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Two patients with orbital vascular malformation ipsilateral facial nevus flammeus were studied. The literature contains one similar case that interestingly shares with our cases the lack of leptomeningeal angiomatosis. Orbitofacial angiomatosis seems to be a distinct entity from meningofacial angiomatosis because of its true vascular malformation of the orbit.
Basically, there are two conditions in which angiomatosis is associated with underlying skeletal disease. The first is Maffucci's syndrome in which angiomatosis is associated with multiple enchondromatosis. Two patients with this disease are presented and its clinical and radiologic features are reviewed. The second is "congenital angiectatic hypertrophy" in which angiomatosis is associated with localized hypertrophy of underlying bones, soft tissues and occasionally internal vercera. Four patients with this condition are presented, illustrating the subtypes of closely related diseases within a broad spectrum.
A 38 year old woman showed first a lobular angiomatosis of the breast with regular neoplastic capillaries. After about one year multiple nodules of an agniosarcoma appeared. Their origin from the angiomatosis is suggested by the sequence of the lesions and for topical and histogenetic reasons.
The angiomatosis uteri shows a low scattering of nuclear DNA content; an euploid DNA stem line suggests a benign behavior in these tumors. This evidence is supported by clinical experiences. The endometrial stromal sarcoma and the angioblastic sarcoma are characterized by a high scattering of nuclear DNA content, an aneuploid DNA stemline and a substantial irregularity of the nuclear area. These difference, suggest the malignancy of stromal and angioblastic sarcoma.
The ophthalmologic and histologic signs of an angiomatosis retinae (et papillae) are presented. Some capillaries are filled abundantly with blood while others appear completely empty. Reticular fibers, starting from the vessels, form dense networks. Large sinusoid venous connections exist between choroid and tumor.
A case of intravascular angiomatosis arising in the pharynx of a 30-year-old man is reported. Gross and microscopic features with differential diagnosis are discussed. The importance of recognition of the lesion as a benign lesion which can arise in the pharynx is stressed.
Ten patients are presented who demonstrate a newly recognized association of macrocephaly with unusual angiomatosis and limb asymmetry in three somewhat similar cutaneous vascular disorders: Klippel-Trenaunay-Weber syndrome, the combination of Sturge-Weber anomaly with Klippel-Trenaunay-Weber syndrome, and cutis marmorata telangiectatica congenita. The etiology of the macrocephaly in patients with these conditions is unknown. The majority (seven of ten) of these children have no evidence of central nervous system dysfunction.
Possibly the first case of Sturge-Weber disease associated with metameric limbs and trunk cutaneous hemangioma is reported. Roentnographic findings including contrast studies and computerized axial tomography are presented and discussed. Comments are made upon the relations between neurocutaneous angiomatosis.
This is a discussion of the clinical condition of a 49-year-old patient who had been presenting intermittent episodes of intracranial hypertension since the age of 9, at which time she suddenly began a serious psychic deterioration which impaired her normal intellectual development. A new episode of intracranial hypertension led to the emergency admittance to the medical center, and was the cause of death. The cerebral post mortem study showed a large ventricular dilatation and the existence of a translucent cysticercus of a lobular shape which was adhered by a fine filament ot the internal wall of the right ventricle, very near to Monro's foramen; the possibility, therefore, was attributed to it of having had the effect of a valve mechanism which might have been causing the crises and the intracranial hypertension. At the same time, it was also observed that there existed an angiomatosis at the level of the temporal lobe, which does not seem to be related to the process, especially because of its small area and the integrity of the blood vessels.
For 55 years much has been written about a peculiar benign intravascular process which can be misdiagnosed as angiosarcoma unless careful attention is paid by the pathologist to its benign cellular morphology and growth pattern. The cases reported in the literature demonstrate an increased incidence in the extremities and the head and neck, with a propensity for the subcutaneous tissues of the perioral region. Four cases are presented to further point out the perioral incidence and emphasize the differential diagnostic criteria for benignancy, including intravascular proliferation without atypical mitotic activity and necrosis in areas devoid of vascular differentiation.
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