I typed my cancer into the Medscape search engine and found a long article that just about makes me want to throw up, or cry, or both. I can't process it enough to be able to write about it, so here are some snippets. I need to go lie down now next to my napping husband and try not to think the worst.
Soft tissue sarcomas are rare, with approximately 8,600 new cases diagnosed annually in the United States -- less than 1% of all newly diagnosed malignancies. ... The rarity of retroperitoneal sarcomas, combined with the vast array of histologic subtypes, has complicated our understanding of these tumors and impeded the development of effective therapies.
Approximately half of retroperitoneal sarcomas are high-grade tumors.
Tumor size has not been identified as a predictor of survival since virtually all retroperitoneal sarcomas are larger than 5 cm at presentation. Tumor grade has been reported as a significant factor in some studies, with the weight of evidence supporting shorter recurrence-free and overall survival for patients with high-grade tumors.
Sarcomas that arise in the retroperitoneum most commonly present as an abdominal mass, often without other symptoms. ... In most cases, retroperitoneal sarcomas smaller than 5 cm are rarely seen since they usually are not noted by the patient until they are larger in size. When symptoms are present, they relate to the mass effect of the tumor or to local invasion. Early satiety, gastrointestinal obstruction or bleeding, lower extremity swelling, or pain are among the first symptoms leading to the discovery of a retroperitoneal sarcoma.
Retroperitoneal soft tissue sarcomas present challenges that distinguish them from the more common soft tissue sarcomas of the extremities. ... While the most common site of first recurrence for patients with extremity sarcomas is a distant site, patients with retroperitoneal sarcomas are more likely to develop recurrences within the abdominal cavity. The overall survival for patients with extremity sarcomas is superior to that of patients with retroperitoneal sarcomas. ... Local failure continues to occur beyond 5 and 10 years following resection, leading some to estimate that the long-term recurrence rate for resectable retroperitoneal sarcomas exceeds 70%.
As with primary sarcomas of the extremities, surgery is the treatment standard for retroperitoneal sarcomas. ... In most reports, complete excision is achieved less than 70% of the time, with local recurrence occurring in approximately half of patients undergoing complete resection. ... A retroperitoneal mass should not be surgically treated unless the surgeon is prepared for the magnitude of the resection that may be required.
Many studies have shown that a significant number of patients experience prolonged disease-free survival when all grossly evident recurrent disease can be resected. The addition of chemotherapy or radiation in the treatment of locally recurrent disease remains the subject of debate.
Following resection of one recurrence, subsequent recurrences have progressively diminishing chances of being successfully resected.
Compared with patients with extremity soft tissue sarcomas, those with retroperitoneal sarcomas have a greater tendency for local recurrence and disseminated disease throughout the abdomen. Potter et al reported that 80% of recurrences occur within 5 years.
The evaluation and treatment of retroperitoneal soft tissue sarcomas remain challenging. The most important factor in the long-term success in the treatment of primary tumors is complete surgical resection. It is important that patients with these tumors be evaluated and treated at centers with multidisciplinary treatment planning and expertise in treating these rare tumors. These centers also can invest time and resources into investigations of novel therapies and have access to clinical trials. Local recurrence remains a difficult problem, with increased associated morbidity and psychological stress for affected patients. We hope that with improved education, early referral of patients with retroperitoneal soft tissue tumors will become the norm, and patients will derive the benefits of multidisciplinary evaluation and treatment of their disease.
— "Retroperitoneal Sarcomas," by T. Christopher Windham, MD; Peter W. T. Pisters, MD, from Cancer Control: Journal of the Moffitt Cancer Center, posted 02/28/2005