Resection and Reconstructive Options in the Management of Dermatofibrosarcoma Protuberans of the Head and Neck.

Aqsa Akhtar,Adeela Hussain Khan,Mamoon Rashid,Haroon Ur Rashid,Farhan Eitezaz

doi:10.7759/cureus.9423

Aqsa Akhtar, Adeela Hussain Khan + Show 3 more

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https://doi.org/10.7759/cureus.9423

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Abstract

ObjectiveTo discuss resection and various reconstructive options in patients with dermatofibrosarcoma protuberans (DFSP).MethodsThis study was conducted at Shifa International Hospital, Islamabad, Pakistan, from May 2018 to December 2019. All patients aged 20 years or above of either gender who were diagnosed to have DFSP over this period were included in the study. All the patients underwent wide local excision of the tumor under general anesthesia. A peroperative frozen section was conducted in all the cases to confirm complete excision. Immediate reconstruction was performed following the tumor excision. The choice of reconstruction, i.e. free, regional, or local flap was based on the size of the resultant defect.ResultsThe mean age of the patients was 37.11 ±10.91 years. There were 12 (66.7%) males and six (33.3%) females. The mean duration of the disease was 11.22 ±2.94 months. The affected anatomical site showed that the face was involved in the majority, nine (50%) patients, followed by the scalp in four (22.2%), nape of the neck in three (16.7%), and supraclavicular region in two (11.1%) patients. In most of the cases, the free flap was observed, i.e. (n=9, 50%), followed by a regional flap in seven (38.9%), and the local flap in two (10.1%) patients.ConclusionWide local excision of the disease, confirmed on frozen section, offers improved survival. Among DFSP of the head and neck, the face was found to be the affected anatomical site in half the cases. Also, reconstruction following tumor excision with a free flap is the most favorable option among patients with DFSP.

Highlights

Dermatofibrosarcoma protuberans (DFSP) is an uncommon tumor of the skin that grows slowly [1]
The affected anatomical site showed that the face was involved in the majority, nine (50%) patients, followed by the scalp in four (22.2%), nape of the neck in three (16.7%), and supraclavicular region in two (11.1%) patients
Radiation therapy has been found to be successful in the treatment of patients with DFSP recently [10]

Summary

Introduction

Dermatofibrosarcoma protuberans (DFSP) is an uncommon tumor of the skin that grows slowly [1]. The tumor was first described by Darier and Ferrand in 1924 [3] It grows slowly over a period of months to years without any definitive symptoms. Delay in diagnosis is not uncommon owing to the benign appearance. It infiltrates into the dermis and subcutaneous tissue but is rarely fixed to the underlying structures [4]. The tumor originates within the dermal layer of the skin and gradually extends into the local tissue to involve the subcutaneous tissue and beyond. The local recurrence rate for patients with DFSP who undergo wide local excision (WLE) of the trunk sites ranges up to 21% [7,8]. Radiation therapy has been found to be successful in the treatment of patients with DFSP recently [10]

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