Nuchal-Type Fibroma in Two Related Patients With Gardner's Syndrome

Abstract

Nuchal-type fibroma is a distinct subcutaneous and dermal fibrous tissue proliferation that has been previously definitely identified in one patient with Gardner's syndrome and has been possibly present in two others. Gardner's syndrome is an autosomal-dominant condition with variable expressivity that comprises epidermoid cysts, fibrous tumors, osteomas, intestinal polyposis, as well as other findings. We report two cases of nuchal-type fibroma presenting in a 13-year-old boy in the right upper back and in his 60-year-old grandfather in the upper chest at the posterior axillary line. Both individuals carried a diagnosis of Gardner's syndrome and neither of them had diabetes. Although the boy has as of now only presented with cutaneous manifestations of Gardner's syndrome, his grandfather has exhibited both cutaneous and intestinal evidence of this syndrome. In addition, the boy's mother and her sister have documented Gardner's syndrome. Light microscopic findings of nuchal-type fibroma from both patients include paucicellular, haphazardly arranged collagen bundles with entrapped adipose tissue. A marked diminution of elastic fibers was noted with Van-Gieson stains. The lesions were diffusely positive for CD34 and contained a few factor XIIIa-positive cells. Electron microscopic analysis revealed no differences between the collagen comprising the nuchal-type fibroma as compared with control dermal collagen obtained from skin away from the tumor. These cases strengthen the view that there is an association between nuchal-type fibroma and Gardner's syndrome.

Nuchal-type fibroma of the shoulder: A case report and review of the literature

“…The tumor typically presents between the third and fifth decades of life, but lesions have additionally been reported in patients aged 3-74 years. Nuchal-type fibroma has been observed to be strongly associated with diabetes mellitus and Gardner's syndrome (12). There is a ratio of 4:1 male to female predominance, although this is not observed in Gardner's syndrome-associated patients (4).…”

CD34+ connective tissue nevi: Are they unusual?

“…1 Other spindle cell neoplasms associated with CD34 expression include cases of sclerotic fibromas, nuchal fibromas, solitary fibrous tumors, cellular blue nevi, nerve sheath tumors, stromal cells surrounding trichoepitheliomas, and Kaposi sarcoma [1][2][3][4][5] (Table II).…”

Nuchal-type Fibroma of the Coccyx

“…It suggests that nuchal-type fibroma with diabetes may occur by tumor-like changes in fibrous tissues, like a link between diabetes and scleredema. And, some authors suggested by their case reports that nuchal-type fibroma is related to Gardner's syndrome [7][8][9] . Gardner syndrome, caused by mutations in the adenomatous polyposis coli (APC) gene, is characterized by polyposis coli, osteomas, and various soft-tissue tumors.…”