Dermatofibrosarcoma protuberans on the right neck with superior vena cava syndrome: Case report and literature review


Dermatofibrosarcoma protuberans (DFSP) is an uncommon dermal soft tissue tumour of intermediate malignancy. A 44-year-old man presented to the hospital with a large lesion on the right upper chest and neck. Despite eight previous surgical excisions, the tumour had continued to recur. Contrast-enhanced computed tomography showed recurrence of the tumour, associated with superior vena cava (SVC) syndrome. He declined radical surgical resection of the recurrent tumour, which may have required right upper limb amputation. Targeted therapy with sunitinib malate was therefore introduced. This case demonstrates the recurrent nature of DFSP and the association of this lesion on the upper chest/neck with SVC syndrome. Primary wide radical resection is essential for better local control and to avoid the development of SVC syndrome.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon dermal soft tissue tumour of intermediate malignancy with a tendency for local recurrence; however metastasis rarely occurs. It accounts for 4% of all soft tissue sarcomas. DFSP tumours occur most commonly in the extremities (47%) and trunk (36%), but can also occur in the head and neck region. It is most common in young adults, and the gender distribution is equal. Clinically, DFSP is an indolent and slow-growing raised skin nodule. Histologically, it is composed of spindle cells arranged in an irregular storiform pattern. It stains positively with CD34. It is thought to originate from within the dermis. With its infiltrative character, the tumour margins can extend far beyond macroscopic boundaries with asymmetric tentacle-like projections. They can exist as a homogeneous group of tumour cells mimicking normal tissue that migrate in a multilayered manner.

The mainstay of treatment for DFSP is radical surgical resection. There is currently no standard guideline for the optimal management of DFSP. Radiotherapy and molecular targeted therapy (imatinib or tyrosine kinase inhibitors, TKIs) are usually used as neoadjuvant and adjuvant treatments. Nevertheless, radical surgical resection can be difficult to achieve in the head and neck region due to anatomical limitations. With its invasive and recurrent nature, DFSP on the right upper chest/neck can sometimes cause superior vena cava (SVC) syndrome, which is associated with significant morbidity. SVC syndrome occurs when there is impaired venous return in the SVC either by extrinsic compression of the adjacent tumour or intrinsic compression from the tumour thrombus.

The purpose of this article is to highlight the clinical association between DFSP in the head and neck region and SVC syndrome. We also discuss the associated treatment dilemma and potential treatment options.

Case report

A 44-year-old man presented to our hospital with a recurrent 13 cm × 17 cm × 11.5 cm lesion on the right upper chest/neck, compressing his right clavicle ( Fig. 1 ). He had had multiple recurrences in the same area since 2006, and had undergone eight surgical resections in the past. In 2006, he presented with a pea-sized lesion on his right upper chest. It was thought to be a keloid and he underwent surgical excision with primary closure. However, histology confirmed DFSP. A similar lesion recurred 2 years later. He underwent a repeat surgical resection with a wider surgical margin, but relapsed again in the following year. He underwent a further surgical resection followed by two cycles of cisplatin chemotherapy. A further five recurrences occurred over the subsequent 3 years despite wide surgical resection. The timing of relapse became shorter, at a mean of 5 months after the third resection, and the size of the recurrence was larger with every relapse. In February 2012, a large (9 cm × 17 cm × 6.5 cm) mass on the root of the right neck was resected and this defect was reconstructed with a latissimus dorsi myocutaneous flap. Histology confirmed a poorly circumscribed infiltrative spindle cell tumour ( Fig. 2 ). It was composed of monotonous fibroblast-like cells demonstrating a storiform pattern with mild mitotic activity. Immunohistochemically, the tumour cells were stained with CD34 ( Fig. 2 C and D). Sarcomatous transformation of the DFSP was ruled out after meticulous microscopic examination ( Fig. 2 B).

Fig. 1
A 13 cm × 17 cm × 11.5 cm large protuberant lesion with profuse bleeding located on the right root of the neck, extending to the right chest.

Jan 24, 2018 | Posted by in Oral and Maxillofacial Surgery | Comments Off on Dermatofibrosarcoma protuberans on the right neck with superior vena cava syndrome: Case report and literature review
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