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Rare disease
CASE REPORT
Acute cor pulmonale due to pulmonary tumour thrombotic microangiopathy from renal cell carcinoma
  1. Maria Story1,
  2. Sook Kyung Kwon2,
  3. Robert Robinson2,
  4. Spyridon Fortis3
  1. 1Department of Internal Medicine, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA
  2. 2Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA
  3. 3Pulmonary, Critical Care and Occupational Medicine, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA
  1. Correspondence to Dr Maria Story, maria.story14{at}gmail.com

Summary

We report the case of a previously healthy man who presented with subacute dyspnoea after a long drive. He developed hypoxic respiratory failure, thought secondary to a massive pulmonary embolism and was treated with tissue plasminogen activator but died in the hospital despite aggressive medical measures. Autopsy revealed pulmonary tumour thrombotic microangiopathy (PTTM) from papillary renal cell carcinoma. PTTM is a rare clinicopathological syndrome that clinically results in symptoms of dyspnoea and right heart failure. Pathologically, a localised paraneoplastic process evolves from tumour microemboli in the pulmonary arterioles, resulting in fibrocellular proliferation and narrowing of the vessels, causing subacute right heart failure. To our knowledge, this is the first case of PTTM due to papillary renal cell carcinoma.

  • Adult Intensive Care
  • Urological Cancer
  • Pulmonary Hypertension
  • Heart Failure

https://doi.org/10.1136/bcr-2017-219730

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    Footnotes

    • Contributors MS took care of the patient, developed the idea for the case report, researched PTTM and wrote the majority of the manuscript. SK performed the autopsy, researched PTTM and wrote the pathology portion of the manuscript. RR assisted with the diagnosis of PTTM based on gross and pathological autopsy findings and reviewed/edited the pathology portion of the manuscript. SF assisted with manuscript preparation, editing and reviewed the manuscript for content and accuracy.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.