Journal of Otology & RhinologyISSN: 2324-8785

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Case Report, J Otol Rhinol Vol: 4 Issue: 3

Rare Pediatric Tumour: Dermatofibrosarcoma - A Case Report

Divya Gupta1*, Shikharani Patel1, Ishwar Singh1 and Nita Khurana2
1Department of Otorhinolaryngology and Head & Neck Surgery, Maulana Azad Medical College and associated Lok Nayak Hospital, New Delhi-110002, India
2Department of Pathology, Maulana Azad Medical College and associated Lok Nayak Hospital, New Delhi-110002, India
Corresponding author : Dr. Divya Gupta, MS
DNB66, Raj Nagar, Pitampura, New Delhi-110034, India
Tel: +91-9810245542
E-mail: [email protected]
Received: February 16, 2015 Accepted: April 25, 2015 Published: May 04, 2015
Citation: Gupta D, Patel S, Singh I, Khurana N (2015) Rare Pediatric Tumour: Dermatofibrosarcoma - A Case Report. J Otol Rhinol 4:3. doi:10.4172/2324-8785.1000229

Abstract

Rare Pediatric Tumour: Dermatofibrosarcoma - A Case Report

Dermatofibrosarcoma protuberans is a very rare dermal tumour in head and neck region, more so in pediatric age group. The characteristic histopathological pattern of the tumour is responsible for easy recurrence if improperly addressed. We present a rare case of this tumour in a 15 year old child situated in supraclavicular region.

Keywords: Pediatric tumour; Dermatofibrosarcoma

Introduction

Dermatofibrosarcoma protuberans is a rare low to intermediate grade dermal tumour, sparsely found in head and neck region [1]. It is a clinically challenging tumour in view of its initial slow idle behaviour that delays its presentation to treatment facilities [2]. It spreads characteristically in a villous pattern in adjoining tissues, thereby recurring commonly if inadequately excised [3]. We present a case of supraclavicular lesion in a child. The objective of this article is to acquaint the clinicians to the possibility of a rare tumour like this in a seemingly benign swelling and to have an idea regarding its management.

Case Report

A 15 year old male presented to ENT OPD of Lok Nayak Hospital, Delhi with chief complaint of swelling on the right side of shoulder for one year which increased gradually from its first appearance as pea size to the current tennis ball size, more prolific in last two months. The swelling, though, did not cause any restriction of movements, tingling, numbness, pain or bleeding. On examination, a single 10×8 cm swelling with smooth and shiny pinkish-red surface with dilated veins was present on the right side of neck in supraclavicular region partially abutting the right shoulder and right clavicle (Figure 1). Local temperature was normal. It was firm to touch, non tender with well defined margins. There was no restriction of shoulder movements and no other swelling was found anywhere in the body. Systemic examination was unremarkable.
Figure 1: Clinical photograph of the patient showing a well defined swelling in right supraclavicular fossa partially abutting the right clavicle.
Contrast enhanced computed tomography of the neck revealed well defined, smooth 10×6 cm soft tissue attenuation, enhancing lesion in subcutaneous plane overlying lateral part of right clavicle and abutting the anterior part of right deltoid muscle. Underlying bone was found intact (Figure 2). FNAC suggested a benign mesodermal tumour.
Figure 2: CECT neck (coronal and axial cuts) showing a well defined, smooth lesion in right supraclavicular fossa with no adjacent bony erosion.
A wide local excision was carried out under general anaesthesia and the resulting defect was repaired using split skin graft. The specimen was sent for histopathological examination which showed cellular spindle cell tumour in dermis consistent with dermatofibrosarcoma protuberans (Figure 3).
Figure 3: Histopathological photograph showing cellular spindle cell tumour in dermis with overlying stretched skin.

Discussion

Dermatofibrosarcoma was described first in 1924 by Darier and Ferrand, however Dermatofibrosarcoma protuberans was christened by Hoffman in 1925. The tumor accounts for less than 0.01% of all the malignancies but 2-6% of all the soft tissue sarcomas [4]. It most commonly involves the trunk (42-72%) followed by proximal extremities (16-30%) and less normally head and neck (10-16%) [5]. It is a low to intermediate grade spindle cell tumour arising from dermis with propensity in males in the third and fourth decades [6]. Less than 10% cases occur in children. Our case represents the rarer of this already rare entity afflicting a young male in his second decade in the head and neck region. Although the metastatic tendency of the tumour is relatively less, it is locally aggressive in nature with high recurrence rates, if inadequately excised. We did a wide local excision without any recurrence in three year follow up.
The tumour starts off usually as a discolored indurate plaque and gradually increases in size. It escapes special notice because of its slow indolent growth pattern, hence is usually big in size at presentation. Our patient also kept dismissing this swelling over his neck for almost a year when sudden increase in its size alerted him to seek medical advice. In literature, mean delay in diagnosis of the tumour has been reported as 10.08 years [6]. It may become nodular or ulcerative or may get fixed to deeper structures like fascia or muscle in later stages. Fortunately our patient’s tumour was still in initial stage where it only involved the deep cervical fascia. Differential diagnosis in earlier stages should include keloid, epidermoids, lipomas and nodular fasciitis and in later stages, it needs to be distinguished from conditions like pyogenic granuloma and other soft tissue sarcomas.
Microscopically, it is characterised by spindle shaped cells arranged in cartwheel pattern with finger-like infiltration into subcutaneous fat, which may extend peripherally till 3 cm. Immunological staining reveals it to be strongly positive for CD34, vimentin and apolipoprotein D and negative for S-100, factor XIIIa and CD44 staining. In this case also similar results were seen.
In more than 90% of cases of dermatofibrosarcoma protuberans, supernumerary ring chromosomes or an inimitable translocation involving chromosomes 17 and 22 is found. The latter fuses the COL1A1 gene on chromosome 17 to the PDGF beta chain on chromosome 22 resulting in autocrine stimulation of PDGF receptors on tumour cells ensuing cellular proliferation and tumour growth [7].
The treatment of choice for dermatofibrosarcoma protuberans remains wide local excision of the tumour with 3 cm margin, given the villous natured extension of the tumour. Our patient also underwent the same and remains tumour free at one year post-surgery. Micrographic controlled excision holds great promise in allowing cancer free margins as has been suggested in various studies [6,8]. Adjuvant radiotherapy should be considered if margins come very close or involved in cases where repeat resection is not feasible because of anatomic limitations [9]. Chemotherapy does not offer a great cure but targeted chemotherapy in the form of tyrosine kinase inhibitor imatinib is often recognised as the gold standard in inoperable or metastatic or recurrent cases of dermatofibrosarcoma protuberans [7].

Conclusion

Aggressive nature of dematofibromosarcoma protuberans and propensity for local invasion necessitates its complete removal with adequate margins. Head and neck region is amongst the most challenging area to get involved, especially in children because of critical structures and aesthetic considerations involved.

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