Fatal pulmonary tumour thrombotic microangiopathy in patient with ovarian adenocarcinoma: review and a case report

Abstract

Background Pulmonary tumour thrombotic microangiopathy (PTTM) is a fatal disease in which tumour cells embolize to the pulmonary vasculature leading to pulmonary hypertension and right heart failure. Early diagnosis is essential for timely treatment which can reduce intimal pulmonary vascular proliferation and prolong survival, improve the symptoms. Due to rare occurrences and no clear diagnostic guidelines the disorder usually is found post-mortem. We present a review of this rare disease and a case of post-mortem diagnosed pulmonary tumour thrombotic microangiopathy in a young female. Case presentation 51 years old woman presented with progressively worsening dyspnea, right ventricular failure signs and symptoms. Computerized tomography denied pulmonary embolism. 2D transthoracic echocardiography demonstrated right ventricle dilatation and dysfunction, severely increased systolic pulmonary pressure. Right heart catheterization revealed pre-capillary pulmonary hypertension with mean pulmonary artery pressure of 78 mmHg, pulmonary wedge pressure of 15 mmHg, reduced cardiac output to 1.78 L/min with a calculated pulmonary vascular resistance of 35 Wood units, and extremely low oxygen saturation (26%) in pulmonary artery. Because of worsening ascites, pelvic magnetic resonance imaging was performed, tumours in both ovaries were diagnosed. Due to the high operative risk, detailed tumour diagnosis surgically was not established. The patient developed progressive cardiorespiratory failure, unresponsive to optimal heart failure drug treatment. A postmortem morphology analyses revealed tumorous masses in pre-capillary lung vessels, right ventricle hypertrophy, ovary adenocarcinoma. Conclusions An early diagnosis of PTTM is essential. Most cases are lethal due to respiratory failure progressing rapidly. Patients with a history of malignancy, symptoms and signs implying of PH should be considered of having PTTM. If detected early enough, combination of chemotherapy with specific PH therapy is believed to be beneficial in reducing intimal proliferation and prolonging survival, along with improving the symptoms.

Pulmonary tumor thrombotic microangiopathy: Two case reports and literature review

“…The search identified 48 eligible clinical articles with 59 cases of PTTM reported nationally and internationally, the remaining 14 articles were excluded due to recurrence or as they were review articles. Among them, 9 Chinese PTTM articles [13–21] were reported 16 cases; 39 English PTTM articles [1,22–59] were reported 43 cases.…”

Chemotherapy-Associated Thrombotic Microangiopathy

“… 30 Cancer-associated TMA: TMA as a direct effect of cancer was historically considered rare, with cases usually occurring in association with mucinous adenocarcinoma. 58 , 59 Recent data suggest that it may be a more common cause among TMAs. Malignancies were deemed the culprit in 19% of cases of TMA in a recent retrospective review of over 500 patients hospitalized with TMA.…”

Pulmonary Tumor Thrombotic Microangiopathy in a Patient with Rapid Progressive Triple-Negative Breast Cancer

“…Activation of the coagulation system on the surface of the pulmonary embolic tumor clot and intimal proliferation is a syndrome called pulmonary tumor thrombotic microangiopathy (PTTM), which is a rare and severe complication and can lead to pulmonary hypertensive arteriopathy, first described in 1990 by von Herbay et al [3,4]. Typical symptoms include cough, dyspnea, and other signs of right heart failure [5,6]. Antemortem diagnosis is difficult due to nonspecific symptoms and lack of clear radiological findings.…”