RÉSUMÉ
BACKGROUND: Cutaneous squamous cell carcinoma (cSCC) is the leading cause of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). We provide the management and prognosis of cSCC in RDEB patients at a Spanish reference center. MATERIALS AND METHODS: We retrospectively included patients with RDEB attended in La Paz University Hospital from November 1988 to October 2018. RESULTS: Fourteen patients developed at least one cSCC. Tumors were predominantly well differentiated. Nearly half of the tumors have recurred. Median time to first recurrence was 23.4 months (95% CI: 17.2-29.5). Five patients have developed distant metastases. Median overall survival (mOS) was 136.5 months since the diagnosis of the first cSCC (95% CI: 30.6-242.3). When distant metastases occurred, mOS was 6.78 months (95% CI: 1.94-11.61). CONCLUSIONS: cSCC is a life-threatening complication of RDEB patients. Although tumors are usually well differentiated, they tend to relapse. This is the first Spanish report of cSCC arising in RDEB patients.
Sujet(s)
Carcinome épidermoïde/étiologie , Épidermolyse bulleuse dystrophique/complications , Tumeurs cutanées/étiologie , Adolescent , Adulte , Carcinome épidermoïde/épidémiologie , Carcinome épidermoïde/mortalité , Carcinome épidermoïde/thérapie , Épidermolyse bulleuse dystrophique/mortalité , Femelle , Humains , Tumeurs du poumon/secondaire , Mâle , Récidive tumorale locale , Pronostic , Études rétrospectives , Tumeurs cutanées/épidémiologie , Tumeurs cutanées/mortalité , Tumeurs cutanées/thérapie , Espagne/épidémiologie , Facteurs temps , Jeune adulteSujet(s)
Antirhumatismaux/effets indésirables , Maladie de Chagas/diagnostic , Étanercept/effets indésirables , Strongyloïdose/diagnostic , Angioedème/complications , Animaux , Anticorps antihelminthe/sang , Anticorps antiprotozoaires/sang , Antiprotozoaires/usage thérapeutique , Antirhumatismaux/usage thérapeutique , Arthrite psoriasique/complications , Arthrite psoriasique/traitement médicamenteux , Bolivie/ethnologie , Maladie de Chagas/traitement médicamenteux , Maladie de Chagas/épidémiologie , Prédisposition aux maladies , Substitution de médicament , Maladies endémiques , Étanercept/usage thérapeutique , Humains , Ivermectine/usage thérapeutique , Nitroimidazoles/usage thérapeutique , Sélection de patients , Espagne , Strongyloïdose/traitement médicamenteux , Strongyloïdose/épidémiologieRÉSUMÉ
Cerebral metastases from colorectal cancer occur in 8% of cases. Diagnosis is usually made when primary disease and widespread metastases are already known. However, the detection of brain metastases as the first sign of colorectal carcinoma without any liver and/or lung involvement is extremely rare. Central nervous system metastases are more commonly seen in rectal cancer and often occur concurrently with lung metastasis. We report a case of a patient with brain metastases as the first clinical manifestation of an adenocarcinoma of caecum without any other organ involvement.