Ghaloo Shayan Khalid, Dhanani Rahim, Pasha Hamdan Ahmed, Awan Mohammad Sohail, Memon Aisha
Aga Khan University Hospital, Karachi.
J Pak Med Assoc. 2019 Jan;69(1):113-115.
In 1924, Darier and Ferrand described Dermatofibrosarcoma Protuberans as a progressive and recurring dermatofibroma. It is a locally aggressive sarcoma originating from dermal and subdermal tissue of the skin. It usually begins as a small plaque that grows over a period and later manifests as multiple small subcutaneous nodules. It is more commonly found in females as compared to males and typically occurs in between 2nd and 5th decades of life. Most frequently involved regions of the body are torso and proximal ends of extremities and very rarely head and neck region is the site of involvement. The mainstay of treatment of this entity is surgery. The rate of recurrence of this disease is very high in about 50% of the cases and it may also express rare distant metastasis. It is a radiosensitive tumour and radiation may play a role in reducing risk of recurrence. We present a case of a 35 years old male with recurrent Dermatofibrosarcoma Protuberans of right parotid gland.
1924年，达里埃和费朗将隆突性皮肤纤维肉瘤描述为一种进行性复发性皮肤纤维瘤。它是一种起源于皮肤真皮和皮下组织的局部侵袭性肉瘤。它通常始于一个小斑块，经过一段时间生长，随后表现为多个小的皮下结节。与男性相比，女性中更常见，通常发生在20至50岁之间。身体最常受累的部位是躯干和四肢近端，头颈部极少受累。该疾病的主要治疗方法是手术。约50%的病例中，这种疾病的复发率非常高，并且也可能出现罕见的远处转移。它是一种对放疗敏感的肿瘤，放疗可能在降低复发风险方面发挥作用。我们报告一例35岁男性右腮腺复发性隆突性皮肤纤维肉瘤的病例。
