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Introduction and objectives: Ovarian tumours are rare in childhood, and account for 1–5% of all tumours. The aim of this study is to determine the epidemiological features, histological subtypes, and therapeutic management of ovarian solid ovarian tumours of the paediatric population of the province of Cordoba, in Spain. Material and methods: A retrospective, descriptive, observational and institutional study was conducted in which a review was made of the clinical histories of patients younger than 14 years-old diagnosed with ovarian tumours, excluding secondary tumours in a University Hospital between 1994 and 2017. A review was carried out on the age, clinical presentation, laterality, diagnostic methodology, treatment, histopathology, and evolution of these tumours. Results: A total of 37 ovarian tumours were reviewed in 31 patients, 6 of them being bilateral. The mean age was 10.3 (0–14) years, with 58% presenting as a palpable mass. There was no predominance of laterality. The tumour markers were negative. Conservative surgery was performed in 29.7% and adnexectomy in 70.3%. Only one case required post-operative adjuvant chemotherapy treatment (stage I immature teratoma with peritoneal gliomatosis). The histological study shows a predominance of germ cell tumours (65%) against those of epithelial lineage (22%). There were 3 stromal tumours that corresponded to fibroma (Gorlin syndrome), and bilateral gonadoblastoma associated with Frasier syndrome. The most frequent type of tumour was mature cystic teratoma (35.1%). There were no complications in the follow-up. Conclusions: Given that most childhood ovarian tumours are benign, conservative surgery is considered as the first choice, being even more important in bilateral tumours. If there is a family history, it is essential to carry out molecular genetic studies, to rule out associated syndromes. Resumen: Introducción y objetivos: Los tumores ováricos son raros en la infancia y representan entre el 1 y el 5% de todos los tumores sólidos. Nuestro objetivo es conocer las características epidemiológicas, los subtipos histológicos y el manejo terapéutico de los tumores sólidos ováricos de la población pediátrica de la provincia de Córdoba. Material y métodos: Se realizó un estudio retrospectivo, descriptivo, observacional, en el que se han revisado las historias clínicas de pacientes ≤ 14 años diagnosticadas de tumores sólidos ováricos en un hospital de tercer nivel entre los años 1994 y 2017, excluyéndose los tumores secundarios. Se revisó la edad, la presentación clínica, la lateralidad, la metodología diagnóstica, el tratamiento, la anatomía patológica y la evolución. Resultados: Se revisaron 37 tumores ováricos en 31 pacientes, siendo 6 bilaterales. La edad media fue de 10,3 años (0-14). El 58% debutaron como masa palpable. No existe predominio de lateralidad. Los marcadores tumorales fueron negativos. Se practicó cirugía conservadora en el 29,7% y anexectomía en el 70,3%. Solo un teratoma inmaduro estadio I con gliomatosis peritoneal precisó tratamiento quimioterápico adyuvante postoperatorio. El estudio histológico demuestra un predominio de tumores de células germinales (65%) frente a los de estirpe epitelial (22%). Destacan 3 tumores estromales que corresponden a fibromas (síndrome de Gorlin) y un gonadoblastoma bilateral asociado a síndrome de Frasier. El tipo de tumor más frecuente fue el teratoma quístico maduro (35,1%). Evolución favorable en todos los casos. Conclusiones: Dada la alta tasa de benignidad de los tumores ováricos en la infancia, la cirugía conservadora debe ser de primera elección, sobre todo en los bilaterales. Si existen antecedentes hereditarios, es imprescindible realizar estudios genéticos moleculares para descartar síndromes asociados.

Resumen: Introducción y objetivos: Los tumores ováricos son raros en la infancia y representan entre el 1 y el 5% de todos los tumores sólidos. Nuestro objetivo es conocer las características epidemiológicas, los subtipos histológicos y el manejo terapéutico de los tumores sólidos ováricos de la población pediátrica de la provincia de Córdoba. Material y métodos: Se realizó un estudio retrospectivo, descriptivo, observacional, en el que se han revisado las historias clínicas de pacientes ≤ 14 años diagnosticadas de tumores sólidos ováricos en un hospital de tercer nivel entre los años 1994 y 2017, excluyéndose los tumores secundarios. Se revisó la edad, la presentación clínica, la lateralidad, la metodología diagnóstica, el tratamiento, la anatomía patológica y la evolución. Resultados: Se revisaron 37 tumores ováricos en 31 pacientes, siendo 6 bilaterales. La edad media fue de 10,3 años (0-14). El 58% debutaron como masa palpable. No existe predominio de lateralidad. Los marcadores tumorales fueron negativos. Se practicó cirugía conservadora en el 29,7% y anexectomía en el 70,3%. Solo un teratoma inmaduro estadio I con gliomatosis peritoneal precisó tratamiento quimioterápico adyuvante postoperatorio. El estudio histológico demuestra un predominio de tumores de células germinales (65%) frente a los de estirpe epitelial (22%). Destacan 3 tumores estromales que corresponden a fibromas (síndrome de Gorlin) y un gonadoblastoma bilateral asociado a síndrome de Frasier. El tipo de tumor más frecuente fue el teratoma quístico maduro (35,1%). Evolución favorable en todos los casos. Conclusiones: Dada la alta tasa de benignidad de los tumores ováricos en la infancia, la cirugía conservadora debe ser de primera elección, sobre todo en los bilaterales. Si existen antecedentes hereditarios, es imprescindible realizar estudios genéticos moleculares para descartar síndromes asociados. Abstract: Introduction and objectives: Ovarian tumours are rare in childhood, and account for 1-5% of all tumours. The aim of this study is to determine the epidemiological features, histological subtypes, and therapeutic management of ovarian solid ovarian tumours of the paediatric population of the province of Cordoba, in Spain. Material and methods: A retrospective, descriptive, observational and institutional study was conducted in which a review was made of the clinical histories of patients younger than 14 years-old diagnosed with ovarian tumours, excluding secondary tumours in a University Hospital between 1994 and 2017. A review was carried out on the age, clinical presentation, laterality, diagnostic methodology, treatment, histopathology, and evolution of these tumours. Results: A total of 37 ovarian tumours were reviewed in 31 patients, 6 of them being bilateral. The mean age was 10.3 (0-14) years, with 58% presenting as a palpable mass. There was no predominance of laterality. The tumour markers were negative. Conservative surgery was performed in 29.7% and adnexectomy in 70.3%. Only one case required post-operative adjuvant chemotherapy treatment (stage I immature teratoma with peritoneal gliomatosis). The histological study shows a predominance of germ cell tumours (65%) against those of epithelial lineage (22%). There were 3 stromal tumours that corresponded to fibroma (Gorlin syndrome), and bilateral gonadoblastoma associated with Frasier syndrome. The most frequent type of tumour was mature cystic teratoma (35.1%). There were no complications in the follow-up. Conclusions: Given that most childhood ovarian tumours are benign, conservative surgery is considered as the first choice, being even more important in bilateral tumours. If there is a family history, it is essential to carry out molecular genetic studies, to rule out associated syndromes.

Abstract In kidney biopsies reviews, scleroderma renal crisis (SRC) is characterized by vascular endothelial injuries, C4d deposits on peritubular vessels, and acute and chronic injuries coexisting on the same biopsy. The clinical signs of thrombotic microangiopathy (TMA) are described in systemic sclerosis (SSc), nevertheless, it has not been related to acute injuries described on kidney biopsies. We report a case of SRC in a patient with scleroderma-dermatomyositis overlap syndrome, which also showed clinical and histopathological data of TMA. On fundus examination, a severe acute hypertensive retinopathy was found. The kidney biopsy showed severe endothelial damage with widening of mucoid cells at the level of the intima, focal concentric proliferation on most small arterioles, and C3, C4d, and IgM deposits along the capillary walls. The genetic study of complement only showed the presence of membrane cofactor protein (MCP) risk haplotypes, without other genetic complement disorders. We understand that in a patient with TMA and SSc, the kidney damage would be fundamentally endothelial and of an acute type; moreover, we would observe clear evidence of complement activation. Once further studies correlate clinical-analytical data with anatomopathological studies, it is likely that we will be forced to redefine the SRC concept, focusing on the relationship between acute endothelial damage and complement activation.