Lessons learned from the study of 10,000 patients with soft tissue sarcoma

Abstract

Background and objective: The management of rare tumors is difficult because of limited information on natural history. Our objective was to describe a long-term comprehensive prospective database with the assumption that with careful attention to patient, predisposing tumor and treatment variables, valuable knowledge could be obtained that could guide management.

Methods: In July of 1982, we began a prospective database of all adult patients admitted to our institution for a surgical procedure for soft tissue sarcoma. Patients were included if they had primary, locally recurrent or metastatic disease undergoing a surgical procedure.

Results: Over 3 decades, we entered 10,000 patients into our prospective soft tissue sarcoma database. Data were entered on a weekly or biweekly schedule with full participation of a multidisciplinary team and a dedicated sarcoma pathologist. Extensive information is available from this database. In this article, we describe distribution by site, histopathology, sex, size, and grade. We utilize this information along with outcome data for local recurrence, distant recurrence, disease specific, and overall survival. The value of molecular diagnosis is illustrated.

Conclusions: Continuous prospective long-term databases are important to obtain knowledge particularly for rare tumors. Such data can be a rich resource for the development of prognostic indicators including nomograms and can be analyzed by Bayesian Belief Networks. These long-term data linked to collection of tumor and germ-line tissue at the time of an initial procedure will remain a resource for future decades.

Figure 1

Site distribution of the primary lesion for soft tissue sarcoma in the extremities.

Figure 2

Soft tissue sarcoma local disease free survival by site. Hash mark indicates censored patients. IA= intraabdominal, Retro= retroperitoneal

Figure 3

Soft tissue sarcoma disease specific survival by site. Hash marks indicate censored patients. IA= intraabdominal, Retro= retroperitoneal

Figure 4

Local recurrence of primary (i.e. initially untreated) extremity soft tissue sarcoma by grade.

Figure 5

Disease specific survival of primary extremity soft tissue sarcoma by grade.

Figure 6

Disease specific survival of primary high grade extremity soft tissue sarcoma by size.

Figure 7

Disease specific survival of primary high grade soft tissue sarcoma by size and depth.

Figure 8

Predominant soft tissue sarcoma histopathology by site. MPNST = malignant peripheral nerve sheath tumor, UPS = undifferentiated pleomorphic sarcoma, GIST = gastrointestinal stromal tumor, IA= intraabdominal, Retro= retroperitoneal

Figure 9

Dominant initial site of metastases dependent on the site of the primary lesion. IA= intraabdominal, Retro= retroperitoneal, GI = gastrointestinal, GU = genitourinary, GYN = gynecological

Figure 10

Age distribution of synovial cell sarcoma, for patients ≥16 years.

Figure 11

Soft tissue sarcoma disease specific survival by lymph node metastases alone (n=119), or with other metastasis (n=128), and all other metastasis (n=1390).