486 - EXTENSIVE DERMATOFIBROSARCOMA PROTUBERANS IN THE CHEST AND BREAST: A CASE REPORT

Abstract

Dermatofibrosarcoma protuberans (DP) is a neoplasm of the deep layer of the dermis and subcutaneous tissue. It presents a rare incidence and constitutes 0.1% of the malignant tumors. It has local aggressive behavior with slow tumor
growth, low metastasis rates of around 5%, but has high rates of local recurrence after surgical excision. The diagnosis
is histopathological through biopsy of the lesion, and the fluorescence in situ hybridization (FISH) method can help in
selected cases by detecting possible chromosomal rearrangements in the tissue. Physical examination, magnetic resonance imaging, and computed tomography may be helpful in assessing the area of tumor extension. The treatment
of choice is resections with 3-cm wide margins or Mohs micrographic surgery. The prognosis is directly related to the
correct excision of the compromised margins. A woman, 51 years old, presented with a raised, brownish, irregular,
13×8 cm multinodular lesion attached to the overlying skin, in the epigastric region, which extended to the left hypochondrium and lower quadrants of the left breast, without local symptoms or lymph node enlargement. She reported
the appearance of a small nodular skin lesion at the site 10 years ago and reports continuous growth of the nodule,
with the involvement of the adjacent skin and the left breast starting 5 years ago, after the formation of a hypertrophic
scar due to two previous local resections of the initial lesion. Mammography showed a nodule of cutaneous origin in
the lower inner quadrant of the left breast, which may correspond to keloids — BIRADS 2. Breast ultrasound showed a
solid, echogenic nodule measuring 1.6×1.2 cm in the left breast at 8 am; 2.5 cm from the nipple — suggestive of lipoma,
and at 7 am, nodule measuring 2.4×1.6 cm that penetrates the breast parenchyma — BIRADS 3. The lesion was diagnosed as dermatofibrosarcoma on histopathological examination of a skin fragment. The patient underwent resection
of the lesion with a safety margin by the mastology team and primary reconstruction using a thigh graft by the plastic
surgery team. A surgical specimen was sent for anatomopathological examination that presented a result compatible
with a previous biopsy, reiterating the diagnosis of DP, and with peripheral and deep surgical margins free of neoplastic involvement; evolved without postoperative complications or restriction of range of motion; and referred to radiotherapy to assess the need for additional treatment.