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Arch Surg. 2001;136(5):598. doi:.
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ANSWER: GASTRIC STROMAL TUMOR (LEIOMYOMA VS LEIOMYOSARCOMA)

Figure 1. Upper gastrointestinal barium x-ray film of a 56-year-old woman after an episode of hematemesis.

Figure 2.Mucosal ulceration over leiomyoma of gastric wall in operative specimen.

Based on the patient's history, physical examination, and the mass in the gastric wall, a presumptive diagnosis of a gastric stromal tumor was made. On exploratory laparotomy, a 4-cm mass was found in the posterior wall of the fundus. A partial gastric resection with a 2-cm rim of normal gastric wall was performed using a stapling device. Examination of the specimen demonstrated a 3-cm ulceration over the mass (Figure 2). Intraoperative pathological consultation described a 4 × 3-cm benign leiomyoma surrounded by a 2-cm rim of normal gastric wall. The patient's postoperative course was uneventful and she has continued to do well.

Reviews of gastric stromal tumors have credited Giovanni Battista Morgagni with the first description of a gastric leiomyoma in 1762.13 Despite numerous publications that have discussed leiomyomas, leiomyosarcomas, and leiomyoblastomas since that time, these diagnoses account for fewer than 2% to 3% of surgical resections performed for gastric neoplasms.2,4,5 Gastric leiomyomas smaller than 1 cm, however, were found in 46% of stomachs carefully sectioned at autopsy in 50 patients aged 19 to 82 years in one older study.6 While children can be affected, the onset of symptoms in most patients occurs in the fifth or sixth decade of life.2,5

Gastric stromal tumors cause epigastric pain and/or upper gastrointestinal hemorrhage. When a leiomyoma or other stromal tumor in the gastric wall grows to a size of 3 to 4 cm, ulceration of the overlying mucosa occurs.2,4,5,7 In some patients, epigastric pain resembling that of a gastric ulcer results.5 Chronic blood loss is a more common presentation, although hematemesis has been reported.2,5,8 With leiomyosarcomas or leiomyoblastomas, hemorrhage has been reported to occur in 50% of patients.8 Finally, asymptomatic gastric stromal tumors may be discovered on radiographs, during endoscopic evaluations of the stomach, or at an exploratory laparotomy for another disease process.4

A rounded mass with smooth edges and reasonably normal overlying mucosa is the characteristic appearance on a barium study (Figure 1). Endoscopic biopsy findings may be deceptive as in this patient unless ulceration is complete and the neoplasm is exposed.2 Also, histologic differentiation between a benign or malignant gastric stromal tumor based on the endoscopic biopsy results may not be possible. On an abdominal computed tomographic scan, larger size (>5 cm), lobulated contour, heterogeneous enhancement, ulceration, and exophytic growth pattern are all statistically significant predictors of a malignant gastrointestinal stromal tumor.9 On endoscopic ultrasound, irregular extraluminal margins, cystic spaces, and malignant lymph nodes had a positive predictive value of 100% for borderline or malignant gastrointestinal stromal cell tumors in one French study.10

There have been continuing difficulties in differentiating benign from low-grade malignant gastric stromal neoplasms for many years.2,1114 For presumably benign lesions, enucleation at operation is contraindicated. Resection of the gastric wall with a 2- to 3-cm cuff of normal tissue is appropriate. A larger exophytic lesion may require a formal gastrectomy with similar margins and resection of adherent omentum, lymph nodes, or adjacent organs.2,4,5,13,14 While this patient underwent surgery in the prelaparoscopic era, laparoscopic resections are now routine for presumably benign lesions.1519

In recent years, acronyms such as GIST (gastrointestinal stromal tumor) and STUMP (smooth muscle tumor of uncertain malignant potential) have been used by pathologists when describing tumors formerly known as leiomyomas, leiomyosarcomas, and leiomyoblastomas.2,20 This reflects the problem of correlating histologic appearance with clinical outcome and continues to prompt newer approaches to differentiation.21,22

Benign leiomyomas are cured by resection of the entire tumor with a rim of normal gastric wall. Patients with gastrointestinal leiomyosarcomas undergoing curative resection have a 5-year actuarial survival rate of 54%.14 This figure is deceptive in light of the 18-month disease-free survival for high-grade lesions vs the 8-year disease-free survival rate of 80% for patients with low-grade lesions reported in one series.21 When only gastric leiomyosarcomas are evaluated, the survival figures are similar.13

Corresponding author and reprints: David V. Feliciano, MD, Grady Memorial Hospital, 69 Butler St, Atlanta, GA 30303 (e-mail: dfelici@emory.edu).

Giberson  RGDockerty  MBGray  HK Leiomyosarcoma of the stomach: clinicopathologic study of 40 cases. Surg Gynecol Obstet. 1954;98186- 196
Basson  MDModlin  IMFlynn  SD Current clinical and pathologic perspectives on gastric stromal tumors. Surg Gynecol Obstet. 1992;175477- 489
Virchow  R Die Krankhaften Geschwvelste.Vol 3. Berlin, Germany: A Hirschwald; 1867:128. Cited by: Giberson RG, Dockerty MB, Gray HK. Leiomyosarcoma of the stomach: clinicopathologic study of 40 cases. Surg Gynecol Obstet. 1954;98186- 196
Sebastian  MW Benign tumors of the stomach. Sabiston  DC  Jred.Textbook of Surgery The Biological Basis of Modern Surgical Practice Philadelphia, Pa WB Saunders Co1997;868- 875
Schorlemmer  GRBenson  MGrimes  M  et al.  Neoplasms of gastric smooth muscle. Contemp Surg. 1983;2289- 100
Meissner  WA Leiomyoma of the stomach. Arch Pathol. 1944;38207- 209
Cathcart  PMCathcart  RSYarborough  DR Tumors of gastric smooth muscle. South Med J. 1980;7318- 20
Link to Article
Appelman  HDHelwig  EB Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastomas). Cancer. 1976;38708- 728
Link to Article
Chun  HJByun  JYChun  KA  et al.  Gastrointestinal leiomyoma and leiomyosarcoma: CT differentiation. J Comput Assist Tomogr. 1998;2269- 84
Link to Article
Palazzo  LLandi  BCellier  C  et al.  Endosonographic features predictive of benign and malignant gastrointestinal stromal cell tumors. Gut. 2000;4688- 92
Link to Article
Golden  TStout  AP Smooth muscle tumors of the gastrointestinal tract and retroperitoneal tissues. Surg Gynecol Obstet. 1941;73784- 810
Stout  AP Bizarre smooth muscle tumors of the stomach. Cancer. 1962;15400- 409
Link to Article
Sanders  LSilverman  MRossi  R  et al.  Gastric smooth muscle tumors: diagnostic dilemmas and factors affecting outcome. World J Surg. 1996;20992- 995
Link to Article
DeMatteo  RPLewis  JJLeung  D  et al.  Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg. 2000;23151- 58
Link to Article
Basso  NSilecchia  GPizzuto  G Laparoscopic excision of posterior gastric wall leiomyoma. Surg Laparosc Endosc Percutan Tech. 1996;665- 67
Link to Article
Seelig  MHHinder  RAFloch  NR  et al.  End-organ and laparoscopy management of gastric leiomyomas. Surg Laparosc Endosc Percutan Tech. 1999;978- 81
Link to Article
Otani  YOhgami  MIgarashi  N  et al.  Laparoscopic wedge resection of gastric submucosal tumors. Surg Laparosc Endosc Percutan Tech. 2000;1019- 23
Heniford  BTArca  MJWalsh  RM The mini-laparoscopic intragastric resection of a gastroesophageal stromal tumor: a novel approach. Surg Laparosc Endosc Percutan Tech. 2000;1082- 85
Matsui  HUyama  IFujita  J  et al.  Gastrointestinal stromal tumor of the stomach successfully treated by laparoscopic proximal gastrectomy with jejunal interposition. Surg Laparosc Endosc Percutan Tech. 2000;10239- 242
Appleman  HD Smooth muscle tumors of the gastrointestinal tract: what we know now that Stout didn't know. Am J Surg Pathol. 1986;10 ((suppl 1)) 83- 99
Dougherty  MJCompton  CTalbert  M  et al.  Sarcomas of the gastrointestinal tract: separation into favorable and unfavorable prognostic groups by mitotic count. Ann Surg. 1991;214569- 574
Link to Article
Miettinen  MSobin  LHSarlomo-Rikala  M Immunohistochemical spectrum of GISTs at different sites and their differential diagnosis with a reference to CD 117 (KIT). Mod Pathol. 2000;131134- 1142
Link to Article

Tables

References

Giberson  RGDockerty  MBGray  HK Leiomyosarcoma of the stomach: clinicopathologic study of 40 cases. Surg Gynecol Obstet. 1954;98186- 196
Basson  MDModlin  IMFlynn  SD Current clinical and pathologic perspectives on gastric stromal tumors. Surg Gynecol Obstet. 1992;175477- 489
Virchow  R Die Krankhaften Geschwvelste.Vol 3. Berlin, Germany: A Hirschwald; 1867:128. Cited by: Giberson RG, Dockerty MB, Gray HK. Leiomyosarcoma of the stomach: clinicopathologic study of 40 cases. Surg Gynecol Obstet. 1954;98186- 196
Sebastian  MW Benign tumors of the stomach. Sabiston  DC  Jred.Textbook of Surgery The Biological Basis of Modern Surgical Practice Philadelphia, Pa WB Saunders Co1997;868- 875
Schorlemmer  GRBenson  MGrimes  M  et al.  Neoplasms of gastric smooth muscle. Contemp Surg. 1983;2289- 100
Meissner  WA Leiomyoma of the stomach. Arch Pathol. 1944;38207- 209
Cathcart  PMCathcart  RSYarborough  DR Tumors of gastric smooth muscle. South Med J. 1980;7318- 20
Link to Article
Appelman  HDHelwig  EB Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastomas). Cancer. 1976;38708- 728
Link to Article
Chun  HJByun  JYChun  KA  et al.  Gastrointestinal leiomyoma and leiomyosarcoma: CT differentiation. J Comput Assist Tomogr. 1998;2269- 84
Link to Article
Palazzo  LLandi  BCellier  C  et al.  Endosonographic features predictive of benign and malignant gastrointestinal stromal cell tumors. Gut. 2000;4688- 92
Link to Article
Golden  TStout  AP Smooth muscle tumors of the gastrointestinal tract and retroperitoneal tissues. Surg Gynecol Obstet. 1941;73784- 810
Stout  AP Bizarre smooth muscle tumors of the stomach. Cancer. 1962;15400- 409
Link to Article
Sanders  LSilverman  MRossi  R  et al.  Gastric smooth muscle tumors: diagnostic dilemmas and factors affecting outcome. World J Surg. 1996;20992- 995
Link to Article
DeMatteo  RPLewis  JJLeung  D  et al.  Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg. 2000;23151- 58
Link to Article
Basso  NSilecchia  GPizzuto  G Laparoscopic excision of posterior gastric wall leiomyoma. Surg Laparosc Endosc Percutan Tech. 1996;665- 67
Link to Article
Seelig  MHHinder  RAFloch  NR  et al.  End-organ and laparoscopy management of gastric leiomyomas. Surg Laparosc Endosc Percutan Tech. 1999;978- 81
Link to Article
Otani  YOhgami  MIgarashi  N  et al.  Laparoscopic wedge resection of gastric submucosal tumors. Surg Laparosc Endosc Percutan Tech. 2000;1019- 23
Heniford  BTArca  MJWalsh  RM The mini-laparoscopic intragastric resection of a gastroesophageal stromal tumor: a novel approach. Surg Laparosc Endosc Percutan Tech. 2000;1082- 85
Matsui  HUyama  IFujita  J  et al.  Gastrointestinal stromal tumor of the stomach successfully treated by laparoscopic proximal gastrectomy with jejunal interposition. Surg Laparosc Endosc Percutan Tech. 2000;10239- 242
Appleman  HD Smooth muscle tumors of the gastrointestinal tract: what we know now that Stout didn't know. Am J Surg Pathol. 1986;10 ((suppl 1)) 83- 99
Dougherty  MJCompton  CTalbert  M  et al.  Sarcomas of the gastrointestinal tract: separation into favorable and unfavorable prognostic groups by mitotic count. Ann Surg. 1991;214569- 574
Link to Article
Miettinen  MSobin  LHSarlomo-Rikala  M Immunohistochemical spectrum of GISTs at different sites and their differential diagnosis with a reference to CD 117 (KIT). Mod Pathol. 2000;131134- 1142
Link to Article

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