A to Z of Desmoid Tumors

Shinagare, Atul B.; Ramaiya, Nikhil H.; Jagannathan, Jyothi P.; Krajewski, Katherine M.; Giardino, Angela; Butrynski, James E.; Raut, Chandrajit P.

doi:10.2214/ajr.11.6657

Cited by 140 publications

(175 citation statements)

References 15 publications

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“…It infiltrates adjacent tissues and may lead to symptoms due to deformation and organ dysfunction. The management of desmoid tumors is controversial and the optimal therapy is still not clear (3,5,7).…”

Section: Discussionmentioning

confidence: 99%

“…It is a rare non-malignant soft tissue neoplasm. (2,3). The etiology of the disease is unknown, but trauma, postsurgical scar, endocrine and genetic causes are being considered as possible risk factors for the development of desmoid tumors (3,4).…”

mentioning

confidence: 99%

“…(2,3). The etiology of the disease is unknown, but trauma, postsurgical scar, endocrine and genetic causes are being considered as possible risk factors for the development of desmoid tumors (3,4). Desmoid tumors have a wide spectrum of heterogeneity in clinical attitude, but they usually have an infiltrative growth and locally aggressive behavioral pattern, and do not distantly metastasize (2,5,6).…”

mentioning

confidence: 99%

See 2 more Smart Citations

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results

Ergen¹,

Tiken²,

Öksüz³

et al. 2016

Balkan Med J

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Background: Desmoid tumors are uncommon and benign mesenchymal neoplasms. The optimal treatment of patients with desmoid tumors is still controversial. Surgery is the primary treatment for locally invasive or recurrent desmoid tumors. Also, radiotherapy is a treatment option for patients at high risk for local failure such as those with positive margins or recurrent and unresectable tumors. Aims: To report our institutional experience and longterm results of patients with desmoid tumors who received radiotherapy. Study Design: Retrospective cross-sectional study. Methods: Between 1980 and 2009, 20 patients who received radiotherapy (RT) in our institution were analyzed. The majority of patients (80%) were referred with a recurrent tumor after previous surgery. Thirteen patients underwent marginal resection, 4 had wide local excision and 3 patients had only biopsy. Resection margin was positive in 15 (75%) patients. All patients received radiation therapy. The median prescribed dose was 60 Gy. Five patients received less than 54 Gy. Results: The median follow-up time was 77.5 months (28-283 months). Nine patients developed local recurrence after RT. Seven local failures (78%) were in field. Time to local recurrence ranged from 3-165 months (median 33 months). The 2-5 year local control (LC) rates were 80% and 69%, respectively. On univariate analysis, the 5 year local control rate was significantly better in the patients treated with ≥54 Gy than in patients who received <54 Gy (p=0.023). The most common acute side effect was grade 1-2 skin toxicity. As a late side effect of radiotherapy, soft tissue fibrosis was detected in 10 patients and lymphangitis was seen in 1 patient. One patient developed radiation-induced sarcoma. Conclusion: According to our results, radiotherapy is especially effective in recurrent disease and provides a high local control rate in the patients received more than 54 Gy.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

See 1 more Smart Citation

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results

Ergen¹,

Tiken²,

Öksüz³

et al. 2016

Balkan Med J

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“…Desmoid tümörler vücuttaki tüm muskuloaponörotik dokulardan gelişebilen benign histolojik karakterde, oldukça nadir görülen fibröz tümörlerdir (1) . Metastatik potansiyelleri olmamasına karşın, cerrahi olarak tam çıkarılsa dahi, lokal olarak tekrarlama riski bulunan lezyonlardır (1,2) .…”

Section: Introductionunclassified

“…Metastatik potansiyelleri olmamasına karşın, cerrahi olarak tam çıkarılsa dahi, lokal olarak tekrarlama riski bulunan lezyonlardır (1,2) . Histolojik olarak benign karakterlerde olmalarına rağmen hayati organ ve dokuları infiltre ederek ölüme yol açabilmektedirler (1) .…”

Section: Introductionunclassified

Abdominal desmoid tumor diagnosed during cesarean section

Üstünyurt¹,

Türkay²,

Ölmez³

et al. 2014

tjod

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Desmoid tümörler metastatik potansiyeli bulunmayan, lokal olarak invazif özellikli, gebelikle nadiren ilişkili olan, yavaş büyüyen benign fibroblastik neoplazmlardır. Bu olgu sunumunda sezaryen esnasında saptanan bir abdominal desmoid tümör olgusunu sunuyoruz. Bu olgu dirençli ağrı semptomları olan gebelerde ayrıntılı değerlendirmenin önemini vurgulamaktadır. J Turk Soc Obstet Gynecol 2014;2:134-36 Anahtar Kelimeler: Desmoid tümör, gebelik, tedavi Desmoid tumors are slowly growing benign fibroblastic neoplasms, with no metastatic potential and local invasive features, which have rarely been described to be pregnancy associated. In this case report we present a case of abdominal desmoid tumor, which was diagnosed during cesarean section. This case emphasizes the importance of detailed examination in pregnant women with refractory pain symptoms. J Turk Soc Obstet Gynecol 2014;2:134-36

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Evolving strategies for management of desmoid tumor

Riedel

Agulnik

2022

Cancer

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Desmoid tumors (DTs) are rare soft tissue mesenchymal neoplasms that may be associated with impairments, disfigurement, morbidity, and (rarely) mortality. DT disease course can be unpredictable. Most DTs are sporadic, harboring somatic mutations in the gene that encodes for βcatenin, whereas DTs occurring in patients with familial adenomatous polyposis have germline mutations in the APC gene, which encodes for a protein regulator of βcatenin. Pathology review by an expert soft tissue pathologist is critical in making a diagnosis. Magnetic resonance imaging is preferred for most anatomic locations. Surgery, once the standard of care for initial treatment of DT, is associated with a significant risk of recurrence as well as avoidable morbidity because spontaneous regressions are known to occur without treatment. Consequently, active surveillance in conjunction with pain management is now recommended for most patients. Systemic medical treatment of DT has evolved beyond the use of hormone therapy, which is no longer routinely recommended. Current options for medical management include tyrosine kinase inhibitors as well as more conventional cytotoxic chemotherapy (e.g., anthracyclinebased or methotrexate-based regimens). A newer class of agents, γsecretase inhibitors, appears promising, including in patients who fail other therapies, but confirmation in Phase 3 trials is needed. In summary, DTs present challenges to physicians in diagnosis and prognosis, as well as in determining treatment initiation, type, duration, and sequence. Accordingly, evaluation by a multidisciplinary team with expertise in DT and patient-tailored management are essential. As management strategies continue to evolve, further studies will help clarify these issues and optimize outcomes for patients.

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A to Z of Desmoid Tumors

Cited by 140 publications

References 15 publications

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results

Abdominal desmoid tumor diagnosed during cesarean section

Evolving strategies for management of desmoid tumor

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