Undifferentiated pleomorphic sarcoma of skin in unusual locations: Report of two cases

Abstract

Undifferentiated pleomorphic sarcoma (UPS) of the skin is a rare soft tissue sarcoma subtype with a high risk of metastasis and local recurrence. Ultraviolet exposure plays a prominent role in its etiology. Herein, we present two rare cases of UPS of the skin with an occurrence in non-ultraviolet exposed locations and discuss the need of wide resection and the identification of the depth of the tumor. Due to the resection of the skin, resulting defect was undertaken with skin grafting. One case was extending to the fascia and, after the resection, vacuum-assisted closure therapy was used for the formation of granulation tissue. No recurrence was seen after three and four years for either cases. In conclusion, UPS is a rare, aggressive malignant tumor with a high local recurrence and metastasis rate. Suspicion of malignancy in a plaque-like or nodular lesion in the skin is crucial, and local recurrence and metastasis risk is significantly correlated to the success of wide excision.

Pleomorphic dermal sarcoma presenting in a child with Li‐Fraumeni syndrome: A case report and review of the literature

“…8 However, in that case, the lesions were confined to the dermis without subcutane- PDS is mostly found on the UV-damaged skin of elderly patients' extremities. 1,2 It is rare in children and may be a manifestation of a familial cancer syndrome such as LFS or retinoblastoma syndrome. 9 LFS is an inherited cancer predisposition disease that is often caused by a germline mutation in TP53, which encodes the antiproliferative role of the p53 protein in response to DNA damage, oncogene activation, and other stress conditions.…”

“…Sarcomas account for less than 1% of all cancers and undifferentiated pleomorphic sarcoma (UPS) is an uncommon subtype of sarcoma with a high risk of both metastasis and local recurrence. 1 While UPS encompasses a diverse group of soft-tissue malignancies arising from internal organ, retroperitoneal, and osteoid origins, the term pleomorphic dermal sarcoma (PDS) is more confined to the tumor of cutaneous origin. PDS typically appears as an asymptomatic, rapidly growing cutaneous or subcutaneous nodule usually on chronically ultraviolet (UV)-damaged skin such as head, neck, and the extremities, and is prevalent around the eighth decade of life.…”

“…PDS typically appears as an asymptomatic, rapidly growing cutaneous or subcutaneous nodule usually on chronically ultraviolet (UV)-damaged skin such as head, neck, and the extremities, and is prevalent around the eighth decade of life. 1,2 We present a rare case of PDS occurring in a non-sun-exposed area of a child with Li-Fraumeni syndrome (LFS). A biopsy was performed, and the histopathology showed an unencapsulated, poorly circumscribed mass involving the dermis and subcutaneous fat (Figure 2A).…”

“…Sarcomas account for less than 1% of all cancers and undifferentiated pleomorphic sarcoma (UPS) is an uncommon subtype of sarcoma with a high risk of both metastasis and local recurrence 1 . While UPS encompasses a diverse group of soft‐tissue malignancies arising from internal organ, retroperitoneal, and osteoid origins, the term pleomorphic dermal sarcoma (PDS) is more confined to the tumor of cutaneous origin.…”

“…While UPS encompasses a diverse group of soft‐tissue malignancies arising from internal organ, retroperitoneal, and osteoid origins, the term pleomorphic dermal sarcoma (PDS) is more confined to the tumor of cutaneous origin. PDS typically appears as an asymptomatic, rapidly growing cutaneous or subcutaneous nodule usually on chronically ultraviolet (UV)‐damaged skin such as head, neck, and the extremities, and is prevalent around the eighth decade of life 1,2 . We present a rare case of PDS occurring in a non‐sun‐exposed area of a child with Li‐Fraumeni syndrome (LFS).…”