Carcinoma of the recto-vaginal septum is an extremely rare entity. We performed a MEDLINE-based search on recto-vaginal septum carcinoma, focussing on its management, in order to clarify which are the best treatment options for this disease. In addition an unpublished case report has been added to the review. 34 case reports were included in our review. Surgery and adjuvant chemoradiation therapy seem to be the most common treatment option. However, since primary surgical treatment leads to mutilation by removing a large portion of the vagina and the anal sphincter with a permanent terminal colostomy, primary platinum-based chemoradiation therapy could be considered. In case of extragastrointestinal stromal tumours primary surgical treatment seems to be the best option. Due to the rarity of this entity only limited data is available. Therefore further investigation is necessary.

RESUMENObjetivos: Analizar el diagnóstico ecográfico, la evolución y el tratamiento de los tumores cardiacos fetales diagnosticados en el Hospital La Paz de Madrid, entre los años 1995 y 2010. Método: Se realizó un estudio retrospectivo descriptivo recogiendo los principales datos ecográficos, de la historia clínica materna, neonatal y pediátrica. Resultados: En el periodo de estudio se diagnosticaron ecográficamente 28 tumores cardiacos fetales, 21 casos fueron catalogados como rabdomiomas. Once de ellos tuvieron una regresión prácticamente completa, con ocho casos diagnosticados hasta el momento de esclerosis tuberosa. Hubo 2 muertes intraútero, 1 interrupción legal del embarazo y 4 diagnósticos ecográficos de rabdomiomas, no se confirmaron al nacimiento. Se diagnosticaron prenatalmente dos fibromas; de ellos una gestante optó por la interrupción legal del embarazo y en el otro caso se produjo la muerte neonatal. Dos neonatos fueron sometidos a cirugía con una resección completa del tumor, con resultado anatomopatológico de hemangioma capilar en uno y teratoma en el otro. En un caso se realizó una biopsia que confirmó la presencia de un hemangiopericitoma auricular que se redujo posteriormente con quimioterapia. Conclusiones: Los tumores cardiacos son una patología poco frecuente. La mayor parte de ellos son rabdomiomas, cuya sintomatología y evolución depende de su localización. Estos suelen regresar espontáneamente, pero pueden asociarse al diagnóstico de esclerosis tuberosa, lo que empeora su pronóstico. PALABRAS CLAVE: Tumores cardiacos fetales, rabdomiomas, esclerosis tuberosa SUMMARY Aims:To analize the diagnosis, clinical course and management of fetal cardiac tumors diagnosed at La Paz Hospital (Madrid) between 1995 and 2010. Methods: We performed a retrospective descriptive study collecting the main ultrasound dates of the maternal, newborn and pediatric history. Results: During the study period, 28 fetal cardiac tumors were dignosed. Rhabdomyomas were diagnosed in 21 fetuses; 11 rhabdomyomas returned almost completely. Eight of them were diagnosed of tuberous sclerosis up to the moment. Other two cases died in utero. One pregnant decided to be practised a miscarriage and four rhabdomyomas which were diagnosed by ultrasound, were not found in the newborns. Two fibroms were diagnosed by ultrasound; one of the pregnant woman decided to be practised a miscarriage and the other fetus died when he was born. Two newborns were operated, with the pathological anatomy result of a hemangioma and a teratoma. A biopsy was made that confirmed the presence of an atrial hemangiopericitoma which was treated by quimiotherapy. Conclusion: Fetal cardiac tumors are a rare disease. Most of them are rhabdomyomas REV CHIL OBSTET GINECOL 2011; 76(3): 147 -154

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