Pulmonary Vascular Disease |

Achieving Early Diagnosis of Pulmonary Tumor Thrombotic Microangiopathy: A Case Series FREE TO VIEW

Rohit Godbole, MD; Alexander Zider, MD; Jaime Betancourt, MD; Rajan Saggar, MD; Nader Kamangar, MD
Author and Funding Information

VA Greater Los Angeles Healthcare System (GLA), Los Angeles, CA

Chest. 2015;148(4_MeetingAbstracts):1003A. doi:10.1378/chest.2276141
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SESSION TITLE: Venous Thromboembolism Posters

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 28, 2015 at 01:30 PM - 02:30 PM

PURPOSE: Pulmonary tumor thrombotic microangiopathy (PTTM) is a disease entity caused by the embolization of tumor cells into the pulmonary vasculature causing severe pulmonary hypertension. PTTM has a poor prognosis and our purpose is to assess the feasibility of an early clinical diagnostic approach.

METHODS: We conducted a retrospective review of seven confirmed cases of PTTM from three institutions in Los Angeles.

RESULTS: All seven patients died. The average time from the initial clinical evaluation to death was 27.5 days (range 1-112 days). The diagnosis of PTTM was made ante-mortem in four cases once the primary malignancy was identified. For the patients with an ante-mortem diagnosis, the time from the initial clinical evaluation to the identification of the primary malignancy was, on average, 32.5 days (median 11.5 days, range 5-102 days) while the time from the initial clinical evaluation to death was, on average, 40 days (median 18.5 days, range 10-112 days). Four of the seven patients had a post-mortem examination (Group A) while three did not (Group B). Comparing these two groups, the presenting symptoms were similar in both. The duration of the symptoms prior to the initial clinical evaluation was, on average, 47.5 days in Group A and 44.5 days in Group B. Both groups had similar examination findings and abnormalities on the plain chest radiography and thoracic CT. There was severe pulmonary hypertension by 2D echocardiogram in both groups with a mean pulmonary artery systolic pressure of 83 mmHg (range 51-140 mmHg) in Group A and 69 mmHg (range 58-86 mmHg) in Group B. Six of the seven patients, three in each group, were diagnosed with gastric adenocarcinoma.

CONCLUSIONS: PTTM should be suspected if the following features are present: (1) symptoms of cough and dyspnea; (2) examination findings of tachycardia, hypoxia, and diffuse rales; (3) imaging showing pulmonary nodules, interlobular septal thickening, and mediastinal and hilar lymphadenopathy; and (4) severe pulmonary hypertension on 2D echocardiogram. Lung pathology is often sought for the classic features of vascular fibrointimal thickening and tumor emboli. We suggest that PTTM may be reliably diagnosed early using the aforementioned criteria and once the primary malignancy is identified, without requiring additional lung tissue. The reliability of the diagnosis increases if the primary malignancy is a gastric adenocarcinoma.

CLINICAL IMPLICATIONS: The approach to expediting the diagnosis of PTTM may facilitate the early initiation of treatment.

DISCLOSURE: The following authors have nothing to disclose: Rohit Godbole, Alexander Zider, Jaime Betancourt, Rajan Saggar, Nader Kamangar

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