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A Brazilian family with hereditary gingival fibromatosis is described with multiple affected subjects in four generations. A total of 50 (48%), of 105 at-risk offspring in a family of 132 members were affected, consistent with an autosomal dominant mode of inheritance. This family appears to represent the largest pedigree with this condition reported in the literature.

Twenty-six persons with Laband syndrome have been reported in the literature to date. We report two further unrelated cases and suggest that a skeletal anterior open bite and symmetric soft tissue swelling of the vault of the palate be added to the variable features associated with the syndrome. There is clear evidence in the literature of autosomal dominant inheritance but our analysis indicates that in some cases recessive transmission is likely.

The clinicopathologic features of 3 new cases and 28 cases obtained from the literature of fibromatosis of the oral and paraoral soft tissues and 9 new cases and 51 cases obtained from the literature of desmoplastic fibroma of the jaws are reported and reviewed. The results of the literature review show that all of the soft tissue lesions occurred before the age of 30 years (mean age, 8.3 +/- 7.0 years). There was a slight male predilection (male to female, 1.2:1). Most cases occurred in the paramandibular soft tissues and mandibular mucosa. Surface resorption of the underlying bone was a common feature. The recurrence rate was 22% and all of these were controlled with further therapy. The desmoplastic fibromas occurred in a slightly older age group (mean age, 14.7 +/- 12.1 years) although 88% of the patients were still younger than 30 years. There was no sex predilection. The vast majority occurred in the area of the mandibular body-angle-ramus. Radiographically, half were unilocular and half were multilocular. Some had poorly defined radiographic margins. The recurrence rate was 25%. The treatment of choice is surgical resection with wide margins, especially for cellular lesions and for those that erode bone with invasion of adjacent soft tissues. Microscopically, both peripheral and central lesions consist of variable proportions of collagen and fibroblasts with a uniform histologic appearance. Hyalinization of collagen is variable but is more frequent in the intraosseous lesions. The cells show no features of anaplasia, and although occasional mitoses may be found, they are always normal in appearance.

The literature recording cases of the rare juvenile hyaline fibromatosis is reviewed. Gingival enlargement is a regular feature of this entity. A case report and treatment of the gingival lesions are described, and the differential diagnosis and prognosis are discussed.

Excessive gingival enlargement has been reported in the literature to be due to and/or accompanied by ingestion of Dilantin sodium, hypertrichosis, hereditary factors, cherubism, or ectodermal dysplasia; gingival enlargement has also been found where none of the above factors could be demonstrated, and this type of enlargement has been generally labeled “idiopathic.” The case reported here appears to be of special interest because of (1) the occurrence of both gingival enlargement and excessive osseous destruction in a very young person and (2) the absence of any marked medical deviation from normal, other than an elevated alkaline phosphatase activity. The latter finding, of course, may be related to the osseous destruction, as reports have implicated this enzyme in the process of bone resorption 5 or have suggested its presence as a sequel to bone resorption. 8