Limb conservation surgery in biphasic synovial sarcoma of thigh with vascular involvement: A race against time

股部双相型滑膜肉瘤伴血管侵犯的保肢手术:与时间赛跑

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Abstract

INTRODUCTION: Synovial sarcomas are malignant soft tissue neoplasm representing 5 to 10 % of all Soft tissue sarcoma with incidence of 2.75 per 100,000. It is presented in particular along with extra articular location with no as such relation to synovial structures. Among various histological pattern biphasic synovial sarcoma (SS) is considered classical type. Involvement of neurovascular structures in synovial sarcoma is least noted and such invasion by malignant cells is considered an indication for amputation. However vessel reconstructive surgeries have also resulted in conservation of limb hence providing good quality of life. CASE PRESENTATION: 13 years old Asian female presented with complaint of exposed synthetic vessel graft from her previous surgical site where femoral vessel reconstruction was carried out for SS of thigh with femoral vessel involvement. Following her (wide local excision) WLE and femoral vessel reconstruction in another center she suffered thrombosis of her femoral vessel following which emergency thrombectomy was also carried out. Later in our center vertical rectus abdominis myocutaneous flap (VRAM) flap was used to manage her exposed synthetic graft. At subsequent follow up patient was ambulating well with no signs of radiological metastasis. CLINICAL DISCUSSION: Surgical resections of mass along with synthetic graft placement of the concomitant vascular bundle have also shown significant improvement in reducing the burden of the severe diseases such as synovial sarcoma. CONCLUSION: SS with its malignant pathophysiology have impacted severely the quality of life of even among the pediatrics group of population. It is utmost need to set up proper and definitive muscular oncological care to reduce the morbidity and mortality associated with such malignancies.

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