Skip to main content

Advertisement

Log in

Pulmonary capillary hemangiomatosis diagnosed by pathology of explanted lungs: a unique etiology serves as a key of clinical diagnosis

  • Case Report
  • Published:
General Thoracic and Cardiovascular Surgery Aims and scope Submit manuscript

Abstract

A 27-year-old female patient had presented progressing exertional dyspnea due to pulmonary hypertension. Chest CT revealed diffusely spread patchy ground-glass opacities sparing subpleural parenchymal areas suggesting the diagnosis of pulmonary veno-occlusive disease (PVOD). Despite the diagnosis of PVOD, she was somehow managed by a repetitive escalation of the epoprostenol dose and oxygen supply during the 12-month waiting period until successful bilateral lung transplantation was performed. Pathology demonstrated capillary proliferation in alveolar septae with scarce lesions of narrowed and/or occluded postcapillary small veins, leading to the final diagnosis of pulmonary capillary hemangiomatosis (PCH), not PVOD. We herein present a case of PCH diagnosed after lung transplantation with a focus on its etiology and a key to clinical diagnosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. O’keeffe MC, Post MD. Pulmonary capillary hemangiomatosis: a rare cause of pulmonary hypertension. Arch Pathol Lab Med. 2015;139:274–7.

    Article  Google Scholar 

  2. Huertas A, Girerd B, Dorfmullar P, O’Callaghan D, Humbert M, Montani D. Pulmonary veno-occlusive disease: advances in clinical management and treatments. Expert Rev Resp Med. 2011;5:217 – 31.

    Article  CAS  Google Scholar 

  3. Frazier AA, Franks TF, Mohammed TLH, Ozbudak IH, Galvin JR. From th archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics. 2007;27:867 – 82.

    Article  PubMed  Google Scholar 

  4. Miura A, Akagi S, Nakamura K, Ohta-Ogo K, Hashioto K, Nagase S, et al. Different size of centrilobular ground-glass opacities in chest high-resolution computed tomography of patients with pulmonary veno-occlusive disease and patients with pulmonary capillary hemangiomatosis. Cardiovasc Pathol. 2013;22:287–93.

    Article  PubMed  Google Scholar 

  5. Montani D, Achouh L, Dorfmuller P, Le Pavec J, Sztrymf B, Tcherakian C, et al. Pulmonary veno-occlusive disease: clinical, functional, radiological, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Boltimore). 2008;87:220 – 33.

    Article  Google Scholar 

  6. Resten A, Maitre S, Humbert M, Rabiller A, Sitbon O, Capron F, et al. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive diease. AJR Am J Roentgenol. 2004;183:65–70.

    Article  PubMed  Google Scholar 

  7. Lantuėjoul S, Sheppard MN, Corrin B, Burke MM, Nicholson AG. Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: a clinicopathologic study of 35 cases. Am J Surg Pathol. 2006;30:850–7.

    Article  PubMed  Google Scholar 

  8. Best DH, Sumner KL, Austin ED, Chung WK, Brown LM, Borczuk AC, et al. EIF2AK4 mutations in pulmonary capillary hemangiomatosis. Chest. 2015;145:231–6.

    Article  Google Scholar 

  9. Owaga A, Miyaji K, Yamadori I, Shinno Y, Miura A, Kusano KF, et al. Safety and efficacy of epoprostenol therapy in pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Circ J. 2012;76:1729–36.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Hironobu Wada.

Ethics declarations

Conflict of interest

The authors have declared that no conflict of interest exists.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Wada, H., Nakajima, T., Suzuki, H. et al. Pulmonary capillary hemangiomatosis diagnosed by pathology of explanted lungs: a unique etiology serves as a key of clinical diagnosis. Gen Thorac Cardiovasc Surg 67, 332–335 (2019). https://doi.org/10.1007/s11748-018-0950-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11748-018-0950-x

Keywords

Navigation