DEEP SPREADING CARCINOMA OF THE STOMACH CAUSING DEATH WITHIN SEVEN MONTHS OF LAST NORMAL X-RAY EXAMINATION

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1 GASTROENTEROLOGY Copyright C 1969 by The Williams & Wilkins Co. Vol. 56, No.1 Printed in U.S.A. DEEP SPREADING CARCINOMA OF THE STOMACH CAUSING DEATH WITHIN SEVEN MONTHS OF LAST NORMAL X-RAY EXAMINATION MUHAMMAD ASLAM, M.D., BENO PACIUC, M.D.,OSVALDO L. RUBINSTEIN, M.D., AND JAMES B. HAMMOND, M.D. Medical, Diagnostic Radiology, and Laboratory Services, Veterans Administration Research Hospital, and Departments of Medicine, Radiology, and Pathology, Northwestern University Medical School, Chicago, Illinois A case of deep spreading carcinoma (linitis plastica) is reported. A radiological examination was performed because of the finding of localized scleroderma, to rule out systemic sclerosis, with normal findings. Six weeks later the patient developed symptoms of a gastric malignancy; 10 weeks later, extensive involvement of the stomach was confirmed radiologically. Fourteen weeks after the initial X-ray study he was explored surgically and considered inoperable. Gastric malignancy and acute complications caused this patient's death 7 months after his initial normal roentgen examination. Necropsy revealed extensive involvement of his entire' stomach by a deep spreading carcinoma, with metastases only to biliary lymph nodes. The case illustrates the rapid course followed by a small proportion of patients bearing this tumor type. Estimates of rates of growth of gastric carcinomas have been based on duration of symptoms prior to diagnosis, and survival afterward. Such observations do not provide any information on the time interval between the development of radiological evidence of tumor and the onset of symptoms. The case to be reported is an exception. A normal radiological examination was made 6 weeks before symptoms developed and 10 weeks prior to radiological detection. The patient was followed and roentgen examinations were repeated serially until death occurred due to deep spreading carcinoma (linitis plastica) and associated acute complications after a 7-month course. To the authors' knowledge, such observations have not been reported for this type of cancer. Received January 29, Accepted June 19, Address requests for reprints to: Dr. James B. Hammond, Veterans Administration Research Hospital, 333 East Huron Street, Chicago, Illinois Case Summary The patient, a 73-year-old white male dye setter, was admitted to Veterans Administration Research Hospital on March 28, 1966, for extraction of bilateral cataracts. He had no digestive complaints at that time; his weight was at its usual level of 175 lb. He was admitted for the second time on July 26, 1966, for investigation of alopecia and patchy hypopigmentation, of 6 years' duration. His past history was unremarkable except for an appendectomy in His sister had died of carcinoma of the stomach. He denied digestive tract symptoms. Physical examination revealed patchy areas of alopecia and scarring over the scalp, and patchy areas of hypopigmentation, hyperpigmentation, and atrophic skin changes over the back and upper extremity consistent with localized scleroderma (morphea) associated with alopecia.cicatricia and vitiligo. He also had Dupytren's contraction of the right hand, an appendectomy scar, and hearing loss of undetermined etiology. His Venereal Disease Research Laboratories test was negative. A roentgenological examination of the upper gastrointestinal tract was performed on

2 138 CASE REPORTS Vol. 56, No. 1 August 1, 1966, to rule out any possibility of systemic scleroderma. The esophagus, stomach, and small bowel were interpreted as normal (fig. la, B, and C), as were the chest and colon. He was treated for localized scleroderma with local injections of methyl prednisolone acetate with no apparent beneficial effect. He was discharged on September 12, Several days afterward he first noted loss of appetite, and approximately 3 weeks after discharge he developed difficulty in swallowing solid food. He was seen by his private physician who advised X-ray studies of his esophagus and stomach, which were performed on October 12, On this examination, narrowing of the terminal esophagus producing delayed transit, very small stomach size with rigidity of the gastric wall, and rapid emptying were noted (fig. 2A). The duodenal bulb and small bowel appeared normal. He was admitted for the third time on October 19, 1966, for further evaluation. On inquiry, the patient described a new symptom- esophageal regurgitation. Physical exam ina- FIG. 1. A, B, and C, normal radiological examination of the stomach on August 1,1966.

3 January 1969 CASE REPORTS 139 FIG. 2. A, X-ray of stomach on October 12, 1966, reveals narrowing of the gastric lumen, diffuse stiffening of the wall of the stomach, and obstruction of the distal esophagus with proximal dilation. B, On October 21, 1966, progression in the abnormalities demonstrated at the previous examination is shown. C, Findings on examination of the stomach on November 9, 1966, are similar to those previously noted. tion revealed that his weight was 164 lb, 11 lb below his usual weight, and that his liver was palpable 3 cm below the costal margin with a total length of 13 cm. The spleen was not palpable. There was some fullness in the epigastrium with minimal tenderness but no definite mass. Minimal spooning of the nails and pretibial edema also were noted. Roentgenological examination was repeated on October 21, 1966, and again showed narrowing of the terminal esophagus with retention and dilation proximal to the narrowed segment (fig. 2B). The stomach lumen was further decreased. as compared with the October 12 X-ray. Peristaltic activity was not observed, on fluoroscopy. On November 9, further decrease in the size of the gastric lumen was demonstrated (fig. 2C). Esophagoscopy with the Eder-Hufford instrument showed that the mucosa of the distal esophagus was pale with a few minute focal hemorrhages. Resistance to further introduction of the instrument was encountered at the level of 36 cm from the incisor teeth. Biopsy was unsuccessful. An attempt at gastroscopy alone was unsuccessful due to distal esophageal resistance to instrumentation. Intubation of the stomach likewise could not be performed, which prevented attempted gastric analysis and gastric washing for cytology. A Diagnex blue test for free Hel was positive. Abnormal laboratory findings included bromsulphthalein retention of 14% after 45 min and a prothrombin activity of 60%. The following determinations were in the normal range: hematocrit, total leucocytes, urinalysis, serum electrolytes, serum proteins, alkaline phosphatase, bilirubin', blood urea nitrogen, fasting blood glucose, transaminase, and lactic dehydrogenase. Occult blood was not detected in fecal specimens.' Electrocardiogram was normal. Pulmonary function studies were consistent with the diagnosis of emphysema. Exploratory laparotomy was performed on November 18, 1966, with a preoperative diagnosis of the linitis plastica type of carcinoma of the stomach. This diagnosis was confirmed by the operative findings. The entire stomach was thickened. A fibrotic attachment of the spleen to the stomach was noted. The splenic lymph nodes were palpably enlarged. A full thickness biopsy of the stomach was obtained, and a feeding tube was placed in the proximal jejunum. The patient underwent an uneventful postoperative course. Subsequently, the gastric biopsy revealed undifferentiated carcinoma of the stomach. When he was discharged on January 12, 1967, he weighed 1211b, 54lb below his usual weight. At home he had one episode of hemoptysis without chest pain. He was readmitted on March 4, 1967, in a terminal condition, confused, cachectic, and hypotensive. A large amount of purulent bile-stained drainage from around the feeding tube was observed. He developed diarrhea terminally, and expired on March 7, Necropsy Findings At necropsy, the body was emaciated. A healed abdominal surgical scar and a

4 140 CASE REPORTS Vol. 56, No.1 jejunostomy with hyperemia and superficial necrosis of the surrounding skin were noted. The stomach appeared to be contracted and roughly spherical in configuration. The serosa appeared normal. The gastric wall was rigid and markedly thickened throughout. On section it measured 0.5 cm, and was diffusely infiltrated by grayish white tissue which penetrated the tunica muscularis. The mucosal surface was flattened and slightly granular, with multiple areas of necrosis. No polypoid or ulcerative lesions were present. Histological examination revealed diffuse infiltration of the gastric mucosa with neoplastic cells. These cells were pleomorphic, with hyperchromatic and occasionally vesicular nuclei, with few mitoses, distributed in dense sheets. The tumor cells were noted between bundles of smooth muscle fibers in the muscularis (figs. 3A and B) and occurred in nests at the serosal surface, which was markedly thickened by a chronic inflammatory process. The tumor cells were surrounded by a dense fibrous stroma in all gastric layers. In addition, large numbers of chronic inflammatory cells, mainly lymphocytes, were present. The duodenum was markedly and uniformly dilated to a circumference of 10 cm. The mucosal surface of the entire duodenum and of the proximal jejunum surrounding the jejunostomy was greenish black in color and foul smelling. The mucosa around the jejunostomy tube was necrotic and sloughing. The ampulla of Vater was patent; however, the common bile duct and pancreatic duct were dilated. Microscopic examination revealed necrotizing duodenitis and jejunitis. The liver was grossly normal in appearance. Microscopically there was evidence of ascending necrotizing cholangitis. The larger bile ducts showed extensive necrosis and contained bacterial colonies. Centrilobular necrosis and focal fatty changes were also noted. The biliary lymph nodes were enlarged to as much as 2 cm in diameter, firm, and on microscopic examination contained metastatic carcinoma. The pancreas also showed acute and chronic interstitial pancreatitis with focal FIG. 3. A, Masses of neoplastic cells are seen infiltrating between smooth muscle bundles of the tunica muscularis (X 50). B, Higher magnification from an adjacent field showing neoplastic cells, smooth muscle, and fibrous stroma (X 235).

5 January 1969 CASE REPORTS 141 hemorrhages and clusters of polymorphonuclear leukocytes in the acini and within the pancreatic ducts. The esophagus was normal. The findings were interpreted as indicating (1) deep spreading carcinoma (linitis plastica), involving the entire stomach, with metastases only to biliary lymph nodes, and (2) severe necrotizing jejunitis and duodenitis, secondary to an indwelling jejunostomy tube, with ascending necrotizing cholangitis and purulent pancreatitis. Multiple pulmonary infarcts associated with phlebothrombosis of the left popliteal and femoral vein and thrombosis of the periprostatic plexus were contributory causes of death. Discussion The development of localized scleroderma in this patient lead to a roentgenological examination of the stomach in the absence of gastrointestinal symptoms. An apparently normal esophagus and stomach were demonstrated. Six weeks later the patient developed symptoms of a gastric malignancy, and 10 weeks later, extensive involvement of the stomach was confirmed radiologically. He was explored surgically 14 weeks after the initial normal X-ray study, and at that time was considered inoperable. His death 7 months following the initial X-ray was due to gastric malignancy and thromboembolic and septic complications. Very likely the carcinoma was present but undetected at the time of the initial examination. The course suggests a rapid evolution from a subclinical lesion to the onset of symptoms and extensive involvement of the entire stomach within 14 weeks. It has been authoritatively stated that the average duration of symptoms of deep spreading carcinoma (linitis plastica) prior to recognition is 1 to 2 years, compared to 6 months for other types of gastric carcinoma. l This estimate is based ' on the series of Lyle 2 and Lyons,3 totaling 70 cases. However, in a relatively uncommon tumor type, averages may be misleading; in Lyons' series of 33 cases, duration of symptoms ranged from 5 weeks to 15 years. Likewise, in a series of 26 patients reported by Saphir and Parker, 4 duration of symptoms prior to diagnosis varied from 3 weeks to 6 years. The case reported falls within these ranges. No large series of linitis plastica has been reported recently, and information on incidence, duration of symptoms, and survival after surgery requires updating. Until this is done it would seem that the protracted, slowly progressive course of linitis plastica has been overemphasized. Lust and Heimlich 5 reported a case in which the patient had symptoms for 3 years before the diagnosis was made. Their patient had two series of roentgen studies 6 months apart showing minimal mucosal changes on the first study to involvement of 7 cm on the greater curvature on the second examination. This is the only previous <;:ase report we have found which documents the progression of this type of carcinoma by serial X-ray studies. The authors theorize that the fibrotic stroma of the tumor was due to "an irritant of low virulence, acting continuously and slowly over a long period." In the case reported, fibrotic changes occurred relatively rapidly, suggesting some other mechanism was operative. Despite the apparent rapid growth of the patient's carcinoma, the only metastases found at necropsy were in intraabdominal lymph nodes. In retrospect, total gastrectomy probably should have been attempted at the time of surgical exploration. Saphir and Parker 4 have emphasized the low incidence of metastases to liver and to extraabdominal sites in linitis plastica, and the relatively high incidence of metastases to abdominal lymph nodes, intestines, ovaries, and spleen. Present evidence suggests that surgical extirpation of the tumor may often be possible even when there is involvement of the entire stomach. The necropsy findings suggested that the feeding jejunostomy caused partial obstruction of the proximal jejunum, and lead to sepsis with necrotizing enteritis,

6 142 CASE REPORTS Vol. 56, No.1 complicated by purulent cholangitis and pancreatitis. Pancreatitis associated with duodenal obstruction from a variety of conditions has been described. 6 REFERENCES 1. Bockus, H. L Gastroenterology, p Ed. 2, Vol. 1. W. B. Saunders Company, Philadelphia. 2. Lyle, H. H. M Linitis plastica (cirrhosis of stomach); with a report of a case cured by gastrojejunostomy. Ann. Surg. 54: Lyons, J. H Linitis plastica. Surg. Gynec. Obstet. 39: Saphir, 0., and M. L. Parker Linitis plastica type of carcinoma. Surg. Gynec. Obstet. 76: Lust, F. J., and H. J. Heimlich. Roentgenographic findings in an early case of linitis plastica. J. A. M. A. 173: Dreiling, D. A., P. A. Kirchner, and H. Nemser Chronic duodenal obstruction: a mechanovascular etiology of pancreatitis. I. Report of 6 cases illustrating this clinical variety. Amer. J. Dig. Dis. N. S. 5: