A CASE REPORT OF METASTATIC ANGIOSARCOMA OF THE SPLEEN

¹Stefanovski PD, ¹Buonadonna A, ²DePaoli A, ¹Bearz A, ¹Berretta M, ³Mazzucato M, ¹Libra M, ⁴Balestreri L, ¹Frustaci S, ¹Division of Medical Oncology, ²Division of Radiation Therapy, ³Blood Bank Service, ⁴Division of Radiology. Centro di Riferimanto Oncologico, Aviano, Italy.

Background: Just over 100 well-documented cases of primary spleen angiosarcoma have been reported since the first description by Langhans in 1879. We report an additional case of metastatic primary spleen angiosarcoma treated with an aggressive chemotherapeutic approach integrated with a planed splenectomy at the time of a substantial response.

Case report: A 28-year-old man was referred to our Institute with asthenia, fever (37-38^oC) and bilateral hypochondrial pain. Clinical hepatosplenomegaly was confirmed by abdominal ultrasound and MR imaging showing central spleen mass with multiple liver lesions. A laparoscopic hepatic tumor biopsies showed angiosarcoma. Laboratory investigations at admission and during the treatment are present at Figure 1. We administered Epirubicin 30mg/m² and Ifosfamide 1500mg/m² for 2 days repeated every 14 days with G-CSF support at 1st, 2nd, 4th and 5th cycle and Epirubicin 60mg/m² for 2 days and Ifosfamide 3000mg/m² for 3 days with G-CSF support at other chemotherapy cycles (Figure 1). Splenectomy was performed on day 64 and diagnosis was poorly differentiated angiosarcoma. After 8 chemotherapy cycles we obtained good partial response at the liver level, complete normalization of the serological markers, XDP products and complete disappearance of any sign and symptom of the disease.

Conclusions: Even in patients with metastatic disease, splenectomy should always be considered and integrated in a multidisciplinary approach with intensive chemotherapy regimens because spleen rupture is the most important adverse prognostic factor determining a very short survival (median 4.4 months after spontaneous spleen rupture). Further approaches are necessary in order to control the chemoresistant clones and anti-angiogenetic agents could be a rationale tool.