|
Advertisement |
|
|
||
 |
|
|||||
|
|||||
|
 |
||||||
|
||||||
|
||||||
|
Journal of Clinical Oncology, Vol 23, No 28 (October 1), 2005: pp. 7221-7223 © 2005 American Society of Clinical Oncology. DOI: 10.1200/JCO.2005.08.158
Double Diagnosis in Cancer Patients and Cutaneous Reaction Related to GemcitabineCASE 1. Synchronous Mucosa-Associated Lymphoid Tissue Lymphoma and Gastrointestinal Stromal Tumors of the StomachDepartments of Clinical Hematology, Surgery, Pathology, and Endoscopy, Hospital del Mar, Barcelona, Spain A 54-year-old woman presented with vague and increasing abdominal discomfort of several months’ duration. She also complained of fatigue but had no complaints of bleeding, fever, night sweats, or weight loss. Her past medical history was unremarkable. There was no family history of gastrointestinal disease. Physical examination was normal. Laboratory investigations showed a hemoglobin count of 11.1 g/dL and a serum ferritin level of 12 ng/mL (normal range, 30 to 400 ng/mL). Computed tomography (CT) scan of the abdomen showed a heterogeneous mass of 6.4 cm in diameter adjacent to the greater curvature of the stomach, and thickening of the gastric wall located immediately contiguous to the mass (Fig 1). Gastric endoscopy showed thickened folds in the greater curvature of the corpus; several biopsies were obtained from the corpus. Histologic examination revealed infiltration of the mucosa by atypical lymphoid cells, characteristic lymphoepithelial lesions, and Helicobacter pylori organisms, consistent with the diagnosis of low-grade lymphoma of the mucosa-associated lymphoid tissue (MALT) type (Fig 2). Eradication therapy was administered against H pylori with amoxicilin, clarithromycin, and omeprazol for 7 days. Three months later, a CT scan showed no change in the size of the gastric mass and the patient was then referred to our center. Endoscopic examination, including gastric mapping,1 confirmed eradication of H pylori, but lymphoma was still seen in the biopsies of the corpus. Polymerase chain reaction for immunoglobulin heavy chain gene rearrangement confirmed clonality, and ruled out t(11;18)(q21;q21). Chemotherapy with intravenous fludarabine was initiated but after three courses no response was seen in the CT of the abdomen. Surgical resection was then considered in order to rule out a different etiology of the gastric tumor. Surgical examination showed a gastric mass that arose from a peduncle of the lesser omentum; it was completely removed without difficulty. Histologically, the tumor was monomorphic, composed of spindle-shaped cells (Fig 3), and immunohistochemical examination showed that it was positive for CD34 (Fig 3) and CD117, but negative for smooth-muscle actin. Proliferation activity was low (Ki67 < 1%) and cytogenetic studies showed a normal karyotype. These findings were consistent with a diagnosis of gastrointestinal stromal tumor (GIST). Of note, lymphoid infiltration consistent with MALT lymphoma could be demostrated in the peduncle of the stomach tumor that had been resected (Fig 4; MALT in the upper left corner and GIST in the lower right corner; arrows). The patient had an uneventful postoperative course. Two months after her operation, CT scan and gastric endoscopy were normal. Histologic and molecular remission were confirmed in all biopsy samples taken during the gastric mapping.
Primary gastric lymphoma is a rare tumor, accounting for fewer than 5% of primary gastric neoplasms.2 However, it is the most frequent stomach malignancy after adenocarcinoma, and the MALT type accounts for approximately 40% to 50% of all gastric lymphomas. These lymphomas develop in response to stimuli such as the presence of H pylori.3 Eradication of this organism has been shown to result in regression of the tumor in many cases, but there are a few tumors that will not respond to this stomach-conservative approach.4 Factors associated with a lower response rate of the lymphoma are an increased number of large cells,5 presence of perigastric lymph nodes, depth of infiltration of the gastric wall measured by endoscopic ultrasound,6 and some translocations involving the bcl-10 gene [t11,18(q21;q21) and t(1;14)(p22;q32)].7 GISTs are rare, with an incidence of 10 per million per annum, but are nevertheless the most common mesenchymal tumors to arise in the gastrointestinal tract.8 These tumors span a wide clinical spectrum from benign to malignant, and most of them are discovered incidentally. Surgery is the standard treatment for primary resectable tumors. However, these neoplasms have long been recognized for their nearly absolute resistance to chemotherapy and radiation treatment. Recently, mutations of the KIT gene have been recognized in the pathogenesis of this tumor, and molecular targeting of this critical mechanism with imatinib mesylate is particularly encouraging, showing potent activity even against metastatic GIST. Synchronous MALT lymphoma and adenocarcinoma of the stomach is a relatively rare occurrence. H pylori infection has been shown to play an early role in the generation of both types of neoplasms.9 To our knowledge, simultaneous MALT lymphoma and GIST of the stomach have been reported only once.10 That patient had H pylori infection and three simultaneous neoplasms of the stomach: adenocarcinoma, MALT lymphoma, and GIST. In the case under discussion, H pylori infection was also noted and could raise the question of a possible pathogenic link with GIST. However, we believe this theory unlikely, due to the high prevalence of H pylori infection and the very low incidence of GIST. The finding of two gastric neoplasms in our patient seems to be coincidental rather than related with the same pathogenic triggering. The need for surgery as a diagnostic or a therapeutic tool in the management of MALT lymphomas of the stomach has decreased in the last decade. Nowadays, staging is usually performed with endoscopy and noninvasive techniques, and a stomach-conserving approach consisting of antibiotic therapy, radiotherapy, or chemotherapy alone or in various combinations is favored. However, in those cases without an appropriate response to these therapeutic options, the presence of foci of large B-cell infiltration or an associated neoplasm should be ruled out. Then, surgical resection of the stomach may provide an accurate diagnosis and an effective treatment, such as in the patient of this report. Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES 1. Genta RM, Graham DY: Comparison of biopsy sites for the histopathologic diagnosis of Helicobacter pylori: A topographic study of H. pylori density and distribution. Gastrointest Endosc 40:342-345, 1994[Medline]
2. Isaacson PG: Gastric MALT lymphoma: From concept to cure. Ann Oncol 10:637-645, 1999
3. Isaacson PG, Spencer J: The biology of low grade MALT lymphoma. J Clin Pathol 48:395-397, 1995 4. 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] 5. de Jong D, Boot H, van Heerde P, et al: Histological grading in gastric lymphoma: Pretreatment criteria and clinical relevance. Gastroenterology 112:1466-1474, 1997[CrossRef][Medline]
6. Nakamura S, Matsumoto T, Suekane H, et al: Predictive value of endoscopic ultrasonography for regression of gastric low grade and high grade MALT lymphomas after eradication of Helicobacter pylori. Gut 48:454-460, 2001 7. Liu H, Ye H, Ruscone-Fourmestrau A, et al: T(11;18) is a marker for all stage gastric MALT lymphoma that will not respond to H. pylori eradication. Gastroenterology 122:1286-1294, 2002[CrossRef][Medline] 8. Joensuu H, Fletcher C, Dimitrijevic S, et al: Management of malignant gastrointestinal stromal tumours. Lancet Oncol 3:655-664, 2002[CrossRef][Medline] 9. Wotherspoon AC, Isaacson PG: Synchronous adenocarcinoma and low grade B-cell lymphoma of mucosa associated lymphoid tissue (MALT) of the stomach. Histopathology 27:325-331, 1995[Medline] 10. Kaffes A, Hughes L, Hollinshead J, et al: Synchronous primary adenocarcinoma, mucosa-associated lymphoid tissue lymphoma and a stromal tumor in a Helicobacter pylori-infected stomach. J Gastroenterol Hepatol 17:1033-1036, 2002[CrossRef][Medline]
This article has been cited by other articles:
|
|||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
|
|
|||||||||||
|
|||||||||||
|
|
Copyright © 2005 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
|
