Surgery Today Jpn. J. Surg. (1992) 22:357-362
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SURGERYTODAY © Springer-Verlag 1992
Case Reports A Case of Duodenal Carcinoma Presenting as a Submucosal Tumor YASUHIKO KOJIMA, 1 TAIZO KOBAYASHI,1 MASAYUKI NOTE, 1 GIZO NAKAGAWARA,t TAKUJI KATO, 2 YOSHIHIRO KOHLI, 2 HIROYUKI SUGIHARA,3 and YOSHIAKI IMAMURA3 The First Department of Surgery,~ The Second Department of Internal Medicine, 2 and The First Department of Pathology, 3 Fukui Medical Scho61, Fukui, Japan
Abstract: This paper describes a patient with duodenal carcinoma showing the features of a submucosal tumor, leading to difficulty in making an accurate preoperative diagnosis. A 63-year-old woman was admitted for investigation of a duodenal mass. An examination of the upper gastrointestinal tract revealed a semicircular compression of the stomach and the duodenum. Endoscopy of the stomach and duodenum disclosed a hemispherical tumor with a deep ulcer in the apex. Computer tomography revealed a tumor of about 5 cm in diameter at the same site. Laparotomy was performed under the tentative diagnosis of a submucosal tumor. A tumor was found occupying the duodenum, which compressed the gastric antrum exteriorly, and was also adherent to the head of the pancreas by direct invasion. A curative resection was performed by combining a pancreatoduodenectomy with a transverse colectomy along with regional lymph node clearance. A microscopic examination showed that the tumor contained neoplastic cells growing in a tubular pattern, particularly in its peripheral regions. Thus, this lesion was finally diagnosed as primary adenocarcinoma of the duodenum. Key Words: duodenal carcinoma, submucosal tumor
features of a submucosal tumor, which leading to difficulties in making an accurate preoperative diagnosis.
Case Report
Patient A 63-year-old small but fairly well nourished Japanese w o m a n was admitted to Fukui Medical School Hospital on July 28, 1987, with a chief complaint of epigastric pain after meals. She began to feel constant epigastric pain since about 2 weeks before admission and had lost 2 kg of body weight. The physical examination on admission revealed an elastic firm tumor approximately 6 × 5 cm in diameter without fluctuation or pulsation in the left epigastrium. The t u m o r was not mobile, and a moderate tenderness was elicited on deep palpation. Auscultation of the lungs and heart was normal. The blood pressure was 134/70 m m Hg, the pulse rate was 84/rain and regular, the respiration rate was 16/min, and the body temperature was 36.5°C.
Introduction
Laboratory Findings
Primary malignant tumors of the duodenum besides the papilla of Vater are rarely encountered in comparison with tumors of the stomach and colon. 1-3 A m o n g them, duodenal carcinoma is the most c o m m o n lesion. This p a p e r presents a patient with carcinoma arising from the second part of the duodenum but not involving the papilla of Vater. This tumor had most of the
The laboratory findings are shown in Table 1. The resuits of various laboratory studies, including urinalysis, complete blood count, stool examination, liver function tests, and urinary 5-hydroxyindoleacetic acid ( 5 H I A A ) showed normal values. However, the serum levels of tumor markers such as carcinoembryonic antigen ( C E A ) and carbohydrate antigen determined 19-9 (CA19-9) were markedly elevated (Table 1).
Reprint requests to: Y. Kojima, The Second Department of Surgery, Kanazawa Medical University, Daigaku 1-1, Uchinadamachi, Kahoku-gun, 920-02 Ishikawa, Japan (Received for publication on Sep. 25, 1990; accepted on May 1, 1992)
Roentgenologic Studies Chest X-ray and plain films of the a b d o m e n showed no remarkable changes. An examination of the upper
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Table 1o Preoperative laboratory findings Hematology WBC RBC Hb Ht Plt Urinalysis Protein Sugar Urobil Stool Occult blood Elastase 1 AFP Ferritin (F) CEA CA19-9 U-5HIAA Na K C1 Ca
8600/ram 3 3.87 × 106/mm3 11.3 g/dl 34.8% 256 × 103/mm3 (-) (-) (+) (-) 326 ng/dl 100 ng/ml 203.3 U/ml 1.8 mg/day 139 mEq/l 4.1 mEq/1 101 mEq/1 4.6 mEq/1
T.P A/G Alb T.Bil D.Bil ZTT TTT GOT GPT LDH ALP LAP 7-GTP ChE BUN Cr S-Amy T-Chol FBS 75 g O-GTT normal PFD
6.9 g/dl 1.09 3.6g/dl 0.3 mg/dl 0.1 mg/dl 10.6U 4.7 U 22 IU/1 19 IU/1 666 IU/I 340 IU/1 45 IU/1 22 !U/1 5.17 IU/ml 18 mg/dl 0.9 mg/dl 326 IU/1 190 mg/dl 97 mg/dl pattern 81.9%
Fig. 2. CT of the upper abdomen revealed a tumor of about 5cm in diameter with an irregular margin. CT, computed tomography
WBC, white blood cells; RBC, red blood cells; Hb, hemoglobin; Ht, hematocrit; Plt, platelets; AFP, alpha-fetoprotein; CEA, carcinoembryonic antigen; CA19-9, carbohydrate antigen determined 19-9; U-5HIAA, urinary 5-1aydroxyindoleacetic acid; Na, sodium; K, potassium; CI, chlorine; Ca, calcium
Fig. 3. Angiography of the celiac artery showed a marked encasement of the gastroduodenal and right gastroepiploic arteries. The anterior superior pancreaticoduodenal artery was distorted by the tumor, and tumor vessels were seen at its periphery
Fig. 1. An upper gastrointestinal tract examination revealed a semicircular compression of the greater curvature of the stomach
gastrointestinal tract revealed a semicircular compression of the gastric antrum by a large extrinsic mass (Fig. 1). Hypotonic duodenography also demonstrated an upward and forward displacement of the first and second parts of the duodenum, together with a slightly serrated pattern. C o m p u t e d t o m o g r a p h y (CT) of the upper a b d o m e n revealed a tumor of about 5 c m in diameter with an irregular margin that was situated between the gastric antrum and the first and second parts of the duodenum. T h e r e was also an enlargement of the para-aortic lymph nodes, but no m a r k e d changes
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359
Fig. 4. A cross-section of the tumor revealed a
yellowish lesion with submucosal growth
were noted in the pancreas, liver, gallbladder, and biliary tract (Fig. 2). Angiography of the celiac and superior mesenteric arteries showed a marked encase: ment of the gastroduodenal and right gastroepiploic arteries. The anterior superior pancreaticoduodenal artery was distorted by the tumor, and tumor vessels were seen at its periphery. The posterior superior pancreaticoduodenal artery was intact (Fig. 3). In the venous phase of angiography, contrast agent accumulation was especially noted in the periphery of the tumor, but no abnomalities were present in either the portal or superior mesenteric veins.
Ultrasonic Study An ultrasound (US) revealed an irregular tumor (6.7 × 6.3cm) situated between the gastric antrum and the first and second parts of the duodenum. The lesion was of a mixed low and high echogenicity and US suggested that this tumor did not originate from the pancreas.
Endoscopic Findings Endoscopy of the stomach and duodenum disclosed a hemispherical tumor in the abovementioned site. The mucosal surface appeared smooth and normal, but there was a deep ulcer near the top of this tumor on the second part of the duodenum. Several specimens taken from the edge of this ulcer showed only an inflammatory duodenal mucosa with chronic inflammatory cells, and so failed to lead to a qualitative pathological diagnosis.
Operative Findings An accurate preoperative diagnosis was difficult, so with a working diagnosis of a submucosal tumor of the
(arrow)
duodenum, laparotomy was performed on October 7, 1987. Under general anesthesia, the peritoneal cavity was opened via an upper middle incision. A tumor was found occupying the duodenum, which compressed the gastric antrum and the transverse colon exteriorly, and also was adherent to the head of the pancreas by direct invasion. There were no visable metastatic lesions in the liver or peritoneal cavity which suggested that the tumor had only spread locally by direct invasion into the adjacent organs, and was not unresectable. Aiming at a curative resection, a pancreatoduodenectomy and transverse colectomy concomitant with regional lymph node clearance was performed. Reconstruction was made by Child's method with some modifications and an ileotransverse colostomy was provided.
Pathological Investigations Macroscopically, the tumor was 8.1 × 7.5 × 7.3cm in size and located in the submucosal layer of the first and second parts of the duodenum. It penetrated the duodenal mucosa in a small region (0.5 × 0.5 cm) at its top, but the papilla of Vater was free of any tumor. A cross-section of the lesion revealed a yellowish tumor located mainly in the submucosa of the duodenum, suggesting that this was a submucosal tumor (Fig. 4). Microscopically, the tumor was largely composed of necrotic tissue, and those cells were surrounded by a pseudocapsule composed of collagen fibers. There were some neoplastic cells growing in a tubular pattern, particularly in the peripheral regions of the tumor (Fig. 5). In some sections, the tumor could be seen to invade the pancreas to a depth of about 0.3 mm, but no duodenal mucosal exposure of neoplastic cells was found except at the top of the lesion. The neoplastic cells had an abundant pale cytoplasm and ovoid vesicular nuclei, and varied mildly in size.
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Fig. 5. A Microscopic examination showed that the tumor was covered with a normal mucosa. B Neoplastic cells growing in a tubuJar pattern were seen in the peripheral part of the tumor. (H&E, ×20)
A r o u n d the tumor nodules, lymphatic involvement was quite commonly seen. This tumor showed the features of a submucosal tumor, as mentioned above. Accordingly, various other stains, such as alcian blue, Grimelius, periodic acid-Schiff (PAS), modified Masson's staining, and immunohistochemical staining, as well as an electron microscopic study were performed on the specimens taken from the entire tumor. The tumor cells were negative for alcian blue, Grimelius, PAS, and modified Masson's staining. Immunohistochemical staining was performed for serotonin, C E A , peanut lectin (PNAlectin), Ulexeuropaeus agglutinin-I (UEA-I), and
Griffonia-simplicifolia agglutin-II (GS-II). 4-6 Serotonin was only weakly positive in some parts of the lesion, while PNA-lectin and GS-II were positive (Fig. 6). No secretory granules were found in the electron microscopic study. Thus, this tumor was considered to be an adenocarcinoma. There were no metastases of the tumor found in the 25 dissected lymph nodes.
Postoperative Course Five days after the operation, a minor leakage from the pancreatojejunostomy was seen. This leakage was cured by sustained aspiration and lavage within about 3
Y. Kojima et al.: Duodenal Carcinoma
361
Fig. 6. A GS-II and B PNA-lectin stainings were positive (×66)
weeks, without any secondary complications such as intraperitoneal hemorrhage or abscess. After surgery, the serum levels of tumor markers C E A and CA19-9 returned to within normal limits. The patient remains in good health 3 years and 5 months after the operation without any evidence of tumor recurrence on regular follow-up CT scans.
Discussion Diseases which present mainly with stenosis or displacement of the second part of the duodenum are normally suspected of being malignant or benign tumors of the duodenal tract, pancreatic or retroperitoneal in origin, or pancreatitis. 7-9 A differential diagnosis of these diseases might be relatively easy to make as a result of new medical imaging techniques including CT, US, X-ray, endoscopic studies of the upper alimentary tract, and angiography. Submucosal tumors are comparatively easy to detect and diagnose, but it is generally difficult to make a definitive diagnosis by biopsy even if an artificial ulcer is induced by needle biopsy or with a high-frequency electrotome.
In the case under discussion, the tumor, for the most part, had a morphologically submucosal growth, and only the top of the tumor was exposed out of the duodenal mucosa, that is, it had the so-called "delle". Based on these ample features of radiographical and endoscopic findings, the patient was considered to most likely to have a stibmucosal tumor of the duodenum before operation. The tissue sample obtained at operation obviously showed an adenocarcinoma, but this finding was coupled with the preoperative suspicions of a submucosal tumor, and thus a metastatic tumor via the lymphatics had to be ruled out. Metastatic carcinoma was ruled out on the grounds that various preoperative examinations failed to reveal the primary growth in any other organ, and intraperitoneal eXamination during surgery failed to reveal other tumor. The patient has now been healthy for 3 years and 5 months since surgery, and no other tumor has been detected by periodical CT scans. Pathologically, the lesion basically showed a pseudocapsulation that is uncommon in metastatic tumors. These facts may also support that the tumor under discussion was a primary carcinoma of the duodenum.
362 P r i m a r y d u o d e n a l c a r c i n o m a is a r a r e t u m o r . D u o d e n a l c a r c i n o m a m a k e s up to 0 . 3 % o f all m a l i g n a n t t u m o r s o f the g a s t r o i n t e s t i n a l t r a c t . t - 3 D u o d e n a l carc i n o m a which l o o k s like s u b m u c o s a l t u m o r , as in o u r case, is still r a r e r . A s a m a t t e r o f fact, t h e r e h a v e o n l y b e e n a few i n s t a n c e s of such a t u m o r in t h e s t o m a c h ( i n c l u d i n g an origin f r o m t h e a b e r r a n t p a n c r e a s ) rep o r t e d so far. 1°-12 T h e p r o b l e m with this t u m o r is t h e histological site o f p r i m a r y g r o w t h . It m a y b e n e c e s s a r y to suspect t h e head of the pancreas, duodenal mucosa, or aberrant p a n c r e a s of t h e d u o d e n u m as p o s s i b l e sites of p r i m a r y g r o w t h . I n the case u n d e r discussion, h o w e v e r , the h e a d of t h e p a n c r e a s a n d d u o d e n a l m u c o s a s h o u l d b e p r e c l u d e d b e c a u s e this t u m o r has a p r e d i l e c t i o n for t h e s u b m u c o s a of t h e d u o d e n u m . O n histological sections, t h e t u m o r gave no clues to its origin, b u t we a r e i n c l i n e d to p o s t u l a t e t h a t t h e a b e r r a n t p a n c r e a s o f t h e d u o d e n u m m a y b e t h e site of p r i m a r y g r o w t h f r o m a h i s t o l o g i c a l view p o i n t . O u r p o s t u l a t i o n is b a s e d on c i r c u m s t a n t i a l e v i d e n c e : T h e d u o d e n u m is o n e of t h e f a v o r e d sites for t h e a b e r r a n t p a n c r e a s . In t h e case o f l a r g e lesions like this t u m o r , t h e a b e r r a n t p a n c r e a s o f t h e d u o d e n u m is o f t e n r e p l a c e d b y a c a n c e r in its e n t i r e t y so t h a t an in situ lesion o f t e n d i s a p p e a r s , a n d t h e t u m o r h i s t o l o g i c a l l y b e a r s a close r e s e m b l a n c e to a c o m m o n t y p e o f p a n c r e a t i c c a n c e r o r i g i n a t i n g in the p a n c r e a t i c duct. A s a l r e a d y m e n t i o n e d , t h e t u m o r g r e w n o t so m u c h in t h e m u c o s a as in t h e s u b m u c o s a , a n d was n o t a m e t a s t a t i c lesion c o n s i s t e n t with t h e diagn o s t i c c r i t e r i a for c a n c e r a t i o n o f t h e a b e r r a n t p a n c r e a s o f t h e d u o d e n u m . 13 T u m o r m a r k e r s , e s p e c i a l l y C A 1 9 - 9 w h i c h usually i n c r e a s e by n e a r l y 100% in p a n c r e a t i c c a r c i n o m a , r e t u r n e d to n o r m a l s o o n a f t e r surgery.
Y. Kojima et al.: Duodenal Carcinoma
References 1. Zenklusen HR, Landmann J, Feess A, Durig M, Kasper M, Oberholzer M (1989) PrimarY duodenal carcinoma arising in a non-Vaterian tubulo-villous adenoma. Virchows Arch [A] 414:529-533 2. Alwmark A, Andersson A, Lasson A (1980) Primary carcinoma of the duodenum. Ann Surg 191:13-18 3. Spira IA, Ghazi A, Wolff WI (1977) Primary adenocarcinoma of the duodenum. Cancer 39:1721-1726 4. Katsuyama T, Ono K, Nakayama J, Akimatsu T, Honda T (1985) Mucosubstance histochemistry of the normal mucosa and carcinoma of the large intestine: Galactose o×idase-Schiff reaction and lectin staining. Acta Pathol Jpn 35:1409-1425 5. Yuan M, Itzkowitz SH, Boland CR, Kim YD, Tomita JT, Palekar A, Bennington JL, Trump BF, Kim YS (1986) Comparison of T-antigen expression in normal, premalignant, and malignant human colonic tissue using lectin and antibody immunohistochemistry. Cancer Res 46:4841-4847 6. Cooper HS, Reuter VE (1983) Peanut lectin-binding sites in polyps of the colon and rectum: Adenomas, hyperplastic polyps, and adenomas with in situ carcinoma. Lab Invest 49:655-661 7. Short WF (1977) Duodenal narrowing associated with chronic pancreatitis. Dig Dis Sci 22:241-242 8. Dodds JJ, Beahrs OH (1963) Leiomyosarcoma of the duodenum. Am J Surg 105:245-249 9. Bruno MS, Fein HD (1970) Primary malignant and benign tumors of the duodenum. Arch Intern Med 125:670-679 10. Ishii K, Kamii H, Mohri K, Kimura T, Teryya M, Hasegawa S, Sugiyama M (1984) A case of gastric cancer showing features of a submucosal tumor in a short term during acute hepatitis: A case report (in Japanese with English Abstract). Prog Diges Endos 24:246-249 11. Hayashi S, Satake T, Koike M, Ema Y, Ohba K (1982) Early gastric mucinous adenicarcinoma showing features of a submucosal tumor: Report of a case (in Japanese with English Abstract). I to Cho (Stomach and Intestine) 17:299-304 12. Hickman DM, Frey CF, Carson JW. Adenocarcinoma arising in gastric heterotopic pancreas. West J Med 1981; 135:57-62 13. Mitsuhayashi Y, Ishikawa Y, Mukawa A, Mori A, Tsunamura Y, Miyanaga M (1983) Carcinoma originating from the heterotopic pancreas in the stomach: Report of a case. I to Cho (Stomach and Intestine) 18:267-272
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SURGERYTODAY © Springer-Verlag 1992
Case Reports A Case of Duodenal Carcinoma Presenting as a Submucosal Tumor YASUHIKO KOJIMA, 1 TAIZO KOBAYASHI,1 MASAYUKI NOTE, 1 GIZO NAKAGAWARA,t TAKUJI KATO, 2 YOSHIHIRO KOHLI, 2 HIROYUKI SUGIHARA,3 and YOSHIAKI IMAMURA3 The First Department of Surgery,~ The Second Department of Internal Medicine, 2 and The First Department of Pathology, 3 Fukui Medical Scho61, Fukui, Japan
Abstract: This paper describes a patient with duodenal carcinoma showing the features of a submucosal tumor, leading to difficulty in making an accurate preoperative diagnosis. A 63-year-old woman was admitted for investigation of a duodenal mass. An examination of the upper gastrointestinal tract revealed a semicircular compression of the stomach and the duodenum. Endoscopy of the stomach and duodenum disclosed a hemispherical tumor with a deep ulcer in the apex. Computer tomography revealed a tumor of about 5 cm in diameter at the same site. Laparotomy was performed under the tentative diagnosis of a submucosal tumor. A tumor was found occupying the duodenum, which compressed the gastric antrum exteriorly, and was also adherent to the head of the pancreas by direct invasion. A curative resection was performed by combining a pancreatoduodenectomy with a transverse colectomy along with regional lymph node clearance. A microscopic examination showed that the tumor contained neoplastic cells growing in a tubular pattern, particularly in its peripheral regions. Thus, this lesion was finally diagnosed as primary adenocarcinoma of the duodenum. Key Words: duodenal carcinoma, submucosal tumor
features of a submucosal tumor, which leading to difficulties in making an accurate preoperative diagnosis.
Case Report
Patient A 63-year-old small but fairly well nourished Japanese w o m a n was admitted to Fukui Medical School Hospital on July 28, 1987, with a chief complaint of epigastric pain after meals. She began to feel constant epigastric pain since about 2 weeks before admission and had lost 2 kg of body weight. The physical examination on admission revealed an elastic firm tumor approximately 6 × 5 cm in diameter without fluctuation or pulsation in the left epigastrium. The t u m o r was not mobile, and a moderate tenderness was elicited on deep palpation. Auscultation of the lungs and heart was normal. The blood pressure was 134/70 m m Hg, the pulse rate was 84/rain and regular, the respiration rate was 16/min, and the body temperature was 36.5°C.
Introduction
Laboratory Findings
Primary malignant tumors of the duodenum besides the papilla of Vater are rarely encountered in comparison with tumors of the stomach and colon. 1-3 A m o n g them, duodenal carcinoma is the most c o m m o n lesion. This p a p e r presents a patient with carcinoma arising from the second part of the duodenum but not involving the papilla of Vater. This tumor had most of the
The laboratory findings are shown in Table 1. The resuits of various laboratory studies, including urinalysis, complete blood count, stool examination, liver function tests, and urinary 5-hydroxyindoleacetic acid ( 5 H I A A ) showed normal values. However, the serum levels of tumor markers such as carcinoembryonic antigen ( C E A ) and carbohydrate antigen determined 19-9 (CA19-9) were markedly elevated (Table 1).
Reprint requests to: Y. Kojima, The Second Department of Surgery, Kanazawa Medical University, Daigaku 1-1, Uchinadamachi, Kahoku-gun, 920-02 Ishikawa, Japan (Received for publication on Sep. 25, 1990; accepted on May 1, 1992)
Roentgenologic Studies Chest X-ray and plain films of the a b d o m e n showed no remarkable changes. An examination of the upper
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Y. Kojima et al.: Duodenal Carcinoma
Table 1o Preoperative laboratory findings Hematology WBC RBC Hb Ht Plt Urinalysis Protein Sugar Urobil Stool Occult blood Elastase 1 AFP Ferritin (F) CEA CA19-9 U-5HIAA Na K C1 Ca
8600/ram 3 3.87 × 106/mm3 11.3 g/dl 34.8% 256 × 103/mm3 (-) (-) (+) (-) 326 ng/dl 100 ng/ml 203.3 U/ml 1.8 mg/day 139 mEq/l 4.1 mEq/1 101 mEq/1 4.6 mEq/1
T.P A/G Alb T.Bil D.Bil ZTT TTT GOT GPT LDH ALP LAP 7-GTP ChE BUN Cr S-Amy T-Chol FBS 75 g O-GTT normal PFD
6.9 g/dl 1.09 3.6g/dl 0.3 mg/dl 0.1 mg/dl 10.6U 4.7 U 22 IU/1 19 IU/1 666 IU/I 340 IU/1 45 IU/1 22 !U/1 5.17 IU/ml 18 mg/dl 0.9 mg/dl 326 IU/1 190 mg/dl 97 mg/dl pattern 81.9%
Fig. 2. CT of the upper abdomen revealed a tumor of about 5cm in diameter with an irregular margin. CT, computed tomography
WBC, white blood cells; RBC, red blood cells; Hb, hemoglobin; Ht, hematocrit; Plt, platelets; AFP, alpha-fetoprotein; CEA, carcinoembryonic antigen; CA19-9, carbohydrate antigen determined 19-9; U-5HIAA, urinary 5-1aydroxyindoleacetic acid; Na, sodium; K, potassium; CI, chlorine; Ca, calcium
Fig. 3. Angiography of the celiac artery showed a marked encasement of the gastroduodenal and right gastroepiploic arteries. The anterior superior pancreaticoduodenal artery was distorted by the tumor, and tumor vessels were seen at its periphery
Fig. 1. An upper gastrointestinal tract examination revealed a semicircular compression of the greater curvature of the stomach
gastrointestinal tract revealed a semicircular compression of the gastric antrum by a large extrinsic mass (Fig. 1). Hypotonic duodenography also demonstrated an upward and forward displacement of the first and second parts of the duodenum, together with a slightly serrated pattern. C o m p u t e d t o m o g r a p h y (CT) of the upper a b d o m e n revealed a tumor of about 5 c m in diameter with an irregular margin that was situated between the gastric antrum and the first and second parts of the duodenum. T h e r e was also an enlargement of the para-aortic lymph nodes, but no m a r k e d changes
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359
Fig. 4. A cross-section of the tumor revealed a
yellowish lesion with submucosal growth
were noted in the pancreas, liver, gallbladder, and biliary tract (Fig. 2). Angiography of the celiac and superior mesenteric arteries showed a marked encase: ment of the gastroduodenal and right gastroepiploic arteries. The anterior superior pancreaticoduodenal artery was distorted by the tumor, and tumor vessels were seen at its periphery. The posterior superior pancreaticoduodenal artery was intact (Fig. 3). In the venous phase of angiography, contrast agent accumulation was especially noted in the periphery of the tumor, but no abnomalities were present in either the portal or superior mesenteric veins.
Ultrasonic Study An ultrasound (US) revealed an irregular tumor (6.7 × 6.3cm) situated between the gastric antrum and the first and second parts of the duodenum. The lesion was of a mixed low and high echogenicity and US suggested that this tumor did not originate from the pancreas.
Endoscopic Findings Endoscopy of the stomach and duodenum disclosed a hemispherical tumor in the abovementioned site. The mucosal surface appeared smooth and normal, but there was a deep ulcer near the top of this tumor on the second part of the duodenum. Several specimens taken from the edge of this ulcer showed only an inflammatory duodenal mucosa with chronic inflammatory cells, and so failed to lead to a qualitative pathological diagnosis.
Operative Findings An accurate preoperative diagnosis was difficult, so with a working diagnosis of a submucosal tumor of the
(arrow)
duodenum, laparotomy was performed on October 7, 1987. Under general anesthesia, the peritoneal cavity was opened via an upper middle incision. A tumor was found occupying the duodenum, which compressed the gastric antrum and the transverse colon exteriorly, and also was adherent to the head of the pancreas by direct invasion. There were no visable metastatic lesions in the liver or peritoneal cavity which suggested that the tumor had only spread locally by direct invasion into the adjacent organs, and was not unresectable. Aiming at a curative resection, a pancreatoduodenectomy and transverse colectomy concomitant with regional lymph node clearance was performed. Reconstruction was made by Child's method with some modifications and an ileotransverse colostomy was provided.
Pathological Investigations Macroscopically, the tumor was 8.1 × 7.5 × 7.3cm in size and located in the submucosal layer of the first and second parts of the duodenum. It penetrated the duodenal mucosa in a small region (0.5 × 0.5 cm) at its top, but the papilla of Vater was free of any tumor. A cross-section of the lesion revealed a yellowish tumor located mainly in the submucosa of the duodenum, suggesting that this was a submucosal tumor (Fig. 4). Microscopically, the tumor was largely composed of necrotic tissue, and those cells were surrounded by a pseudocapsule composed of collagen fibers. There were some neoplastic cells growing in a tubular pattern, particularly in the peripheral regions of the tumor (Fig. 5). In some sections, the tumor could be seen to invade the pancreas to a depth of about 0.3 mm, but no duodenal mucosal exposure of neoplastic cells was found except at the top of the lesion. The neoplastic cells had an abundant pale cytoplasm and ovoid vesicular nuclei, and varied mildly in size.
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Y. Kojima et al.: Duodenal Carcinoma
Fig. 5. A Microscopic examination showed that the tumor was covered with a normal mucosa. B Neoplastic cells growing in a tubuJar pattern were seen in the peripheral part of the tumor. (H&E, ×20)
A r o u n d the tumor nodules, lymphatic involvement was quite commonly seen. This tumor showed the features of a submucosal tumor, as mentioned above. Accordingly, various other stains, such as alcian blue, Grimelius, periodic acid-Schiff (PAS), modified Masson's staining, and immunohistochemical staining, as well as an electron microscopic study were performed on the specimens taken from the entire tumor. The tumor cells were negative for alcian blue, Grimelius, PAS, and modified Masson's staining. Immunohistochemical staining was performed for serotonin, C E A , peanut lectin (PNAlectin), Ulexeuropaeus agglutinin-I (UEA-I), and
Griffonia-simplicifolia agglutin-II (GS-II). 4-6 Serotonin was only weakly positive in some parts of the lesion, while PNA-lectin and GS-II were positive (Fig. 6). No secretory granules were found in the electron microscopic study. Thus, this tumor was considered to be an adenocarcinoma. There were no metastases of the tumor found in the 25 dissected lymph nodes.
Postoperative Course Five days after the operation, a minor leakage from the pancreatojejunostomy was seen. This leakage was cured by sustained aspiration and lavage within about 3
Y. Kojima et al.: Duodenal Carcinoma
361
Fig. 6. A GS-II and B PNA-lectin stainings were positive (×66)
weeks, without any secondary complications such as intraperitoneal hemorrhage or abscess. After surgery, the serum levels of tumor markers C E A and CA19-9 returned to within normal limits. The patient remains in good health 3 years and 5 months after the operation without any evidence of tumor recurrence on regular follow-up CT scans.
Discussion Diseases which present mainly with stenosis or displacement of the second part of the duodenum are normally suspected of being malignant or benign tumors of the duodenal tract, pancreatic or retroperitoneal in origin, or pancreatitis. 7-9 A differential diagnosis of these diseases might be relatively easy to make as a result of new medical imaging techniques including CT, US, X-ray, endoscopic studies of the upper alimentary tract, and angiography. Submucosal tumors are comparatively easy to detect and diagnose, but it is generally difficult to make a definitive diagnosis by biopsy even if an artificial ulcer is induced by needle biopsy or with a high-frequency electrotome.
In the case under discussion, the tumor, for the most part, had a morphologically submucosal growth, and only the top of the tumor was exposed out of the duodenal mucosa, that is, it had the so-called "delle". Based on these ample features of radiographical and endoscopic findings, the patient was considered to most likely to have a stibmucosal tumor of the duodenum before operation. The tissue sample obtained at operation obviously showed an adenocarcinoma, but this finding was coupled with the preoperative suspicions of a submucosal tumor, and thus a metastatic tumor via the lymphatics had to be ruled out. Metastatic carcinoma was ruled out on the grounds that various preoperative examinations failed to reveal the primary growth in any other organ, and intraperitoneal eXamination during surgery failed to reveal other tumor. The patient has now been healthy for 3 years and 5 months since surgery, and no other tumor has been detected by periodical CT scans. Pathologically, the lesion basically showed a pseudocapsulation that is uncommon in metastatic tumors. These facts may also support that the tumor under discussion was a primary carcinoma of the duodenum.
362 P r i m a r y d u o d e n a l c a r c i n o m a is a r a r e t u m o r . D u o d e n a l c a r c i n o m a m a k e s up to 0 . 3 % o f all m a l i g n a n t t u m o r s o f the g a s t r o i n t e s t i n a l t r a c t . t - 3 D u o d e n a l carc i n o m a which l o o k s like s u b m u c o s a l t u m o r , as in o u r case, is still r a r e r . A s a m a t t e r o f fact, t h e r e h a v e o n l y b e e n a few i n s t a n c e s of such a t u m o r in t h e s t o m a c h ( i n c l u d i n g an origin f r o m t h e a b e r r a n t p a n c r e a s ) rep o r t e d so far. 1°-12 T h e p r o b l e m with this t u m o r is t h e histological site o f p r i m a r y g r o w t h . It m a y b e n e c e s s a r y to suspect t h e head of the pancreas, duodenal mucosa, or aberrant p a n c r e a s of t h e d u o d e n u m as p o s s i b l e sites of p r i m a r y g r o w t h . I n the case u n d e r discussion, h o w e v e r , the h e a d of t h e p a n c r e a s a n d d u o d e n a l m u c o s a s h o u l d b e p r e c l u d e d b e c a u s e this t u m o r has a p r e d i l e c t i o n for t h e s u b m u c o s a of t h e d u o d e n u m . O n histological sections, t h e t u m o r gave no clues to its origin, b u t we a r e i n c l i n e d to p o s t u l a t e t h a t t h e a b e r r a n t p a n c r e a s o f t h e d u o d e n u m m a y b e t h e site of p r i m a r y g r o w t h f r o m a h i s t o l o g i c a l view p o i n t . O u r p o s t u l a t i o n is b a s e d on c i r c u m s t a n t i a l e v i d e n c e : T h e d u o d e n u m is o n e of t h e f a v o r e d sites for t h e a b e r r a n t p a n c r e a s . In t h e case o f l a r g e lesions like this t u m o r , t h e a b e r r a n t p a n c r e a s o f t h e d u o d e n u m is o f t e n r e p l a c e d b y a c a n c e r in its e n t i r e t y so t h a t an in situ lesion o f t e n d i s a p p e a r s , a n d t h e t u m o r h i s t o l o g i c a l l y b e a r s a close r e s e m b l a n c e to a c o m m o n t y p e o f p a n c r e a t i c c a n c e r o r i g i n a t i n g in the p a n c r e a t i c duct. A s a l r e a d y m e n t i o n e d , t h e t u m o r g r e w n o t so m u c h in t h e m u c o s a as in t h e s u b m u c o s a , a n d was n o t a m e t a s t a t i c lesion c o n s i s t e n t with t h e diagn o s t i c c r i t e r i a for c a n c e r a t i o n o f t h e a b e r r a n t p a n c r e a s o f t h e d u o d e n u m . 13 T u m o r m a r k e r s , e s p e c i a l l y C A 1 9 - 9 w h i c h usually i n c r e a s e by n e a r l y 100% in p a n c r e a t i c c a r c i n o m a , r e t u r n e d to n o r m a l s o o n a f t e r surgery.
Y. Kojima et al.: Duodenal Carcinoma
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