Introduction

Primary splenic angiosarcoma (PSA) is a very rare vascular neoplasm, even though it is the most common non hematolymphoid malignant tumor of the spleen. Prognosis is poor and almost all patients die within 1 year of diagnosis. It is found more frequently in older patients (sixth or seventh decade of life), with an annual incidence of one case per four million persons and there is no gender predilection [1].

The symptoms and signs of PSA are not specific, and they include fever, fatigue, weight loss, left-sided abdominal or chest pain; hematologic disorders and splenomegaly are almost always identified. On contrast-enhanced computed tomography (CT) scan, the PSA shows up as an ill-defined splenic mass with heterogeneous contrast enhancement and areas of necrotic degeneration [2]. Spontaneous rupture is a known complication in up to 30% of patients, in these cases patients with PSA may also present with signs and symptoms of hemoperitoneum [3].

PSA can metastasize and the most affected sites of distant metastases are liver, lungs, bone marrow, and lymphatic system.

Due to the rarity of this malignancy, the optimal treatment of PSA remains under investigation. The initial treatment frequently involves splenectomy in order to avoid splenic rupture while the role of adjuvant chemotherapy is unknown [4]. Embolization is not common but may be useful in stabilizing hemodynamics [5].

Case presentation

A 69-year-old healthy female, with a negative medical history for serious illness, presented with left-sided abdominal pain for few days. The patient denied abdominal trauma, fevers and significant family history. Clinical examination revealed splenomegaly, pallor and signs of abdominal tenderness; however, the patient was hemodynamically stable. Initial laboratory work-up revealed anemia (Hb 9 g/dL; normal range, 11.6–14.8 g/dL), thrombocytosis (platelet count, 918 × 10ˆ3/μl; normal range, 158–348 × 10ˆ3/μL) and normal white blood cell count. The ultrasound examination (US) of the abdomen showed free intraperitoneal fluid and splenomegaly with inhomogeneous parenchymal echotexture due to areas of cystic appearance with internal echoes. The presence of free intraperitoneal fluid led us to perform an emergency abdominal contrast-enhanced CT that confirmed the splenomegaly and showed the presence of hemoperitoneum and a splenic mass with areas of necrotic degeneration and heterogeneous contrast enhancement (Fig. 1). No other abdominal lesions were described. Due to the sudden worsening of the patient’s clinical conditions (Hb 7 g/dl) and the unavailability of the operating room, embolization of the splenic artery was performed, under suspicion of splenic rupture, as a bridge to the subsequent splenectomy. Under local anesthesia, the right femoral artery was punctured, and a 5-Fr catheter was inserted in the right femoral artery. Celiac arteriography and selective splenic arteriography (Fig. 2) demonstrated inhomogeneous splenic perfusion, without any contrast extravasation. Therefore, proximal splenic artery embolization was performed, by using 0.035’’steel coils and 0.020’’ platinum coils, achieving a significant flow reduction in the main splenic artery (Figs. 3, 4). Following the emergency vascular embolization, elective surgery was performed the following day without significant blood loss. The findings of pathological examination of the spleen were suggestive of PSA and the patient was referred to an oncological center.

Fig. 1
figure 1

Portal phase CT scan shows splenomegaly, splenic mass with areas of necrotic degeneration, heterogeneous contrast enhancement and free peritoneal fluid

Full size image
Fig. 2
figure 2

Arteriography of the celiac trunk (A) and selective splenic arteriography (B)

Full size image
Fig. 3
figure 3

Arterial phase CT scan with splenic artery diameter (17.5 mm)

Full size image
Fig. 4
figure 4

Coil embolization of the splenic artery showing exclusion of splenic parenchyma from blood flow

Full size image

Discussion

PSA is a rare aggressive neoplasm that arises from the endothelial lining of splenic blood vessels and it is the most common non hematolymphoid malignant tumor of the spleen. The patient age range is 50–79 years at manifestation of symptoms and even though there is no gender predilection a slight male predominance is reported in the literature [1]. The etiology of PSA is unknown although some authors believe that PSA develops from previously existing benign tumors such as hemangiomas and lymphangiomas. These tumors with others such as littoral cell angioma, lymphangioma, hemangiopericytoma, lymphoid-origin neoplasia, and metastatic disease are included in the differential diagnosis of splenomegaly with a complex mass. Among metastatic disease, ovarian carcinoma is one of the more common primary tumors that metastasizes to the spleen and should be considered in the differential diagnosis [6].

The clinical presentation of PSA is often nonspecific making it difficult to detect early and this relates with a poor prognosis [4]. The symptoms generally include fever, fatigue, weight loss, upper abdominal pain, left flank pain, or chest wall pain. Laboratory evaluation may present anemia, leukocytosis, or thrombocytopenia. Splenic rupture is the most feared complication and may be the first sign of illness [3].

PSA may present as diffuse involvement of the spleen either as a single large lesion or as a large mass with multiple smaller nodules that could show central necrosis with a rim of soft tissue. At US examination, the necrotic central areas tend to appear cystic, with internal echoes secondary to debris or hemorrhage, while the soft-tissue rim appears heterogeneous in echotexture. Typically, on CT images, the PSA is poorly defined (60% of cases) and irregularly shaped, and it shows irregular heterogeneous enhancement on postcontrast images [7]. In particular on CT scan PSA often demonstrates a hypodense mass, which may be either homogeneous or heterogeneous [8].

Pathologic examination of tissue is necessary for the diagnosis of PSA. Percutaneous biopsy of a potentially malignant lesion remains challenging due to the possibility of tumor seeding along the needle tract and the risk of hemorrhage. Additionally, patients may have thrombocytopenia, which can further alter the ability to achieve hemostasis after the procedure. This knowledge will help the physician to choose the best technique and location for biopsy and to manage potentially catastrophic complications [9].

Frequently PSA presents with associated metastatic disease at the time of diagnosis. The most common sites of metastases involve liver and bone, specifically the vertebrae but also lungs, lymph nodes, gastrointestinal tract, brain, adrenals, abdominal wall, heart, and pancreas [10]. In patients presenting with widely metastatic disease, it is often impossible to identify the site of the primary origin of the tumor.

Due to the rarity of this malignancy, the optimal treatment of PSA remains unknown and systemic chemotherapy regimens have not been standardized. In general patients with PSA should undergo early splenectomy whenever possible to avoid splenic rupture [11]. Treatment of splenic rupture must take into account several factors including the etiology of the rupture, hemodynamic stability, patient status and volume of intraperitoneal bleeding.

Transcatheter arterial embolization (TAE) of splenic artery for PSA is safe and effective in maintaining the hemodynamic stability [12].

PSA can reach large dimensions due to its aggressive and often asymptomatic nature, increasing the risk of hemorrhagic complications during splenectomy. In such cases, TAE can minimize blood loss during surgery.

According to the literature, there are few cases of splenic artery embolization in patients with PSA, and all are followed by splenectomy [13]. Splenectomy is indeed the best treatment for PSA, but due to the poor prognosis of this pathology, it is exclusively diagnostic. Regardless of any treatment, the prognosis remains poor, with the average survival interval in patients with PSA being about six months from diagnosis.

Conclusion

PSA is a rare tumor with a nonspecific clinical presentation, making its diagnosis and treatment challenging.

Splenic artery embolization is uncommon but can be useful for maintaining hemodynamic stability in patients with splenic rupture due to the neoplastic mass. TAE can also reduce blood loss during splenectomy, minimizing hemorrhagic complications.

However, surgery remains the treatment of choice for PSA, usually in combination with adjuvant chemotherapy and radiation therapy [4].