Intestinal Leiomyosarcoma Workup

  • Chief Editor: Julian Katz, MD   more...
 
Updated: Jan 7, 2010
 

Laboratory Studies

  • Unless obstruction or perforation occurs, low hemoglobin caused by acute or chronic bleeding may be the only evidence of this tumor. If the bleeding is chronic, the appropriate indices will be affected.
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Imaging Studies

  • Notably, as many as 40% of these tumors are discovered incidentally. With this in mind, the principle symptom or sign of leiomyosarcomas is GI bleeding, sometimes massive.
  • If findings on esophagogastroduodenoscopy (EGD) and colonoscopy are normal, always consider a focused contrast study of the small bowel followed by a CT scan in case the source of bleeding is not identified. Unfortunately, early detection of the tumor by CT scan depends on size and may not be helpful.
  • A single report describes the use of preoperative endoscopic ultrasound in helping to plan resection.[6] Ludwig postulated that this modality might be useful; further study is necessary.
  • A 2004 study by Ping-Hong Zhou and colleagues evaluated the use of miniprobe ultrasonography during colonoscopy in diagnosing submucosal tumors of the large intestine.[7] Leiomyosarcomas were identified as having inhomogeneous echoes and irregular borders. Zhou et al also concluded that this technique provided information about size and layer of origin. As leiomyosarcoma account for only 0.1% of colonic malignancies, miniprobe ultrasonography is more useful in other submucosal tumors, such as lipomas and leiomyomas.
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Procedures

  • Because these tumors are intramural in origin and tend to grow extraluminally, biopsy findings from the luminal wall obtained from over the tumor often will be reported as benign mucosa.
  • If possible, endoscopic ultrasound with guided biopsy may be diagnostic.[8]
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Histologic Findings

These tumors are spindle cell in character, with high cellularity. The mitotic figure count is of supreme importance. A count of more than 5 mitotic figures per 10 high-powered fields places a tumor into the high-grade category. Necrosis often occurs with high-grade tumors. (See the images below.)

Colonic mucosa with gastrointestinal stromal tumorColonic mucosa with gastrointestinal stromal tumor (GIST) involving adjacent submucosa (hematoxylin and eosin [H&E] stain, medium power). Clusters of tumor cells separated by a hyaline andClusters of tumor cells separated by a hyaline and mucin-rich stroma (hematoxylin and eosin [H&E] stain, medium power). Oval- to spindle-shaped cells forming a fascicle (Oval- to spindle-shaped cells forming a fascicle (hematoxylin and eosin [H&E] stain, high power). CD-34 stain showing a tumor (medium power). CD-34 CD-34 stain showing a tumor (medium power). CD-34 is a myeloid progenitor cell antigen. High-power magnification with CD-34 antigen immunoHigh-power magnification with CD-34 antigen immunohistochemical stain showing membrane positivity of tumor cells.
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Contributor Information and Disclosures
Coauthor(s)

George Brasinikas, MD  Staff Physician, Department of Pathology, Birmingham Baptist Medical Center

George Brasinikas, MD is a member of the following medical societies: American Medical Association, College of American Pathologists, and Medical Association of the State of Alabama

Disclosure: Nothing to disclose.

Specialty Editor Board

Rajeev Vasudeva, MD, FACG  Clinical Professor of Medicine, Consultants in Gastroenterology, University of South Carolina School of Medicine

Rajeev Vasudeva, MD, FACG is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, Columbia Medical Society, South Carolina Gastroenterology Association, and South Carolina Medical Association

Disclosure: Pricara Honoraria Speaking and teaching; UCB Consulting fee Consulting

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Noel Williams, MD  Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada

Noel Williams, MD is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

References

  1. Martin RG. Malignant tumors of the small intestine. Surg Clin North Am. Aug 1986;66(4):779-85. [Medline].

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

  3. Zhan J, Xia ZS, Zhong YQ, et al. Clinical analysis of primary small intestinal disease: A report of 309 cases. World J Gastroenterol. Sep 1 2004;10(17):2585-7. [Medline].

  4. Evans HL. Smooth muscle tumors of the gastrointestinal tract. A study of 56 cases followed for a minimum of 10 years. Cancer. Nov 1 1985;56(9):2242-50. [Medline].

  5. Hill MA, Mera R, Levine EA. Leiomyosarcoma: a 45-year review at Charity Hospital, New Orleans. Am Surg. Jan 1998;64(1):53-60; discussion 60-1. [Medline].

  6. Ludwig DJ, Traverso LW. Gut stromal tumors and their clinical behavior. Am J Surg. May 1997;173(5):390-4. [Medline].

  7. Zhou PH, Yao LQ, Zhong YS, et al. Role of endoscopic miniprobe ultrasonography in diagnosis of submucosal tumor of large intestine. World J Gastroenterol. Aug 15 2004;10(16):2444-6. [Medline].

  8. Yoshida S, Yamashita K, Yokozawa M, et al. Diagnostic findings of ultrasound-guided fine-needle aspiration cytology for gastrointestinal stromal tumors: proposal of a combined cytology with newly defined features and histology diagnosis. Pathol Int. Oct 2009;59(10):712-9. [Medline].

  9. Tien YW, Lee CY, Huang CC, Hu RH, Lee PH. Surgery for gastrointestinal stromal tumors of the duodenum. Ann Surg Oncol. Oct 20 2009;epub ahead of print. [Medline].

  10. D'Adamo D. Advances in the treatment of gastrointestinal stromal tumor. Adv Ther. Oct 2 2009;epub ahead of print. [Medline].

  11. Guo T, Hajdu M, Agaram NP, et al. Mechanisms of sunitinib resistance in gastrointestinal stromal tumors harboring KITAY502-3ins mutation: an in vitro mutagenesis screen for drug resistance. Clin Cancer Res. Nov 15 2009;15(22):6862-70. [Medline].

  12. Miki Y, Kurokawa Y, Hirao M, et al. Survival analysis of patients with duodenal gastrointestinal stromal tumors. J Clin Gastroenterol. Oct 3 2009;epub ahead of print. [Medline].

  13. Artigau Nieto E, Luna Aufroy A, et al. Gastrointestinal stromal tumors: experience in 49 patients. Clin Transl Oncol. Aug 2006;8(8):594-8. [Medline].

  14. Demetri GD, van Oosterom AT, Garrett CR, et al. Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: a randomised controlled trial. Lancet. Oct 14 2006;368(9544):1329-38.

  15. Hines OJ, Nelson S, Quinones-Baldrich WJ, Eilber FR. Leiomyosarcoma of the inferior vena cava: prognosis and comparison with leiomyosarcoma of other anatomic sites. Cancer. Mar 1 1999;85(5):1077-83. [Medline].

  16. Yang WL, Yu JR, Wu YJ, et al. Duodenal gastrointestinal stromal tumor: clinical, pathologic, immunohistochemical characteristics, and surgical prognosis. J Surg Oncol. Dec 1 2009;100(7):606-10. [Medline].

  17. Zhan WH, Wang PZ, Shao YF, et al. [Efficacy and safety of adjuvant post-surgical therapy with imatinib in gastrointestinal stromal tumor patients with high risk of recurrence: interim analysis from a multicenter prospective clinical trial] [Chinese]. Zhonghua Wei Chang Wai Ke Za Zhi. Sep 2006;9(5):383-7. [Medline].

 
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Colonic mucosa with gastrointestinal stromal tumor (GIST) involving adjacent submucosa (hematoxylin and eosin [H&E] stain, medium power).
Clusters of tumor cells separated by a hyaline and mucin-rich stroma (hematoxylin and eosin [H&E] stain, medium power).
Oval- to spindle-shaped cells forming a fascicle (hematoxylin and eosin [H&E] stain, high power).
CD-34 stain showing a tumor (medium power). CD-34 is a myeloid progenitor cell antigen.
High-power magnification with CD-34 antigen immunohistochemical stain showing membrane positivity of tumor cells.
 
 
 
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