We analyzed the clinical and histopathologic characteristics of verrucous hemangioma, compared these findings to hyperkeratotic mimickers such as capillary-lymphatic malformation or capillary-venous malformation and angiokeratoma circumscriptum, and reconsidered whether the term verrucous hemangioma is appropriate in the current nosology of vascular anomalies. Fourteen similar-appearing localized hyperkeratotic vascular lesions were identified by one surgeon as either angiokeratoma, angiokeratoma circumscriptum, capillary-venous malformation, capillary-lymphatic malformation, or verrucous hemangioma. All lesions were located on an extremity, except for one lesion on the trunk, and were single or grouped and 2.6 to 8 cm in diameter. All were raised, red-to-purple, variably keratotic with irregular borders, and several manifested intermittent bleeding and oozing. Excision was performed at 4 months to 16 years of age. After histologic review, three lesions were designated as combined vascular malformations composed of capillaries, lymphatics, and veins, and none was designated as angiokeratoma. Eleven of 14 specimens met the histologic criteria for verrucous hemangioma: a hyperkeratotic epidermis with small, thick-walled, blood-filled vessels with multilamellated basement membrane involving the entire dermis as well as the subcutis. Immunostaining showed focal GLUT1 endothelial positivity (7/11) and low-level MIB-1 reactivity (8/11). Verrucous hemangioma has the accepted clinical features of vascular malformation, specifically presence at birth and proportionate growth. Microscopic features, such as thick vascular walls, multilamellated basement membrane, relatively uniform channel size, and GLUT1 immunopositivity are reminiscent of infantile hemangioma, particularly in its involutive phase. No firm conclusion seems possible as to whether verrucous hemangioma is a malformation or an indolent tumor, but clinical evidence favors the former category.
PTEN hamartoma tumor syndrome (PHTS) presents in a spectrum that encompasses the epononymous disorders, Cowden’s and Bannayan-Riley-Ruvalcaba. Herein, we delineate the distinctive histopathology of a predominantly intramuscular lesion in PHTS, often called “arteriovenous malformation” because of certain imaging and histopathologic features. Cases were identified by review of lesions resected from patients with PHTS registered in our Vascular Anomalies Center and of unusual intramuscular vascular anomalies in our pathology database from 1985 to 2008. Thirty-four patients with this lesion were identified: 20 had a clinical diagnosis of, or were suspected to have, PHTS (genetically confirmed in 16). In 4 patients without clinical manifestations of PHTS, 2 had PTEN mutations, 1 did not, and in another the mutation was intronic. In the remaining 10, there was insufficient clinical information to fully assess whether they had manifestations of PHTS. Lesions manifested by 15 years of age, normally with pain and swelling, most often located in the lower extremity. The major mass was usually intramuscular, but often there were fascial and subcutaneous components and not infrequently a cutaneous vascular stain. MRI generally showed an infiltrative soft tissue lesion involving muscle, fascia and subcutis with frequent enlarged, serpiginous vessels and a prominent adipocytic component. Some lesions involved contiguous muscles and 20% were multifocal. Resected specimens ranged in size from 3–25 cm; in one patient, amputation was necessary. Histopathologically, these unencapsulated masses, oftentimes with a nodular appearance at scanning magnification, consisted of: 1) variable admixture of mature adipocytic and dense and/or myxoid fibrous tissues (50–90% of surface area); 2) vascular component (10–50% of surface area) with: a) clusters of venous channels, some with excessively and irregularly muscularized complex walls and lumens, and others with thin walls resembling pulmonary alveoli, b) tortuous, thick-walled arteries with concentric muscular hyperplasia and relatively small lumens, c) numerous small vessels (arteries, veins and indeterminate channels), and d) occasional arteriovenous communications; 3) lymphoid follicles (50%); 4) foci of bone (20%); 5) hypertrophic nerves with “onion bulb” proliferation of periaxonal spindled cells (9%). We designate this disorganized overgrowth of essentially mesenchymal elements, PTEN hamartoma of soft tissue (PHOST). It differs from other vascular and connective tissue lesions that occur in patients with PHTS. PHOST is histopathologically distinctive and its identification should prompt a thorough investigation for PHTS.
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