key: cord-0945780-rlkkpjjk
authors: Weijmans, Maaike; Vermeulen, Rosa; Kleijwegt, Fleur S.; Boiten, Henk-Jan
title: An abdominal mass with thrombosis due to inferior vena cava agenesis mimicking lymphoma
date: 2021-08-02
journal: Ann Hematol
DOI: 10.1007/s00277-021-04626-5
sha: 7a97cfe1c38d96d5fc507db439eeb9141a410d91
doc_id: 945780
cord_uid: rlkkpjjk

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these findings were consistent with a diagnosis of agenesis of the infrarenal inferior vena cava (IVC).

The etiology by which central vein obstruction occurs can be classified as thrombotic and those that are nonthrombotic. Thrombotic etiologies include inherited thrombophilia, acquired hypercoagulable conditions, immobilization, recent surgery, and testicular tumors. Non-thrombotic obstruction can be categorized into extrinsic (e.g., compression by a tumor, cyst, or aneurysm) and intrinsic causes (e.g., intraluminal device). Although rare, agenesis of the IVC has been reported previously [1] [2] [3] [4] [5] .

Agenesis of the IVC (IVCA), a type of IVC anomaly, is estimated to occur in 0.0005-1% of the general population [6] . The embryologic development of the infrarenal IVC is complex deriving from supracardinal veins approximately 6 weeks after conception. It is interesting that our patient presented with thrombosis at the age of 34 years without a history of thrombosis. In a report of 10 patients with IVCA [7] , venous thrombosis followed intense physical activity in 8 patients, as was the case in our patient who was active in bicycle racing. We hypothesize that the collaterals (who developed before the venous thrombosis) were unable to assure the increased blood flow due to major physical activity, thereby generating venous stasis and thrombosis.

Due to inadequate drainage of venous blood, IVCA is a risk factor for deep venous thrombosis (DVT). In young (< 40 years old) patients with IVCA, the rate of DVT is relatively high (5%) compared to that of the same patients without IVCA (0.5%) [8] . In a study of 97 patients with DVT, a total of 31 patients showed thrombotic occlusion of iliac veins. Five of the 31 patients had an anomaly of the IVC of which only one patient had IVCA [9] .

IVCA is a rare but important congenital condition that predisposes to thrombosis. This diagnosis should be considered in the young especially in active male patients presenting with a clinical picture similar to that of the inherited thrombophilias.

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Congenital agenesis of inferior vena cava: a rare cause of unprovoked deep venous thrombosis

Pyrexia of unknown origin: inferior vena cava agenesis

IVC agenesis: a rare cause of deep vein thrombosis

Recurrent deep venous thrombosis in a patient with agenesis of inferior vena cava

The typical presentation spectrum of deep vein thrombosis associated with inferior vena cava malformations

Absence of the retrohepatic inferior vena cava: what the surgeon should know

Inferior vena cava agenesis and deep vein thrombosis: 10 patients and review of the literature

Congenital absence of the inferior vena cava: a rare risk factor for idiopathic deep-vein thrombosis

Anomalies of the inferior vena cava in patients with iliac venous thrombosis