Pulmonary tumor thrombotic microangiopathy presenting as recurrent syncope

Pulmonary tumor thrombotic microangiopathy is a rare condition in which embolization of tumor cells to the pulmonary arterioles causes fibrocellular intimal thickening and activation of the coagulation cascade resulting in pulmonary hypertension and right heart failure. Herein, we highlight a young 35-year-old male with no known past medical history who presented with recurrent syncope and dyspnea, and was found to have severe right heart failure and pulmonary hypertension. He developed sudden clinical deterioration and died after a cardiac arrest. Autopsy revealed poorly differentiated gastric adenocarcinoma and pulmonary tumor thrombotic microangiopathy. New onset severe pulmonary hypertension and right heart failure without any other obvious etiology should encourage the reader to evaluate for pulmonary tumor thrombotic microangiopathy and undergo a diligent search for underlying malignancy. This case highlights recurrent syncope as a rare presentation of this rapidly fatal disease.

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Pulmonary Tumor Microemboli from Prostatic Adenocarcinoma Causing Acute Right Heart Failure and Circulatory Collapse; a Rare Presentation

10.1164/ajrccm-conference.2020.201.1_meetingabstracts.a7309 ◽

2020 ◽

Author(s):

K. Nunna ◽

P.B. Datar ◽

N.A. Hanania

Keyword(s):

Heart Failure ◽

Right Heart Failure ◽

Prostatic Adenocarcinoma ◽

Circulatory Collapse ◽

Right Heart ◽

Rare Presentation ◽

Pulmonary Tumor

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Fatal Microscopic Pulmonary Tumor Thromboemboli: Unusual Presentation of Occult Cervical Carcinoma. Two Case Reports and Review of the Literature

Academic Forensic Pathology ◽

10.1177/1925362119851131 ◽

2019 ◽

Vol 9 (1-2) ◽

pp. 81-92

Author(s):

Daniel Kirsch ◽

Irini Scordi-Bello

Keyword(s):

Heart Failure ◽

Pulmonary Hypertension ◽

Sudden Death ◽

Case Reports ◽

Metastatic Cancer ◽

Right Heart Failure ◽

Right Heart ◽

Pulmonary Tumor ◽

Advanced Tumor ◽

Tumor Emboli

Cor pulmonale (right heart failure) due to microscopic pulmonary tumor emboli (MPTE) can arise from a variety of malignancies including breast, lung, and liver and carries significant morbidity and mortality. Tumor cell aggregates spread hematogenously to the lungs and occlude small pulmonary vessels leading to pulmonary hypertension through either a mechanical process or inducing vascular remodeling as a downstream result of interactions between the embolus and the vessel wall. Its presentation includes unexplained dyspnea, hypoxemia, tachycardia, pulmonary hypertension, right heart failure, and in some cases sudden death. The symptoms may suggest the more common entity of pulmonary thromboembolism, particularly in the setting of previously known metastatic cancer; however, computed tomography scans may appear normal and ventilation-perfusion scans which are the preferable diagnostic modality are not always ordered. In most cases of MPTE, the presentation reflects metastasis of an already known and advanced tumor, but, in rare cases, it may be the primary manifestation of an occult malignancy. We present here 2 unique cases of MPTE in women with occult cervical cancer. In both cases, the malignancy was discovered and diagnosed at autopsy. Microscopic pulmonary tumor emboli can be easily overlooked, and therefore, forensic pathologists and other death investigators should be aware of it and trained to at least consider the possibility in appropriate situations. Thorough microscopic examination of apparently normal tissues may be necessary, particularly in cases of unexplained right heart failure and sudden death, even if the decedent has few or no identifiable risk factors for cancer.

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Insights on pulmonary tumor thrombotic microangiopathy: a seven-patient case series

Pulmonary Circulation ◽

10.1177/2045893217728072 ◽

2017 ◽

Vol 7 (4) ◽

pp. 813-820 ◽

Cited By ~ 8

Author(s):

Rohit Godbole ◽

Rajan Saggar ◽

Alexander Zider ◽

Jamie Betancourt ◽

William D. Wallace ◽

...

Keyword(s):

Heart Failure ◽

Los Angeles ◽

Early Diagnosis ◽

Thrombotic Microangiopathy ◽

Case Series ◽

Disease Process ◽

Heart Catheterization ◽

Right Heart ◽

Pulmonary Tumor ◽

Pulmonary Tumor Thrombotic Microangiopathy

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 cases of PTTM from hospitals in the greater Los Angeles metropolitan area. Patients in this series exhibited: symptoms of cough and progressive dyspnea; PH and/or heart failure on physical exam; laboratory abnormalities of anemia, thrombocytopenia, elevated LDH, and elevated D-dimer; chest computed tomography (CT) showing diffuse septal thickening, mediastinal and hilar lymphadenopathy and nodules; elevated pulmonary artery pressures on transthoracic echocardiogram and/or right heart catheterization; and presence of malignancy. Tumor emboli and fibrocellular intimal proliferation were seen in pulmonary arterioles, while two patients had pulmonary venopathy. PTTM is a devastating disease occurring in patients with metastatic carcinoma. An early diagnosis is challenging. Understanding the clinical presentation of PTTM and distinguishing PTTM from clinical mimics may help achieve an early diagnosis and allow time for initiation of treatment.

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Near syncope is not always benign

Challenging Concepts in Congenital and Acquired Heart Disease in the Young ◽

10.1093/med/9780198759447.003.0016 ◽

2020 ◽

pp. 211-226

Author(s):

Paraskevi Theocharis ◽

Roberta Bini

Keyword(s):

Heart Failure ◽

Pulmonary Hypertension ◽

Correct Diagnosis ◽

Rare Condition ◽

Right Heart Failure ◽

Therapeutic Strategies ◽

Right Heart ◽

Bilateral Lung Transplantation ◽

Starting Point ◽

Bilateral Lung

A ‘not uncommon’ episode of near syncope in a 6-year-old child is the starting point for cardiology screening of pulmonary hypertension, a rare condition in paediatrics, posed two questions—was SVT secondary to right heart failure that is secondary to pulmonary hypertension? Or was SVT precipitated by right heart failure in the presence of pulmonary hypertension? The chapter goes on to describe the meticulous and extensive screening necessary to establish the correct diagnosis, aiming to ascertain the presence, type, and severity of pulmonary hypertension. The differential diagnosis is fundamental to the therapeutic strategies. There is no cure. Palliative procedures and drug therapy improve symptoms and prolong life, but eventually the disease progresses. When the response to therapeutic strategies decreases and symptoms worsen, the only option that remains is bilateral lung transplantation.

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