Splanchnic vein
thrombosis (SVT), which includes portal, mesenteric, and splenic vein
thrombosis and the
Budd-Chiari syndrome, is an infrequent manifestation of
venous thromboembolism (VTE). Like typical site VTE, SVT is also frequently associated with
cancer, particularly intra-abdominal solid
malignancies and myeloproliferative
neoplasms (MPNs). The clinical presentation of SVT is nonspecific. Symptoms may be related to the underlying
malignancy, and
thrombosis is incidentally diagnosed by imaging studies for
cancer staging or follow-up in a substantial proportion of cases. The occurrence of SVT predicts worse prognosis in patients with liver or
pancreatic cancer and, not uncommonly, SVT may precede the diagnosis of
cancer. Therefore, the occurrence of an apparently unprovoked SVT should prompt careful patient evaluation for the presence of an underlying
malignancy or MPN.
Cancer patients carry a high risk of VTE extension and recurrence and long-term
anticoagulant treatment is suggested in the absence of high risk of
bleeding. Either
LMWH or direct oral
anticoagulants (DOACs) are suggested for the treatment of patients with
cancer-related SVT, although limited experience is available on the use of DOACs in these settings.
Vitamin K antagonists (VKAs) are suggested for the short and long-term treatment of SVT associated with MPN. This review outlines the epidemiological aspects, pathogenesis, risk factors, and diagnosis of
cancer-associated SVT, and addresses questions regarding the management of this challenging condition.