Skip to main content

Advertisement

SpringerLink
Log in

Small Bowel Sarcoma: Analysis of Survival From the National Cancer Data Base

  • Original Articles
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background: Small bowel sarcomas (SBS) are rare, accounting for 10% of small bowel cancers. As a result, few studies of SBS have had enough patients to accurately define their natural history and to determine the factors that have an impact on patient survival. The objective of this study was to examine patient and tumor factors in SBS and to determine prognostic factors for disease-specific survival (DSS) using the National Cancer Data Base.

Methods: Data from the National Cancer Data Base for patients diagnosed with primary SBS between 1985 and 1995 were analyzed. The χ2 statistic was used to determine significant differences between groups of patient, tumor, and treatment factors. DSS was calculated for patients diagnosed between 1985 and 1990. Significant differences in survival were determined using the Wilcoxon statistic for univariate analyses and by Cox regression in multivariate analyses.

Results: Of 14,253 small bowel tumors diagnosed between 1985 and 1995, sarcomas represented 10.1%. Overall, 5-year DSS was 38.9%, with a median survival of 34.1 months (n = 590). By univariate analysis, patient age, sex, tumor size, tumor grade, histologic type, general summary stage, nodal status, and whether cancer-directed surgery was performed were significantly correlated with DSS. In multivariate analysis, tumor size <5 cm, leiomyosarcoma histology, and localized disease were found to be significant favorable prognostic factors for DSS.

Conclusions: SBS are rare tumors that are challenging in terms of their histopathologic classification, grading, and staging. Patients with SBS were treated predominantly by surgery, with a minority receiving adjuvant therapy. Tumor size, histologic type, and general summary stage were independent prognostic factors for 5-year DSS in patients with SBS, which is improved relative to 5-year DSS seen in patients with small bowel adenocarcinoma.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

REFERENCES

  1. Martin RG. Malignant tumors of the small intestine. Surg Clin North Am 1986; 66: 779–85.

    CAS  PubMed  Google Scholar 

  2. Howe JR, Karnell LH, Menck HR, Scott-Conner C. The American College of Surgeons Commission on Cancer and the American Cancer Society. Adenocarcinoma of the small bowel: review of the National Cancer Data Base, 1985–1995. Cancer 1999; 86: 2693–706.

    Article  CAS  PubMed  Google Scholar 

  3. Greenlee RT, Murray T, Bolden S, Wingo PA. Cancer statistics, 2000. CA Cancer J Clin 2000; 50: 7–33.

    CAS  PubMed  Google Scholar 

  4. Weiss NS, Yang CP. Incidence of histologic types of cancer of the small intestine. J Natl Cancer Inst 1987; 78: 653–6.

    CAS  PubMed  Google Scholar 

  5. DiSario JA, Burt RW, Vargas H, McWhorter WP. Small bowel cancer: epidemiological and clinical characteristics from a population-based registry. Am J Gastroenterol 1994; 89: 699–701.

    CAS  PubMed  Google Scholar 

  6. Conlon KC, Casper ES, Brennan MF. Primary gastrointestinal sarcomas: analysis of prognostic variables. Ann Surg Oncol 1995; 2: 26–31.

    CAS  PubMed  Google Scholar 

  7. Skandalakis JE, Gray SW. Smooth-muscle tumors of the alimentary tract.In: Ariel IM, ed. Progress in Clinical Cancer, vol 1. New York: Grune & Stratton, 1965: 692–708.

    Google Scholar 

  8. Lewin KJ, Riddell RH, Weinstein WM. Gastrointestinal Pathology and Its Clinical Implications. New York: Igaku-Shoin Medical Publishers, 1992.

    Google Scholar 

  9. Erlandson RA, Klimstra DS, Woodruff JM. Subclassification of gastrointestinal stromal tumors based on evaluation by electron microscopy and immunohistochemistry. Ultrastruct Pathol 1996; 20: 373–93.

    PubMed  CAS  Google Scholar 

  10. Dougherty MJ, Compton C, Talbert M, Wood WC. Sarcomas of the gastrointestinal tract: separation into favorable and unfavorable prognostic groups by mitotic count. Ann Surg 1991; 214: 569–74.

    CAS  PubMed  Google Scholar 

  11. Ng EH, Pollock RE, Munsell MF, Atkinson EN, Romsdahl MM. Prognostic factors influencing survival in gastrointestinal leiomyosarcomas: implications for surgical management and staging. Ann Surg 1992; 215: 68–77.

    Article  CAS  PubMed  Google Scholar 

  12. Cancer Co. Data Acquisition Manual. 2nd ed. Chicago: American College of Surgeons, 1994.

  13. Beahrs O, Henson DE, Hutter RVP, Kennedy BJ. Manual for Staging of Cancer. 4th ed. Philadelphia: JB Lippincott, 1992.

    Google Scholar 

  14. World Health Organization. International Classification of Diseases for Oncology. 2nd ed. Geneva, Switzerland: WHO, 1992.

    Google Scholar 

  15. Jessup JM, Steele GD, Winchester DP. Introduction.In: Steele GD, Jessup JM, Winchester DP, Menck HR, Murphy GP, eds. National Cancer Data Base: Annual Review of Patient Care 1995. Atlanta: American Cancer Society, 1995: 1–11.

    Google Scholar 

  16. Wattenberg LW. Carcinogen-detoxifying mechanisms in the gastrointestinal tract. Gastroenterology 1966; 51: 932–5.

    CAS  PubMed  Google Scholar 

  17. Lowenfels AB. Why are small-bowel tumours so rare? Lancet 1973; 1: 24–6.

    CAS  PubMed  Google Scholar 

  18. Wilson JM, Melvin DB, Gray GF, Thorbjarnarson B. Primary malignancies of the small bowel: a report of 96 cases and review of the literature. Ann Surg 1974; 180: 175–9.

    CAS  PubMed  Google Scholar 

  19. Shiu MH, Farr GH, Egeli RA, Quan SH, Hajdu SI. Myosarcomas of the small and large intestine: a clinicopathologic study. J Surg Oncol 1983; 24: 67–72.

    CAS  PubMed  Google Scholar 

  20. Emory TS, Sobin LH, Lukes L, Lee DH, O’Leary TJ. Prognosis of gastrointestinal smooth-muscle (stromal) tumors: dependence on anatomic site. Am J Surg Pathol 1999; 23: 82–7.

    CAS  PubMed  Google Scholar 

  21. DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg 2000; 231: 51–8.

    Article  CAS  PubMed  Google Scholar 

  22. Chiotasso PJ, Fazio VW. Prognostic factors of 28 leiomyosarcomas of the small intestine. Surg Gynecol Obstet 1982; 155: 197–202.

    CAS  PubMed  Google Scholar 

  23. McGrath PC, Neifeld JP, Lawrence W Jr, Kay S, Horsley JS, Parker GA. Gastrointestinal sarcomas: analysis of prognostic factors. Ann Surg 1987; 206: 706–10.

    CAS  PubMed  Google Scholar 

  24. He LJ, Wang BS, Chen CC. Smooth muscle tumours of the digestive tract: report of 160 cases. Br J Surg 1988; 75: 184–6.

    CAS  PubMed  Google Scholar 

  25. Chou FF, Eng HL, Sheen-Chen SM. Smooth muscle tumors of the gastrointestinal tract: analysis of prognostic factors. Surgery 1996; 119: 171–7.

    CAS  PubMed  Google Scholar 

  26. Golden T, Stout AP. Smooth muscle tumors of the gastrointestinal tract and retroperitoneal tissues. Surg Gynecol Obstet 1941; 73: 784–810.

    Google Scholar 

  27. Hirota S, Isozaki K, Moriyama Y, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 1998; 279: 577–80.

    Article  CAS  PubMed  Google Scholar 

  28. Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol 1998; 152: 1259–69.

    CAS  PubMed  Google Scholar 

  29. Miettinen M, Sarlomo-Rikala M, Lasota J. Gastrointestinal stromal tumors: recent advances in understanding of their biology. Hum Pathol 1999; 30: 1213–20.

    Article  CAS  PubMed  Google Scholar 

  30. Wang WL, Healy ME, Sattler M, et al. Growth inhibition and modulation of kinase pathways of small cell lung cancer cell lines by the novel tyrosine kinase inhibitor STI 571. Oncogene 2000; 19: 3521–8.

    Article  CAS  PubMed  Google Scholar 

  31. Heinrich MC, Griffith DJ, Druker BJ, Wait CL, Ott KA, Zigler AJ. Inhibition of c-kit receptor tyrosine kinase activity by STI 571, a selective tyrosine kinase inhibitor. Blood 2000; 96: 925–32.

    CAS  PubMed  Google Scholar 

  32. Franquemont DW. Differentiation and risk assessment of gastrointestinal stromal tumors. Am J Clin Pathol 1995; 103: 41–7.

    CAS  PubMed  Google Scholar 

  33. Evans HL. Smooth muscle tumors of the gastrointestinal tract: a study of 56 cases followed for a minimum of 10 years. Cancer 1985; 56: 2242–50.

    CAS  PubMed  Google Scholar 

  34. Fleming ID, Cooper JS, Henson DE, et al. AJCC Cancer Staging Manual. 5th ed. Philadelphia: Lippincott, Williams & Wilkins, 1998.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Surgery, University of Iowa College of Medicine, Iowa City, Iowa

    James R. Howe MD

  2. Department of Otolaryngology, University of Iowa College of Medicine, Iowa City, Iowa

    Lucy H. Karnell PhD

  3. Department of Surgery, University of Iowa College of Medicine, Iowa City, Iowa

    Carol Scott-Conner MD, PhD

  4. Department of Surgery, University of Iowa College of Medicine, 200 Hawkins Drive, Iowa City, IA, 52242

    James R. Howe MD

Authors
  1. James R. Howe MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lucy H. Karnell PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Carol Scott-Conner MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to James R. Howe MD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Howe, J.R., Karnell, L.H. & Scott-Conner, C. Small Bowel Sarcoma: Analysis of Survival From the National Cancer Data Base. Ann Surg Oncol 8, 496–508 (2001). https://doi.org/10.1007/s10434-001-0496-4

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10434-001-0496-4

Key Words

Search

Navigation