Descrição de neoplasia no lúpus eritematoso sistêmico pediátrico : um estudo multicêntrico brasileiro
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Data
2022-12-01
Autores
Brufatto, Matheus Zanata
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Universidade Estadual Paulista (Unesp)
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Introdução: O Lúpus Eritematoso Sistêmico (LES) é uma doença crônica, autoimune com etiologia multifatorial. A forma pediátrica LESp é responsável por 10 a 15 % de todos os casos. A associação do LES a neoplasias ocorre como comorbidade ou, possivelmente, associada ao tratamento imunossupressor, com frequência maior em relação à população de referência, porém os dados da população pediátrica são escassos. Objetivos: Analisar a frequência de neoplasias em associação ao LESp, em uma serie de casos, descrevendo-se os dados demográficos, características clínicas/laboratoriais, atividade e dano cumulativo do LESp mediante o desfecho neoplasia. Comparar com a frequência dos diagnósticos específicos na população de referência. Método: Análise retrospectiva de uma coorte multicêntrica de LESp em 27 serviços de Reumatologia Pediátrica do Grupo Brasileiro de Lúpus Pediátrico. Os parâmetros descritivos das neoplasias foram compilados, considerando-se o diagnóstico e a sua relação com o status de atividade e dano cumulativo, explorando-se também o tratamento imunossupressor. Foram incluídos casos com diagnóstico histopatológico de neoplasia, realizado no serviço de origem, e os dados clínicos e laboratoriais foram compilados de acordo com um protocolo único para todas as manifestações raras do LESp. Resultados: Entre os 1757 pacientes incluídos, A frequência do desfecho neoplasia no LESp foi 0,7 % (n=12), sendo 91% feminino e com neoplasias hematológicas 0,2% (n=4) e tumores sólidos 0,4% (n=8). Destes, onze tiveram localização única, e um com múltiplas localizações concomitantes. A média de idade ao diagnóstico de Neoplasia foi de 22 (DP ± 7) anos. Tempo médio entre o diagnóstico de Lúpus e o da Neoplasia foi 9 (DP± 6) anos. A idade média do diagnóstico do lúpus foi 12 anos (DP±2). A média de SLEDAI-2K: 12 (DP±12), e a média de SLICC/ACR-DI (SDI): 1,5 (DP±1). Entre os diagnósticos de Neoplasias, um terço foi linfo-hematopoiética, sendo um Linfoma Hodgkin, dois Linfoma não Hodgkin e um Leucemia Linfóide Aguda; quatro foram Neoplasias do Trato Gastrointestinal, sendo dois Carcinomas de células escamosas (um na língua e um no ânus); um Adenocarcinoma de cólon sigmóide e um Tumor carcinoide de estômago; três apresentaram Neoplasias ginecológicas, sendo um Carcinoma de vulva, um de colo de útero e um de vulva associado à de colo de útero; um caso de neoplasia de sistema nervoso central, do tipo oligodendroglioma; um por tumor urogenital de células germinativas do tipo teratoma. Conclusão: A frequência de malignidade no LESp foi de 0,7% (n=12) uma multiplos locais, onze de local único, 91% feminino. Quatro hematológicas (0,22%) e oito sumores sólidos (0,45%). A proporção de Neoplasias linfo-hematopoiéticas foi semelhante à de Tumores sólidos. Houve maior frequência de linfomas, leucemias, tumores de colo de útero, vulva/vagina, orofaringe e gástrico. A associação com neoplasia colo-retal e cerebral observada nesta série como a neoplasia de sigmóide e oligodendroglioma não havia sido descrita anteriormente em estudos de metanálise. O status de atividade e dano cumulativo tiveram maiores índices, comparados com as coortes de adultos, com baixos índices de atividade do Lúpus quando do desenvolvimento de Neoplasias
Introduction: Systemic Lupus Erythematosus (SLE) is a chronic, multifactorial autoimmune disease. The pediatric pSLE diagnoses correspond to 10 to 15% of all SLE cases. The association with cancer occurs as comorbidity with possible immunosuppressive treatment implications, with higher frequency compared with reference population in epidemiological registries, but robust data about pediatric population are scarce. Objectives: Explore the frequency of malignancy outcome associated with pSLE, describe demographic data, clinical/laboratory characteristics, activity and cumulative damage of pSLE at the malignancy diagnosis. Also, compare the frequency of specific malignancy diagnoses to the reference population. Method: A retrospective analysis of the multicentric cohort of the Brazilian Group of Pediatric Lupus in 27 Pediatric Rheumatology centers. Descriptive parameters, type of malignancy documented by histopathology were compiled, considering the diagnoses and its relation with the status of activity and cumulative damage, and exploring the immunosuppressive treatment. The histopathological diagnoses were performed in the local service, as well as clinical and laboratory data that were compiled according to the a standardized protocol. Results: Of the 1757 patients included, twelve (0.7%) developed any type of malignancy; eleven in a single-site, one with concomitant multiple sites, being 91% of those female, four lymphohematopoietic (0,22%) and eight solid tumors (0,45%). Average age at diagnosis of malignancy was 22 (SD ± 7) years. Average time between lupus and malignancy diagnosis: 9 (SD± 6) years. The average age of lupus diagnosis was 12 years (SD±2). The average of SLEDAI-2K was 12 (SD±12) and the average of SLICC/ACR-DI (SDI) was 1.5 (SD±1), by the time of malignancy diagnosis. Of 12 cases, one third had lymphohematopoietic cancer, one Hodgkin's lymphoma, two Non-Hodgkin's lymphoma and one acute lymphoblastic leukemia; four had gastrointestinal tract neoplasms, two of which were squamous cell carcinoma (one from the tongue and one anal cancer); a sigmoid colon adenocarcinoma and a stomach carcinoid tumor; three had genital cancer, one vulva carcinoma, one of the cervix and one of the vulva associated with cervix; one case had central nervous system (CNS) cancer due to oligodendroglioma; and one urogenital germ cell teratoma tumor. Conclusion: The frequency (number) of malignancy in pSLE was 0.7% (n=12), of those hematological malignancy was 0.2% (n=4) and solid tumors to 0.4% (n=8). The proportion of lymphohematopoietic malignancy and solid tumors was similar. There was a higher frequency of lymphomas, leukemias; uterine cervix, vulva/vagina, oropharynx and gastric tumors. The association between colorectal and brain malignancy observed in this series such as sigmoid neoplasia and oligodendroglioma had not been previously reported in published metanalysis. Activity status and cumulative damage were higher compared to other adult SLE-malignancy cohorts, where low rates of disease activity status were reported.
Introduction: Systemic Lupus Erythematosus (SLE) is a chronic, multifactorial autoimmune disease. The pediatric pSLE diagnoses correspond to 10 to 15% of all SLE cases. The association with cancer occurs as comorbidity with possible immunosuppressive treatment implications, with higher frequency compared with reference population in epidemiological registries, but robust data about pediatric population are scarce. Objectives: Explore the frequency of malignancy outcome associated with pSLE, describe demographic data, clinical/laboratory characteristics, activity and cumulative damage of pSLE at the malignancy diagnosis. Also, compare the frequency of specific malignancy diagnoses to the reference population. Method: A retrospective analysis of the multicentric cohort of the Brazilian Group of Pediatric Lupus in 27 Pediatric Rheumatology centers. Descriptive parameters, type of malignancy documented by histopathology were compiled, considering the diagnoses and its relation with the status of activity and cumulative damage, and exploring the immunosuppressive treatment. The histopathological diagnoses were performed in the local service, as well as clinical and laboratory data that were compiled according to the a standardized protocol. Results: Of the 1757 patients included, twelve (0.7%) developed any type of malignancy; eleven in a single-site, one with concomitant multiple sites, being 91% of those female, four lymphohematopoietic (0,22%) and eight solid tumors (0,45%). Average age at diagnosis of malignancy was 22 (SD ± 7) years. Average time between lupus and malignancy diagnosis: 9 (SD± 6) years. The average age of lupus diagnosis was 12 years (SD±2). The average of SLEDAI-2K was 12 (SD±12) and the average of SLICC/ACR-DI (SDI) was 1.5 (SD±1), by the time of malignancy diagnosis. Of 12 cases, one third had lymphohematopoietic cancer, one Hodgkin's lymphoma, two Non-Hodgkin's lymphoma and one acute lymphoblastic leukemia; four had gastrointestinal tract neoplasms, two of which were squamous cell carcinoma (one from the tongue and one anal cancer); a sigmoid colon adenocarcinoma and a stomach carcinoid tumor; three had genital cancer, one vulva carcinoma, one of the cervix and one of the vulva associated with cervix; one case had central nervous system (CNS) cancer due to oligodendroglioma; and one urogenital germ cell teratoma tumor. Conclusion: The frequency (number) of malignancy in pSLE was 0.7% (n=12), of those hematological malignancy was 0.2% (n=4) and solid tumors to 0.4% (n=8). The proportion of lymphohematopoietic malignancy and solid tumors was similar. There was a higher frequency of lymphomas, leukemias; uterine cervix, vulva/vagina, oropharynx and gastric tumors. The association between colorectal and brain malignancy observed in this series such as sigmoid neoplasia and oligodendroglioma had not been previously reported in published metanalysis. Activity status and cumulative damage were higher compared to other adult SLE-malignancy cohorts, where low rates of disease activity status were reported.
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Lúpus eritematoso sistêmico pediátrico, Registro, Neoplasias, Linfomas, Tumores sólidos, Childhood-onset systemic lupus erithematosus, Registry, Malignancy, Lymphoma, Solid tumors