Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Review Article
Review Articles
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Letter to the Editor - Case Letter
doi: 10.4103/0378-6323.168347
PMID: 26515854

Infantile dermatofibrosarcoma protuberans: An underrecognized diagnosis?

Giulio Gualdi1 , Laura Pavoni1 , Paola Monari1 , Lucia Corasaniti2 , Pierluigi Pedersini2 , Maria Pia Bondioni3 , Paolo Incardona4 , Daniele Alberti2 , PierGiacomo Calzavara-Pinton1 , Ausilia Maria Manganoni1
1 Department of Dermatology, A.O. Spedali Civili, Brescia, Italy
2 Department of Pediatric Surgery, Presidio Ospedale dei Bambini- A.O. Spedali Civili, Brescia, Italy
3 Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, Brescia, Italy
4 Department of Pathology, Spedali Civili, Brescia, Italy

Correspondence Address:
Giulio Gualdi
Department of Dermatology, University Hospital Spedali Civili, P.le Spedali Civili 1, 25123 Brescia
How to cite this article:
Gualdi G, Pavoni L, Monari P, Corasaniti L, Pedersini P, Bondioni MP, Incardona P, Alberti D, Calzavara-Pinton P, Manganoni AM. Infantile dermatofibrosarcoma protuberans: An underrecognized diagnosis?. Indian J Dermatol Venereol Leprol 2015;81:635-637
Copyright: (C)2015 Indian Journal of Dermatology, Venereology, and Leprology


Dermatofibrosarcoma protuberans (DFSP) is a rare intermediate-grade malignancy considered uncommon in infancy, childhood and adolescence whereas it is usually described in middle-aged men.[1] We report a pediatric case of dermatofibrosarcoma protuberans in a 5-year-old girl from Bangladesh who was admitted to the University Hospital Spedali Civili of Brescia, Italy for a small asymptomatic subcutaneous mass on the anterior side of the left leg [Figure - 1]. The lesion was evident since she was 5 months old and an ultrasound scan had been routinely performed since she was 2 years old showing an oval subcutaneous mass of 1-cm with a moderately irregular appearance without evident vascular flows on Doppler examination suggesting the diagnosis of lipoma. The size had remained almost unchanged until the past year when it began to increase. The ultrasound scan performed before the admission of the patient in our unit confirmed the presence of an oval subcutaneous lesion of 1.7 × 0.8 cm that showed an irregular appearance characterized by a high vascular flow on Doppler examination [Figure - 2]. Considering the clinical and the imaging findings, a surgical excision was suggested. The patient underwent surgery under general anesthesia. During the surgical procedure, we found an oval, nodular, subcutaneous yellowish color mass of 3 × 2 cm with a solid and fatty appearance extending until the muscular fascia without macroscopic infiltration of the muscle tissues. The lesion was easily excised from the surrounding fat and did not appear to be encapsulated.

Figure 1: Subcutaneous mass on the anterior side of the left leg increasing in diameter for one year
Figure 2: Ultrasound scan: (a) Oval-shaped lesion with heterogeneous echogenicity (clippers) characterized by mixed hypo-and hyperechoic components is localized to the subcutaneous tissue near the underlying muscle fascia. The margins are blurred focally. (b) On color Doppler evaluation, intralesional vascular structures can be detected

Histology showed a lesion suggestive for a dermatofibrosarcoma protuberans infiltrating the surrounding fat tissue [Figure - 3].

Figure 3: (a) Panoramic vision of dermatofibrosarcoma protuberans infi ltrating the surrounding fat tissue (H and E, ×20);(b) Typical spindle cell proliferation (these spindle cell were positive when labeled with anti-CD34 antibodies [not shown]) (H and E, ×400)

In view of this diagnosis, at re-excision we kept a 3-cm resection margin around the previous scar, including cutaneous, subcutaneous and muscular tissues. Primary closure of the wound was not done which was covered with a sterile dressing at the end of the procedure. Since histology did not reveal residual tumor tissue, the girl underwent surgery for closing the wound through local rotation flap after 3 days. On the 7th postoperative day, the patient was discharged. Currently, the patient is being regularly monitored with clinical evaluation of the skin and scans of soft tissues and lymph nodes. There were no signs of recurrence at follow up 2 years after surgery.

Recently, we conducted a review of the literature on dermatofibrosarcoma protuberans in infancy and adolescence emphasizing that this tumor is not so rare.[2] Dermatofibrosarcoma protuberans does not have a distinctively recognisable manifestation in adulthood, and even less so in childhood. Thus, the apparently benign appearance, variable location, slow growth and the lack of subjective symptoms can lead to a delay or misdiagnosis of the disease.[3],[4] The tumour may consist of an asymptomatic, atrophic or exophytic, indurated plaque/nodule or compressible plaques similar to atrophoderma lesions.[2],[3],[4] Moreover, as in our case, it can simulate a lipoma and also other tumors such as sarcoma, neuroblastoma and other mesenchymal tumors.[5]

Histologically, dermatofibrosarcoma protuberans is characterized by a CD34+ spindle-cell proliferation arranged in a cartwheel or storiform pattern infiltrating deep into the dermis and adipose layers in a fascicular growth pattern. The histopathological differential diagnoses may include dermatofibroma, thymoma, or malignant fibrous histiocytoma-pleomorphic or atypical fibroxanthoma which also show a storiform pattern but are usually CD34 negative. Cytogenetic abnormalities that characterize dermatofibrosarcoma protuberans can be shown through fluorescence in situ hybridization and multiplex reverse transcription polymerase chain reaction for the detection of the distinctive gene rearrangements of chromosomes 17 and 22. The standard treatment for dermatofibrosarcoma protuberans is wide local excision with resection of at least 3 cm of normal looking surrouding skin and block removal of the underlying fascia. Revision surgery may be performed to ensure complete excision and reduce the risk of local recurrence. Alternatively, surgical excision with Mohs technique could be employed. Due to re-growth of residual tumor following incomplete removal of microscopic projections of the neoplastic cells, the local recurrence, rate of dermatofibrosarcoma protuberans is high (32–76%).[2] For this reason, follow-up visits are required in order to recognize any unusual changes in the scar of the previous excision. In case of recurrence a further wide excision is recommended. Radiation therapy shows good control in recurrent disease. Moreover, chemotherapy (e.g., vinorelbine and methotrexate) and more recently imatinib mesylate have been reported to be effective. However, the metastatic rate of dermatofibrosarcoma protuberans is low (4%), occurring principally as lung and lymph node metastases. Careful regional lymph node examination is necessary.

In conclusion, slow growth and difficulties in recognising the standard clinical appearance may delay diagnosis of the disease. In our case, a lesion was present at the age of 5 months but interpreted at ultrasound examination as a lipoma. However, we can assume that the initial lesion was a dermatofibrosarcoma protuberans because its evolution from another tumor has not been reported.

Cai H, Wang Y, Wu J, Shi Y. Dermatofibrosarcoma protuberans: Clinical diagnoses and treatment results of 260 cases in China. J Surg Oncol 2012;105:142-8.
[Google Scholar]
Manganoni AM, Pavoni L, Gualdi G, Marocolo D, Chiudinelli M, Sereni E, et al. Dermatofibrosarcoma protuberans in an adolescent: A case report and review of the literature. J Pediatr Hematol Oncol 2013;35:383-7.
[Google Scholar]
Iqbal CW, St Peter S, Ishitani MB. Pediatric dermatofibrosarcoma protuberans: Multi-institutional outcomes. J Surg Res 2011;170:69-72.
[Google Scholar]
Posso-De Los Rios CJ, Lara-Corrales I, Ho N. Dermatofibrosarcoma protuberans in pediatric patients: A report of 17 cases. J Cutan Med Surg 2014;18:1-6.
[Google Scholar]
Manganoni AM, Farisoglio C, Lonati A, Zorzi F, Tucci G, Pinton PG. Cutaneous epithelioid malignant schwannoma: Review of the literature and case report. J Plast Reconstr Aesthet Surg 2009;62:e318-21.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections