Br. J. Surg. Vol. 63 (1976) 542-545
Colonic lymphoma complicating ulcerative colitis P. RENTON A N D A. J. BLACKSHAW* SUMMARY
Colonic lymphoma is a rare complication of ulcerative colitis. Two cases are described in patients who had had ulcerative colitis for I 2 and 22 years respectively. Both patients presented with a recent change in their symptoms, which had become increasingly severe and which had not remitted with customary treatment for ulcerative colitis. Physical and haematological examinations revealed no evidence of generalized lymphoma, though barium enema studies indicated the sites of’ the lymphomatous lesions superimposed on chronic ulcerative colitis which were confirmed at operation and biopsy. Case reports Case 1 : A 52-year-old Portuguese woman was admitted to St Mark’s Hospital, London, on 12 June 1974 with a 12-year history of diarrhoea. This had increased in severity in the 6 months before admission, and had recently been accompanied by rectal bleeding for which blood transfusion was necessary. Pain was not a feature of her symptoms. She had lost 18 kg in weight in 6 months and had been treated with steroid enemas and Salazopyrin, having been diagnosed as a case of ulcerative colitis. Physical examination was unremarkable, but on sigmoidoscopy the rectal mucosa was irregular. Pseudo-polyps were seen in the lower rectum together with an atypical large isolated ulcer on the posterior rectal wall. Investigations on admission were as follows: haemoglobin 13.0 g, WBC 10 500/mm3 and ESR 62 mm/h. Urine analysis revealed no abnormality. Plasma electrolytes were within normal limits. Total plasma proteins were 6.4 g per cent and albumin was 3.0 g per cent. Plasma electrophoresis showed a non-specific slight increase in alpha-2-globulin. Measurement of immunoglobulin levels gave results within normal limits. An ‘instant’ (unprepared) barium enema examination showed the large bowel devoid of faeces other than in the caecum. The rectum was narrow and rigid. The post-rectal space was 3 cm. There was angulation at the upper rectum above which the bowel was slightly distensible. Irregular ulceration, as well as a polyp, was seen o n the posterior wall. There was loss of the normal haustral pattern throughout the colon and fine ulceration in the region of the hepatic flexure (Fig. 1). A chest X-ray was normal. In view of the length of history, recent change in symptoms, radiological evidence of total colitis and atypical rectal ulceration seen at sigmoidoscopy and o n barium enema, a proctocolectomy was performed (Mr H. E. Lockhart-Mummery and Mr P. R. Hawley). The operative specimen showed shortening and narrowing of the colon from the caecum to the splenic flexure with muscular thickening of the bowel wall and atrophic mucosa. In the region of the splenic flexure there was an extensive area of ulceration involving the entire circumference of the bowel wall for 15 cni. The edges were well demarcated, elevated and hard, and the cut surface showed white turnour infiltrating through the wall to the serosal aspect. The lower 10 cm of the rectum showed similar appearances (Fig. 2). Histology (Dr B. C. Morson) of the two ulcerated areas showed the appearances of malignant lymphoma. Tumour was invading the mesenteric and pararectal fatty tissue. Sixty-two mesocolic and pararectal lymph nodes were examined and none showed involvement by malignant lymphoma. The remaining colon showed the histological appearances o f
542
chronic ulcerative colitis in remission, with mucosal atrophy throughout the whole length of the specimen. Case 2: A 49-year-old English woman had a history of proctocolitis of 22 years’ duration, with attacks of diarrhoea and bloody mucous discharge, usually in the summer and of 1-2 months’ duration. A barium enema study performed elsewhere 4 years before the admission to St Mark’s Hospital was reported to have shown total chronic ulcerative colitis. In the 6 months before admission her symptoms had altered to bloody diarrhoea and urgency associated with lower abdominal and rectal pain and three episodes of pyrexia. The customary autumnal remission of her colitis did not occur. On admission t o St Mark’s Hospital physical examination was unremarkable. There were n o abdominal masses or peripheral lymphadenopathy. Sigmoidoscopy was uninformative because of fluid faeces. Investigations were as follows: haemoglobin 86 per cent, WBC 7000/mms and ESR 20 mmih. Analysis of urine revealed no abnormality. Plasma electrolytes were within normal limits, as was the alkaline phosphatase level. A barium enema study showed a 4-cm polypoid mass with an irregular base in the rectum, the appearances being compatible with a rectal carcinoma. The rectum was narrow and the post-rectal space was wide at 2.5 cm. The distal descending colon and sigmoid colon were tubular, the appearances being compatible with ulcerative colitis. A chest X-ray showed healed tuberculous disease only. Excision of the sigrnoid colon, rectum and anus was performed (Mr I. P. Todd). The specimen showed an ulcerating tumour of 7 x 8 cm with elevated margins in the rectum. The remaining mucosa of the colon and rectum was atrophic Fig. 3 ) . Histology (Dr B. C. Morson) of the tumour showed a malignant lymphoma which had penetrated through to the pararectal tissues. Four of 25 regional nodes examined contained malignant lymphoma. The remaining colon and rectum showed chronic ulcerative colitis in remission with mucosal atrophy.
Discussion Colonic malignancy is a well-recognized complication of ulcerative colitis, though controversy exists as to the magnitude of the danger. According to Bargen et al. (1954) the death rate from carcinoma of the colon in patients with ulcerative colitis is thirty times that in the general population. Edwards and Truelove (1964) gave an overall incidence of 3.5 per cent for carcinoma in ulcerative colitis, with a cumulative risk of 12.6 per cent at 20 years’ duration and an average age at development of carcinoma of 41 years, while de Dombal et al. (1966) gave an incidence of 1.7 per cent in 465 cases, with a cumulative incidence of 26 per cent after 25 years. Hinton (1966) has shown that the major risk is in total colitis. In contrast, primary malignant lymphoma of the colon is a rare development. Three cases of malignant lymphoma developing in ulcerative colitis have been seen at St Mark’s in a 16-year period (the present 2 cases plus a case
* Departments of Radiology and Pathology, St Mark’s Hospital, London.
Colonic lymphoma in ulcerative colitis
Fig. 1. Cuse 1. ‘Instant’ barium enema film showing total chronic ulcerative colitis with rectal narrowing and irregularity due to a lymphoma.
Fig. 3. Cuse 2. Operative specimen showing a rectal tumour with atrophic mucosa elsewhere.
Fig. 2. Case 1 . Operative specimen showing localized tumours at the rectum and splenic flexure with total chronic ulcerative colitis.
previously reported by Cornes et al., 1961). During this time 33 adenocarcinomas complicating ulcerative colitis have been seen. Fewer than 20 cases of malignant lymphoma in ulcerative colitis have been described in the literature. A review of the more recent of these cases reveals an average age at the discovery of lymphoma of 48 years, and an average length of colitic history of 12 years. The male to female sex ratio is 7 : 3. The present 2 cases are, therefore, in the same age group and show at least an equivalent length of colitic history. From our cases and those in the literature it is clear that alteration in a patient’s symptomatology should alert the clinician to the possibility of malignant change in ulcerative colitis. There is often an increase in the severity and duration of attacks of diarrhoea, which may not respond to the customary treatment. Blood may be passed either for the first time or in greater amounts. Many of these patients are febrile, and colicky or cramp-like lower abdominal pains are a commonly noted feature. An abdominal mass may be noted. These alterations in symptomatology, which are often severe and intractable, a r e usually-of short duration before the patient presents. The average duration of altered symptoms of 9 cases in the literature was 10 weeks, and in both our cases 6 months. 543
P. Renton and A. J. Blackshaw
Fig. 4. Case 2. Microscopic detail of the lymphoma. H E . ( x 185.)
Fig. 5. Case 2. Microscopic detail of the lymphoma showing malignant cells within blood vessels. HE. (330.)
Radiologically, most of the reported cases show evidence of chronic total ulcerative colitis with an anhaustral, tubular, featureless and shortened colon. The superimposed lymphoid tumour is usually unicentric. However, one of our cases had two separate lymphoid tumours, as did the cases of Cornes et al. (1961) and Sataline et al. (1963). Two further cases (Warren, 1959; Nugent et al., 1972) also had two separate malignancies, each having a lymphoma accompanied by a carcinoma. Some cases may show a diffuse involvement of the whole or part of the colon by lymphoma which apparently simulates ulcerative colitis and makes a diagnosis of supervening malignancy difficult, if not impossible (Friedman et al., 1968). Federman et al. (1963) reported a case which they believed to be of malignant lymphoma of over 15 years’ duration masquerading as ulcerative colitis. Where localized lymphomas are found, they seem to arise mainly distal to the splenic flexure, usually in the rectum or sigmoid colon. They may be annular or polypoid, but a review of the cases reported previously does not reveal any relationship of microscopiccellular type to macroscopic appearance. The terminology employed in the reports of these lymphomas is varied and probably reflects differences in usage between pathologists and the lack of a universally accepted classification of lymphoid tumours in general. However, it appears that most cases are of a poorly
differentiated type with presumably a correspondingly poor prognosis. The microscopic appearances of the tumours in the present cases were very similar. In both, the lymphoma was of diffuse type without any evidence of a follicular pattern. Individual tumour cells were large with a moderate amount of amphophilic cytoplasm and large nuclei many of which had a folded or indented outline. Nuclear chromatin tended to be fine, but in many cells there was peripheral condensation into coarser clumps at the nuclear membrane. Nucleoli were mostly single and often eosinophilic. In many instances they were thrown into prominence by a relative lack of surrounding nuclear chromatin, producing a clear halo. The tumour cells thus displayed many of the morphological characteristics of the primitive lymphoid precursor cells which are presently known as immunoblasts. Occasional banal inflammatory cells, including eosinophils and lymphocytes, were scattered throughout the tumour but no Sternberg-Reed type of cells were seen. Figs. 4 and 5 show the microscopic appearance of the tumour from Case 2. Cellular details are best seen where tumour has invaded small blood vessels. Microscopic examination of colonic mucosa uninvolved by tumour showed the characteristic atrophy and crypt distortion of ulcerative colitis in a quiescent phase as stated above. This not only confirmed the
544
Colonic lymphoma in ulcerative colitis clinical and radiological diagnoses but also excluded mimicry of ulcerative colitis by colonic lymphoma per se. Follow-up information is not included in most reports, but the case of Walker and Weaver (1964) was alive and well 17 months after operation. Case 1 of Cornes et al. (1961), who was a patient at St Mark’s Hospital, subsequently died 21 months after operation. The cause of death was given as ‘carcinoma of the rectum’ but no autopsy was performed. However, she was known to have had recurrent intra-abdominal disease 4 months after operation, for which she was treated with radiotherapy (Middlesex Hospital). The second case reported by Cornes et al. (1961) also had recurrent intra-abdominal growth and died 14 months after operation. Again no autopsy was performed. The present Case 1 returned to Portugal after being discharged and has been lost to follow-up. Case 2 is known to have died 41 months after operation. She was treated with radiotherapy and cytotoxic combination chemotherapy (St Bartholomew’s Hospital) for disseminated malignant lymphoma which became apparent 1 month after operation, with the development of cervical and later axillary lymphadenopathy. The cause of her eventual death was given as ‘mediastinal obstruction due to reticulum cell sarcoma’. Autopsy was not performed. We believe that the lymphomas in our cases arose primarily in the colon. Dawson et al. (I 961) laid down five criteria to distinguish primary colonic lymphoma from colonic involvement evolving in generalized lymphoma : 1. There must be no superficial enlarged lymph nodes when the patient is first seen. 2. The chest X-ray must show no enlargement of mediastinal nodes. 3. The total and differential white cell count must be normal. 4. At laparotomy only regional nodes should be affected by disease. 5 . The liver and spleen should be unaffected. Our 2 cases meet these criteria. In neither of the cases was a specific diagnosis of malignant lymphoma made before histological examination. If a tumour complicating ulcerative colitis is suspected because of the history and radiological appearances, then obviously a carcinoma is more likely. The diagnosis of malignant lymphoma complicating ulcerative colitis is
52
generally made from the postoperative specimen or at post-mortem (Walker and Weaver, 1964).
Acknowledgements We would like to thank Mr H. E. Lockhart-Mummery and Mr I. P. Todd, Consultant Surgeons at St Mark’s Hospital, for permission to report their patients, Mr N. Mackie for the preparation of the illustrations and Miss C. Liggins for secretarial assistance. References et al. (1954) The development of cancer in ulcerative colitis. Gastroenterology 26, 32-37. CORNES J. s., SMITH J . c. and SOUTHWOOD w . F. w . (1 961) Lymphosarcoma in chronic ulcerative colitis. Br. J . Surg. 49, 50-53. DAWSON I. M. P., CORNES J. s. and MORSON B. c . (1961) Primary malignant lymphoid tumours of the intestinal tract. Br. J . Surg. 49, 80-89. DE DOMBAL F. T., WATTS J. M., WATKINSON G. et al. (1966) Local complications of ulcerative colitis, stricture pseudopolyposis and carcinoma of the colon and rectum. Br. Med. J. 1, 1442-1447. EDWARDS F. c. and TRUELOVE s. G. (1964) The course and prognosis of ulcerative colitis. Gut 5, 1-22. FEDERMAN J . , GOLDSTEIN M. and WEINGARTEN B. (1 963) Malignant lymphoma of over fifteen years’ duration masquerading as ulcerative colitis. Am. J. Roentgenol. Radium Ther. Nucl. Med. 89, 771178. FRIEDMAN H . B., SILVER G . M. and BROWN c . H. (1968) Lymphoma of the colon simulating ulcerative colitis. Am. J . Dig. Dis. 13, 910-917. HINTON J . M. (1966) Carcinoma in ulcerative colitis Proc. R. SOC.Med. 59, 632-633. NUGENT F. w . , ZUBERI s., BULAN M. B. et al. (1972) Colonic lymphoma in ulcerative colitis. Lahey Clin. Foundn Bull. 21, 104-111. SATALINE L. R., MORLEY E. M. and KIRKHAM w . (1963) Ulcerative colitis complicated by colonic lymphoma. Gastroenterology 44, 342-347. WALKER F. c. and WEAVER J. P. A . (1964) Lymphosarcoma in ulcerative colitis Br. J . Surg. 51, 475471. WARREN K . (1959) Malignant lymphoma of the duodenum, small intestine and colon. Surg. Clin. North Am. 39, 725-135. BARGEN J . A . , SAUER w . G., SLOAN w . P.
545
Colonic lymphoma complicating ulcerative colitis P. RENTON A N D A. J. BLACKSHAW* SUMMARY
Colonic lymphoma is a rare complication of ulcerative colitis. Two cases are described in patients who had had ulcerative colitis for I 2 and 22 years respectively. Both patients presented with a recent change in their symptoms, which had become increasingly severe and which had not remitted with customary treatment for ulcerative colitis. Physical and haematological examinations revealed no evidence of generalized lymphoma, though barium enema studies indicated the sites of’ the lymphomatous lesions superimposed on chronic ulcerative colitis which were confirmed at operation and biopsy. Case reports Case 1 : A 52-year-old Portuguese woman was admitted to St Mark’s Hospital, London, on 12 June 1974 with a 12-year history of diarrhoea. This had increased in severity in the 6 months before admission, and had recently been accompanied by rectal bleeding for which blood transfusion was necessary. Pain was not a feature of her symptoms. She had lost 18 kg in weight in 6 months and had been treated with steroid enemas and Salazopyrin, having been diagnosed as a case of ulcerative colitis. Physical examination was unremarkable, but on sigmoidoscopy the rectal mucosa was irregular. Pseudo-polyps were seen in the lower rectum together with an atypical large isolated ulcer on the posterior rectal wall. Investigations on admission were as follows: haemoglobin 13.0 g, WBC 10 500/mm3 and ESR 62 mm/h. Urine analysis revealed no abnormality. Plasma electrolytes were within normal limits. Total plasma proteins were 6.4 g per cent and albumin was 3.0 g per cent. Plasma electrophoresis showed a non-specific slight increase in alpha-2-globulin. Measurement of immunoglobulin levels gave results within normal limits. An ‘instant’ (unprepared) barium enema examination showed the large bowel devoid of faeces other than in the caecum. The rectum was narrow and rigid. The post-rectal space was 3 cm. There was angulation at the upper rectum above which the bowel was slightly distensible. Irregular ulceration, as well as a polyp, was seen o n the posterior wall. There was loss of the normal haustral pattern throughout the colon and fine ulceration in the region of the hepatic flexure (Fig. 1). A chest X-ray was normal. In view of the length of history, recent change in symptoms, radiological evidence of total colitis and atypical rectal ulceration seen at sigmoidoscopy and o n barium enema, a proctocolectomy was performed (Mr H. E. Lockhart-Mummery and Mr P. R. Hawley). The operative specimen showed shortening and narrowing of the colon from the caecum to the splenic flexure with muscular thickening of the bowel wall and atrophic mucosa. In the region of the splenic flexure there was an extensive area of ulceration involving the entire circumference of the bowel wall for 15 cni. The edges were well demarcated, elevated and hard, and the cut surface showed white turnour infiltrating through the wall to the serosal aspect. The lower 10 cm of the rectum showed similar appearances (Fig. 2). Histology (Dr B. C. Morson) of the two ulcerated areas showed the appearances of malignant lymphoma. Tumour was invading the mesenteric and pararectal fatty tissue. Sixty-two mesocolic and pararectal lymph nodes were examined and none showed involvement by malignant lymphoma. The remaining colon showed the histological appearances o f
542
chronic ulcerative colitis in remission, with mucosal atrophy throughout the whole length of the specimen. Case 2: A 49-year-old English woman had a history of proctocolitis of 22 years’ duration, with attacks of diarrhoea and bloody mucous discharge, usually in the summer and of 1-2 months’ duration. A barium enema study performed elsewhere 4 years before the admission to St Mark’s Hospital was reported to have shown total chronic ulcerative colitis. In the 6 months before admission her symptoms had altered to bloody diarrhoea and urgency associated with lower abdominal and rectal pain and three episodes of pyrexia. The customary autumnal remission of her colitis did not occur. On admission t o St Mark’s Hospital physical examination was unremarkable. There were n o abdominal masses or peripheral lymphadenopathy. Sigmoidoscopy was uninformative because of fluid faeces. Investigations were as follows: haemoglobin 86 per cent, WBC 7000/mms and ESR 20 mmih. Analysis of urine revealed no abnormality. Plasma electrolytes were within normal limits, as was the alkaline phosphatase level. A barium enema study showed a 4-cm polypoid mass with an irregular base in the rectum, the appearances being compatible with a rectal carcinoma. The rectum was narrow and the post-rectal space was wide at 2.5 cm. The distal descending colon and sigmoid colon were tubular, the appearances being compatible with ulcerative colitis. A chest X-ray showed healed tuberculous disease only. Excision of the sigrnoid colon, rectum and anus was performed (Mr I. P. Todd). The specimen showed an ulcerating tumour of 7 x 8 cm with elevated margins in the rectum. The remaining mucosa of the colon and rectum was atrophic Fig. 3 ) . Histology (Dr B. C. Morson) of the tumour showed a malignant lymphoma which had penetrated through to the pararectal tissues. Four of 25 regional nodes examined contained malignant lymphoma. The remaining colon and rectum showed chronic ulcerative colitis in remission with mucosal atrophy.
Discussion Colonic malignancy is a well-recognized complication of ulcerative colitis, though controversy exists as to the magnitude of the danger. According to Bargen et al. (1954) the death rate from carcinoma of the colon in patients with ulcerative colitis is thirty times that in the general population. Edwards and Truelove (1964) gave an overall incidence of 3.5 per cent for carcinoma in ulcerative colitis, with a cumulative risk of 12.6 per cent at 20 years’ duration and an average age at development of carcinoma of 41 years, while de Dombal et al. (1966) gave an incidence of 1.7 per cent in 465 cases, with a cumulative incidence of 26 per cent after 25 years. Hinton (1966) has shown that the major risk is in total colitis. In contrast, primary malignant lymphoma of the colon is a rare development. Three cases of malignant lymphoma developing in ulcerative colitis have been seen at St Mark’s in a 16-year period (the present 2 cases plus a case
* Departments of Radiology and Pathology, St Mark’s Hospital, London.
Colonic lymphoma in ulcerative colitis
Fig. 1. Cuse 1. ‘Instant’ barium enema film showing total chronic ulcerative colitis with rectal narrowing and irregularity due to a lymphoma.
Fig. 3. Cuse 2. Operative specimen showing a rectal tumour with atrophic mucosa elsewhere.
Fig. 2. Case 1 . Operative specimen showing localized tumours at the rectum and splenic flexure with total chronic ulcerative colitis.
previously reported by Cornes et al., 1961). During this time 33 adenocarcinomas complicating ulcerative colitis have been seen. Fewer than 20 cases of malignant lymphoma in ulcerative colitis have been described in the literature. A review of the more recent of these cases reveals an average age at the discovery of lymphoma of 48 years, and an average length of colitic history of 12 years. The male to female sex ratio is 7 : 3. The present 2 cases are, therefore, in the same age group and show at least an equivalent length of colitic history. From our cases and those in the literature it is clear that alteration in a patient’s symptomatology should alert the clinician to the possibility of malignant change in ulcerative colitis. There is often an increase in the severity and duration of attacks of diarrhoea, which may not respond to the customary treatment. Blood may be passed either for the first time or in greater amounts. Many of these patients are febrile, and colicky or cramp-like lower abdominal pains are a commonly noted feature. An abdominal mass may be noted. These alterations in symptomatology, which are often severe and intractable, a r e usually-of short duration before the patient presents. The average duration of altered symptoms of 9 cases in the literature was 10 weeks, and in both our cases 6 months. 543
P. Renton and A. J. Blackshaw
Fig. 4. Case 2. Microscopic detail of the lymphoma. H E . ( x 185.)
Fig. 5. Case 2. Microscopic detail of the lymphoma showing malignant cells within blood vessels. HE. (330.)
Radiologically, most of the reported cases show evidence of chronic total ulcerative colitis with an anhaustral, tubular, featureless and shortened colon. The superimposed lymphoid tumour is usually unicentric. However, one of our cases had two separate lymphoid tumours, as did the cases of Cornes et al. (1961) and Sataline et al. (1963). Two further cases (Warren, 1959; Nugent et al., 1972) also had two separate malignancies, each having a lymphoma accompanied by a carcinoma. Some cases may show a diffuse involvement of the whole or part of the colon by lymphoma which apparently simulates ulcerative colitis and makes a diagnosis of supervening malignancy difficult, if not impossible (Friedman et al., 1968). Federman et al. (1963) reported a case which they believed to be of malignant lymphoma of over 15 years’ duration masquerading as ulcerative colitis. Where localized lymphomas are found, they seem to arise mainly distal to the splenic flexure, usually in the rectum or sigmoid colon. They may be annular or polypoid, but a review of the cases reported previously does not reveal any relationship of microscopiccellular type to macroscopic appearance. The terminology employed in the reports of these lymphomas is varied and probably reflects differences in usage between pathologists and the lack of a universally accepted classification of lymphoid tumours in general. However, it appears that most cases are of a poorly
differentiated type with presumably a correspondingly poor prognosis. The microscopic appearances of the tumours in the present cases were very similar. In both, the lymphoma was of diffuse type without any evidence of a follicular pattern. Individual tumour cells were large with a moderate amount of amphophilic cytoplasm and large nuclei many of which had a folded or indented outline. Nuclear chromatin tended to be fine, but in many cells there was peripheral condensation into coarser clumps at the nuclear membrane. Nucleoli were mostly single and often eosinophilic. In many instances they were thrown into prominence by a relative lack of surrounding nuclear chromatin, producing a clear halo. The tumour cells thus displayed many of the morphological characteristics of the primitive lymphoid precursor cells which are presently known as immunoblasts. Occasional banal inflammatory cells, including eosinophils and lymphocytes, were scattered throughout the tumour but no Sternberg-Reed type of cells were seen. Figs. 4 and 5 show the microscopic appearance of the tumour from Case 2. Cellular details are best seen where tumour has invaded small blood vessels. Microscopic examination of colonic mucosa uninvolved by tumour showed the characteristic atrophy and crypt distortion of ulcerative colitis in a quiescent phase as stated above. This not only confirmed the
544
Colonic lymphoma in ulcerative colitis clinical and radiological diagnoses but also excluded mimicry of ulcerative colitis by colonic lymphoma per se. Follow-up information is not included in most reports, but the case of Walker and Weaver (1964) was alive and well 17 months after operation. Case 1 of Cornes et al. (1961), who was a patient at St Mark’s Hospital, subsequently died 21 months after operation. The cause of death was given as ‘carcinoma of the rectum’ but no autopsy was performed. However, she was known to have had recurrent intra-abdominal disease 4 months after operation, for which she was treated with radiotherapy (Middlesex Hospital). The second case reported by Cornes et al. (1961) also had recurrent intra-abdominal growth and died 14 months after operation. Again no autopsy was performed. The present Case 1 returned to Portugal after being discharged and has been lost to follow-up. Case 2 is known to have died 41 months after operation. She was treated with radiotherapy and cytotoxic combination chemotherapy (St Bartholomew’s Hospital) for disseminated malignant lymphoma which became apparent 1 month after operation, with the development of cervical and later axillary lymphadenopathy. The cause of her eventual death was given as ‘mediastinal obstruction due to reticulum cell sarcoma’. Autopsy was not performed. We believe that the lymphomas in our cases arose primarily in the colon. Dawson et al. (I 961) laid down five criteria to distinguish primary colonic lymphoma from colonic involvement evolving in generalized lymphoma : 1. There must be no superficial enlarged lymph nodes when the patient is first seen. 2. The chest X-ray must show no enlargement of mediastinal nodes. 3. The total and differential white cell count must be normal. 4. At laparotomy only regional nodes should be affected by disease. 5 . The liver and spleen should be unaffected. Our 2 cases meet these criteria. In neither of the cases was a specific diagnosis of malignant lymphoma made before histological examination. If a tumour complicating ulcerative colitis is suspected because of the history and radiological appearances, then obviously a carcinoma is more likely. The diagnosis of malignant lymphoma complicating ulcerative colitis is
52
generally made from the postoperative specimen or at post-mortem (Walker and Weaver, 1964).
Acknowledgements We would like to thank Mr H. E. Lockhart-Mummery and Mr I. P. Todd, Consultant Surgeons at St Mark’s Hospital, for permission to report their patients, Mr N. Mackie for the preparation of the illustrations and Miss C. Liggins for secretarial assistance. References et al. (1954) The development of cancer in ulcerative colitis. Gastroenterology 26, 32-37. CORNES J. s., SMITH J . c. and SOUTHWOOD w . F. w . (1 961) Lymphosarcoma in chronic ulcerative colitis. Br. J . Surg. 49, 50-53. DAWSON I. M. P., CORNES J. s. and MORSON B. c . (1961) Primary malignant lymphoid tumours of the intestinal tract. Br. J . Surg. 49, 80-89. DE DOMBAL F. T., WATTS J. M., WATKINSON G. et al. (1966) Local complications of ulcerative colitis, stricture pseudopolyposis and carcinoma of the colon and rectum. Br. Med. J. 1, 1442-1447. EDWARDS F. c. and TRUELOVE s. G. (1964) The course and prognosis of ulcerative colitis. Gut 5, 1-22. FEDERMAN J . , GOLDSTEIN M. and WEINGARTEN B. (1 963) Malignant lymphoma of over fifteen years’ duration masquerading as ulcerative colitis. Am. J. Roentgenol. Radium Ther. Nucl. Med. 89, 771178. FRIEDMAN H . B., SILVER G . M. and BROWN c . H. (1968) Lymphoma of the colon simulating ulcerative colitis. Am. J . Dig. Dis. 13, 910-917. HINTON J . M. (1966) Carcinoma in ulcerative colitis Proc. R. SOC.Med. 59, 632-633. NUGENT F. w . , ZUBERI s., BULAN M. B. et al. (1972) Colonic lymphoma in ulcerative colitis. Lahey Clin. Foundn Bull. 21, 104-111. SATALINE L. R., MORLEY E. M. and KIRKHAM w . (1963) Ulcerative colitis complicated by colonic lymphoma. Gastroenterology 44, 342-347. WALKER F. c. and WEAVER J. P. A . (1964) Lymphosarcoma in ulcerative colitis Br. J . Surg. 51, 475471. WARREN K . (1959) Malignant lymphoma of the duodenum, small intestine and colon. Surg. Clin. North Am. 39, 725-135. BARGEN J . A . , SAUER w . G., SLOAN w . P.
545
