Surgery Today Jpn. J. Surg. (1992) 22:363-367

~

SURGERYTODAY © Springer-Verlag 1992

Lymphangioma in the Small Intestine: Report of a Case and Review of the Japanese Literature TAKESHI HANAGIRI, 1 MEGUMI BABA,2 TAKASHI SHIMABUKURO,2MITSUNORI HASHIMOTO,2 HIROSHI TAKEMOTO,2 AKIRA INOUE, 2 AKIRA SUGITANI,2 and TAKAYUKISHIRAKUSA1 1The Second Department of Surgery, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Kitakyusyu 807, Japan and 2 the Department of Surgery, Hamamatsu Rousai Hospital, Hamamatsu, Japan

Abstract: We report herein a rare case of a 53 year old man with a benign lymphangioma of the small intestine. He presented with a complete obstruction of the small intestine and radiological examination revealed a small intestinal tumor. A long intestinal tube was passed, the intestinograms from which detected a submucosal tumor of the small intestine. Partial resection of the small intestine was thus performed and the tumor was found to be located mainly in the submucosa. The final pathological diagnosis was made as cavernous lymphangioma. This case is presented with a description of the roentgen appearance, followed by a review of the Japanese literature. Key Words: lymphangioma, small intestine

Introduction

Lymphangioma of the gastrointestinal tract is a very rare entity. 1.2 The following report describes a case of lymphangioma of the small intestine. The tumor is usually asymptomatic but the patient may present with signs and symptoms of intestinal obstruction. There have been few reports which describe the X-ray findings of this lesion in any part of the small intestine other than the duodenum. Local resection is the treatment of choice once the diagnosis has been made. Recently, endoscopic examinations of the small intestine have been reported, 3'4 but it is difficult to examine the proximal portion of the ileum, as needed to be done in the present case. X-ray examination using a long intestinal tube is a very useful method for detecting organic lesions in the middle part of the small intestine, and we describe the use of this method in the case presented herein. Reprint requests to: T. Hanagiri (Received for publication on Nov. 8, 1990; accepted on May 1, 1992)

Case R e p o r t

A 53 year old man was admitted to the Department of Surgery, Hamamatsu Rousai Hospital, in March, 1988 complaining of abdominal pain and nausea. On physical examination, tenderness in the left lower quadrant of the abdomen and abdominal distention were evident. He had undergone no previous surgery. Laboratory data revealed a hematocrit of 33.7 per cent, a hemoglobin of ll.0g/dl, and a leukocyte count of 7,700. Serum chemistry and urine analysis were normal. A test for occult blood in the stool was positive. Plain abdominal roentgenograms showed an air-fluid level of the small intestine. An obstruction of the small intestine was thus diagnosed and a long intestinal tube inserted, the selective intestinograms of which revealed a sharply defined oval filling defect, measuring 2.5 x 2.0cm (Figs. 1 and 2). Laparotomy was performed on March 28, 1988. A soft pliable tumor was observed in the small intestine about 250 cm distal from the ligament of Treitz and the segment of small intestine containing this tumor was resected. Macroscopically a polypoid mass with a broad sessile base was located in the ventral side of the intestine and the mucosal surface of the tumor was smooth and yellowish (Fig. 3). Histologically, this tumor was diagnosed as a cavernous lymphangioma from the following findings: 1. the prominence of cystic dilatations of varying sizes separated by a thin septum and lined by endothelium, mo~inly in the submucosa; 2. the presence of a homogeneous pink-staining material and; 3. histologically, the lesion resembled a cavernous hemangioma except that lymph instead of blood was filling the dilated spaces (Figs. 4 and 5). The patient's discomfort was relieved after the operation and he was discharged following postoperative barium studies.

364

T. Hanagiri et al.: Small Intestinal Lymphangioma

Fig. 3. Gross findings of the lymphangioma (arrows). The tumor is spherical in shape with a smooth surface

Fig. 1. Upright film taken after the long intestinal, or ileus, tube had been inserted. The intestinal obstruction was released and double-contrast X-ray examination revealed an elevated polypoid tumor (arrows) located 250cm distal from the ligament of Treitz

Fig. 4. Cross section of the resected specimen revealed many dilated lymphatic vessels of varying sizes, mainly in the submucosa

Fig. 5. Higher-power view revealed cystic spaces separated by a thin septum and lined with endothelium, which contained a homogeneous pink-staining material and scattered lymphocytes. (H&E xl00) Fig. 2. Selective intestinogram. The sharply defined oval filling defect seen in the small intestine (arrows) measured 25 x 20 mm and had a vaguely nodular surface

365

T. Hanagiri et al.: Small Intestinal Lymphangioma Table 1. Reported cases of lymphangioma in the small intestine in Japan Case

Author

Year

A g e (years)

Sex

Location

1 2 3 4 5 6 7 8 9

W a t a n a b e et a1.16 Satou et al. 17 Furukawa et al. 18 Sugiura et a1.19 Miyamoto et al. ~° T a m u r a et al. 21 Nishimoto et al.13 Kuroda et al. 9 T a n a k a et al. 22

1967 1969 1970 1971 1974 1979 1981 1981 1981

34 38 11

M F F

35 32 40 29 55

M M M M F

J I I J J J I I I

10 11 12 13

Inoue et al. 3 Yoshioka et al. 23 Fujimaki et al. 12 Inoue et al. z4

1982 1984 1984 1984

56 21 51 6

F M F M

J, I J I J

14 15 16 17 18 19 20

Kitaguchi et al. ~5 K a m i b a y a s h i e t al. 26 H a t a n a k a et al. a7 Imai et al. ~8 Syundou et al. TM Kourogi et al. 29 Kourogi et al. ~9

1984 1984 1984 1985 1985 1985 1985

43

M

65 54 62 55 55

F M M M F

I J J I J J I

21

N a k a m u r a et al. 3°

1986

67

M

I

22 23

Y a m a m u r a et al. 31 Hoshika et al. 15

1986 1986

29 76

M F

J, I J

24 25 26 27

Niiyama et al. 32 E n o m o t o et al. 33 Shigematsu et al. 4 Shigematsu et al. 4

1988 1988 1988 1988

32 36 55 65

F M M F

J I J, I J

28 29 30 31 32 33 34

Shigematsu et al. 4 Shibata et al. 34 Kano et al. 8 Hayashi et al. 35 Koike et al. 36 Sawai et al. 37 Present case

1988 1989 1889 1989 1989 1990 1991

46 42 74 29 46 30 53

M M M M M F M

J I I I J J I

Size (cm) 7.0 x 5.0 × 1.0 7.5 x 7.3 10.0 × 20.0 16.0 × 7.0 3.8 × 2.9 Polyposis 1.5 × 1.1, 3.0 × 1.7 Polyposis 6.5 × 7.5 x 15 1.8 x 1.5 × 3.0

10.0 x 6.0 Polyposis 0.8 × 0.7 1.2 × 1.2 x 0.4 3.0 × 3.0 3.0 × 3.0, 1.5 x 1.5 1.0 × 0.6 × 0.6 Polyposis 1.1 × 0.9 × 0.6, 0.3 × 0.3 x 0.3

Polyposis 1.6 × 1.2, 1.1 × 0 . 7 1.5 × 0.8

Symptom M, A Intussusception P, V P, V Tumor Intussusception Intussusception P P, H, M P Torsion, chylous ascites M, A

M, A A , tarry stool A, Tarry stool Intussusception

Examination op op op

R R echo E R autopsy R

R, R, R, R R R, R

op op op op op op op

E' E E'

E

E' R

Endoscopic polypectomy op op

R R R, E R, E

op op op op

R, E

op op op op op op op

5.0 x 8.0 × 4.0 P A P, V P, V

op op op op

Intussusception

3.0 × 3.0 10.0 × 8.5 2.5 × 2.0

op op op

R, E R echo, R, E

E A, M P, diarrhea, constipation P, V P, V A, tarry stool

Therapy

R Echo, CT R

Abbreviations: 1, ileum; J, jejunum; A, anemia; M, melena; H, hematemesis; P, abdominal pain; V, vomiting; R, radiography; E, endoscopy; E', endoscopy during surgery

Discussion

Lymphangioma is one of the least frequently encountered types of benign tumor of the small intestine. Good s reported finding 16 cases of lymphangioma among a total 659 tumors of the small intestine in a 20 year period from 1938 through 1957 at the Mayo Clinic, while Fleming et al. 6 reported finding 9 cases of lymphatic cysts among 1,437,767 patients on whom 453,708 roentgenographic examinations of the gastrointestinal tract were performed between 1959 and 1968 at the Mayo Clinic. Three of these 9 cysts were located in the duodenum, 2 in the stomach, 1 in the jejunum, 1 in the descending colon and the other 2 patients had multiple cysts. In Japan, Yao et al. 7 reviewed primary neoplasms of the jejunum and ileum during a ten-year period from 1970 to 1979 and reported finding 3 cases

of lymphangioma among 273 cases of a benign neoplasms. Our review of the Japanese literature revealed a total 34 reported cases of lymphangioma in this country (Table 1). In most of these reported cases, the diagnosis was made coincidentally at the time of operation s or autopsy. 9 However, when symptoms are present, they can be divided into three basic types depending upon the type of tumor and its location, namely: (1) obstruction; 1° (2) bleeding; and (3) symptoms arising from local irritability. According to the reports, jejunal tumors most often manifest as a tarry stool or melena, followed by crampy abdominal pain, whereas ileal tumors may manifest as intussusception or subileus. 11-13 Some of these tumors have been detected by radiological and endoscopic examinations before the

366 o p e r a t i o n . 3"4 If the t u m o r is located in the p r o x i m a l p o r t i o n of the j e j u n u m or n e a r the ileal e n d , e n d o scopic e x a m i n a t i o n is a very useful m e t h o d for diff e r e n t i a l e x a m i n a t i o n . H o w e v e r , w h e n the t u m o r is located in the distal p o t i o n of the j e j u n u m , it is difficult to e x a m i n e by e n d o s c o p y . T h e radiological findings of l y m p h a n g i o m a have b e e n r e p o r t e d to p r e s e n t as a filling defect which reveals a r o u n d s m o o t h mass. 14,15 T h e findings are those of a s u b m u c o s a l i n t e s t i n a l t u m o r , mostly with overlying intact m u c o s a . T h e t u m o r shows a p r o p e n s i t y for c h a n g i n g its size a n d shape o n c o m P r e s s i o n or following the use of d o u b l e contrast m e t h o d s . T h e p e r f o r m i n g of selective i n t e s t i n o g r a m s by a long i n t e s t i n a l t u b e is a useful m e t h o d for detecting lesions of the small i n t e s t i n e , a n d w h e n the cause of i n t e s t i n a l o b s t r u c t i o n is u n e x p l a i n e d , radiological e x a m i n a t i o n by gastrografin is r e c o m m e n d e d . A f t e r the i n t e s t i n a l o b s t r u c t i o n has b e e n released, r e e x a m i n a t i o n with b a r i u m studies s h o u l d be p e r f o r m e d as in the p r e s e n t case.

References 1. Rainford TS (1932) Tumors of the small intestine. Arch Surg 25:122- 77 2. River L, Silverstein J, Tope JW (1956) Benign neoplasms of small intestine. Int Abst Surg 102:1-38 3. Inoue Y, Ohkubo N, Fukuzawa M, Imura K, Kitazume H, Miyata M, Okada T, Nakao K, Kawashima Y (1982) A lymphangioma found multiply in the duodenum, the upper jejunum, jejunal mesenterium and the ileum (in Japanese). Nippon Shokakibyo Gakkai Zashi 15:1411-1415 4. Shigematsu A, Iida M, Hatanaka M, Kohrogi N, Mastui T, Fujishima M, Itoh H, Fuchigami T, Iwashita A (1988) Endoscopic diagnosis of lymphangioma of the small intestine. Am J Gastroentero| 83:1289-1293 5. Good CA (1962) Tumors of the small intestine. Am J Roentg 25:122-77 6. Fleming MP, Carlson HC (1970) Submucosal lymphatic cysts of the gastrointestinal tract: A rare case of submucosal mass lesion. Am J Roentg 110:842-845 7. Yao T, Hiyoshi Y, Tanaka K, Fujita K, Yamamoto T, Hita K (1981) Primary benign neoplasm of the jejunum and ileum: Review of the Japanese literature (in Japanese). I to Chou (Stomach and Intestine) 16:1049-1056 8. Kano N, Yamada N, Hara S, Kayama H, Wada E, Inada K, Matsunami E, Kimura M, Ikeda T, Shimokawa K (1989) A case of lymphangioma in small intestine (in Japanese). Nippon Shoukaki Geka Gakkai Zasshi (Jpn J Gastroenterol Surg) 22:867-870 9. Kuroda M, Takano E, Yamamoto N, Kasahara M, Watanabe Y, Watanabe K (1981) An autopsy case of lymphangioma in the jejunum with multiple enchondroma (in Japanese). Nippon Byouri Gakkai Kaishi (Tr Soc Pathol Jpn) 70:394 10. Neidich GA, Mills K, Ohrt DW (1989) Intestinal lymphangiomas: A rare cause of intestinal obstruction. J Pediatr Gastroenterol Nutr 8:122-124 11. Lain A, Ternberg JL (1975) Cystic lymphangioma of the cecum with ileocecal intussusception: Case report. Am Surg 41:648-649

T. Hanagiri et al.: Small Intestinal Lymphangioma 12. Fujimaki E, Kano A, Orii S, Kato K, Sugai S, Katafuku K, Ataka R, Shozushima M, Tochinai H, Suzuki T (1984) Lymphangioma of the ileum: Report of a case (in Japanese). Gastroenterol Endosc 26:1337-1343 13. Nishimoto K, Sasaki N, Kawaguchi M, Muto H, Mizuuchi K (1981) A case of adult intussusception caused by lymphangioma of ileum (in Japanese). Hiroshima Igaku (J Hiroshima Med Assoc) 34:795-7 14. Syundo J, Ogawa T, Sakashita O, Takeuchi Y (1986) Jejunal lymphangioma. Report of a case (in Japanese). I to Chou (Stomach and Intestine) 21:565-568 15. Hoshika K, Uchida J, Kihara T (1986) Lymphangioma of small intestine (in Japanese). Sougon Rinshou (Clinic All-Round) 35:2223-2228 16. Watanabe M, Terasaki T (1967) A case of lymphangioma in the jejunum (in Japanese). Iryo 21:766-770 17. Satou J, Watanabe H, Maeda H, Yamaguchi K (1969) A case of lymphangioma in the ileum (in Japanese). Nippon Rynshou Geka Gakkai Zasshi. (J Jpn Soc Clin Surg) 30:577 18. Furukawa M, Tadashi Y, Saeki S, Uchimura M, Kondou N (1970) A case of lymphangioma in the jejunum (in Japanese). Geka Shinryou 12:1492-1494 19. Sugiura J, Nakayama T (1971) Lymphangioma in the jejunum. Report of a case (in Japanese). Nippon Shoukakibyou Gakkai Zasshi (Jpn J Gastroenterol) 68:67 20. Miyamoto Y, Kuroiwa M, Kohazu M, Oohama H (1974) Large cavernous lymphangioma of the jejunum and the jejunal mesentery (in Japanese). Okinawa Igakkai Zasshi (Okinawa Med J) 11:136-137 21. Tamura N, Furujyou H, Miyoshi Y, Fukumoto S, Hirayama S (1979) Lymphangioma of the small intestine. Report of three cases (in Japanese). Gastroenterol Endosc 21:1272 22. Tanaka K (1981) Roentgenologic diagnosis for the tumors of the small intestine (in Japanese). I to Chou (Stomach and Intestine) 16:971-990 23. Yoshioka K, Kubota H, Miyaka A, Yamada T, Nakamura N, Takada Y, Kawaguchi Y, Yamamoto M, Izumi H (1984) Lymphangioma of the small intestine. Report of a case (in Japanese). Geka (Surg) 46:657-659 24. Inoue S, Murakami T, Sugiyama Y, Tsuchida H, Yamagata N (1984) Cystic lymphangioma and massive chylous ascites with volvulus of common mesentery. Report of a case (in Japanese). Nippon Shouni Geka Gakkai Zasshi (J Jpn Soc Pediatr Surg) 20:1269 25. Kitaguchi Y, Nakano H, Sasaki K, Kitagawa Y, Gotoh M (1984) Lymphangioma in the ileum, report of a case (in Japanese). Nippon Shoukakibyou Gakkai Zasshi (Jpn J Gastroenterol) 81:2680 26. Kamibayashi S, Aratani K, Mitsui T, Kobayashi O (1984) Lymphangioma in the jejunum (in Japanese). Nippon Shoukakibyou Gakkai Zasshi (Jpn J Gastroenterol) 81:1347 27. Hatanaka M, Iida M, Matsuzaka T, Iwashiro A, Iwashita A (1984) Multiple lymphangioma of the small intestine. Report of a case (in Japanese). Nippon Shoukakibyou Gakkai Zasshi (Jpn J Gastroenterol) 81:1322 28. Imai Y, Sugino Y, Sekine M, Kumakura K (1985) Submucosal tumor of the small intestine (in Japanese). Nippon Igaku Houshasen Gakkai Zasshi (Nippon Act Radiol) 45:118 29. Kourogi N, Iida M, Itou H, Shigematsu A, Kuroiwa S, Kimura Y (1985) Lymphangioma of the small intestine (in Japanese). Report of two cases. Gastroenterol Endosc 27:1452 30. Nakamura M, Kikuchi N, Hoshino K, Ohno G, Namiki M (1986) Lymphangioma of the distal ileum removed endoscopically. Report of a case (in Japanese). Gastroenterol Endosc 28: 2616-2620 31. Yamamura T, Kouno T, Satou M, Mita K, Yamamoto T, Koyama M, Kawahara H, Soejima K, Kamimura N (1985) Lymphangioma of the small intestine. Report of a case (in Japanese). Geka Shinryou 28:223-226

T. Hanagiri et al.: Small Intestinal Lymphangioma 32. Niiyama T, Misono T, Yamaguchi K, Baba Y, Yoshikawa J (1988) Lymphangioma of the small intestine. Report of a case (in Japanese). Nippon Shoukakibyou Gakkai Zasshi (Jpn J Gastroenterol) 85:1615 33. Enomoto H, Yamashita Y, Honda M, Yoshida M, Ono T, Asada S, Kojima T (1988) Lymphangioma of the ileum and the mesentery of the ileum. Report of a case (in Japanese). Nippon Shoukaki Geka Gakkai Zasshi (Jpn J Gastroenterol Surg) 21:945-948 34. Shibata H, Yanagisawa N, Niino N, Hanamura N, Fujita H (1989) Lymphangioma of the ileum. Report of a case (in Japanese). Shinsyu Igaku Zasshi (Shinsyu Med J) 37:413 35. Hayashi H, Satou T, Henmi A, Watanabe O, Matsumura M

367 (1989) Lymphangioma of the small intestine. Report of a case (in Japanese). Juntendou Igaku (Juntendou Med J) 35: 118 36. Koike M, Ishiyama T, Wakabayashi Y, Hirose S, Kitamura S, Kuwabara N (1989) Jejunal hamartomatous lymphangioma revealed iron deficiency anemia as a main symptom (in Japanese). Naika (Internal Medicine) 64:1165-1167 37. Sawai T, Nakao S, Ishii T, Eida K, Noguchi K, Fujita A, Kurouji K, Abe K, Takahara O (1990) A case of lymphangioma of the jeiunum and a review of 28 cases in Japan (in Japanese). Nippon Rynsyou Geka Gakkai Zasshi (J Jpn Soc Clin Surg) 51:327330

Lymphangioma in the small intestine: report of a case and review of the Japanese literature.

We report herein a rare case of a 53 year old man with a benign lymphangioma of the small intestine. He presented with a complete obstruction of the s...
1MB Sizes 0 Downloads 0 Views