Primary renal angiosarcoma with progressive clinical course despite surgical and adjuvant treatment: A case report

Filiz Celebi,Kezban Nur Pilanci,Sezer Saglam,Numan Cem Balci

doi:10.3892/ol.2015.2902

Filiz Celebi, Kezban Nur Pilanci + Show 2 more

Open Access

https://doi.org/10.3892/ol.2015.2902

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Abstract

Angiosarcoma is an extremely rare, high-grade malignancy, which accounts for <2% of all soft-tissue sarcomas. Cases of primary renal angiosarcoma represent 1% of these. Angiosarcomas involving the kidney usually originate from metastatic skin lesions or primary visceral lesions and most often occur in the sixth and seventh decades of life. The present study describes a case of primary renal angiosarcoma that presented as a large right-sided renal mass with symptoms of flank pain. Despite surgical removal of the tumor, recurrent disease with associated lung metastases was identified at the surgical site following adjuvant chemotherapy. The patient succumbed to the disease 13 months after the diagnosis.

Highlights

Angiosarcoma is a rare malignant neoplasm that affects the endothelial‐type cells lining the vessel walls [1]
Visceral sarcomas are less common than soft‐tissue and skin sarcomas, and renal involvement is generally associated with metastasis [3]
We present the case of a patient with primary renal angiosarcoma with pleuropulmonary metastasis

Summary

Introduction

Angiosarcoma is a rare malignant neoplasm that affects the endothelial‐type cells lining the vessel walls [1]. Primary renal angiosarcoma usually affects Caucasians in the sixth and seventh decades of life. It presents with macroscopic hematuria (81%), pain in the flank (38%) or a palpable renal mass (31%) [4]. We present the case of a patient with primary renal angiosarcoma with pleuropulmonary metastasis. Radiological differential diagnosis concluded that the lesion was most likely renal cell carcinoma or a metastasis from another primary tumor. Immunohistochemical findings revealed that the neoplastic cells were highly positive for cluster of differentiation (CD) and CD34, which supported the diagnosis of primary renal angiosarcoma. The abdominal MRI scan revealed a heterogenous contrast‐enhanced necrotic mass measuring 8x9.5x12.5 cm (Fig. 2) in the right nephrectomy space, and a 6.5x11 cm pelvic metastatic mass. There was no response to three cycles of the antiangiogenic therapy and the patient succumbed to the disease 13 months after the initial diagnosis

Discussion

Findings

Prince CL