Oral and maxillofacial desmoid-type fibromatoses in an eastern Chinese population: a report of 20 cases.
OBJECTIVE
Primary desmoid-type fibromatoses arising from the oral and maxillofacial region are uncommon and hold the character of local invasion and regional recurrence. The purpose of this study was to analyze the clinicoepidemiologic characteristics of this rare disease in an eastern Chinese population.


METHODS
Epidemiological and clinicopathological data on 20 patients were reviewed from the hospital records and analyzed accordingly.


RESULTS
Twelve males and 8 females with a ratio of 3:2 were involved. The mandible was the most common site of presentation. Suspected malignant change was found in 6 cases and an extremely high recurrence rate was also found in up to 53%. Abnormally high blood levels of alkaline phosphatase were found in 65% of patients, which had a close relation with tumor malignant change.


CONCLUSIONS
Chinese people are more susceptible to desmoid-type fibromatoses and are at an extremely high risk of regional malignant change. Elevated serum alkaline phosphatase levels may be taken as an indicator for malignant transformation.
Department of Oral & Maxillofacial Surgery, Ninth People's Hospital, Shanghai Jiao Tong University, School of Medicine, Shanghai Key Laboratory of Stomatology, Shanghai, P R China.
Ruan
R
Min
Zhang
Z
Zun
Wang
W
Lizheng
Dong
D
Minjun
Liu
L
Shengwen
Yang
Y
Wenjun
Zhang
Z
Chenping
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000469:Alkaline Phosphatase / Q000097:blood; D014408:Biomarkers, Tumor / Q000097:blood; D002471:Cell Transformation, Neoplastic; D002648:Child; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D035301:Cranial Fossa, Middle / Q000473:pathology; D005153:Facial Neoplasms / Q000453:epidemiology*; D005260:Female; D005350:Fibroma / Q000453:epidemiology*; D018222:Desmoid Tumors / Q000453:epidemiology*; D005500:Follow-Up Studies; D006801:Humans; D007573:Jaw Neoplasms / Q000453:epidemiology*; D008297:Male; D008339:Mandibular Neoplasms / Q000453:epidemiology; D008441:Maxillary Neoplasms / Q000453:epidemiology; D008875:Middle Aged; D008940:Mitotic Index; D009062:Mouth Neoplasms / Q000453:epidemiology*; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D035583:Rare Diseases / Q000453:epidemiology; D012189:Retrospective Studies; D012737:Sex Factors; D019292:Skull Base Neoplasms / Q000453:epidemiology; D055815:Young Adult
D014408:Biomarkers, Tumor; D000469:Alkaline Phosphatase