Desmoid tumors: clinical features and treatment options for advanced disease.

@article{Kasper2011DesmoidTC,
  title={Desmoid tumors: clinical features and treatment options for advanced disease.},
  author={Bernd Kasper and Philipp Str{\"o}bel and Peter Hohenberger},
  journal={The oncologist},
  year={2011},
  volume={16 5},
  pages={
          682-93
        }
}
Desmoid tumors describe a rare monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Although histologically benign, desmoids are locally invasive and associated with a high local recurrence rate, but lack metastatic potential. On the molecular level, desmoids are characterized by mutations in the β-catenin gene, CTNNB1, or the adenomatous polyposis coli gene, APC. Proof of a CTNNB1 mutation may be useful when the pathological differential… 

Figures and Tables from this paper

Extra-abdominal desmoid tumor: A case report

This case report presents the experience with the treatment of recurrent extra-abdominal desmoid tumor and establishes an optimal therapy protocol for this disease.

DESMOID TUMORS: PATHOPHYSIOLOGY,EPIDEMIOLOGY, DIAGNOSIS AND TREATMENT MODALITIES

The etiology,pathophysiology and treatment modalities are reminded under guidance of the literature and there is no standard consensus on treatment approach.

Evolving strategies for management of desmoid tumor

In summary, DTs present challenges to physicians in diagnosis and prognosis, as well as in determining treatment initiation, type, duration, and sequence, so evaluation by a multidisciplinary team with expertise in DT and patient‐tailored management are essential.

Systemic Treatment Approaches for Sporadic Desmoid-Type Fibromatosis: Scarce Evidence and Recommendations

  • B. Kasper
  • Medicine
    Oncology Research and Treatment
  • 2015
To better define possible therapeutic strategies, a consensus approach has been initiated, bringing together sarcoma experts from the European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG) with patient advocates from SARcoma Patients EuroNet (SPAEN).

Desmoid-Type Fibromatosis

This is a narrative review of this uncommon disease and presents current knowledge about molecular pathogenesis, diagnosis and treatment of desmoid tumors.

Medical treatment of mammary desmoid-type fibromatosis: which benefit?

TKI had been an interesting alternative option to initial surgery, providing at least a partial response and potentially allowing less mutilating surgery, however, no pharmacological mechanism can unequivocally explain TKI efficacy.

Immunohistochemical evaluation of potential molecular targets of desmoid-type fibromatosis.

Desmoid-type fibromatosis is locally aggressive tumor rare in general population, although commonly present in patients with familial adenomatous polyposis, significantly contributing to the

Desmoid tumors of the head and neck: A therapeutic challenge

Although the margin status seems to be of importance, operations that avoid function loss and esthetic disfigurement should be the primary goal and the efficacy of postoperative radiotherapy is controversial.

Desmoid-Type Fibromatosis: Who, When, and How to Treat

Desmoid-type fibromatosis is a sarcoma subtype that gathers some singular characteristics, making it a difficult challenge to face in clinical practice, and the Notch pathway inhibition in DF is one of the most promising potential targets to explore.
...

References

SHOWING 1-10 OF 70 REFERENCES

Desmoid tumor: from surgical extirpation to molecular dissection

Assessment and treatment of individual patients in a multidisciplinary setting is critical to achieve the most favorable outcome in this disease and additional study of the molecular determinates of desmoid behavior is needed to guide therapeutic selection.

The enigma of desmoid tumors

  • S. Okuno
  • Medicine
    Current treatment options in oncology
  • 2006
The treatment of desmoid tumors remains an enigma, and because desmoids spontaneously regress, any claim of successful intervention must be viewed skeptically.

Multimodality treatment of mesenteric desmoid tumours.

Desmoid tumors

  • J. Biermann
  • Medicine
    Current treatment options in oncology
  • 2000
Patients with desmoid tumors are optimally managed in a multidisciplinary setting with close collaboration between surgeon, pathologist, diagnostic radiologist, radiation oncologist, and medical oncologists.

Multimodality management of desmoid tumors: How important is a negative surgical margin?

The current evidence for multimodality therapy of desmoids is reviewed, with a focus on the importance of resection margins, and an algorithm for treatment is presented.

Desmoid tumour in familial adenomatous polyposis. A review of literature

The prognosis in mesenteric DT is serious, and improvement of the therapeutic strategy awaits current international studies.

Natural course of desmoid-type fibromatosis

Simple observation is a noninvasive and function-preserving treatment for desmoid-type fibromatosis and is effective for two patients in whom the tumors were initially incorrectly diagnosed as other tumors.

Immunohistochemical analysis of desmoid tumours

The data from this immunohistochemical study show that the published effects of antioestrogens and imatinib mesylate in the treatment of aggressive fibromatoses may not be attributable to oestrogen receptor α or c-KIT expression.

Response of extraabdominal desmoid tumors to therapy with imatinib mesylate

Surgical resection with adjuvant radiotherapy for a positive surgical margin remains the standard approach and Imatinib mesylate appears to demonstrate inhibitory activity against multiple class 3 receptor tyrosine kinases, including platelet‐derived growth factor receptor (PDGFR)‐α and PDGFR‐β, as well as c‐kit.

Low grade fibromyxoid sarcoma: problems in the diagnosis and management of a malignant tumour with bland histological appearance

LGFMS is a tumour with low grade histological features but a high risk of local recurrence and a significant risk of metastasis which can be very late, there should be a high index of suspicion for this rare tumour and a low threshold for sending tissue for cytogenetics and/or molecular genetics.
...