Late isolated regional lymph node recurrence of dermatofibrosarcoma protuberans of the abdominal wall

Abstract

This report is of a rare occurrence of lymph node relapse in a 46-year-old Chinese man with dermatofibrosarcoma protuberans of the abdominal wall. At initial presentation, the patient underwent wide local excision of the primary lesion. He developed local recurrences twice, at 11 months and 2 years after diagnosis, both of which were treated with wide local excision. Adjuvant radiotherapy was given to the abdominal wall after his second relapse. Subsequently he developed right groin lymph node metastasis 17 years after the initial presentation. There was no evidence of other sites of metastasis on positron emission tomography / computed tomography scan. He underwent right groin lymph node dissection followed by postoperative radiotherapy because of the presence of extracapsular extension of the nodal metastasis. He was disease-free at the last follow-up visit two months after completion of radiotherapy. Literature review for the incidence, characteristics, treatment, and outcome of lymph node relapse showed less than 20 reported cases of dermatofibrosarcoma protuberans with lymph node metastasis. Patients with repeated local recurrences were found to be at higher risk for lymph node metastasis. The prognosis for lymph node metastasis was poor for patients with concomitant distant metastasis or those in whom radical lymph node dissection was not feasible, with most patients dying of metastatic disease within 2 years. This case illustrates that the clinical course of dermatofibrosarcoma protuberans could be long, and it is important to examine the regional lymphatic system in addition to the primary site during follow-up, especially for patients with repeated local recurrences. When there is no distant metastasis, the treatment of choice for lymph node metastasis is radical lymph node dissection. Close surveillance for subsequent distant metastasis after the regional treatment is warranted. © 2012 Hong Kong College of Radiologists.