Non-Hodgkin’s

Lymphoma Presenting By

Ray C. Pais, Amir Hammami,

as a Rectal Polyp in a Child

Hahk Kim, and Abdel H. Ragab

A t/an ta, Georgia 0 Primary lymphoma of the rectum is extremely rare in children. We report here a lo-year-old boy with localized non-Hodgkin’s lymphoma, discovered within a rectal polyp. The literature on childhood rectal lymphomas is reviewed. This case illustrates the importance of considering the possibility of malignancy with rectal polyps, even in children. @ 1990 by W. B. Saunders Company. INDEX WORDS:

Lymphoma,

rectal.

DISCUSSION

I

NVOLVEMENT of the rectum by malignant iymphoma, either primary or secondary, is uncommon in adults.“4 Rectal lymphoma in children is even more rare. We were able to identify only four cases from the English language literature of primary malignant lymphoma of the rectum in children.5-8 We report here a child with a rectal polyp found to be a localized non-Hodgkin’s lymphoma. CASE

REPORT

A lo-year-old

boy presented with a 2- to 3-week history of hematochezia. He had no history of night sweats, constipation, diarrhea, or abdominal pain. Other than a transient rash and fever 5 days prior to admission, he had no additional symptoms. Past medical history was unremarkable. Physical examination showed a well-nourished, cheerful, and cooperative boy. The general examination was normal, with no adenopathy, hepatosplenomegaly, or abdominal mass. On rectal examination, a mass was palpable. Sigmoidoscopy demonstrated a single 1.5~cm sessile polyp on the anterior rectal wall. The polyp did not resemble a common juvenile polyp, and was completely removed with wide margins. The final histological diagnosis was malignant lymphoma, small noncleaved cell, by the Working Formulation classification. (Rappaport classification: diffuse, undifferentiated non-Burkitt’s lymphoma.) Figure I shows the histological details of the polyp. A complete blood count was normal. Blood chemistries, including uric acid and liver enzymes, were completely normal. A metastatic

From the Division of Pediatric HematologylOncology and the Department of Pathology, Emory University School of Medicine, and the Department of Surgery, Georgia Baptist Medical Center, Atlanta, GA. Dr Pais is the recipient of Ameriean Cancer Society Clinical Oncology Fellowship No. 88-39. Dr Ragab is the recipient of a Faculty Research Award from the American Cancer Society. Supported in part by the Cure Foundation of Georgia and The Georgia Department of Human Resources, Atlanta, GA. Address reprint requests to Ray C. Pats, MD. Emory University School of Medicine. Division of Pediatric Hematology/Oncology, 2040 Ridgewood Dr NE. Atlanta, GA 30322. 0 1990 by W.B. Saunders Company. 0022-3468/X3/2512-0028$03.00/O

1280

workup was negative, including a barium enema, bone scan, chest x-ray, computed axial tomography (chest, abdomen, and pelvis), spinal tap, and bone marrow biopsy. The patient was subsequently treated with a ‘I-week course of chemotherapy (cyclophosphamide, doxorubicin, prednisone, and vincristine) as per a Pediatric Oncology Group protocol for localized non-Hodgkin’s lymphoma. He is currently free of disease and doing well, 7 months after diagnosis and 5 months after completing chemotherapy.

Rectal malignancies are extremely rare in children. Although occasional cases of rectal carcinoma are reported in children,5*g*‘0 primary lymphoma of the rectum is even rarer. We could identify only four clear-cut cases of primary rectal lymphoma in children from the English language literature.5-8 These four cases, as well as this case, are summarized in Table 1. Berry and Keeling include a 7%year-old boy with rectal involvement in their series of children with gastrointestinal lymphosarcoma (poorly differentiated, lymphocytic Iymphoma), but it is unclear whether or not the rectum was the primary site because that patient also had involvement of the ileum and omenturn.” Glick and Soule briefly mention a 15yearold girl with ulcerative colitis who developed a primary colon reticulum cell sarcoma (histiocytic lymphoma), which also involved the terminal ileum and rectum.12 Reports by Rankin and Chumley,‘3 Kennedy,14 and Naqvi et al” have occasionally been cited as examples of malignant rectal lymphoma in children; however, the original papers do not state where in the large intestine these lymphomas occurred, and therefore, one may doubt whether or not the children included in these series actually had rectal lymphomas. A series of children with malignant lymphoma reported by Lemerle et all6 included one case of localized lymphoma of the anus, but no case of rectal lymphoma. A useful definition of primary intestinal lymphoma was put forth by Dawson et al,17 which requires the following conditions at the time of diagnosis: the absence of lymphadenopathy, a normal chest x-ray, a normal complete blood count, predominance of the bowel lesion at laparotomy, and the absence of tumor in the liver and spleen. Lewin et al’* used a less restrictive definition of primary gastrointestinal lymphoma, requiring only that the presenting symptom be related to the gastrointestinal tract. By any definition, it is clear that primary gastrointestinal lymphoma is not rare in children. The American Burkitt’s Lymphoma Registry for 197 1 to 1979 lists 110 cases of

Journal of Pediatric Surgery, Vol 25, No 12 (December), 1990: pp 1280-1282

RECTAL LYMPHOMA

1281

sites within the gastrointestinal tract is quite different than in adults. In adults the stomach is the principal site in 44% to 74% of all primary gastrointestinal lymphomas,~,‘5.‘7,‘8,*3-2~ whereas virtually none of the pediatric cases have gastric involvement.1’~‘6~‘8~21~23~29’30 In children, nearly all cases of primary gastrointestinal lymphoma involve the distal ileum or ileocecal reIn children with so-called “Meditergion. 11~16~18,21-23,29V30 ranean Lymphoma,” also known as immunoproliferative small intestinal disease, diffuse involvement of the proximal small bowel is more likely.29 Primary rectal lymphoma in adults is infrequent, although certainly not as infrequent as in childhood. Many small series of adults with primary rectal lymphoma have been published.‘-4*‘5>‘7.3’ Rectal lymphoma represents from 0% to 9% of all adult cases of primary gastrointestinal lymphoma.4,‘5,23-25,2*In adults with primary rectal lymphoma, rectal bleeding is the most frequent presenting symptom, with other common complaints being rectal pain, tenesmus, diarrhea, constipation, and/or weight ~oss.‘*~~~~” Great strides have been made in the treatment of localized childhood non-Hodgkin’s lymphoma during the past decade. Nelson et al reported a long-term survival rate of only 41% for children with localized gastrointestinal non-Hodgkin’s lymphoma treated at the Sidney Farber Cancer Center from 1948 to 1974.32 More recently (1987), the Pediatric Oncology Group reported a 95% 2-year relapse-free survival rate for children with localized non-Hodgkin’s lymphoma.20 In the randomized prospective Pediatric Oncology Group Study, the children treated with chemotherapy alone did just as well as those children treated with radiation therapy and chemotherapy.*’ Our current treatment strategy for localized gastrointestinal non-Hodgkin’s Lymphoma is to attempt to reduce the side effects of treatment, while still maintaining the excellent success rate cited above. This case is unusual, in that nearly all rectal polyps in children are benign. Of 103 colorectal polyps in

Fig 1. Low-power photomicrograph showing residual rectal mucosa and a diffuse infiltrate by malignant lymphoma. Inset shows cytologic details of the lymphoma cells at higher magnification. (H&E, original magnification x40: insert, x 1.000.)

children with primary gastrointestinal Burkitt’s lymphoma.” In a recent Pediatric Oncology Group series of children with localized non-Hodgkin’s lymphoma, 26% of the children had a gastrointestinal primary site.*’ Other series indicate that primary gastrointestinal lymphoma represents 10% to 20% of pediatric lymphomas.2’*22 Although primary gastrointestinal lymphoma in childhood is not unusual, the distribution of primary

Table 1. Primary Malignant Lymphoma of the Rectum in Children Age Study

Bacon et aI6

IV)

3B/~~

Outcome

Presenting Symptoms SW

(Duration)

M

Constipation (1 mo), urine

Treatment

Histology

~Follow-Up)

Reticulum cell sarcoma*

Surgery, radiation

Died (19 mo)

Reticulum cell sarcoma*

Surgery, radiation

Disease-free (not stated)

retention Cutler et al7

2%

M

Hematochezia (3% mo), anorexia

Gupta’ Baliga et al’

4% 10

? M

Hematochezia (6 mo)

Lymphosarcomat

Refused

Lost to follow-up

Diarrhea (6 mol. anorexia/

Lymphoblastic lymphoma

Surgery

Disease-free (8 mo)

Diffuse undifferentiated non-

Surgery, chemotherapy

Disease-free (7 mo)

cachexia (1 mo) Pais et al

10

M

Hematochezia (2-3 wk)

Burkitt’s *Corresponds to “histiocytic lymphoma” by the Rappaport classification. tcorresponds to “poorly differentiated lymphocytic lymphoma” by the Rappaport classification.

1282

PAIS ET AL

children reported by Horrillero et al, 79 were of rectal origin. None were malignant, all being classified as juvenile ~01~~s.~~Mestre reported 76 cases of childhood intestinal polyps, with none being malignant.34 In Louw’s series of 194 children with colorectal polyps (76% rectal), he identified only two polyps containing carcinoma, five adenomas, and no malignant lymphomas, with most of the polyps being juvenile polyps. Five polyps did demonstrate benign lymphoid hyperplasia (four of these cases were from the same family).9

Even benign lymphoid rectal polyps are relatively uncommon in childhood, with only 44 cases identified in a recent review of the literature.35 The case reported here illustrates the soundness of the recommendation by Gryboski36 and by Mestre,34 that all rectal polyps in children be removed, rather than complacent “watchful waiting.” ACKNOWLEDGMENT We would like to thank Suzan Tibor for typing this manuscript.

REFERENCES 1. Devine RM, Beart RW, Wolff BG: Malignant lymphoma of the rectum. Dis Colon Rectum 29:821-824, 1986 2. Egeli RA, Quan SH: Lymphome malin primaire du gros intestin. Schweiz Med Wschr 110:1045-1047, 1980 3. Perry PM, Cross RM, Morson BC: Primary malignant lymphoma of the rectum (22 cases). Proc R Sot Med 6572, 1972 4. Vanden Heule B, Taylor CR, Terry R, et al: Presentation of malignant lymphoma in the rectum. Cancer 49:2602-2607, 1982 5. Bacon HE, Wolfe FD, Archambault RA: Rectal malignant tumor in childhood. Am J Dis Child 64:70-79, 1942 6. Cutler CD, Stark RB, Scott HW: Lymphosarcoma of the bowel in childhood. N Engl J Med 232:665-670,1945 7. Gupta SD: Primary lymphomas of the gastrointestinal tract. Indian J Surg 38:106-l 10, 1976 8. Baliga M, Shenoy MG, Warrier PKR, et al: Primary lymphoma of rectum. Indian Pediatr 18:835-837, 1981 9. Louw JH: Polyp&d lesions of the large bowel in children with particular reference to benign lymphoid polyposis. J Pediatr Surg 3:195-209, 1968 10. Satyanand, Rana BS: Primary carcinoma of the rectum in children and young adults. Indian J Cancer 6:38-43, 1969 11. Berry CL, Keeling JW: Gastrointestinal lymphoma in childhood. J Clin Pathol23:459-463,197O 12. Glick DD, Soule EH: Primary malignant lymphoma of colon or appendix: Report of 27 cases. Arch Surg 92:144-151, 1966 13. Rankin FW, Chumley CL: Lymphosarcoma of the colon and rectum. Minnesota Med 12:247-253, 1929 14. Kennedy RLJ: Polyps of the rectum and colon in infants and in children. Am J Dis Child 62:481-488, 1941 15. Naqvi MS, Burrows L, Kark AE: Lymphoma of the gastrointestinal tract: Prognostic guides based on 162 cases. Ann Surg 170:221-231, 1969 16. Lemerle M, Gerard-Marchant R, Sarrazin D, et al: Lymphosarcoma and reticulum cell sarcoma in children: A retrospective study of 172 cases. Cancer 32:1499-1507, 1973 17. Dawson IMP, Cornes JS, Morson BC: Primary malignant lymphoid tumors of the intestinal tract; report of 37 cases with a study of factors influencing prognosis. Br J Surg 49:80-89, 1961 18. Lewin KJ, Ranched M, Dorfman RF: Lymphomas of the gastrointestinal tract: A study of 117 cases presenting with gastrointestinal disease. Cancer 42:693-707, 1978 19. Levine PH, Kamaraju LS, Connelly RR, et al: The American Burkitt’s Lymphoma Registry: Eight year’s experience. Cancer 49:1016-1022, 1982

20. Link MP, Donaldson SS, Berard CW, et al: High cure rate with reduced therapy in localized non-Hodgkin’s lymphoma of childhood. Proc Am Sot Clin Oncol6:190, 1987 21. Jenkin DT, Sonley JA, Stephens CA, et al: Primary gastrointestinal tract lymphoma in childhood. Radiology 92:763-767, 1969 22. Jones B, Klineberg WG: Lymphosarcoma in children: A report of 43 cases and a review of the literature. J Pediatr 63:1 l-20, 1963 23. Bugat R, Voigh JJ, Delsol G, et al: Non-Hodgkin’s primary lymphomas of the gastrointestinal tract. Front Gastrointest Res 4:192-197,1979 24. Braendstrup 0, Johansen A: Malignant non-Hodgkin lymphoma of the gastrointestinal tract: A histopathological review of 34 cases. Acta Path01 Microbial Stand 89:389-392, 1981 25. Contreary K, Nance FC, Becker WF: Primary lymphoma of the gastrointestinal tract. Ann Surg 191:593-598, 1980 26. Fitch DD, Wilson JAP: Primary gastrointestinal lymphoma. South Med J 78:909-913,1985 27. Gray GM, Rosenberg SA, Cooper AD, et al: Lymphomas involving the gastrointestinal tract. Gastroenterology 82:143-152, 1982 28. Siegert W, Hack1 G, Lohrs U, et al: Non-Hodgkin’s lymphomas presenting with gastrointestinal involvement. Klin Wochenschr 63:56-61, 1985 29. Al-Bahrani Z, Al-Mondhiry H, Al-Saleem T, et al: Primary intestinal lymphoma in Iraqi children. Oncology 43:243-250, 1986 30. Mestel AL: Lymphosarcoma of the small intestine in infancy and childhood. Ann Surg 149:87-94, 1959 31. Culp CE, Hill JR: Malignant lymphoma involving the rectum. Dis Colon Rectum 5:426-436, 1962 32. Nelson DF, Cassady JR, Traggis D, et al: The role of radiation therapy in localized resectable intestinal non-Hodgkin’s lymphoma in children. Cancer 39:89-97, 1977 33. Horrilleno EG, Eckert C, Ackerman LV: Polyps of the rectum and colon in children. Cancer 10:1210- 1220, 1957 34. Mestre JR: The changing pattern of juvenile polyps. Am J Gastroenterol81:312-314, 1986 35. Byrne WJ, Jimenez JF, Euler AR, et al: Lymphoid polyps (focal lymphoid hyperplasia) of the colon in children. Pediatrics 69:598-600, 1982 36. Gryboski JD: All juvenile polyps are not benign. Am J Gastroenterol (editorial) 81:397, 1986

Non-Hodgkin's lymphoma presenting as a rectal polyp in a child.

Primary lymphoma of the rectum is extremely rare in children. We report here a 10-year-old boy with localized non-Hodgkin's lymphoma, discovered withi...
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