Pancreatic metastasis of angiosarcoma (Stewart–Treves syndrome) diagnosed using endoscopic ultrasound-guided fine needle aspiration

Abstract

Background:Pancreatic involvement of angiosarcoma is extremely rare.Methods:We herein report a rare case of angiosarcoma associated with chronic lymphedema (Stewart–Treves syndrome) with pancreatic metastasis that was diagnosed using endoscopic ultrasound (EUS)/fine needle aspiration (FNA).Results:A 43-year-old woman with a history of radical hysterectomy with bilateral inguinal lymphadenectomy and chemoradiotherapy for cervical cancer 15 years prior noticed the presence of erythematous indurative plaques on her right femoral region, where chronic lymphedema had developed. Contrast-enhanced computed tomography (CT) revealed not only multiple nodules in the subcutaneous tissue of the right femoral region but also a 25 mm × 20 mm solid mass in the region of the pancreatic tail. A histological analysis of the specimens obtained using EUS/FNA revealed angiosarcoma that was immunohistochemically positive for platelet/endothelial cell adhesion molecule-1 but negative for cytokeratin. The patient was diagnosed as Stewart–Treves syndrome that had metastasized to the pancreas. Chemotherapy was performed, but the patient died 14 months after her diagnosis.Conclusion:Unfortunately, this patient was not followed up, even though she had chronic lymphedema of the right femoral region due to the repeated occurrence of phlegmon. To improve the survival rate of this fatal secondary malignant complication of radical lymphadenectomy, an early diagnosis with consecutive and long-term clinical follow-up and close monitoring for Stewart–Treves syndrome is therefore important.

Cutaneous angiosarcoma: update on biology and latest treatment

“…Secondary angiosarcoma is usually related to a history of radiation treatment and chronic lymphedema. Therefore, secondary angiosarcoma is often termed ‘radiation-associated angiosarcoma (RAAS)’ in the first scenario, or Stewart–Treves syndrome in the latter [ 3 , 4 ]. The term, ‘breast angiosarcoma’ encompasses both cutaneous and noncutaneous lesions whose cause is presumably secondary.…”

Comments on and Illustrations of the EFSUMB CEUS Guidelines: Transabdominal and Endoscopic Ultrasound Features of Intrapancreatic Metastases and the Role of Multiparametric Imaging and EUS-Guided Sampling in Rare Pancreatic Tumors

“…In the surgical resection statistics, only individual cases are reported that do not allow generalization [32,34,35,37,40,48,49]. A few case reports are described [113][114][115][116]. Mostly, these are patients who have already undergone surgery for other metastases.…”