Malignant Appendiceal Carcinoid with Intussusception of the Base Manifesting as a Cecal Tumor: Report

of a Case*

PER SKAANE, M.D., TOR JACOB EIDE, M . D .

From the Department of Surgery, Harstad Hospital, and the Institute o[ Medical Biology, University o] Tromsr Norway

CARCINOID TUMOR Of the vermiform appendix is a relatively rare entity, found in

about 0.3 per cent of all surgically removed appendices, s Appendiceal carcinoids usually a p p e a r as small nodular or bulbar swellings of the tip, in most cases not exceeding 1.5 cm in diameter.S, x0 T h e r e are no specific signs or symptoms to suggest the presence of an appendiceal carcinoid. Occasionally, the preoperative diagnosis is appendicitis, but most of the lesions are discovered incidentally in appendices removed during other abdominal operations, s, 10 Intussusception of the vermiform appendix is also uncommon. Simple appendiceal intussusceptions have been classified according to McSwain into five types, s, 7 Intussusception of the base of the appendix into the cecum, the cecum being the intussuscipiens, is numerically the most common type. 1 In these cases, i.e., an "inversion" of the appendix, the appendix retains its form and is only dragged into the cecum. T h i s report describes an extremely rare combination of intussusception of the proximal a p p e n d i x with a large malignant carcinoid. T h e lesion was preoperatively diagnosed as a large polypoid t u m o r of the cecum. * Read at the Annual meeting of the Norwegian Surgical Association, Oslo, October 21 to 23, 1976. Address reprint requests to Dr. Skaane: Harstad Sykehus, Kirurgisk Avdeling, 9400 Harstad, Norway.

R e p o r t of a Case A 42-year-old woman was admitted to the hospital on July 29, 1975, with the diagnosis of a polypoid tumor of the cecum. Past history included typhoid fever, iridocyclitis, papilloma of the bladder, and pleuritis. In 1968, radical hysterectomy a n d lymphadenectomy, after preoperative radium, irradiation, were performed because of cervical cancer. There was no spread of the tumor, and the postoperative course was uncomplicated. Later, however, chronic recurrent cystitis and stenosis of the urethra developed. The patient had been referred to our outpatient department in August 1974 because of urethral stricture and pyuria. Since February 1974, she had noticed abdominal discomfort and recurrent attacks of pain in the right lower quadrant. At that time, being a sailor, she had consulted different general practitioners, but no thorough investigation had been performed. On a routine visit occasioned by her urethral stricture in our outpatient department on July 9, 1975, she mentioned having frequent pains in the right lower quadrant. On physical examination a small, tender tumor could be palpated in the right lower quadrant. Three examinations for occult blood in the feces were negative. Barium-enema examination disclosed a large polypoid tumor approximately 3.5 cm in diameter (Fig. 1). The tumor was situated in the medial part of the cecum below the ileocecal valve, and had a smooth, regular surface (Fig. 2). On admission, physical examination disclosed no abnormality except for the above-mentioned tumor in the right lower quadrant. Blood pressure, pulse rate and temperature were normal. Hemoglobin was 13.2 g/100 ml, leukocyte count 6,200/cu ram. After the usual preoperative bowel preparation, laparotomy was performed on July 31, 1975. T h e appendix was found to be firm, thickened, and enlarged throughout its length. T h e color was greyishwhite, with yellowish areas at the proximal part. An enlarged firm lymph node, approximately 1.5 cm in diameter, was seen in the mesoappendix

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intussuscepted base could stiI1 be seen projecting into the cecum (Fig. 4). T h e length of the appendix ~as 6.5 o n . T h e appendiceal lumen was obliterated in its distal part. Microscopically, the wall of the vermiform ap-

FIg. 1. Barium-enema examination, showing a large, polypoid tumor in the cecum, approximately 3.5 by S.5 cm. Fro. 3. Surgical specimen, demonstrating the palpated polypoid tumor, p r o t r u d i n g approximately 4 cm into lumen from the base of the appendix, the top indicated by a line. This t u m o r proved to be an intussusception of the proximal appendix. I n the mesoappendix, the enlarged l y m p h node can be seen (arrow).

F:g. 2. Barium-filled coton with intracecal welldefined filling defect inferior to the ileocecal valve. (Fig. 3). T h e rest of the bowel and abdominal o r g a n were normal. As the enlarged lymph node in the mesoappendix was assumed to represent metastasis, a malignant tumor of the appendix was suspected, and primary right hemicolectomy with an end-to-end ileotransversostomy was performed. Before the cecum was opened, a smooth polypoid tumor protruding from the base of the a p p e n d i x about 4 cm into the cecal cavity was palpated (Fig. 3). On opening the cecum, the size of this tumor was markedly reduced by manipulation, b u t the

Flc. 4. T h e opened surgical specimen, showing superiorly the inverted base of the a p p e n d i x still projecting into the cecal lumen. T h e ileocecal valve ties inferiorly.

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Fro. 5. The appendiceal wall, with solid aggregates of tumor tissue in the mucosal membrane with infiltration deep in the muscle coat (hematoxylin and eosin; • 45).

per~dix was infiltrated by epithelial tumor cells, mainly arranged in solid nests, hut also with areas of trabecular pattern (Fig. 5). The cells were fairly uniform, with an ovoid shape and round ,mclei, aqd contained both argentaffin and argyrophil ga'anules in the cytoplasm, as demonstrated by the Fontana-Masson and Bodian methods. The lymph node in the mesoappendix and one of two ileocecal lymph nodes embedded for histologic examination contained nests of tumor cells with the same appearance as cells in the primary tumor. T h u s , histologic and histochemicat examination confirmed the diagnosis of an enterochromaffin carcinoid tumor of the appendix with regional lymph-node metastases. A year postoperatively, the patient had no evidence of recurrent or metastatic disease.

Discussion C a r c i n o i d t u m o r of the v e r m i f o r m a p p e n d i x m a y show local i n v a s i o n histologically, b u t it is g e n e r a l l y b e n i g n i n its course, the f r e q u e n c y of metastases b e i n g o n l y a b o u t 2 per cent.8 T h e o n l y m o r p h o l o g i c feature

indicating malignancy in carcinoid tumors is the size of the t u m o r . T h e i n c i d e n c e of metastasis is very low for t u m o r s less t h a n 2 cm i n diameter, i n contrast to the h i g h e r i n c i d e n c e a m o n g the rare t u m o r s larger t h a n 2 cm. M o e r t e t et al., i n their study of 144 cases of a p p e n d i c e a l carcinoids, f o u n d o n l y three t u m o r s m o r e t h a n 2 cm i n d i a m eter, a n d o n l y two of these (1.4 per cent) h a d p r o v e d metastasis. T h e i r review lists o n l y 35 w e l l - d o c u m e n t e d a p p e n d i c e a l carcinoids t h a t have metastasized.S T h e s y m p t o m a t o l o g y of a p p e n d i c e a l intussusception may be d i v i d e d i n t o four patterns of clinical p r e s e n t a t i o n : acute a p p e n dicitis, i n t e s t i n a l i n t u s s u s c e p t i o n , c h r o n i c i n t e r m i t t e n t c r a m p y p a i n i n the r i g h t lower q u a d r a n t , a n d n o symptoms.3 T h e radiog r a p h i c a p p e a r a n c e of a p p e n d i c e a l intussusception is that of a n oval, r o u n d or

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fingerlike filling defect in the m e d i a l w a l l of the cecum, a n d no v i s u a l i z a t i o n of the a p p e n d i x . I . 6 W h e n the a p p e n d i x is partially i n t u s s u s c e p t e d it is s o m e t i m e s possible to r e d u c e it b y s i m p l e m a n i p u l a t i o n , as in o u r case, or b y c o m p r e s s i n g the cecum. 2, 6 Even b a r i u m - e n e m a e x a m i n a t i o n m a y reduce the a p p e n d i c e a l i n t u s s u s c e p t i o n , as r e c e n t l y r e p o r t e d by B a c h m a n a n d C l e m e t t . I C a r e f u l r e v i e w Of the l i t e r a t u r e reveals o n l y three w e l l - d o c u m e n t e d cases of a p p e n diceal c a r c i n o i d c a u s i n g i n t u s s u s c e p t i o n of the a p p e n d i x . H i c k i n b o t h a m , in 1951, p u b lished the first case, t h a t of a s m a l l c a r c i n o i d t u m o r at the t i p of a c o m p l e t e l y intussuspected a p p e n d i x . 4 P e a r l m a n a n d Srinivasan r e p o r t e d a case of i n v e r s i o n of t h e a p p e n d i x caused by a small c a r c i n o i d tumor.9 I n e i t h e r case a well-defined, p o l y p o i d filling defect was seen o n b a r i u m - e n e m a e x a m i n a t i o n . P u r i t a r e p o r t e d a case of a n a p p e n d i c e a l c a r c i n o i d associated w i t h intussusception i n c i d e n t a l l y f o u n d d u r i n g a n a p p e n d e c t o m y , u I n a d d i t i o n to these t h r e e cases, K i e r a l d o et al. briefly m e n t i o n e d a case m a n i f e s t i n g as acute a p p e n d i c i t i s in w h i c h a g a n g r e n o u s a n d i n t u s s u s c e p t e d app e n d i x was r e m o v e d a n d h i s t o l o g i c examin a t i o n r e v e a l e d a c a r c i n o i d t u m o r of the tip.S O u r p a t i e n t c o m p l a i n e d of r e c u r r e n t p a i n in the r i g h t lower q u a d r a n t for m o r e t h a n 18 m o n t h s . I t is i m p o s s i b l e to say w h e t h e r the t u m o r itself o r a n intussusception of i o n g s t a n d i n g caused h e r symptoms. T h e e x t r a o r d i n a r y size of this t u m o r , inv a d i n g the 6.5 cm l o n g a p p e n d i x t h r o u g h o u t its length, is r e m a r k a b l e . T h e t u m o r o u s a p p e n d i x , in a d d i t i o n to l y m p h - n o d e metastasis in the m e s o a p p e n d i x , i n d i c a t e d a m a l i g n a n c y of the a p p e n d i x . R i g h t hemicolectomy c o u l d t h e r e f o r e be c a r r i e d o u t as a p r i m a r y o p e r a t i o n , this p r o c e d u r e b e i n g r e c o m m e n d e d by most surgeons for the

e x c e e d i n g l y rare a p p e n d i c e a l c a r c i n o i d tum o r s m o r e t h a n 2 cm i n d i a m e t e r , o r w h e n l y m p h - n o d e metastasis is present.S. 10 Summary

A case of a n a p p e n d i c e a l c a r c i n o i d t u m o r w i t h r e g i o n a l l y m p h - n o d e m e t a s t a s e s is presented. T h e size of the t u m o r was rem a r k a b l e , as the a p p e n d i x ,,'as i n f i l t r a t e d t h r o u g h o u t its length. T h e p r o x i m a l p a r t of the a p p e n d i x was i n t u s s u s c e p t e d i n t o the cecum. P r e o p e r a t i v e l y , t h e lesion was diagnosed as a p o l y p o i d t u m o r of the cecum. A p r i m a r y r i g h t h e m i c o l e c t o m y was c a r r i e d OUt.

References 1. B a c h m a n AL, C l e m e t t A R : R o e n t g e n aspects of

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primary appendiceal intussusception. Radiology 101: 531, 1971 Day WA: Appendiceal intussusception simulating cecal tumor: Case report. Dis Colon Rectum 6:118, 1963 Fink VH, Santos AL, Goldberg SL: Intussusception of the appendix: Case reports and reviews of the literature. Am J Gastroenterol 42: 431, 1964 Hickinbotham P: Inversion of the appendix. Br J Surg 39: 84, i951 Kieraldo J, Eversole S, Allen R: Carcinoid tumor of the vermiform appendix with distant metastasis: A review of the literature and report of two cases, one in a 14-year-old girl. Calif Med 99: 161, 1963. Kloppedal EA, Toledo-Pereyra LH: Intussusception of the base of the appendix. Minn Med 55: 1139, 1972 McSwain B: Intussception of the appendix: Review of the literature and report of a case. South Med J. 34: 263, 1941 Moertel CG, Dockerty MB, Judd ES: Carcinoid tumors of the vermiform appendix. Cancer 21: 270, 1968 Pearlman DM, Srinivasan K: Malignant carcinoid of appendix: Metastasis from small primary tumor which appeared as appendiceal intussusception. NY State J Med 71: 1529, 1971 Ponka JL: Carcinoid tumors of the appendix: Report of thirty-five cases. Am J Surg 126: 77, 1973 Purita F: Intussuscepcao do At~ndice Ileocecal corn Carcindide. Rev Bras Cir 42: 85, 1961

Malignant appendiceal carcinoid with intussusception of the base manifesting as a cecal tumor: report of a case.

Malignant Appendiceal Carcinoid with Intussusception of the Base Manifesting as a Cecal Tumor: Report of a Case* PER SKAANE, M.D., TOR JACOB EIDE, M...
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