Volume 27, Issue 2, November 2016, Pages 250–256
Fethi Jebri1, Tarek Damak2, Montassar Ghalleb3, Zied Benzarti4, Maher Slimane5, Riadh Chargui6, Tarek Dhieb7, Monia Hechiche8, and Khaled Rahal9
1 Surgical oncology Department, Salah Azeiez Institute of oncology, Tunis, Tunisia
2 Service de chirurgie carcinologique, Institut Salah Azaiez, Boulevard 9 Avril, 1006 Tunis, Tunisia
3 Service de chirurgie carcinologique, Institut Salah Azaeiz, Tunis, Tunisia
4 Service de chirurgie carcinologique, Institut Salah Azaeiz, Tunis, Tunisia
5 Service de chirurgie carcinologique, Institut Salah Azaiez, Boulevard 9 Avril, 1006 Tunis, Tunisia
6 Service de chirurgie carcinologique, Institut Salah Azaiez, Boulevard 9 Avril, 1006 Tunis, Tunisia
7 Surgical oncology Department, Salah Azeiez Institute of oncology, Tunis, Tunisia
8 Service de chirurgie carcinologique, Institut Salah Azaiez, Boulevard 9 Avril, 1006 Tunis, Tunisia
9 Service de chirurgie carcinologique, Institut Salah Azaiez, Boulevard 9 Avril, 1006 Tunis, Tunisia
Original language: English
Copyright © 2016 ISSR Journals. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Background: The dermatofibrosarcoma protuberans is a rare skin tumor with a low grade malignancy. It is characterized by a random and asymmetric local extension and a spontaneous tendency to local recurrence. This characteristic is the main difficulty in the management of dermatofibrosarcoma protuberans. The aim of our study was to evaluate our experience in the management of this disease and to clarify the epidemiological, clinical, therapeutic and prognostic characteristics of this rare tumor. Methods: This was a retrospective, single-center, covering 120 cases of dermatofibrosarcoma protuberans treated at the Institute Salah Azaiez, over a period of 21 years from 1 January 1992 to 31 December 2012. Results: The average age at diagnosis was 41.5 years, ranging from 7 to 82 years. . Our population was consisted of 51 women and 69 men. The chief complaint most frequently found was the rapid growth of a nodule (39.2% of cases). The average consultation time was 51 months. The predilection was the trunk in 71 patients (59%) and members in 42 patients (35%). The average lesion size was 6 cm, with a range of 1 to 20 cm. The most common appearance was an isolated nodule in 60% of cases. Ultrasound soft tissue was done in 15 cases (12.5%). Therapeutically, our patients underwent a wide excision in 118 cases and adjuvant radiotherapy in two cases. We observed nine cases of recurrence after a median follow up of 29 months. We observed ten cases of sarcomatous transformation six of which have developed lethal metastases. Conclusion: The prognosis of this disease depends on the surgery performed in first intention which must be complete from the start. Any inappropriate surgery increases the risk of recurrence, sarcomatous degeneration and metastasis.
Author Keywords: Dermatofibrosarcoma protuberans, local malignancy, surgery, recurrence, prognosis.
Fethi Jebri1, Tarek Damak2, Montassar Ghalleb3, Zied Benzarti4, Maher Slimane5, Riadh Chargui6, Tarek Dhieb7, Monia Hechiche8, and Khaled Rahal9
1 Surgical oncology Department, Salah Azeiez Institute of oncology, Tunis, Tunisia
2 Service de chirurgie carcinologique, Institut Salah Azaiez, Boulevard 9 Avril, 1006 Tunis, Tunisia
3 Service de chirurgie carcinologique, Institut Salah Azaeiz, Tunis, Tunisia
4 Service de chirurgie carcinologique, Institut Salah Azaeiz, Tunis, Tunisia
5 Service de chirurgie carcinologique, Institut Salah Azaiez, Boulevard 9 Avril, 1006 Tunis, Tunisia
6 Service de chirurgie carcinologique, Institut Salah Azaiez, Boulevard 9 Avril, 1006 Tunis, Tunisia
7 Surgical oncology Department, Salah Azeiez Institute of oncology, Tunis, Tunisia
8 Service de chirurgie carcinologique, Institut Salah Azaiez, Boulevard 9 Avril, 1006 Tunis, Tunisia
9 Service de chirurgie carcinologique, Institut Salah Azaiez, Boulevard 9 Avril, 1006 Tunis, Tunisia
Original language: English
Copyright © 2016 ISSR Journals. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Background: The dermatofibrosarcoma protuberans is a rare skin tumor with a low grade malignancy. It is characterized by a random and asymmetric local extension and a spontaneous tendency to local recurrence. This characteristic is the main difficulty in the management of dermatofibrosarcoma protuberans. The aim of our study was to evaluate our experience in the management of this disease and to clarify the epidemiological, clinical, therapeutic and prognostic characteristics of this rare tumor. Methods: This was a retrospective, single-center, covering 120 cases of dermatofibrosarcoma protuberans treated at the Institute Salah Azaiez, over a period of 21 years from 1 January 1992 to 31 December 2012. Results: The average age at diagnosis was 41.5 years, ranging from 7 to 82 years. . Our population was consisted of 51 women and 69 men. The chief complaint most frequently found was the rapid growth of a nodule (39.2% of cases). The average consultation time was 51 months. The predilection was the trunk in 71 patients (59%) and members in 42 patients (35%). The average lesion size was 6 cm, with a range of 1 to 20 cm. The most common appearance was an isolated nodule in 60% of cases. Ultrasound soft tissue was done in 15 cases (12.5%). Therapeutically, our patients underwent a wide excision in 118 cases and adjuvant radiotherapy in two cases. We observed nine cases of recurrence after a median follow up of 29 months. We observed ten cases of sarcomatous transformation six of which have developed lethal metastases. Conclusion: The prognosis of this disease depends on the surgery performed in first intention which must be complete from the start. Any inappropriate surgery increases the risk of recurrence, sarcomatous degeneration and metastasis.
Author Keywords: Dermatofibrosarcoma protuberans, local malignancy, surgery, recurrence, prognosis.
How to Cite this Article
Fethi Jebri, Tarek Damak, Montassar Ghalleb, Zied Benzarti, Maher Slimane, Riadh Chargui, Tarek Dhieb, Monia Hechiche, and Khaled Rahal, “DermatoFibrosarcoma Protuberans - A rare dermatologic malignancy: 120 case reports,” International Journal of Innovation and Scientific Research, vol. 27, no. 2, pp. 250–256, November 2016.
