Extra-Abdominal Desmoid Tumor: Local Recurrence and Treatment Options

Teixeira, Luiz Eduardo Moreira; Arantes, Eugênio Costa; Villela, Rafael Freitas; Soares, Cláudio Beling Gonçalves; Costa, Roberto Bitarães de Carvalho; Andrade, Marco Antônio Percope de

doi:10.1590/1413-785220162403142182

Cited by 8 publications

(9 citation statements)

References 20 publications

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“…A study of 21 patients with DT has shown that gender, age, localization and number of previous recurrences does not affect the recurrence rate of the tumor [11]. We presented a challenging case of DT in which we supported the notion of surgery as treatment of choice, but with functional preservation of anatomical structures.…”

Section: Discussionsupporting

confidence: 61%

“…Patients with DT usually are young females between 15-35 years [11]. Our patient was 36 year old male, and he was younger, compared to patients described in publications with similar localization (lower limbs) of this tumor type [9,10,12,17,18].…”

Section: Discussionmentioning

confidence: 46%

“…The lowest rate of recurrence was after surgical treatment with wide resection of tumor margins. If histopathological findings observe tumor infiltration in resected margins, increased rate of recurrence is expected [1,11]. Sometimes surgical wide resection is not possible (as was in our case), because of difficult anatomic localization and often functionally unacceptable results [3].…”

Section: Discussionmentioning

confidence: 87%

“…The recurrence rate of DT according to Teixeira LE et al is 33%, with 85% presenting in first 18 months after the end of treatment [11]. A study of 21 patients with DT has shown that gender, age, localization and number of previous recurrences does not affect the recurrence rate of the tumor [11].…”

Section: Discussionmentioning

confidence: 99%

“…So far, the literature describes only several cases of anterior lower limb presentation of desmoid tumor, one case presentation in bilateral lower limbs and there are no literature findings of ipsilateral knee and ankle joint presentation (Table 1) [7][8][9][10]. Volume Patients with DT usually are young females between 15-35 years [11]. Genetical predisposing plays an important role in development od DT, and is often associated with the familial adenomatous polyposis and Gradner's syndrome.…”

Section: Introductionmentioning

confidence: 99%

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Limb Salvage Treatment of Large Multicentric Extra-Abdominal Desmoid Tumor of Lower Extremity

2017

JACCOA

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This article presents a case report of large isolated extra-abdominal desmoid tumor on ipsilateral knee and ankle joint, which was successful treated with combination of surgical -limb salvage procedure and oncologist therapy in the form of hormone and anti-infammatory therapy. A 36 years old male comes, five years after primary treatment of tumor mass of the anterior abdominal wall to Department of Orthopedic surgery because he has new two large tumor changes in the front of the right knee (approximate dimensions 26x10x22 cm (CCxLLxAP)) and ankle joint (16 x 5 cm (APxLL)), which have been progressively increased over the time. The optimal management of desmoid tumor has not be established, because this tumor entity is rare and all recommendations for treatment come from the individual cases described in the literature. Combination of surgery, postoperative hormone and anti-inflammatory therapy is best way of treatment for non resectable tumors.

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Section: Discussionsupporting

confidence: 61%

Section: Discussionmentioning

confidence: 46%

Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Limb Salvage Treatment of Large Multicentric Extra-Abdominal Desmoid Tumor of Lower Extremity

2017

JACCOA

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Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study

et al. 2021

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Background Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate. Questions/purpose This paper aimed to (1) to compare the outcomes of DTF after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation. Methods This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). All seven centres involved were tertiary referral centres for soft tissue tumours. Baseline demographic data was collected for all patients as well as data on the diagnosis, tumour characteristics, clinical features, treatment modalities and whether they had any predisposing factors for DTF. Results Three hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3–85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group. Conclusion Local recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle.

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

et al. 2017

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BackgroundBreast fibromatosis is a rare disease characterized by monoclonal fibroblast proliferation. It has no ability to metastasize but has a high local recurrence rate and often infiltrates surrounding tissues. Surgical treatment is the reference, but recently, new targeted therapies have emerged. We report an original case of a patient with breast fibromatosis who received exclusive medical treatment. Our aim was to analyze these treatments based on the clinical and radiological outcome, iatrogenic effects, and pharmacological action.Case presentationWe report the case of a 19-year-old woman who developed a desmoid-type fibromatosis of the lower inner quadrant of the right breast, measuring 50 × 25 mm (i.e., a volume of 27.4 cm3). Initial surgery was not possible because of potential esthetic and functional prejudice. Thus, she had an exclusive medical treatment including several lines: NSAIDs with tamoxifen and triptorelin, followed by sorafenib, then interferon α2b, and finally sunitinib. With tyrosine-kinase inhibitors (TKIs) (sunitinib), a significant partial response was observed (57% reduction of the maximal tumoral volume). For each treatment, we provided the clinical and radiological outcome in association with known pharmacological action.ConclusionsTKI 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. In general, breast fibromatosis should be treated along with oncologist and interventional radiologists in a trans-disciplinary modality, thus offering an adapted treatment for this particular desmoid-type fibromatosis localization.

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Extra-Abdominal Desmoid Tumor: Local Recurrence and Treatment Options

Cited by 8 publications

References 20 publications

Limb Salvage Treatment of Large Multicentric Extra-Abdominal Desmoid Tumor of Lower Extremity

Limb Salvage Treatment of Large Multicentric Extra-Abdominal Desmoid Tumor of Lower Extremity

Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study

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

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