Recurrent dermatofibrosarcoma protuberans treated with neoadjuvant imatinib mesylate followed by Mohs micrographic surgery

Fontecilla, Natalia M.; Kittler, Nicole W.; Geskin, Larisa J.; Niedt, George W.; Imahiyerobo, Thomas; Schwartz, Gary N.; Ingham, Matt; Lewin, John M.

doi:10.1016/j.jdcr.2017.06.019

Cited by 10 publications

(9 citation statements)

References 9 publications

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“…Surgical treatment for DSFP is wide local excision and Mohs micrographic surgery. Different recurrence rates are reported for both procedures (2,6). Surgical excision with a margin of at least 2 to 3 cm is recommended for treatment because the local recurrence rate is 20% to 50% in cases of incomplete resection (7).…”

Section: Discussionmentioning

confidence: 99%

“…In 90 % of cases, there is translocation between 17. and 22. chromosomes causing overproduction of platelet derived growth factor β (PDGF β) and stimulating DFSP cell growth (9). This started the use of imitanib, C-kit and PDGF-R inhibitor adjuvant and neoadjuvant therapies for DFSP (6). We vigorously recommended the patient medical oncology consultation, but she did not admit.…”

Section: Discussionmentioning

confidence: 99%

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Dermatofibrosarcoma Protuberans: A Rare Cause of Giant Breast Mass

2019

GMJ

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Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor of dermis and subcutaneous tissue, mostly seen in trunk and extremities. Here we present a case of DFSP in the breast of a young women. A 26 years old female admitted to hospital with a 7 cm painless mass at right breast. Trucut biopsy reported spindle cell tumor. PET/CT and thorax CT revealed no metastases. Breast conserving surgery and sentinel lymph node biopsy was done. Pathological examination report was DFSP and reactive lymphadenopathy. Due to high recurrence rates in DFSP cases, wide local excision for acceptable oncologic margins and follow up is necessary.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Dermatofibrosarcoma Protuberans: A Rare Cause of Giant Breast Mass

2019

GMJ

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“…Neoadjuvant therapy with imatinib mesylate aims to reduce the size or extent of the tumour before using radical treatment intervention, surgery. This drug is given for at least 6 weeks prior excision of 24,30,31 the tumour.…”

Section: Discussionmentioning

confidence: 99%

Dermatofibrosarcoma Protruberans A Locally Aggressive And Recurent Malignant Tumour Of The Skin: Review And Case Report

Malumani

Quan

et al. 2018

MJZ

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Dermatofibrosarcoma protuberans (DFSP) is uncommon but represents one of the most common dermal sarcomas, which is said to be a locally aggressive, low grade, and relatively uncommon cutaneous tumour, which has a high propensity for local relapse with low metastatic potential. Over 90% DFSPs are genetically characterized by chromosomal rearrangements (translocation t(17;22) (q22; q13), resulting in the collagen type-1 alpha 1 (COL1A1)-platelet-derived growth factor â (PDGFB) fusion gene with well-known risk factors for developing DFSP, some cases develop at the site of previous trauma and reports have included a burn scar and the site of vaccination. The mainstay mode of treatment of DFSPs is wide local excision with traditionally 3-cm gross margins excision surgery. Mohs Micrographic Surgery (MMS) is highly recommended surgery for DFSP and is very useful treatment choice for recurrent DFSP. This case report discusses two patients with confirmed DFSP and have received adequate therapy. It also reviews salient features of evaluation and prompt management of DFSP and much emphasis on long-term follow-up of patients. Key Messages: Early diagnosis of pediatric DFSP is critical to minimize surgical disfiguration. We emphasize early evaluation of any suspicious skin lesion should not be ignored and should be excised and sent for histopathology. Undertreating a DFSP is even more problematic, as the tumour is locally destructive, can metastasize, and can prove fatal without treatment. A full metastatic workup should be done and patient should be regularly followed up, if it turns out to be malignant.

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“…4,7,8 As a consequence, the era of systemic therapy has begun for DFSP with Imatinib being the first successful systemic therapy. 8,[16][17][18][19] Radiotherapy details…”

Section: Clinicopathologic Featuresmentioning

confidence: 99%

Dermatofibrosarcoma protuberans – the impact of radiation therapy: a single institution series

et al. 2018

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BackgroundDermatofibrosarcoma protuberans (DFSP) is a locally aggressive intermediate malignancy.ObjectiveThe purpose of this retrospective analysis is to determine the efficacy of radiation therapy (RT) in local control of DFSP.Patients and methodsThe recurrence-free survival (RFS) for 45 patients treated for DFSP at our institution was estimated and compared between surgery alone and postoperative RT groups.ResultsAge range of the patients were in the third and fourth decades; males:females=2:1; most common site: anterior abdominal wall; tumours >5 cm in size in 75%; low grade in 77·8%; margins positive in 31·8% and <5 mm margins in 45·5%. Two-thirds of patients had at least one recurrence before presentation to our institution. RT dose was >50 Gy in 88% of patients. The patients treated with postoperative RT had poorer prognostic factors compared with surgery alone: they were males (17 versus 13 patients), and presented with high-grade tumours (5 versus 1 patients), multiple recurrences prior to presentation (25 versus 20 patients) and positive or <5 mm margins (22 versus 12 patients). Median follow-up for surgery alone group was 17 (1–152) months and for postoperative RT group, this was 54 (5–121) months. RFS at 5 years was 77·1% for surgery alone and 87·9% for postoperative RT group but was not statistically significant. The median time to recurrence was 4 years.ConclusionRT delays the time to recurrence in DFSP. RT improves the outcome of DFSP for recurrent tumours and with positive margins.

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Recurrent dermatofibrosarcoma protuberans treated with neoadjuvant imatinib mesylate followed by Mohs micrographic surgery

Cited by 10 publications

References 9 publications

Dermatofibrosarcoma Protuberans: A Rare Cause of Giant Breast Mass

Dermatofibrosarcoma Protuberans: A Rare Cause of Giant Breast Mass

Dermatofibrosarcoma Protruberans A Locally Aggressive And Recurent Malignant Tumour Of The Skin: Review And Case Report

Dermatofibrosarcoma protuberans – the impact of radiation therapy: a single institution series

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