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Endoscopic appearance of primary appendiceal lymphoma
BRIEF REPORTS Endoscopic appearance of primary appendiceal lymphoma Masanori Furuse, MD Kunihiko Aoyagi, MD Motohiro Esaki, MD Tsuyoshi Shigemura, MD Shotaro Nakamura, MD Kazuoki Hizawa, MD Toshio Kozuki, MD Takashi Yao, MD Yutaka Kimura, MD Masatoshi Fujishima, MD
Primary appendiceal lymphoma is an extremely rare disease,1-8 and its endoscopic appearance has not been described. Because of the rarity of the disease and the similarity of its presentation to that of appendicitis, preoperative diagnosis is difficult.5 In a previous report, abdominal ultrasound was performed, but no specific finding for appendiceal lymphoma was found.7 This is the first report of the endoscopic appearance of a primary appendiceal lymphoma. CASE REPORT A 67-year-old man presented with epigastric pain for 2 to 3 days. Although he had not felt pain in the right lower quadrant, physical examination revealed tenderness (without rebound tenderness) at that location. No superficial lymphadenopathy could be detected. Laboratory studies, including a complete blood count, urinalysis, and serum chemistry were unremarkable. Stool samples were positive for occult blood. Upper gastrointestinal endoscopy was normal. Abdominal ultrasound and CT showed a hemispheric mass at the tip of cecum. Colonoscopy disclosed a polypoid lesion at the appendiceal orifice. The overlying mucosa was ulcerated and irregular (Fig. 1A). The mass was circumscribed by folds (Fig. 1B). Biopsies from the surface mucosa showed aggregation of atypical lymphoid cells, suggesting lymphoma. Malignant lymphoma was mainly suspected based on the biopsy findings. An ileocecal resection with mesenteric lymph node dissection was performed. Macroscopically, the resected appendix was swollen, measuring From Second Department of Internal Medicine, Faculty of Medicine, the Second Department of Pathology, Kyushu University, and Kimura Surgical Hospital, Fukuoka, Japan. Reprint requests: Masanori Furuse, MD, Second Department of Internal Medicine, Faculty of Medicine, Kyushu Univeristy, Maidashi 3-1-1, Higashi-ku, Fukuoka 812-8582, Japan. Copyright © 1998 by the American Society for Gastrointestinal Endoscopy 0016-5107/98/$5.00 + 0 37/54/89876 86
GASTROINTESTINAL ENDOSCOPY
Figure 1. A, Endoscopic view of the appendiceal lymphoma protruding into the cecum, accompanied by irregular shaped ulceration and erosion. B, “Vortical folds” observed at the base of the lesion indicative of the appendiceal origin. 3 × 3 × 7 cm (Fig. 2). Histologically the tumor was composed of a diffuse proliferation of medium-sized atypical lymphoid cells (centrocytes) admixed with large centroblasts, forming sparse lymphomatous follicles (Fig. 3). Immunohistochemically, the neoplastic cells were Kappa(+), Lambda(–), CD3(–), CD20(+), CD43(–), CD45(+), CD45RO(–), and bcl-2(+). Based on these findings, the tumor was diagnosed as low-grade B-cell centroblasticcentrocytic lymphoma, according to the updated Kiel classification.9 The patient underwent postoperative CHOP chemotherapy (cyclophosphamide 750 mg/m3, pirarubicin 50 mg/m3, vincristine 1.4 mg/m3 each i.v. bolus, and prednisolone 50 mg/m3 i.v. for 5 days). Twenty months after admission, the patient was doing well, in full remission with no evidence of recurrence.
DISCUSSION A review of the literature disclosed that the symptoms of appendiceal lymphoma include right VOLUME 48, NO. 1, 1998
Brief Reports
K Aoyagi, M Esaki, T Shigemura, et al.
Figure 3. Photomicrograph showing diffuse, medium-sized atypical lymphoid cells (hematoxylin and eosin staining, original magnification × 510). Figure 2. Resection specimen with longitudinal section of the appendix. The tumor is primarily confined to the appendix.
lower quadrant pain, nausea, and vomiting5 and that patients ranged in age from 4 to 77 years. Because of similar symptoms, most patients are misdiagnosed as having appendicitis and undergo surgical exploration without prior endoscopic evaluation. In the present case, colonoscopy revealed a polypoid mass with circumscribed folds at the base of the tumor. These folds may correspond to the socalled “vortical folds” that have been used as a radiographic indicator of appendiceal masses including mucoceles.10,11 These folds have also been observed endoscopically in some appendiceal tumors.12,13 However this endoscopic appearance is not specific for appendiceal lymphoma. In this case, the vortical folds indicated appendiceal origin and the irregularly shaped ulceration suggested malignancy. Biopsy was indicative of lymphoma. It may be difficult to preoperatively diagnose a primary appendiceal lymphoma because it is extremely rare and because prompt surgical intervention is indicated for appendicitis. Fewer than 60 cases of primary appendiceal lymphomas have been reported worldwide.1-8 Most were found incidentally and diagnosed postoperatively by pathologic findings in resected specimens. To our knowledge, there is no case where preoperative endoscopy was performed. Further characterization of the endoscopic features of primary appendiceal lymphomas would be desirable. Furthermore preoperative colonoscopy with biopsy, if possible, may be helpful for identifying a VOLUME 48, NO. 1, 1998
patient with a primary appendiceal lymphoma. This may be particularly useful in patients who present with atypical clinical features of acute appendicitis. REFERENCES 1. Lewin KJ, Ranchod M, Dorfman RF. Lymphomas of the gastrointestinal tract. A study of 117 cases presenting with gastrointestinal disease. Cancer 1978;42:693-707. 2. Loehr WJ, Mujahed Z, Zahn FD, Gray GF, Thorbjarnarson B. Primary lymphoma of the gastrointestinal tract: a review of 100 cases. Ann Surg 1969;170:232-8. 3. Contreary K, Nance FC, Becker WF. Primary lymphoma of the gastrointestinal tract. Ann Surg 1980;191:593-8. 4. Collins DC. 71000 human appendix specimens. A final report summarizing 40 years’ study. Am J Proctol 1963;14:365-81. 5. Pasquale MD, Shabahang M, Bitterman P, Lack EE, Evans SRT. Primary lymphoma of the appendix. Case report and review of the literature. Surg Oncol 1994;3:243-8. 6. Stewart RJ, Mirakhur M. Primary malignant lymphoma of the appendix. Ulster Med J 1986;55:187-9. 7. Mori M, Kusunoi T, Kikuchi M, Motoori T, Sugimachi K. Primary malignant lymphoma of the appendix. Jpn J Surg 1985;15:230-3. 8. Rao SK, Aydinalp S. Appendiceal lymphoma: a case report. J Clin Gastroenterol 1991;13:588-90. 9. Stansfeld AG, Diebold J, Kapanci Y, Kelenyi G, Lennert K, Mioduszewska O, et al. Updated Kiel classification for lymphomas. Lancet 1988;1:292-3,603. 10. Euphrat EJ. Roentgen features of mucocele of the appendix. Radiology 1947;48:113-7. 11. Dachman AH, Lichtenstein JE, Friedman AC. Mucocele of the appendix and pseudomyxoma peritonei. Am J Roentgenol 1985;144:923-9. 12. Raijman I, Leong S, Hassaram N, Marcon NE. Appendiceal mucocele: endoscopic appearance. Endoscopy 1994;26: 326-8. 13. Hy Trinh D, Rether L, Ronsse H, Juvin T, Mouton A, Peron R. Adenocarcinoma of the appendix: a radioendoscopic diagnosis. Gastrointest Endosc 1988;34:155-6. GASTROINTESTINAL ENDOSCOPY
87
Primary appendiceal lymphoma is an extremely rare disease,1-8 and its endoscopic appearance has not been described. Because of the rarity of the disease and the similarity of its presentation to that of appendicitis, preoperative diagnosis is difficult.5 In a previous report, abdominal ultrasound was performed, but no specific finding for appendiceal lymphoma was found.7 This is the first report of the endoscopic appearance of a primary appendiceal lymphoma. CASE REPORT A 67-year-old man presented with epigastric pain for 2 to 3 days. Although he had not felt pain in the right lower quadrant, physical examination revealed tenderness (without rebound tenderness) at that location. No superficial lymphadenopathy could be detected. Laboratory studies, including a complete blood count, urinalysis, and serum chemistry were unremarkable. Stool samples were positive for occult blood. Upper gastrointestinal endoscopy was normal. Abdominal ultrasound and CT showed a hemispheric mass at the tip of cecum. Colonoscopy disclosed a polypoid lesion at the appendiceal orifice. The overlying mucosa was ulcerated and irregular (Fig. 1A). The mass was circumscribed by folds (Fig. 1B). Biopsies from the surface mucosa showed aggregation of atypical lymphoid cells, suggesting lymphoma. Malignant lymphoma was mainly suspected based on the biopsy findings. An ileocecal resection with mesenteric lymph node dissection was performed. Macroscopically, the resected appendix was swollen, measuring From Second Department of Internal Medicine, Faculty of Medicine, the Second Department of Pathology, Kyushu University, and Kimura Surgical Hospital, Fukuoka, Japan. Reprint requests: Masanori Furuse, MD, Second Department of Internal Medicine, Faculty of Medicine, Kyushu Univeristy, Maidashi 3-1-1, Higashi-ku, Fukuoka 812-8582, Japan. Copyright © 1998 by the American Society for Gastrointestinal Endoscopy 0016-5107/98/$5.00 + 0 37/54/89876 86
GASTROINTESTINAL ENDOSCOPY
Figure 1. A, Endoscopic view of the appendiceal lymphoma protruding into the cecum, accompanied by irregular shaped ulceration and erosion. B, “Vortical folds” observed at the base of the lesion indicative of the appendiceal origin. 3 × 3 × 7 cm (Fig. 2). Histologically the tumor was composed of a diffuse proliferation of medium-sized atypical lymphoid cells (centrocytes) admixed with large centroblasts, forming sparse lymphomatous follicles (Fig. 3). Immunohistochemically, the neoplastic cells were Kappa(+), Lambda(–), CD3(–), CD20(+), CD43(–), CD45(+), CD45RO(–), and bcl-2(+). Based on these findings, the tumor was diagnosed as low-grade B-cell centroblasticcentrocytic lymphoma, according to the updated Kiel classification.9 The patient underwent postoperative CHOP chemotherapy (cyclophosphamide 750 mg/m3, pirarubicin 50 mg/m3, vincristine 1.4 mg/m3 each i.v. bolus, and prednisolone 50 mg/m3 i.v. for 5 days). Twenty months after admission, the patient was doing well, in full remission with no evidence of recurrence.
DISCUSSION A review of the literature disclosed that the symptoms of appendiceal lymphoma include right VOLUME 48, NO. 1, 1998
Brief Reports
K Aoyagi, M Esaki, T Shigemura, et al.
Figure 3. Photomicrograph showing diffuse, medium-sized atypical lymphoid cells (hematoxylin and eosin staining, original magnification × 510). Figure 2. Resection specimen with longitudinal section of the appendix. The tumor is primarily confined to the appendix.
lower quadrant pain, nausea, and vomiting5 and that patients ranged in age from 4 to 77 years. Because of similar symptoms, most patients are misdiagnosed as having appendicitis and undergo surgical exploration without prior endoscopic evaluation. In the present case, colonoscopy revealed a polypoid mass with circumscribed folds at the base of the tumor. These folds may correspond to the socalled “vortical folds” that have been used as a radiographic indicator of appendiceal masses including mucoceles.10,11 These folds have also been observed endoscopically in some appendiceal tumors.12,13 However this endoscopic appearance is not specific for appendiceal lymphoma. In this case, the vortical folds indicated appendiceal origin and the irregularly shaped ulceration suggested malignancy. Biopsy was indicative of lymphoma. It may be difficult to preoperatively diagnose a primary appendiceal lymphoma because it is extremely rare and because prompt surgical intervention is indicated for appendicitis. Fewer than 60 cases of primary appendiceal lymphomas have been reported worldwide.1-8 Most were found incidentally and diagnosed postoperatively by pathologic findings in resected specimens. To our knowledge, there is no case where preoperative endoscopy was performed. Further characterization of the endoscopic features of primary appendiceal lymphomas would be desirable. Furthermore preoperative colonoscopy with biopsy, if possible, may be helpful for identifying a VOLUME 48, NO. 1, 1998
patient with a primary appendiceal lymphoma. This may be particularly useful in patients who present with atypical clinical features of acute appendicitis. REFERENCES 1. Lewin KJ, Ranchod M, Dorfman RF. Lymphomas of the gastrointestinal tract. A study of 117 cases presenting with gastrointestinal disease. Cancer 1978;42:693-707. 2. Loehr WJ, Mujahed Z, Zahn FD, Gray GF, Thorbjarnarson B. Primary lymphoma of the gastrointestinal tract: a review of 100 cases. Ann Surg 1969;170:232-8. 3. Contreary K, Nance FC, Becker WF. Primary lymphoma of the gastrointestinal tract. Ann Surg 1980;191:593-8. 4. Collins DC. 71000 human appendix specimens. A final report summarizing 40 years’ study. Am J Proctol 1963;14:365-81. 5. Pasquale MD, Shabahang M, Bitterman P, Lack EE, Evans SRT. Primary lymphoma of the appendix. Case report and review of the literature. Surg Oncol 1994;3:243-8. 6. Stewart RJ, Mirakhur M. Primary malignant lymphoma of the appendix. Ulster Med J 1986;55:187-9. 7. Mori M, Kusunoi T, Kikuchi M, Motoori T, Sugimachi K. Primary malignant lymphoma of the appendix. Jpn J Surg 1985;15:230-3. 8. Rao SK, Aydinalp S. Appendiceal lymphoma: a case report. J Clin Gastroenterol 1991;13:588-90. 9. Stansfeld AG, Diebold J, Kapanci Y, Kelenyi G, Lennert K, Mioduszewska O, et al. Updated Kiel classification for lymphomas. Lancet 1988;1:292-3,603. 10. Euphrat EJ. Roentgen features of mucocele of the appendix. Radiology 1947;48:113-7. 11. Dachman AH, Lichtenstein JE, Friedman AC. Mucocele of the appendix and pseudomyxoma peritonei. Am J Roentgenol 1985;144:923-9. 12. Raijman I, Leong S, Hassaram N, Marcon NE. Appendiceal mucocele: endoscopic appearance. Endoscopy 1994;26: 326-8. 13. Hy Trinh D, Rether L, Ronsse H, Juvin T, Mouton A, Peron R. Adenocarcinoma of the appendix: a radioendoscopic diagnosis. Gastrointest Endosc 1988;34:155-6. GASTROINTESTINAL ENDOSCOPY
87