The Department of Oral Pathology reported the presence of desmoplastic fibroma recurrence with bundles of spindle-shaped, fibroblast-like cells in a collagen matrix (Figure 5(a)).
There is little information about the combination of desmoplastic fibroma and tuberous sclerosis; this combination is uncommon [8, 10, 11].
Demographic data of patients Patient Gender Age Etiology Location of defect 1 M 16 Ameloblastoma LEFT 2 M 48 SCC LEFT 3 M 33 SCC RIGHT 4 M 30 Ameloblastoma LEFT 5 M 7 Desmoplastic Fibroma
LEFT 6 M 29 Ameloblastome LEFT 7 M 54 SCC RIGHT 8 M 24 SCC RIGHT 9 M 77 SCC LEFT 10 F 46 SCC BILATERAL 11 M 27 SCC LEFT Patient Defect type Recipient vein Complication 1 HC + m + t STA and lJV 2 H +c STA and lJV Donor area-VAC-Graft 3 L + m + t FA and FV 4 L + m STA and lJV 5 L + c STA and lJV 6 L + m STA and lJV 7 L + m + t STA and lJV 8 L + c STA and lJV 9 H + c + m + t STA and lJV Flap necrosis- Pec.
DISCUSSION: Jaffe was the first to describe desmoplastic fibroma in 1958 and distinguish it from fibrosarcoma.
The desmoplastic fibroma is a slowly progressing locally invasive tumor characterized by a production of the collagen fibres by its tumor cells.
E, A spindle cell stroma-predominant area with dense collagen deposition can sometimes be seen at the periphery of the tumor, simulating desmoplastic fibroma
. F, Cartilage differentiation of a parosteal osteosarcoma reveals irregularly arranged chondrocytes with mild to moderate pleomorphism and occasional binucleation (arrow).
The WHO defines a desmoplastic fibroma as a rare, benign bone tumour composed of spindle cells with minimal atypia and abundant collagen production (3).
Desmoplastic fibroma: report of a rare tumor of the oral structures.
Selected characteristics of 28 cases of benign palatal tumor Variable n (%) Sex Male 15 (53.6) Female 13 (46.4) Tumor location Hard palate 23 (82.1) Soft palate 5 (17.9) Chief complaint Palatal mass * 14 (50.0) Difficulty chewing * 5 (17.9) Bleeding * 4 (14.3) Throat lump ([dagger]) 3 (10.7) Snoring ([dagger]) 1 (3.6) Dysphagia ([dagger]) 1 (3.6) Variable n (%) Treatment Surgery 26 (92.9) Steroid and ablation ([dagger]) (7.1) Histopathologic diagnosis Pleomorphic adenoma * 9 (32.1) Pyogenic granuloma * 5 (17.9) Hemangioma ([double dagger]) 4 (14.3) Papilloma ([section]) 4 (14.3) Median palatine cyst * 2 (7.1) Leiomyoma * 1 (3.6) Desmoplastic fibroma
* 1 (3.6) Osteoma * 1 (3.6) Myoepithelioma * 1 (3.6) Recurrence Yes * 2 (7.1) No 26 (92.9) * All patients had a hard-palate tumor.
Key words: Desmoplastic fibroma, mandible, limited mouth opening.
Desmoplastic fibroma of bone is a rare benign intraosseous tumor of connective tissue origin with locally aggressive behaviour, which may lead to infiltration of the surrounding soft tissues [Shafer et al., 1974; Templeton et al., 1997; Wippold et al., 2005].
We present a case of desmoplastic fibroma in the rib of a 19-year-old man, adding a fourth case to the 3 previously reported cases involving this unusual location.
On histologic examination, the tumor was classified as a desmoplastic fibroma. It was hypocellular to moderately cellular and composed of bland fibroblasts in a background of numerous thick and wavy collagen fibers (Figure 3).
Desmoplastic fibroma (DF) was first described as a distinct entity and given its present name by Jaffe in 1958.
Desmoplastic fibroma is one of the rarest bone tumors.