Rare giant dermatofibrosarcoma protuberans with uncommon fibrosarcomatous transformation: case report and review of literature

Authors

  • Manisha Aggarwal Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India
  • Dinesh Manchikanti Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India
  • Sunayana Misra Department of Pathology, ABVIMS and Dr. RML Hospital, New Delhi, India
  • Shaji Thomas Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India
  • Ashish Arsia Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India
  • Rahul Pusuluri Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India
  • Sanjay Kumar Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India

DOI:

https://doi.org/10.18203/2349-2902.isj20214018

Keywords:

Dermatofibrosarcoma protuberans, Wide local excision, Recurrence, Adjuvant radiotherapy, Imatinib

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma with aggressive local behavior but with a low metastatic potential. Although slow growing and indolent, they rarely reach huge sizes. Very uncommonly, these locally invasive tumors undergo fibrosarcomatous transformation with a more aggressive clinical course, with higher rate of recurrence risk and distant metastases. A 32-years-old lady, presented with a gradually progressive lump in the upper central back for the past 6 years, with rapid progression in size during the last 6 months. On examination, she had a single lump of size 18×18 cm in the midline of the upper back, with prominent veins over its surface. Magnetic resonance imaging (MRI) showed no connection with the spinal canal and appeared flush with the paraspinal muscles. Core needle biopsy showed DFSP. The patient underwent a wide local excision with split skin grafting. The histopathology now showed a fibrosarcomatous transformation of DFSP. The patient again underwent a wide re-excision with a 3 cm margin. Histology reported no evidence of tumor cells in the specimen. The patient’s postoperative period was uneventful and she was referred for adjuvant radiotherapy. DFSP is a rare, slow-growing malignant fibroblastic mesenchymal skin tumor with low metastatic potential. However, in any patient with long standing DFSP with a recent increase in size, this fibrosarcomatous transformation must be kept in mind as it represents an uncommon form of DFSP that tends to follow a more aggressive clinical course, with higher rate of recurrence risk and distant metastases.

Author Biographies

Manisha Aggarwal, Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India

Junior Resident,

Department of Surgery

Dinesh Manchikanti, Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India

Junior Resident,

Department of Surgery

Sunayana Misra, Department of Pathology, ABVIMS and Dr. RML Hospital, New Delhi, India

Assistant Professor,

Department of Pathology

Shaji Thomas, Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India

Director-Professor of Surgery,

Department of Surgery

Ashish Arsia, Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India

Professor of Surgery,

Department of Surgery

Rahul Pusuluri, Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India

Professor of Surgery,

Department of Surgery

Sanjay Kumar, Department of Surgery, Lady Hardinge Medical College and Dr. RML Hospital, New Delhi, India

Professor of Surgery,

Department of Surgery

References

Kampshoff JL, Cogbill TH. Unusual skin tumors: Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans. Surg Clin North Am. 2009;89(3):727-38.

Mentzel T, Beham A, Katenkamp D, Dei Tos AP, Fletcher CD. Fibrosarcomatous ("high-grade") dermatofibrosarcoma protuberans: clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance. Am J Surg Pathol. 1998;22(5):576-87.

Gloster HM. Dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996;35(3):355-74.

Stivala A, Lombardo GA, Pompili G, Tarico MS, Fraggetta F, Perrotta RE. Dermatofibrosarcoma protuberans: Our experience of 59 cases. Oncol Lett. 2012;4(5):1047-55.

Sanmartín O, Llombart B, López-Guerrero JA, Serra C, Requena C, Guillén C. Dermatofibrosarcoma protuberans [Dermatofibrosarcoma protuberans]. Actas Dermosifiliogr. 2007;98(2):77-87.

Monnier D, Vidal C, Martin L, Danzon A, Pelletier F, Puzenat E, et al. Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002. J Eur Acad Dermatol Venereol. 2006;20(10):1237-42.

Suehara Y, Yazawa Y, Hitachi K. Metastatic Bednar tumor (pigmented dermatofibrosarcoma protuberans) with fibrosarcomatous change: a case report. J Orthop Sci. 2004;9(6):662-5.

Abbott JJ, Oliveira AM, Nascimento AG. The prognostic significance of fibrosarcomatous transformation in dermatofibrosarcoma protuberans. Am J Surg Pathol. 2006;30(4):436-43.

Patel KU, Szabo SS, Hernandez VS, Prieto VG, Abruzzo LV, Lazar AJ, López-Terrada D. Dermatofibrosarcoma protuberans COL1A1-PDGFB fusion is identified in virtually all dermatofibrosarcoma protuberans cases when investigated by newly developed multiplex reverse transcription polymerase chain reaction and fluorescence in situ hybridization assays. Hum Pathol. 2008;39(2):184-93.

Llombart B, Sanmartín O, López-Guerrero JA, Monteagudo C, Serra C, Requena C, et al. Dermatofibrosarcoma protuberans: clinical, pathological, and genetic (COL1A1-PDGFB) study with therapeutic implications. Histopathology. 2009;54(7):860-72.

Sundram UN. Review: Dermatofibrosarcoma protuberans: histologic approach and updated treatment recommendations. Clin Adv Hematol Oncol. 2009;7(6):406-8.

Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F. WHO Classification of Tumors of Soft tissue and Bone. IARC, Lyon. 2013:77-9.

Llombart B, Serra-Guillén C, Monteagudo C, López Guerrero JA, Sanmartín O. Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. Semin Diagn Pathol. 2013;30(1):13-28.

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(4):781-6.

Lemm D, Mügge LO, Mentzel T, Höffken K. Current treatment options in dermatofibrosarcoma protuberans. J Cancer Res Clin Oncol. 2009;135(5):653-65.

Eguzo K, Camazine B, Milner D. Giant dermatofibrosarcoma protuberans of the face and scalp: a case report. Int J Dermatol. 2014;53(6):767-72.

Nouri K, Lodha R, Jimenez G, Robins P. Mohs micrographic surgery for dermatofibrosarcoma protuberans: University of Miami and NYU experience. Dermatol Surg. 2002;28(11):1060-4.

Snow SN, Gordon EM, Larson PO, Bagheri MM, Bentz ML, Sable DB. Dermatofibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature. Cancer. 2004;101(1):28-38.

Fiore M, Miceli R, Mussi C, Lo Vullo S, Mariani L, Lozza L, et al. Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. J Clin Oncol. 2005;23(30):7669-75.

Mendenhall WM, Zlotecki RA, Scarborough MT. Dermatofibrosarcoma protuberans. Cancer. 2004;101(11):2503-8.

Dagan R, Morris CG, Zlotecki RA, Scarborough MT, Mendenhall WM. Radiotherapy in the treatment of dermatofibrosarcoma protuberans. Am J Clin Oncol. 2005;28(6):537-9.

Ballo MT, Zagars GK, Pisters P, Pollack A. The role of radiation therapy in the management of dermatofibrosarcoma protuberans. Int J Radiat Oncol Biol Phys. 1998;40(4):823-7.

Handolias D, McArthur GA. Imatinib as effective therapy for dermatofibrosarcoma protuberans: proof of concept of the autocrine hypothesis for cancer. Future Oncol. 2008;4(2):211-7.

Labropoulos SV, Fletcher JA, Oliveira AM, Papadopoulos S, Razis ED. Sustained complete remission of metastatic dermatofibrosarcoma protuberans with imatinib mesylate. Anticancer Drugs. 2005;16(4):461-6.

Bhambri S, Desai A, Del Rosso JQ, Mobini N. Dermatofibrosarcoma protuberans: a case report and review of the literature. J Clin Aesthet Dermatol. 2008;1(1):34-6.

Gloster HM. Dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996;35(3):355-74.

Downloads

Published

2021-09-28

Issue

Section

Case Reports