Angiosarcoma of the Spleen and traumatic splenic laceration

Clinical History

A 40-year-old male presented with abdominal pain and dizziness 2 days after a casual trauma, while he was practicing canoeing. His medical history included a testicular neoplasm (non-seminomatous germ cell tumour) with surgery and chemotherapy 15 years ago. The last control CT, performed 6 months ago, showed no pathology.

Imaging Findings

US revealed intraperitoneal particulate free fluid (haemoperitoneum), an enlarged spleen with ill-defined, heterogeneous masses with increased flow on Doppler images and nodular areas of decreased echogenicity, as well as a splenic laceration and hepatic hypoechoic nodules (Fig.1).
Contrast enhanced CT showed splenomegaly, multiple splenic masses that ranged in size from 3 mm to 33 mm, with little or no enhancement, that almost completely replaced the spleen, a splenic laceration, intraabdominal free fluid with densities of blood, and new hypodense hepatic nodules (Fig.2).
After splenectomy, the diagnosis of angiosarcoma was made with histology. CT was repeated after 40 days and revealed peritoneal carcinomatosis and increased number of the hypodense liver metastases, suggesting fulminant disease progression (Fig.3).

Discussion

Angiosarcoma of the spleen is a very rare neoplasm, but represents the most common primary nonlymphoid malignant tumour of the spleen [1, 2]. The pacient age range is 50-79 years [1]. In some case reports has been described an association with previous chemotherapy for lymphoma and radiation therapy for breast cancer [2, 3]. Unlike primary hepatic angiosarcoma, splenic angiosarcoma has no documented association with exposure to chemicals, such as vinyl chloride, arsenic or thorium dioxide [1, 2, 4]. Abdominal pain, fever, malaise and weight loss are usually the most common symptoms [1]. Spontaneous splenic rupture is a common complication, in up to 30% of patients [1, 2, 4-6]. In most cases the spleen is enlarged and contains haemorrhagic nodules [1]. Diffuse infiltration of the spleen is frequent and solitary masses are less common [1, 2]. Splenic angiosarcoma arises from splenic sinus endothelial cells [1, 2].
Splenomegaly is almost always identified [1].
On sonograms, these lesions usually appear as multiple heterogeneous masses with cystic areas –areas of necrosis and haemorrhage. The solid regions of the tumour can show increased Doppler flow [1, 2].
On CT images, splenic angiosarcomas are depicted as nodular masses (solitary mass, multiple masses or replacement of spleen by mass), ill-defined, with irregular margins and heterogeneous low attenuation [1, 2, 8]. May be present peripheral enhancement similar to hepatic hemangiomas [1, 7-9], calcifications [1, 2], metastases (liver metastases generally hypervascular [1]), haemoperitoneum [1, 2, 8].
On MR images there is a variable appearance due to haemorrhage and necrosis as well as heterogeneous enhancement [2].
Prognosis is poor (the majority of the patients die within 1 year of diagnosis) [1, 2, 8], with early metastases: liver (70%), lungs, pleura, bones, lymphatic system and brain [1, 2]. Angiosarcoma can be associated with synchronous malignant pathology, such as non-Hodgkin's lymphoma, colon and breast carcinomas [5, 8].
Surgical resection is always performed; chemotherapy is not effective [2, 4].
The diagnosis can be made using the fine needle aspiration biopsy. Large needle biopsies have a higher risk of haemorrhage among patients with refractory thrombocytopenia [1, 10].

Differential Diagnosis List

Final Diagnosis

Angiosarcoma of the Spleen and traumatic splenic laceration

URL:	https://www.eurorad.org/case/12994
DOI:	10.1594/EURORAD/CASE.12994
ISSN:	1563-4086