|
Advertisement |
|
|
||
 |
|
|||||
|
|||||
|
 |
||||||
|
||||||
|
||||||
|
© 2003 American Society for Clinical Oncology
Challenging and Unusual CasesCASE 2. CONCURRENT GASTROINTESTINAL STROMAL TUMOR AND BURKITT’S LYMPHOMADepartments of Medicine and Pathology, Queen Mary Hospital, Hong Kong
A 76-year-old human immunodeficiency virus–negative Chinese woman presented with intestinal obstruction. Laparotomy showed a 10 x 8 cm fleshy tumor encasing multiple small intestinal segments (Fig 1A
Sporadic Burkitt’s lymphoma is uncommon in Chinese, and 73% of cases are Epstein-Barr virus (EBV)-negative.1 The markers c-myc and Ki67 occur in 47% of GIST cases and are associated with aggressive disseminated disease.2 Our novel patient case with near-concomitant aggressive c-myc–related GIST and BL in an immunocompetent patient raise several interesting points. First, the clustering of two short-latency malignancies is unlikely to be a chance occurrence. Both tumors were EBV-negative, in contrast to opportunistic human immunodeficiency virus–related GIST and BL in immunocompromised hosts.3 Although lymphoma with c-kit mutation has been reported,4 c-kit was not expressed by our BL case. In the absence of apparent tumor-prone phenotype and common etiology, an immune trigger (eg, major abdominal surgery) is a plausible link.5 The presence of unknown BL trigger factors is evidenced from the time and place clustering of African BL cases.6 We have previously reported aggressive natural killer lymphoma triggered by resection of colonic carcinoma.7 Second, in all patients with known primary malignancy, secondary tumor masses withunusual location, growth, and treatment responses should always undergo biopsy for concurrent second malignancies. Finally, complete response of GIST to combination chemotherapy is rare, and the optimal regimen is undefined. Recently, the novel tyrosine kinase inhibitor imatinib mesylate (Gleevec [Novartis, Basel, Switzerland], STI-571) have shown promising results in patients with advanced GIST. However, complete response was rare, and early resistance is not uncommon.8 Our experience indicated that in c-myc–expressing GIST, a cyclophosphamide/methotrexate-based regimen may be an additional option.
REFERENCES 1. Chan JK, Tsang WY, Ng CS, et al: A study of the association of Epstein-Barr virus with Burkitt’s lymphoma occurring in a Chinese population. Histopathology 26:239–245, 1995[Medline]
2. Panizo-Santos A, Sola I, Vega F, et al: Predicting metastatic risk of gastrointestinal stromal tumors: Role of cell proliferation and cell cycle regulatory proteins. Int J Surg Pathol 8:133–144, 2000 3. Kubben FJ, Kroon FP, Hogendoorn PC, et al: Absence of Epstein-Barr virus (EBV) in a gastrointestinal stromal cell tumour (GIST) in an adult human immunodeficiency virus-seropositive patient with past EBV infection. Eur J Gastroenterol Hepatol 9:721–724, 1997[Medline] 4. Kuwahara Y, Hirata A, Miwa H, et al: Epstein-Barr virus associated B-cell lymphoma of brain developing in myelodysplastic syndrome with c-kit mutation (Try-557 ->stop). Am J Hematol 65:234–238, 2000[CrossRef][Medline] 5. Au WY, Yeung CK, Chan HH, et al: Cutaneous CD30-positive T cell lymphoma with concurrent solid tumor. Br J Dermatol 146:1091–1095, 2002[CrossRef][Medline] 6. van den Bosch C, Hills M, Kazembe P, et al: Time-space case clusters of Burkitt’s lymphoma in Malawi. Leukemia 7:1875–1878, 1993[Medline] 7. Chim CS, Au WY, Shek TW, et al: Primary CD56 positive lymphomas of the gastrointestinal tract. Cancer 91:525–533, 2001[CrossRef][Medline]
8. Demetri GD, von Mehren M, Blanke CD, et al: Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med 347:472–480, 2002
|
||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
|
|
|||||||||||
|
|||||||||||
|
|
Copyright © 2003 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
|
