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Retroperitoneal cavernous hemangioma
RETROPERITONEAL GRANT
E.
WARD,
CAVERNOUS
M.D.
AND
EDWIN
H.
HEMANGIOMA* STEWART,
JR.,
M.D.
Baltimore, Maryland ETROPERITONEAL hemangiomas are rare but must be considered in the differentia1 diagnosis of retroperitonea tumors. The pathogenesis of hemangiomas is stilI undetermined. A deveIopmenta1 anomaly in the structure of certain vascular
R
FIG. I. Picture taken prior to operation.
segments may be responsib1e for this Iesion. Matas,’ and DeIafieId and Prudden2 maintain that hemangiomas are composed of the same ceI1uIar e1ements that give rise to primordia1 blood vesseIs. In accordance with this they beIieve that the hemangioma is the resuIt of disordered, spontaneous growth of these primitive ceIIs. Why hemangiomas assume a capiIIary or cavernous configuration and why they occur at al1 is imperfect1y understood. An angioma is considered a true neopIastic process invo1ving vascu1ar and/or Iymphatic tissue. It is generaIIy be1ieved that angiomas are congenita1 in nature and have their origin in mispIaced mesoderma1 tissue. In the Iiterature extrarena retroperitonea1 hemangiomas are found to be very rare. HiIse3 in 1928 reported a Iarge recurring retro-
peritoneal Iipoma that had areas of cavernous hemangioma in it. In Ig2g Harris4 reported the case of a fifty-nine year oId white maIe who had an encapsuIated retroperitonea1 perirenal fibrohemangioma about the size of a grapefruit. The Iesion was removed but the patient died eteven days postoperativeIy probabIy from a cerebral embo1ism. A retroperitonea1 peIvic hemangioma was reported by Frank5 in 1930. The patient was a thirty-five year oId unmarried female admitted to Mt. Sinai Hospital because of abdomina1 pain of four hours’ duration. A hemangioma was removed from the Ieft broad ligament six years previousIy. Operation at this time reveaIed a large hemangioma of the peIvic floor, which surrounded the uterus and extended on to the urinary bIadder and sigmoid. The author states that with much diffrcuIty this retroperitonea1 cavernous hemangioma was removed a1ong with the uterus and that convaIescence was uneventful. Two years later the patient was reoperated upon and recurrence noted. RemovaI of the Iesion was impossib1e at this time. Radium, 19,250 mg. hours, was appIied per vagina and over the abdomen and buttock (treatment factors not known). Tapfer and Just? in 1935 reported a case of capiIIary hemangioma of the connective tissue of the pe1vis. In 1944 MilIman7 reported a retroperitonea1 cavernous hemangioma in a white maIe thirty-two years of age who had a Iarge, non-tender, smooth mass in the right Iower quadrant. The cecum and ascending coIon were dispIaced to the midIine. At operation a cavernous hemangioma about the size of a pineappIe Iying on the right iIeopsoas muscIe was encountered. The tumor which was moderateIy adherent to the retroperitonea1 tissues was removed.
* From the Department of Oncology of the Department of Surgery, University of Maryland School of Medicine, BaItimore, Md.
470
American
Journal
of Surgery
Ward,
Stewart-Retroperitoneal
Hemangioma
3
2
FIG. 2. Anterior surface of the bladder. FIG. 3. Photograph”showing posterior wail of bladder surface of sipmoid (c).
FIG. 4. Photograph showing beneath the mucosa of sigmoid.
hemorrhagic
(A)
hydro-ureter,
Ieft
(B)
and hemangioma
on anterior
areas
The purpose of this paper is to report a case of a retroperitonea1 cavernous hemangioma which was a compIicating factor to what appeared to be a subcutaneous plexiform hemangioma of the buttocks. CASE REPORT The patient, a white maIe, was first seen at the age of five months because of rectal bleeding and two hemangiomas, one overlying the right buttock and the other over the mid-sacra1 crest. The hemangioma of the sacra1 crest measured z by I cm.; the one of the right buttock which extended beyond the midIine in the intergIutea1 foId measured 835 by 6 cm. (Fig. I.) The smaIIer hemangioma was treated with radium on February 5, 1946 (I 20 mg. hours (2 T.E.D.), fiItration I mm. pt., S.T.D. 0.5 cm.). Recta1 and proctoscopic examinations were normal. On February 22, 1946, the child received 340 r over the large hemangioma (technic 140 kiIovoIt, 15 miIIiampere, filtration none, S.T.D. 35 cm., field size 4 cm.). On March 22, 1946, x-ray examination of pelvis reveaIed the pelvic bones to be normal in denOctober, 1950
471
FIG. 3. Photomicrograph waI1.
of hemangioma
of bIadder
sity, and a soft tissue mass Iying posterior to the coccyx was noted. On March 27, 1946, and September 16, 1946, the chiId received 408 r over the large hemangioma using the same technic as previousIy described, making a tota of I, 156 r. During this time the hemangiomas were paIer and onIy occasionalIy was recta1 bIeeding noted. Barium enema on May 20, I 947, was essentialIy normaI; but because satisfactory regression of these Iesions was not forthcoming and intermittent recta1 bleeding still persisted, operative intervention was deemed advisabIe. On JuIy 20, 1947, the patient was admitted to the University Hospital for surgery. On JuIy 21, 1947, under ether anesthesia an attempt was made to remove the hemangiomas. In the subcutaneous tissue overlying the buttock many Iarge veins and venous sinuses were encountered and excised. The hemangiomas were connected by Iarge veins. The larger
372
Ward,
Stewart-RetroperitoneaI
hemangioma was made up of sinuses, some containing bIood and others cIear fluid. The dissection was carried to the right of the coccyx and it was obvious that the Iesion extended into the pelvic floor. At this point the chiId had what was thought to be an ether convuIsion, and responded we11 to oxygen. SyInasoI, 6 cc., was injected into the Iargest sinus to the right of the coccyx with the hope of obIiterating this retroperitonea1 extension. The wound was closed with interrupted derma1 sutures. About one hour foIIowing operation hyperpyrexia and cIonic convuIsions deveIoped. It was thought that the patient’s symptoms couId be expIained on the basis of cerebra1 anoxemia. The chiId continued to remain comatose. FinaIIy decerebrate rigidity deve1oped and on August 24, I 947, respirations ceased. At autopsy a Iarge retroperitonea1 cavernous hemangioma of the pe1vic fIoor was found to be continuous with the excised Iesion. From the peIvic floor the Iesion surrounded the neck of the urinary bladder and extended over the posterior waI1 and fundus of the bIadder. (Figs. 2 and 3.) The Iesion surrounded the ureterovesica1 junction to cause biIatera1 hydro-ureters. A smaI1 ramification extended beneath the peritoneal covering of the sigmoid. (Fig. 3.) On opening the sigmoid small punctate hemorrhagic spaces were found beneath the mucosa. (Fig. 4.) This probably accounts for the patient’s recta1 bleeding. Careful examination revealed venous communications between the iliac veins and the previously described tumor mass. DiIated retroperitoneal pelvic Iymphatics were noted and thought to be due to a Iymphatic bIockage caused by this retroperitonea1 hemangioma. An attempt at autopsy to re-
Hemangioma move this Iesion was impossibIe. Microscopic examination revea1ed a cavernous hemangioma. (Fig. r.) The brain showed diffuse encephaIomaIacia of the cerebra1 cortex, pons and cerebeIIum (post anoxic), cerebra1 congestion and edema. CONCLUSIONS
Retroperitoneal hemangiomas are rare but should be considered in the differential diagnosis of retroperitoneal tumors. Diagnosis as we11 as therapy is difficult. Complete extirpation shouId be performed whenever possible. Use of x-ray and radium is an important adjunct in the treatment but, because of the inaccessible location, it has IittIe vaIue in curing the lesion. This case should bring to mind the importance of recta1 bIeeding in association with hemangioma in the perinea1 area as we11 as the futility in treating such extensive lesions. REFERENCES 1.
2.
3.
4. 5.
6.
7.
MATAS, R. VascuIar tumors. In: PiersaI, G. M. and Bortz, E. L. The Cyclopedia of Medicine. Vol. I 2, p. 814. Philadelphia, ,934. F. A. Davis Co. DELAFIELD, F. and PRUDDEK, A. A Textbook of 16th ed. BaItimore, 1936. William Pathology. Wood & Co. HILSE, A. Ubcr Retroperitonealc MesodermaIe Geschwiilste mit einem Beitrag zu ihrer Morphogenese. Arch. j. klin Cbir., I p: 251-258, 1928. HARRIS, A. Retroperitoneal angioma. J. Llrol., 2 I : 181-191, 1929. FRANK, R. Hemangioma of pelvic connective tissue. Am. J. Obst. & Gynec., 20: 81-84, ,930. TAPFER, S. and JUST, E. Uber ein Kapillares Hamangiom des Beckenbindegewebes. Zentralbl. f. Gyd. 59: 727-735. 1935. MILLMAN, M. RetroperitoneaI cavernous hemangioma. J. A. M. A., 124: 773-774, 1944.
American
Journal of Surgery
E.
WARD,
CAVERNOUS
M.D.
AND
EDWIN
H.
HEMANGIOMA* STEWART,
JR.,
M.D.
Baltimore, Maryland ETROPERITONEAL hemangiomas are rare but must be considered in the differentia1 diagnosis of retroperitonea tumors. The pathogenesis of hemangiomas is stilI undetermined. A deveIopmenta1 anomaly in the structure of certain vascular
R
FIG. I. Picture taken prior to operation.
segments may be responsib1e for this Iesion. Matas,’ and DeIafieId and Prudden2 maintain that hemangiomas are composed of the same ceI1uIar e1ements that give rise to primordia1 blood vesseIs. In accordance with this they beIieve that the hemangioma is the resuIt of disordered, spontaneous growth of these primitive ceIIs. Why hemangiomas assume a capiIIary or cavernous configuration and why they occur at al1 is imperfect1y understood. An angioma is considered a true neopIastic process invo1ving vascu1ar and/or Iymphatic tissue. It is generaIIy be1ieved that angiomas are congenita1 in nature and have their origin in mispIaced mesoderma1 tissue. In the Iiterature extrarena retroperitonea1 hemangiomas are found to be very rare. HiIse3 in 1928 reported a Iarge recurring retro-
peritoneal Iipoma that had areas of cavernous hemangioma in it. In Ig2g Harris4 reported the case of a fifty-nine year oId white maIe who had an encapsuIated retroperitonea1 perirenal fibrohemangioma about the size of a grapefruit. The Iesion was removed but the patient died eteven days postoperativeIy probabIy from a cerebral embo1ism. A retroperitonea1 peIvic hemangioma was reported by Frank5 in 1930. The patient was a thirty-five year oId unmarried female admitted to Mt. Sinai Hospital because of abdomina1 pain of four hours’ duration. A hemangioma was removed from the Ieft broad ligament six years previousIy. Operation at this time reveaIed a large hemangioma of the peIvic floor, which surrounded the uterus and extended on to the urinary bIadder and sigmoid. The author states that with much diffrcuIty this retroperitonea1 cavernous hemangioma was removed a1ong with the uterus and that convaIescence was uneventful. Two years later the patient was reoperated upon and recurrence noted. RemovaI of the Iesion was impossib1e at this time. Radium, 19,250 mg. hours, was appIied per vagina and over the abdomen and buttock (treatment factors not known). Tapfer and Just? in 1935 reported a case of capiIIary hemangioma of the connective tissue of the pe1vis. In 1944 MilIman7 reported a retroperitonea1 cavernous hemangioma in a white maIe thirty-two years of age who had a Iarge, non-tender, smooth mass in the right Iower quadrant. The cecum and ascending coIon were dispIaced to the midIine. At operation a cavernous hemangioma about the size of a pineappIe Iying on the right iIeopsoas muscIe was encountered. The tumor which was moderateIy adherent to the retroperitonea1 tissues was removed.
* From the Department of Oncology of the Department of Surgery, University of Maryland School of Medicine, BaItimore, Md.
470
American
Journal
of Surgery
Ward,
Stewart-Retroperitoneal
Hemangioma
3
2
FIG. 2. Anterior surface of the bladder. FIG. 3. Photograph”showing posterior wail of bladder surface of sipmoid (c).
FIG. 4. Photograph showing beneath the mucosa of sigmoid.
hemorrhagic
(A)
hydro-ureter,
Ieft
(B)
and hemangioma
on anterior
areas
The purpose of this paper is to report a case of a retroperitonea1 cavernous hemangioma which was a compIicating factor to what appeared to be a subcutaneous plexiform hemangioma of the buttocks. CASE REPORT The patient, a white maIe, was first seen at the age of five months because of rectal bleeding and two hemangiomas, one overlying the right buttock and the other over the mid-sacra1 crest. The hemangioma of the sacra1 crest measured z by I cm.; the one of the right buttock which extended beyond the midIine in the intergIutea1 foId measured 835 by 6 cm. (Fig. I.) The smaIIer hemangioma was treated with radium on February 5, 1946 (I 20 mg. hours (2 T.E.D.), fiItration I mm. pt., S.T.D. 0.5 cm.). Recta1 and proctoscopic examinations were normal. On February 22, 1946, the child received 340 r over the large hemangioma (technic 140 kiIovoIt, 15 miIIiampere, filtration none, S.T.D. 35 cm., field size 4 cm.). On March 22, 1946, x-ray examination of pelvis reveaIed the pelvic bones to be normal in denOctober, 1950
471
FIG. 3. Photomicrograph waI1.
of hemangioma
of bIadder
sity, and a soft tissue mass Iying posterior to the coccyx was noted. On March 27, 1946, and September 16, 1946, the chiId received 408 r over the large hemangioma using the same technic as previousIy described, making a tota of I, 156 r. During this time the hemangiomas were paIer and onIy occasionalIy was recta1 bIeeding noted. Barium enema on May 20, I 947, was essentialIy normaI; but because satisfactory regression of these Iesions was not forthcoming and intermittent recta1 bleeding still persisted, operative intervention was deemed advisabIe. On JuIy 20, 1947, the patient was admitted to the University Hospital for surgery. On JuIy 21, 1947, under ether anesthesia an attempt was made to remove the hemangiomas. In the subcutaneous tissue overlying the buttock many Iarge veins and venous sinuses were encountered and excised. The hemangiomas were connected by Iarge veins. The larger
372
Ward,
Stewart-RetroperitoneaI
hemangioma was made up of sinuses, some containing bIood and others cIear fluid. The dissection was carried to the right of the coccyx and it was obvious that the Iesion extended into the pelvic floor. At this point the chiId had what was thought to be an ether convuIsion, and responded we11 to oxygen. SyInasoI, 6 cc., was injected into the Iargest sinus to the right of the coccyx with the hope of obIiterating this retroperitonea1 extension. The wound was closed with interrupted derma1 sutures. About one hour foIIowing operation hyperpyrexia and cIonic convuIsions deveIoped. It was thought that the patient’s symptoms couId be expIained on the basis of cerebra1 anoxemia. The chiId continued to remain comatose. FinaIIy decerebrate rigidity deve1oped and on August 24, I 947, respirations ceased. At autopsy a Iarge retroperitonea1 cavernous hemangioma of the pe1vic fIoor was found to be continuous with the excised Iesion. From the peIvic floor the Iesion surrounded the neck of the urinary bladder and extended over the posterior waI1 and fundus of the bIadder. (Figs. 2 and 3.) The Iesion surrounded the ureterovesica1 junction to cause biIatera1 hydro-ureters. A smaI1 ramification extended beneath the peritoneal covering of the sigmoid. (Fig. 3.) On opening the sigmoid small punctate hemorrhagic spaces were found beneath the mucosa. (Fig. 4.) This probably accounts for the patient’s recta1 bleeding. Careful examination revealed venous communications between the iliac veins and the previously described tumor mass. DiIated retroperitoneal pelvic Iymphatics were noted and thought to be due to a Iymphatic bIockage caused by this retroperitonea1 hemangioma. An attempt at autopsy to re-
Hemangioma move this Iesion was impossibIe. Microscopic examination revea1ed a cavernous hemangioma. (Fig. r.) The brain showed diffuse encephaIomaIacia of the cerebra1 cortex, pons and cerebeIIum (post anoxic), cerebra1 congestion and edema. CONCLUSIONS
Retroperitoneal hemangiomas are rare but should be considered in the differential diagnosis of retroperitoneal tumors. Diagnosis as we11 as therapy is difficult. Complete extirpation shouId be performed whenever possible. Use of x-ray and radium is an important adjunct in the treatment but, because of the inaccessible location, it has IittIe vaIue in curing the lesion. This case should bring to mind the importance of recta1 bIeeding in association with hemangioma in the perinea1 area as we11 as the futility in treating such extensive lesions. REFERENCES 1.
2.
3.
4. 5.
6.
7.
MATAS, R. VascuIar tumors. In: PiersaI, G. M. and Bortz, E. L. The Cyclopedia of Medicine. Vol. I 2, p. 814. Philadelphia, ,934. F. A. Davis Co. DELAFIELD, F. and PRUDDEK, A. A Textbook of 16th ed. BaItimore, 1936. William Pathology. Wood & Co. HILSE, A. Ubcr Retroperitonealc MesodermaIe Geschwiilste mit einem Beitrag zu ihrer Morphogenese. Arch. j. klin Cbir., I p: 251-258, 1928. HARRIS, A. Retroperitoneal angioma. J. Llrol., 2 I : 181-191, 1929. FRANK, R. Hemangioma of pelvic connective tissue. Am. J. Obst. & Gynec., 20: 81-84, ,930. TAPFER, S. and JUST, E. Uber ein Kapillares Hamangiom des Beckenbindegewebes. Zentralbl. f. Gyd. 59: 727-735. 1935. MILLMAN, M. RetroperitoneaI cavernous hemangioma. J. A. M. A., 124: 773-774, 1944.
American
Journal of Surgery