A Rare Case of Misdiagnosis: Recurrence of Dermatofibrosarcoma Protuberans That Was Treated Surgicallyas a Keloid

Uçak, Murat

doi:10.5455/medarh.2018.72.74-75

Cited by 5 publications

(7 citation statements)

References 7 publications

(9 reference statements)

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“…Usually patients may present rather late when the tumor is already several centimeters in size due to its indolent onset. Misdiagnoses of the tumor for a simple scar, keloid [10], or cyst, lump is often the case [11]. As it has been shown to arise from regions or areas of previous trauma.…”

Section: Introductionmentioning

confidence: 99%

The efficacy of Mohs micrographic surgery over the traditional wide local excision surgery in the cure of dermatofibrosarcoma protuberans

2019

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Usually most patients with dermatofibrosarcoma protuberans (DFSP) may present rather late when the tumor is in protuberant phase due to its rarity and indolent onset. It has a high propensity for local recurrence and destructive nature. Management of DFSP requires a biopsychosocial and Multidisplinary approach regardless of the clinical or immunohistochemical variant. Surgery is the Gold standard management of localized disease. DFSP rarely exhibits any lymphatic or hematogenous dissemination. It is because of its high recurrence rate associated with Wide Local Excision (WLE), the introduction of Mohs micrographic surgery (MMS) has really helped in reducing the rates of recurrence of DFSP. Thus, the aim of this meta-analysis and systemic review is to advocate for MMS over WLE for DFSP and other cutaneous malignancies using DFSP as a prototype. The objective of this study were to conduct a meta-analysis on comparative surgical methods used in the cure of DFSP with regards to WLE verses MMS, to evaluate the cure rates with relation to recurrence rates, offer a recommendation on the various treatment modalities based on the location of lesion, and use of adjuvant therapy in different clinical-medical setups. A comprehensive retrospective analysis search in EMBASE, Google Scholar and Medline (PubMed) for studies published from 2008 to 2018 containing the surgical management of DFSP with WLE verses MMS were reviewed. Five studies of moderate-quality evidence (level B) with a pooled patient load of 684 was analyzed and found for recurrence of DFSP after WLE and MMS to be 9.10% and 2.72% respectively after an average follow-up time for both groups of 5.32 years with a female predominance of 1.58. The trunk is the commonest site for the DFSP lesion which was at 52.80% then the upper and lower extremities zones and the head and neck zones at 31.75% and 15.45% respectively. The pooled adjusted odds ratio (OR) analysis indicated that there was a direct relationship with regards the reduced recurrence rate of DFSP in the MMS group compared to the WLE group (OR:0.31;95%; CI :0.17-0.56). Furthermore, there was significant association between the reduced recurrence rate with the MMS in DFSP patients with a statistical P-value of 0.0001 at 95% CI. The expected increased recurrence rate by zones was in WLE head and neck zone at 38.19% then trunk and extremities zone at 13.34%. In the MMS group it was at of 23.4% as compared to 16.0% in the head and neck zone. Mohs Micrographic Surgery (MMS) is more efficacious in the cure rate and recurrence reduction of DFSP and should be advocated for as first line therapy especially in high recurrence prone zones.

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Section: Introductionmentioning

confidence: 99%

The efficacy of Mohs micrographic surgery over the traditional wide local excision surgery in the cure of dermatofibrosarcoma protuberans

2019

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“…Differential diagnoses of keloids include complications of acne, hyperplastic scars, and keloidal dermatofibroma. DFSP can be misdiagnosed as a keloid scar [3].…”

Section: Discussionmentioning

confidence: 99%

“…Keloid is a benign fibroproliferative disorder of the skin that presents in scar tissue when a wound heals. Although keloid scars can be difficult to be differentiated from dermatofibrosarcoma protuberans (DFSP) [3], there are no reports of UPS development in a keloid scar.…”

Section: Introductionmentioning

confidence: 99%

Giant Protruding High-Grade Undifferentiated Pleomorphic Sarcoma Arising in a Keloid Scar on the Abdominal Wall

Kinoshita

Tsukanishi

Ishii

et al. 2020

Case Reports in Dermatological Medicine

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Undifferentiated pleomorphic sarcoma (UPS) is a high-grade, aggressive, soft tissue sarcoma that is often fatal. Although there are reports describing associations of sarcoma and skin lesions such as burns, radiation, and trauma, to our knowledge, UPS development in a keloid scar has not been reported. Herein, we present the case of a 76-year-old woman who had undergone surgery for endometrial cancer, 5 years before. She presented with a protruding lesion that was continuous to a keloid scar on the abdominal wall. The tumor appeared clinically malignant as it was protruding and doubled in size within three weeks, reaching approximately 6 × 6 × 2 cm. Since the tumor was diagnosed as UPS after pathological evaluation by needle biopsy, wide resection was performed. Intraoperatively, the tumor was apparently continuous to the keloid, protruding and pedunculated outside the body, and had not invaded the abdominal cavity. Histopathological examination of the resected tumor showed evidence of UPS and no suspicion of metastasis of endometrial cancer. No recurrence, metastases, or other complications were noted 6 months after surgery. The current case study reminds us that keloids may cause high-grade sarcoma such as UPS, and careful follow-up is required.

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“…Due to the indolent nature of DFSP onset or misdiagnosis of the tumor, it may be diagnosed late when the tumor is already several centimeters. However, even following many years, the tumor may be asymptomatic …”

Section: Introductionmentioning

confidence: 99%

“…However, even following many years, the tumor may be asymptomatic. 7,8 Studies in the literature have shown the association of DFSP with CD34, and the chromosomal studies have revealed 17:22 translocation causing overexpression of tyrosine kinase PDGFB as the etiology of DFSP. Therefore, imatinib, a tyrosine kinase inhibitor, has been considered as a choice for medical treatment of this sarcoma.…”

Section: Introductionmentioning

confidence: 99%

An experience of slow‐Mohs micrographic surgery for the treatment of Dermatofibrosarcoma protuberans: A long‐term cohort study

Asilian

Honarjou

Faghihi

et al. 2020

J of Cosmetic Dermatology

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Background Dermatofibrosarcoma protuberans (DFSP) is a rare dermal mesenchymal tumor known as a low‐grade, slow‐growing malignancy. The local invasion and high rate of recurrence following surgical treatment are the main concerns to plan the best surgical approach of treatment. Aims In the current study, it is aimed to provide an experience of slow‐Mohs surgery for the treatment of patients with DSFP. Patients/Methods Number of 25 patients with the diagnosis of DFSP through histological and immunostaining study was included. The slow‐Mohs was performed by excision of the tumor with margins accounting for 1–2 cm from both the tumor margins and three‐dimensional thickness. The obtained tissue margins were horizontally, and if any of the specimens was not margin‐free, the procedure was repeated. The patients' opinion about the procedure was assessed using Patient‐Observer Scar Assessment Scale (POSAS). Results Number of 25 patients were included and followed for a median of 46.9 months. The median of the area of excision was 35.56 cm2, and the median clinical excision margins were 19 mm (tumor excision margins + thickness of the three‐dimensional excision). The surgery was performed once for 16 (64%), and postoperative skin closure within 5–7 days after the procedure was performed for 19 (76%) patients. None of the patients represented any recurrence. The patients' overall opinion and satisfaction POSAS score accounted for 2.3 ± 1.65 and 1.6 ± 0.59, respectively. Conclusion The findings of the current study are in favor of slow‐Mohs surgery for the management of DFSP, while more extensive studies are strongly recommended for generalization of this procedure.

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A Rare Case of Misdiagnosis: Recurrence of Dermatofibrosarcoma Protuberans That Was Treated Surgicallyas a Keloid

Cited by 5 publications

References 7 publications

The efficacy of Mohs micrographic surgery over the traditional wide local excision surgery in the cure of dermatofibrosarcoma protuberans

The efficacy of Mohs micrographic surgery over the traditional wide local excision surgery in the cure of dermatofibrosarcoma protuberans

Giant Protruding High-Grade Undifferentiated Pleomorphic Sarcoma Arising in a Keloid Scar on the Abdominal Wall

An experience of slow‐Mohs micrographic surgery for the treatment of Dermatofibrosarcoma protuberans: A long‐term cohort study

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