RESUMO
Solitary fibrous tumours (SFTs) are a rare mesenchymal neoplasm with an incidence of 2.8 per 100 000 of which only 1% occur in the female genital tract. Doege-Potter syndrome is a paraneoplastic phenomenon associated with approximately 5%-10% of SFTs and is characterised by non-islet cell hypoglycaemia due to tumour production of low molecular weight insulin-like growth factor-II. We present the fourth confirmed case of female pelvic SFT with Doege-Potter syndrome and a literature review.
Assuntos
Nefropatias , Síndromes Paraneoplásicas , Tumores Fibrosos Solitários , Feminino , Humanos , Anormalidades Congênitas , Rim/anormalidades , Nefropatias/congênito , Síndromes Paraneoplásicas/diagnóstico , Síndromes Paraneoplásicas/etiologia , Tumores Fibrosos Solitários/diagnóstico por imagem , Tumores Fibrosos Solitários/cirurgiaRESUMO
BACKGROUND: Fournier gangrene is a rare and rapidly progressive necrosis of the perineal and genitourinary region. Although predominantly affecting men, we describe an unusual presentation in pregnancy. CASE: A 30-year old primigravid woman in the third trimester of pregnancy presented with a history of low back pain. Subsequently, a diagnosis of perianal sepsis and associated Fournier gangrene was established. The patient underwent an emergency cesarean delivery followed by radical surgical debridement and colostomy. CONCLUSION: Expedited delivery, timely diagnosis of gangrene, urgent, extensive debridement, and broad-spectrum antibiotic coverage contributed largely to effective management of this rare presentation in pregnancy.