Desmoid Tumors

1. Front Matter

   Pages 1-1

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2. The Identification and Treatment of Desmoid Tumors

   1. Front Matter

      Pages 3-3

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   2. Introduction

      • Charisse Litchman

      Pages 1-2

   3. Clinical Presentation of Desmoid Tumors

      • Anastasia Constantinidou, Michelle Scurr, Ian Judson, Charisse Litchman

      Pages 5-16

   4. Pathology of Desmoid Tumors

      • Wai Chin Foo, Alexander J. Lazar

      Pages 17-28

   5. APC/β-Catenin Deregulation in Desmoid Tumors: Important Implications for Diagnosis, Prognosis, and Therapy

      • Chiara Colombo, Dina Lev

      Pages 29-46

   6. Imaging Techniques in Desmoid Tumors

      • Robert A. Lefkowitz, Sinchun Hwang, Jonathan Landa

      Pages 47-75

   7. Surgical Management of Desmoid Tumors

      • Paxton V. Dickson, Raphael Pollock

      Pages 77-90

   8. Systemic Therapy in the Treatment of Desmoid Tumors

      • Andrea Marrari, Suzanne George

      Pages 91-104

   9. Radiation Therapy for Desmoid Tumors

      • Hani O. Al-Halabi, Yen-Lin Chen, John T. Mullen, Sam S. Yoon, Francis J. Hornicek, Thomas F. DeLaney

      Pages 105-125

   10. Interventional Radiology

       • David S. Pryluck, Joseph P. Erinjeri

       Pages 127-144

3. Special Populations with Desmoid Tumors

   1. Front Matter

      Pages 145-145

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   2. Desmoid Disease in Familial Adenomatous Polyposis

      • James Church

      Pages 147-157

   3. Desmoid Tumor in Children and Adolescents: The Influence of Age

      • Aaron R. Weiss, Anthony Montag, Stephen X. Skapek

      Pages 159-178

4. Considerations for Current and Future Advancement in the Search for a Cure

   1. Front Matter

      Pages 179-179

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   2. Microarrays and High-Throughput Sequencing in Desmoid-Type Fibromatosis and Scar

      • Robert T. Sweeney, Matt van de Rijn

      Pages 181-193

   3. Desmoid Tumors: Are They Benign or Malignant?

      • Benjamin Alman

      Pages 195-203

   4. The Role of Patient Advocacy Groups in Rare Tumors Such as Desmoid Tumors

      • Oakleigh Ryan

      Pages 205-215

5. Back Matter

   Pages 209-209

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Desmoid Tumors (DT), also called Aggressive Fibromatoses, are a rare fibroblastic proliferative disease, with an incidence of 2 to 4 new cases per million people per year. Despite the absence of a metastatic potential, DT cause significant morbidity and at times mortality due to its locally invasive behaviour. The anatomical locations can be abdominal, extra-abdominal (often in the extremities) and intra-abdominal. This book is the first manuscript dedicated entirely to Desmoid Tumors. Written by prominent clinicians, researchers and advocacy group experts, patients and professionals alike will find this to be a comprehensive review. Clinical presentation, imaging guidelines and treatment paradigms are highlighted. Both the sporadic and heredity forms (Familial Adenomatous Polyposis) will be discussed. A thorough discussion on the unique issues in children with DT is included. A portion of the book will address the role of the APC gene, the β-catenin protein and the role of mutations in the genesis of DT. Emerging cutting edge research techniques will be revealed. Also included is a thoughtful discussion on the controversial labelling of Desmoid Tumors as benign and the consequences of such a designation. The role of advocacy groups in supporting research and in promoting awareness of rare diseases such as DT will be outlined. This book will serve as basis to prepare clinicians, researchers and patients to embark on the quest for a cure for Desmoid Tumors.

• APC
• Aggressive Fibromatosis
• Desmoid Tumor
• Familial Adenomatous Polyposis
• β-catenin
• surgical oncology
• abdominal surgery

• , Department of Neurology, Columbia University, Stamford, USA

  Charisse Litchman

.

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