Primary extramedullary plasmacytoma of the omentum associated with recurrent adenocarcinoma of the colon: First case report

MEDICAL 1N T E L L I G E N C E 17. Madonia. T.: Sulla grande emorragic cerebrale da Icsioni vascolari letncemiche, ttaematologica,37:693, 1953. 18. Volland. W.: Beitrag zur Kerntis der apoplexien im Kindesaher. Dtsch. Z. Nervenheilk.,168:418, 1952. 19. Conne)', T., and Sweene)., E.: The belt's tootln: arterial aneur)sm resuhing from invasion by lgD myeloma cells. Irish J. Med. Sci. 146:295, 1977. 20. Rappaport, H., Strum, S. B.. llutchinson, G., and Allen, L. W.: The clinical and biologicalsignificanceof vascular invasion in llodgkin's disease. Cancer Res..31:179t, 1971.

PRIMARY EXTRAMEDULLARY PLASMACYTOMA OF THE OMENTUM ASSOCIATED WITH RECURRENT ADENOCARCINOMA OF THE COLON: FIRST CASE REPORT BERNARD PEISON, *I.D.,* BAR~V BE,XXSCH, M.D.,l" MAXLEY C. WIX.LtAMS, M.D.,~AND RICIIARO NEWMAN, M.I).w

Abstract Extramedullmy plasmacytomas are rare tumors, with 272 cases reported in the literature in English. Less than 10 per cent of these are located in the gastrointestinal tract. This report documenls the first prima~y plasmacytoma of the omentum in association with'a recurrent colonic adenocarcinoma. Extramedullaly plasmacytoma, a B cell neoplasm, characteristically arises in areas containing lymphoid tissue. In our case the tumor most likely arose in a lymph node or nodes in the omental fat, with subsequent replacement of the entire greater omentum and involvement of the colonic serosa by direct exteasion. Although the extent and ~tature of the association between myloma and carcinoma remain obscure, a review of the literature suggests that such association may occur more frequently than has been supposed. Further investigation u,ould appear to be warranted. Primary extramedttllary plasmacytoma is a rare tumor, 272 cases lmving been r e p o r t e d in the literatnre in English? T h e y most commonly invoh'e the head and neck regions where they account for about 90 p e r cent o f the r e p o r t e d cases. Less titan 10 p e r cent o f these tumors are located in the gastrointestinal tract; the stomach and small intestine are nearly eqnally affected a n d the colorectum is less frequently involved, z It is tim p u r p o s e o f tiffs article to report a unique case o f p r i m a r y extramedttllary plasmacytoma o f the o m e n t u m associated with a r e c u r r e n t adenocarcinoma o f left colon. T o o u r knowledge this is tire first such r e c o r d e d case.

CASE REPORT A 58 )'ear old N e g r o male was admittcd to Rahway Hospital on Marcia 6, 1976, because o f episodes o f diarrhea and tar D- stools. A barium enema revealed a fiat defect in the sigmoid colon, a biopsy specimen o f which showed aclenocarcinoma. T h e leukocyte cotmt was 4400 pet- cu. rant. with a differential count o f 68 p o l y m o r p h o nuclear leukocytes, 2 b a n d forms, 26 lymphocytes, 2 monocytes, 1 eosinophil, a n d I basoplfil. T h e hemoglobin level was 7.5 gm. p e r 100 ml. and the hematocrit, 23.2 per cent. T h e total serum protein concentration was 15 gin. p e r 100 ml. with a g a m m a globnlin level o f 12.7 gin. per 100 nil. Serttm electrophoresis revealed a lfigh 110mogenous spike in tim g a m m a globulin zone identified by immunoelectrophoresis as IgG kappa. A Bence Jones protein determination was negative. A bone scan, liver scan, and x-ray examination o f the skull and ribs were all negative. Bone m a r r o w aspiration in tim sternal region and a hiopsy p e r f o r m e d in the right posterior iliac crest revealed no evidence o f plasma cell ntyeloma. Surgical exploration revealed nodules o f fleshy tumor-like tissne involving the peritoneal cavity with replacement o f the g r e a t e r ontentum (Fig. 1). Frozen section examination revealed plasmacytoma. Because o f tim infihration o f the peritoneal cavity by myeloma cells, which ntade snrgery difficult, a n d since obstrnction was not present, it was d e c i d e d to do an omentectonty and postpone surgical resection o f the colonic carcinoma until after therapy for the ntyeloma. Microscopic exanfination of the ontentnm revealed it to be entirely replaced by sheets o f plasma cells varying moderately in size and sltape (Fig. 2). T h e patient was treated with Adriamycin (50 rag. intravenously), Alkeran (2 mg. four times daily for five days), and p r e d n i s o n e (10 mg. four times daily for five days) and made fairly g o o d progress. A tltird bone ntarrow aspiration and biopsy o f the left posterior iliac crest revealed no evidence o f plasma cell myeloma. On July 28, 1976, the patient was readmitted to Rahway Hospital where the sigmoid colon was resected with an e n d to e n d anastomosis. During snrgery it was noted that a h h o u g h tim plasmacytoma was mnch improved, there was still evidence o f its presence within the perito-

Accepted for publication November 28, 1978. *Clinical Assistant l'rofessor of Pathology, New York University School of Medicine, New York, New York. Director of Laboratories, Rahway Hospital, Rahway, New Je'rse).. tAssistaut Professor of Pathology, Mr. Sinai School of Medicine, New York, New York. Co-director of Laboratories, Rahway Hospital, Rahwa)', New jersey. ~-Attending Surgeon, Rahway Hospital, Rahway, New Jersey. w

Surgeon, Rahway Hospital, Rahway, New Jersey.

Figure 1. Greater omentnln totally replaced by plasmacytoma. Note diffuseness of tumor replacement.

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H U M A N I ' A T H O I , O G Y - - V O L U M E 11, NUMBER 4 July 1980

Figure 2. Omental plasmacytoma. Note uniformity of m)eloma cells with a few hyperchromatic nuclei. (llematoxylin and eosin stain, x 200.)

neal cavity. Microscopic examination revealed that the adenocarcinoma had invaded the muscularis and serosal adipose tissue. Large sheets of myeloma cells in the colon infiltrated the serosal adipose tissue and mtiscularis externa and were in close proximity to the invading adenocarcinoma cells..A few regional lymph nodes revealed focal infihration by myeloma but not by the adenocarcinoma. Tim patient was given additional chemmherapy (AIkeran and prednisone) and returned to work. He did well until March 3, 1978, when he developed rectal bleeding; a second primary adenocarcinoma of the colon was encountered at a point above the previous anastonmsis. T h e hemoglobin level was 9.5 gin. per 100 ml., hematocrit 28.1 per cent, and total serum protein level 11.8 gin. per 100 ml. T h e resected tumor was a typical primary adenocarcinoma, measuring 3 by 3.5 by 1.5 cm. Microscopically it was deeply invasive with diffnse perinenral lymphatic permeation. Portions o f tile serosal adipose tissue and muscularis were infiltrated by sheets o f myeloma cells in close proximity to the invading adenocarcinoma. T h e patient, at tim present time, is continuing to take chemotlierapy (Alkeran and prednisone) and shows clinical and radiological evidence o f abdominal spread of the colonic adenocarcinotna. A fourth bone marrow examination revealed no evidence of plasma cell myeloma. DISCUSSION T h e term extramedullary plasmacytonm applies to plasma cell tumors that present in an extl,'amedullary site. Extramedullary plasmacytomas are usually located in the nasoplmrynx, upper respiratory tract, lamina propria of tlmgastrointestinal tract, and lymph nodes. Histologically they show the same pattern as other plasma cell tumors. These tumors can arise in almost any organ, and in Wiltshaw's series they were present in the vagina, breast, pancreas, and parotid as well as the thyroid and testis. ~ O u r patient was studied by skeletal roentgenograms, serum and urine protein determinations, several bone marrow aspirations and biopsies done at different sites and intervals, and peroxidase staining of the formaldehyde fixed, paraffin embedded tissue, using antisera to h u m a n gamma, k a p p a , and lambda chains, tim latter showing an occasional cell with positive staining with the

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gamma and kappa antisera. Nielsen et al. a have stated, however, that fixation with formaldehyde often depresses tile antigenicity o f tile immunoglobulins, and that unless tile)' are present in large amonnts, they would not be discernible. The histopathologic diagnosis in o u r case was primary pl;ismacytoma o f the omentum, with direct extension into the colon and focal involvement of the regional l)'mpll nodes. One could argue tlmt the plasmacytoma rather than being primary in the o m e n t u m had arisen from the sigmoid colon. The sigmoid, however, was involved only peripherally and had an intact mucosa and lamina propria. Tim recurrent colonic adenocarcinoma was clearly primary rather than metastatic, since one could clearly delineate its origin from adjacent benign colonic mucosa. T h e histogenesis o f the plasma cell is controversial. The bulk o1 evidence favors the view that plasma cells are ultimately derived fi'om Iymphocytes. Stages o f transforntation from lymplmcyte to plasma cell have been documented. 4 Therefore, plasmacytoma could be primary in lymph nodes, l'rimary origin in l)'mpll nodes, althougll rare, has been described, s" ~' In our case the tumor most likely also arose in a lympla node or nodes in the omental fat, with subsequent replacement of the entire greater omentum and involvement of the colonic serosa by direct extension. T h e occurrence o f M type gamma globulin abnormalities in association with various neoplasms other than those of tim reticuloendothelial syslem is well documented. In a review o f gamma globulin structure and function, Osserman and Takatsuki 7 cite 31 patients witli monoclonal gannnopathies o f unknown etiology associated with an epithelial neoplasm. Hallen 8 found 37 subjects in whom serum M components were ln'esent in association with nonreticular malignant tumors. T h e literature contains fewer reports of cases in which overt nffeloma and nonreticular neoplasia arc well established. According to I.ynch and Joske 9 there is an associated carcinoma in over 5 per cent of the patients with paraproteinemia, but these patients rarely show evidence o f myelomatosis. On the other band, Weitzel, TM in a review of patients with malignant plasma cell disorders coming to autopsy during the period 1930 to 1956, found that coexistent carcinohaa was present in 11 of 57 cases

EDITORIAl. (19.3 p e r cent). More recently Hosle) 'n studied 14 cancer patients with serum globtdin abnormalities on electrophorcsis for the presence o f muhiple ntyelonm; five of these had osteolytic bone lesions and a p p e a r e d to have coincidental xnycloma. T h e relationship, if any, between plasina cell dyscras i a s - - malignant o r o t h e r w i s e - - and p r i m a r y malignant epltlmlial cell tumors is unknown; however, observations by several investigators raise the possibility that such an occurrence is more titan coincidental, particularly in rectosigmoid, oroplmryngeal, breast, and biliary tract carcinomas. In the autopsy survey by WeitzeP ~ the incidence o f coexistent carcinoma in patients with malignant plasma cell disorders was considerably Ifigher (19.3 per cent) than the incidence o f carcinoma associated with lymphoma o r l e u k e m i a (7.96 per cent). Some observers have suggested that a benign plasma cell o r lymphocyte proliferation may be a p a r t of a response to a tumor3, tt A n o t h e r postulate is tlmt cancer and m)'cloma ill some patients might have a commou etiology, such as a genetic defect or a c o m m o n carcino g e n . " Although the extent and nature o f the association between myeloma and carcinoma remain obscure, a review o f the literature suggests that such an association nmy occur more frequently than has previously been supposed. F u r t h e r investigation would a p p e a r to be warranted.

Acknowledgments We are indebted to Dr. Frederick Miller for the imm u n o p e r o x i d a s e staining aud to Martha Bradley, Susan Benisch, a n d Karen Peison for their tcclmical assistance. References I. Wihshaw, E.: The natural history of extramcdullar)' plasnmcytoma and its relation to solitary myeloma of bone and myelomatosis. Medicine, 55:217, 1976. 2. Sharma, K. D., and Shrivastar, J. D.: Extramedullary plasmacytoma of gastrointestinal tract. Arch. Path., 71:229, 1961. 3. Nielsen, S. M., Schenken, J. R., and Cawley, L. P.: Primary colonic plasmacytoma. Cancer, 30:261, 1972. 4. Roberts, J. C.: Role of the lymphocyte in antibody formation. In Rebuck, J. W. (Editor): T h e Lynq)hoc)te and Lymphoc}tic Tissue. New York, Paul B. lloeber, 1960, p. 82. 5. Nelson, M. G., and Lyons, A. B.: Plasmacytoma of lymph glands. Cancer, 10:1275, 1957. 6. Simon, M. A., and Eidlow, S.: Plasmacytoma of lymph node; report of case with multiple myeloma. New Engl. J. Med., 243:335, 1950. 7. Osserman, E. F., and Takatsuki, K.: Plasma cell myeloma; gamma globulin synthesis and structure. Medicine, 42:357, 1963. 8. ilallen, J.: Discrete gammaglobulin (M-) components in serum. Clinical stud}' of 150 sul~ects without m)elomatosis. Acta Med. Scand., 462 (Suppl.):16, 1966. 9. Lynch, IV. J., and Joske, R. A.: The occurrence of abnormal serum proteins in patients with epithelial neoplasma. J. Clin. Pathol., 19:461, 1966. 10. Weitzcl, R. A.: C21rcinoma coexistem with malignant disorders of plasnm cells. Cancer, 11:5t6, 1958. 1I. ttosley, 1t. F.: M-proteins, plasnmcytosis and cancer. Cancer, 20:295, 1967.

Editorial

HUMAN P A T H O L O G Y - T H E FIRST DECADE T h e first issue o f Human PatholoD' a p p e a r e d in March 1970. For some time prior to this, a dedicated g r o u p o f five p a t h o l o g i s t s - S t a n l e y Robbins, Gns Dammin, Whitey T h u r l b e c k , Bob Scull)', and the late Cesare T e d e s c l f i - h a d d e v e l o p e d the idea o f a n e w j o t t r n a l dedicated to the needs o f the practicing pathologist. Fortunately the W. B. S a u n d e r s Coral)any agreed with the concept and decided to move ahead. T h e original fountlers o f the j o u r n a l worked as an Editorial Committee with Ccsare Tedeschi as its secretary. An Editorial Board was recruited and tire j o u r n a l was launched on an uncertain f l m n e . T h e Sa.unders Company and tim Editorial Committee decided that thtman Pathol%9' would a p p e a r six times a year. By the e n d of 1971, it was a p p a r e n t that the j o u r n a l had a modest n u m b e r o f subscribers and the decision was m a d e to continue. At a meeting o f the Editorial Committee in March 1971, I was elected Editor a n d the Committee m e m b e r s became Associate Editors. We felt that if we could survive for five )'ears, we would probably xnake it as a responsible publication. Over tim )'cars it Ires been In)" p r M l e g c to work with an outstanding Editorial Board, all o f whom lmvc given unselfishly o f their time and expertise. T h e publishers have been equally supportive, especially in the cooperation o f Kendall *lcNally. As the j o u r n a l grew so did the dcnmnds for space, m o r e pages, and more sections. Each ) c a r the total nuinber

o f papers submitted increased, and delay in publication became a critical issue. T h e entire Editorial Board was committed to maintaining a n d e x p a n d i n g Human Pathol%% After considerable discussion and planning the format was changed in J a n u a r y 1980 to a larger page and better type. In addition, advertising began to a p p e a r for tim first time. It is a pleasure for me to announce to o u r loyal readers that a special s u p p l e m e n t n u m b e r will a p p e a r tiffs fall. But most important, b e g i n n i n g in J a n u a r y 1981, Human Patholo~" will be published monthly. In 1972 a p p r o x i m a t e l y 3000 copies o f each issue were published. T o d a y 10,500 copies o f each issue arc published and distributed on a world-wide basis. As the Editorial Board and the W. B. S a u n d e r s Company p r e p a r e for 1981, I can assttre you that o u r dedication to the p u r p o s e o f the j o u r n a l remains firm. W e will increase the n u m b e r o f symlmsium issues a n d e x p a n d existing sections. New and innovative sections will be a d d e d as the need arises. We look forward to the second decade o f Human Patholo,.U confident o f its future success. As always, this success d e p e n d s on the r e a d e r and we expect to h e a r from you. C o r r e s p o n d e n c e is most welcome and tire j o u r n a r s varied format will a t t e m p t to touch on the most intportant areas necessary for tim c o n t e m p o r a r y practice o f pathology. As a nmnthly publication Human Pathology will significantly increase its ability to provide its readers with the highest quality o f usefid information. BERNARD h i . WAGNER

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