Extraabdominal Desmoid Tumor

Pignatti, G.; Barbanti-Br??dano, G.; Ferrari, Domenico; Gherlinzoni, Filíppo; Bertoni, Franco; Bacchini, Patrizia; Barbieri, Enza; Giunti, Armando; Campanacci, Mario

doi:10.1097/00003086-200006000-00025

Cited by 113 publications

(80 citation statements)

References 23 publications

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“…A randomized prospective study with long-term follow-up might solve this issue. However, our findings together with case reports on spontaneous regression (Dahn et al 1963, Rock et al 1984) and a report on stable tumors (Pignatti et al 2000) suggest that most desmoid tumors should probably be left alone. Whether tumors may occasionally progress indefinitely is still unclear.…”

Section: Local Recurrence After Surgical Removal Of Desmoid Tumors Issupporting

confidence: 62%

“…For this reason, many alternative treatments have been tried but with varying results. Radiotherapy, alone or given postoperatively, has been associated with high local control rates in some studies (Kiel and Suit 1984, McCollough et al 1991, Karakousis et al 1993, Kamath et al 1996, Nuyttens et al 2000 but not in others (Merchant et al 1999, Pignatti et al 2000. Chemotherapy has also been tried in the treatment of desmoid tumors with variable results (Easter and Halasz 1989, Raney 1991, Patel et al 1993.…”

Section: Extra-abdominal Desmoid Tumorsmentioning

confidence: 99%

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Clinical and imaging observations of desmoid tumors left without treatment

et al. 2006

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Background Until now, surgical treatment has been the mainstay in the treatment of desmoid tumors, even though it is associated with a high recurrence rate. There have, however, been occasional case reports showing that desmoid tumors may spontaneously decrease in size or even disappear.Patients and methods This is a retrospective review of 8 patients with abdominal (5) or extra-abdominal (3) desmoid tumors who were followed both clinically and with imaging techniques (sonography, CT or MRI). Mean follow-up time was 4.4 (0.8-7.5) years. Tumor volume was assessed in each investigation and followed over time.Results 3 tumors disappeared, 2 diminished in size, 1 did not change and 2 tumors became larger, 1 of which had trippled in volume at the latest follow-up.Interpretation Desmoid tumors have probably been overtreated in the past. Many of them tend to regress spontaneously.

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Section: Local Recurrence After Surgical Removal Of Desmoid Tumors Issupporting

confidence: 62%

Section: Extra-abdominal Desmoid Tumorsmentioning

confidence: 99%

Clinical and imaging observations of desmoid tumors left without treatment

et al. 2006

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“…Desmoid tumours are locally aggressive, non-metastasising tumours [9,13,15,22]. Not only are they rare, they also have an unpredictable clinical course, which makes their treatment difficult.…”

Section: Discussionmentioning

confidence: 99%

The management of extra-abdominal desmoid tumours

Duggal

Dickinson

Sommerville

et al. 2004

International Orthopaedics (SICOT)

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We performed a retrospective analysis of 35 cases of desmoid tumours (aggressive fibromatoses) that underwent treatment at our institutions between 1987 and 2002. The purpose was to evaluate the rate of local recurrence of desmoid tumours treated with surgical excision, to assess the impact of surgical margins on local recurrence and to define the role of radiotherapy in the treatment. Nine patients experienced a recurrence at an average of 16 months after initial treatment. Seven of the 15 patients with a less-than-wide margin had a local recurrence. Comparatively, only two of the 20 patients with a wide margin had a local recurrence. Thirty-three of the 35 patients were disease free at the last follow-up. We recommend wide excision with clear margins whenever possible. Marginal resections are appropriate when wide excision would severely compromise the function of the limb. Surgical resections and selective supplementation of adjuvant radiotherapy give excellent control rates.Résumé Nous avons exécuté une analyse rétrospective de 35 cas de tumeurs desmoides (fibromatose agressive) traitées dans notre institution entre 1987 et 2002. Le but était d'évaluer le taux de récidive locale après traitement par excision chirurgicale, d'étudier le rôle des marges chirurgicales sur la récidive et de définir le rôle de la radiothérapie dans le traitement. Neuf malades ont eu une récidive à une moyenne de 16 mois après le traitement initial. Sept des 15 malades opérés avec une marge qui n'était pas large, avait une récidive locale. Comparativement, seulement 2 des 20 malades avec une marge large avaient une récidive locale. Trente trois des 35 malades étaient sans maladie au dernier suivi. Nous recommandons l'excision large avec des marges claires toutes les fois que possible. Les résections marginales sont appropriées quand l'excision large compromettrait sévèrement la fonction du membre. Les résections chirurgicales avec, sélectivement, un traitement adjuvant par radiothérapie donnent d'excellents taux de contrôle de la maladie.

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“…Local control rate of patients with negative margin was better than those with positive margin (8). Zeng et al (9) reported that tumor larger than 5 cm, extra-abdominal tumor and R1 resection status were associated with worse recurrence-free survival. The only factor associated with low recurrence is negative margin (10).…”

Section: Surgerymentioning

confidence: 99%

Efficacy of vinorelbine combined with low-dose methotrexate for treatment of inoperable desmoid tumor and prognostic factor analysis

Fan

Fang

et al. 2017

Chinese Journal of Cancer Research

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Results: Of the 71 patients, 55% were female. Age of onset varied from 1 to 47 years, and the median age was 14 years. Only 11 (15.5%) cases suffered primary tumor. The distribution of the site of tumors was: 31 (43.7%) in the trunk, 36 (50.7%) in the limbs, and 4 (5.6%) in the peritoneal and pelvic cavity. The size of tumor (the maximum diameter) differed from 2 to 37 cm with a mean of 9.3 cm. The median follow-up duration was 28 (range, 6-87) months. Common side effects included: nausea and vomiting, liver injury, bone marrow suppression and oral ulcers.When the chemotherapy finished, 1 (1.4%) case achieved complete response, 24 (33.8%) achieved partial response, 37 (52.1%) achieved stable disease and 9 (12.7%) had progressive disease. The overall response rate was 87.3%.The progression-free survival (PFS) of the participants were from 6 to 87 months, and the 2-, 3-and 5-year PFS was 79.9%, 68.4% and 36.3%, respectively. No significant difference was identified in PFS in subgroups of gender, age of onset, age of chemotherapy, tumor site and tumor size.Conclusions: For recurrent, inoperable and progressive DT, enough course of chemotherapy with vinorelbine combined with low-dose methotrexate was an optional choice for local control.

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Extraabdominal Desmoid Tumor

Cited by 113 publications

References 23 publications

Clinical and imaging observations of desmoid tumors left without treatment

Clinical and imaging observations of desmoid tumors left without treatment

The management of extra-abdominal desmoid tumours

Efficacy of vinorelbine combined with low-dose methotrexate for treatment of inoperable desmoid tumor and prognostic factor analysis

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