Pulmonary tumor thrombotic microangiopathy

Last revised by Daniel J Bell on 24 Oct 2021

Pulmonary tumor thrombotic microangiopathy occurs when there are non-occlusive tumor cell microemboli with fibrointimal remodeling in small pulmonary arteries, veins and lymphatics. It rarely causes pulmonary hypertension.

At autopsy, approximately 25% patients with pulmonary tumor thrombotic microangiopathy are found to have an extrathoracic malignancy. Most commonly 6:

The diagnosis of pulmonary tumor thrombotic microangiopathy rests upon histological confirmation, most commonly from a lung biopsy 6

Progressive breathlessness is the key symptom of pulmonary tumor thrombotic microangiopathy.

Thrombotic microangiopathy is a consequence of tumor cells metastasizing to the distal tiny arterioles, venules and lymphatics of the lungs. The embolized cells induce an intravascular procoagulant effect resulting in fibrocellular overgrowth of the intima and vasoconstriction. Occasionally, this proceeds to overt pulmonary hypertension.

Structural imaging is usually unremarkable in pulmonary tumor thrombotic microangiopathy:

Pulmonary tumor thrombotic microangiopathy heralds a dismal prognosis. Although treatment of the primary tumor improves prognosis 6.

It was first described by Von Herbay et al. in 1990 2.

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