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Special Feature |

Image of the Month—Diagnosis FREE

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Section Editor: Carl E. Bredenberg, MD

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Arch Surg. 2009;144(1):88. doi:10.1001/archsurg.2008.533-b.
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ANSWER: GASTROINTESTINAL STROMAL TUMOR

The patient was taken to the operating room and first underwent diagnostic laparoscopy revealing a significant amount of periappendiceal purulent fluid. The appendix was removed laparoscopically. The procedure was then converted to an open exploration through a periumbilical midline incision. An approximately 5 × 5-cm pedunculated mass was identified arising from the proximal jejunum approximately 40 cm from the ligament of Treitz (Figure 2). Perforation of the mass with its associated bowel was noted, and no other abnormalities were noted. The involved segment of bowel with its associated mesentery was resected with 10-cm margins and a stapled anastomosis performed.

Place holder to copy figure label and caption
Figure 2.

Portion of resected jejunum with associated mass. The specimen has been opened longitudinally to demonstrate both the luminal extension of the tumor as well as the region of central tumor necrosis and perforation. Photograph courtesy of Victor Davis, MD.

Graphic Jump Location

Small-bowel cancers are relatively rare. Adenocarcinomas are the most common, typically located in the duodenum. Small-bowel tumors most often present with nonspecific symptoms such as pain and cramping but can also present with hemorrhage, as was most likely the case with this patient who had melena a month prior to this admission when he presented with peritonitis secondary to perforation.1,2

On final pathologic review, this patient was found to have a gastrointestinal stromal tumor (GIST). Gastrointestinal stromal tumors account for only 14% of small-bowel neoplasms and most commonly (60%) arise from the stomach.3Though extraluminal in origin as mesenchymal tumors, they can perforate through the mucosa as well as intraperitoneally, as was the case with this patient. On pathologic examination, this patient had an 8-cm tumor with more than 5 mitoses per 50 high-power fields, suggesting a high-risk tumor.4The tumor core was necrotic, leading to perforation, further classifying this as a high-risk tumor. The tumor was c-KIT (CD117) positive and negative for S-100 and desmin, all typical for GISTs.3,5Margins and associated mesenteric lymph nodes were negative.

Complete tumor resection with negative margins is the definitive treatment for GIST, with routine lymphadenectomy unnecessary as lymph node extension is very rare. Effective treatment of GISTs with activating mutations in the proto-oncogene C-KIThas been achieved with imatinib mesylate in a number of recent studies.3,4,6,7Mutations in c-KITand the platelet-derived growth factor receptor α gene (PDGFRA) have been used to predict response to imatinib3,5but were unavailable in this patient. A number of ongoing trials are evaluating adjuvant treatment with imatinib.8

The differential diagnosis in this patient also included Mediterranean intestinal lymphoma. This is typified by diffuse bowel thickening and multiple lesions resulting from lymphoplasmacytic infiltration of the bowel wall. These malignancies are low-grade B-cell mucosa-associated lymphoid tissue lymphomas. However, patients do not classically present with bleeding and perforation. Parasitic and bacterial intestinal infections are present in many patients and early disease is often responsive to antibiotic therapy.9,10

This patient recovered uneventfully and was discharged home with plans to start imatinib therapy in 6 weeks.

Return to Quiz Case.

ARTICLE INFORMATION

Accepted for Publication:March 12, 2007.

Correspondence:Mitchell Jay Cohen, MD, Department of Surgery, San Francisco General Hospital, Ward 3A, 1001 Potrero Ave, San Francisco, CA 94110 (mcohen@sfghsurg.ucsf.edu).

Author Contributions:Study concept and design: Lord, Ozgediz, and Cohen. Acquisition of data: Lord and Ozgediz. Analysis and interpretation of data: Lord and Ozgediz. Drafting of the manuscript: Lord and Ozgediz. Critical revision of the manuscript for important intellectual content: Lord, Ozgediz, and Cohen. Administrative, technical, and material support: Lord and Ozgediz. Study supervision: Lord and Cohen.

Financial Disclosure:None reported.

REFERENCES

Brunicardi  FCAndersen  DKBilliar  TR  et al. Schwartz's Principles of Surgery. 8th ed. New York, NY McGraw-Hill Companies, Inc2005;
Catena  FAnsaloni  LGazzotti  F  et al.  Small bowel tumours in emergency surgery: specificity of clinical presentation. ANZ J Surg 2005;75 (11) 997- 999
PubMed Link to Article
D’Amato  GSteinert  DM McAuliffe  JCTrent  JC Update on the biology and therapy of gastrointestinal stromal tumors. Cancer Control 2005;12 (1) 44- 56
PubMed
Fletcher  CDBerman  JJCorless  C  et al.  Diagnosis of gastrointestinal stromal tumors: a consensus approach. Int J Surg Pathol 2002;10 (2) 81- 89
PubMed Link to Article
Shinomura  YKinoshita  KTsutsui  SHirota  S Pathophysiology, diagnosis, and treatment of gastrointestinal stromal tumors. J Gastroenterol 2005;40 (8) 775- 780
PubMed Link to Article
Demetri  GDvon Mehren  MBlanke  CD  et al.  Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med 2002;347 (7) 472- 480
PubMed Link to Article
Verweij  JCasali  PGZalcberg  J  et al.  Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib. Lancet 2004;364 (9440) 1127- 1134
PubMed Link to Article
van der Zwan  SMDeMatteo  RP Gastrointestinal stromal tumor: 5 years later. Cancer 2005;104 (9) 1781- 1788
PubMed Link to Article
Ben-Ayed  FHalphen  MNajjar  T  et al.  Treatment of alpha-chain disease.  Cancer 1989;63 (7) 1251- 1256
PubMed Link to Article
Lecuit  MAbachin  EMartin  A  et al.  Immunoproliferative small intestinal disease associated with Campylobacter jejuni. N Engl J Med 2004;350 (3) 239- 248
PubMed Link to Article

Figures

Place holder to copy figure label and caption
Figure 2.

Portion of resected jejunum with associated mass. The specimen has been opened longitudinally to demonstrate both the luminal extension of the tumor as well as the region of central tumor necrosis and perforation. Photograph courtesy of Victor Davis, MD.

Graphic Jump Location

Tables

References

Brunicardi  FCAndersen  DKBilliar  TR  et al. Schwartz's Principles of Surgery. 8th ed. New York, NY McGraw-Hill Companies, Inc2005;
Catena  FAnsaloni  LGazzotti  F  et al.  Small bowel tumours in emergency surgery: specificity of clinical presentation. ANZ J Surg 2005;75 (11) 997- 999
PubMed Link to Article
D’Amato  GSteinert  DM McAuliffe  JCTrent  JC Update on the biology and therapy of gastrointestinal stromal tumors. Cancer Control 2005;12 (1) 44- 56
PubMed
Fletcher  CDBerman  JJCorless  C  et al.  Diagnosis of gastrointestinal stromal tumors: a consensus approach. Int J Surg Pathol 2002;10 (2) 81- 89
PubMed Link to Article
Shinomura  YKinoshita  KTsutsui  SHirota  S Pathophysiology, diagnosis, and treatment of gastrointestinal stromal tumors. J Gastroenterol 2005;40 (8) 775- 780
PubMed Link to Article
Demetri  GDvon Mehren  MBlanke  CD  et al.  Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med 2002;347 (7) 472- 480
PubMed Link to Article
Verweij  JCasali  PGZalcberg  J  et al.  Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib. Lancet 2004;364 (9440) 1127- 1134
PubMed Link to Article
van der Zwan  SMDeMatteo  RP Gastrointestinal stromal tumor: 5 years later. Cancer 2005;104 (9) 1781- 1788
PubMed Link to Article
Ben-Ayed  FHalphen  MNajjar  T  et al.  Treatment of alpha-chain disease.  Cancer 1989;63 (7) 1251- 1256
PubMed Link to Article
Lecuit  MAbachin  EMartin  A  et al.  Immunoproliferative small intestinal disease associated with Campylobacter jejuni. N Engl J Med 2004;350 (3) 239- 248
PubMed Link to Article

Correspondence

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