Rapid Review·Cardiovascular

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PATHOLOGY

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Vascular Tumors & Malformations

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a benign capillary tumour.
  • Infantile (strawberry) hemangiomas appear in the first weeks of life and regress by about 5 to 8 years, so the answer is usually observation.
  • Cherry hemangiomas are common in adults and do NOT regress.
a rapidly growing red polypoid lesion that bleeds easily. Linked to trauma and pregnancy, and despite the name it is neither pyogenic nor a granuloma.
benign, painful, red-blue tumour under the nail bed, arising from the modified smooth muscle of the thermoregulatory glomus body.
a benign vascular proliferation caused by Bartonella in immunocompromised (HIV) patients. Can mimic Kaposi sarcoma, and the distinction matters because this one is curable with antibiotics.
a lymphatic malformation of the neck, associated with Turner syndrome.
malignant and HHV-8 driven, of endothelial origin. Seen in AIDS, transplant recipients, and older Eastern European men.
a highly aggressive malignant endothelial tumour.
  • Hepatic angiosarcoma is linked to vinyl chloride, arsenic, and Thorotrast.
  • Also arises in chronic lymphedema after mastectomy (Stewart-Treves syndrome) and after radiation.
an abnormal high-flow artery-to-vein connection that bypasses the capillary bed, raising the risk of high-output cardiac failure. The most common cause of intracranial hemorrhage in children.
autosomal dominant. Mutations in the genes for TGF-beta receptors weaken the vessel wall, blood pools in the post-capillary venules, and small and large arteriovenous shunts form.
  • Findings: blanching telangiectasias on skin and mucous membranes including the tongue, recurrent epistaxis, and arteriovenous malformations in the brain (hemorrhagic stroke), lung (hemoptysis, and platypnea, dyspnea that worsens on sitting up), liver, GI tract (bleeding), and kidney (hematuria).
  • Complications: the systemic high-volume shunts cause high-output heart failure and pulmonary hypertension; pulmonary AVMs let clots and bacteria bypass the lung filter (paradoxical embolism, brain abscess); and the chronic bleeding gives iron deficiency anemia.

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A patient and several family members present with blanching skin lesions, frequent epistaxis, arteriovenous malformations, hematuria, and gastrointestinal bleeding. What is the diagnosis?

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