Bioscience Chapter Database :: 3618 Chapters Now Online

Chapter category: Cancer Genetics

Chromosome Translocations in Dermatofibrosarcoma

This chapter appears in the following book:

Translocations in Solid Tumors

Edited by: Colin Cooper
ISBN: 1-58706-033-7
» Get more information about this book at landesbioscience.com «

Chapter authors:
Claude Turc-Carel


[+] view image
Dermatofibrosarcoma Protuberans (DP), also named Darier–Ferrand tumor, is a soft tissue tumor typically located in the deep dermis. It arises at any site but preferentially in the subcutaneous tissue of the trunk and proximal extremities. DP occurs during early and mid–adult life.1 Although authentic DP may also but seldomly, occurs in children, a distinct entity, giant cell fibroblastoma (GCF), has been considered as a juvenile form of DP.1,2 DP is locally invasive and can be classed among tumors of low or borderline malignancy. Current therapy is purely surgical. The overall high recurrence rate, up to 50% and the repetition of recurrences are common, reflecting the extensive infitrative growth of the tumor and the difficulty to recognize it at the time of the surgery.1,3 To avoid recurrence, such wide margins are required that secondary reconstructive surgery and skin grafts are often needed. An accurate diagnosis differentiating DP from other tumors, benign or malignant, is therefore crucial. It is sometimes particularly puzzling, requiring highly reliable diagnostic markers as are recurrent chromosome translocations and their subsequent specific molecular rearrangements.4,5 In this context, we investigated the features of DP as part of a program on cytogenetic and molecular characterization of soft tissue tumors.

» Access chapter for $19



Additional chapters from this book:

Toward New Approaches: Targeting Tumor Specific Molecular Alterations with Immune Based Therapy

Crystal L. Mackall, Theresa J. Goletz, Jay A. Berzofsky, and Lee J. Helman

Effective immune based therapies for cancer have long been sought by immunologists and oncologists. Indeed many of the basic immunologic principles of transplantation were discovered serendipi...

Molecular Diagnostics

Marc Ladanyi

Specific chromosomal translocations are found in several solid tumors, including many sarcomas (Table 1) and some epithelial tumors (Table 2). Sever...

Renal Carcinoma

Colin S. Cooper and Janet M. Shipley

Renal neoplasms account for 3% of adult malignancies with approximately 27,000 cases and more than 10,000 deaths in the US each year.1 In children renal tumors, predominantly Wilm's...

Thyroid Cancer

Marco A. Pierotti, Italia Bongarzone, Maria Grazia Borrello, Angela Greco, Silvana Pilotti and Gabriella Sozzi

The thyroid gland manifests a wide spectrum of malignant neoplasms, including medullary thyroid carcinoma (MTC), which develop from the neural–crest derived C cells, and tumors originatin...

ETV6–NTRK3 Gene Fusions in Spindle Cell Tumors of Early Childhood

Poul H.B. Sorensen

Molecular analysis of gene fusions expressed in human tumors is providing numerous insights into the oncogenic process. Most chimeric oncoproteins characterized to date appear to act as aberra...

Chromosome Translocations in Dermatofibrosarcoma

Claude Turc-Carel

Dermatofibrosarcoma Protuberans (DP), also named Darier–Ferrand tumor, is a soft tissue tumor typically located in the deep dermis. It arises at any site but preferentially in the subcuta...

Synovial Sarcoma

Janet M. Shipley and Colin S. Cooper

Synovial sarcoma accounts for about 10% of adult soft tissue sarcomas with approximately 200 new cases a year in the UK and 800 in the USA. They occur mainly in adolescence although can presen...

The TLS-CHOP Oncoprotein and Human Liposarcoma

Masahiko Kuroda, John Sok, and David Ron

In thinking about cancers that may be amenable to specific therapeutic approaches, one is drawn to the relatively small group of neoplasms, mainly leukemias, lymphomas and sarcomas, that have ...

Chromosome Translocations in the Ewing Family of Tumors

Olivier Delattre and Nicolas Sévenet

Ewing's sarcoma of bone was originally identified by James Ewing in 1921 as a tumor of long bones, distinct from osteosarcoma, sensitive to radiation and of possible endothelial origin. Subseq...

Chromosomal Translocations in Alveolar Rhabdomyosarcoma

Frederic G. Barr

Alveolar rhabdomyosarcoma (ARMS) is one subtype of rhabdomyosarcoma (RMS), a heterogeneous family of soft tissue tumors that generally occur in pediatric patients and are related to the skelet...

Chromosome Translocations in Benign Solid Tumors

Wim J.M. Van de Ven, Koen Kas, Marleen M.R. Petit, Eric F.P.M. Schoenmakers and Marianne L. Voz

The evolution from classical genetics to molecular genetics and genetic engineering has revealed the immense potential of gene manipulation for basic science, medicine, and biotechnology. The ...

Molecular Cytogenetics of Complex Chromosomal Rearrangements

R.F. Suijkerbuijk, A. Simons, and A. Geurts van Kessel

Since the end of the last century karyotypicabnormalities have been known to occur in neoplastic cells.1–2 Significant progress in the interpretation of these aberrations was, ...

Cytogenetics of Solid Tumors

Jonathon A. Fletcher

Most of the chromosome translocations discussed in this book were discovered through cytogenetic analyzes. In fact, traditional cytogenetic approaches, in which various stains are used to prod...

Toward New Approaches: Targeting Tumor Specific Molecular Alterations with Immune Based Therapy

C. L. Mackall, T. J. Goletz, J. A. Berzofsky and L.J. Helman

Effective immune based therapies for cancer have long been sought by immunologists and oncologists. Indeed many of the basic immunologic principles of transplantation were discovered serendipi...

Molecular Diagnostics

Marc Ladanyi

Specific chromosomal translocations are found in several solid tumors, including many sarcomas (Table 1) and some epithelial tumors (Table 2). Several lines of evidence suggest that the fusion...

Renal Carcinoma

Colin S. Cooper and Janet M. Shipley

Renal neoplasms account for 3% of adult malignancies with approximately 27,000 cases and more than 10,000 deaths in the US each year.1 In children renal tumors, predominantly Wilm's...

Thyroid Cancer

M. A. Pierotti, I. Bongarzone, M. G. Borrello, A. Greco, S. Pilotti and G. Sozzi

The thyroid gland manifests a wide spectrum of malignant neoplasms, including medullary thyroid carcinoma (MTC), which develop from the neural–crest derived C cells, and tumors originatin...

ETV6–NTRK3 Gene Fusions in Spindle Cell Tumors of Early Childhood

Poul H.B. Sorensen

Molecular analysis of gene fusions expressed in human tumors is providing numerous insights into the oncogenic process. Most chimeric oncoproteins characterized to date appear to act as aberra...

Chromosome Translocations in Dermatofibrosarcoma

Claude Turc-Carel

Dermatofibrosarcoma Protuberans (DP), also named Darier–Ferrand tumor, is a soft tissue tumor typically located in the deep dermis. It arises at any site but preferentially in the subcuta...

Synovial Sarcoma

Janet M. Shipley and Colin S. Cooper

Synovial sarcoma accounts for about 10% of adult soft tissue sarcomas with approximately 200 new cases a year in the UK and 800 in the USA. They occur mainly in adolescence although can presen...

The TLS-CHOP Oncoprotein and Human Liposarcoma

Masahiko Kuroda, John Sok and David Ron

In thinking about cancers that may be amenable to specific therapeutic approaches, one is drawn to the relatively small group of neoplasms, mainly leukemias, lymphomas and sarcomas, that have ...

Chromosomal Translocations in Alveolar Rhabdomyosarcoma

Frederic G. Barr

Alveolar rhabdomyosarcoma (ARMS) is one subtype of rhabdomyosarcoma (RMS), a heterogeneous family of soft tissue tumors that generally occur in pediatric patients and are related to the skelet...

Chromosome Translocations in Benign Solid Tumors

Wim J.M. Van de Ven, Koen Kas, Marleen M.R. Petit, Eric F.P.M. Schoenmakers, and Marianne L. Voz

The evolution from classical genetics to molecular genetics and genetic engineering has revealed the immense potential of gene manipulation for basic science, medicine, and biotechnology. The ...

Molecular Cytogenetics of Complex Chromosomal Rearrangements

R.F. Suijkerbuijk, A. Simons, and A. Geurts van Kessel

Since the end of the last century karyotypicabnormalities have been known to occur in neoplastic cells.1–2 Significant progress in the interpretation of these aberrations was, ...

Cytogenetics of Solid Tumors

Jonathan A. Fletcher

Most of the chromosome translocations discussed in this book were discovered through cytogenetic analyzes. In fact, traditional cytogenetic approaches, in which various stains are used to prod...


SIGN IN

Email:


Password:


lost password?




[ Home | Authors | Editors | Custom Books | Chapter Reprints | Subscribe | Contact | Biotoons ]