DescriptionWe present the case of a 75-year-old woman referred for a liver biopsy. In her medical history, she had hypertension, well controlled with an ACE-inhibitor and a beta-blocker. Seven months prior to referral, she started to have B symptoms, including fatigue, weight loss and anorexia. A CT scan showed multiple splenic and hepatic nodules. Additional MRI imaging was most suggestive for multiple haemangiomata. A CT scan of the lumbar vertebral column three months later because of back pain revealed multiple new bone lesions that were suspect for bone metastases or multiple haemangiomata on subsequent MRI imaging. Her primary physician referred her to the haematology department. The work up for multiple myeloma and lymphoma with a lumbar puncture was negative. A PET-CT showed FDG-positive lesions in spleen and liver, and she was referred to our gastro-enterology service for a biopsy of a hepatic FDG-positive lesion. At the time of admission, there was progressive hepatic failure with increased bilirubin and INR and with low platelets and hyponatremia. Tumour markers CEA, CA 19.9, alfa-foetoprotein were normal. Immunohistochemical and histopathological results confirmed a metastatic lesion originating from a primary splenic angiosarcoma. She was unfit for chemotherapy because of rapidly progressive hepatic failure. There was clinical deterioration with fatal outcome shortly after.
Splenic angiosarcoma is a rare type of sarcoma with an annual incidence of 0.2/million. However, it remains the most common primary non-lymphoid, non-hematopoietic malignant tumour of the spleen. There is a very high metastatic risk of 69-100%, primarily to the liver. There are no specific diagnostic clinical or biochemical signs. Differentiation on imaging is challenging since imaging shows an overlap with other (benign) vascular lesions such as haemangiomata. Histopathological and immunohistochemical tissue analysis is therefore essential for diagnosis, but biopsies obtained by punction may lead to seeding of the tumour cells and rapid development of metastases. A splenic angiosarcoma has a very poor prognosis with a median overall survival of 5-6 months. In early stages, splenectomy can prolong median survival to 14 months. So, whenever possible splenectomy is advised for both diagnostic and therapeutic goals. Chemotherapy is often based on regimens for other (angio)sarcomas, but none have shown a significant effect on overall survival. However, randomised prospective studies in this setting are not feasible because of the aggressive character and rarity of the disease. Based on imaging, the lesions in our patient were too quickly labelled as being benign haemangiomata. She developed bone metastasis and hepatic failure during the diagnostic delay of seven months.
The key points of this case are to remember that B symptoms and rapidly evolving lesions of the spleen and liver should always be alarming even if they look like benign lesions on imaging modalities. Histology is essential in diagnosis since radiographic differentiation between a benign haemangioma and angiosarcoma is often impossible. Splenic angiosarcoma is rare but sometimes we have to look for the zebra in a herd of horses.
|28 feb 2023
|Belgian week of Gastro-Enterology
|Mate van erkenning