Hachiya Tsutomu

Matsuoka Sadayuki

Ohta Hitoya

Shintaku Masayuki

Two cases of cavernous lymphangioma arising in the intestinal wall are reported. The first case, a 7 A-year-old man, had an intramural cavernous lymphangioma in the duodenum detected on computerized tomographic examination and magnetic resonance imaging. The second case, a 59-year-old woman, had a large polypoid cavernous lymphangioma in the ascending colon detected on barium enema examination. No clinical symptoms referrable to lymphangiomas were found in both cases. Radiological examination is thought to be a useful mea-ns of detecting lymphangiomas of the intestines, although preoperative diagnosis of the exact nature of these lesions can be difficult because of their extreme rarity.technical repor

Shintaku, Masayuki

Hachiya, Tsutomu

Matsuoka, Sadayuki

Ohta, Hitoya

Japanese Red Cross Repository / 赤十字リポジトリ

Cavernous Lymphangioma of the Intestines:Report of Two Cases (日赤医学 第48巻 第2号 1996 9 15)(247)く症 例 告〉Cavernous Lymphangioma of the lntestines :Report of Two CasesMasayuki Shintaku, M.D.Department of Pathology, Osaka Red Cross HospitalTsutomu Hachiya, M.D.Department of Health Care, Osaka Red Cross HospitalSadayuki Matsuoka, M.D.Department of Surgery, Osaka Red Cross HospitalHitoya Ohta, M.D.Department of Laboratories, Osaka Red Cross HospitalABSTRACTTwo cases of cavernous lymphangioma arising in the intestinal wall are reported. The first case, a 7 A-year-old man, had an intramural cavernous lymphangioma in the duodenum detected on computerized tomographicexamination and magnetic resonance imaging. The second case, a 59-year-old woman, had a large polypoidcavernous lymphangioma in the ascending colon detected on barium enema examination. No clinical symptomsreferrable to lymphangiomas were found in both cases. Radiological examination is thought to be a usefulmea-ns of detecting lymphangiomas of the intestines, although preoperative diagnosis of the exact nature ofthese lesions can be difficult because of their extreme rarity.Key words : lymphangioma, duodenum, colonINTRODUCTIONLymphangiomas arising in the abdominal organs are rare, and the majority of them arises inthe mesentery, omentum, mesocolon, and retroperitoneumr). Those arising in the wall of thegastrointestinal tract are even more rare, and according to Davis et al.2) only 39 cases ofintestinal lymphangioma have been reported until 1987. Although the diagnosis of lymphan-giomas is usually established by pathological examination, the preoperative radiologicalexamination occasionally enables the'precise delineation of the lesions and suggests correctdiagnosis.We present here the radiological and pathological features of two adult cases of abdominalcavernous lymphangioma arising from unusual locations. In the first case, the tumor was foundwithin the duodenal wall on computerized tomographic examination (CT) and magneticresonance imaging (MRI). In the second case, the tumor had grown to a large pedunculatedpolyp of the ascending colon and was detected on barium enema examination.CASE REPORTCasθ I: The patient was a 74-year―old man,who presented、vith lower abdominal pain and(248) Masayuki SHINTAKU, Tsutomu HACHIYASadayuki MATSUOKA, Hitoya OIITAFig.1 Case 1. CT scan with contrast en-hancement of the abdomen demon-strated a round, non-enhanced cysticmass outside of the duodenal loop.Fig.2 & 3 Case 1. MRI of the duodenal cystic lesion showed a low signal intensity on T1-weighted image(Fig.2) and a very high signal intensity on T2-weighted image (Fie.3).Fig.4 Case 1. Gross appearance of theduodenal tumor at autopsy. Thetumor showed a multilocular cysticappearance with a smooth transpar'ent surface.lumbago. Thorough examination revealed adenocarcinoma of the lung with metastasis to thevertebral column and retroperitoneum. CT of the abdomen disclosed a low density masswithout contrast enhancement, measuring about 4 cm in diameter, just lateral to the duodenalloop (Fig. 1). On MRI, the lesion showed a low signal on T1 weighted image (600/13) (Fig. 2)and a very high signal on T2-weighted image (2500/80) (Fig. 3). Ultrasound-guided punctureof this cystic lesion yielded clear, serous fluid. The patient developed paraplegia, then died ofcarcinomatous cachexia and bronchopneumonia. Autopsy revealed extensive metastasis ofpulmonary adenocarcinoma to various organs.In the lateral wall of the second portion of the duodenum, a multilocular cystic tumor.measuring 4.5x3.5x3.5 cm was found outside of the duodenal muscle layer (Fig.4). It had aCavernous i-y'nrphangioma of the lrrtestinesReport of Two Cases (日赤医学 第48巻 第2号 1996 9.15)(249)Fig. 6 Case 2. Barium enema examinationshowed a large pedunculated polypoidlesion in the ascending colon. (P-A view)smooth transparent surface and containedclear serous fluid. On cut section, the tumorwas comprised of aggregates of tiny irregularspaces, each measuring a few millimeters indiameter, which were separated from eachother by thin fibrous septa.Histopathologically, the tumor consisted oflymphatic spaces of irregular shapes andsizes separated by fibrous septa occasionallycontaining small amounts of smooth musclefibers (Fig. 5). Each space was lined by asingle layer of bland-appearing, flat endoth-elial cells. Mild, focal lymphocytic infiltra-tion was noted in the septa.Case 2: The patient was a S9-year-oldwoman, who presented with anal bleedingthat persisted for two months. Bariumenema and endoscopic examination revealeda large, circumferentially protruding lesion inthe sigmoid colon, and biopsy specimenstaken from this lesion were diagnosed asCase 1. The tumor consisted of irregularlymphatic spaces lined by f lat endothelialcells. The spaces were separated by thinfibrous septa. (Hematoxylin - Eosinstain, X25)Case 2. ln the mucosal portion of thetumor, many intestinal crypts separatedthe dilated lymphatic spaces. (Hematox-ylin-Eosin stain, x 10)貯ずず臀ト′r 、ヽ、Fig.8 Case 2. Lymphatic spaces with irregularshapes and sizes were lined by flat endoth-elial cells and contained palely eosinophilicfluid. (Hematoxylin-Eosin stain, x25)膠心ダ鰺 ???(250) Masayuki SHINTAKU, Tsutomu HACHIYASadayuki MATSUOKA, Hitoya OHTAadenocarcinoma. While the fiberscope could not be passed beyond this lesion, barium enemastudy depicted a large pedunculated polyp having a smooth surface and a long stalk in theascending colon (Fig. 6). Sigmoidectomy and resection of the polyp by means of colotomy wasperformed, and the patient recovered uneventfully. Hypoalbuminemia and other symptoms andsigns suggestive of a protein-losing enteropathy were not found.The resected polyp measured 25xl6x 17 mm and had a yellowish, transparent smoothsurface. It was connected to the colonic mucosa with a thin stalk approximately 24 mm long.On cut section, the polyp, which showed a honeycomb appearance, consisted of aggregates ofmicrocysts, each measuring less than2 mm in diameter and containing clear fluid. The centralarea of. the polyp was slightly hard and whitish.Histopathologically a large number of irregularly shaped lymphatic spaces filled with palelyeosinophilic fluid were found extending from the lamina propria to the submucosal region of thepolyp (Fig. 7). Each of these spaces was lined by flat endothelial cells showing no cytologicalatypism and separated by a thin fibrous septum (Fig.8). In the intramucosal portion of thetumor, each lymphatic space was separated by intervening mucosal tissue containing intestinalcrypts. Variable amounts of smooth muscle fibers, some of which were in continuity with themuscularis mucosae around the lesion, were found in the septa.DISCUSSIONThe two cases presented here showed almost typical gross and microscopic features ofcavernous lymphangioma. In the second case, histopathological appearance of the tumor wassomewhat unusual in that the tumor involved the lamina propria extensively and each dilatedlymphatic channel was separated by mucosal tissue containing intestinal crypts. Lymphan-giomas of the intestines are usually located in the submucosa with the overlying mucosaremaining intact3)-s). The diagnostic possibility of intestinal lymphangiectasia6rT)was consid-ered in this case, but similar lymphangiectatic lesions were not found either in the stalk of thepolyp or in the resected specimen of the sigmoid colon, and there were no other clinical orradiological features suggesting the presence of intestinal iymphangiectasia.Most of the examples of lymphangioma of the abdominal cavity arise in the mesentery'r'),and those arising within the intestinal wall or presenting as intraluminal polypoid lesions arevery rareoru). Lymphangiomas are in general regarded as a kind of hamartomatous ormalformative lesions, and many of them are detected during childhood'). However, intra-abdominal lymphangiomas found in adults are not uncommon. All reported cases of lymphan-gioma of the large intestine which were reviewed by Berardiu)had been found in adults. Theseare occasionally asymptomatic and found incidentally at autopsy or surgical exploration forother reasons'). Because of its extreme rarity, specific preoperative radiological diagnosis oflymphangiomas of the intestines is very difficult, even if the presence of lesions can be detected.According to a review by Berardis), none of the cases of lymphangioma of the large intestinewas diagnosed preoperatively. Elucidation of the exact nature of the lesions must usually awaitpathololgical examination of the resected specimens.In our first case, the cystic nature of the duodenal lesion was clearly demonstrated by CT,and the MRI indicated that the content of the cyst was not viscid. Clear, serous fluid wasobtained by puncture of the lesion. From these findings, duplication cyst of the duodenum andmicrocystic adenoma of the pancreatic head were considered the most likely diagnosticpossibilities. The extreme rarity of lymphangioma arising in the duodenum'r') prevented theCavernous Lymphangioma of the Intestines:Report of Two Cases (日赤医学 第48巻 第2号 1996.9.15)(251)correct antemortem diagnosis. Accordi.,g to Davis et al.2) only eight of 39 reported casesinvolving lympangioma of the intestines arose in the duodenum. Lymphangioma should beincluded in the list of radiological differential diagnosis of intramural tumors of the intestines,although its incidence is extremely low.In the second case, polypoid lesion of the ascending colon was considered radiographicallymost likely to be an epithlial (adenomatous) polyp. In a series of lymphangiornas of the iargeintestine reviewed by Berardis), most of the cases were diagnosed as adenomatous polyp oradenocarcinoma preoperatively. However, the mucosal surface of polypoid lesions is unusualll,smooth, which, as noted earlier by Arnett and Friedman3), is rather suggestive of submucosalmesenchymal neoplasm, especially lipoma.It is generally agreed that lymphangiomas are hamartomatous or malformative lesions whicharise from sequestrations of lymphatic tissue that failed to communicate normally with thelymphatic SyStemrrz). Most of them are considered present at birthl). However, in cases oflymphangioma appearing late in adulthood, the possibility that the lesions have developedsecondarily from acquired lymphangiectasia resulting from local disturbance of lymphaticcirculation is not excludedto). In our first case, extensive metastasis of pulmonary adenocar-cinoma was found in the vertebral column and the surrounding retroperitoneal tissue. It islikely that the local disturbance of lymphatic circulation caused by metastatic carcinomatriggered lymph stasis and secondary lymphangiectasia which in turn caused the formation ofa lymphangioma in that very unusual location.REFERENCES1 . Enzinger FM & weiss SW: Soft tissue tumors, 3rd ed., Mosby, St. Louis, 19g4, p. 6T9-700.2 . Davis M, Fenoglio-Preiser CM, Haque AK: Cavernous lymphangioma of the duodenum.case report and review of the literature. Gastrointest Radiol 12 10-12, lg}7.3 . Arnett NL & Friedman PS: Lymphangioma of the colon : Roentgen aspects. A casereport. Radiology 67: 882-885, 1956.4 . Greene EI, Kirshen MM, Greene JM: Lymphangioma of the transverse colon. Amer JSurg 103: 723 726,1962.5 . Berardi RS: Lymphangioma of the large intestine. Report of a case and review of theliterature. Dis Colon Rectum 17: 265-272,1974.6 . Ivey K, DenBesten L, et al. : Lymphangiectasia of the colon with protein loss and malabsor-ption. Gastroenterol 57 : 709-714, 1969.7 . Fenoglio-Preiser CM, Pascal RR, Perzin KH: Tumors of the intestines. Atlas of TumorPathology, Second series, Fasc. 27. Armed Forces Institute of Pathology, Washington, D.C., 1990, p.494-500.8 . Daniel S, Lazarevic B, Attia A: Lymphangioma of the mesentery of the jejunum. Reportof a case and a brief review of the literature. Amer J Gastroenterol 78: 726-729, 1983.9 . Elliott RL, Williams RD, et al.: Lymphangioma of the duodenum. Case report with lightand electron microscopic observation. Ann Surg 163: 86 92, 1966.10. Fisher I & Orkin M : Acquired lymphangioma (lymphangiectasis). Report of a case. ArchDermat l0l: 230-234. 1970.*tt'e6.10.11

Cavernous Lymphangioma of the lntestines :Report of Two Cases

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Cavernous Lymphangioma of the lntestines :Report of Two Cases

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