Abstract
PurposeAngiosarcoma (AS) is an aggressive malignant tumour of endothelial origin, most frequently compelling the skin. The kidney is a rare site of primary occurrence of AS with a quite challenging diagnosis and poor prognosis, mainly due to its raw metastasising power. We described our first case of AS treatment. A comprehensive literature review in this field is also carried out.Patients and methodsWe report the case of a 56-years-old man with radiological appearance of an 11 cm poor vascularised left renal mass. The pre-operative imaging ruled out distant metastases and inferior vena cava thrombosis. Thus, after radioembolization, we performed a transperitoneal open radical nephrectomy with regional lymphadenectomy with the removal of the middle portion of the ileo-psoas muscle, followed by two lines of adjuvant chemotherapy. We analysed all the papers concerning primary kidney AS until December 2018.ResultsEstimated intraoperative blood loss was 200 mL with an operative time of 100 minutes. No postoperative complications were recorded, with the patient discharge in the 8th postoperative day. The pathological assessment showed a pT3a N0, M0 tumour compatible with AS of the kidney. An immune-histochemical study showed a vascular positive for CD31, CD34, Fli-1 with a Ki-67/Mib 1 of 50%. One month after surgery the patient began adjuvant chemotherapy with gemcitabine. After 45 days from surgery, a Computed Tomography scan showed lung and liver metastases with disease relapse in the left kidney lodge. The response to the first-line treatment was excellent, unlike the second-line, encountering a chemo-refractory disease. The cancer-specific survival was of 15 months. We included 66 cases in our review.ConclusionThis is the first case of treatment of kidney AS performed at our Institute. Our findings added new information about the unclear biology and progression of this subset of tumours, demonstrating an abysmal prognosis.
Highlights
Angiosarcoma (AS) is a rapid growth tumour of endothelial origin, which accounts for less than 2% of soft tissue sarcomas [1]
We included 66 cases in our review. This is the first case of treatment of kidney AS performed at our Institute
The immune-histochemical study showed strongly and diffusely positive staining for CD31 (Figure 3b), CD34, Fli-1, and remained harmful for pan-cytokeratin, MelanA, HMB45, PGM1, PAX8, GATA3, CD10, racemase, p63, smooth muscle actin, desmin, HHV8; Ki-67/ Mib 1:50%. These findings indicated that the tumour was an AS of the kidney
Summary
Angiosarcoma (AS) is a rapid growth tumour of endothelial origin, which accounts for less than 2% of soft tissue sarcomas [1]. Most cases happen in older white men with an extremely high mortality rate mainly due to distant metastasis at the time of diagnosis or shortly afterwards. According to the World Health Organization (WHO), mean survival of 7.7 months was met [2]. Endothelial cells are considered the source of AS, but an origin from circulating stem cells recruited from bone marrow or extra-medullary hematopoiesis is argued [3, 4]. The skin accounts for approximately one-third of cases, especially in the head and neck region or the extremities [5]. Regardless of the primary organ affected, the five years overall survival rate is estimated to be 10-35%, with
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