RESUMO
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.
Assuntos
Carcinoma de Células Escamosas/etiologia , Epidermólise Bolhosa Distrófica/complicações , Neoplasias Cutâneas/etiologia , Adolescente , Adulto , Carcinoma de Células Escamosas/epidemiologia , Carcinoma de Células Escamosas/mortalidade , Carcinoma de Células Escamosas/terapia , Epidermólise Bolhosa Distrófica/mortalidade , Feminino , Humanos , Neoplasias Pulmonares/secundário , Masculino , Recidiva Local de Neoplasia , Prognóstico , Estudos Retrospectivos , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/mortalidade , Neoplasias Cutâneas/terapia , Espanha/epidemiologia , Fatores de Tempo , Adulto JovemAssuntos
Antirreumáticos/efeitos adversos , Doença de Chagas/diagnóstico , Etanercepte/efeitos adversos , Estrongiloidíase/diagnóstico , Angioedema/complicações , Animais , Anticorpos Anti-Helmínticos/sangue , Anticorpos Antiprotozoários/sangue , Antiprotozoários/uso terapêutico , Antirreumáticos/uso terapêutico , Artrite Psoriásica/complicações , Artrite Psoriásica/tratamento farmacológico , Bolívia/etnologia , Doença de Chagas/tratamento farmacológico , Doença de Chagas/epidemiologia , Suscetibilidade a Doenças , Substituição de Medicamentos , Doenças Endêmicas , Etanercepte/uso terapêutico , Humanos , Ivermectina/uso terapêutico , Nitroimidazóis/uso terapêutico , Seleção de Pacientes , Espanha , Estrongiloidíase/tratamento farmacológico , Estrongiloidíase/epidemiologiaRESUMO
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.