Soft tissue sarcomas are malignant tumors that may arise in any of the mesodermal tissues of the extremities (50%), trunk and retroperitoneum (40%), or head and neck (10%). Retroperitoneal sarcomas have vary clinical courses depending on their histologic subtype and grade: approximately half of retroperitoneal sarcoma are high-grade tumors and most are liposarcoma (41%), leiomyosarcoma (28%), malignant fibrous histiocytoma (7%), fibrosarcoma (6%), and malignant peripheral nerve sheath tumor (3%). Surgery is the standard treatment for retroperitoneal sarcomas but complete margin-negative resections can be difficult to achieve because of their large size and the complexity of the retroperitoneal anatomy. Radiation therapy is widely used as an adjuvant therapy to surgery but it is more problematic in the treatment of retroperitoneal sarcomas. Preoperative radiation therapy has the theoretical advantages of using the tumor's bulk to displace uninvolved intraabdominal viscera out of the radiation field, thereby decreasing local toxicity and increasing the ability to administer therapeutic radiation doses. Postoperative external-beam radiation at the doses that are most likely to be effective can be associated with acute and delayed bowel toxicity. We report the case of an 60-year-old man whith a long history of ingravescent lower limb edema, an abdominal mass and loss of weight who was found to have a large retroperitoneal mass confirmed to be a leiomyosarcoma. The clinical features are outlined and in addition the treatment and prognosis.
I sarcomi dei tessuti molli sono tumori maligni che derivano dai tessuti di origine mesodermica a livello delle estremità (50%), del tronco e del retroperitoneo (40%) o della testa e del collo (10%). I sarcomi retroperitoneali hanno un decorso clinico variabile a seconda dell’istotipo e del grading : circa il 50% sono tumori di alto grado e l’istotipo in assoluto più frequente è il liposarcoma (41%), seguito dal leiomiosarcoma (28%), dal fibroistiocitoma maligno (7%), dal fibrosarcoma (6%), e dallo schwannosarcoma (3%). Il cardine del trattamento è chirurgico ma la resezione completa del sarcoma pùo risultare difficile a causa delle dimensioni del tumore e della complessità dell’anatomia del retroperitoneo. La radioterapia è ampiamente utilizzata come terapia adiuvante ma da non sottovalutare è il problema della radiotossicità nel postoperatorio, in quanto le dosi di radiazioni da elargire nella sede della neoplasia sono limitate dalla tolleranza dei tessuti sani circostanti; quindi l’ulteriore applicazione della radioterapia pre-operatoria, in combinazione con la brachiterapia o la radioterapia intraoperatoria, permetterebbe un frazionamento delle radiazioni raggiungendo dosi elevate che altrimenti non sarebbero possibili soltanto nel postoperatorio. Riportiamo il caso di un paziente di sesso maschile di 60 anni con anamnesi positiva per edema bilaterale agli arti inferiori, massa addominale e perdita di peso cui è stato diagnosticata una grossa massa retroperitoneale rivelatasi all’esame istologico un leiomiosarcoma (28 x 24 x 22) di alto grado. Sono descritti gli aspetti clinici, il trattamento e la prognosi del caso.
An unusual retroperitoneal leiomyosarcoma: A case report / A. Antonino, F. Guida, G. Formisano, D. Esposito, P. Conte, M. Celiento, G. Mastrobuoni, G. Aprea, U. Avallone. - In: CHIRURGIA. - ISSN 0394-9508. - 20:5(2007 Oct), pp. 269-271.
An unusual retroperitoneal leiomyosarcoma: A case report
G. Formisano;
2007
Abstract
Soft tissue sarcomas are malignant tumors that may arise in any of the mesodermal tissues of the extremities (50%), trunk and retroperitoneum (40%), or head and neck (10%). Retroperitoneal sarcomas have vary clinical courses depending on their histologic subtype and grade: approximately half of retroperitoneal sarcoma are high-grade tumors and most are liposarcoma (41%), leiomyosarcoma (28%), malignant fibrous histiocytoma (7%), fibrosarcoma (6%), and malignant peripheral nerve sheath tumor (3%). Surgery is the standard treatment for retroperitoneal sarcomas but complete margin-negative resections can be difficult to achieve because of their large size and the complexity of the retroperitoneal anatomy. Radiation therapy is widely used as an adjuvant therapy to surgery but it is more problematic in the treatment of retroperitoneal sarcomas. Preoperative radiation therapy has the theoretical advantages of using the tumor's bulk to displace uninvolved intraabdominal viscera out of the radiation field, thereby decreasing local toxicity and increasing the ability to administer therapeutic radiation doses. Postoperative external-beam radiation at the doses that are most likely to be effective can be associated with acute and delayed bowel toxicity. We report the case of an 60-year-old man whith a long history of ingravescent lower limb edema, an abdominal mass and loss of weight who was found to have a large retroperitoneal mass confirmed to be a leiomyosarcoma. The clinical features are outlined and in addition the treatment and prognosis.Pubblicazioni consigliate
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