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Recurrent uterine myomata in three sisters — an uncommon occurrence
Znt. J. Gynecol. O&ret., 1988,27: 289-291 Intemational Federation of Gynecology&Obstetrics
289
Recurrent uterine myomata in three sisters - an uncommon occurrence A. Kulenthran
and V. Sivanesaratnam
Department Obstetrics and Gynaecology. University Hospital. 59100 Kuala Lumpur (blalaysia) (Received September lOth, 1987) (Accepted November 1lth, 1987)
Abstract
Three sisters who developed recurrent uterine myomata from a very young age are presented. Despite repeated attempts at myomectomy, all three cases had hysterectomies ultimately. Complications encountered during surgery were severe hemorrhage, inadvertent injury to bladder and bowel in two patients and a rare complication of colonicuteric-cutaneous fZ.stulaoccurring post-operatively in one patient. Histology of the final hysterectomy specimens in two cases showed low grade leiomyosarcoma and cellular myoma, respectively. Keywords: Recurrent uterine myomata. Introduction Veit in 1907 reported that he knew several families where members developed myoma in youth but he did not give the patients ages [8]. Since then there have been no reports in the literature suggesting a familial basis for the etiology of uterine myoma. Furthermore Novak [7] states that heredity does not appear to be an important factor in the etiology of uterine myoma. We would like to present case reports of three sisters in whom uterine 002O-7292/88/$03.50 0 1988 International Federation of Gynecology & Obstetrics Published and Printed in Ireland
myoma occurred at an early age and despite repeated surgical attempts at conservatism, hysterectomy had to be resorted to eventually. The difficulties and complications encountered both during surgery and postoperatively are discussed. case1 A single, nulliparous, Indian female had multiple uterine myomectomy performed at 19 years of age in a peripheral hospital in Malaysia in January 1971. Three years later, in 1974, she had recurrence of uterine myomata, the uterus corresponding to a 32 week gravid uterus. A second multiple myomectomy was carried out at a neighboring private hospital, In 1976, the myomata recurred; the uterus was approximately 26 week size. She refused a hysterectomy. A third myomectomy was performed with some difficulty. No evidence of malignancy was noted on histoexamination of the myomata logical removed. The myomata recurred again in 1979; the uterus corresponding to a 28 week gravid uterus. At laparotomy in the same neighboring hospital, extensive adhesions of omenturn, bowel and parietal peritoneum to the uterus made mobilisation of the uterus difficult. As a result of severe intra-operative Case Report
290
Kulenthran and Sivansaratnam
bleeding, she developed a cardiac arrest. After resuscitation with massive blood transfusion and external cardiac massage, a subtotal hysterectomy was done. At surgery, the rectum was inadvertently injured, but was repaired. The post-operative recovery was “stormy”. She developed a fecal fistula in her abdominal wound which healed with conservative measures. No malignancy was noted on histological examination of the myomata removed. In February 1981 she was referred to the University Hospital Kuala Lumpur for recurrence of myomata. On examination, a large fixed pelvic mas about 28 week size was palpable. At laparotomy the bladder was pulled up to the fundus of the uterus and densely adherent to it and the anterior abdominal wall, making it difficult to obtain a plain of cleavage. Posteriorly the sigmoid colon was densely adherent to the uterine mass. Multiple small bowel adhesions were present over the fundus. With difficulty a total hysterectomy was performed after lysis of adhesions. Both the bladder and rectum were inadvertently injured and were repaired. Exteriorisation of the transverse colon for subsequent colostomy and a supra-pubic catheter drainage of bladder was carried out. The total blood loss during surgery was 111. Despite adequate blood replacement intraoperatively and in the immediate post-operative period, a second laparotomy had to be performed 24 h later because of severe intra-abdominal hemorrhage. Both internal iliac arteries were ligated and the pelvis packed with roll gauze and the abdomen closed The pack was removed 24 h later at a third laparotomy, and a colostomy was done; the latter was closed 6 weeks later. The histopathology showed a low grade leiomyosarcoma. Five courses of adjuvant adriamycin were given. She remains well.
mass, the size of a 16 week gravid uterus. At laparotomy, myomectomy was done for multiple myomata. In August 1974 she developed recurrence of myomata; the uterus corresponding to a 28 week gravid uterus. As she refused a hysterectomy, multiple myomectomy was performed after lysis of large bowel and omental adhesions. As it was difficult to establish hemostasis, both internal iliac arteries were ligated. Ten units of blood were transfused intra-operatively. A second laparotomy had to be performed 12 h later because of intra-abdominal hemorrhage; approximately 1 1 of blood was evacuated from the abdominal cavity. The myomectomy incisions were reopened to evacuate the clots that had accumulated within the uterus. Hemostasis was re-established and the incisions resutured. With intensive care management she made a satisfactory recovery and was discharged 2 weeks later. Six weeks later she developed a fecal fistula between the recta-sigmoid, uterus and incisional site, requiring fistulectomy. In January 1985, she developed a recurrence of the myomata and this time the uterus was fixed and corresponded to a 30 week gravid uterus. She agreed to have a hysterectomy. At laparotomy the uterus was enlarged with multiple myomata. It had prominent feeder vessels from the abdominal wall and bowel. The uterus was mobilised with much difficulty; a total hysterectomy, bilateral salpingectomy and a left oophorectomy was performed. Intra-operatively she required 15 units of blood. With intensive post-operative care, the recovery was uneventful. Histopathology of the specimens revealed cellular myomata with no evidence of malignant change. Up to the time of writing she remains well.
Case 2
The third sister had a myomectomy at the age of 24 years. The myomata recurred at the age of 29 and a total hysterectomy was done. She is now asymptomatic.
The second sister presented at the age of 18 years in July 1973 with a lower abdominal Int. J Gynecol Obstet 27
case3
Uterine myomata
Discussion Leiomyomata are the commonest tumors of the uterus, occurring in 20-309’0 of women above the age of 30 years [ 11. They are said to be rare in women below the age of 18 and 13 years is the youngest patient on record [9]. Two of our patients presented initially with the problem at 18 and 19 years, respectively, and the third sister at 24 years. Myomectomy is done when conservation of reproductive function is desired. In two of our patients, despite strong advice for a hysterectomy during the third recurrence, they declined. The number and/or the size of the myomata are no contra-indications to a myomectomy; Bonney [2] reports the removal of 125 myomata at a single operation. However, the operation is often incomplete because innumerable tiny tumors invisible to the naked eye are inevitably left behind. It is thus not surprising that myomata have been reported to recur in approximately 30070 of cases; recurrence after myomectomy for a solitary myoma, however, is lower [6]. When surgery is performed for the first time in women aged 35 years or more, the incidence of recurrence is also lower [S]. Eleven to 27.9% [5,6] who have had repeated myomectomy will finally require a hysterectomy as seen in the three sisters described above. Difficulty is usually encountered during surgery for recurrent uterine myomata as a result of adhesions of the bowel to the uterus and anterior abdominal wall [3]. In one of our patients, large feeder vessels to the uterus were seen arising from the bowel and anterior abdominal wall. In this same patient a rare complication of colonic-uterine-cutaneous fistula was evident on the 39th post-operative day. Inadvertent injury to the ureter may occur and hence a pre-operative intravenous pyelogram is essential. The use of a ureteric stent introduced via cystoscopy prior to surgery would fascilitate identification of the ureter. As intra-operative hemorrhage is one of the major problems at surgery, an adequate amount of blood must be available. This is
291
clearly illustrated in the first two cases described above; the second required 15 units of blood intra-operatively to maintain her blood pressure throughout surgery. Morphological and histological features of the tumors removed may be of prognostic significance in terms of recurrence. A problem for the gynecologic pathologist is that of differentiating between a cellular and benign myoma, a typical leiomyoma and a low grade leiomyosarcoma. The three features that are of importance are increased cellularity, mitotic activity and cellular atypia. A mitotic rate of greater than 5 per 10 high power fields correlates well with a malignant clinical behavior [4]. In Case 1, though the tumor removed during the first two surgeries were histologically benign, the final surgery revealed a low grade leiomysarcoma. In Case 2, although the myomata were rather cellular, the other features of malignancy were absent. References Blaustein A: Pathology of the female genital tract, 2nd edn, ~354. Springer-Verlag. Berlin, 1982. Bonney V: The technique and results of myomectomy. Lancet i: 171,193l. Brown AB, Chamberlain R, TeLinde RW: Myomectomy. Lancet i: 171,193l. Burns B, Curry RH, Bell MEA: Morphologic features of prognostic significance in uterine smooth muscle tumours: a review of eighty-four cases. Am J Obstet Gynaecoll35: 109,1979. Loeffler FE, Noble AB: Myomectomy at the Chelsea Hospital for women. J Obstet Gynaecol Br Commonw 70: 167, 1970. Malorie LJ: Myomectomy. Recurrence after removal of solitary and multiple myoma. Obstet Gynaecol 34:200, 1969. Novak ER, Woodruft JD: Novak’s GynecologicandObstetric Pathology, 6th edn, p 212. W.B. Saunders Co., PhiladelphiaandLondon, 1%7. Veit J: Uterine myoma. In Handbuch der Gynakologie, 2nd edn. J.F. Bergman, Weisbaden, 1907(quotedinRef. 9). Wisof AL, Neimand KM, Rosenthal AH: Symptomatic myomaina 13yearoldgirl. Am JObstet GynaecolZ05:639, 1%9. Address for reprints: A. Kuienthran Department of Obstetrics and Gyaaecology University Hospital 59100 Knala Lumpur Malaysia Case Report
289
Recurrent uterine myomata in three sisters - an uncommon occurrence A. Kulenthran
and V. Sivanesaratnam
Department Obstetrics and Gynaecology. University Hospital. 59100 Kuala Lumpur (blalaysia) (Received September lOth, 1987) (Accepted November 1lth, 1987)
Abstract
Three sisters who developed recurrent uterine myomata from a very young age are presented. Despite repeated attempts at myomectomy, all three cases had hysterectomies ultimately. Complications encountered during surgery were severe hemorrhage, inadvertent injury to bladder and bowel in two patients and a rare complication of colonicuteric-cutaneous fZ.stulaoccurring post-operatively in one patient. Histology of the final hysterectomy specimens in two cases showed low grade leiomyosarcoma and cellular myoma, respectively. Keywords: Recurrent uterine myomata. Introduction Veit in 1907 reported that he knew several families where members developed myoma in youth but he did not give the patients ages [8]. Since then there have been no reports in the literature suggesting a familial basis for the etiology of uterine myoma. Furthermore Novak [7] states that heredity does not appear to be an important factor in the etiology of uterine myoma. We would like to present case reports of three sisters in whom uterine 002O-7292/88/$03.50 0 1988 International Federation of Gynecology & Obstetrics Published and Printed in Ireland
myoma occurred at an early age and despite repeated surgical attempts at conservatism, hysterectomy had to be resorted to eventually. The difficulties and complications encountered both during surgery and postoperatively are discussed. case1 A single, nulliparous, Indian female had multiple uterine myomectomy performed at 19 years of age in a peripheral hospital in Malaysia in January 1971. Three years later, in 1974, she had recurrence of uterine myomata, the uterus corresponding to a 32 week gravid uterus. A second multiple myomectomy was carried out at a neighboring private hospital, In 1976, the myomata recurred; the uterus was approximately 26 week size. She refused a hysterectomy. A third myomectomy was performed with some difficulty. No evidence of malignancy was noted on histoexamination of the myomata logical removed. The myomata recurred again in 1979; the uterus corresponding to a 28 week gravid uterus. At laparotomy in the same neighboring hospital, extensive adhesions of omenturn, bowel and parietal peritoneum to the uterus made mobilisation of the uterus difficult. As a result of severe intra-operative Case Report
290
Kulenthran and Sivansaratnam
bleeding, she developed a cardiac arrest. After resuscitation with massive blood transfusion and external cardiac massage, a subtotal hysterectomy was done. At surgery, the rectum was inadvertently injured, but was repaired. The post-operative recovery was “stormy”. She developed a fecal fistula in her abdominal wound which healed with conservative measures. No malignancy was noted on histological examination of the myomata removed. In February 1981 she was referred to the University Hospital Kuala Lumpur for recurrence of myomata. On examination, a large fixed pelvic mas about 28 week size was palpable. At laparotomy the bladder was pulled up to the fundus of the uterus and densely adherent to it and the anterior abdominal wall, making it difficult to obtain a plain of cleavage. Posteriorly the sigmoid colon was densely adherent to the uterine mass. Multiple small bowel adhesions were present over the fundus. With difficulty a total hysterectomy was performed after lysis of adhesions. Both the bladder and rectum were inadvertently injured and were repaired. Exteriorisation of the transverse colon for subsequent colostomy and a supra-pubic catheter drainage of bladder was carried out. The total blood loss during surgery was 111. Despite adequate blood replacement intraoperatively and in the immediate post-operative period, a second laparotomy had to be performed 24 h later because of severe intra-abdominal hemorrhage. Both internal iliac arteries were ligated and the pelvis packed with roll gauze and the abdomen closed The pack was removed 24 h later at a third laparotomy, and a colostomy was done; the latter was closed 6 weeks later. The histopathology showed a low grade leiomyosarcoma. Five courses of adjuvant adriamycin were given. She remains well.
mass, the size of a 16 week gravid uterus. At laparotomy, myomectomy was done for multiple myomata. In August 1974 she developed recurrence of myomata; the uterus corresponding to a 28 week gravid uterus. As she refused a hysterectomy, multiple myomectomy was performed after lysis of large bowel and omental adhesions. As it was difficult to establish hemostasis, both internal iliac arteries were ligated. Ten units of blood were transfused intra-operatively. A second laparotomy had to be performed 12 h later because of intra-abdominal hemorrhage; approximately 1 1 of blood was evacuated from the abdominal cavity. The myomectomy incisions were reopened to evacuate the clots that had accumulated within the uterus. Hemostasis was re-established and the incisions resutured. With intensive care management she made a satisfactory recovery and was discharged 2 weeks later. Six weeks later she developed a fecal fistula between the recta-sigmoid, uterus and incisional site, requiring fistulectomy. In January 1985, she developed a recurrence of the myomata and this time the uterus was fixed and corresponded to a 30 week gravid uterus. She agreed to have a hysterectomy. At laparotomy the uterus was enlarged with multiple myomata. It had prominent feeder vessels from the abdominal wall and bowel. The uterus was mobilised with much difficulty; a total hysterectomy, bilateral salpingectomy and a left oophorectomy was performed. Intra-operatively she required 15 units of blood. With intensive post-operative care, the recovery was uneventful. Histopathology of the specimens revealed cellular myomata with no evidence of malignant change. Up to the time of writing she remains well.
Case 2
The third sister had a myomectomy at the age of 24 years. The myomata recurred at the age of 29 and a total hysterectomy was done. She is now asymptomatic.
The second sister presented at the age of 18 years in July 1973 with a lower abdominal Int. J Gynecol Obstet 27
case3
Uterine myomata
Discussion Leiomyomata are the commonest tumors of the uterus, occurring in 20-309’0 of women above the age of 30 years [ 11. They are said to be rare in women below the age of 18 and 13 years is the youngest patient on record [9]. Two of our patients presented initially with the problem at 18 and 19 years, respectively, and the third sister at 24 years. Myomectomy is done when conservation of reproductive function is desired. In two of our patients, despite strong advice for a hysterectomy during the third recurrence, they declined. The number and/or the size of the myomata are no contra-indications to a myomectomy; Bonney [2] reports the removal of 125 myomata at a single operation. However, the operation is often incomplete because innumerable tiny tumors invisible to the naked eye are inevitably left behind. It is thus not surprising that myomata have been reported to recur in approximately 30070 of cases; recurrence after myomectomy for a solitary myoma, however, is lower [6]. When surgery is performed for the first time in women aged 35 years or more, the incidence of recurrence is also lower [S]. Eleven to 27.9% [5,6] who have had repeated myomectomy will finally require a hysterectomy as seen in the three sisters described above. Difficulty is usually encountered during surgery for recurrent uterine myomata as a result of adhesions of the bowel to the uterus and anterior abdominal wall [3]. In one of our patients, large feeder vessels to the uterus were seen arising from the bowel and anterior abdominal wall. In this same patient a rare complication of colonic-uterine-cutaneous fistula was evident on the 39th post-operative day. Inadvertent injury to the ureter may occur and hence a pre-operative intravenous pyelogram is essential. The use of a ureteric stent introduced via cystoscopy prior to surgery would fascilitate identification of the ureter. As intra-operative hemorrhage is one of the major problems at surgery, an adequate amount of blood must be available. This is
291
clearly illustrated in the first two cases described above; the second required 15 units of blood intra-operatively to maintain her blood pressure throughout surgery. Morphological and histological features of the tumors removed may be of prognostic significance in terms of recurrence. A problem for the gynecologic pathologist is that of differentiating between a cellular and benign myoma, a typical leiomyoma and a low grade leiomyosarcoma. The three features that are of importance are increased cellularity, mitotic activity and cellular atypia. A mitotic rate of greater than 5 per 10 high power fields correlates well with a malignant clinical behavior [4]. In Case 1, though the tumor removed during the first two surgeries were histologically benign, the final surgery revealed a low grade leiomysarcoma. In Case 2, although the myomata were rather cellular, the other features of malignancy were absent. References Blaustein A: Pathology of the female genital tract, 2nd edn, ~354. Springer-Verlag. Berlin, 1982. Bonney V: The technique and results of myomectomy. Lancet i: 171,193l. Brown AB, Chamberlain R, TeLinde RW: Myomectomy. Lancet i: 171,193l. Burns B, Curry RH, Bell MEA: Morphologic features of prognostic significance in uterine smooth muscle tumours: a review of eighty-four cases. Am J Obstet Gynaecoll35: 109,1979. Loeffler FE, Noble AB: Myomectomy at the Chelsea Hospital for women. J Obstet Gynaecol Br Commonw 70: 167, 1970. Malorie LJ: Myomectomy. Recurrence after removal of solitary and multiple myoma. Obstet Gynaecol 34:200, 1969. Novak ER, Woodruft JD: Novak’s GynecologicandObstetric Pathology, 6th edn, p 212. W.B. Saunders Co., PhiladelphiaandLondon, 1%7. Veit J: Uterine myoma. In Handbuch der Gynakologie, 2nd edn. J.F. Bergman, Weisbaden, 1907(quotedinRef. 9). Wisof AL, Neimand KM, Rosenthal AH: Symptomatic myomaina 13yearoldgirl. Am JObstet GynaecolZ05:639, 1%9. Address for reprints: A. Kuienthran Department of Obstetrics and Gyaaecology University Hospital 59100 Knala Lumpur Malaysia Case Report