Desmoid tumor medical therapy

Desmoid tumor Microchapters
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Differentiating Desmoid tumor from other Diseases
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Case #1

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]Faizan Sheraz, M.D. [3]

Overview

Wait and watch strategy is applied to desmoid tumors which are asymptomatic, unresectable, non-life threatening, not causing any significant impairment, and resectable tumors with increased morbidity associated with surgery.Different drugs including chemotherapeutic agents, NSAIDs, anti-hormonal agents and tyrosine kinase inhibitors can be used to shrink or stabilize the tumor size and to improve the symptoms. Radiation therapy and tumor ablation with heat, cold, microwave and/or high-frequency ultrasound waves can also be of therapeutic use as required.

Medical Therapy

• Ideally, patients with desmoid tumors should be evaluated by a multi-disciplinary team which includes surgeons, medical oncologists, radiation oncologists, geneticists and nurses
• Patients with desmoid tumors should be evaluated in a hospital with expertise in sarcoma (usually such hospitals are designated as NCCN [National Comprehensive Cancer Network] centers)
• Wait and watch or observation of the tumor carefully with images and/or physical examination, is implied as a primary therapeutic option for desmoid tumors with following features:
  • Potentially resectable but asymptomatic or minimally symptomatic
  • Non-life threatening
  • Not causing any significant impairment
  • Stable appearance on screening modalities
  • Unresectable tumors
  • Resectable but surgery would lead to unacceptable morbidity
  • How often scans and/or physical exams are needed during a “wait and watch” period varies with each situation
• Treatment is recommended for extra-abdominal or abdominal desmoid tumors associated with:
  • Symptoms
  • Progressively enlarging tumors irrespective of symptoms
  • Imminent risk to adjacent structures
  • Tumor creating cosmetic concerns
• Goals of medical therapy are:
  • Shrinkage of tumor size
  • Stabilization of tumor
  • Improvement in symptoms after a very wide variety of treatments
• Following table shows drugs used in medical therapy for desmoid tumors:

Different drugs used in medical therapy of desmoid tumors

Drug class	Drug name
Chemotherapeutic agents	Doxorubicin (Adriamycin, Rubex) Doxil (liposomal doxorubicin) Dacarbazine (DTIC-Dome) Methotrexate Vinorelbine Vinblastine Carboplatin (Paraplatin)
NSAIDs	Sulindac
Anti-hormonal agents	Tamoxifen (Soltamox, Nolvadex) Prostaglandin inhibitors
Tyrosine kinase inhibitors (Novel molecular targeted therapies)	Sorafenib Imatinib (Gleevec)

Desmoid tumour before (A, C) and 2 years after (B, D) the commencement of NSAID. Multi planner reformation (MPR)-CT demonstrates the sporadic desmoid tumours originating from the intra-abdominal cavity (arrows). Frontal (A, B) and axial (C, D) images are shown. The tumour has shown a remarkable shrinkage with a regression rate of 68.5% along with disappearance of intratumoural septa.Source: Tanaka K. et al, Department of Surgery, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan

Radiation Therapy

• Desmoids are radiosensitive tumors
• Radiation therapy uses high-energy waves such as X-rays to kill or shrink tumor cells
• Rarely, radiation therapy is used if:
  • Patient is symptomatic
  • Medical therapy fails
  • Surgery fails
  • Patient is not a good surgical candidate
  • Patient declines surgery
  • Surgical morbidity would be excessive
  • Margins are positive post-resection
  • Tumor recurrence post-resection
• Depending on individual patient characteristics, the radiation may be applied by following two methods:
  • Conventional linear accelerators (radiotherapy treatment machines)
  • Proton beam therapy
• Depending on the size and location of the tumor, radiation is delivered either in low doses over the course of 5 to 6 weeks or in a single high dose
• Radiation therapy is effective in approximately 30% of cases
• Following complications can occur from radiation therapy due to late radiation effects:
  • Secondary malignancies (especially in younger patients)
  • Fibrosis associated with radiation therapy

Tumor ablation

• In some rare cases, ablation of desmoid tumors can be used as a therapeutic option
• Following different kinds of ablation can be used:
  • Cold ablation
  • Heat/thermal ablation
  • Microwave ablation
  • High-frequency ultrasound ablation