Necrosis of a Skin Autograft After Short-Term Treatment with Sunitinib in a 14-Year-Old Girl with Metastatic Alveolar Soft Part Sarcoma of the Thigh

Abstract

A 14-year-old girl was diagnosed with alveolar soft part sarcoma (ASPS) of the thigh and lung metastases. She underwent tumor resection and pulmonary metastasectomy followed by hyperfractionated local radiotherapy (44.8 Gy). A mesh graft transplant was used to cover the postoperative skin defect on the thigh. Since ASPS do not respond to conventional chemotherapy antiangiogenic treatment with peginterferon alfa-2b and thalidomide was started. Immunohistochemical analysis of tumor tissue showed expression of vascular endothelial growth factor receptors (VEGFR) 1, 2, 3, and platelet derived growth factor receptor (PDGFR)-alpha and -beta. Hence, additional treatment with multitargeted receptor tyrosine kinase inhibitor sunitinib (Sutent) was started on a compassionate use basis. 2 weeks later the patient presented with necrosis of the skin transplant requiring necrectomy and skin grafting. This case illustrates that drugs inhibiting vascular endothelial growth factor receptors have to be used very cautiously in cancer patients with severe pre-existing skin damage.

Alveolar soft part sarcoma in childhood and adolescence: Report of three cases and review of literature

“…The maximum tolerated dose for patients without cardiac risk factors is 15 mg/m 2 for four consecutive weeks and rest for 2 weeks (54). Studies have shown that sunitinib has a good effect on children with advanced ASPS and can improve their quality of life (51), but it should be used cautiously in patients with severe skin injury (34). ( 5) Immune checkpoint inhibitors: immune checkpoint inhibitors can activate the effect of human autoimmune response against tumors and have a more lasting effect.…”

The current management of alveolar soft part sarcomas

“…For multiple brain metastases, some researchers administered palliative whole-brain radiotherapy with 30 Gy in 10 fractions, but prognosis was poor because of the widespread metastases in advanced cases [25] . For single lung metastasis of ASPS, hyperfractionated local radiotherapy could be considered with total dose of 44.8 Gy [26] …”

“…[25] For single lung metastasis of ASPS, hyperfractionated local radiotherapy could be considered with total dose of 44.8 Gy. [26] Carbon-ion radiotherapy also showed satisfactory effects. There was a case report that a 9-year old girl who was diagnosed as ASPS with the tumor locating in the upper third of the vagina, received carbon-ion radiotherapy with 67.2 Gy in 16 fractions at the part of the tumor adhered to the posterior pubis which was residual after resection, the tumor decreased gradually without recurrence for 20 months, the patient had not any adverse effects.…”

Advances in treatment of alveolar soft part sarcoma: an updated review

“…For the lung metastasis of ASPS, whole-lung irradiation is generally not performed as for other subtypes of STS except Ewing sarcoma. Strategies combining metastasectomy and RT may be used; a case report indicated the use of hyperfractioned local RT with a total dose of 44.8 Gy (2 × 1.6 Gy daily) following pulmonary metastasectomy ( 59 ).…”