Case Number : Case 758 - 14th May Posted By: Guest

This lesion is within the capillary malformation / portwine stain (nevus flammeus)category including 'salmon patch. Is there any evidence of this lesion being related to a congenital clinical syndrome such as Sturge Webber, or Klipper-Trenaunay?

The absence of a lobular pattern militates against a diagnosis of capillary or infantile hemangioma. The involvement of deep dermis and subcutaneous tissue does not favor a salmon patch. I think it is a vascular hamartoma, more in keeping with a port-wine stain.

Microvenular hemangima

The hyalinised stroma and the thin walled capillaries are probably more in keeping with a microvenular haemangioma (though not seeing the infiltration of arrector pili, typical of this entity).

Thick-walled and thin-walled blood vessels with round or oval contours evenly distributed throughout a fibrotic dermis and extending into subcutaneous tissue without clear limits. These features suggest a high-flow vascular malformation with direct arteriovenous shuntings or fistulas.
I think it is arteriovenous malformation. Maybe there are other clinical signs in addition to the visual ones, like pulsation, murmur and warmness, but it seems to be in quiescent stage.

This is killing me. Ducking instead of answering the challenge I say that vascular lesions in infancy are disproportionately benign and localized in nature, plaques or segmental lesions are of certain risk and mutifocally cutaneous lesions associated with internal hemangiomas as well. If this is a segmental stuff, [b]PHACE [/b]syndrome should be considered in the workup. Having said that, my ignoramus perception is that this lesion is not well lobulated, rather diffuse, with primitive vessels and hyalinized ones, fat and myfibroblastoid whorls semi-glomeruloid funny structures. Help me GLUT-1! and vascular hamartoma of some kind are in my list. Certainly not a capillary hemangioma, not so sure about infantile hemangio(endothelio)ma (GLUT-1?). Kassabach-Merrit, nop, I do not think so. Microvenular hemangioma, never seen one in a child, can be. You can tell, I am dumbfounded on this. Wait to see the results.

I think this does not belong to vascular malformations because there is actual vascular proliferation. The pattern of the proliferation, the hyalinized stroma and the plump nuclei are making me consider microvenular hemangioma however it is more likely to be present in young adults.

This was a difficult one, the diagnosis is involuting infantile hemangioma.

Thanks.

What microscopic features is one supposed to pursue in order to diagnose an infantile hemangioma as an involuting or as an involuted lesion?