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Table of Contents
Year : 2019  |  Volume : 2  |  Issue : 2  |  Page : 256-257

Head-and-neck dermatofibrosarcoma protuberans: Scooping out data even in dearth of evidence

Department of Medical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute (HBNI), Mumbai, Maharashtra, India

Date of Web Publication20-Dec-2019

Correspondence Address:
Akhil Kapoor
Department of Medical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra; Homi Bhabha National Institute (HBNI), Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/CRST.CRST_70_19

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How to cite this article:
Kapoor A, Kumar A. Head-and-neck dermatofibrosarcoma protuberans: Scooping out data even in dearth of evidence. Cancer Res Stat Treat 2019;2:256-7

How to cite this URL:
Kapoor A, Kumar A. Head-and-neck dermatofibrosarcoma protuberans: Scooping out data even in dearth of evidence. Cancer Res Stat Treat [serial online] 2019 [cited 2020 Mar 31];2:256-7. Available from: http://www.crstonline.com/text.asp?2019/2/2/256/273685

We read with great interest the article 'Dermatofibrosarcoma protuberans of head and neck: Clinical outcome of nine cases treated with imatinib' published in the last issue of your journal.[1]

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive, cutaneous, malignant tumor characterized by a high propensity for local relapse and low metastatic potential.[2] Although 90% of all DFSPs represent low-grade tumors, 10-15% contain a component of high-grade fibrosarcoma which is characterized by a higher incidence of local relapse and distant metastasis.[2] The accompanying editorial by Pandey and Singh noted that the majority of patients had high-risk features such as over 50% had a tumor size more than 5 cm, with 44% having fibrosarcoma variant and one-third of patients presenting with metastatic disease.[3] The reason for this higher percentage of fibrosarcomatous component (44%) in this study as compared to the usual 10-15% could be the referral bias to the medical oncology department for neoadjuvant or adjuvant therapy.

The authors have also reported the use of imatinib as neoadjuvant therapy in borderline operable head-and-neck DFSP, yielding 66% partial response rates with all the three patients achieving wide excision with negative margins. This is in congruence with the results of an European Organisation for Research and Treatment of Cancer (EORTC) study in which out of 16 patients of DFSP treated for 14 weeks with 800 mg daily of neoadjuvant imatinib, 4 (25%) achieved complete remission after wide local excision.[4] The cons of neoadjuvant imatinib should be also kept in mind, one of which being long-term outcomes with respect to local margin control and disease-free survival. In addition, imatinib use may lead to reduced cellularity and increased hyalinization in patchy areas, leading to subsequent generation of skip lesions that could obscure identification of the true negative margins.

In a pooled analysis of EORTC and SWOG studies, it was observed that a daily dose of 400 mg has similar efficacy to 800 mg daily. In addition, as higher doses are related to more myelosuppression and diarrhea, use of 400 mg dose seems to be the best possible balance between benefits and adverse effects. The authors have reported the Grade 3 toxicities in 33.3% of patients which included anemia, anorexia, and fatigue. These toxicities could stem from vitamin B12 deficiency in patients on long-term imatinib.[5] However, the evaluation of the cause of these toxicities was not reported by the authors, and we suggest checking the records for these values. Furthermore, routine workup for anemia profile for patients planned for starting imatinib should be done in order to correct nutritional deficiencies, especially in Indian settings where a large chunk of population is nutritionally compromised. Use of these simple measures can reduce toxicities such as anemia and fatigue which are an important cause of poor compliance and possible poor outcomes.

The authors' work serves as an addition to the limited available literature in head-and-neck DFSP, and such studies should be highly encouraged. It is also suggested to form inter-institutional DFSP data-sharing groups so that combined data can generate better evidence to select the best possible option in an individual patient of DFSP.

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There are no conflicts of interest.

  References Top

Kashyap L, Noronha V, Patil V, Joshi A, Mahajan A, Mittal N, et al. Dermatofibrosarcoma protuberans of head and neck: Clinical outcome of nine cases treated with imatinib. Cancer Res Stat Treat 2019;2:112-8.  Back to cited text no. 1
  [Full text]  
Angouridakis N, Kafas P, Jerjes W, Triaridis S, Upile T, Karkavelas G, et al. Dermatofibrosarcoma protuberans with fibrosarcomatous transformation of the head and neck. Head Neck Oncol 2011;3:5.  Back to cited text no. 2
Pandey AV, Singh A. Head-and-neck dermatofibrosarcoma protuberans: Where does imatinib 'fit in'? Cancer Res Stat Treat 2019;2:74-5.  Back to cited text no. 3
  [Full text]  
Rutkowski P, Van Glabbeke M, Rankin CJ, Ruka W, Rubin BP, Debiec-Rychter M, et al. Imatinib mesylate in advanced dermatofibrosarcoma protuberans: Pooled analysis of two phase II clinical trials. J Clin Oncol 2010;28:1772-9.  Back to cited text no. 4
Moura MS, Benevides TC, Delamain MT, Duarte GO, Percout PO, Dias MA, et al. Evaluation of anemia after long-term treatment with imatinib in chronic myeloid leukemia patients in chronic phase. Hematol Transfus Cell Ther 2019. pii: S2531-1379(19)30089-6.  Back to cited text no. 5

This article has been cited by
1 Author Reply to Kapoor et al.
Lakhan Kashyap,Vanita Noronha,Vijay Patil,Amit Joshi,Abhishek Mahajan,Neha Mittal,Kumar Prabhash
Cancer Research, Statistics, and Treatment. 2019; 2(2): 257
[Pubmed] | [DOI]


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