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.

Keywords: Ovary adenocarcinoma; Pulmonary tumour thrombotic microangiopathy; Right heart failure.

Fig. 1.

2D Doppler echocardiography—four chambers apical view A, B, C, D. Revealing tricuspid regurgitation, maximal tricuspid regurgitation velocity (TR Vmax)—4.1 m/s indicating pulmonary hypertension, right heart enlargement and reduced RV free wall longitudinal function (S’—8.0 cm/s)

Fig. 2

Reconstruction of computerized tomography pulmonary angiography (CTPA). Axial CT angiography scans: A the dilatation of the pulmonary artery trunk and both main pulmonary arteries with no filling defects within pulmonary vasculature. B The dilatation of right ventricle (RV), atrium (RA) and pericardial effusion (black arrow). C multiple perivascular areas of hyperattenuation in keeping with PH. Note the increased segmental artery-to-bronchus ratio up to 2,5:1

Fig. 3

Postmortem histological evaluation results (hematoxylin and eosin staining). A Microscopic findings in left ovary with low-grade serous carcinoma—moderately atypical cells with round chromatic nuclei and moderate amount of pale eosinophilic cytoplasm are observed; tumor cells form solid pattern and some primitive papillary structures. B, C Microscopic findings in pulmonary tissue—multiple veins and lymphatic vessels, some of these vascular lumens are obliterated by tumorous thrombi consisting of ovarian tumor`s structures (picture B); dilated vein with tumorous thrombus which is partially recanalized (picture C). D Microscopic findings in thyroid gland tissue—similar to those observed in pulmonary tissue