Oncología quirúrgica pediátrica / Pediatric surgical oncology

Las neoplasias suponen la primera causa de muerte en niños mayores de 1 año en países desarrollados. la cirugía pediátrica juega un importante papel terapéutico en el cáncer infantil a distintos niveles: extirpación tumoral, implantación de accesos vasculares, manejo de metástasis y complicaciones. La oncología pediátrica es una especialidad muy dinámica con actualizaciones y cambios en protocolos constantemente. el objetivo de este artículo es la revisión actualizada de los tumores sólidos más frecuentes y con características específicas en relación con la cirugía pediátrica: neuroblastoma, tumor de Wilms, hepatoblastoma, tumores ováricos, testiculares y de partes blandas. El neuroblastoma es el tumor sólido extracraneal más común en la infancia. tanto el neuroblastoma como el tumor de Wilms suelen presentarse como una masa abdominal asintomática. la combinación del grupo de riesgo, edad, factores biológicos y resultados histológicos permite asignar a cada paciente un estadio de riesgo con valor pronóstico, y establecer una estrategia terapéutica específica en cada caso de neuroblastoma. el nefroblastoma o tumor de Wilms es el tumor renal maligno más frecuente en niños. en nuestro medio el nuevo protocolo establecido por la Siop se conoce como UMBrella que se basa en la quimioterapia preoperatoria con el objetivo de reducir masa tumoral seguido de cirugía. En los casos de hepatoblastoma es importante la clasificación preteXt (Pretreatment Extent of Disease) porque define la extensión de parénquima hepático afectada y enfermedad extrahepática dando información sobre la resecabilidad del tumor y su respuesta a la quimioterapia. Ante una masa de tejido blando debe plantearse en primer lugar que se trate de procesos reactivos y tumores benignos, los tumores malignos de partes blandas son raros. dentro de estos, el rabdomiosarcoma se caracteriza por su buena respuesta a quimioterapia, por lo que está en desuso la cirugía con resecciones agresivas o mutilantes. Respecto a los tumores gonadales en la infancia, a diferencia de los adultos, predominan los germinales, en concreto el teratoma. la cirugia tiende a ser conservadora para respetar la fertilidad futura. en las últimas décadas, los avances en tratamientos oncológicos han logrado un aumento de la supervivencia en la mayoría de tumores infantiles, en parte gracias al enfoque multidisciplinar necesario desde el diagnóstico de cada caso

Malignant neoplasms constitute the first cause of death in children over 1 year of age in developed countries. Pediatric surgery plays an important therapeutic role in childhood cancer on different sides: removing tumors, placing vascular access devices, metastasis management and complications. Pediatric oncology is a dinamyc specialty with constant updates and changes in protocols. the principal aim of this report is an updated review of more common solid tumors and their specific surgical aspects: neuroblastoma, Wilms' tumor, hepatoblastoma, ovarian tumor, testicular tumor and rhabdomyosarcoma. Neuroblastoma is the most common extracranial solid tumor in chidhood. Wilms' tumor and neuroblastoma usually appears as an asyntomatic abdominal mass. the risk group, age, biological factors and histologic analysis allow to assign a risk stage to each patient, that has prognostic value and determines the specific treatment for each case of neuroblastoma. Nephroblastoma or Wilms' tumor is the most frequent malignant kidney tumor in children. UMBrella is the new protocol of Siop based on preoperative chemotherapy to reduce the size of the tumor and surgery after. The classification PRETEXT (Pretreatment Extent of Disease) is important in cases of hepatoblastoma because defines the extent of hepatic infiltrated parenchyma, so we can know about its resectability and the response to chemotherapy. A soft-tissue mass is probably a reactive process or a benign tumor, since malignant soft-tissue tumors are rare. rhabdomyosarcoma has a good response to chemotherapy, so wide resections and radical surgery are not current techniques. With regard to gonadal tumors in childhood, unlike adults, germ tumors predominate, in particular teratoma. Sparing surgery is the current treatment to preserve future fertility. over the last several decades, the advances in cancer treatment have achieved an increased survival in most of childhood tumors, thanks to multidisciplinary approach from diagnosis

Malignant extra-cranial germ cell tumors in children and adolescents. Results following the guidelines of SFOP/SFCE 95 Protocol.

Between September 1995 and December 2010, 99 new consecutive assessable patients with extra-cranial MGCT were treated according to SFOP/SFCE TGM95 Protocol. A "watch and wait" strategy for completely resected stage I-II was observed in cases with preoperative high tumor markers levels. Metastatic disease or alpha fetoprotein levels > 15 000 ng/ml cases were treated by VIP chemotherapy (etoposide, ifosfamide and CDDP) 4-6-courses. All other cases were treated by VBP (vinblastine, bleomycin, and CDDP) 3-5 courses. Median age for the whole group was 11.1 (r: 0-17) years. Males: 49, females: 50. Stage I: 19 patients, stage II: 16, stage III: 31 and stage IV: 3. Gonadal disease occurred in 77 cases. Of 21 completely resected stage I-II patients with MGCT who did not receive chemotherapy after surgery, 6 presented disease progression and were successfully treated by chemotherapy and remained disease-free. There were no significant differences in outcome according to age, gender, initial site, staging, and histological variant or high levels of alpha-fetoprotein. Initial non-responsiveness to VIP chemotherapy was the only significant unfavorable prognostic feature. With a median follow-up of 64 (r: 5-204) months, at 10 years EFS and OS estimates for the whole group were 0.82 (SE = 0.05) and 0.90 (SE = 0.03) respectively. Therapy results of MGCT treated with the SFOP/SFCE 95 strategy were excellent. Initial non-response to front line chemotherapy was the only significant adverse prognostic feature. The "watch and wait" strategy for completely resected disease with initial positive markers proved to be safe with optimal outcome.

Myoid gonadal stromal tumor: a clinicopathologic study of three cases of a distinctive testicular tumor.

OBJECTIVES: To report three new cases of testicular myoid gonadal stromal tumor to better characterize its features. METHODS: The clinicopathologic findings (including follow-up) were evaluated and a review of the literature was performed. RESULTS: The patients were 38, 43, and 59 years old, and tumor sizes were 1.2, 1.3, and 3.2 cm. All were unilateral, well circumscribed, adjacent to the rete testis, and composed exclusively of spindled cells with elongated nuclei and occasional nuclear grooves arranged in fascicles with admixed variably ectatic blood vessels. Nucleoli were inconspicuous, and the cytoplasm was scant, ill-defined, and pale/lightly eosinophilic. No sex cord component was present. Mitotic figures ranged from zero to five per 10 high-power fields. Significant atypia, lymphovascular invasion, and necrosis were absent. All were consistently positive for smooth muscle actin, S100 protein, FOXL2, and steroidogenic factor 1 but negative for h-caldesmon, calretinin, and SOX9. Inhibin and calponin were focally positive. All patients were alive and well at 5, 31, and 58 months postorchiectomy. Combining our cases with those previously reported (n = 6) shows that this neoplasm occurs mostly in younger men (mean, 37 years), and all follow-up thus far (mean, 25 months) has been benign. CONCLUSIONS: Myoid gonadal stromal tumors are small (<4 cm) indolent testicular tumors distinctly different from other sex cord-stromal tumors and are adequately managed by orchiectomy.

Current status of the treatment of gynecologic cancer by site: ovary.

Cancer of the ovary is the leading cause of death from gynecologic cancer. The constant challenge presented by ovarian cancer is that about 11,000 women die from ovarian cancer each year and the results in 1974 are no better than have been achieved in the previous two decades. Standard practice of treatment for truly invasive common epithelial ovarian cancer includes total hysterectomy, bilateral salpingo-oophorectomy, appendectomy, omentectomy, and post-surgical insertion of tubes and administration of P32 (if the disease is of limited extent). Although it is occasionally necessary to resect isolated segments of bowel, exenterative or ultraradical surgery in the management of ovarian cancer is not usually chosen because of the natural history of the disease. However, aggressive surgery is indicated not so much because it is curative, but because it potentiates other forms of treatment. All stages I through IV are treated surgically, to remove as much tumor as possible without running a risk of a gastrointestinal or genitourinary fistula. Radiation therapy has been utilized in addition to the surgical therapy in stage IV to control supraclavicular and/or inguinal node involvement. Single agent alkylating chemotherapy is chosen for the treatment of common epithelial ovarian cancers. Combination chemotherapy does not produce better results at this time, except in the treatment of embryonal tumors. The treatment of the common epithelial tumors by stage is outlined. The treatment of germ cell tumors, gonadal stromal tumors, ovarian tumors in childhood, ovarian tumors in pregnancy, as well as tumors not specific for the ovary, will also be discussed.