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The endoscopic appearance of Burkitt's lymphoma involving the stomach and colon
with PEG in 25 patients with previous abdominal surgery. However, none of the cases reported had a cervical esophagogastrostomy with the stomach pulled into the chest cavity. To our knowledge, there are no previous reports of PED. Certainly, the placement of duodenal feeding tubes using the percutaneous endoscopic technique is a procedure that should be performed by an experienced endoscopist and in selected cases such as the one described here.
REFERENCES 1. Ponsky JL, Gauderer MWL. Percutaneous endoscopic gastrostomy: a nonoperative technique for feeding gastrostomy. Gastrointest Endosc 1981;27:9-11. 2. Ponsky JL, Aszodi A. Percutaneous endoscopic jejunostomy. Am J GastroenteroI1984;79:113-6. 3. Gottfried EB, Plumser AB. Endoscopic gastrojejunostomy: a technique to establish small bowel feeding without laparotomy. Gastrointest Endosc 1984;30:355-7. 4. Stellato TA, Gauderer MWL, Ponsky JL. Percutaneous endoscopic gastrostomy following previous abdominal surgery. Ann Surg 1984;200:46-50.
Case Reports The endoscopic appearance of Burkitt's lymphoma involving the stomach and colon W. Michael Priebe, MD
Burkitt's lymphoma, a distinct histopathological entity, l seems to primarily involve the gastrointestinal tract in American patients2 - 4 and less commonly in African patients in whom jaw tumors predominate. 1 ,4, 5 The Epstein-Barr virus probably has etiologic significance in Africans, but the role of this virus in causing American Burkitt's lymphoma is less clear. 4 ,6 Because of the rarity of this undifferentiated malignant lymphoma of the B cell type, endoscopic descriptions are lacking. Colonoscopic and upper gastrointestinal endoscopic findings in a case of Burkitt's lymphoma involving the colon and stomach are presented.
CASE REPORT A 27-year-old welder noted episodic, severe, crampy, left upper quadrant and lower abdominal pain in June 1981. For the preceding 2 weeks he had also noted the passage of bright red blood with stools and a small volume of red blood between bowel movements. An outpatient barium enema demonstrated a 5-cm sessile, rounded mass filling the lumen in the splenic flexure and a second similar lesion in the proximal sigmoid colon. An upper gastrointestinal series was reported as normal. Physical examination on an August hospital admission was unremarkable. The hemoglobin was 10.3 gjdl. Colonoscopy revealed a smooth, polypoid, friable mass, 4 cm in diameter, in the proximal sigmoid colon. The surface was hard with shallow lobulation and a central umbilication; From St. Joseph Hospital and Health Care Center, Tocoma, Washington. Reprint requests: W. Michael Priebe, MD, Gastroenterology, 721 Fawcett, Suite 103, Tocoma, Washington 98402.
there was a light green hue to the tumor (Fig. 1). A second, slightly larger lesion with a similar appearance could be seen in the splenic flexure. The remainder of the colon was normal. Biopsies of both lesions revealed an undifferentiated small cell malignant tumor of uncertain etiology along with extensive superficial necrosis. A laparotomy demonstrated intussusception of the sigmoid colon lesion, but no evidence of extraintestinal tumor. An unexpected finding was a large mass involving the midbody of the stomach. Since resection would have resulted in a near total gastrectomy, the stomach was left in place and a left hemicolectomy was performed (Fig. 2). Histologic sections of both colonic lesions revealed monotonous sheaths of malignant round cells with interspersed histocytes giving the classical "starry sky" appearance of Burkitt's lymphomaS (Fig. 3). Mesenteric lymph node histology was normal. Upper gastrointestinal endoscopy postoperatively demonstrated a single "doughnut-shaped" mass, 6 cm in diameter, involving the mid-gastric body along the greater curvature with extension onto the anterior wall (Fig. 4). The margin was heaped up, irregular, and friable. A large, central, excavated ulcer crater was covered with a greenish yellow exudate. The mucosa proximal and distal to the mass was normal, as was the duodenum to the third portion. Biopsies of the tumor confirmed the presence of gastric Burkitt's lymphoma, but specimens proximal and distal to the mass showed only normal gastric epithelium. Over the ensuing months the patient received combination chemotherapy including cyclophosphamide, doxorubicin, vincristine, prednisone, intrathecal cytosine arabinoside, and high dose methotrexate with leucovorin rescue. At repeat upper gastrointestinal endoscopy in November 1981, the gastric mass had melted away, leaving only a small residual ulcer crater 0.5 cm in diameter with radiating gastric folds (Fig. 5). Biopsies of the ulcer margin showed only mild nonspecific gastritis. Follow-up endoscopy in March 1982, after completion of chemotherapy, revealed re-epithelization of the previous ulcer crater and persistent radiating gastric
Figure 1. Colonoscopic view of a 4-cm sessile mass in the sigmoid colon with a distinctive olive green color. The mass was later shown to be Burkitt's lymphoma. Figure 2. Left hemicolectomy specimen showing polypoid masses of Burkitt's lymphoma involving the splenic flexure and sigmoid colon. 352
GASTROINTESTINAL ENDOSCOPY
Figure 3. Histologic section of the sigmoid colon mass showing sheets of malignant lymphocytes with interspersed histocytes, the classical "starry sky" appearance of Burkitt's lymphoma (H & E, X100). Figure 4. Upper endoscopic view of the large "doughnut-shaped" gastric mass of Burkitt's lymphoma with a large central crater. Figure 5. Residual small gastric ulcer with radiating folds following disappearance of the Burkitt's lymphoma mass after chemotherapy.
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folds. Biopsies showed a mild chronic inflammation. In April, a second-look laparotomy including a full-thickness biopsy of the gastric wall demonstrated a normal-appearing stomach without evidence of residual Burkitt's lymphoma either grossly or microscopically. The patient currently remains in complete remission from Burkitt's lymphoma and has been free of gastrointestinal symptoms for 2.5 years following completion of chemotherapy. DISCUSSION
Burkitt's lymphoma in American patients typically presents in the young, with a mean of 11 years of age (range, 2 to 30).7 The male to female ratio is approximately 2:1.4.7 Like other non-Hodgkin lymphomas, this entity commonly involves the gastrointestinal tract, with the ileum, colon, and stomach most often involved in decreasing frequency.2,3,7,8 Patients usually present with a short history of abdominal pain. 9 Gastrointestinal bleeding occurred in this patient, but the incidence in patients with Burkitt's lymphoma is unclear from the literature. Gastrointestinal blood loss may occur in lymphomas of non-Burkitt's type in 15% to 30% of patients.9 , 10 In a previous endoscopic description of Burkitt's lymphoma,l1 the tumor involved the stomach and duodenum and was also widespread within the abdominal and pelvic cavities. Endoscopic features included large gastric ulcers with nodular borders, 3- to 5-mm, "coffee cup"-shaped ulcers of the gastric body, and a friable mass in the duodenal second stage. In contrast, the present case had three large isolated areas of involvement (two polypoid colonic lesions and a single ulcerative gastric mass), and the lymphoma was confined strictly to the gastrointestinal tract. The colonic lesions differed from the gastric mass in color, size, shape, texture, and surface characteristics. The unusual olive green surface color of both colonic masses (Fig. 1) may have represented biliverdin formation due to the extensive superficial necrosis. Necrotic tumors typically have varied surface coloration depending upon the degree of necrosis and hemorrhage. 12 The endoscopic features of Burkitt's lymphoma in-
354
volving the gastrointestinal tract will probably vary with the areas involved, the degree of tumor extension, and the number of tumor foci but, in general, will resemble those in other non-Hodgkin's lymphomas. This unusual tumor should be kept in mind as a cause of gastrointestinal bleeding. ACKNOWLEDGMENTS
Drs. R. Garth McBride, H. Irving Pierce, and William H. Martin assisted in patient care. Dr. Jerome B. Simon reviewed the manuscript and Patricia Weiding and Patricia Marek provided secretarial assistance.
REFERENCES 1. O'Connor GT. Malignant lymphoma in African children. II. A pathological entity. Cancer 1961;14:270-83. 2. Arseneau JC, Canellos GP, Banks PM, Berard CW, Gralnick HR, DeVita VT. American Burkitt's lymphoma: a clinicopathologic study of 30 cases. I. Clinical factors relating to prolonged survival. Am J Med 1975;58:314-21. 3. Dunnick NR, Reaman GH, Head GL, Shawker TH, Ziegler JL. Radiographic manifestations of Burkitt's lymphoma in American patients. Am J Radiol 1979;132:1-6. 4. Ziegler JL. Burkitt's lymphoma. Med Clin North Am 1977;61:1073-82. 5. Wright DH. Microscopic features, histochemistry, histogenesis and diagnosis. In: Burkitt DP, Wright DH, eds. Burkitt's lymphoma. London: E&S Livingstone, 1970:82-102. 6. Judson SC, Henle W, Henle G. A cluster of Epstein-Barr virusassociated American Burkitt's lymphoma. N Engl J Med 1977;297:464-8. 7. Banks PM, Arseneau JC, Gralnick HR, Canellos GP, DeVita VT, Berard CWo American Burkitt's lymphoma: a clinicopathologic study of 30 cases. II. Pathologic correlations. Am J Med 1975;58:322-9. 8. Lewin KJ, Ranchod M, Dorfman RF. Lymphomas of the gastrointestinal tract. Cancer 1978;42:693-707. 9. Ziegler JL, Magrath IT, Burkitt's lymphoma. In: Ioachim HL, ed. Pathobiology annual. New York: Appleton-Century-Crofts, 1974:129-42. 10. Gray GM, Rosenberg SA, Cooper AD, Gregory PB, Stein DT, Herzenberg H. Lymphomas involving the gastrointestinal tract. Gastroenterology 1982;82:143-52. 11. Collins J, Katon R, Harty-Golder B. Burkitt's lymphoma presenting with gastroduodenal involvement-endoscopic description and review of the literature. Gastroenterology 1983;85:4259. 12. Sandritter W, Thomas C. Introduction-diagnostic criteria. In: Kirsten WH, ed. Color atlas and textbook of macropathology. Chicago: Year Book Medical Publishers Inc, 1976.
GASTROINTESTINAL ENDOSCOPY
REFERENCES 1. Ponsky JL, Gauderer MWL. Percutaneous endoscopic gastrostomy: a nonoperative technique for feeding gastrostomy. Gastrointest Endosc 1981;27:9-11. 2. Ponsky JL, Aszodi A. Percutaneous endoscopic jejunostomy. Am J GastroenteroI1984;79:113-6. 3. Gottfried EB, Plumser AB. Endoscopic gastrojejunostomy: a technique to establish small bowel feeding without laparotomy. Gastrointest Endosc 1984;30:355-7. 4. Stellato TA, Gauderer MWL, Ponsky JL. Percutaneous endoscopic gastrostomy following previous abdominal surgery. Ann Surg 1984;200:46-50.
Case Reports The endoscopic appearance of Burkitt's lymphoma involving the stomach and colon W. Michael Priebe, MD
Burkitt's lymphoma, a distinct histopathological entity, l seems to primarily involve the gastrointestinal tract in American patients2 - 4 and less commonly in African patients in whom jaw tumors predominate. 1 ,4, 5 The Epstein-Barr virus probably has etiologic significance in Africans, but the role of this virus in causing American Burkitt's lymphoma is less clear. 4 ,6 Because of the rarity of this undifferentiated malignant lymphoma of the B cell type, endoscopic descriptions are lacking. Colonoscopic and upper gastrointestinal endoscopic findings in a case of Burkitt's lymphoma involving the colon and stomach are presented.
CASE REPORT A 27-year-old welder noted episodic, severe, crampy, left upper quadrant and lower abdominal pain in June 1981. For the preceding 2 weeks he had also noted the passage of bright red blood with stools and a small volume of red blood between bowel movements. An outpatient barium enema demonstrated a 5-cm sessile, rounded mass filling the lumen in the splenic flexure and a second similar lesion in the proximal sigmoid colon. An upper gastrointestinal series was reported as normal. Physical examination on an August hospital admission was unremarkable. The hemoglobin was 10.3 gjdl. Colonoscopy revealed a smooth, polypoid, friable mass, 4 cm in diameter, in the proximal sigmoid colon. The surface was hard with shallow lobulation and a central umbilication; From St. Joseph Hospital and Health Care Center, Tocoma, Washington. Reprint requests: W. Michael Priebe, MD, Gastroenterology, 721 Fawcett, Suite 103, Tocoma, Washington 98402.
there was a light green hue to the tumor (Fig. 1). A second, slightly larger lesion with a similar appearance could be seen in the splenic flexure. The remainder of the colon was normal. Biopsies of both lesions revealed an undifferentiated small cell malignant tumor of uncertain etiology along with extensive superficial necrosis. A laparotomy demonstrated intussusception of the sigmoid colon lesion, but no evidence of extraintestinal tumor. An unexpected finding was a large mass involving the midbody of the stomach. Since resection would have resulted in a near total gastrectomy, the stomach was left in place and a left hemicolectomy was performed (Fig. 2). Histologic sections of both colonic lesions revealed monotonous sheaths of malignant round cells with interspersed histocytes giving the classical "starry sky" appearance of Burkitt's lymphomaS (Fig. 3). Mesenteric lymph node histology was normal. Upper gastrointestinal endoscopy postoperatively demonstrated a single "doughnut-shaped" mass, 6 cm in diameter, involving the mid-gastric body along the greater curvature with extension onto the anterior wall (Fig. 4). The margin was heaped up, irregular, and friable. A large, central, excavated ulcer crater was covered with a greenish yellow exudate. The mucosa proximal and distal to the mass was normal, as was the duodenum to the third portion. Biopsies of the tumor confirmed the presence of gastric Burkitt's lymphoma, but specimens proximal and distal to the mass showed only normal gastric epithelium. Over the ensuing months the patient received combination chemotherapy including cyclophosphamide, doxorubicin, vincristine, prednisone, intrathecal cytosine arabinoside, and high dose methotrexate with leucovorin rescue. At repeat upper gastrointestinal endoscopy in November 1981, the gastric mass had melted away, leaving only a small residual ulcer crater 0.5 cm in diameter with radiating gastric folds (Fig. 5). Biopsies of the ulcer margin showed only mild nonspecific gastritis. Follow-up endoscopy in March 1982, after completion of chemotherapy, revealed re-epithelization of the previous ulcer crater and persistent radiating gastric
Figure 1. Colonoscopic view of a 4-cm sessile mass in the sigmoid colon with a distinctive olive green color. The mass was later shown to be Burkitt's lymphoma. Figure 2. Left hemicolectomy specimen showing polypoid masses of Burkitt's lymphoma involving the splenic flexure and sigmoid colon. 352
GASTROINTESTINAL ENDOSCOPY
Figure 3. Histologic section of the sigmoid colon mass showing sheets of malignant lymphocytes with interspersed histocytes, the classical "starry sky" appearance of Burkitt's lymphoma (H & E, X100). Figure 4. Upper endoscopic view of the large "doughnut-shaped" gastric mass of Burkitt's lymphoma with a large central crater. Figure 5. Residual small gastric ulcer with radiating folds following disappearance of the Burkitt's lymphoma mass after chemotherapy.
VOLUME 32, NO.5, 1986
353
folds. Biopsies showed a mild chronic inflammation. In April, a second-look laparotomy including a full-thickness biopsy of the gastric wall demonstrated a normal-appearing stomach without evidence of residual Burkitt's lymphoma either grossly or microscopically. The patient currently remains in complete remission from Burkitt's lymphoma and has been free of gastrointestinal symptoms for 2.5 years following completion of chemotherapy. DISCUSSION
Burkitt's lymphoma in American patients typically presents in the young, with a mean of 11 years of age (range, 2 to 30).7 The male to female ratio is approximately 2:1.4.7 Like other non-Hodgkin lymphomas, this entity commonly involves the gastrointestinal tract, with the ileum, colon, and stomach most often involved in decreasing frequency.2,3,7,8 Patients usually present with a short history of abdominal pain. 9 Gastrointestinal bleeding occurred in this patient, but the incidence in patients with Burkitt's lymphoma is unclear from the literature. Gastrointestinal blood loss may occur in lymphomas of non-Burkitt's type in 15% to 30% of patients.9 , 10 In a previous endoscopic description of Burkitt's lymphoma,l1 the tumor involved the stomach and duodenum and was also widespread within the abdominal and pelvic cavities. Endoscopic features included large gastric ulcers with nodular borders, 3- to 5-mm, "coffee cup"-shaped ulcers of the gastric body, and a friable mass in the duodenal second stage. In contrast, the present case had three large isolated areas of involvement (two polypoid colonic lesions and a single ulcerative gastric mass), and the lymphoma was confined strictly to the gastrointestinal tract. The colonic lesions differed from the gastric mass in color, size, shape, texture, and surface characteristics. The unusual olive green surface color of both colonic masses (Fig. 1) may have represented biliverdin formation due to the extensive superficial necrosis. Necrotic tumors typically have varied surface coloration depending upon the degree of necrosis and hemorrhage. 12 The endoscopic features of Burkitt's lymphoma in-
354
volving the gastrointestinal tract will probably vary with the areas involved, the degree of tumor extension, and the number of tumor foci but, in general, will resemble those in other non-Hodgkin's lymphomas. This unusual tumor should be kept in mind as a cause of gastrointestinal bleeding. ACKNOWLEDGMENTS
Drs. R. Garth McBride, H. Irving Pierce, and William H. Martin assisted in patient care. Dr. Jerome B. Simon reviewed the manuscript and Patricia Weiding and Patricia Marek provided secretarial assistance.
REFERENCES 1. O'Connor GT. Malignant lymphoma in African children. II. A pathological entity. Cancer 1961;14:270-83. 2. Arseneau JC, Canellos GP, Banks PM, Berard CW, Gralnick HR, DeVita VT. American Burkitt's lymphoma: a clinicopathologic study of 30 cases. I. Clinical factors relating to prolonged survival. Am J Med 1975;58:314-21. 3. Dunnick NR, Reaman GH, Head GL, Shawker TH, Ziegler JL. Radiographic manifestations of Burkitt's lymphoma in American patients. Am J Radiol 1979;132:1-6. 4. Ziegler JL. Burkitt's lymphoma. Med Clin North Am 1977;61:1073-82. 5. Wright DH. Microscopic features, histochemistry, histogenesis and diagnosis. In: Burkitt DP, Wright DH, eds. Burkitt's lymphoma. London: E&S Livingstone, 1970:82-102. 6. Judson SC, Henle W, Henle G. A cluster of Epstein-Barr virusassociated American Burkitt's lymphoma. N Engl J Med 1977;297:464-8. 7. Banks PM, Arseneau JC, Gralnick HR, Canellos GP, DeVita VT, Berard CWo American Burkitt's lymphoma: a clinicopathologic study of 30 cases. II. Pathologic correlations. Am J Med 1975;58:322-9. 8. Lewin KJ, Ranchod M, Dorfman RF. Lymphomas of the gastrointestinal tract. Cancer 1978;42:693-707. 9. Ziegler JL, Magrath IT, Burkitt's lymphoma. In: Ioachim HL, ed. Pathobiology annual. New York: Appleton-Century-Crofts, 1974:129-42. 10. Gray GM, Rosenberg SA, Cooper AD, Gregory PB, Stein DT, Herzenberg H. Lymphomas involving the gastrointestinal tract. Gastroenterology 1982;82:143-52. 11. Collins J, Katon R, Harty-Golder B. Burkitt's lymphoma presenting with gastroduodenal involvement-endoscopic description and review of the literature. Gastroenterology 1983;85:4259. 12. Sandritter W, Thomas C. Introduction-diagnostic criteria. In: Kirsten WH, ed. Color atlas and textbook of macropathology. Chicago: Year Book Medical Publishers Inc, 1976.
GASTROINTESTINAL ENDOSCOPY