Primary Malignant Tumors of the Small Intestine*

T. COUTSOFTIDES,M.D.,? H. R. SHIBATA,M.D.

PRIMARY MALIGNANTTUMORS o f the small intestine constitute only a p p r o x i m a t e l y 0.3 per cent o f all n e o p l a s m s ( N C I T h i r d N a t i o n a l C a n c e r Survey). N u m e r o u s d e s c r i p t i o n s o f this e n t i t y h a v e appeared.,, 2, 6, t0 T h e aim o f this report was to review o u r experience with these t u m o r s a n d to try to c o r r e l a t e t h e i r pathologic aspects (risk factors) with survival.

From the Department of Surgery, Royal Victoria Hospital, Montreal, Canada TABLE l. Presenting Symptoms and Signs in Patients with MaligvLant Tumors of the Small Intestine Per Cent of Patients

Symptoms

Materials a n d M e t h o d s

Pain Nausea, vomiting Weight loss Constipation Diarrhea Small-bowel obstruction Gastrointestinal-tract bleeding Perforation

T h e records o f more than 100 patients at the Royal Victoria H o s p i t a l who were d i a g n o s e d as h a v i n g malignant t u m o r s o f the small intestine between 1935 and 1973 were reviewed. Fifty-four patients were inc l u d e d in this review. T h e excluded patients either had lymphoproliferative disease that had spread bey o n d the paraintestinal nodes, m a k i n g it impossible to d e t e r m i n e the origin of the tumor, or had metastases o f the small intestine from a n o t h e r primary site. Also, p a t i e n t s who h a d lesions o f the a m p u l l a r y a n d p e r i a m p u l l a r y regions of the d u o d e n u m were excluded f r o m this study. Follow-up data were available for all cases t h r o u g h the Central T u m o r Registry o f the hospital. Results o f p o s t m o r t e m studies were available for 85 per cent o f the patients who died.

63 44 44 22 20 9 9 5

TABLE 2. Radiologic Findings Per Cent of Patients Small-intestinal obstruction Positive upper gastrointestinal-tract series Negative upper gastrointestinal-tract series Free air X-rays not taken

28 21 17 6 24

Results Results of physical examinations and laboratory investigations generally did not help to make a diagnosis, except in five cases where a mass was palpable. Table 2 shows the results o f radiologic studies. Fifty-one patients (95 per cent) u n d e r w e n t operations, and in three patients the diagnosis was made only at p o s t m o r t e m examination. In 80 per cent o f the cases, the operation was u n d e r t a k e n with intent to cure, while in eight patients extensive disease f o u n d at laparotomy precluded an operation for cure. Eleven patients died d u r i n g the initial hospitalization, resuiting in a mortality rate o f 21.5 per cent. T h e overall five-year survival rate for the 40 patients who left the hospital was 32 per cent. Most o f the patients (88 per cent) who died did so within two years o f diagnosis.

T h e r e were 22 men (40 per cent) and 32 w o m e n (60 per cent). Eighty-seven per cent o f the patients were m o r e than 41 years o f age, and 65 per cent were m o r e than 51 years o f age, indicating that o l d e r people are more p r o n e to this disease. Durations o f s y m p t o m s f r o m onset to diagnosis varied, r a n g i n g f r o m a few hours to 30 months. Table 1 shows the p r e s e n t i n g symptoms and signs. * Received for publication August 21,'1978. Address reprint requests to Dr. Shibata: Department of Surgery, Royal Victoria Hospital, 687 Pine Avenue West, Montreal, P. Q., Canada. t Current address: Department of Surgery, University of California, Irvine, and Long Beach Veterans Administration Hospital, Long Beach, California.

0012-3706/79/0100/0024/$00.65 9 American Society of Colon and Rectal Surgeons

24

V o l u m e 22 Number 1

25

SMALL-INTESTINAL PRIMARY TUMORS TABLE 3. Tumor Locations Found at Operation Location Duodenum

Adenocarcinoma Carcinoid Lymphosarcoma Reticulum-cell sarcoma Leiomyosarcoma Cell tumor Anaplastic "tOTAL

Jejunum

IIeum

Total

6 0 0 0 i 1 2

9 0 3 1 1 0 I

11 9 5 2 1 0 1

26 9 8 3 3 1 4

10

15

29

54

T h e locations a n d histologic features o f t u m o r s as d e t e r m i n e d at l a p a r o t o m y or post m o r t e m are shown in T a b l e 3. T h e correlation between location o f t u m o r and five-year survival rate is shown in T a b l e 4.

Discussion In o u r hospital, the incidence o f p r i m a r y m a l i g n a n t neoplasms of the small intestine over a 20-year p e r i o d was 2.5 per cent o f all intestinal neoplasms. But a large p e r c e n t a g e o f t h e m are malignant, 5'~"10 a n d they tend to p r o d u c e s y m p t o m s r a t h e r late in their course, leading to late diagnosis a n d a c o r r e s p o n d i n g l y low survival rate. T h e majority of patients in o u r series were w o m e n (60 per cent) a n d were m o r e than 40 years old (87 per cent); these figures are in a g r e e m e n t with those in previous reports, a's'8 T h e periods f r o m onset of s y m p t o m s to diagnosis varied, r a n g i n g f r o m a few hours to 30 months. All but three patients h a d a n t e c e d e n t nonspecific gastrointestinal complaints, the most c o m m o n o f which were pain, nausea a n d vomiting, and weight loss. When first seen, a p p r o x i m a t e l y a third o f the patients had small-intestinal obstruction which, at l a p a r o t o m y , was f o u n d to be d u e to the tumor. This high incidence o f small-intestinal obstruction was also f o u n d by o t h e r a u t h o r s ? '5'7 Gastrointestinal b l e e d i n g a n d free p e r f o r a t i o n were seen in 9 per cent a n d 5 per cent of patients, respectively. Results o f physical examinations and l a b o r a t o r y investigations generally p r o v i d e d no clue to the diagnosis. Radiologic e x a m i n a t i o n s d e m o n s t r a t e d the lesion in 50 per cent o f patients who u n d e r w e n t u p p e r gastrointestinal-tract series. In the two cases in which patients u n d e r w e n t a n g i o g r a p h i c examinations, the results were positive. T h e use o f a n g i o g r a p h y m u s t be c o n s i d e r e d w h e n small-intestinal t u m o r s are suspected. We cannot r e c o m m e n d using c o m p u t e r i z e d

axial t o m o g r a p h y or u l t r a s o n o g r a p h y without f u r t h e r experience. T h e general principle at l a p a r o t o m y was to r e m o v e the lesion with an a d e q u a t e m a r g i n o f n o r m a l intestine on b o t h sides, as well as any n o d e - b e a r i n g tissue. W h e n distal ileal lesions were present, this was easily accomplished by right colectomy with ileocolic anastomosis. For lesions o f the j e j u n u m and p r o x i m a l ileum, a m o r e limited resection was carried out because o f the a n a t o m i c p r o x i m i t y o f the s u p e r i o r mesenteric vessels. Lesions o f the d u o d e n u m that app e a r e d curable were treated by p a n c r e a t i c o d u o d e n e c tomy. Evidence of extensive disease was found intraoperatively in eight patients, and thus only a palliative bypass p r o c e d u r e was carried out. In analyzing o u r results, a l t h o u g h the n u m b e r s of patients in each g r o u p are small, the following factors a p p e a r e d to be significant in d e t e r m i n i n g the prognosis of patients that survived the o p e r a t i o n . Location of Lesion: T h e best five-year survival rate was obtained in patients with lesions o f the jejun u m (50 per cent) a n d the worst in patients with lesions o f the d u o d e n u m (28 per cent). Lesions in the ileum were associated with a 42 per cent five-year survival rate (Table 4). Similar results have b e e n rep o r t e d by o t h e r investigators3' 7 Histology of Lesion: T h e best five-year survival r a t e , 67 p e r c e n t , was s e e n in p a t i e n t s w i t h TABLE 4.

Location Duodenum Jejunum Ileum

Location,'of Tumors and Five-Year Survival Rates

Five-Year Survival Rate (Per Cent) 28 50 42

26

Dis. CoL & Rect. Jan.-Feb. 1979

COUTSOFTIDES AND SHIBATA TABLE 5. Types of Tumors and Fize-year Survival Rates

Type

Number of Patients

Five-year Survival Rate (Per Cent)

3 21

67 32

26 3 1

50 0 0

Leiomyosarcoma Adenocarcinoma Carcinoid, lymphoma, and anaplastic tumor Reticulum sarcoma Others

TABLE 6. Staging of Tumors and Five-year Survival Rates

Location

Number of Patients

Five-year Survival Rate (Per Cent)

17 9 9 6

71 44 14 0

Confined to bowel wall Extends to mesentery Positive lymph nodes Distant metastases

TABLE 7. Differentiation of CeUs and Five-year Survival Rates

Well or moderately well differentiated Poorly differentiated

Number of Patients

Five-year Survival Rate (Per Cent)

23

56

8

12

m e s e n t e r y was 44 p e r c e n t ; t h a t f o r n i n e p a t i e n t s w h o h a d m e t a s t a s e s to l y m p h n o d e s was 14 p e r c e n t ; n o n e o f t h e six p a t i e n t s w h o h a d d i s t a n t m e t a s t a s e s s u r vived five y e a r s . S i m i l a r o b s e r v a t i o n s h a v e b e e n m a d e by other investigators/ Differentiation: T h e p a t h o l o g i s t g r a d e d t h e d e g r e e s o f d i f f e r e n t i a t i o n o f l e s i o n s f r o m 31 p a t i e n t s . F o r 23 p a t i e n t s (74 p e r c e n t ) w h o s e t u m o r s w e r e well d i f f e r e n t i a t e d o r m o d e r a t e l y well d i f f e r e n t i a t e d , t h e f i v e - y e a r s u r v i v a l r a t e was 56 p e r cent. F o r e i g h t patients (26 p e r c e n t ) w h o s e t u m o r s w e r e p o o r l y d i f f e r e n t i a t e d , t h e f i v e - y e a r s u r v i v a l r a t e was o n l y t 2 p e r c e n t ( T a b l e 7). T h u s , t h e r e s u l t s o f this s t u d y d o n o t s u p p o r t a pessimistic outlook for patients who have smalli n t e s t i n a l n e o p l a s m s ? S u r v i v a l in this g r o u p o f p a tients d e p e n d s o n t h e f a c t o r s m e n t i o n e d a b o v e , a n d it is t h u s p o s s i b l e to i m p r o v e s u r v i v a l rates b y d i a g n o s i n g t h e s e lesions e a r l i e r . As t h e s e t u m o r s a r e o f t e n associated with vague gastrointestinal symptoms, diagnostic investigations, including upper gastrointestinal-tract series and angiography, should be undertaken early? Early diagnosis, while the t u m o r was still c o n f i n e d to t h e b o w e l wall, y i e l d e d 71 per cent five-year survival, but when the tumor had s p r e a d to the r e g i o n a l l y m p h n o d e s , t h e s u r v i v a l r a t e d r o p p e d to 14 p e r cent. W i t h t h e s e lesions, e a r l y d i a g n o s i s is i m p e r a t i v e , f o r t h e i r l o c a t i o n p r e c l u d e s w i d e e'n bloc e x c i s i o n .

References

l e i o m y o s a r c o m a . P a t i e n t s with a d e n o c a r c i n o m a , t h e most common tumor found, had a five-year survival r a t e o f 32 p e r cent. C a r c i n o i d s , a n a p l a s t i c c a r c i n o m a s , a n d l y m p h o s a r c o m a s w e r e a s s o c i a t e d with 50 p e r c e n t f i v e - y e a r survival, while no p a t i e n t with r e t i c u l u m - c e l l s a r c o m a s u r v i v e d five y e a r s ( T a b l e 5). S i m i l a r t r e n d s w e r e f o u n d by o t h e r i n v e s t i g a t o r s , 4-6'10 a l t h o u g h their absolute five-year survival rates differ from ours.

Extent o f S p r e a d o f T u m o r : It was p o s s i b l e to c o r relate five-year survival rates with the extent of s p r e a d o f t u m o r ( T a b l e 6). T h e f i v e - y e a r s u r v i v a l r a t e f o r 17 p a t i e n t s in w h o m t h e t u m o r s w e r e c o n f i n e d to t h e b o w e l wall was 71 p e t cent; t h a t f o r n i n e p a t i e n t s in w h o m t h e t u m o r s h a d i n v a d e d t h e s u r r o u n d i n g

1. Botsford TW, Crowe P, Crocker DW: Tumors of the small intestine: A review of experience with 115 cases including a report of a rare case of malignant hemangio-endothelioma. A m j Surg 103: 358, 1962 2. Brookes VS, Waterhouse JA, Powell DJ: Malignant lesions of the small intestine: A ten-year survey. Br J Surg 55: 405, 1968 3. Croom RD III, NewsomJF: Tumors of the small intestine. Am Surg 41: 160, 1975 4. DormanJE, Floyd CE, Cohn I Jr: Malignant neoplasms of the small bowel. AmJ Surg 113: 131, 1967 5. Ostermiller W, Joergenson EJ, Weibel L: A clinical review of tumors of the small bowel. A m J Surg 111: 403, 1966 6. Pagtalunan RJ, Mayo CW, Dockerty MB: Primary malignant tumors of the small intestine. AmJ Surg 108: 13, 1964 7. Reyes EL, Talley RW: Primary malignant tumors of the small intestine. Am J Gastroenterol 54: 30, 1970 8. Sethi G, Hardin CA: Primary malignant tumors of the small bowel. Arch Surg 98: 659, 1969 9. Silberman H, Crichlow RW, Cap!an HS: Neoplasms of the small bowel. Ann Surg 180: 157, 1974 10. Wilson JM, Melvin DB, Gray GF, et al: Primary malignancies of the small bowel: A report of 96 cases and review of the literature. Ann Surg 180: 175, 1974

Primary malignant tumors of the small intestine.

Primary Malignant Tumors of the Small Intestine* T. COUTSOFTIDES,M.D.,? H. R. SHIBATA,M.D. PRIMARY MALIGNANTTUMORS o f the small intestine constitut...
235KB Sizes 0 Downloads 0 Views