Unsuspected Perforated Adenocarcinoma of the Appendix as a Second Colonic Neoplasm: Report STEVEN BROZINSKY,
of a Case*
M.D., HOWARD GOLDSON, M.D.,
GEORGE STERGIOPOULOS, M . D . , SAUL
J.
GROSBERG, M . D .
From the Departments of Gastroenterology and Surgery, The Brooklyn Veterans Administration Hospital, and The Downstate Medical Center, Brooklyn, New Yorh
the appendix is not often suspected preoperatively, 2, 3, 9, 10 and has never been diagnosed prior to surgical exploration. A case of perforated adenocarcinoma of the appendix as a second primary neoplasm in a patient who had relatively few symptoms is presented. PRIMARY ADENOCARCINOMA Of
P~eport o f a C a s e A 78-year-old black man was admitted to the hospital complaining of vague right and left lower q u a d r a n t pain and constipation of two months' duration. T h e r e was no weight loss, hematemesis, or melena, nor was there any history of a previous surgical procedure or significant illness. Results of physical examination were normal, including persistently guaiac-negative stools. Laboratory data were: hemoglobin 11.4 g/100 ml, hematocrit 36 per cent, leukocyte count, 4,400, with a normal differential. Blood urea nitrogen, glucose, electrolytes, and SMA-12 values were normal, as was the chest x-ray. T h e EKG revealed complete right bundle-branch block. Barium-enema examination showed segmental narrowing of the midsigmoid colon with a 1.5 • 1.0-cm sessile polyp in the proximal sigmoid. Pathologic examination of material obtained by colonoscopic polypectomy of sessile polyps at 27 and 30 cm revealed villous adenoma. T h e cecum was visualized to be. normal. Repeat colonoscopic polypectomy of a more proximal polyp (35 cm) revealed infiltrating adenocarcinoma (Fig. 1). A repeat barium-enema examination, done in preparation for definitive sigmoidal resection, revealed only the previously observed segmental narrowing. * Received for publication July 19, 1976. Address reprin.t requests to Dr. Grosberg: Veterans Administration Hospital, 800 Poly Place, Brooklyn, New York 11209.
T h e abdomen was explored via a left paramedian incision. A large mass in the pelvis, adherent to two loops of small bowel and to the bladder, was found. Further dissection revealed what appeared to be an old perforated appendicitis. T h e appendix was about 2.5 cm in diameter. Its tip was lying in a large cavity adherent to the urinary bladder. A frozen section of the material within the appendiceal mass showed adenocarcinoma. T h e r e was no evidence of hepatic or peritoneal metastases. Based u p o n the possible diagnosis of adenocarcinoma of the a p p e n d i x and an infiltrating carcinoma of the sigmoid colon, subtotal colectomy with ileoproctostomy was performed. T h e final pathology report revealed an infiltrating carcinoma of the sigmoid colon at the base of the previous polypectomy site and a primary perforated adenocarcinoma, mucinous type, o f the a p p e n d i x (Fig. 2). T h e cecum was not involved. T h e patient did well postoperatively and is being followed as an outpatient.
Discussion Primary adenocarcinoma of the appendix is rare. In one large series it was found in 0.03-0.08 per cent of all appendices removed. 4 In a more recent study t0 of 101 tumors found in 8,699 surgically removed appendices, only two were adenocarcinomas. A review of medical literature 1, 2, 5, demonstrates that the diagnosis is not often suspected preoperatively, and has never actually been made before operation. Presumptive preoperative diagnoses include acute appendicitis, - appendiceal abscess, uterine fibroid, carcinoma of the cecum, ovarian tumor, and acute and chronic cholecystitis. In many reports, 3, t0 carcinoma of the appendix has been an incidental finding dur-
263 Dis. Col. & Rect. April, 1977
Volume 20 Number 3
264
BROZINSKY, ET AL.
Dis. Col. & Rect.
ApriI, 1977
Fie. 1. This focus of adenocarcinoma in close proximity to the base of the sigmoid polyp prompted the decision for a definitive operative procedure (hematoxylin and eosin; x 32) .
FIG. 2. Pyknotic nuclei, aberrant glandular structure, and mucin-containing cells (center field) of appendiceal carcinoma (hematoxylin and eosin; • 240).
Volume 20 Number 3
PERFORATED
APPENDICEAL
i n g a b d o m i n a l e x p l o r a t i o n for o t h e r pathologic conditions. Such was the case in o u r p a t i e n t w h o was o r i g i n a l l y s c h e d u l e d to u n d e r g o s e g m e n t a l s i g m o i d a l resection for an invasive c a r c i n o m a in a s i g m o i d a l polyp. M a n y a b n o r m a l - a p p e a r i n g a p p e n d i c e s are r e m o v e d w i t h o u t benefit of frozen section. T h i s is especially true when a n a p p e n d i c e a l abscess is present, l e a d i n g the s u r g e o n to believe t h a t a n o l d p e r f o r a t i o n can account for the diseased a p p e n d i x . Forsgren e t al. 6 c a u t i o n t h a t histologic e x a m i n a t i o n s of all a p p e n d i c e s s h o u l d be done, a n d t h a t the a p p e n d i x should be p a l p a t e d at every laparotomy. C a r c i n o m a of the a p p e n d i x occurs m o r e often in males, w i t h the highest i n c i d e n c e in the fifth decade of life2 I t is m o r e freq u e n t in the base t h a n in the tip a n d , because of a p o o r l y d e v e l o p e d m u s c u l a r coat, metastasizes r e a d i l y b y local invasion. I t also spreads via l y m p h a t i c a n d h e m a t o genous routes. T h e most c o m m o n sites of metastasis are the liver, p e r i t o n e u m , a n d lung. P e r f o r a t i o n of the c a r c i n o m a does n o t alter survival figures. 9 O u r p a t i e n t p r o b a b l y h a d an " o l d " p e r f o r a t i o n , b u t h a d no evidence of metastatic disease. T h e o p e r a t i v e p r o c e d u r e of choice is app e n d e c t o m y w i t h r i g h t hemicolectomy. T h i s increased the five-year survival r a t e b y 43 p e r cent over t h a t f o u n d w i t h a p p e n d e c t o m y alone in one series. 8 B a r i u m - e n e m a e x a m i n a t i o n of o u r pat i e n t d i d n o t suggest disease in the a p p e n d i x . T h i s has been the usual experience, 12 alt h o u g h a n occasional case r e p o r t m e n t i o n s t h a t a d e n o c a r c i n o m a of the a p p e n d i x was s t r o n g l y suggested w h e n the clinical p i c t u r e a n d b a r i u m - e n e m a studies p o i n t e d towards a p p e n d i c e a l disease, z, 11 T h e s e x-ray studies s h o w e d eccentric filling of the a p p e n d i c e a l
ADENOCARCINOMA
265
l u m e n , a n extrinsic mass compressing the m e d i a l wall of the cecum, a n d a lesion of the c a p u t cecum i n the r e g i o n of the a p p e n d i c u l a r orifice.
Summary T h e case of a p a t i e n t who h a d an unsuspected p e r f o r a t e d a d e n o c a r c i n o m a of the a p p e n d i x discovered d u r i n g o p e r a t i o n for a s i g m o i d c a r c i n o m a is presented. T h e problems in d i a g n o s i n g c a r c i n o m a of the a p p e n d i x are discussed a n d the l i t e r a t u r e is reviewed. References 1. Berman AT, James PM Jr: Adenocarcinoma of the vermiform appendix. Am J Surg 119: 733, 1970 2. Case Records of the Massachusetts General Hospital: Case 46202. N Engl J Med 262: 1033, 1960 3. Coblentz MG, Filippone DR: Primary adenocarcinoma of the jejunum and coexisting primary adenocarcinoma of the vermiform appendis: A case report. Surgery 77: 160, 1975 4. Coltins DC: 71,000 human appendix specimens: A final report, summarizing forty years' study. Am J Proctot I4: 365, 1963 5. Edmonson HT Jr, Hobbs ML: Primary adenocarcinoma of the appendix. Am Surg 33: 717, 1967 6. Yorsgren L, Molin K, Rieger A: Adenocarcinoma of the vermiform appendix. Acta Chir Scand 140: 486, 1974 7. Get R, Buch J, Held BT, et al: Carcinoma of the appendix: Report of two cases. Dis Colon Rectum 17: 370, 1974 8. Hesketh KT: The management of primary adenocarcinoma of the vermiform appendix. Gut 4: 158, 1963 9. Hopkins GB, Tullis RH, Kristensen KA: Primary adenocarcinoma of the vermiform appendix: Report of seven cases and review of the literature. Dis Colon Rectum 16: 140, 1973 10. Schmutzer KJ, Bayar M, Zaki AE, et al: Tumors of the appendix. Dis Colon Rectum 18: 324, 1975 11. Smith EB: Primary adenocarcinoma of the appendix. J Natl Med Assoc 55: 220, 1963 12. Watkins E, Khazei AM: Adenocarcinoma of the vermiform appendix: A review of the literature and case reports. Lahey Clin Found Bull 13: 273, 1964
of a Case*
M.D., HOWARD GOLDSON, M.D.,
GEORGE STERGIOPOULOS, M . D . , SAUL
J.
GROSBERG, M . D .
From the Departments of Gastroenterology and Surgery, The Brooklyn Veterans Administration Hospital, and The Downstate Medical Center, Brooklyn, New Yorh
the appendix is not often suspected preoperatively, 2, 3, 9, 10 and has never been diagnosed prior to surgical exploration. A case of perforated adenocarcinoma of the appendix as a second primary neoplasm in a patient who had relatively few symptoms is presented. PRIMARY ADENOCARCINOMA Of
P~eport o f a C a s e A 78-year-old black man was admitted to the hospital complaining of vague right and left lower q u a d r a n t pain and constipation of two months' duration. T h e r e was no weight loss, hematemesis, or melena, nor was there any history of a previous surgical procedure or significant illness. Results of physical examination were normal, including persistently guaiac-negative stools. Laboratory data were: hemoglobin 11.4 g/100 ml, hematocrit 36 per cent, leukocyte count, 4,400, with a normal differential. Blood urea nitrogen, glucose, electrolytes, and SMA-12 values were normal, as was the chest x-ray. T h e EKG revealed complete right bundle-branch block. Barium-enema examination showed segmental narrowing of the midsigmoid colon with a 1.5 • 1.0-cm sessile polyp in the proximal sigmoid. Pathologic examination of material obtained by colonoscopic polypectomy of sessile polyps at 27 and 30 cm revealed villous adenoma. T h e cecum was visualized to be. normal. Repeat colonoscopic polypectomy of a more proximal polyp (35 cm) revealed infiltrating adenocarcinoma (Fig. 1). A repeat barium-enema examination, done in preparation for definitive sigmoidal resection, revealed only the previously observed segmental narrowing. * Received for publication July 19, 1976. Address reprin.t requests to Dr. Grosberg: Veterans Administration Hospital, 800 Poly Place, Brooklyn, New York 11209.
T h e abdomen was explored via a left paramedian incision. A large mass in the pelvis, adherent to two loops of small bowel and to the bladder, was found. Further dissection revealed what appeared to be an old perforated appendicitis. T h e appendix was about 2.5 cm in diameter. Its tip was lying in a large cavity adherent to the urinary bladder. A frozen section of the material within the appendiceal mass showed adenocarcinoma. T h e r e was no evidence of hepatic or peritoneal metastases. Based u p o n the possible diagnosis of adenocarcinoma of the a p p e n d i x and an infiltrating carcinoma of the sigmoid colon, subtotal colectomy with ileoproctostomy was performed. T h e final pathology report revealed an infiltrating carcinoma of the sigmoid colon at the base of the previous polypectomy site and a primary perforated adenocarcinoma, mucinous type, o f the a p p e n d i x (Fig. 2). T h e cecum was not involved. T h e patient did well postoperatively and is being followed as an outpatient.
Discussion Primary adenocarcinoma of the appendix is rare. In one large series it was found in 0.03-0.08 per cent of all appendices removed. 4 In a more recent study t0 of 101 tumors found in 8,699 surgically removed appendices, only two were adenocarcinomas. A review of medical literature 1, 2, 5, demonstrates that the diagnosis is not often suspected preoperatively, and has never actually been made before operation. Presumptive preoperative diagnoses include acute appendicitis, - appendiceal abscess, uterine fibroid, carcinoma of the cecum, ovarian tumor, and acute and chronic cholecystitis. In many reports, 3, t0 carcinoma of the appendix has been an incidental finding dur-
263 Dis. Col. & Rect. April, 1977
Volume 20 Number 3
264
BROZINSKY, ET AL.
Dis. Col. & Rect.
ApriI, 1977
Fie. 1. This focus of adenocarcinoma in close proximity to the base of the sigmoid polyp prompted the decision for a definitive operative procedure (hematoxylin and eosin; x 32) .
FIG. 2. Pyknotic nuclei, aberrant glandular structure, and mucin-containing cells (center field) of appendiceal carcinoma (hematoxylin and eosin; • 240).
Volume 20 Number 3
PERFORATED
APPENDICEAL
i n g a b d o m i n a l e x p l o r a t i o n for o t h e r pathologic conditions. Such was the case in o u r p a t i e n t w h o was o r i g i n a l l y s c h e d u l e d to u n d e r g o s e g m e n t a l s i g m o i d a l resection for an invasive c a r c i n o m a in a s i g m o i d a l polyp. M a n y a b n o r m a l - a p p e a r i n g a p p e n d i c e s are r e m o v e d w i t h o u t benefit of frozen section. T h i s is especially true when a n a p p e n d i c e a l abscess is present, l e a d i n g the s u r g e o n to believe t h a t a n o l d p e r f o r a t i o n can account for the diseased a p p e n d i x . Forsgren e t al. 6 c a u t i o n t h a t histologic e x a m i n a t i o n s of all a p p e n d i c e s s h o u l d be done, a n d t h a t the a p p e n d i x should be p a l p a t e d at every laparotomy. C a r c i n o m a of the a p p e n d i x occurs m o r e often in males, w i t h the highest i n c i d e n c e in the fifth decade of life2 I t is m o r e freq u e n t in the base t h a n in the tip a n d , because of a p o o r l y d e v e l o p e d m u s c u l a r coat, metastasizes r e a d i l y b y local invasion. I t also spreads via l y m p h a t i c a n d h e m a t o genous routes. T h e most c o m m o n sites of metastasis are the liver, p e r i t o n e u m , a n d lung. P e r f o r a t i o n of the c a r c i n o m a does n o t alter survival figures. 9 O u r p a t i e n t p r o b a b l y h a d an " o l d " p e r f o r a t i o n , b u t h a d no evidence of metastatic disease. T h e o p e r a t i v e p r o c e d u r e of choice is app e n d e c t o m y w i t h r i g h t hemicolectomy. T h i s increased the five-year survival r a t e b y 43 p e r cent over t h a t f o u n d w i t h a p p e n d e c t o m y alone in one series. 8 B a r i u m - e n e m a e x a m i n a t i o n of o u r pat i e n t d i d n o t suggest disease in the a p p e n d i x . T h i s has been the usual experience, 12 alt h o u g h a n occasional case r e p o r t m e n t i o n s t h a t a d e n o c a r c i n o m a of the a p p e n d i x was s t r o n g l y suggested w h e n the clinical p i c t u r e a n d b a r i u m - e n e m a studies p o i n t e d towards a p p e n d i c e a l disease, z, 11 T h e s e x-ray studies s h o w e d eccentric filling of the a p p e n d i c e a l
ADENOCARCINOMA
265
l u m e n , a n extrinsic mass compressing the m e d i a l wall of the cecum, a n d a lesion of the c a p u t cecum i n the r e g i o n of the a p p e n d i c u l a r orifice.
Summary T h e case of a p a t i e n t who h a d an unsuspected p e r f o r a t e d a d e n o c a r c i n o m a of the a p p e n d i x discovered d u r i n g o p e r a t i o n for a s i g m o i d c a r c i n o m a is presented. T h e problems in d i a g n o s i n g c a r c i n o m a of the a p p e n d i x are discussed a n d the l i t e r a t u r e is reviewed. References 1. Berman AT, James PM Jr: Adenocarcinoma of the vermiform appendix. Am J Surg 119: 733, 1970 2. Case Records of the Massachusetts General Hospital: Case 46202. N Engl J Med 262: 1033, 1960 3. Coblentz MG, Filippone DR: Primary adenocarcinoma of the jejunum and coexisting primary adenocarcinoma of the vermiform appendis: A case report. Surgery 77: 160, 1975 4. Coltins DC: 71,000 human appendix specimens: A final report, summarizing forty years' study. Am J Proctot I4: 365, 1963 5. Edmonson HT Jr, Hobbs ML: Primary adenocarcinoma of the appendix. Am Surg 33: 717, 1967 6. Yorsgren L, Molin K, Rieger A: Adenocarcinoma of the vermiform appendix. Acta Chir Scand 140: 486, 1974 7. Get R, Buch J, Held BT, et al: Carcinoma of the appendix: Report of two cases. Dis Colon Rectum 17: 370, 1974 8. Hesketh KT: The management of primary adenocarcinoma of the vermiform appendix. Gut 4: 158, 1963 9. Hopkins GB, Tullis RH, Kristensen KA: Primary adenocarcinoma of the vermiform appendix: Report of seven cases and review of the literature. Dis Colon Rectum 16: 140, 1973 10. Schmutzer KJ, Bayar M, Zaki AE, et al: Tumors of the appendix. Dis Colon Rectum 18: 324, 1975 11. Smith EB: Primary adenocarcinoma of the appendix. J Natl Med Assoc 55: 220, 1963 12. Watkins E, Khazei AM: Adenocarcinoma of the vermiform appendix: A review of the literature and case reports. Lahey Clin Found Bull 13: 273, 1964
