open access

Vol 6, No 3 (2010)
Case report
Published online: 2010-08-27
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Giant, transformed dermatofibrosarcoma protuberans in female patient with newly diagnosed type 1 neurofibromatosis — a case report

Bartosz Wilk, Sebastian Ochenduszko, Joanna Dąbrowska
Onkol. Prak. Klin 2010;6(3):116-119.

open access

Vol 6, No 3 (2010)
CASE REPORTS
Published online: 2010-08-27

Abstract

Dermatofibrosarcoma protuberans (DFS) is a rare, cutaneous, locoregionally aggressive malignancy with a low risk of distant metastases. Neurofibromatosis type 1 (NF1, von Recklinghausen disease) is a genetical predisposition to the development of malignant tumors. The presented case describes atypical coincidence of DFS and NF1 in 30 year old female. The giant tumor was localized on the left side of the neck. It was non-radically excised. The pathological report described dermatofibrosarcoma protuberans progressing into epithelioid sclerosing fibrosarcoma. Adjuvant sequential chemotherapy and radiotherapy was planned. After 3 cycles of doxorubicin, the chest X-ray revealed pulmonary metastases; the radical therapy was stopped with no subsequent radiotherapy. Systemic treatment with ifosfamide was started. After 6 cycles the progression was shown. Considering the agressiveness of the disease and the patient’s excellent performance status, the next line of chemotherapy was started with doxorubicin and dacarbazine.
According to our knowledge, there is only one report of coincidence of DFS and NF1, but without histological progression into more aggressive type of sarcoma.
Onkol. Prak. Klin. 2010; 6, 3: 116–119

Abstract

Dermatofibrosarcoma protuberans (DFS) is a rare, cutaneous, locoregionally aggressive malignancy with a low risk of distant metastases. Neurofibromatosis type 1 (NF1, von Recklinghausen disease) is a genetical predisposition to the development of malignant tumors. The presented case describes atypical coincidence of DFS and NF1 in 30 year old female. The giant tumor was localized on the left side of the neck. It was non-radically excised. The pathological report described dermatofibrosarcoma protuberans progressing into epithelioid sclerosing fibrosarcoma. Adjuvant sequential chemotherapy and radiotherapy was planned. After 3 cycles of doxorubicin, the chest X-ray revealed pulmonary metastases; the radical therapy was stopped with no subsequent radiotherapy. Systemic treatment with ifosfamide was started. After 6 cycles the progression was shown. Considering the agressiveness of the disease and the patient’s excellent performance status, the next line of chemotherapy was started with doxorubicin and dacarbazine.
According to our knowledge, there is only one report of coincidence of DFS and NF1, but without histological progression into more aggressive type of sarcoma.
Onkol. Prak. Klin. 2010; 6, 3: 116–119
Get Citation

Keywords

dermatofibrosarcoma protuberans; neurofibromatosis type I (von Recklinghausen disease); distant metastases; chemotherapy; radiotherapy

About this article
Title

Giant, transformed dermatofibrosarcoma protuberans in female patient with newly diagnosed type 1 neurofibromatosis — a case report

Journal

Oncology in Clinical Practice

Issue

Vol 6, No 3 (2010)

Article type

Case report

Pages

116-119

Published online

2010-08-27

Bibliographic record

Onkol. Prak. Klin 2010;6(3):116-119.

Keywords

dermatofibrosarcoma protuberans
neurofibromatosis type I (von Recklinghausen disease)
distant metastases
chemotherapy
radiotherapy

Authors

Bartosz Wilk
Sebastian Ochenduszko
Joanna Dąbrowska

References (16)
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