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Journal of Clinical Oncology, Vol 25, No 9 (March 20), 2007: pp. 1136-1138 © 2007 American Society of Clinical Oncology. DOI: 10.1200/JCO.2006.09.8228
Simultaneous Gastrointestinal Stromal Tumor and Mucosa-Associated Lymphoid Tissue Lymphoma of the StomachDepartment of Surgery, St Luke's General Hospital, Kilkenny, County Kilkenny, Republic of Ireland; Department of Surgery, Morriston Hospital, Swansea, Wales
Department of Anatomy, Royal College of Surgeons Ireland, Dublin, Republic of Ireland
Department of Pathology, Morriston Hospital, Swansea, Wales
Department of Surgery, St Luke's General Hospital, Kilkenny, County Kilkenny, Republic of Ireland A 77-year-old man presented with a 4-week history of anorexia, abdominal pain, and bloody diarrhea. The comorbid conditions included chronic obstructive airway disease and angina. He had cardiac bypass in the past. Physical examination revealed a palpable nonpulsatile epigastric mass. Laboratory investigations (full blood count, urea, electrolytes, and liver function test) were within normal range. Fecal occult blood was positive. Gastroscopy showed a golf ball size sessile growth in the fundus of the stomach. Gastric biopsy histology was suggestive of a malignant tumor and non-Hodgkin's lymphoma. The computed tomography (CT) scan of the abdomen confirmed the presence of mass on the posterior wall of the gastric fundus and the features of a malignant tumor (Fig 1). He had a partial gastrectomy, gastrojejunostomy, and splenectomy (due to presence of suspicious lesions on the spleen). His postoperative recovery was uneventful. He received prophylactic (pneumococcus, influenza, and meningitis) vaccination before discharge.
Histologic examination confirmed coexisting gastrointestinal stromal tumor (GIST) and mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach. The GIST component was predominantly composed of spindle shaped cells (Fig 2). Immunohistochemical examination showed that the cells were positive for C-kit (Fig 3) and CD34 and negative for vimentin, smooth muscle actin, S-100, and desmin. Mitotic count was fewer than 5 and there was no atypia.
MALT lymphoma was confirmed by the presence of a diffuse monotonous lymphoid infiltrate of monotypic (lambda restrictive) B cells (CD20 positive centrocyte-like cells; Figs 4 and 5). Classical lymphoepithelial lesions were noted in addition to surface ulceration and deep infiltration of the stomach wall with extension to serosal surface. There was also patchy involvement of the adjacent mucosa. The spleen was enlarged and showed the presence of marginal zone B-cell lymphoma located within white pulp with early infiltration of the red pulp. He received mitoxanthane (instead of doxorubicin because of his suboptimal cardiac function) as cyclophospamide, mitoxanthane, vincristine and prednisolone (CNOP) plus tituximab (anti-CD20 monoclonal antibody). He did well and did not show any evidence of recurrence (normal gastroscopy and CT scan) at 18 months follow-up.
GISTs are rare, affecting middle and older age groups with equal sex distribution.1 The presentation pattern varies from being asymptomatic to vomiting, abdominal pain, obstruction, and abdominal mass. They may also present unusually with hematamesis, melaena, or anemia.2 The main diagnostic tools are gastroscopy and CT scan, but accurate diagnosis can only be reached by histologic examination and immunohistochemistry of the tissue. The expression of the KIT receptor tyrosine kinase (CD117: 98% to 100% of cases) and CD34 (60% to 70% cases)1 play a crucial role in the diagnosis and treatment of GISTs.3,4 The clinical spectrum varies from very low risk (benign) to high risk (malignant). Surgery is the standard treatment for the primary resectable GIST. They are resistant to chemotherapy and radiotherapy. Treatment options are even more limited for recurrence. The probability of second recurrence after re-excision reaches 100%.5 In the recent years, the prognosis of patients with metastatic disease has changed dramatically with the availability of specific inhibitor of KIT receptor tyrosine kinase called imatinib mesylate (STI-571; Gleevec; Novartis Pharma, Basel, Switzerland). It is a derivative of 2-phenylaminopyrimidine that targets specific molecules crucial in etiology of cancer3,5 and may even cause complete remission of the recurrent disease.6 MALT lymphoma occurs in patients older than 50 years with equal sex distribution and presents with symptoms like gastritis and peptic ulcer disease.7 The gastric MALT lymphoma remains localized to the stomach and rarely spreads to lymph nodes or bone marrow, though frequency of this spread may have been underestimated.8 H pylori has a role in the etiology of MALT lymphoma,3 but has no association in the pathogenesis of GIST (high incidence of H.pylori and rarity of GIST in general population).3 The early chromosomal abnormalities include trisomy 3 (60% cases), translocations t (11; 8), p53 mutations or loss of heterozygosity, and c-myc mutation. This results in an abnormal clone which undergo clonal expansion and gives rise to low-grade MALT lymphoma,9 the early phase of which responsive to H pylori eradication.10 High-grade MALT lymphoma is more common and is thought to require the development of T-cell and H pylori independence of B-cell clone with further genetic abnormalities.10 The diagnosis is confirmed by histology and immunohistochemical examination of the tissue biopsy positive for CD20. The treatment options are antibiotics (H pylori eradication therapy), chemotherapy, or radiotherapy alone, or a combination. Simultaneous (synchronous) GIST and MALT lymphoma of the stomach are very rare and have been reported only once in the literature.3 There is one published report of synchronous primary adenocarcinoma, MALT lymphoma, and GIST.11 The presence of two malignancies in our patient seems to be a coincidental rather than due to a common etiological triggering factor. There was no incidence of H pylori infection either. Surgery is the standard treatment for GIST (5-year survival of 48% to 65%).2 Imitinib mesylate results in encouraging responses particularly in patients with metastatic GIST. Chemotherapy is the first-line treatment in MALT lymphoma (5-year survival is 91%).12 In our patient, there was a strong suspicion of coexisting tumors of the stomach, so a decision was made to proceed with surgery. It provided effective treatment and resulted in an accurate diagnosis of both malignancies. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The authors indicated no potential conflicts of interest.
REFERENCES 1. Florence Duddaud, Jean-Yves Blay: Gastrointestinal stromal tumours: Biology and treatment. Oncology 65:187-197, 2003[CrossRef][Medline] 2. Connolly EM, Gaffney E, Reynolds JV: Gastrointestinal stromal tumours. Brit J Surg 90:1178-1186, 2003[CrossRef][Medline] 3. Salar A, Ramon JM, Barranco C, et al: Synchronous mucosa-associated lymphoid tissue lymphoma and gastrointestinal stromal tumors of the stomach. J Clin Oncol 23:7221-7223, 2005 4. Nakagawa M, Akasaka Y, Kanai T, et al: Clinicopathological and immunohistochemical features of extragastrointestinal stromal tumours: Report of two cases. Surg Today 35:336-340, 2005[CrossRef][Medline] 5. DeMatteo RP, Heinrich MC, El-Rifai W, et al: Clinical management of gastrointestinal stromal tumours: Before and after ST1-571. Hum Pathol 33:446-447, 2002 6. Kobayashi M, Okamoto K, Nakatani H, et al: Complete remission of recurrent gastrointestinal stromal tumours after treatment with imatinib: Report of a case. Surg Today 36:727-732, 2006[CrossRef][Medline] 7. Montalban C, Castrillo JM, Abraira V, et al: Gastric B-cell mucosa–associated lymphoid tissue (MALT) lymphoma: Clinicopathological study and evaluation of the prognostic factors in 143 patients. Ann Oncol 6:355-362, 1995 8. Du MQ, Isaacson PG: Recent advances in our understanding of the biology and pathogenesis of gastric mucosa-associated lymphoid tissue (MALT) lymphoma. Forum (Genova) 8:162-173, 1998 9. Wotherspoon AC, Doglioni C, Diss TC, et al: Regression of primary low-grade B-cell gastric lymphoma of mucosa-associated lymphoid tissue type after eradication of Helicobacter pylori. Lancet 342:575-577, 1993[CrossRef][Medline] 10. Zucca E, Bertoni F, Roggero E, et al: Molecular analysis of the progression from Helicobacter pylori-associated chronic gastritis to mucosa-associated lymphoid- tissue lymphoma of the stomach. N Engl J Med 338:804-810, 1998 11. Kaffes A, Hughes L, Hollinshead J, et al: Synchronous primary adenocarcinoma, mucosa-associated lymphoid tissue lymphoma and a stromal tumour in a Helicobacter pylori-infected stomach. J Gastroenterol Hepatol 17:1033-1036, 2002[CrossRef][Medline] 12. Didley P, Bennett MK: Pathology of benign, malignant and premalignant oesophageal and gastric tumours, in Griffin SM, Raimes SA (ed): A Companion to Specialist Surgical Practice: Upper Gastrointestinal surgery (ed 2). Oxford, United Kingdom, Harcourt Publishers Limited, 2001, pp 25-27
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Copyright © 2007 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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