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Hemangioma of the colon
Hemangioma ROBERT E. CONDON,
M.D. AND
of the Colon R. DANIEL LOYD, M.D., Houston, Texas
From the Surgical Service, Veterans Administ, ation Hospital, Houston, Texas, and the Cora and Webb Mading Department of Surgery, Baylor University College of Medicine, Houston, Texas.
tive diagnosis and treatment during adolescence or early adulthood. This report of a case of hemangioma of the colon concerns a patient who had an atypical history. His symptoms first occurred late in life since there was no gastrointestinal bleeding or other symptoms until he was sixty-four years old. Bleeding from a single small tumor had produced severe symptoms of anemia and had precipitated heart failure.
EMANGIOMA is a common skin tumor, but is unusual in the gastrointestinal tract, and is one of the rarest benign tumors found in the colon. Gentry, Dockerty, and Clagett [I 1, reviewing the world literature through 1935, summarized 255 cases of hemangioma involving the gastrointestinal tract; in only thirty-eight cases did the lesion involve the intra-abdominal colon. Rissier [Z] tabulated eighteen cases and reports of eight other cases were found in our survey of the literature [3-9 1. Gastrointestmal hemangiomas may enlarge sufficiently to produce symptoms of obstruction in a minority of patients, and intussusception has been reported, particularly in cases from eastern Europe. The commonest complication of hemangioma of the colon is bleeding, the reported incidence varying from 60 to 90 per cent. Characteristically, colonic hemangiomas bleed episodically, slowly and persistently so that the symptoms complained of are melena and the consequences of anemia, that is, weakness and fatigue. The degree of bleeding usually correlates with the size, number, and type of hemangioma, cavernous hemangiomas tending to bleed massively much more frequently than capillary hemangiomas. The bleeding is painless and not accompanied by diarrhea unless precipitated by an intercurrent enteritis. Typically, since these tumors are congenital in melena begins in childhood and is origin, recurrent throughout adolescence, resulting in intermittent symptomatic anemia. The bleeding tends to become more severe with each recurrent episode. The early onset of bleeding and its recurrent nature usually lead to defini-
H
CASE REPORT The patient (D.H., No. 419~0%i741), a sixtyfour year old man, was referred for treatment of anemia and after a barium enema examination gave abnormal results. About eight months previously the patient first noted dyspnea on exertion and began to have fainting spells during which he became tremulous and fell to the floor, allegedly without losing consciousness. His exertional dyspnea progressed in severity and paroxysmal nocturnal dyspnea and swollen ankles and feet developed. Administration of digitalis and diuretics by his physician failed to alleviate the symptoms. Five months prior to admission he fractured his ankle during a fainting spell. At that time he was found to have a hematocrit of 28 per cent, the red blood cells were hypochromic and microcytic, serum iron was 33 pg. per cent, and stools were positive for occult blood. The patient was treated for fracture of the ankle, two units of whole blood were given, and oral iron therapy was begun. No investigation was made of the gastrointestinal tract. The fainting spells were relieved for a short time, but dpspnea and edema persisted. In the four month interval prior to admission, the patient first noticed intermittent tarry stools and sustained a weight loss of 15 pounds. A barium enema examination was carried out and his physician noted a “suspicious alea in the cecum” and referred the patient to us. The patient denied having had melena in the past and also denied having had diarrhea or mucoid stools during this present illness. There was no history of antecedent heart disease. Four years previously he 720
American
Journal
of Surgery
Hemangioma of Colon had had a small subcutaneous nodule excised from his forearm. This initially had been reported to be a pyogenic granuloma but a current reappraisal of the biopsy showed the tissue to be a hemangioma. There was no history of hemangioma in any memhers of his family. The patient had been admitted several times to this hospital for psychiatric treatment. His hematocrit on each such occasion was normal, the last admission having been three years previous. He was thin, pale, and looked chronically ill. His blood pressure was 140/75 mm. Hg, and the pulse rate was 95 per minute and regular. The jugular veins were distended 2 to 3 cm. above the clavicle with the patient in a sitting position. There was a grade II/VI systolic ejection murmur audible along the left sternal border. No gallop was detected. There was firm, pitting edema of the lower part of the legs extending up to the knees. There were no cutaneous or oral lesions. The remainder of the physicial examination and sigmoidoscopy to 25 cm. revealed nothing abnormal. The initial hemoglobin was 6.2 gm. per cent, hematocrit 23 per cent, and the total and differential leukocyte count was normal. Hypochromic, microcytic erythrocytes were found on smear examination, and the reticulocyte count was 6.5 per cent. Stools were strongly positive for occult blood. The serum iron was 17 pg. per cent and the iron binding capacity 393 pg. per cent. Serum total protein was 6.6 gm. per cent and albumin 3.6 gm. per cent. The
barium enema showed no abnormality of the cecum, but did demonstrate a filling defect in the descending colon. (Fig. 1.) All other laboratory and x-ray investigations gave normal results. Abdominal exploration was carried out after appropriate preparation of the patient. A soft nodule was palpable in the midportion of the descending colon; there were no other abnormal findings. A sessile smal1 tumor, 1.8 cm. in maximal dimension and surrounded by fresh and clotted blood, was visualized through a colotomv. This tumor was not ulcerated. The tumor was excised submucosally and its henign nature confirmed by frozen section examination. Permanent sections showed a small localized cavernous hemangioma. (Fig. 2.) There were no postoperative complications and the patient was discharged one week after operation. His symptoms of dyspnea and edema of the ankles disappeared. He noted increased vigor and gamed 10 pounds in weight during the first postoperative month. Melena has not recurred, and the patient remains asymptomatic. COMMENTS
In patients with hemangiomas of the colon presenting with painless rectal bleeding it may be difficult to make a diagnosis prior to operative exploration of the abdomen. Of course, a Vol. 115, May 1968
721
FIG. 1. Barium enema examination demonstrating filling defect in the descending colon. The remainder of the intra-abdominal colon was well visualized films and no other lesions were found.
on other
positive family history of a similar problem may be helpful. Physical examination is often unremarkable, except for signs of anemia, but the presence of hemangiomas of the skin or mucous membranes should raise a suspicion that a similar lesion might be present in the colon. Barium enema examination may reveal a filling defect, as in our case, and, infrequently, phleboliths may be noted within the filling defect. The occurrence of phleboliths is probably related to thrombosis within the tumor and is seen particularly with cavernous hemangiomas of the colon [g-11], but was not present in our patient. This patient is distinctly unusual in that his symptomatic history began at sixty-four years of age. He could recollect no episodes of rectal bleeding or symptoms of anemia in the past. The absence of anemia had been confirmed on several occasions in the last decade during admissions to the psychiatric wards. The most likely explanation for this atypical history is that the patient’scongenitalcolonichemangioma must have been very small and had grown exceedingly slowly, if at all, during most of his life. Recently, it probably underwent a period
722
Condon
and Loyd
FIG. 2. Representative portion of submucosal cavernous hemangioma excised from the colon. The overlying mucosa was intact and histologically normal. The blood-filled vascular spaces lined by endothelium and supported by a fibrous stoma are characteristic of cavernous hemangioma.
of rapid growth leading to the onset of colonic bleeding. Hemangiomas in other sites are known to undergo irregular periods of rapid acceleration in growth [I]. Major blood loss resulting in severe anemia is more usual with diffuse and extensive involvement of the colon by cavernous hemangioma. Our patient had marked symptoms both of diminished red blood cell and total blood volume (exertional dyspnea and fainting) and of congestive heart failure (dyspnea and peripheral edema in the absence of hypoproteinemia) due to anemia brought about by bleeding from the hemangioma. This degree of blood loss from a single small hemangioma is unusual. Resection of a bleeding colonic hemangioma is the optimal treatment. If the benign nature of the tumor can be determined at laparotomy and confirmed by adequate frozen section examination, local excision of the hemangioma is sticient. In our case, submucosal excision of the hemangioma was performed, preserving the seromuscular bowel wall. Of course, more extensive colectomy must be carried out if the colon is involved with diffuse or multiple hemangiomas, or if the surgeon cannot be assured of the benign nature of the colonic tumor. After complete resection, patients are invariably relieved of their symptoms. Treatment with rutin, 5_hydroxytrytamine, injection of sclerosants, and irradiation have been
none of these modalities reported [2,12,13]; produces a completely satisfactory result. SUMMARY
Hemangioma of the colon is a rare benign tumor and should be considered in the differential diagnosis of patients presenting with painless rectal bleeding and anemia in childhood or early adulthood. il case is reported in which a sixty-four year old man who had never had bleeding previously was found to be markedly anemic as a result of bleeding from a single small hemangioma of the descending colon. Symptoms of heart failure had been precipitated by the severe anemia. The late onset of symptoms in this patient and the degree of bleeding from a single small tumor are atypical. Excision of the colonic hemangioma resulted in the relief of all symptoms.
Achzowledgment: We are indebted to Drs. Majdalena J. Pogonowska and Jansz Gorzynsky for translations of articles from Polish and Russian. REFERENCES
1. GENTRY, R. W., DOCKERTY,M. B., and CLAGETT, 0. T. Vascular malformations and vascular tumors of the gastrointestinal tract. Inlernat. Abstr. Surg., 88: 281, 1949. 2. RISSIER, H. L. Hemangiomatosis of the intestine: discussion, review of the literature, and report of American Journal of Surgery
Hemangioma two new cases. Gastroente7ologia, 93: 357, 1960. 3. FIGLIOLINI. F. J., CUTAIT, M. R., and BASTOS, E. S. Rectosigmoidal hemangioma. Dis. Colon b Rectum, 4: 349, 1961. 4. KITORAGA, N. F. Hemangioma of the large intestine causing profuse hemorrhage. Vestnik khir., 88: 125, 1962. 5. MENDOZA, C. C. Arteriovenous angioma of the colon. South. M. J., 55: 40, 1962. 6. RUIZ-MORENO, F. Hemangiomatosis of the colon: report of a case. Dis. Colon & Rectum, 5: 453, 1962. 7. SHKLOWSKIJ, G. S. and KADYROW, F. A. Hemangioma of colon with intussusception. Vestnik khir., 93: 114, 1964.
Vol. 115, May 1968
of Colon
723
S. DZIOBA, H. and KABZA, R. Hemangiomata of colon. Polski tygodnik lek., 20: 147, 1965. 9. OLSICK, H. M., WOODHALL, J. P., JR., and CLAY, C. B. Hemangioma of the colon. J.M.A. Georgia, 46: 383, 1957. 10. HOLLINGSWORTH, G. Hemangiomatous lesions of the colon. Brit. J. Radial., 24: 220, 1951. 11. BAII.EY, J. J., BARRICK, C. W., and JENKINSON, E. L. Hemangioma of the colon. J.A.M.A., 160: 658, 1956. 12. JAQUES, A. A. Cavernous hemangioma of the rectum and rectosigmoid colon. Anr. J. Surg., 84: 507, 1952. 13. KUSHLAN, S. D. Hereditary hemorrhagic telangiectasia. Angiology, 4: 346, 1953.
M.D. AND
of the Colon R. DANIEL LOYD, M.D., Houston, Texas
From the Surgical Service, Veterans Administ, ation Hospital, Houston, Texas, and the Cora and Webb Mading Department of Surgery, Baylor University College of Medicine, Houston, Texas.
tive diagnosis and treatment during adolescence or early adulthood. This report of a case of hemangioma of the colon concerns a patient who had an atypical history. His symptoms first occurred late in life since there was no gastrointestinal bleeding or other symptoms until he was sixty-four years old. Bleeding from a single small tumor had produced severe symptoms of anemia and had precipitated heart failure.
EMANGIOMA is a common skin tumor, but is unusual in the gastrointestinal tract, and is one of the rarest benign tumors found in the colon. Gentry, Dockerty, and Clagett [I 1, reviewing the world literature through 1935, summarized 255 cases of hemangioma involving the gastrointestinal tract; in only thirty-eight cases did the lesion involve the intra-abdominal colon. Rissier [Z] tabulated eighteen cases and reports of eight other cases were found in our survey of the literature [3-9 1. Gastrointestmal hemangiomas may enlarge sufficiently to produce symptoms of obstruction in a minority of patients, and intussusception has been reported, particularly in cases from eastern Europe. The commonest complication of hemangioma of the colon is bleeding, the reported incidence varying from 60 to 90 per cent. Characteristically, colonic hemangiomas bleed episodically, slowly and persistently so that the symptoms complained of are melena and the consequences of anemia, that is, weakness and fatigue. The degree of bleeding usually correlates with the size, number, and type of hemangioma, cavernous hemangiomas tending to bleed massively much more frequently than capillary hemangiomas. The bleeding is painless and not accompanied by diarrhea unless precipitated by an intercurrent enteritis. Typically, since these tumors are congenital in melena begins in childhood and is origin, recurrent throughout adolescence, resulting in intermittent symptomatic anemia. The bleeding tends to become more severe with each recurrent episode. The early onset of bleeding and its recurrent nature usually lead to defini-
H
CASE REPORT The patient (D.H., No. 419~0%i741), a sixtyfour year old man, was referred for treatment of anemia and after a barium enema examination gave abnormal results. About eight months previously the patient first noted dyspnea on exertion and began to have fainting spells during which he became tremulous and fell to the floor, allegedly without losing consciousness. His exertional dyspnea progressed in severity and paroxysmal nocturnal dyspnea and swollen ankles and feet developed. Administration of digitalis and diuretics by his physician failed to alleviate the symptoms. Five months prior to admission he fractured his ankle during a fainting spell. At that time he was found to have a hematocrit of 28 per cent, the red blood cells were hypochromic and microcytic, serum iron was 33 pg. per cent, and stools were positive for occult blood. The patient was treated for fracture of the ankle, two units of whole blood were given, and oral iron therapy was begun. No investigation was made of the gastrointestinal tract. The fainting spells were relieved for a short time, but dpspnea and edema persisted. In the four month interval prior to admission, the patient first noticed intermittent tarry stools and sustained a weight loss of 15 pounds. A barium enema examination was carried out and his physician noted a “suspicious alea in the cecum” and referred the patient to us. The patient denied having had melena in the past and also denied having had diarrhea or mucoid stools during this present illness. There was no history of antecedent heart disease. Four years previously he 720
American
Journal
of Surgery
Hemangioma of Colon had had a small subcutaneous nodule excised from his forearm. This initially had been reported to be a pyogenic granuloma but a current reappraisal of the biopsy showed the tissue to be a hemangioma. There was no history of hemangioma in any memhers of his family. The patient had been admitted several times to this hospital for psychiatric treatment. His hematocrit on each such occasion was normal, the last admission having been three years previous. He was thin, pale, and looked chronically ill. His blood pressure was 140/75 mm. Hg, and the pulse rate was 95 per minute and regular. The jugular veins were distended 2 to 3 cm. above the clavicle with the patient in a sitting position. There was a grade II/VI systolic ejection murmur audible along the left sternal border. No gallop was detected. There was firm, pitting edema of the lower part of the legs extending up to the knees. There were no cutaneous or oral lesions. The remainder of the physicial examination and sigmoidoscopy to 25 cm. revealed nothing abnormal. The initial hemoglobin was 6.2 gm. per cent, hematocrit 23 per cent, and the total and differential leukocyte count was normal. Hypochromic, microcytic erythrocytes were found on smear examination, and the reticulocyte count was 6.5 per cent. Stools were strongly positive for occult blood. The serum iron was 17 pg. per cent and the iron binding capacity 393 pg. per cent. Serum total protein was 6.6 gm. per cent and albumin 3.6 gm. per cent. The
barium enema showed no abnormality of the cecum, but did demonstrate a filling defect in the descending colon. (Fig. 1.) All other laboratory and x-ray investigations gave normal results. Abdominal exploration was carried out after appropriate preparation of the patient. A soft nodule was palpable in the midportion of the descending colon; there were no other abnormal findings. A sessile smal1 tumor, 1.8 cm. in maximal dimension and surrounded by fresh and clotted blood, was visualized through a colotomv. This tumor was not ulcerated. The tumor was excised submucosally and its henign nature confirmed by frozen section examination. Permanent sections showed a small localized cavernous hemangioma. (Fig. 2.) There were no postoperative complications and the patient was discharged one week after operation. His symptoms of dyspnea and edema of the ankles disappeared. He noted increased vigor and gamed 10 pounds in weight during the first postoperative month. Melena has not recurred, and the patient remains asymptomatic. COMMENTS
In patients with hemangiomas of the colon presenting with painless rectal bleeding it may be difficult to make a diagnosis prior to operative exploration of the abdomen. Of course, a Vol. 115, May 1968
721
FIG. 1. Barium enema examination demonstrating filling defect in the descending colon. The remainder of the intra-abdominal colon was well visualized films and no other lesions were found.
on other
positive family history of a similar problem may be helpful. Physical examination is often unremarkable, except for signs of anemia, but the presence of hemangiomas of the skin or mucous membranes should raise a suspicion that a similar lesion might be present in the colon. Barium enema examination may reveal a filling defect, as in our case, and, infrequently, phleboliths may be noted within the filling defect. The occurrence of phleboliths is probably related to thrombosis within the tumor and is seen particularly with cavernous hemangiomas of the colon [g-11], but was not present in our patient. This patient is distinctly unusual in that his symptomatic history began at sixty-four years of age. He could recollect no episodes of rectal bleeding or symptoms of anemia in the past. The absence of anemia had been confirmed on several occasions in the last decade during admissions to the psychiatric wards. The most likely explanation for this atypical history is that the patient’scongenitalcolonichemangioma must have been very small and had grown exceedingly slowly, if at all, during most of his life. Recently, it probably underwent a period
722
Condon
and Loyd
FIG. 2. Representative portion of submucosal cavernous hemangioma excised from the colon. The overlying mucosa was intact and histologically normal. The blood-filled vascular spaces lined by endothelium and supported by a fibrous stoma are characteristic of cavernous hemangioma.
of rapid growth leading to the onset of colonic bleeding. Hemangiomas in other sites are known to undergo irregular periods of rapid acceleration in growth [I]. Major blood loss resulting in severe anemia is more usual with diffuse and extensive involvement of the colon by cavernous hemangioma. Our patient had marked symptoms both of diminished red blood cell and total blood volume (exertional dyspnea and fainting) and of congestive heart failure (dyspnea and peripheral edema in the absence of hypoproteinemia) due to anemia brought about by bleeding from the hemangioma. This degree of blood loss from a single small hemangioma is unusual. Resection of a bleeding colonic hemangioma is the optimal treatment. If the benign nature of the tumor can be determined at laparotomy and confirmed by adequate frozen section examination, local excision of the hemangioma is sticient. In our case, submucosal excision of the hemangioma was performed, preserving the seromuscular bowel wall. Of course, more extensive colectomy must be carried out if the colon is involved with diffuse or multiple hemangiomas, or if the surgeon cannot be assured of the benign nature of the colonic tumor. After complete resection, patients are invariably relieved of their symptoms. Treatment with rutin, 5_hydroxytrytamine, injection of sclerosants, and irradiation have been
none of these modalities reported [2,12,13]; produces a completely satisfactory result. SUMMARY
Hemangioma of the colon is a rare benign tumor and should be considered in the differential diagnosis of patients presenting with painless rectal bleeding and anemia in childhood or early adulthood. il case is reported in which a sixty-four year old man who had never had bleeding previously was found to be markedly anemic as a result of bleeding from a single small hemangioma of the descending colon. Symptoms of heart failure had been precipitated by the severe anemia. The late onset of symptoms in this patient and the degree of bleeding from a single small tumor are atypical. Excision of the colonic hemangioma resulted in the relief of all symptoms.
Achzowledgment: We are indebted to Drs. Majdalena J. Pogonowska and Jansz Gorzynsky for translations of articles from Polish and Russian. REFERENCES
1. GENTRY, R. W., DOCKERTY,M. B., and CLAGETT, 0. T. Vascular malformations and vascular tumors of the gastrointestinal tract. Inlernat. Abstr. Surg., 88: 281, 1949. 2. RISSIER, H. L. Hemangiomatosis of the intestine: discussion, review of the literature, and report of American Journal of Surgery
Hemangioma two new cases. Gastroente7ologia, 93: 357, 1960. 3. FIGLIOLINI. F. J., CUTAIT, M. R., and BASTOS, E. S. Rectosigmoidal hemangioma. Dis. Colon b Rectum, 4: 349, 1961. 4. KITORAGA, N. F. Hemangioma of the large intestine causing profuse hemorrhage. Vestnik khir., 88: 125, 1962. 5. MENDOZA, C. C. Arteriovenous angioma of the colon. South. M. J., 55: 40, 1962. 6. RUIZ-MORENO, F. Hemangiomatosis of the colon: report of a case. Dis. Colon & Rectum, 5: 453, 1962. 7. SHKLOWSKIJ, G. S. and KADYROW, F. A. Hemangioma of colon with intussusception. Vestnik khir., 93: 114, 1964.
Vol. 115, May 1968
of Colon
723
S. DZIOBA, H. and KABZA, R. Hemangiomata of colon. Polski tygodnik lek., 20: 147, 1965. 9. OLSICK, H. M., WOODHALL, J. P., JR., and CLAY, C. B. Hemangioma of the colon. J.M.A. Georgia, 46: 383, 1957. 10. HOLLINGSWORTH, G. Hemangiomatous lesions of the colon. Brit. J. Radial., 24: 220, 1951. 11. BAII.EY, J. J., BARRICK, C. W., and JENKINSON, E. L. Hemangioma of the colon. J.A.M.A., 160: 658, 1956. 12. JAQUES, A. A. Cavernous hemangioma of the rectum and rectosigmoid colon. Anr. J. Surg., 84: 507, 1952. 13. KUSHLAN, S. D. Hereditary hemorrhagic telangiectasia. Angiology, 4: 346, 1953.