Hemangioma of the Colon MAJ Harold D. Head, MC, Washington, DC MAJ John Q. Baker, MC, Washington, DC COL Robert W. Muir, MC, Washington, DC
Vascular malformations of the gastrointestinal tract have been reported since 1839 when Phillips Ill described the case of a servant who had three severe hemorrhages from a walnut-sized rectal t u m o r . Although vascular malformations have been found in virtually every human organ system, hemangiomas of the colon and rectum are quite uncommon. Only fifty cases of hemangioma confined to the intra-abdominal colon have been described in the world literature (Table [), including thirty-one cases tabulated by Gentry, Dockerty, and Clagett [2] in 1959 in their extensive review of 283 benign vascular tumors of the gastrointestinal tract. Eight additional cases of hemangioma of the colon have been found in a review Of the recent literature [5-II], including four of the rectosigmoid colon. The present case report is illustrative of this unusual malformation.
Case Report A twenty-two year old soldier was admitted to USAH Heidelberg, Germany, in March 1972 because of intermittent hematochezia for four months. The patient first noted bright red blood in the stool five months prior to admission, and he subsequently had intermittent episodes of painless bleeding. He was hospitalized in March 1972 after having a large, bloody bowel movement associated with. dull, left lower abdominal pain. On admission the stool was guaiac-positive and
From the Department of Surgery, Walter Reed General Hospital, Walter Reed Army Medical Center, Washington, DC. Reprint requests should be addressed to Dr Head, [)epartment of Surgery, Walter Reed General Hospital, Wallet Reed Army Medical Center, Washington, DC 20012,
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the hematocrit was 44 per cent. A plain radiograph of the abdomen demonstrated numerous lower abdominal phleboliths (Figure i), which were subsequently found to be associated with a large constricting mass of the sigmoid colon on barium enema. He was transferred for further evaluation to USAGH Landstuhl, Germany, with a presumptive diagnosis of hemangioma of the sigmoid colon. At this time the stool was guaiac-negatire, and the hematocrit was 43 per cent. A percutaneous arteriogram with selective injection of the inferi. or mesenteric artery demonstrated a hemangioma of the sigmoid colon (Figure 2), and he was transferred to Walter Reed General Hospital. A complete review of the patient's past and family history was noncontributory, and physical examination including proctosigmoidoscopy to 25 em showed no abnormalities. On April 7, 1972 he was taken to surgery at which time a 6 by 13 cm venous angioma of the sigmoid colon was identified and segmentally resected. (Figure 3.) A radiograph of the resected specimen revealed the phleboliths that were apparent on preoperative flat plate of the abdomen. (Figure 4.) His postoperative course was uneventful, ~/nd he has subsequently had no further rectal bleeding. TABLE I Hemangioma of the Colon and Rectum: Summary of Reported Cases
Author
Colon
Gentry, Dockerty, 31 and Clagett [2] Rissier [3] 11 Condon and Loyd [4] 8 Total 50
RecRec- tosigturn moid 45
12
18 ... 63
14 1 27
Multiple Involvement* 37 (appendix 5) 9 ... 46
*Multiple organ system involvement including colon, rectum, or both,
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Pathology An early classification of benign vascular malformations was proposed by Obendorfer and popularized in 1936 by Kaijser [12], as follows: I. Multiple phlebectasia II. Cavernous hemal~gioma A. Diffuse infiltrating type B. Polypoid type III. Capillary hemangioma (simplex) IV. Special group: gastrointestinal localization, hereditary or nonhereditary
Figure 1. Admission. radiograph o f abdomen demonstrating numerous phleboliths in lower abdomen.
Figure 3. Gross appearance of the sigmoid colon with a 6 by 13 cm venous angioma at operation.
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Despite its widespread use, the ObendorferKaijser classification has met several criticisms. No distinction is made between the benign hemangiomas and their rare malignant counterparts, and there is no clear histologic differentiation between multiple phlebectasias and many of the multiple, hereditary hemangiomas of the special group. Thin-walled vessels of capillary and cavernous sizes are often present in the same tumor, and the classification of benign hemangiomas presented by Gentry, Dockerty, and Clagett [2] includes this group of "mixed" hemangiomas:
Figure 2. Venous phase of selective inferior mesenteric arteriogram demonstrating "puddling" of contrast material in the area associated with phleboliths.
Figure 4. Radiograph of resected angioma containing calcified venous thrombi identified as phleboliths on preoperative radiographs.
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Hemangioma of Colon
I. Capillary (simplex, mostly single) II. Mixed III. Cavernous A. Multiple phlebectasias (small cavernous) B. Simple polypoid (single cavernous) C. Diffuse expansive (single, contiguous) D. Diffuse expansive (multiple, noncontiguous) The Armed Forces Institute of Pathology (Landing and Farber [13]) includes venous angiomas in its list of benign vascular malformations as well as arteriovenous angiomas (racemose aneurysms), plexiform angiomas, and several other variants of hemangiomas. The pathogenesis of these tumors is not weJl defined, although they are generally agreed to be congenital with their origins in embryonic sequestrations of mesodermal tissue. Whether these growths are neoplastic or congenital is a matter of controversy, but enlargement occurs by projections of budding endothelial cells. The capillary hemangioma consists of small, thin-walled, closely packed vessels with a well differentiated, hyperplastic endothelial lining. These tumors are distributed equally throughout the gastrointestinal tract, and comprise 6 per cent of benign vascular tumors. They arise from the submocosal vascular plexus and are often encapsulated. Mucosal ulceration occurs in half of these lesions. The cavernous hemangioma is composed of large, thin-walled vessels which are much larger than those of the capillary hemangioma. The supporting stroma contains scant connective tissue and may contain smooth muscle fibers. These lesions may be of the "multiple phlebectasia" type, characterized by a multitude of small (less than 1 era) discrete tumors found in any or all parts of the intestinal tract. Although they are often overlooked, they constitute one third of all the benign vascular tumors of the gastrointestinal tract. The simple polypoid type of cavernous hemangioma represents 10 per cent of benign vascular intestinal malformations and is usually of sufficient size to produce symptoms such as ulceration, hemorrhage, and luminal obstruction. The diffuse expansive type of cavernous hemangioma is quite variable in size a n d s h a p e and frequently involves 20 to 30 cm of intestine, occasionally in multiple locations. Diffuse cavernous hemangiomas comprise about 20 per cent of intestinal angiomas and produce severe symptoms at a relatively early age. Mixed hemangiomas are characterized microscopically by areas resembling capillary hemangi-
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omas which are interspersed with large vessels similar to those of cavernous hemangiomas. They represent 6 per cent of benign vascular tumors, and most of them occur in the stomach, small intestine, or appendix. Venous angiomas are frequently confused with cavernous hemangiomas since both are composed of large, thin-walled vessels with large lumens. The walls of these vessels, however, contain various amounts of smooth muscle and usually resemble veins. Many of these tumors are extensive, especially those in extremities. Thrombosis is common in venous as well as in cavernous hemangiomas, and calcification often occurs in these thrombi and in the surrounding interstitial tissue. Comments
The great majority of patients who have symptoms of hemangioma of the colon present early in adult life, or even in childhood, with recurrent, painless, and often massive rectal bleeding. The age at onset, the type of bleeding, and the frequency and severity of hemorrhage are related to the size, number, and type of vascular malformations. Capillary hemangiomas may present with slow and persistent bleeding, producing melena and anemia, or they may be incidental findings at autopsy. Diffuse cavernous hemangiomas, conversely, may present very early i n life with severe and even fatal hemorrhage. Rectal bleeding is frequently painless and usually more severe with each dpisode. The incidence of bleedingin colorectal hemangioma is reported as 60 to 90 per cent, and the early onset and frequency of hemorrhage often lead to diagnosis in adolescence or early adulthood, as typified in the case reported herein. Complete or partial intestinal obstruction occurs in about 17 per cent of cases of hemangioma of the colon and may be the result of luminal occlusion due to the size of the tumor, intussusception, or volvulus. Involvement of the rectum by hemangioma, as by other rectal tumors, may produce tenesmus, rectal urgency, and incomplete defecation. The diagnosis of hemangioma of the colon is likewise related to the type, size, and location of the tumor. A family history of such malformations and the finding of similar mucocutaneous lesions may occasionally lead to the diagnosis of a gastrointestinal hemangioma. Anemia, frank tmmorrhage, or signs of obstruction will frequently lead to further diagnostic investigation or abdominal exploration. If there is rectal involvement, the tumor may be palpable on digital examination or
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it may be visible through the proctoscope as a bluish, nodular mass. Calcifications within hemangiomas occasionally occur, and the presence of multiple phleboliths in a young patient should arouse suspicion of a vascular malformation. Hollingsworth [14J first documented the association of a narrowing of the sigmold colon on barium enema with an area of surrounding phleboliths and was thus able to make the diagnosis of a vascular tumor in a nine year old girl. Selective aagiography may also reveal a vascular malformation, particularly in the late vascular phases, All of these radiographic findings served to establish the diagnosis of a vascular malformation of the colon in this patient, and the phleboliths were radiographically apparent in the resected specimen. (Figure 4.) The treatment of a symptomatic intestinal hemangioma is primarily surgical. Small malformations can sometimes be excised submucosally with preservation of the seromuscular bowel wall. In larger lesions, if the surgeon is assured that the tumor is benign, local segmental resection is curative. If malignancy cannot be excluded after adequate frozen section examination, radical extirpation of the segment with its mesentery should be undertaken. Malignant vascular tumors include hemangioendothelioma, angiosarcoma, Kaposi's sarcoma, and "benign metastasizing hemangioma," and represent approximately 13 per cent of all vascular lesions of the cotorectum. Large, diffuse cavernous hemangiomas and multiple hemangiomas frequently require extensive colectomy for cure, including abdominoperineal resection for rectosigmoid hemangiomas. Nonresective management of intestinal hemangiomas may occasionally be necessary because of the extensive involvement of the lesion or because the patient is a poor surgical risk. Intestinal diversions have been performed for these lesions, but hemorrhage remains a constant threat. Diverting colostomy combined with high voltage radiotherapy has been successful in a few instances. The use of sclerosing agents has seldom been successful, and |igation of the blood supply of the tumor, with its danger of intestinal necrosis, is seldom helpful.
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Summary Hemangioma is a rare lesion of the colon, with only fifty-five cases being reported in the world literature. The histopathologic characteristics of these tumors are reviewed, and an additional case of a sigmoid hemangioma occurring in a young adult is described. Rectal hemorrhage is the most frequent symptom of hemangioma of the colon, and the diagnosis may often be confirmed by selective angiography. The presence of multiple phleboliths and a filling defect on barium enema is highly suggestive of a cavernous or venous hemangioma. Complete surgical extirpation, when possible, is the treatment of choice.
Referenr, es 1. Phillips B: London Med Gaz 1: 514, 1639. 2. Gentry RW, Dockerty MB, Clagett OT: Vascular malformations and vascular tumors of the gastrointestinal tract. Int Abstr Surg 88: 281, 1949 3. Rissier HL: Hemangiomatosis of the intestine: discussion, review of the literature, and report of two new cases. Gastroenterologia (Basel) 93: 357, 1960. 4. Condon R, Loyd D: Hemangioma of the colon. Am J Surg 115: 720, 1968. 5. Gotdlust D, Chalat J, Rault JJ, et al: Recto-sigmoid hemangiomatosis. J Radiol Electrol Med Nuct 52: 108, 1971. 6. Mot{ FK, Dortenmann J: Hemangioma of the colon. Med Welt 45: 2483, 1968. 7. Paris J, Goudemand M, Leduc M, et al: Isolated angioma of the Tight colon with repeated gastrointestinal hemorrhage for 15 years. Lille Med 12: 592, 1967. 8. Ragulin VS: Capillary hemangioma of the large intestine. Vestn Khir 97: 129, 1966, 9, Reiss H, Ry~ K: Extensive angioma of the colon and rectum of mixed structure, Pol Przegl Chit" 43:115, 1971. 10. Stening SG, Heptinstall DP: Diffuse cavernous haemangioma of the rectum and sigmoid colon. Br J Surg 57: 186, 1970. 11. Upson JF, Bunnell J, Kokkinopoulis E: Hemangioma of the cecum: diagnosis by angiography. JAMA 217: 1104, 1971. 12. Kaijser R: Hemangioma of the gastrointestinal tract. Arch Klin Chit 187: 351, 1936. 13. Landing BH, Farber S: Tumors of the cardiovascular system, fast 7, p 45. Atlas of Tumor Pathology. Washington, D C , Armed Forces Institute of Pathology, 1956. 14. Hollingsworth G: Haemangiomatous lesions of the colon. Br J Radio124: 220, 1951.
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