Solitary extrapleural fibrous tumor with hepatic bilobar metastases: multimodal approach treatment.

BACKGROUND: Solitary fibrous tumor is an unusual fibroblastic mesenchymal neoplasm typically described in the pleura. It may appear anywhere with a varied anatomic distribution and essentially it can develop from any soft tissue or visceral location. Its course is usually indolent and it rarely causes distant metastases, so it has a prolonged survival rate. It sometimes presents itself as a disseminate disease being the liver the most frequently involved location. In these occasions, the management should be discussed in a multidisciplinary tumor committee formed by surgeons, oncologists and radiologists. Surgery remains the gold standard for treatment. CASE REPRESENTATION: We present the case of a woman with a tumor in the left abdominal wall and bilobar massive liver metastases, both locations histologically diagnosed as solitary fibrous tumor. She receives biological treatment for a severe case of Crohn´s disease. Evaluated in a multidisciplinary committee, surgery was recommended for both the primary lesion and the liver metastases. The hepatobiliary surgeons considered a two-stage hepatectomy with portal vein embolization (PVE) as the best strategy. After the first procedure consisting in cleaning the left hepatic lobe followed by PVE the future liver remnant volume (FLRV) was considered inadequate, so the patient was also treated with right transarterial radioembolizacion with yttrium 90 (TARE-Y90) intending a double goal: to treat the tumor and to increased the FLRV. Furthermore, a severe flare of Crohn´s disease forced us to intensify the patient's treatment with the addition of biological agents (infliximab and adalimumab) until complete remission of the symptoms. The second stage of the liver surgery had to be postponed for more than 6 months and could finally be carried out without complications, achieving an R0 resection. The postoperative course was uneventful and the follow up has showed no recurrence to date. CONCLUSION: Solitary fibrous tumours with extensive liver metastases are infrequent but when they appear modern surgical strategies like two stage hepatectomy are the treatment of choice and must be carried out by specialised units. The therapeutic decisions should be guided by a multidisciplinary committee.

[Intraoperative therapeutic intensification techniques in locally advanced abdominal sarcomas]. / Intensificación terapéutica intraoperatoria en el tratamiento de los sarcomas abdominales localmente avanzados.

INTRODUCTION: Sarcomas are rare tumors that develop from mesenchymal cells. Their management is difficult due to their changing histology, location, and behavior. In this article, we discuss the use of two intraoperative therapeutic intensification techniques, intraoperative radiotherapy (IORT) and hyperthermic intraoperative intraperitoneal chemotherapy (HIIC), in the treatment of locally advanced abdominal sarcomas and peritoneal sarcomatosis. MATERIAL AND METHODS: We analyzed a series of 20 consecutive patients diagnosed with advanced abdominal sarcoma and 5 patients with a diagnosis of peritoneal sarcomatosis who were evaluated and treated in our department from December 1996 to October 2005. In advanced abdominal sarcoma, we performed complete or maximal resection followed by IORT. In peritoneal sarcomatosis we performed massive cytoreduction followed by HIIC. RESULTS: The survival rate in advanced abdominal sarcomas without sarcomatosis was 65% at 26 months. Among the 5 patients diagnosed with peritoneal sarcomatosis, 3 were alive, and 2 were without recurrence at 20 months of follow-up. CONCLUSIONS: IORT associated with radical surgery seems to improve local control and survival in advanced abdominal sarcomas. Maximal cytoreduction plus HIIC used as treatment of peritoneal sarcomatosis is a feasible technique that offers a therapeutic option with curative intent.

Intensificación terapéutica intraoperatoria en el tratamiento de los sarcomas abdominales localmente avanzados / Intraoperative therapeutic intensification techniques in locally advanced abdominal sarcomas

Introducción. Los sarcomas son tumores infrecuentes que se desarrollan a partir de células mesenquimales. Su estudio y tratamiento es difícil debido a una histopatología, localización y comportamiento variables. En este artículo estudiamos el papel de las técnicas de intensificación terapéutica intraoperatoria: radioterapia intraoperatoria (RIO) y quimioterapia intraperitoneal intraoperatoria hipertérmica (QIOH), en el tratamiento de los sarcomas abdominales localmente avanzados y la sarcomatosis peritoneal. Material y métodos. Analizamos una serie de 20 pacientes consecutivos diagnosticados de sarcoma abdominal avanzado y 5 diagnosticados de sarcomatosis peritoneal, evaluados y tratados en nuestro servicio entre diciembre de 1996 y octubre de 2005. En el sarcoma localmente avanzado se realizó resección completa o máxima asociada a RIO. En sarcomatosis peritoneal se llevó a cabo la máxima citorreducción asociada a QIOH. Resultados. La tasa de supervivencia de los sarcomas abdominales avanzados sin sarcomatosis fue del 65% a los 26 meses. De los 5 pacientes con sarcomatosis peritoneal, 3 estaban vivos y 2 de ellos libres de enfermedad a los 20 meses de seguimiento. Conclusiones. La RIO asociada a cirugía radical parece mejorar el control local y la supervivencia en sarcomas abdominales avanzados. La citorreducción máxima más QIOH usada como tratamiento de la sarcomatosis peritoneal es una técnica factible y que ofrece una opción terapéutica con intención curativa (AU)

Introduction. Sarcomas are rare tumors that develop from mesenchymal cells. Their management is difficult due to their changing histology, location, and behavior. In this article, we discuss the use of two intraoperative therapeutic intensification techniques, intraoperative radiotherapy (IORT) and hyperthermic intraoperative intraperitoneal chemotherapy (HIIC), in the treatment of locally advanced abdominal sarcomas and peritoneal sarcomatosis. Material and methods. We analyzed a series of 20 consecutive patients diagnosed with advanced abdominal sarcoma and 5 patients with a diagnosis of peritoneal sarcomatosis who were evaluated and treated in our department from December 1996 to October 2005. In advanced abdominal sarcoma, we performed complete or maximal resection followed by IORT. In peritoneal sarcomatosis we performed massive cytoreduction followed by HIIC. Results. The survival rate in advanced abdominal sarcomas without sarcomatosis was 65% at 26 months. Among the 5 patients diagnosed with peritoneal sarcomatosis, 3 were alive, and 2 were without recurrence at 20 months of follow-up. Conclusions. IORT associated with radical surgery seems to improve local control and survival in advanced abdominal sarcomas. Maximal cytoreduction plus HIIC used as treatment of peritoneal sarcomatosis is a feasible technique that offers a therapeutic option with curative intent (AU)

Tumores sacropélvicos primarios y secundarios. Tratamiento con cirugía radical y radioterapia intraoperatoria / Primary and secondary sacropelvic tumors. Treatment with radical surgery and intraoperative radiation therapy (IORT)

Introducción. La resección sacropélvica es el tratamiento de elección de los tumores primitivos o secundarios de los huesos pélvicos. Sin embargo, es una técnica compleja asociada a una elevada tasa de recidiva local. Para optimizar el control local y la supervivencia proponemos la asociación de radioterapia intraoperatoria (RIO) a la cirugía radical. Pacientes y método. Desde 1997-2002 hemos evaluado a 17 pacientes, de los que 15 fueron operados. En 13 casos se realizó sacrectomía (siete por invasión de cáncer de recto, tres por cordomas sacros, uno por histiocitoma maligno, uno por neurofibrosarcoma y uno por condrosarcoma). En 2 pacientes se llevó a cabo una escisión subtotal del ilion izquierdo y del pubis por sarcomas de la región. En 7 casos se practicaron procedimientos asociados (3 exenteraciones pélvicas completas, 2 posteriores y 2 resecciones intestinales). En todos los casos se aplicó un componente de RIO sobre el área de resección, con dosis entre 10-12,5 Gy. Resultados. Un paciente murió (6,5 por ciento) en el postoperatorio inmediato por infarto agudo de miocardio. Las complicaciones mayores incluyeron dos reoperaciones y 7 infecciones o retraso en la curación de la herida pelviperineal. El seguimiento medio ha sido de 26 meses (rango, 6-60 meses). Dos pacientes presentaron una recaída sistémica con metástasis a distancia, sin recidiva local, a los 41-48 meses de la cirugía. Los otros 13 pacientes siguen vivos, sin evidencia de enfermedad, a los 6-60 meses de la intervención quirúrgica. Conclusiones. La invasión tumoral sacropélvica no debe ser considerada como signo de inoperabilidad. La prolongada supervivencia con control de los síntomas locales en el 86 por ciento de nuestros pacientes sometidos a cirugía radical y RIO avalan esta terapia multimodal. El presente artículo contribuye a la descripción de las indicaciones de resección sacropélvica, los tiempos técnicos y los resultados a medio plazo. Consideramos que la RIO durante la cirugía radical ha sido de gran importancia para el control local de la enfermedad. Según nuestro conocimiento, ésta es la primera descripción en España de una serie clínica de resección sacropélvica asociada a radioterapia intraoperatoria (AU)