Desmoplastic Fibroma

A extremely rare bone tumor characterized by abundant collagen formation and a fibrous stroma, without evidence of mitosis or pleomorphism. It appears on x-rays as an osteolytic lesion with well-defined margins and must be differentiated from primary fibrosarcoma of bone. (DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1441)

Also Known As:

Networked: 24 relevant articles (0 outcomes, 2 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Aggressive Fibromatosis (Desmoid)
2.	Neoplasms (Cancer)
3.	Fibroma (Fibromatosis)
4.	Malignant Fibrous Histiocytoma
5.	Osteosarcoma (Osteogenic Sarcoma)

Experts

1.	Takagi, Tatsuya: 2 articles (05/2014 - 01/2013)
2.	Okubo, Taketo: 2 articles (05/2014 - 01/2013)
3.	Suehara, Yoshiyuki: 2 articles (05/2014 - 01/2013)
4.	Saito, Tsuyoshi: 2 articles (05/2014 - 01/2013)
5.	Kaneko, Kazuo: 2 articles (05/2014 - 01/2013)
6.	Jiao, Y: 1 article (07/2015)
7.	Li, J: 1 article (07/2015)
8.	Guo, Z: 1 article (07/2015)
9.	Ji, Ch: 1 article (07/2015)
10.	Wang, Z: 1 article (07/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Desmoplastic Fibroma:

1.	beta CateninIBA 09/01/2005 - "Desmoplastic fibroma of bone: an immunohistochemical study including beta-catenin expression and mutational analysis for beta-catenin." 09/01/2005 - "The aim of this study is to investigate the immunohistochemical profile and the involvement of the beta-catenin pathway in desmoplastic fibroma as it is known in desmoid-type fibromatosis. " 09/01/2005 - "However, the beta-catenin pathway does not seem to have the same essential role in the tumorigenesis of desmoplastic fibroma, as it has in desmoid-type fibromatosis." 09/01/2005 - "Immunohistochemistry was performed on 13 cases of desmoplastic fibroma for muscle-specific markers, estrogen and progesterone receptors, CD117, beta-catenin, and the potential downstream target of beta-catenin, namely, cyclin D1. "
2.	ParaffinIBA 03/01/1999 - "In this study, 22 specimens from 19 patients diagnosed with desmoid tumor, desmoplastic fibroma, periosteal desmoid tumor, osteofibrous dysplasia, or fibrous dysplasia were examined by cytogenetic analysis of short-term cultures and bi-color fluorescence in situ hybridization of cytological touch preparations or paraffin-embedded tissue with centromeric probes for chromosomes 8 and 20. "
3.	CollagenIBA 01/01/2013 - "A biopsy specimen from the intraosseous lesion showed a hypocellular area of spindle cell proliferation with dense collagen deposition, which is reminiscent of a histological feature of desmoplastic fibroma. " 05/01/2014 - "Desmoplastic fibroma (DF) is a rare, locally aggressive, solitary tumor microscopically composed of well-differentiated myofibroblasts with abundant dense collagen deposition. " 12/01/2013 - "Desmoplastic fibroma (DF) is a benign intra-osseous neoplasm characterized by the formation of abundant collagen fibers. " 06/01/1985 - "Desmoplastic fibroma of bone is a rare benign tumor consisting of thin, wavy fibroblasts set in an abundant matrix of collagen fibers. "
4.	Cyclin D1IBA 09/01/2005 - "Immunohistochemistry was performed on 13 cases of desmoplastic fibroma for muscle-specific markers, estrogen and progesterone receptors, CD117, beta-catenin, and the potential downstream target of beta-catenin, namely, cyclin D1. "
5.	Progesterone Receptors (Progesterone Receptor)IBA 09/01/2005 - "Immunohistochemistry was performed on 13 cases of desmoplastic fibroma for muscle-specific markers, estrogen and progesterone receptors, CD117, beta-catenin, and the potential downstream target of beta-catenin, namely, cyclin D1. "
6.	Indomethacin (Indometacin)FDA LinkGeneric 04/01/1984 - "Treatment with indomethacin and ascorbic acid is proposed for cases of desmoplastic fibroma of bone when mutilating operations are the alternative or in inoperable patients."
7.	GadoliniumIBA 01/01/2001 - "Desmoplastic fibroma showed a distinct, inhomogeneous gadolinium enhancement. "
8.	Estrogens (Estrogen)FDA Link 09/01/2005 - "Immunohistochemistry was performed on 13 cases of desmoplastic fibroma for muscle-specific markers, estrogen and progesterone receptors, CD117, beta-catenin, and the potential downstream target of beta-catenin, namely, cyclin D1. "
9.	Ascorbic Acid (Vitamin C)FDA LinkGeneric 04/01/1984 - "Treatment with indomethacin and ascorbic acid is proposed for cases of desmoplastic fibroma of bone when mutilating operations are the alternative or in inoperable patients."
10.	trisomy Chromosome 8IBA 08/01/2013 - "Collagenous fibroma (desmoplastic fibroblastoma) with trisomy 8 as the sole cytogenetic abnormality."

Therapies and Procedures

1.	Curettage 04/01/1990 - "It was originally misdiagnosed as a desmoplastic fibroma, and after initial curettage continued to grow for twelve years. " 11/01/1987 - "A case of two recurrences of desmoplastic fibroma of the mandible after vigorous curettage is presented. " 03/01/1999 - "A calcaneal desmoplastic fibroma in a 24-year-old woman had previously been treated with repeated curettage and bone grafting. " 09/01/1987 - "A desmoplastic fibroma of the mandible in an 11-year-old girl provided a difficult diagnosis because of its similarity, both clinically and histologically, to a low-grade fibrosarcoma following its rapid recurrence after primary local curettage. "
2.	Transplants (Transplant) 07/01/2013 - "The authors present three cases of huge mandibular desmoplastic fibromas in paediatric patients treated with mandibulectomy and immediate reconstruction using rib grafts. " 03/01/2013 - "Desmoplastic fibroma of ulna: Excision and reconstruction of olecranon with a fibular graft." 03/01/1999 - "Desmoplastic fibroma of the calcaneus treated with a microvascular bone graft. "
3.	Homologous Transplantation (Allograft) 03/01/1994 - "Allograft bone in the treatment of desmoplastic fibroma. " 07/01/2015 - "All cases of osteoarticular allograft reconstruction with or without the S-K procedure following resection of distal radial tumours (giant-cell tumour in 13, desmoplastic fibroma in two, osteosarcoma in one and malignant fibrous histiocytoma in one) performed from 2000 to 2009 were evaluated by clinical and radiologic examinations; the complications, functional outcomes and Musculoskeletal Tumor Society (MSTS) score were recorded. " 03/01/1998 - "Twenty-four patients had reconstruction of the distal aspect of the radius with use of an osteoarticular allograft, between 1974 and 1992, after excision of a giant-cell tumor (twenty patients), a desmoplastic fibroma (two patients), a chondrosarcoma (one patient), or an angiosarcoma (one patient). "
4.	Spinal Fusion (Spondylosyndesis) 06/01/2000 - "The authors describe a 20-year-old woman with a thoracic epidural desmoplastic fibroma treated by complete resection and posterior spinal fusion. " 09/01/1988 - "A 24-year-old male with desmoplastic fibroma of the axis treated by complete resection and posterior spinal fusion is reported. "
5.	Radiotherapy 06/01/2003 - "Radiotherapy for desmoplastic fibroma of bone: a case report." 12/01/1995 - "Desmoplastic fibroma: a role for radiotherapy?"