Pulmonary tumor thrombotic microangiopathy: A systematic review of the literature. Issue 1 (2nd January 2021)

• Record Type: Journal Article
• Authors: Morin-Thibault, L. V.; Wiseman, D.; Joubert, P.; Paulin, R.; Bonnet, S.; Provencher, S.
• Is Part Of: Canadian journal of respiratory, critical care, and sleep medicine =. Volume 5:Issue 1(2021)
• Page Start: 20
• Page End: 27
• Publication Date: 2021-01-02
• Subjects: Cancer -- pulmonary hypertension -- pulmonary tumor emboli -- pulmonary tumor thrombotic microangiopathy; Lungs -- Diseases -- Periodicals; Critical care medicine -- Periodicals; Sleep apnea syndromes -- Periodicals; 616.2005
• DOI: 10.1080/24745332.2020.1724061
• Languages: English
• ISSNs: 2474-5332

Abstract

Abstract: BACKGROUND: Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare complication of cancer that was described almost 30 years ago. However, a minimal amount is known about this condition making pre-mortem diagnosis and treatment difficult. OBJECTIVES: We aimed to systematically review and summarize the epidemiology, clinical manifestations, radiologic and hemodynamics features, diagnostic procedures and outcomes of PTTM. METHODS: MEDLINE was searched (January 1990–November 2019) for case reports on PTTM. RESULTS: A total of 132 studies were included (217 cases), including 126 case reports and 6 autopsy series. Gastric cancer (53%) and adenocarcinoma (87%) were the most common site of cancer and histological subtype, respectively. Mean age at presentation was 56 ± 16 years old. Most patients reported dyspnea, although symptoms were variable and nonspecific. Pulmonary hypertension was frequently documented either by transthoracic echocardiogram (97/103) or right heart catheterization (39/39). The diagnosis was made antemortem in only 29 patients using open lung biopsy (n = 12), transbronchial biopsy (n = 14) and CT-guided needle biopsy (n = 1), pulmonary microvascular cytology (n = 6); whereas, clinical presentation was suggestive of PTTM in 2 patients. Anticoagulation (n = 39), pulmonary hypertension-targeted treatments (n = 27), chemotherapy (n = 21), imatinib (n = 6) and thrombolysis (n = 5) were used as treatment strategies. Prognosis was poor with most patients dying within days or weeks of their admission. CONCLUSIONS: PTTM is a rare syndrome that manifests as progressive dyspnea, pulmonary hypertension and right heart failure. PTTM is associated with a poor prognosis. Transbronchial biopsy and pulmonary microvascular cytology may represent alternatives to open lung biopsy to achieve an antemortem diagnosis of PTTM, although the best treatment strategy remains elusive.