Incidence and Outcomes of Dermatofibrosarcoma Protuberans in the US Pediatric Population

Rubio, Gustavo A.; Alvarado, Andrea; Gerth, David J.; Tashiro, Jun; Thaller, Seth R.

doi:10.1097/scs.0000000000003203

Cited by 30 publications

(45 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Other affected areas include the proximal extremities and less commonly the head and neck . Visceral or lymph node metastasis is rare despite the locally aggressive behavior of the tumor …”

Section: Discussionmentioning

confidence: 99%

“…This oral medication inhibits platelet‐derived growth factor receptor and may be used in the future as neoadjuvant chemotherapy for DFSP . DFSP has one of the most favorable overall prognoses among soft tissue sarcomas . Criscione and Weinstock found the relative 5‐ and 15‐year prognoses to be 99.2% and 97.2%, respectively …”

Section: Discussionmentioning

confidence: 99%

“…4 Delayed diagnosis is common in this rare condition, resulting in large tumors that can complicate surgical management. 5 Mohs micrographic surgery (MMS) and wide local excision (WLE) with 2-4 cm margins have been recommended for these patients. 5 Significantly lower recurrence rates have been seen in patients who undergo MMS compared to WLE.…”

Section: Discussionmentioning

confidence: 99%

“…5 Mohs micrographic surgery (MMS) and wide local excision (WLE) with 2-4 cm margins have been recommended for these patients. 5 Significantly lower recurrence rates have been seen in patients who undergo MMS compared to WLE. 9 Most recurrences after local resection occur within 3 years but recurrence after more than 10 years has been reported.…”

Section: Discussionmentioning

confidence: 99%

“…4 Visceral or lymph node metastasis is rare despite the locally aggressive behavior of the tumor. 5 Histologically, DFSP arises in the dermis and penetrates the subcutis creating a characteristic honeycomb appearance. 3 Other histologic findings include dense, monomorphic cells containing spindle-shaped cells in a storiform pattern, with limited mitotic figures.…”

Section: Histopath Ologymentioning

confidence: 99%

See 4 more Smart Citations

Enlarging lesion on the back of a 17‐year‐old boy

Glade

Gillooly²,

Browning³

2019

Pediatric Dermatology

View full text Add to dashboard Cite

“…Other affected areas include the proximal extremities and less commonly the head and neck . Visceral or lymph node metastasis is rare despite the locally aggressive behavior of the tumor …”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Histopath Ologymentioning

confidence: 99%

See 3 more Smart Citations

Enlarging lesion on the back of a 17‐year‐old boy

Glade

Gillooly²,

Browning³

2019

Pediatric Dermatology

View full text Add to dashboard Cite

Dermatofibrosarcoma protuberans misdiagnosed as sebaceous adenoma and incompletely resected in a child: A case report

Zhang

Chen

Wang

2022

Clinical Laboratory Analysis

View full text Add to dashboard Cite

BackgroundDermatofibrosarcoma protuberans is extremely rare in children, making a correct diagnosis by clinicians is usually difficult due to its nonspecific manifestations, the recurrence of dermatofibrosarcoma protuberans after resection has always been a perplexing problem for clinicians. Ultrasound plays an irreplaceable role in the assessment of dermatofibrosarcoma protuberans, although there is a limitation in the diagnosis of the tumor.Case ReportA 10‐year‐old boy led by his parents sought for surgical treatment because of the growing mass. Physical examination and preoperative ultrasonography showed that the mass was clear, and the routine resection of mass was performed. Six days postoperatively, histopathological examination indicated that the mass was dermatofibrosarcoma protuberans, and the peripheral and deep resection margins were positive. The patient was informed that a second extended resection was required. The second postoperative pathology showed a negative margin, and the patient was discharged. Postoperative follow‐up was assessed by ultrasound examination, and the patient had no abnormalities.ConclusionsDermatofibrosarcoma protuberans should be included in the differential diagnosis when the wide base subcutaneous lesion has suspicious features with high vascularity on ultrasound. If an ultrasound reveals a mass with abundant blood flow, clinicians should routinely perform the extended resection. Wide surgical excision may reduce the risk of reoperation and recurrence, and long‐term follow‐up is necessary to evaluate postoperative outcomes.

show abstract

Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease—Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS)

Krewer

Rolle

Kościelniak

et al. 2020

Journal of Surgical Oncology

View full text Add to dashboard Cite

Background Dermatofibrosarcoma protuberans (DFSP) is a rare low‐grade tumor. Little is known about best treatment of primary and relapsed disease (RD). Methods Treatment and outcome of 40 patients with DFSP prospectively registered within the CWS‐96 and ‐2002P trials and the registry SoTiSaR (1996‐2016) were analysed. Results Median age was 8 years (range, 0.64‐17.77). Fluorescence in situ hybridization analysis to detect COL1A1‐PDGFB fusion genes was positive in 86% (12/14) of evaluated patients. Primary resection was performed in all patients. Patients had IRS group I (n = 28), II (n = 9), and III (n = 2); not available (n = 1). To achieve complete remission (CR), a secondary resection was performed in 18 patients resulting in microscopically complete (R0, n = 34/40) and microscopically incomplete (R1, n = 5/40) resection. All patients achieved CR. The 5‐year event‐free survival (EFS) and overall survival was 86% (±12; CI, 95%) and 100% (±0; CI, 95%), respectively. R0 resection/IRS I was significantly favorable for the 5‐year EFS. Local relapse occurred after a median time of 1.1 years (range, 0.04‐5.1) in 15% (6/40) after CR. All patients with RD underwent resection and achieved CR. Three patients had fibrosarcomatous DFSP, two were alive after R0 resection. Conclusion Complete surgical resection is mandatory to prevent relapse of DFSP.

show abstract

Incidence and Outcomes of Dermatofibrosarcoma Protuberans in the US Pediatric Population

Abstract: Pediatric DFSP has lower incidence but similar clinical characteristics to adults. Incidence is higher in black children and in the trunk region. While prognosis is favorable, male sex is associated with decreased OS.

Cited by 30 publications

References 22 publications

Enlarging lesion on the back of a 17‐year‐old boy

Enlarging lesion on the back of a 17‐year‐old boy

Dermatofibrosarcoma protuberans misdiagnosed as sebaceous adenoma and incompletely resected in a child: A case report

Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease—Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS)

Contact Info

Product

Resources

About