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CASE #4 (presented by Wesley King Galen)
This 4 year old girl was noted to have red, infiltrated plaques on the skin with a few surface erosions shortly after birth. She also had generalized lymphadenopathy, hepatomegaly and splenomegaly at birth. The skin has become slightly more indurated producing a rugose red appearance. The lymphadenopathy has for the most part resolved , but the patient still has significant hepatosplenomegaly. Currently, the patient generally has red skin with a peau d’orange surface and rugose folds over back and thighs. A rubbery, 1cm nodule is present on the left thigh.
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Fig. 17: The lesion is polypoid at the surface of the skin. Rete ridges are paritally effaced and a tumor in the dermis presses upon the epidermis. Nuclei of the tumor cells are regularly spaced and the resulting pattern is one of a “solid” tumor. A follicle is preserved in the lesion and focally shows epithelial hyperplasia. At the right lateral margin the tumor cells form traceries among collagen bundles of the reticular dermis. The lesion is not encapsulated. The infiltrate involved the entire thickness of the dermis.
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Fig. 18: The tumor cells have sufficient cytoplasm to provide a regularly spaced pattern of nuclei. The nuclei are uniform. The cytoplasms of the tumor cells provide a lavender background.
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Fig. 19: The tumor cells are irregular in outline and show uniform nuclear characteristics. The nuclei are enlarged and show slightly open chromatin patterns. Nuclear outlines are irregular and the cells have lavender cytoplasm.
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Fig. 20: Nuclei are enlarged and variable in outline. A blue arrow points to an eosinophil. The cells are loosely spaced in a clear matrix.
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Fig. 21: Some of the nuclei are notched (red arrows). Some of the cytologic features of the cells are “histiocytoid.” There is a degenerating cell in the center of the field (blue arrow).
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Fig. 22: At a higher magnification with a different technique, the nuclear irregularities are a prominent feature. Some of the nuclei are notched or grooved (arrows).
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