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Case report: Malignant lymphomatous polyposis of the colon
CASE REPORTS
797
Clinical Radiology (1997) 52, 797-798
Case Report: Malignant Lymphomatous Polyposis of the Colon M. P. CALLAWAY, D. G. O'DONOVAN* and S. H. LEE
Departments of Radiology and *Histopathology, Manchester Royal Infirmary, Manchester, UK A rare case of malignant lymphomatous polyposis (MLP) of the colon as demonstrated on double contrast barium enema (DCBE) is presented in a patient with an altered bowel habit. The diagnosis was made on combined clinical, radiological and pathological criteria following endoscopic biopsy of multiple colonic polyps. Histological examination showed the features of a non-Hodgkin's lymphoma of mantle cell type of the Revised European American Lymphoma (REAL) classification which is equivalent to the centrocytic type of the updated Kiel classification.
CASE REPORT A 65-year-old man was referred from the renal physicians for DCBE with a month's history of alternating bowel habit without rectal bleeding. He had recently started continuous ambulatory peritoneal dialysis for idiopathic end-stage renal failure. He also had a 10-year history of respiratory failure recently requiring continuous positive airways pressure (CPAP) ventilation to prevent noctural hypoxia. DCBE showed multiple submucosal polypoid filling defects in the colon with sparing of the sigmoid and rectum (Fig. 1). There was also a large filling defect in the caecum involving the terminal ileum (Fig. 2). Ultrasound examination of the abdomen confirmed the presence of polyps in the right hemicolon together with a large caecal mass (Fig. 3). Contrastenhanced computed tomography of the abdomen excluded the presence of retroperitoneal lymphadenopathy and confirmed the findings of a normal liver and spleen. The differential diagnosis included multiple adenomas and/or adenocarcinomas of the colon which suggested the syndrome of familial polyposis coli. Pneumatosis coli was a possibility especially in view of the patient's respiratory disease and its treatment with CPAP. However, sigmoid and rectal involvement would be expected in both these conditions. The patient underwent colonoscopy when multiple biopsies were taken from masses of pale grey tissue measuring up to 0.5 cm in diameter. Histological examination confirmed the polypoidal nature of the colonic mucosa and revealed a diffuse infiltrate of mantle cells in the lamina propria (Fig. 4). There were no lymphoepithelial lesions. Immunohistochemistry showed the cells of the neoplastic infiltrate were of a B-cell lineage. These appearances indicated an extranodal mantle cell lymphoma characteristic of malignant lymphomatous polyposis (MLP) of the colon.
Fig. 1 - Double contrast barium enema showing multiple broad based filling defects throughout the colon, with sigmoid sparing.
DISCUSSION Primary malignant lymphoma of the colon is rare accounting for 0.05% of gastrointestinal malignancies and about 0.1% of rectal malignancies [1,2]. The incidence is higher in men, with a sex ratio of 2:1, and the majority of patients are symptomatic complaining of abdominal pain, altered bowel habit and weight loss. They can be solitary or multiple on DCBE and may mimic other more common colonic tumours. Several studies note an increased incidence of colonic malignant lymphoma in patients with ulcerative colitis [1,3,4]. MLP is a primary malignant lymphoma arising in the gastrointestinal tract. It is a non-Hodgkin's lymphoma recently classified as an extranodal mantle cell lymphoma (REAL) [5] and it corresponds to an extranodal form of a pure centrocytic lymphoma (Kiel classification) [6-8]. Correspondence to: Dr S. H. Lee, Department of Radiology, Manchester Royal Infirmary, Oxford Road, Manchester MI3 9WL, UK. © 1997 The Royal College of Radiologists, ClinicalRadiology, 52, 794-802.
Fig. 2 - Caecum and right colon showing a large polypoid filling defect in the caecum.
Mantle cell lymphoma of the gastrointestinal tract differs histologically from other lymphomas of mucosal associated lymphoid tissue (MALToma) which show little tendency to dissemination beyond the mucosa and remain localized. Therefore, MLP, as in this case, carries a worse prognosis.
798
CLINICAL RADIOLOGY
Fig. 4 - Histological examination of the colon, revealing diffuse infiltrate of mantle cells in the lamina propria. Fig. 3 - Ultrasound of the caecum and terminal ileum showing polypoid tumour within the caecum, (white arrow) and solid tumour involving the terminal ileum (black arrow).
REFERENCES
Characteristically, MLP occurs at multiple sites in the colon and in a proportion of patients there may also be multiple lesions in the small bowel or stomach [10]. The sub-mucosal infiltrate generally projects into the bowel lumen and forms a sessile or pedunculated polyp [9]. MLP has a typical appearance .on DCBE of either a single or multiple submucosal polyps, with a wide sessile base, and often a central depression [10]. Other MALTomas tend to be localized and do not normally form polypoid masses. The appearances of MLP on DCBE may be confused with pneumatosis coli which typically shows the presence of submucosal gas on DCBE not seen in MLP or other polypoid tumours. Indeed one unusual case report described both diffuse colonic lymphoma and pneumatosis coli occurring together [11]. MLP is an aggressive mantle cell lymphoma. Its prognosis is worst among the low-grade B-cell lymphomas, due to widespread involvement of the gastrointestinal tract, and tendency for extra intestinal spread lead to a high stage at diagnosis. However, although no extraintestinal spread was demonstrated in this case, surgery was not performed due to the poor general condition of the patient. Surgical resection is often not feasible in MLP contrasting with observations in other MALTomas and management mainly involves the use of chemotherapy but the prognosis remains poor [8].
1 Dodd GD. Lymphoma of the hollow abdominal viscera. Radiologic Clinics of North America 1990;28:771-783. 2 Craig O, Gregson R. Primary lymphomas of the gastrointestinal tract. Clinical Radiology 1981 ;32:63-71. 3 Sheperd NA, Hall PA, Williams GT et al. Primary lymphoma of the large intestine complicating inflammatory bowel disease. Histopathology 1989;16:95-97. 4 Hill DH, Manton Mills JO, Maxwell RJ. Synchronous colonic lymphomas complicating chronic ulcerative colitis. Abdominal Imaging 1993;18:369-370. 5 Chan JKC. A new classification of lymphomas: the revised EuropeanAmerican lymphoma classification. Advances in Anatomical Pathology 1994;1:166-172. 6 Isaacson PG, Norton AJ. Mantle cell lymphoma (lymphomatous polyposis). In: Extranodal Lymphomas, 1st ed. Edinburgh: Churchill Livingstone, 1994:50-52. 7 Stansfeld AG, Diebold J, Noel H e t al. Updated Kiel classification for lymphomas. Lancet 1988;1:292-293. 8 Banks PM, Chan J, Cleary ML et al. Mantle cell lymphoma. A proposal for unification of morphologic, immunologic, and molecular data. American Journal of Surgical Pathology 1992;16:637-640. 9 Sheperd NA, Hall PA, Coates PJ, Levison DA. Primary lymphoma of the colon and rectum. A histological and immunohistochemical analysis of 45 cases with cfinicopathological correlations. Histopathology 1988; 12:235-252. 10 Kawamoto K, Motooka M, Hirata N e t al. Colonic submucosal tumors: a new classification based on radiologic characteristics. American Journal of Roentgenology 1993;160:315-320. 11 O'Connell DJ, ThompsonAJ, Lymphoma of the colon: the spectrumof radiologic changes. Gastrointestinal Radiology 1978;2:377-385.
Clinical Radiology (1997) 52, 798-800
Case Report: Cardiac Arrest Due to Coronary Artery Spasm During Angiographic Procedure I. SAKAMOTO, T. UCHIDA and K. HAYASHI
Department of Radiology, Nagasaki University School of Medicine, Nagasaki, Japan It is well known that coronary artery spasm (CAS) can be induced by exercise, cold pressor test, Valsalva manoeuvre, Correspondence to: Dr I. Sakamoto, Department of Radiology, Nagasaki University School of Medicine, 1-7-1 Sakamoto, Nagasaki 852, Japan.
and the administration of pharmacological agents such as acetylcholine [1]. However, CAS following the use of contrast material is extremely rare and all previously reported cases developed after direct intracoronary injection of contrast agents [2,3]. We describe a patient who © 1997 The Royal College of Radiologists, ClinicalRadiology, 52, 794-802.
797
Clinical Radiology (1997) 52, 797-798
Case Report: Malignant Lymphomatous Polyposis of the Colon M. P. CALLAWAY, D. G. O'DONOVAN* and S. H. LEE
Departments of Radiology and *Histopathology, Manchester Royal Infirmary, Manchester, UK A rare case of malignant lymphomatous polyposis (MLP) of the colon as demonstrated on double contrast barium enema (DCBE) is presented in a patient with an altered bowel habit. The diagnosis was made on combined clinical, radiological and pathological criteria following endoscopic biopsy of multiple colonic polyps. Histological examination showed the features of a non-Hodgkin's lymphoma of mantle cell type of the Revised European American Lymphoma (REAL) classification which is equivalent to the centrocytic type of the updated Kiel classification.
CASE REPORT A 65-year-old man was referred from the renal physicians for DCBE with a month's history of alternating bowel habit without rectal bleeding. He had recently started continuous ambulatory peritoneal dialysis for idiopathic end-stage renal failure. He also had a 10-year history of respiratory failure recently requiring continuous positive airways pressure (CPAP) ventilation to prevent noctural hypoxia. DCBE showed multiple submucosal polypoid filling defects in the colon with sparing of the sigmoid and rectum (Fig. 1). There was also a large filling defect in the caecum involving the terminal ileum (Fig. 2). Ultrasound examination of the abdomen confirmed the presence of polyps in the right hemicolon together with a large caecal mass (Fig. 3). Contrastenhanced computed tomography of the abdomen excluded the presence of retroperitoneal lymphadenopathy and confirmed the findings of a normal liver and spleen. The differential diagnosis included multiple adenomas and/or adenocarcinomas of the colon which suggested the syndrome of familial polyposis coli. Pneumatosis coli was a possibility especially in view of the patient's respiratory disease and its treatment with CPAP. However, sigmoid and rectal involvement would be expected in both these conditions. The patient underwent colonoscopy when multiple biopsies were taken from masses of pale grey tissue measuring up to 0.5 cm in diameter. Histological examination confirmed the polypoidal nature of the colonic mucosa and revealed a diffuse infiltrate of mantle cells in the lamina propria (Fig. 4). There were no lymphoepithelial lesions. Immunohistochemistry showed the cells of the neoplastic infiltrate were of a B-cell lineage. These appearances indicated an extranodal mantle cell lymphoma characteristic of malignant lymphomatous polyposis (MLP) of the colon.
Fig. 1 - Double contrast barium enema showing multiple broad based filling defects throughout the colon, with sigmoid sparing.
DISCUSSION Primary malignant lymphoma of the colon is rare accounting for 0.05% of gastrointestinal malignancies and about 0.1% of rectal malignancies [1,2]. The incidence is higher in men, with a sex ratio of 2:1, and the majority of patients are symptomatic complaining of abdominal pain, altered bowel habit and weight loss. They can be solitary or multiple on DCBE and may mimic other more common colonic tumours. Several studies note an increased incidence of colonic malignant lymphoma in patients with ulcerative colitis [1,3,4]. MLP is a primary malignant lymphoma arising in the gastrointestinal tract. It is a non-Hodgkin's lymphoma recently classified as an extranodal mantle cell lymphoma (REAL) [5] and it corresponds to an extranodal form of a pure centrocytic lymphoma (Kiel classification) [6-8]. Correspondence to: Dr S. H. Lee, Department of Radiology, Manchester Royal Infirmary, Oxford Road, Manchester MI3 9WL, UK. © 1997 The Royal College of Radiologists, ClinicalRadiology, 52, 794-802.
Fig. 2 - Caecum and right colon showing a large polypoid filling defect in the caecum.
Mantle cell lymphoma of the gastrointestinal tract differs histologically from other lymphomas of mucosal associated lymphoid tissue (MALToma) which show little tendency to dissemination beyond the mucosa and remain localized. Therefore, MLP, as in this case, carries a worse prognosis.
798
CLINICAL RADIOLOGY
Fig. 4 - Histological examination of the colon, revealing diffuse infiltrate of mantle cells in the lamina propria. Fig. 3 - Ultrasound of the caecum and terminal ileum showing polypoid tumour within the caecum, (white arrow) and solid tumour involving the terminal ileum (black arrow).
REFERENCES
Characteristically, MLP occurs at multiple sites in the colon and in a proportion of patients there may also be multiple lesions in the small bowel or stomach [10]. The sub-mucosal infiltrate generally projects into the bowel lumen and forms a sessile or pedunculated polyp [9]. MLP has a typical appearance .on DCBE of either a single or multiple submucosal polyps, with a wide sessile base, and often a central depression [10]. Other MALTomas tend to be localized and do not normally form polypoid masses. The appearances of MLP on DCBE may be confused with pneumatosis coli which typically shows the presence of submucosal gas on DCBE not seen in MLP or other polypoid tumours. Indeed one unusual case report described both diffuse colonic lymphoma and pneumatosis coli occurring together [11]. MLP is an aggressive mantle cell lymphoma. Its prognosis is worst among the low-grade B-cell lymphomas, due to widespread involvement of the gastrointestinal tract, and tendency for extra intestinal spread lead to a high stage at diagnosis. However, although no extraintestinal spread was demonstrated in this case, surgery was not performed due to the poor general condition of the patient. Surgical resection is often not feasible in MLP contrasting with observations in other MALTomas and management mainly involves the use of chemotherapy but the prognosis remains poor [8].
1 Dodd GD. Lymphoma of the hollow abdominal viscera. Radiologic Clinics of North America 1990;28:771-783. 2 Craig O, Gregson R. Primary lymphomas of the gastrointestinal tract. Clinical Radiology 1981 ;32:63-71. 3 Sheperd NA, Hall PA, Williams GT et al. Primary lymphoma of the large intestine complicating inflammatory bowel disease. Histopathology 1989;16:95-97. 4 Hill DH, Manton Mills JO, Maxwell RJ. Synchronous colonic lymphomas complicating chronic ulcerative colitis. Abdominal Imaging 1993;18:369-370. 5 Chan JKC. A new classification of lymphomas: the revised EuropeanAmerican lymphoma classification. Advances in Anatomical Pathology 1994;1:166-172. 6 Isaacson PG, Norton AJ. Mantle cell lymphoma (lymphomatous polyposis). In: Extranodal Lymphomas, 1st ed. Edinburgh: Churchill Livingstone, 1994:50-52. 7 Stansfeld AG, Diebold J, Noel H e t al. Updated Kiel classification for lymphomas. Lancet 1988;1:292-293. 8 Banks PM, Chan J, Cleary ML et al. Mantle cell lymphoma. A proposal for unification of morphologic, immunologic, and molecular data. American Journal of Surgical Pathology 1992;16:637-640. 9 Sheperd NA, Hall PA, Coates PJ, Levison DA. Primary lymphoma of the colon and rectum. A histological and immunohistochemical analysis of 45 cases with cfinicopathological correlations. Histopathology 1988; 12:235-252. 10 Kawamoto K, Motooka M, Hirata N e t al. Colonic submucosal tumors: a new classification based on radiologic characteristics. American Journal of Roentgenology 1993;160:315-320. 11 O'Connell DJ, ThompsonAJ, Lymphoma of the colon: the spectrumof radiologic changes. Gastrointestinal Radiology 1978;2:377-385.
Clinical Radiology (1997) 52, 798-800
Case Report: Cardiac Arrest Due to Coronary Artery Spasm During Angiographic Procedure I. SAKAMOTO, T. UCHIDA and K. HAYASHI
Department of Radiology, Nagasaki University School of Medicine, Nagasaki, Japan It is well known that coronary artery spasm (CAS) can be induced by exercise, cold pressor test, Valsalva manoeuvre, Correspondence to: Dr I. Sakamoto, Department of Radiology, Nagasaki University School of Medicine, 1-7-1 Sakamoto, Nagasaki 852, Japan.
and the administration of pharmacological agents such as acetylcholine [1]. However, CAS following the use of contrast material is extremely rare and all previously reported cases developed after direct intracoronary injection of contrast agents [2,3]. We describe a patient who © 1997 The Royal College of Radiologists, ClinicalRadiology, 52, 794-802.