Late Isolated Regional Lymph Node Recurrence of Dermatofibrosarcoma Protuberans of the Abdominal Wall

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YK Chik, AKC Leung, KC Ngan

Hong Kong J Radiol 2012;15:181-6

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.





本文報告一名46歲華籍男性,其腹壁隆突性皮膚纖維肉瘤出現罕見的淋巴結復發。首發時患者接受 廣泛局部切除原發病灶。術後第十一個月及兩年後分別有局部復發,兩次均施以廣泛局部切除。第 二次復發後,病人同時接受了腹壁輔助放療。病人首發十七年後出現右腹股溝淋巴結轉移。正子攝 影及電腦斷層攝影均顯示未有其他部位的轉移。由於病人有淋巴腺包膜外侵犯,遂進行右腹股溝淋巴結切除及術後放療。放療後兩個月的跟進顯示病人未有復發。記載有關淋巴結復發的病發率、特 性、治療及結果的文獻中,只有20宗關於隆突性皮膚纖維肉瘤併發淋巴結復發的病例。有重復原位 性復發的病人,其淋巴結轉移的風險會增加。有遠處轉移或未能接受根治性淋巴結切除的病人,其 淋巴結轉移的預後會很差。大部分的病人都因轉移灶而於兩年內死亡。本病例顯示隆突性皮膚纖維 肉瘤的臨床過程可以相當長,所以跟進期內檢查病人的原發及區域淋巴系統非常重要,尤其對於有 多次原位復發的病人。如果沒有遠處轉移,淋巴結轉移的治療方法為根治性淋巴結切除。術後要密 切監察病人是否有遠處轉移。