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Diffuse pulmonary lymphangiomatosis

H D Tazelaar1, D Kerr, S A Yousem

  • 1Division of Anatomic Pathology, Mayo Clinic, Rochester, MN 55905.

Human Pathology
|December 1, 1993
PubMed
Summary
This summary is machine-generated.

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Diffuse pulmonary lymphangiomatosis is a rare lung lymphatic vascular lesion. This study characterizes its distinct histology, clinical presentation, and aggressive nature, particularly in children.

Area of Science:

  • Pulmonary Medicine
  • Vascular Pathology
  • Pediatric Oncology

Background:

  • Angiomatous lesions of the lung are uncommon and poorly understood.
  • Diffuse pulmonary lymphangiomatosis is a distinct lymphatic vascular lesion.
  • This condition primarily affects children and presents with respiratory symptoms.

Purpose of the Study:

  • To describe the clinicopathological features of diffuse pulmonary lymphangiomatosis.
  • To differentiate this entity from other pulmonary vascular lesions.
  • To understand its progression and outcomes.

Main Methods:

  • Retrospective analysis of nine patient cases.
  • Review of clinical presentations, imaging, and pulmonary function tests.
  • Histopathological examination of lung biopsies and autopsy specimens, including immunohistochemical staining.

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Main Results:

  • Patients presented with wheezing, asthma, or dyspnea.
  • Radiographs showed bilateral interstitial infiltrates; effusions were common.
  • Histology revealed anastomosing endothelial-lined spaces along lymphatic routes with spindle cells; immunohistochemistry supported a lymphatic origin.

Conclusions:

  • Diffuse pulmonary lymphangiomatosis is a distinct lymphatic vascular lesion of the lung.
  • The condition is progressive and aggressive, especially in younger children.
  • It differs from lymphangiectasis, lymphangioleiomyomatosis, and other vascular tumors.