Análisis de los tumores sólidos ováricos pediátricos en nuestra población / Analysis of solid ovarian tumours in a Spanish paediatric population

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

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 14years-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

[Analysis of solid ovarian tumours in a Spanish paediatric population]. / Análisis de los tumores sólidos ováricos pediátricos en nuestra población.

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 14years-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 (stageI 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.

Tumores testiculares y paratesticulares en la edad pediátrica / Testicular and paratesticular tumors in children

OBJETIVOS: Los tumores testiculares (TT) y paratesticulares (TP) constituyen el 1-2% de los tumores sólidos infantiles. Estudios recientes recomiendan un manejo conservador, ante la mayor frecuencia de tumores benignos. Con estas premisas, revisamos nuestra experiencia, así como la actitud terapéutica adoptada. MÉTODOS: Todos los TT y TP tratados desde 1998 hasta 2016 se analizaron de manera retrospectiva. Entre los datos recogidos se encuentran la edad, clínica, lateralidad de la tumoración, pruebas de imagen, tratamiento realizado, tipo histológico y evolución. RESULTADOS: Se revisaron un total de 19 casos de TT y TP en 17 pacientes. El 79% de los casos debutaron como una masa escrotal asintomática con marcadores tumorales negativos. El estudio anatomopatológico demostró una proporción similar de TT estromales y de células germinales. En cuanto a los TP se evidenció una proporción similar entre los tumores de características benignas y malignas. Se practicó cirugía conservadora en el 58% de los TT y tumorectomía en el 57% de los TP. CONCLUSIONES: La alta incidencia de benignidad de los TT y TP en la infancia, sobre todo con marcadores tumorales normales, hace que deba considerarse la cirugía conservadora como primera opción terapéutica

OBJECTIVES: Testicular (TT) and paratesticular (PT) tumors account for 1-2% of all infant solid tumors. Due to the increased frequency of benign tumors, conservative management is recommended. Our experience and the therapeutic approach adopted considering testis-sparing surgery, was reviewed. METHODS: A retrospective observational study concerning testicular and paratesticular tumors in our hospital between 1998 and 2016, was performed. Age, side, symptoms, imaging, treatment methods, histological findings and evolution were reviewed. RESULTS: Nineteen cases of TT and PT were reviewed in 17 patients. A painless scrotal mass was found in most cases as the initial presentation (79%). Tumor markers were normal in all cases. Similar distribution between germ cell and stromal testicular tumors was found. Nevertheless, benign and malignant PT proportion was similar. Testis preserving surgery was performed in 58% of TT and in 57% of PT. CONCLUSIONS: Due to the high incidence of the benign histological findings, testicular sparing surgery should be considered as a first therapeutic option, especially in those cases with normal tumor markers

Testicular and paratesticular tumors in children. / Tumores testiculares y paratesticulares en la edad pediátrica.

OBJECTIVES: Testicular (TT) and paratesticular (PT) tumors account for 1-2%of all infant solid tumors. Due to the increased frequency of benign tumors, conservative management is recommended. Our experience and the therapeutic approach adopted considering testis-sparing surgery, was reviewed. METHODS: A retrospective observational study concerning testicular and paratesticular tumors in our hospital between 1998 and 2016, was performed. Age, side, symptoms, imaging, treatment methods, histological findings and evolution were reviewed. RESULTS: Nineteen cases of TT and PT were reviewed in 17 patients. A painless scrotal mass was found in most cases as the initial presentation (79%). Tumor markers were normal in all cases. Similar distribution between germ cell and stromal testicular tumors was found Nevertheless, benign and malignant PT proportion was similar. Testis preserving surgery was performed in 58% of TT and in 57% of PT. CONCLUSIONS: Due to the high incidence of the benign histological findings, testicular sparing surgery should be considered as a first therapeutic option, especially in those cases with normal tumor markers.

Beneficial effects of growth hormone therapy for ossification defects after bone distraction in X linked hypophosphataemic rickets.

A report on two homozygous twin girls affected by X linked hypophosphataemic rickets. They were examined due to short stature and genu varum of both tibias. They were treated with calcitriol and Joulie's solution, whereon it was observed that serum parathyroid hormone and phosphaturia decreased while phosphataemia increased. They underwent a tibial osteotomy (by means of the insertion of Kirchner needles) at 7.7 years of age for correction of genu varum and a normal consolidation was reached 1 month later. Nonetheless, height was percentile <1 after menarche, so both sisters asked for bone lengthening. Because of this, at 15 years of age femoral distraction was performed, but no bone callus was observed 14 months later. Consequently, they were treated with subcutaneous growth hormone, showing bone callus at 6 months. Finally, the external fixators were removed due to ossification in the lengthened segments.

Abdomen agudo como presentación de absceso tubo-ovárico en adolescente sexualmente inactiva / Acute abdomen as the form of presentation of tubo-ovarian abscess in a sexually inactive adolescent

El absceso tubo-ovárico es una complicación de la enfermedad inflamatoria pélvica (EPI), entidad poco frecuente en mujeres sin actividad sexual previa. Presentamos el caso de una paciente de 13 años, sin relaciones sexuales, que consultó por cuadro de dolor abdominal, fiebre y diarrea. Fue diagnosticada de absceso tubo-ovárico bilateral que se resolvió mediante antibioterapia por vía intravenosa y drenaje laparoscópico. El diagnóstico del absceso tubo-ovárico y su tratamiento precoz son esenciales para prevenir posibles secuelas (infertilidad, embarazo ectópico o dolor abdominal crónico) y debe ser sospechado aun en ausencia de relaciones sexuales. Actualmente, la laparoscopia es el tratamiento quirúrgico de elección porque genera menos morbilidad postoperatoria (AU)

Tubo-ovarian abscess (TOA) is a complication of pelvic inflammatory disease (PID) rarely found in sexually inactive girls. We report the case of a 13-year-old sexually inactive adolescent who presented with abdominal pain, fever and diarrhea. The patient was diagnosed with bilateral tubo-ovarian abscess, which was resolved by laparoscopic drainage of the abscess and antibiotic therapy. Early diagnosis and treatment are essential to prevent further sequelae including infertility, ectopic pregnancy and chronic pelvic pain. TOA should be included in the broad differential diagnosis of abdominal pain with fever in adolescent girls regardless of sexual history. Currently, laparoscopy is the surgical treatment of choice because it produces less postoperative morbidity (AU)

Quiste paratubárico en la infancia. Manejo laparoscópico / Paratubal Cyst in Childhood. Laparoscopic Management

Los quistes paratubáricos son de diagnóstico excepcional en la edad pediátrica. Presentamos el caso de una niña de 12 años con dolor abdominal intermitente de 2 años de evolución a la que se le detectó por pruebas de imagen una masa quística y homogénea en pelvis compatible con quiste paratubárico. Tras completar el estudio diagnóstico se llevó a cabo la quistectomía laparoscópica con bisturí armónico. La resección quirúrgica es el tratamiento de elección, siendo el abordaje laparoscópico la primera opción en la actualidad (AU)

Diagnosis of paratubal cysts in children is exceptional. We present the case of a 12-year-old girl with a 2-year history of intermittent abdominal pain. Imaging studies revealed a homogeneous cystic mass in the pelvis compatible with a paratubal cyst. After completing the diagnostic workup, laparoscopic cystectomy was performed by harmonic scalpel. Surgical resection is the treatment of choice in this entity, the laparoscopic approach currently being the first option (AU)