Benign Lymphoid Hyperplasia Manifesting as a Cecal Mass" Report o f a Case*

RANDLE C. JOHNSON, M.D., MAJOR, U S A F , MC, MICHAEL H. BLESHMAN, M . D . , t JAMES W. DEFORD, M.D.S

THE BOWEL is a l a r g e r e s e r v o i r o f l y m p h o i d tissue that may become involved in several diseases: leukemia lymphoma, dysgammaglobulinemia, and benign lymphoid hyperplasia. B e n i g n l y m p h o i d h y p e r p l a s i a ( B L H ) is a collection o f l y m p h o i d tissue w i t h i n the bowel wall, c a u s i n g elev a t i o n s o f the n o r m a l m u c o s a . T h e u s u a l l o c a t i o n o f b e n i g n l y m p h o i d h y p e r p l a s i a is the t e r m i n a l i l e u m , b u t it m a y be d i s t r i b u t e d t h r o u g h o u t the e n t i r e c o l o n o r l i m i t e d to the r e c t u m . B e n i g n l y m p h o i d h y p e r p l a s i a is u s u a l l y s e e n in the y o u n g , a n d is u s u a l l y a s s o c i a t e d w i t h few o r n o symptoms. We have s e e n a y o u n g boy who h a d i n t e r m i t t e n t e p i s o d e s o f f e v e r , chills, d i a r r h e a a n d a b d o m i n a l p a i n . D u r i n g o u r e v a l u a t i o n he was f o u n d to have a cecal mass. C o l o n o s c o p y a n d o p e r a t i o n r e v e a l e d this c e c a l m a s s to b e l o c a l i z e d b e n i g n l y m p h o i d h y p e r p l a s i a with p a r t i a l a p p e n d i c e a l o b s t r u c t i o n . App e a r a n c e o f c e c a l a b n o r m a l i t y d u e to b e n i g n lymphoid hyperplasia has not been previously reported. Report of a Case A 15-year-old Caucasian boy was referred because of a ninemonth history of episodes of dull abdominal pain associated with fever, chills, diarrhea, nausea and vomiting. Typically, each episode would last approximately two hours and then resolve, leaving the patient feeling well. From December 1974 to October 1975, he had five of these episodes. In October 1975, he had a typical attack of abdominal pain and was seen in the emergenc?, room, where he was referred for further evaluation to the Gastroenterology Clinic. Emergency room data included temperature of 102 F, a leukocyte count of 8,050 with 83 per cent polymorphonuclear Ieukocytes and band forms. Serum glutamic oxaloacetic transaminase (SGOT) was 240 * Received for publication May 8, 1978. +Current address: Phoenixville Hospital, Department ot" Radiology, Pheonixville. Pennsylvania. - C u r r e n t address: 907 S.W. Fourth Avenue, Gainesville, Florida. Address reprint requests to Dr. johnson: Wilford Hail USAF Medical Center/SGHMG, Lackland AFB, Texas 78936.

From the Gastroenterology. Service and Department of Radiology., Wilford Hall USAF Medical Center, Lackland AFB, Texas

units (normal less than 38 units), alkaline phosphatase was 400 units, and bilirubin was 1.6 rag/100 ml total with 0.4 mg/100 ml direct. A stool specimen was positive for Giardia lamblia cysts. The patient was admitted to the hospital. A history of travel to Japan, Florida, and most recently, Colorado Springs, Colorado, was obtained. Other than his current problem his review of systems was unremarkable. Physical examination showed no abnormality. The foUowing laboratory data were negative or normal: complete blood count, erythrocyte sedimentation rate, reticulocyte count, serum electrolytes, blood urea nitrogen (BUN), creatinine, uric acid, calcium, phosphate, results of urinalysis, prothrombintime, partial thromboplastin time SPE, serum iron and ironbinding capacity, anti-smooth-muscle antibody, antimitochondrial antibody, antinuclear antibody, hepatitis B antigen, haptoglobin, serum copper, serum IgG, IgA, IgM, creatine phosphokinase, lactate dehydrogenase, serology, stool culture, ECG, chest x-ray, oral cholecvstogram and intermediate PPD skin test. The' following data were obtained and were abnormal: examination of stools for ova and parasites--all positive for Giardia lamblia; SGOT 67 units, returning to norntal range during evaluation; bromsulphalein 10 per cent retention at 45 minutes; gammaglutamyl transpeptidase 42 IUll (normal less than 40 lull), alkaline phosphatase 260 units with a normal thermostable portion. Metronidazole was begun as therapy for giardiasis. An upper gastrointestinal series with small-bowel follow-through, obtained shortly after institution of therapy, revealed a cecal deformity, which was felt to be compatible with ameboma. The metronidazole dosage was increased to 750 mg three times a day for two weeks. Barium-enema examination confirmed the cecal abnormality (Figs. 1 and 2). Repeat sigmoidoscopy and rectal biopsy showed no abnormality. Barium-enema examination at the completion of metronidazole therapy revealed no change in the cecal deformity. Colonoscopy showed small nodules in the cecum with normalappearing mucosa. Examination of colonoscopic biopsies revealed benign lymphoid hyperplasia of the cecum (Fig. 3). However, because of continuing concern for a neoplastic or an infectious process right ileocolectomy was accomplished. At operation and pathologic examination there were small polypoid masses in the terminal ileum and in the cecum. Histologic examination revealed benign lvnmhoid hvperplasia (Fi~. 4). This diagnosis was . Forces . . Institute of Patho 1ogy. T he apconfirmed by. the .Armed pendiceal lumen was partially occluded by a group of these polypoid masses, with purulent material found in the lumen of the appendix. The patient recovered from the operation uneventfully. Outpatient follow-up for 18 months has been unremarkable, without recurrence of symptoms.

0012-3706/78/1000/0510/$00.70 9 American Society of Colon and Rectal Surgeons

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Fro. 1

(left).

FIC. 2

(right).

5 11

B a r i u m - e n e m a study, s h o w i n g an a b n o r m a l i t y in the medial a n d inferior c e c u m . C o m p r e s s i o n view, s h o w i n g mass effect in the c e c u m on b a r i u m - e n e m a s t u d y

Discussion

Lymphoid diseases of the bowel are confusing entities. Benign lymphoid hyperplasia has been described as gastrointestinal pseudoleukemia, a'l~''la lymphoid hyperplasia, H lymphoid polyps,' lymphoid polyposis,9 lymphoid hyperplasia of the terminal ileum, 4 and multiple lymphomatous polyposis.2 The lymphoid nodule may involve the gastrointestinal tract from the stomach to the anus. 12 Benign lymphoid hyperplasia of the colon and rectum usually has a peak incidence in children 1 to 3 years of age. The polypoid lesions are usually diffuse, but may be localized to the rectum. It is previously unreported for lymphoid nodules to manifest as a limited cecal abnormality. Symptoms, when present, are usually related to rectal bleeding from ulceration of a nodule, but intussusception can occur. 1~ Radiographic examination of the intestinal tract may reveal m a n y nodules, e i t h e r localized or

generalized. A central dimple in the nodules is cons i d e r e d good evidence for b e n i g n l y m p h o i d hyperplasia. 8 Other entities which may produce a similar radiographic appearance of many nodules are i m m u n o g l o b u l i n deficiencies, especially IgA and IgM,6 leukemia, lymphoma, Hodgkin's disease,2 and multiple lymphoid nodules in familial polyposis and Gardner's syndrome2 '9 Colonoscopy is a diagnostic approach in benign lymphoid hyperplasia. Colonoscopy in our patient permitted visualization of the cecal nodules, A histologic diagnosis of benign lymphoid hyperplasia was established by biopsy. Colonoscopy is helpful in eliminating a diagnosis of neoplasia. We were not convinced that the cecal n o d u l e s visualized by colonoscopy explained the mass effect and the clinical course. Gross examination of the pathologic specimens will show many nodules covered by normal mucosa. The central dimple may be seen on gross examination,s

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JOHNSON, ET AL.

October 1978

Fro. 3. Colonoscopic biopsy specimens, showing benign lymphoid tissue underlying normal mucosa (x 33).

Ftc;. 4. Pathologyspecimen section, showingbenign lymphoid nodules (x 33).

Microscopically, benign lymphoid hyperplasia is represented by lymphoid nodules with germinal centers covered by normal mucosa. Other features of benign disease include the presence of chronic inflammatory cells, germinal centers with mitotic activity, separation of germinal centers by orderly reticular strands, and mature lymphocytes?. 8 Therapy for benign lymphoid hyperplasia has included observation, steroids, radiation therapy, and surgery.2, ~,~o,~ In our patient, the mass effect of the lymphoid nodules was an unexpected finding. The initial manifestation in our patient was very similar to that in other cases in the literature: intermittent episodes of abdominal pain. v,l~ We feel that these episodes of pain, fever, nausea and vomiting may have been related to intermittent obstruction of the appendiceal orifice by one or more of the hyperplastic lymphoid polyps (nodules). We do not feel that giardiasis was related to our patient's benign lymphoid hyperplasia because serum immunoglobulins were normal. Our patient underwent surgical treatment for a benign process, as has happened to many others reported in the literature. However, we felt, as have

others, that a neoplastic process had not been sufficiently excluded. Colonoscopy will allow future patients to be followed conservatively after colonoscopic biopsy establishes a diagnosis of benign lymphoid hyperplasia. Those patients who are followed conservatively need r e p e a t e d e v a l u a t i o n s - - b o t h radiographic and colonoscopic--because the lymphoid nodules may be one of the first signs of malignant lymphoma,z When there is a familial history of other polypoid diseases, these patients should be followed for the development of adenomatous polyps.

Summary We have presented an unusual case of benign lymphoid hyperplasia, which manifested as a cecal deformity in a 15-year-old boy. The clinical manifestation may have been related to partial occlusion of the appendiceal orifice. In future cases of benign lymphoid hyperplasia, colonoscopy may be diagnostic, and if it is used for continuing observation, may avert unnecessary surgical procedures in children and young adults.

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References 8. 1. Anthony JE Jr: Hyperplastic lymphoid polyps of the terminal ileum. South M e d J 61: 571, 1968 2. Comes JS: Multiple lymphomatous polyposis of the gastrointestinal tract. Cancer 14: 249, 1961 3. Cosens CG: Gastro-intestinal pseudoleukemia: A case report. Ann Surg 148: 129, 1958 4. Fieber SS, Schaefer HJ: Lymphoid hyperplasia of the terminal ileum--a clinical entity? Gastroenterology 50: 83, 1966 5. Gruenberg J, Mackman S: Multiple lymphoid polyps in familial polyposis. Ann Surg 175: 553, 1972 6. Hermans PE, Huizenga KA, Hoffman HN II, et al: Dysgammaglobulinemia associated with n o d u l a r lymphoid hyperplasia of the small intestine. Am J bled 40: 78, 1966 7. Jona j z , Belin RP, Burke JA: Lymphoid hyperplasia of the

9. 10.

11. 12. 13.

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bowel and its surgical significance in children. J Pediatr Surg 11: 997, 1976 Shaw EB Jr, Hennigar GR: Intestinal lymphoid polyposis. Am J Clin Pathol 61: 417, 1974 Shull LN Jr, Fitts CT: Lymphoid polyposis associated with familial potyposis and Gardner's syndrome. Ann Surg 180: 319, 1974 Silverman A, Roy CC, Cozzetto FJ: Lymphoid polyposis (lymphoid nodular hyperplasia of the colon and rectum). In: Pediatric Clinical Gastroenterology. St. Louis, The C. V. Mosby Company, 1971, p 266 Swartley RN, Stayman JW Jr: Lymphoid hyperplasia of the intestinal tract requiring surgical intervention. Ann Surg 155: 238, 1962 Tannhauser S, Davison R: Gastro-intestinaI pseudoleukemia (report of a case). Am J Dig Dis 7(os): 45, 1940 Wells HG, Mayer MB: Pseudoleukaemia gastrointestinalis. Am J Med Sci 128: 837, 1904

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Benign lymphoid hyperplasia manifesting as a cecal mass: report of a case.

Benign Lymphoid Hyperplasia Manifesting as a Cecal Mass" Report o f a Case* RANDLE C. JOHNSON, M.D., MAJOR, U S A F , MC, MICHAEL H. BLESHMAN, M . D...
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