RESUMO
A multicenter review of Periorbital Necrotizing Fasciitis including nine cases, aged 41 to 82, mostly female, and mainly post-traumatic or idiopathic. Streptococcus pyogenes was the most frequent pathogen. Treatment involved debridement alongside antibiotic therapy in all cases. Two cases experienced toxic shock, with no fatalities. Visual outcomes varied from exenteration to preserved visual acuity with minimal aesthetic impact. Early detection and prompt intervention are paramount due to the significant risks associated with this condition, which may lead to severe complications ranging from vision loss to systemic decline or death.
RESUMO
Hybrid neurofibroma/schwannoma is a rare variant of hybrid peripheral nerve sheath tumours (HPNST). A Medline search up to December 2021 identified only six cases of this tumour in the orbit. We report the case of a 78-year-old man who presented with left exophthalmos. Computed tomography showed a left intraconal orbital mass. The clinico-radiological diagnosis was consistent with an intraconal cavernous angioma. Orbitotomy was performed, obtaining an 18×16×11mm mass. Two different morphologies were seen microscopically, diagnostic of hybrid neurofibroma/schwannoma. HPNSTs of the orbit are uncommon and most reported cases showed a hybrid neurofibroma/schwannoma morphology. Hybrid neurofibroma/schwannomas have been associated with neurofibromatosis and schwannomatosis. Local recurrences have been reported. The correct identification of these tumours is important due to their potential use as a syndromic marker.