Abstract:Pulmonary tumor thrombotic microangiopathy (PTTM) is a disease process wherein tumor cells are thought to embolize to the pulmonary circulation causing pulmonary hypertension (PH) and death from right heart failure. Presented herein are clinical, laboratory, radiographic, and histologic features across seven cases of PTTM. Highlighted in this publication are also involvement of pulmonary venules and clinical features distinguishing PTTM from clinical mimics.We conducted a retrospective chart review of seven ca… Show more
“…No evidence of infection or pneumonia was identified. Collectively, these findings were most consistent with a primary diagnosis of PTTM with features of PVOD [ 1 , 4 ]. Fig.…”
BackgroundPulmonary 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 presentationA 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).ConclusionPTTM 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.
“…No evidence of infection or pneumonia was identified. Collectively, these findings were most consistent with a primary diagnosis of PTTM with features of PVOD [ 1 , 4 ]. Fig.…”
BackgroundPulmonary 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 presentationA 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).ConclusionPTTM 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.
“…Tumour cell nests are also evident in pulmonary veins and lymphatics [16,21,24], with evidence for lymphatic tumour invasion (figure 2c). Fibrointimal proliferation in pulmonary veins in PTTM lesions has been reported in several recent reports [21,31], and may be analogous to the remodelling of pulmonary veins seen in distal chronic thromboembolic PH (CTEPH) [32]. Putative interactions between tumour cells, macrophages and vascular cells are summarised in figure 3.…”
Tumoral pulmonary hypertension (PH) comprises a variety of subtypes in patients with a current or previous malignancy. Tumoral PH principally includes the tumour-related pulmonary microvascular conditions pulmonary tumour microembolism and pulmonary tumour thrombotic microangiopathy. These inter-related conditions are frequently found inpost mortemspecimens but are notoriously difficult to diagnoseante mortem. The outlook for patients remains extremely poor although there is some emerging evidence that pulmonary vasodilators and anti-inflammatory approaches may improve survival. Tumoral PH also includes pulmonary macroembolism and tumours that involve the proximal pulmonary vasculature, such as angiosarcoma; both may mimic pulmonary embolism and chronic thromboembolic PH. Finally, tumoral PH may develop in response to treatments of an underlying malignancy. There is increasing interest in pulmonary arterial hypertension induced by tyrosine kinase inhibitors, such as dasatanib. In addition, radiotherapy and chemotherapeutic agents such as mitomycin-C can cause pulmonary veno-occlusive disease. Tumoral PH should be considered in any patient presenting with unexplained PH, especially if it is poorly responsive to standard approaches or there is a history of malignancy. This article will describe subtypes of tumoral PH, their pathophysiology, investigation and management options in turn.
“…70,74 Although cough may be present in CTEPH, it is not a common symptom like in PTTM. 70 In more advanced cases, patients can present with decompensated heart failure. 70 On laboratory analysis, signs of microangiopathic hemolytic anemia like thrombocytopenia, anemia, elevated D-dimer, and elevated LDH may be seen.…”
Section: Sarcoidosismentioning
confidence: 99%
“…70 In more advanced cases, patients can present with decompensated heart failure. 70 On laboratory analysis, signs of microangiopathic hemolytic anemia like thrombocytopenia, anemia, elevated D-dimer, and elevated LDH may be seen. 70 The VQ scans of these patients typically show multiple symmetric peripheral subsegmental mismatched perfusion defects that are distributed evenly throughout the lungs.…”
Section: Sarcoidosismentioning
confidence: 99%
“…76 A Chest CT scan may show asymmetric intra and interlobular septal thickening that may be nodular in appearance secondary to lymphangitic infiltration by tumor cells or tumoral edema. 70,76 There may also be mediastinal or hilar lymphadenopathy or lung nodules, which may represent metastatic disease. A case series reported four patients with a 'dilated and beaded' appearance of the peripheral pulmonary arteries due to tumor emboli.…”
Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by mechanical obstruction of large pulmonary arteries secondary to one or more episodes of pulmonary embolism. Ventilation perfusion scan is the recommended initial screening test for this condition and typically shows multiple large mismatched perfusion defects. However, not all patients with an abnormal ventilation perfusion scan have CTEPH since there are other conditions that be associated with a positive ventilation perfusion scan. These conditions include in situ thrombosis, pulmonary artery sarcoma, fibrosing mediastinitis, pulmonary vasculitis and sarcoidosis, among others. Although these conditions cannot be distinguished from CTEPH using a ventilation perfusion scan, they have certain characteristic radiological features that can be demonstrated on other imaging techniques such as computed tomography scan and can help in differentiation of these conditions. In this review, we have summarized some key clinical and radiological features that can help differentiate CTEPH from the CTEPH mimics.
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