radiation Oncology, Gunma University Graduate School of Medicine, Gunma, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20180410 PL - Denmark TA - Int J Oral Maxillofac Surg JT - International journal of oral and maxillofacial surgery JID - 8605826 SB - IM MH - Biopsy MH - Diagnosis, Differential MH - Female MH - Fibroma/diagnostic imaging/*pathology/*surgery MH - Humans MH - Immunohistochemistry MH - Odontogenic Tumors/diagnostic imaging/*pathology/*surgery MH - Tuberous Sclerosis/diagnostic imaging/*pathology MH - Young Adult OTO - NOTNLM OT - mammalian target of rapamycin OT - odontogenic fibroma OT - tuberous sclerosis complex EDAT- 2018/04/15 06:00 MHDA- 2018/12/12 06:00 CRDT- 2018/04/15 06:00 PHST- 2018/01/12 00:00 [received] PHST- 2018/02/18 00:00 [revised] PHST- 2018/03/26 00:00 [accepted] PHST- 2018/04/15 06:00 [pubmed] PHST- 2018/12/12 06:00 [medline] PHST- 2018/04/15 06:00 [entrez] AID - S0901-5027(18)30112-7 [pii] AID - 10.1016/j.ijom.2018.03.025 [doi] PST - ppublish SO - Int J Oral Maxillofac Surg. 2018 Jul;47(7):918-922. doi: 10.1016/j.ijom.2018.03.025. Epub 2018 Apr 10.