Journal of Clinical Oncology, Vol 21, Issue 3
(February), 2003: 565-567
© 2003 American Society for Clinical Oncology
Uncommon Presentations of Non-Hodgkin’s Lymphoma
Case 2. Non-Hodgkin’s Lymphoma of the Ileocecal Region
Mario Chenal,
Hernan Molina,
Vinicio Mendez,
John Poole,
Carlos Parellada
Hospital General San Juan de Dios, Ciudad Guatemala, Guatemala
A 14-year-old Hispanic man presented with a 3-month history of abdominal pain, fever, weight loss, and episodes of bloody stools. Six years earlier, he had undergone an appendectomy for acute appendicitis. On physical examination, he had a temperature of 38°C, no superficial adenopathy, and a 12-cm palpable mass in the right-lower quadrant of the abdomen. Computed tomography scan of the abdomen showed thickening of the bowel wall at the level of the terminal ileum and cecum, without metastatic lesions in the retroperitoneum and abdominal viscera (Fig 1 ; 1 and 2 = tumor in bowel, 3 = normal liver). Chest x-ray and bone marrow biopsy were normal. A colonoscopy demonstrated a normal appearance of the large bowel mucosa from the rectum to the ascending colon. The mucosa at the cecum had an abnormal appearance with thickening, friability, and ulceration extending up to 8 cm into the terminal ileum (Fig 2; arrow). Biopsies taken from the disease area of the cecum showed colonic mucosa and submucosa infiltrated by abnormal large mononuclear cells (Fig 3; hematoxylin and eosin, x10). These cells had a round rhomboid or cerebriform enlarged nuclei; thick, irregular nuclear membrane and prominent nucleoli; and abundant clear or slightly basophilic cytoplasm resembling large lymphoma cells. Interspersed in this infiltrate there were small, normal-appearing lymphocytes (Fig 4; hematoxylin and eosin, x40). Immunophenotype of the large atypical lymphoid cells showed CD3-, CD20+, CD30-, CD45+, and CD68- cells, confirming their lymphoid nature, and AE1- and S-100- cells, ruling out epithelial origin. These findings were consistent with a diagnosis of diffuse large B-cell lymphoma.
Because of a poor nutritional condition and a large tumoral mass, the patient was initially treated with combination chemotherapy consisting of cyclophosphamide, doxorubicin, vincristine, and prednisone. By the end of six cycles of therapy, his symptoms had resolved and a complete response was established clinically, radiographically (as evidenced by repeated computed tomography scans), and endoscopically. He also achieved a pathologic complete response documented in biopsies taken during colonoscopy and in the right hemicolectomy/terminal ileum specimen obtained after surgical resection. One year after initial presentation, he remains in clinical complete response.
Primary gastrointestinal (GI) non-Hodgkin’s lymphoma (NHL) is a rare occurrence. It can be diagnosed by examining biopsies taken during endoscopic procedures, and treatment options include antibiotics, cytotoxic chemotherapy, surgery, and radiation. Population-based studies report an annual age-standardized incidence rate for GI lymphomas of less than 1 per 100,000 population, with the GI tract being the primary site of disease in 9% to 15% of all lymphomas.1–3 The intestine, and specifically the ileocecal region, is affected in 16% to 54% and 3% to 9% of GI NHL, respectively.1,3–5 The clinical presentation is characterized by abdominal pain, loss of appetite, constipation, and bleeding, with symptoms depending on the anatomic location of disease.2,4,6 For all GI lymphomas, the most common histologic subtypes are in the high-grade category, and this feature is more remarkable for disease localized to the intestine, where high-grade lymphomas represent a 53% to 85% frequency.1,3,4 Prognostic factors associated with better survival in GI lymphomas include young age, early stage, gastric localization, mucosa-associated lymphoid tissue (MALT) histology, radical or incomplete surgical resection, and achievement of a complete response.1,4–6
Survival for GI NHL is in the range of 44% to 75%. This rate can be improved for patients with intestinal lymphomas by performing surgical resection in addition to chemotherapy as the single therapeutic modality.1,5,6 Involvement of the ileocecal region has an event-free and overall survival rate similar to that of the gastric lymphomas, a feature that may relate to early disease and possibly local treatment of such areas.4 More studies addressing the sequence and results of single or combined therapy will help to clarify the role of surgery and radiation in cases like this.
REFERENCES
1. d’Amore F, Brincker H, Gronbaek K, et al: Non-Hodgkin’s lymphoma of the gastrointestinal tract: A population-based analysis of incidence, geographic distribution, clinicopathologic presentation features and prognosis. J Clin Oncol 12:1673–1684, 1994[Abstract/Free Full Text]
2. Ducreux M, Boutron MC, Piard F, et al: A 15-year series of gastrointestinal non-Hodgkin’s lymphomas: A population-based study. Br J Cancer 77:511–514, 1998[Medline]
3. Gurney KA, Cartwright RA, Gilman EA: Descriptive epidemiology of gastrointestinal non-Hodgkin’s lymphoma in a population-based registry. Br J Cancer 79:1929–1934, 1999[CrossRef][Medline]
4. Koch P, del Valle F, Berdel W, et al: Primary gastrointestinal non-Hodgkin’s lymphoma: I. Anatomic and histologic distribution, clinical features, and survival data of 371 patients registered in the German Multicenter Study GIT NHL 01/92. J Clin Oncol 19:3861–3873, 2001[Abstract/Free Full Text]
5. Morton JE, Leyland MJ, Vaughan Hudson G, et al: Primary gastrointestinal non-Hodgkin’s lymphoma: A review of 175 British National Lymphoma Investigation cases. Br J Cancer 67:776–782, 1993[Medline]
6. Ruskone-Fourmestraux A, Aegerter P, Delmer A, et al: Primary digestive tract lymphoma: A prospective multicentric study of 91 patients—Groupe d’Etude des Lymphomes Digestifs. Gastroenterology 105:1662–1671, 1993[Medline]
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