Pulmonary tumor thrombotic microangiopathy and pulmonary veno-occlusive disease in a woman with cervical cancer treated with cediranib and durvalumab

• Published in: BMC pulmonary medicine Vol. 18; no. 1; pp. 112 - 7
• Main Authors: Suffredini, Dante A., Lee, Jung-Min, Peer, Cody J., Pratt, Drew, Kleiner, David E., Elinoff, Jason M., Solomon, Michael A.
• Format: Journal Article
• Language: English
• Published: London BioMed Central 11.07.2018; BioMed Central Ltd; BMC
• Subjects: Analysis; Angiogenesis; Antibodies, Monoclonal - therapeutic use; Autopsy; Cancer therapies; Carcinoma, Squamous Cell - drug therapy; Carcinoma, Squamous Cell - pathology; Case Report; Cediranib; Cervical cancer; Cervix; Clinical trials; Critical Care Medicine; Diagnosis; Dosage and administration; Drug therapy; Durvalumab; Dyspnea; Fatal Outcome; Female; Heart failure; Heart rate; Humans; Immunotherapy; Intensive; Internal Medicine; Ligands; Lung - blood supply; Lung - pathology; Lung - physiopathology; Lung cancer; Lung diseases; Lung Neoplasms - complications; Lung Neoplasms - secondary; Medicine; Medicine & Public Health; Metastasis; Middle Aged; Monoclonal antibodies; Neoplastic Cells, Circulating - pathology; PD-1 protein; PD-L1 protein; Pneumology/Respiratory System; Pulmonary arteries; Pulmonary hypertension; Pulmonary tumor thrombotic microangiopathy; Pulmonary Veno-Occlusive Disease - etiology; Pulmonary Veno-Occlusive Disease - pathology; Pulmonology; Quinazolines - therapeutic use; Respiratory critical care; Risk factors; Targeted cancer therapy; Thrombosis; Thrombotic Microangiopathies - etiology; Thrombotic Microangiopathies - pathology; Thrombotic microangiopathy; Tumors; Uterine Cervical Neoplasms - drug therapy; Uterine Cervical Neoplasms - pathology; Vascular endothelial growth factor; Veno-occlusive disease; Women's health; Pulmonary tumor thrombotic microangiopathy; Durvalumab; Pulmonary hypertension; Cediranib; Cervical cancer
• ISSN: 1471-2466; 1471-2466
• DOI: 10.1186/s12890-018-0681-x

Background Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare cause of pulmonary hypertension that is associated with malignancies and is marked by the presence of non-occlusive tumor emboli and fibrocellular intimal proliferation of small pulmonary arteries leading to increased pulmonary vascular resistance and right heart failure. The diagnosis of PTTM is challenging to make pre-mortem and guidelines on treatment are lacking. Case presentation A 45-year-old woman with advanced squamous cell carcinoma of the cervix developed symptoms of dyspnea and evidence of right heart failure during a phase I clinical trial with cediranib and durvalumab. After an extensive evaluation, pre-capillary pulmonary hypertension was confirmed by right heart catheterization. Vasodilator therapy was initiated but resulted in the development of symptomatic hypoxemia and was discontinued. Despite continued supportive care, she continued to decline and was transitioned to hospice care. At autopsy, the cause of her right heart failure was found to be due to PTTM with features of pulmonary veno-occlusive disease (PVOD). Conclusion PTTM and PVOD are important diagnoses to consider in patients with a malignancy and the development of right heart failure and may be manifestations of a spectrum of similar disease processes.