Early childhood–onset mucosal neuromas of the lips, tongue, and eyelids are pathognomonic for multiple endocrine neoplasia (MEN) 2b syndrome, which is also associated with medullary thyroid carcinoma and pheochromocytoma. It has recently been suggested that multiple mucocutaneous neuromas may also represent an early manifestation of PTEN hamartoma-tumor syndrome (PHTS), including Cowden syndrome and others.1,2 Mucocutaneous neuromas in PHTS appear in early childhood and characteristically involve acral sites.1,2
Figure 1. Skin-colored, translucent, or brownish papules and nodules. Lesion appear on the upper and lower lips (A), back side of the left hand and middle and index fingers (B), palmar side of the right hand and fingers (C), and lower back (D).
Figure 2. Hematoxylin-eosin–stained specimens of the palisaded encapsulated neuroma (A and B) and neuroma lesions (C and D). A, Large neoplastic aggregation with a smaller adjacent aggregation in the dermis, both of which are composed of intersecting fascicles separated by clefts (original magnification ×10). B, Close-up view of the intersecting fascicles with vertical and cross-sections (original magnification ×100). C, Several neighboring or scattered, thick and proliferated nerve bundles in the dermis (original magnification ×25). D, A close-up view of the proliferated nerve bundles with vertical and cross-sections associated with mucinous stroma (original magnification ×100).
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