Skip to main page content
U.S. flag

An official website of the United States government

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2017 May;6(5):665-668.
doi: 10.3892/mco.2017.1197. Epub 2017 Mar 17.

Programmed death-ligand 1 expression is associated with fibrosarcomatous transformation of dermatofibrosarcoma protuberans

Kenji Tsuchihashi  1 , Hitoshi Kusaba  1 , Yuichi Yamada  2 , Yuta Okumura  1 , Hozumi Shimokawa  3 , Masato Komoda  4 , Keita Uchino  3 , Tomoyasu Yoshihiro  1 , Nobuhiro Tsuruta  1 , Fumiyasu Hanamura  1 , Kyoko Inadomi  1 , Mamoru Ito  1 , Kosuke Sagara  1 , Michitaka Nakano  1 , Kenta Nio  1 , Shuji Arita  5 , Hiroshi Ariyama  1 , Kenichi Kohashi  2 , Ryuji Tominaga  6 , Yoshinao Oda  2 , Koichi Akashi  1 , Eishi Baba  5
Affiliations

Programmed death-ligand 1 expression is associated with fibrosarcomatous transformation of dermatofibrosarcoma protuberans

Kenji Tsuchihashi et al. Mol Clin Oncol. 2017 May.

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a locally invading tumor, characterized by the presence of the collagen type I α 1 (COL1A1)-platelet-derived growth factor (PDGF) β fusion gene. We herein report the case of a 31-year-old man with a history of resection of an abdominal wall DFSP. The patient presented with chest pain and a computed tomography scan revealed a large mass in the posterior mediastinum and another mass in the right lung. The mediastinal mass was a sarcomatous lesion expressing the COL1A1-PDGFβ fusion gene, suggesting that it represented a metastasis of the DFSP following fibrosarcomatous (FS) transformation. Following resection of the mediastinal metastasis and subsequent radiotherapy, the mass in the right lung was also resected. Due to the emergence of pleural and pancreatic tail metastases, the patient was treated with a combination therapy of adriamycin and ifosfamide. After five courses, the disease progressed and the patient was subsequently treated with pazopanib for ~2 months until further progression. Three years after the diagnosis of the mediastinal metastasis of DFSP, the patient was referred to another hospital for palliative care. The expression of programmed cell death 1 ligand (PD-L1) in the primary and metastatic tumors was investigated: PD-L1 expression was detected in the metastasis but not in the primary tumor. Given that the metastatic tumor exhibited FS transformation (DFSP-FS), PD-L1 expression may be induced by FS transformation, contributing to the metastasis through escape from immune surveillance. Further investigation of the PD-L1 pathway in DFSP and DFSP-FS in primary as well as metastatic sites is required to evaluate the clinical efficacy of therapies targeting the PD-L1 signaling cascade.

Keywords: dermatofibrosarcoma protuberans; metastasis; programmed death-ligand 1; sarcomatous transformation.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Computed tomography scan showing (A) the posterior mediastinal tumor and (B) the tumor in the right lung.
Figure 2.
Figure 2.
Hematoxylin and eosin staining of (A) the primary tumor and (B) the posterior mediastinal metastasis (magnification, ×200).
Figure 3.
Figure 3.
Programmed death-ligand 1 expression analysis in the primary and metastatic sites using (A and B) clone E1L3N (Cell Signaling Technology), (C and D) clone SP142 (Spring Bioscience) and (E and F) clone 28–8 (Abcam) antibodies, respectively.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Hoesly PM, Lowe GC, Lohse CM, Brewer JD, Lehman JS. Prognostic impact of fibrosarcomatous transformation in dermatofibrosarcoma protuberans: A cohort study. J Am Acad Dermatol. 2015;72:419–425. doi: 10.1016/j.jaad.2014.11.020. - DOI - PubMed
    1. Shimizu A, O'Brien KP, Sjöblom T, Pietras K, Buchdunger E, Collins VP, Heldin CH, Dumanski JP, Ostman A. The dermatofibrosarcoma protuberans-associated collagen type Ialpha1/platelet-derived growth factor (PDGF) B-chain fusion gene generates a transforming protein that is processed to functional PDGF-BB. Cancer Res. 1999;59:3719–3723. - PubMed
    1. Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol. 2014;71:781–786. doi: 10.1016/j.jaad.2014.03.018. - DOI - PubMed
    1. Llombart B, Monteagudo C, Sanmartín O, López-Guerrero JA, Serra-Guillén C, Poveda A, Jorda E, Fernandez-Serra A, Pellín A, Guillén C, et al. Dermatofibrosarcoma protuberans: A clinicopathological, immunohistochemical, genetic (COL1A1-PDGFB), and therapeutic study of low-grade versus high-grade (fibrosarcomatous) tumors. J Am Acad Dermatol. 2011;65:564–575. doi: 10.1016/j.jaad.2010.06.020. - DOI - PubMed
    1. Stacchiotti S, Astolfi A, Gronchi A, Fontana A, Pantaleo MA, Negri T, Brenca M, Tazzari M, Urbini M, Indio V, et al. Evolution of Dermatofibrosarcoma protuberans (DFSP) to DFSP-derived fibrosarcoma (FS-DFSP): An event marked by epithelial mesenchymal transition-like process and 22q loss. Mol Cancer Res. 2016;14:820–829. doi: 10.1158/1541-7786.MCR-16-0068. - DOI - PubMed