Dermatofibrosarcoma protuberans: surgical management of a challenging mesenchymal tumor

Abstract

Introduction Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant mesenchymal tumor of the soft tissue, characterized by slow infiltrative growth and common local recurrence, with rare distant metastases. Patients and methods We present a retrospective study of nineteen patients who were diagnosed with DFSP and operated at our institution in > 10-year period. We examined the clinicopathological parameters with special emphasis on the margin status regarding the clinical outcome and the follow-up. Results A total of eight cases underwent re-excision at our institution following primary excision or incisional biopsy performed at a different institution. Seven cases received excision after incisional biopsy at our institution. Four patients developed recurrent disease following primary excision with histological R0 margins at other institutions and received re-excision at our institution. All excisions at our institution resulted in R0 margins with no recurrence recorded at last follow-up (6 to 175; mean 84 months). The mean margin for those who received resection at our institution was 1.67 cm. Negative margins upon primary excision were achieved using a mean margin width of 2.04 cm. Most common tumor localization was the trunk (10 cases). Conclusion Awareness of this rare entity is important for a prompt diagnosis and a proper management of the disease. The greatest clinical challenge in the management of DFSP is achieving local control. Complete excision of the tumor with surgical margin widths of at least 2 cm is recommended.

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

“…The value of Mohs surgery can be better clarified considering the high rate of recurrence due to wide local excisions with less than 3 cm of margins. Despite the advantages of wide local excision, including the less required time, the fewer costs, and more comfortable performance, a high rate of recurrence poses patients to undergo further procedures . Monnier et al conducted a study on 66 patients undergoing wide local excision and followed them for the median duration of 9 years.…”

Dermatofibrosarcoma protuberans pigmentado: descripción de un caso pediátrico

“…El tratamiento de elección es la extirpación quirúrgica completa, lo cual está dificultado por el crecimiento asimétrico e infiltrativo del tumor. La cirugía de Mohs es la técnicade elección 22,23 ; en caso de tener que recurrir a cirugía convencional, se deben asegurar márgenes amplios (2 a 4 cm). En nuestro caso, se realizó una variante de la cirugía micrográfica con incisión a 90º, (dado que por definición en esta técnica se practica a 45º), con la intención de asegurar márgenes en todos los planos cutáneos (epidermis, dermis e hipodermis).…”

Congenital giant Bednar tumor in a child: surgical challenges in a young infant: a case report

“…Most of the times it leaves a large defect requiring flap cover. Mohs's micrographic surgery is the alternative treatment of choice, avoiding excessive excision in critical locations like head or hand; however, this may not be readily available [4,5], leaving behind positive margins or base make the disease prone for recurrence. Wide local excision with 2 to 3 cm margin involving visibly uninvolved tissue may be difficult in infants and young children.…”