10.5826/dpc.1201a15
text/xml
2160-9381
1681
en
Haci Mustafa Ozdemir
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
imatinib
Dermatofibrosarcoma Protuberans in the Palm of a 3-Year-Old Child
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2022-02-02
2021-02-12
2022-02-02
2022-02-09
e2022015
Dermatol Pract Concept
Haci Mustafa Ozdemir
2022/02/02
10.5826/dpc.1201a15
e2022015
Department of Orthopaedics and Traumatology, Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey
2160-9381
imatinib
en
e2022015
Tsai YJ, Lin PY, Chew KY, Chiang YC. Dermatofibrosarcoma protuberans in children and adolescents: Clinical presentation, histology, treatment, and review of the literature. J Plast Reconstr Aesthet Surg. 2014;67(9):1222-1229. DOI: 10.1016/j.bjps.2014.05.031. PMID: 24973861.
Dermatofibrosarcoma Protuberans in the Palm of a 3-Year-Old Child
https://dpcj.org/index.php/dpc/article/download/1681/1477
       Dermatofibrosarcoma Protuberans in the Palm
                   of a 3-Year-Old Child
 Omer Faruk Kumbuloglu1, Fatih Barishan2, Fevziye Kabukcuoglu3, Haci Mustafa Ozdemir2


1 Department of Orthopaedics and Traumatology, Hand Surgery Division, Sisli Hamidiye Etfal Training and Research Hospital,
  Istanbul, Turkey
2 Department of Orthopaedics and Traumatology, Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey
3 Department of Pathology, Istanbul Sisli Hamidiye Etfal Application and Research Center, University of Health Sciences, Istanbul, Turkey



Key words: pediatric dermatofibrosarcoma protuberans, handpalm masses, Mohs micorgraphic surgery, imatinib
Citation: Kumbuloglu OF, Barishan F, Kabukcuoglu F, Ozdemir HM. Dermatofibrosarcoma protuberans in the palm of a 3-year-old child.
Dermatol Pract Concept. 2022;12(1):e2022015. DOI: https://doi.org/10.5826/dpc.1201a15
Accepted: May 5, 2021; Published: January 2022
Copyright: ©2022 Kumbuloglu et al. This is an open-access article distributed under the terms of the Creative Commons
Attribution-NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted
noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding author: Fatih Barishan, MD, Department of Orthopaedics and Traumatology, Sisli Hamidiye Etfal Training and Research
Hospital, Istanbul, Turkey. E-mail: drbarishan@hotmail.com




Introduction                                                             underwent surgical excision on the palm of his right hand
                                                                         at another clinic. The histological diagnosis was reported as
Dermatofibrosarcoma protuberans (DFSP) is a low-grade,                   lipofibromatosis at that time. Physical examination revealed
locally invasive tumor that originates from cutaneous tis-               a 1.5 cm × 2 cm mass in the same region (Figure 1A).
sues. DFSP is the second most frequent skin sarcoma and                       In the second surgical excision of the patient, the mass
is generally diagnosed between the ages of 30 and 50. It is              ­lesion was excised together with the overlying skin. The defec-
mostly localized in the trunk, proximal extremities, or in the           tive area that occurred at the excision site was closed with a
head and neck region [1]. There are a few reports showing                full-thickness skin graft obtained from the right inguinal area.
DFSP in the dorsum of the hand of pediatric cases [2]. In this           The histological examination showed a spindle cell lesion
study, a case is presented of a 3-year-old boy that underwent            that started just below the epidermis and reaching the der-
3 operations for DFSP in the palm of the hand.                           mis and subcutaneous fat tissue, with short bundles crossing
                                                                         each other and continued beyond the surgical border. Marked
                                                                         atypia or mitotic activity was not observed. Immunohisto-
Case Presentation
                                                                         chemical studies revealed widespread CD34 staining. The
A 3-year-old male child presented to our clinic with a mass              focal staining properties were observed using caldesmon and
lesion at the level of the second metacarpophalangeal joint in           Alpha Smooth Muscle Actin stains. Desmin stain, HMB45,
the palm of the right hand. Two months before, the patient               pancytokeratin, CD31, and calponin were all negative. The




Research Letter | Dermatol Pract Concept. 2022;12(1):e2022015                                                                               1
                                                                          Figure 1. (A) Nodular lesion at the volar side
                                                                          of the right hand before the second surgical
                                                                          ­excision. (B) Tissue specimen evaluation after the
                                                                           second surgical excision: spindle cells arranged in
                                                                           a storiform pattern (H&E, ×400) and (C) diffuse
                                                                           CD34 immunostaining (×400).




Ki67 proliferation index was approximately 2-5% and the        need for radiotherapy or imatinib treatment. There was no
lesion was diagnosed as DFSP (Figure 1, B and C).              sign of recurrence at the end of the first postoperative year
    The patient was reevaluated following the histological     (Figure 2, D-F).
diagnosis of DFSP and a subsequent chest X-ray did not
show metastasis. Physical examination did not show any
                                                               Conclusions
sign of lymphadenopathy. The third surgical excision was
planned for the patient.                                       A diagnosis of DFSP is made upon clinical examination and
    In the third surgical excision Mohs micrographic sur-      histological evaluation of the patient and is often difficult
gery was used. The surgical excision site was mapped with      due the frequently asymptomatic and slow progression. His-
the tumor in the middle (Figure 2, A and B). The whole         tological diagnosis cannot be made with routine staining.
epidermal tissue, dermal tissue, first lumbrical muscle, and   Clinicians should consider DFSP in the differential diagnosis
common palmar digital nerve were excised together with a       for pediatric palmar cutaneous masses; otherwise, a delay in
tissue margin of 1 cm (Figure 2C). Since intraoperative fro-   diagnosis may cause a loss of hand function.
zen examination was reported as negative for the surgical
border, a 4 cm × 3 cm wound site was left open and covered     Informed consent: Written informed consent for publication

with surgical dressing, and a plaster was applied. The wound   of clinical details and clinical images was obtained from the

healed with a secondary closure in 6 weeks. There was no       patient.




2                                                                  Research Letter | Dermatol Pract Concept. 2022;12(1):e2022015
        Figure 2. (A) Appearance of the hand before Mohs micrographic surgery. (B) Tumor mapping, placing the lesion in the
        center. (C) After Mohs micrographic surgery. (D) Wound healing process following Mohs micrographic surgery 4 weeks
        postoperatively. (E, F) Appearance of the hand following Mohs micrographic surgery 1 year postoperatively with good
        function and no contracture development.




Research Letter | Dermatol Pract Concept. 2022;12(1):e2022015                                                                 3
References                                                              2. Tsai YJ, Lin PY, Chew KY, Chiang YC. Dermatofibrosarcoma
                                                                           protuberans in children and adolescents: Clinical presenta-
1. Shah KK, McHugh JB, Folpe AL, Patel RM. Dermatofibrosarcoma             tion, histology, treatment, and review of the literature. J Plast
   protuberans of distal extremities and acral sites: a clinicopatho-      ­Reconstr Aesthet Surg. 2014;67(9):1222-1229. DOI: 10.1016/j.
   logic analysis of 27 cases. Am J Surg Pathol. 2018;42(3):413-419.        bjps.2014.05.031. PMID: 24973861.
   DOI: 10.1097/PAS.0000000000000998. PMID: 29240584.




4                                                                          Research Letter | Dermatol Pract Concept. 2022;12(1):e2022015
