XXXX . Diagnosis and treatment.

@entity5 @entity3852 @entity1 @entity75
BACKGROUND: @entity3852 is an uncommon @entity5 of the skin that often portends a poor prognosis. Since its first description by Toker in 1972, a plethora of case reports and articles regarding the etiopathogenesis and treatment have been published spanning multiple medical and surgical disciplines. Much confusion still exists regarding the diagnosis and treatment of this @entity5 . OBJECT: Through extensive review of the medical, surgical, and pathological literature, to collate the observations of multiple investigators and summarize these findings. METHODS: Articles from journals of multiple subspecialties were carefully reviewed with particular emphasis placed on epidemiology, prognosis, histology, immunohistochemistry, electron microscopy, @entity5 origin, treatment, and work-up of @entity3852 . RESULTS: @entity3852 is an aggressive @entity5 . Local recurrence develops in 26-44% of @entity1 despite therapy. Up to three-fourths of @entity1 eventually develop regional nodal metastases with distant metastases occurring in one-third of all @entity1 . Reported overall 5-year survival rates range from 30% to 64%. CONCLUSION: Treatment recommendations unfortunately are based more on anecdotal than scientific data because of the rarity of the @entity5 and its recognized high risk. Most authors recommend wide local excision of the @entity75 and regional lymph node resection if lymph nodes are palpable followed by x-irradiation of both the postsurgical bed and lymph node basin. The role of elective lymph node resection in the absence of clinically positive nodes remains controversial.
