Amyloidosis of gastrointestinal tract simulating gastric carcinoma

Amyloidosis of Gastrointestinal Tract Simulating Gastric Carcinoma PAUL H . KLINGENBERG, M .D .,

From the Surgical Service, Veterans Administration Center Hospital, Dayton, Obio and the Department of Surgery, Ohio State University, Columbus, Obio . TTENTION

has been drawn in the literature

A to the fact that gastrointestinal involve-

ment with deposits of amyloid occurs . The most frequent signs and symptoms have been gastrointestinal hemorrhage (mucosal and submucosal ulceration), abdominal pain, and vomiting secondary to pyloric obstruction [r,4] . Complete pyloric obstruction has been described with filling defects seen on gastrointestinal series that strongly suggest a malignant lesion . Although frequently pathological specimens must be obtained for proper diagnosis, occasionally laboratory, clinical and x-ray findings [5] are sufficiently suggestive of amyloidosis that useless extensive surgical treatment can be avoided . (Fig. i .) Very often, definitive therapy for pyloric tumor that later is found to be amyloid results in unpleasant complications with high mortality . Although this disease is uncommon, a familiarity with its existence may help avoid hazardous surgery which is most likely to end in failure . There is little therapeutic value in the surgical care of primary gastrointestinal amyloidosis . The prognosis is poor when involvement of gastrointestinal tract is severe enough to cause symptoms . Anastomosis of bowel containing amyloid is very likely to be unsuccessful, partly because of the poor nutrition associated with this pathological process . Nutrition deteriorates as mucosal atrophy interferes with gastrointestinal absorption and muscular involvement with amyloid deposits prevent normal peristalsis . A functional obstruction may be present and a pyloric tumor mass may cause organic obstruction by narrowing the lumen . Patients with this condition become markedly debilitated . Golden [21 described

Covington, Kentucky

death from general debility several weeks after gastric resection . There is high incidence of surgical complications . Shnider and Burka [3] report leakage of duodenal stump with peritonitis and death . It is very difficult to remove all lesions because of the extent of the disease process . There is no known satisfactory therapy for primary amyloidosis [q,] . Patients respond poorly to medical management . If suspicion is aroused that the surgeon is dealing -with amyloidosis, then diagnostic biopsy is of most benefit . Knowledge of the disease may prevent needless surgery in an effort to establish diagno-

FiG . i . Atonicity of stomach and loss of mucosal pattern of small bowel is typical of amyloidosis .

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;3 American Journal of Surgery, Volume 96, November, 1o

Klingenberg

FIG . 3 . Small bowel showing diffuse thickening and the glistening, granular serosa with longitudinal striations . developed . No abdominal masses were felt ; there was no lymphadenopathy . Roentgenograms showed incomplete obstruction at the pylorus by a smooth, round tumor on the lesser curvature of the stomach . (Fig. 2 .) A malignancy could not be ruled out . Hemogram was normal . No free acid was found in gastric contents ; total protein was 4.5 gm . with 3 .0 gm. of albumin and 1 .5 gm . of globulin . An attempt at gastric evacuation with a Levin tube and subsequent oral feedings were not successful . Two pints of blood were given to correct the blood volume deficit for a weight loss of i o pounds in the hospital . Following the correction of electrolyte imbalance and the use of intravenous albumin, laparotomy was completed on November 1, 1 955 . Marked thickening of the entire gastrointestinal tract by a leathery disease process (Fig. 3) involved the muscle layers primarily and produced longi-

FIG . 2 . Smooth tumor on lesser curvature of stomach at level of pylorus producing incomplete obstruction . sis and treat adequately a lesion that appears to be a malignancy .

CASE REPORT T . H ., a sixty-three year old white man, was admitted on September 30, 1955, with pain in both arms, hands and wrists, and left hip, and stomach trouble described subjectively as "gas," present for about one and a half years . Swelling of hands and fingers had been marked for ten days prior to admission . On physical examination the presence of numerous small miliary, firm, subcutaneous nodules scattered all over the body were noted . The patient appeared emaciated and was very weak . Six days after admission nausea and vomiting

FIG . 5 . Marked atrophy of mucosa of stomach .

FIG . 4 . Marked thickening at pylorus (tumor mass) by amyloid narrowing the entrance into duodenum . 714

Amyloidosis of Gastrointestinal Tract tudinal serosal striations . The pyloric lumen was markedly narrowed by this diffuse process. (Fig. 4 .) The mucosa was atrophic (Fig . 5) ; serosa was glistening and granular . (Fig . 3 .) Frozen section was reported as showing chronic inflammation . Because of the incomplete obstruction, hemigastrectomy was completed with gastroduodenostomy . The patient withstood the surgical procedure well ; permanent microscopic sections of the stomach were reported to show primary amyloidosis of the stomach and duodenum, with marked thickening of the pylorus . On the third postoperative day, confusion and further progression of the weakness and debility developed in the patient . He died on November 6, 1955 ; autopsy indicated moderate pulmonary edema, several small pulmonary emboli, bronchopneumonia and primary amyloidosis of the entire gastrointestinal tract, bladder, skin, right knee joint, heart and blood vessels . The gastrointestinal anastomosis was intact .

Occasionally, surgery must be attempted, for example, to stop severe bleeding . Knowledge of amyloid in bowel may suggest a less extensive procedure than otherwise is indicated . SUMMARY

A case of primary amyloidosis involving the entire gastrointestinal tract is presented, showing typical roentgenographic, clinical and pathological findings and simulating obstruction at pylorus by malignancy . Surgery is beneficial only (i) as a diagnostic measure and (2) as an emergency measure to control bleeding, relieve obstruction, etc . Radical surgery is frequently unsuccessful . REFERENCES 1 . COOLEY, R . N . Primary amyloidosis with involvement of stomach . Am . J. Roentgenol ., 70 : 428, 1953 . 2 . GOLDEN, A . Primary systemic amyloidosis of alimentary tract . Arch . Int . Med ., 75 :4 1 3 -416, 19453 . SHNIDER, B . I . and BURKA, P . Amyloid disease of stomach simulating gastric carcinoma . Gastroenterology, 28 : 424 -430, 1955 . 4- BERO, G. Amyloidosis, its clinical and pathologic manifestations . Ann . Int . Med., 46 : 931, 1956 . 5 . KORELITZ, B . and SPINDELL, L. Gastrointestinal amyloidosis . J. Mt. Sinai Hosp ., 23 : 683, 1956 .

COMMENTS

This case fits the criteria described by Lubarsch as constituting primary amyloidosis [r] . Biopsy of the skin which was not performed most likely would have been diagnostic and might have served to contraindicate surgery since x-ray films of the stomach and small bowel were highly suggestive of this disease .

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