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Management strategy in patient with familial gigantiform cementoma: A case report and analysis of the literature.

Abstract Familial gigantiform cementoma (FGC) is a rare benign autosomal dominant fibrocemento-osseous lesion generally limited to the facial bones, typically in the anterior portion of the mandible; it is often associated with abnormalities of the long bones and prepubertal pathologic fractures. Owing to the small number of such patients, a uniform treatment criterion has not been established. This paper presents a patient with FGC who was treated in our department, and offers a systematic review of the patients reported in the literature. Our aim was to explore the treatment strategy for patients with FGC.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 29390315
OWN - NLM
STAT- In-Process
LR  - 20180202
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 50
DP  - 2017 Dec
TI  - Management strategy in patient with familial gigantiform cementoma: A case report
      and analysis of the literature.
PG  - e9138
LID - 10.1097/MD.0000000000009138 [doi]
AB  - RATIONALE: Familial gigantiform cementoma (FGC) is a rare benign autosomal
      dominant fibrocemento-osseous lesion generally limited to the facial bones,
      typically in the anterior portion of the mandible; it is often associated with
      abnormalities of the long bones and prepubertal pathologic fractures. Owing to
      the small number of such patients, a uniform treatment criterion has not been
      established. This paper presents a patient with FGC who was treated in our
      department, and offers a systematic review of the patients reported in the
      literature. Our aim was to explore the treatment strategy for patients with FGC. 
      PATIENT CONCERNS: Our patient, a 13-year-old boy, presented with a painless
      enlargement of the mandible first noted 2 years earlier. It had grown rapidly
      over the preceding 8 months, affecting both his appearance and ability to chew.
      DIAGNOSIS: Based on the pathologic, clinical, and radiographic features, FGC was 
      diagnosed. INTERVENTIONS: Mandibuloectomy was performed. The mandibular defect
      was immediately reconstructed with his right vascularized iliac crest flap. At
      the same time, a PubMed search was conducted to identify studies reporting on
      other patients with FGC. OUTCOMES: A 3-dimensional computed tomography (3D-CT)
      scan demonstrated appropriate height of the new alveolar bone. Follow-up results 
      showed recovery of the patient's appearance and mandibular function. He was free 
      of recurrence at 4-year follow-up. LESSONS: FGC is a rare benign
      fibrocemento-osseous lesion of the jaws that can cause severe facial deformity.
      Incomplete removal leads to more rapid growth of the residual lesion. Therefore, 
      extensive resection is a suitable strategy to avoid recurrence. Defects of the
      facial bones found intraoperatively should be repaired with resort to an
      appropriate donor site. However, it is important to be aware that patients with
      FGC always have concomitant abnormalities of skeletal metabolism and structure,
      as well as a vulnerability to fractures of the long bones of the lower extremity.
      Therefore, the optimal management strategy should include a review of treatment
      options for other patients as reported in the literature. An optimal protocol can
      not only provide sufficient high-quality bone suitable for the reconstruction of 
      bone defects, but also minimize complications and maximize quality of life.
CI  - Copyright (c) 2017 The Authors. Published by Wolters Kluwer Health, Inc. All
      rights reserved.
FAU - Wang, Hong-Wei
AU  - Wang HW
AD  - Department of Oral and Maxillofacial-Head and Neck Oncology, Ninth People's
      Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai Key
      Laboratory of Stomatology, Shanghai, China.
FAU - Ma, Chun-Yue
AU  - Ma CY
FAU - Qin, Xing-Jun
AU  - Qin XJ
FAU - Zhang, Chen-Ping
AU  - Zhang CP
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
EDAT- 2018/02/03 06:00
MHDA- 2018/02/03 06:00
CRDT- 2018/02/03 06:00
PHST- 2018/02/03 06:00 [entrez]
PHST- 2018/02/03 06:00 [pubmed]
PHST- 2018/02/03 06:00 [medline]
AID - 10.1097/MD.0000000000009138 [doi]
AID - 00005792-201712150-00065 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Dec;96(50):e9138. doi: 10.1097/MD.0000000000009138.