EXTRAMEDULLARY PLASMA CELL TUMOR OF THE STOMACH: A CASE REPORT*

Transcription

1 EXTRAMEDULLARY PLASMA CELL TUMOR OF THE STOMACH: A CASE REPORT* JOHN S. COURET, M.D. From the Department of Pathology, Hotel Dieu, New Orleans, La. Extramedullar plasma cell tumor is also referred to as extramedullary plasmoma, plasmacytoma or plasma cell sarcoma. Schridde first described a case in Hellwig 5 recently reviewed the literature and collected 126 other cases besides Schridde's and added one of his own. Not included in Hellwig's review are McNamara and Rogers' patient 6 ; Gordon and Walker's 3 report of one case, and their mention of the unpublished reports of two others coming under the observation of Stewart at the Memorial Hospital; and the reports of one case each by Esposito and Stout 2, Standi and Tomlinson 7, and Haines 4. Thus 135 cases have been reviewed in the English language literature and in only one instance has this disease been found involving the stomach. This case, reported by Vasiliu and Popa and quoted by Brown and Liber 1 and by Hellwig 5, presented multiple ulcerated plasma cell tumors of the stomach and jejunum with metastases in the mesenteric lymph nodes. Hellwig found that most plasmomata originate in the mouth and upper air passages where about half are malignant, and where most cases of malignancy occur in the middle aged male patient. The invasion of bone or the occurrence of distant metastasis presaged a universally fatal outcome within two years, in spite of any form of therapy. The disease in this location has been treated by surgery, X-ray, and a combination of both with apparently inconclusive results. The next most frequent location in which this tumor occurs is the conjunctiva where it is relatively benign and usually responds readily to surgery. Other primary sites include the lymph nodes, pleura, lung, mediastinum, thyroid, lacrimal gland, intestine and stomach, skin, ovary and other urogenital organs. Since this disease is apparently rather rare in any extramedullary focus, and particularly in the stomach wall, the following case was thought worthy of publication, especially in view of the diagnostic problem it presented. Case Hi Q-1066 was that of a white female, aged 48, a patient of Dr. C.J. Brown, admitted to Hotel Dieu on February 12, 1943, complaining of epigastric pain, distention and "gas". Her illness began at the time of the menopause, 8 years before, with sticking pain in the region of the left scapula which recurred from time to time and persisted. Sometime after the onset she began to have epigastric pain, distention and vomiting. One year after the onset she had a left nephropexy, but experienced no relief. The digestive symptoms, bearing no relation to meals or type of food, gradually became worse, and also persisted until the time of admission. She lost a moderate amount of weight. Physical examination at the time of admission revealed a rather poorly nourished white female, not acutely ill. Temperature, pulse, respiration and blood pressure were normal, and no other positive findings were recorded. Laboratory findings: 5,500 WBC, with 72 per cent neutrophiles and 28 per cent lymphocytes; 3.2 million RBC, with marked polychromatophilia, anisocytosis, and poikilocytosis; hemoglobin 55 per cent (Tallqvist). The blood NPN was 27 mg. per 100 cc. of blood, the blood sugar 76 mg. The urine contained a trace of albumin, many pus cells and an occasional erythrocyte. The Kolmer Wassermann and the Kline flocculation tests were negative. One gastric analysis was reported as "unsatisfactory". The phenolphthalein excretin was 40 per cent after 2 hours. The pertinent findings observed by X-ray studies of the G.I. tract was a persistent filling defect in the prepyloric region of the stomach with distention of the fundus. The concluion: "findings consistent with malignancy of the prepyloric region of the stomach". * Presented before the Louisiana Association of Pathologists, New Orleans, Louisiana, January 4,

2 214 JOHN S. COTJRET The upper abdomen was explored surgically. Adhesions were found about the gall bladder, duodenum and pyloric end of the stomach. The latter was opened and the wall of the pyloric region was discovered to be edematous and thickened, causing almost complete pyloric occlusion. The pyloric portion of the stomach and the pyloric valve were resected and the end of the duodenum sutured to the lower angle of the gastric wound. The pathologic report on this tissue was: "The pyloric portion of the stomach measured 8x5 cm. The serosa showed nothing of note. The walls averaged 18 mm. in thickness. The rugae were swollen and confluent, so that the mucosal surface presented a lobated appearance. It was pale gray in color, but necked with numerous bright red ecchymotic areas, and had lost its normal velvety character. Small segments of normal mucosa were present at each extremity of the specimen. The increased thickness of the wall, which was stiff, firm, and edematous, was due to an increase of tissue in the submucosal layer, that presented cut surfaces of a pale ivory-gray color and a moist homogenous appearance. Microscopically, the gastric epithelium was found to be atrophied due to the pressure of a dense mass of cells in the submucosa which also extended into the edematous stromal elements of the muscle layers. The gastric glands had been almost completely obliterated, but the few remaining showed evidence of attempting regeneration. There was little evidence of fibrous tissue proliferation, and that present was compressed and often hyaline. The blood vessels were numerous in the submucosa and usually formed by a single endothelial layer. The cells of the mass itself were of two types. The larger and more numerous were roughly oval or polygonal in shape and had fairly well defined cell membranes. Their cytoplasm was slightly basophilic and glassy or finely granular in character. No perinuclear pallor was noted. The nuclei were relatively large and eccentrically placed. Most of the chromatin was arranged as granules and small masses immediately beneath the well denned nuclear membrane; and in many cells these chromatin masses were larger, so that a definite "cartwheel" effect was produced. In other cells, however, the nuclei were vesicular, while in still others they were small and dense. One or two nucleoli were usually present. Occasionally one of these cells was larger and contained two or even three nuclei, and an occasional mitotic figure was seen. The other type of cell, which in some areas predominated numerically (fig. 1), was smaller; but varied more in size, had an indefinite cell membrane, scanty palestaining cytoplasm, and a small, dense, well-defined nucleus. These cells were identified as rapidly proliferating plasma cells and lymphocytes and the lesion was diagnosed as inflammatory. The presence of small groups of similar cells in lymph spaces in the muscular coats of the stomach was observed, but no significance was attached thereto. The post-operative course was uneventful. She was discharged on March 10, and experienced symptomatic relief for about two months, but returned to the hospital on October 5, complaining of the same symptoms. Nausea and vomiting had become very severe, she had lost considerable weight and strength, and appeared acutely ill. A "knot" had appeared in the abdomen, which, she said,

3 PLASMA CELL TUMOR OF STOMACH 215 varied in size from time to time. She also complained of constipation. Examination revealed marked emaciation and a palpable mass at, and to the left of, the umbilicus, 8 cm. in length, with its long axis directed transversely. It was not very hard, did not move on respiration, but was slightly movable on manipulation. The laboratory examinations were noncontributory. The pertinent findings on roentgenologic studies revealed a moderate-sized defect of the lesser curvature of the pars media of the stomach, with the remaining portion of the pyloric region narrowed. It was concluded that the filling defect of the lesser curvature could have been due to an inflammatory cicatrization, and that the narrowing of the distal part of the stomach was possibly due to the previous operative procedure. No external pressure seat could be demonstrated. A second exploration was performed on October 25. The mass was found to be in the stomach, which was opened and its distal part discovered to be filled with a large fungating growth. A small biopsy of this mass was performed and FIG. 1 (950X). AREA FBOM STOMACH TUMOR, SHOWING MANY SMALL LYMPHOID AND LARGER PLASMA CELLS CELLS the operation terminated. This tissue showed numerous lymphocytes and relatively few plasma cells, all giving evidence of rapid proliferation. The diagnosis was now changed to lymphosarcoma. The post-operative course was stormy. There was much distention, and, on the ninth day the wound began to discharge a large amount of fluid. The general condition deteriorated progressively, and the patient expired on November 13. At the autopsy the pertinent morbid changes, except for the evidence of extreme emaciation, were mostly located in and about the stomach. A fistulous tract connected the cavity of this organ with the exterior through the operative wound; while a gastro-duodenostomy joined it to that of the proximal end of the duodenum. The upper of the stomach appeared normal, but the distal portion presented walls which measured from 2 to 2.5 cm. in thickness and were composed of confluent tumor masses, firm in consistency, with the inner surfaces red in color and thrown up into nodules. On section, these tumors were found to be com-

4 216 JOHN S. COURET posed of pale ivory-colored, moist, homogenous tissue, located mostly in the submucosa, but appearing to infiltrate and disorganize the underlying muscle layers. Some of this tissue had penetrated into the gastric attachment of the greater omentum in this region, and had also partially obstructed the opening between the stomach and duodenum. The remainder of the gastro-intestinal tract showed nothing of note. In the retroperitoneal space, just above the origin of the superior mesenteric artery, there was found an encapsulated oval mass, measuring 4x6 cm. Its outer surface was smooth and ivory in color, and on section it presented cut surfaces similar to those of the tumors in the gastric wall. Of note also was a lesion of the urinary bladder. On the mucosal surface there was a poorly denned, round, shallow ulcer, with an indurated base, 1 cm. in diameter, and 1 cm. below and medial to the left ureteral orifice. No evidence of skeletal disease was found. FIG. 2 (950X). AREA FROM METASTATIC RETROPERITONEAL TUMOR, SHOWING MORE TYPICAL PLASMA CELLS PREDOMINATING Microscopic examination showed the stomach lesion to be essentially the same as that found and removed at the first operation, except that the gastric epithelium over the tumors had been completely destroyed. In the retroperitoneal tumor the cells were nearly all of the larger type described in the first stomach biopsy (fig. 2). Numerous small areas of coagulation necrosis were scattered throughout. Normal lymph node architecture could not be identified. The bladder ulcer was shallow, with slight fibrosis of the base which was the site of a dense accumulation of lymphocytes, plasma cells and macrophages, the latter containing finely granular cytoplasm. It was not believed that any of these cells were the same as those described in the other lesions, but the distortion due to inflammation had altered them to an extent where this possibility could not be definitely ruled out. The remainder of the microscopic findings were not pertinent to this discussion.

5 PLASMA CELL TUMOR OF STOMACH 217 Anatomic diagnosis: 1) Malignant plasma cell tumor of the stomach, with metastasis to the retroperitoneal space. (Lymph node?) 2) Extreme inanition. 3) Ulcer of bladder Non-specific. 4) Hypoplasia of left kidney. 5) Chronic cholecystitis and pericholecystitis. DISCUSSION The histologic resemblance between plasma cell tumors and actively proliferating chronic inflammatory cells renders the differentiation between these two conditions often troublesome, as pointed out by Hellwig, and as illustrated in this present instance. In fact, many regard certain of the non-metastasizing plasmomata to be more granulomatous than neoplastic. Hellwig also states that not only is identification a difficult problem, but the histologic discrimination between the benign and malignant forms of the disease unsatisfactory. Hence this method is not to be depended upon too heavily for arriving at the diagnosis, prognosis or therapeutic indications in a given case. Had a proper evaluation, at the time of the first operation, been placed on the gross characteristics of the stomach tumor, which were certainly those of a neoplasm, it is probable that the true nature of the condition would have been recognized at that time. Instead, too much reliance was placed on the histologic picture. When the second biopsy was performed, the presence of a neoplasm could no longer be ignored; but, because of the preponderance of lymphocytes in the particular piece of tissue studied, the lesion was then thought to have been an atypical lymphosarcoma. It was only at the autopsy, when the metastatic tumor was found to be made up of almost typical plasma cells, that the true nature of the disease was discovered. And it was then also that the cell masses in the lymph channels of the stomach wall, observed in the first biopsy were recognized to be tumor thrombi. While, as indicated above, too much reliance should not be placed on the microscopic picture for the diagnosis of a given case, it is of course true that the recognition of a plasmoma rests finally on the microscopic identification of plasma cells as the characteristic elements of the tumor. For this Marschalko has given certain criteria, which are: 1) round, oval or polygonal cells with abundant, nongranular and basophilic cytoplasm; 2) eccentrically placed nuclei; 3) paranuclear pallor; and 4) the arrangement of the relatively large chromatin masses within the nucleus so as to produce the so called "cart-wheel" nucleus. In the case under discussion the characteristic cells, best exemplified in the metastatic nodule found at autopsy, but also present in the primary stomach tumor, fulfilled most of these conditions. However, perinuclear pallor was not observed, while in some cells the cytoplasm was slightly granular, and in some others the nuclei were more vesicular than "cart-wheel". But on the whole, the resemblence to typical plasma cells was quite plain (fig. 2). No Russel bodies were found in any of the cells in the sections studied. Staining according to Pappenheim's technic was not done as the tissue had not been fixed in alcohol. Lymphocytes, which were so numerous in certain areas of the gastric lesion, are said to be characteristic

6 218 JOHN S. COURET of many of these tumors. In fact, their presence, together with the belief that plasma cells are derived from them, lead many to consider plasmomata as a form of lymphoma or lymphosarcoma. It should also be noted that the eight year duration of this patient's symptoms need not be considered as the duration of her neoplasm, but could well be accounted for by the low grade chronic cholecystitis found at autopsy. However, it is impossible to state, from her history, when the plasmoma first manifested itself. The immediate cause of death was attributed to extreme inanition, resulting from the tumor's location. CONCLUSION A case of malignant extramedullary plasma cell tumor arising in the stomach and metastasizing to the retroperitoneal space is reported. The tumor was not recognized histologically until the autopsy; although the clinical, radiologic and gross pathologic findings indicated a malignant obstructive lesion of the pyloric region. The difficulty of the microscopic diagnosis of this condition is discussed. REFERENCES 1. BROWN, C. R., AND LIBER, A. F.: Multiple plasmoma of the ileum and colon. Arch. Path., 28: 112, ESPOSITO, J. J., AND STOTJT, A. P.: Multiple plasmocytoma of the jejunum. Am. J. Roentgenol., 53: 33, GORDON, J., AND WALKER, G.: Plasmocytoma of the lung. Arch. Path., 37: 222, HAINES, M.: Metastasizing plasma cell tumor of pharyngeal tonsil. J. Laryng. and Otol., 57: 264, HELLWIG, C. A.: Extramedullary plasma cell tumors as observed in various locations. Arch. Path., 36: 95, MCNAMARA, W. L., AND ROGERS, R. J.: Extramedullary plasma cell tumor of a tonsil with metastasis. Arch. Path., 36: 89, STANCIL, J. R., AND TOMMNSON, W. J.: Extramedullary plasma cell tumor of the mouth. Arch. Otolaryng., 40: 139, 1944.