A 36-year-old woman presented to the Emergency Department (ED) with left shoulder pain lasting for 3 months, followed by diffuse abdominal pain, both refractory to analgesia. The physical examination showed no alterations in the shoulder, but it was noticed an exuberant hepatomegaly and a hard and tender splenomegaly.

Thoracoabdominal computed tomography (Fig. 1) showed a splenic heterogeneous mass of 14 centimeters with signs of intracapsular hemorrhage, and hepatomegaly with uncountable nodules histologically compatible with splenic angiosarcoma.

She started chemotherapy with doxorubicin, dacarbazine, ifosfamide and etoposide followed by paclitaxel and goserreline with reduction of tumor volume (Fig. 2), with splenectomy now a therapeutic hypothesis. The patient remains alive 10 months after the onset of symptoms.

Splenic angiosarcoma is a rare and rapidly fatal tumor, most often presenting with spontaneous splenic rupture. No consensual or effective treatment is known to date^1,2 however, combination therapies with paclitaxel may be the future^3,4.

Figura I

Figure 1- Splenic heterogeneous mass of 14 centimeters (arrow) with signs of intracapsular hemorrhage (arrow head), and hepatomegaly with numerous metastases (star

Figura II

Figure 2- Reduced volume of splenic angiosarcoma (arrow) after chemotherapy, surrounded by normal spleen, and hepatomegaly with numerous metastases (star)

  BIBLIOGRAFIA  

1- Deng R, Chang W, Wu X, et al. Primary splenic angiosarcoma with fever and anemia: a case report and literature review. International journal of clinical and experimental pathology. 2015;8(11):14040–14044.

2- Coppola S, Leva A, Pagni F, et al. Demanding Diagnosis of Splenic Angiosarcoma as Cause of Delayed Treatment of Spontaneous Splenic Rupture: A Case Report and Literature Review. Case reports in surgery. 2017.

3- Vakkalanka, Bhanu, and Mohammed Milhem. Paclitaxel as neoadjuvant therapy for high grade angiosarcoma of the spleen: a brief report and literature review. Clinical Medicine Insights. Oncology. 2010;4:107–110

4- Ferreira BP, Rodler ET, Loggers ET, et al. Systemic therapy in primary angiosarcoma of the spleen. Rare Tumors. 2012;10; 4(4): e55.