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Pelvic hemangiopericytoma
PELVIC HEMANGIOPERICYTOMA D. I. ROBERTS,
F.R.C.S.
From the Department of Surgery, Launceston General Hospital, Launceston, Tasmania, Australia
ABSTRACT -An additional case of pelvic hemangiopericytoma is described, emphasizing difficulty in diagnosis. If surgery is undertaken, excessive blood loss must be anticipated.
In 1942, Stout and Murray’ described a tumor arising from the capillaries and surrounded by one or more layers of pericytes which distinguished it from a hemangioma. Unlike the glomus tumor, neural elements were lacking, and this neoplasm was distinguished by the name hemangiopericytoma. The hemangiopericytoma is commonly found in skin and subcutaneous tissue but may occur at any site in the body, some 20 per cent arising in the intra- and retroperitoneal spaces. It grows locally, sometimes with limited infiltration and rarely with metastasis. It is frequently extremely vascular, and when large in size may be associated with hypoglycemia. 2 The following report records a case in which diagnosis was difficult. Radical surgical management has proved successful in a three-year follow-up. Case Report A fit fifty-eight-year-old man was seen in October, 1972, with symptoms of bladder neck obstruction. Rectal examination revealed what appeared to be an enormous prostate with the possibility of malignancy in the right lateral lobe. A cystoscopy was attempted, but because of urethral distortion, the instrument could not be passed. Examination under anesthesia confirmed the presence of a large pelvic mass, hard on the right, probably arising from the prostate and extending lateral to the rectum on both sides. A further attempt at cystoscopy revealed a normal bladder and bladder neck. A transrectal biopsy of the mass was performed using a Vim-Silverman needle. The
684
the
puncture site bled profusely, but the bleeding was controlled by finger pressure. The biopsy showed a cellular mass of fibrous tissue, and no specific diagnosis was made. A repeat biopsy reported “active fibrous tissue.” In view of the indeterminate nature of the lesion, exploratory laparotomy was undertaken. This revealed a huge pelvic tumor, solid but vascular, apparently arising from the right lobe of the prostate. There was no evidence of distant spread of the disease. A cystotomy was performed and a biopsy taken through the posterior wall of the bladder. Since it was obvious that removal of the tumor would involve a cystoprostatectomy, the operation was terminated at this stage. On this occasion, the biopsy report suggested a slowly growing fibrosarcoma. On February 7, 1973, total cystoprostatectomy with removal of the tumor was performed and an ileal conduit was fashioned. The tumor filled the pelvis, but was surrounded by a capsule and was removed without spillage. Profuse pelvic oozing was controlled by packs which were uneventfully removed forty-eight hours later. The tumor surrounded but apparently did not arise from the right lobe of the prostate which was otherwise normal. The cut surface was white with a whorled appearance (Fig. 1A). Histology revealed a hemangiopericytoma of a low degree of malignancy. Numerous small capillary type vessels were present with intervening groups of mature pericytes. Mitoses were infrequent (Fig. 1B and C). The patient left the hospital on the seventeenth postoperative day and has remained free from clinical recurrence of the neoplasm.
UROLOGY
I
JUNE 1977 / VOLUME
IX, NUMBER
6
(A) Vertical section of surgical specimen __ ” showing whorled surface of tumor, and size of tumor in relation to bladder cavity on left and prostate below. (B) Low-power photomicrograph of tumor showing densely cellular nature. (C) High-power photomicrograph of tumor demonstrating pericytes and absence of mitoses.
10 units mediately
of blood were given during and imOf the 12 cases of after operation.
extrauterine pelvic hemangiopericytoma detailed by Spiro, Brockunier, and Brunschwig,5 7 metastasized or recurred locally and 3 patients died of their disease. The lifetime recurrence rate has been estimated by Backwinkel and Diddams’ to be 50 per cent. Radiotherapy may have a place in inoperable or incompletely removed lesions. Friedman and Egan* state that the dose required to kill the tumor cells is in the range 7,500 to 9,000 rads in thirty to sixty days, while palliation may be airied with 3,000 rads in eighteen days. Launceston,
Comment Hemangiopericytoma is not usually diagnosed clinically when it arises in an unusual site. Needle biopsy produces equivocal results but usually causes brisk hemorrhage which responds to finger pressure. Since pelvic hemangioperi-
cytoma is only locally malignant in most cases, surgical removal should be attempted. Wise3 and Carroll and Laughton detail a case similar to the foregoing, in which the vascular extraperitoneal mass was removed without the need for cystectomy. A number of other cases have been recorded in which surgery has varied from simple piecemeal removal to posterior exenteration.5 Internal iliac artery ligation has been employed to control the extreme vascularity of some of the tumors.” If a pelvic hemangiopericytoma is suspected, adequate blood should be available for replacement. In the present case,
UROLOGY I JUNE 1977 /
VOLUMEIX,
NUMBER6
Tasmania,
Australia
7250
References 1. STOUT, A. P.,and MURRAY, M. R.: Hemangiopericytoma, Ann. Surg. 116:26 (1942). 2. CROCKER, D. W., and VEITH, F. J,: Mesodermal tumors associated with hypoglycemia, ibid. 161: 418 (1965).
3, WISE, R. A.: 4. 5, 6. 7 6.
Hemangiopericytoma, Arch. Surg. 63: 201 (1952). CARROLL, R., and LAUGHTON, J. M.: Obstructive uropathy due to unusual pelvic swellings, Proc. Roy. Sot. Med. 66: IO47 (1973). SPIRO, R. H., BROCKUNIER,A., and BRUNSCHWIG,A.: Pelvic hemangiopericytoma, Obstet. Gynecol. 24: 402 (1964). DOUGLAS, J. L., MITCHELL, R., and SHORT, D. W.: Retroperitoneal haemangiopericytoma, Br. J. Surg. 53: 31 (1966). BACKWINKEL, K. D., and DIDDAMS,J. A.: Hemangiopericytoma, Cancer 25: 896 (1970). Irradiation of FRIEDMAN, M., and EGAN, J. W.: hemangiopericytoma of Stout, Radiology 74: 721 (1960).
685
F.R.C.S.
From the Department of Surgery, Launceston General Hospital, Launceston, Tasmania, Australia
ABSTRACT -An additional case of pelvic hemangiopericytoma is described, emphasizing difficulty in diagnosis. If surgery is undertaken, excessive blood loss must be anticipated.
In 1942, Stout and Murray’ described a tumor arising from the capillaries and surrounded by one or more layers of pericytes which distinguished it from a hemangioma. Unlike the glomus tumor, neural elements were lacking, and this neoplasm was distinguished by the name hemangiopericytoma. The hemangiopericytoma is commonly found in skin and subcutaneous tissue but may occur at any site in the body, some 20 per cent arising in the intra- and retroperitoneal spaces. It grows locally, sometimes with limited infiltration and rarely with metastasis. It is frequently extremely vascular, and when large in size may be associated with hypoglycemia. 2 The following report records a case in which diagnosis was difficult. Radical surgical management has proved successful in a three-year follow-up. Case Report A fit fifty-eight-year-old man was seen in October, 1972, with symptoms of bladder neck obstruction. Rectal examination revealed what appeared to be an enormous prostate with the possibility of malignancy in the right lateral lobe. A cystoscopy was attempted, but because of urethral distortion, the instrument could not be passed. Examination under anesthesia confirmed the presence of a large pelvic mass, hard on the right, probably arising from the prostate and extending lateral to the rectum on both sides. A further attempt at cystoscopy revealed a normal bladder and bladder neck. A transrectal biopsy of the mass was performed using a Vim-Silverman needle. The
684
the
puncture site bled profusely, but the bleeding was controlled by finger pressure. The biopsy showed a cellular mass of fibrous tissue, and no specific diagnosis was made. A repeat biopsy reported “active fibrous tissue.” In view of the indeterminate nature of the lesion, exploratory laparotomy was undertaken. This revealed a huge pelvic tumor, solid but vascular, apparently arising from the right lobe of the prostate. There was no evidence of distant spread of the disease. A cystotomy was performed and a biopsy taken through the posterior wall of the bladder. Since it was obvious that removal of the tumor would involve a cystoprostatectomy, the operation was terminated at this stage. On this occasion, the biopsy report suggested a slowly growing fibrosarcoma. On February 7, 1973, total cystoprostatectomy with removal of the tumor was performed and an ileal conduit was fashioned. The tumor filled the pelvis, but was surrounded by a capsule and was removed without spillage. Profuse pelvic oozing was controlled by packs which were uneventfully removed forty-eight hours later. The tumor surrounded but apparently did not arise from the right lobe of the prostate which was otherwise normal. The cut surface was white with a whorled appearance (Fig. 1A). Histology revealed a hemangiopericytoma of a low degree of malignancy. Numerous small capillary type vessels were present with intervening groups of mature pericytes. Mitoses were infrequent (Fig. 1B and C). The patient left the hospital on the seventeenth postoperative day and has remained free from clinical recurrence of the neoplasm.
UROLOGY
I
JUNE 1977 / VOLUME
IX, NUMBER
6
(A) Vertical section of surgical specimen __ ” showing whorled surface of tumor, and size of tumor in relation to bladder cavity on left and prostate below. (B) Low-power photomicrograph of tumor showing densely cellular nature. (C) High-power photomicrograph of tumor demonstrating pericytes and absence of mitoses.
10 units mediately
of blood were given during and imOf the 12 cases of after operation.
extrauterine pelvic hemangiopericytoma detailed by Spiro, Brockunier, and Brunschwig,5 7 metastasized or recurred locally and 3 patients died of their disease. The lifetime recurrence rate has been estimated by Backwinkel and Diddams’ to be 50 per cent. Radiotherapy may have a place in inoperable or incompletely removed lesions. Friedman and Egan* state that the dose required to kill the tumor cells is in the range 7,500 to 9,000 rads in thirty to sixty days, while palliation may be airied with 3,000 rads in eighteen days. Launceston,
Comment Hemangiopericytoma is not usually diagnosed clinically when it arises in an unusual site. Needle biopsy produces equivocal results but usually causes brisk hemorrhage which responds to finger pressure. Since pelvic hemangioperi-
cytoma is only locally malignant in most cases, surgical removal should be attempted. Wise3 and Carroll and Laughton detail a case similar to the foregoing, in which the vascular extraperitoneal mass was removed without the need for cystectomy. A number of other cases have been recorded in which surgery has varied from simple piecemeal removal to posterior exenteration.5 Internal iliac artery ligation has been employed to control the extreme vascularity of some of the tumors.” If a pelvic hemangiopericytoma is suspected, adequate blood should be available for replacement. In the present case,
UROLOGY I JUNE 1977 /
VOLUMEIX,
NUMBER6
Tasmania,
Australia
7250
References 1. STOUT, A. P.,and MURRAY, M. R.: Hemangiopericytoma, Ann. Surg. 116:26 (1942). 2. CROCKER, D. W., and VEITH, F. J,: Mesodermal tumors associated with hypoglycemia, ibid. 161: 418 (1965).
3, WISE, R. A.: 4. 5, 6. 7 6.
Hemangiopericytoma, Arch. Surg. 63: 201 (1952). CARROLL, R., and LAUGHTON, J. M.: Obstructive uropathy due to unusual pelvic swellings, Proc. Roy. Sot. Med. 66: IO47 (1973). SPIRO, R. H., BROCKUNIER,A., and BRUNSCHWIG,A.: Pelvic hemangiopericytoma, Obstet. Gynecol. 24: 402 (1964). DOUGLAS, J. L., MITCHELL, R., and SHORT, D. W.: Retroperitoneal haemangiopericytoma, Br. J. Surg. 53: 31 (1966). BACKWINKEL, K. D., and DIDDAMS,J. A.: Hemangiopericytoma, Cancer 25: 896 (1970). Irradiation of FRIEDMAN, M., and EGAN, J. W.: hemangiopericytoma of Stout, Radiology 74: 721 (1960).
685