Dermatofibrosarcoma Protuberans of Scalp With Cervical Lymph Node Metastasis

Abstract

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, slow growing and locally aggressive tumor of the skin with a high rate of recurrence even after supposedly wide excision. The reports of regional lymph node metastasis and distant metastasis are very rare. Because of the extreme rarity of these cases with metastasis, the experience with management of such patients is very limited. A case of recurrent DFSP of scalp, with metastasis to the regional lymph nodes, in a 17-year-old boy is reported here. This is the second case of DFSP involving scalp and 16th case of DFSP of all sites metastasizing to the regional lymph nodes reported in literature. The patient was treated with wide excision of the lesion and ipsilateral radical neck dissection (including excision of overlying involved skin).

Dermatofibrosarcoma protuberans with fibrosarcomatous transformation of the head and neck

“…Regional lymphatic metastasis is uncommon, approximately 1%, even less frequent than distant metastasis with only a few cases reported in the literature up to date [10,34]. …”

Imaging Findings of Head and Neck Dermatofibrosarcoma Protuberans

“…3,4 DFSP grows in an infiltrative manner and has the capacity for local recurrence, though it rarely metastasizes. 5,6 The imaging features of DFSP of the trunk and extremities are nonspecific, including low signal on T1 and high on T2 on MR imaging, with uniform enhancement after contrast administration. 7,8 CT findings include isoattenuated to hypoattenuated lesions without calcifications and uniform (small lesions) to heterogeneous (large lesions) enhancement after contrast administration.…”

Metastasis of soft tissue sarcomas in lymph node: A cytomorphological study

“…Very few such cases are available in literature. [22][23][24][25][26] Various studies have shown synchronous involvement of regional lymph node in patients with STS ranging from 5.9% to 63.15% of cases. In our study, 15.8% (3 of 19) cases showed synchronous involvement.…”