Int J Gynecol Obster, 1992, 37: 285-288
285
International Federation of Gynecology and Obstetrics
Intravenous A.M.A.
Mullings,
leiomyomatosis
with massive ascites
G. Char”, M.P. Martina,
J. Frederick,
N. Walton
and K. Pickering
Departments of Obstetrics and Gynaecology and ‘Pathology, University of The West Indies. Kingston (Jamaicu)
(Received July 14th, 1991) (Revised and accepted August 22nd. 1991)
Abstract
A case of intravenous leiomyomatosis with massive ascites is reported, This is the first such recorded case. The patient was treated with a subtotal abdominal hysterectomy and bilateral salpingo-oophorectomy. Pathological examination established a vessel wall origin. There is no evidence of recurrence up to 20 months after initial treatment. Keywords: Intravenous leiomyomatosis; Ascites; Clinico-pathological. Introduction
A rare gynecological tumor, the intravenous leiomyoma (IVL), is a benign smooth muscle tumor growing within veins [9]. It may arise primarily from the blood vessel or from the uterus itself with extension into the venous system. Though slow growing and benign it may be fatal largely due to its location [2,3,10]. A review of the literature reveals 60 reported cases including a recent report [2,12]. This is the first with ascites and the first from the Caribbean. Case report
A 46-year-old hypertensive unmarried negro woman was admitted to the University 0020-7292/92/$05.00 0 1992 International Federation of Gynecology and Obstetrics Printed and Published in Ireland
Hospital in April 1989 giving a 4-month history of swollen abdomen with a rapid increase in size over the previous 7 days. Of significance in the history was weight loss, anorexia, weakness and abdominal pain but no menstrual or reproductive organ dysfunction. Examination revealed a moderately distressed middle-aged lady with a grossly distended abdomen. No masses were palpable and a fluid thrill was elicited. Abdominal ultrasound showed a predominantly solid pelvic mass with cystic areas and the ascites. Liver architecture was normal. Abdominal paracentesis was performed and 5.5 1 of straw colored fluid drained. Cytological studies of this fluid were negative for malignancy showing only polymorphs, lymphocytes and mesothelial cells. A barium series was normal. At surgery a further 10 1 of ascitic fluid was drained and a vascular cystic tumor measuring approximately 12 x 18 cm involving the right side of the uterus, round ligament, parietal peritoneum and dome of bladder was seen. There were no palpable nodes. A subtotal hysterectomy with bilateral salpingooophorectomy was performed. On gross pathological examination of the uterus it was found to be markedly distorted on the right by a large lobulated mass extending laterally from the uterine wall. The cut surface showed coiled, nodular, well demarcated firm masses in dilated myometrial veins within the right broad ligament from the Case Report
286
Mullings ef al.
mesosalpinx superiorly to the vault at the lower margin. Laterally the intravenous worm-like masses were expanding to form a large mass which was partly cystic, partly solid with areas of infarction and recent and old hemorrhages (Fig. 1). Microscopically the tumor was present in multiple venous channels lined by endotheliurn and grew within these channels (Fig. 2). In places these were attached to the wall as if arising from it. All the intravenous masses and the largest were composed of spindle shaped smooth muscle fibers arranged in whorls and interlacing bundles (Fig. 3). In places they appeared epitheloid. Stromal fibrosis, hyalinization and presence of
Fig. 1. venous
Cut surface of the uterus showing coiled nodular masses with parametrial
Int J Gynecol Obsret 37
extension.
intra-
numerous thick walled blood vessels were prominent features. Nuclear atypia and mitotic activity were absent. The overall histological picture was that of intravenous leiomyomatosis. Discussion The condition of intravenous leiomyomatosis (IVL) was first described by Birch Hirschfield in 1896 and the first case of intracardiac spread by Durek in 1907 [lo]. Reference in English language textbooks to a similar condition was found as early as 1922 [6]. Mukherjee [6] in his report agrees with others that the clinical features are essentially the same as for fibromyomata and that the surprise usually comes with the surgery. Roman and Mirchandani [lo], in their report, attempted to give a clinical profile. From their review of 50 cases 48 or 96% of cases were in women, possibly implying that it may occur in males. The age range was 28-76 with a median of 46 years, the age of this patient, and a mean of 46.4 years. No specific data are given as to race or parity, but from the published data reviewed parity is variable’. None of the reports reviewed mentioned ascites as a feature of the disease and this was the most striking feature in our patient. The nonrecurrence of ascites up to 20 months after treatment is strong evidence to suggest a direct relationship to IVL. At laparotomy the differential diagnosis would include endometrial stromatosis, uterine sarcoma, disseminated peritoneal leiomyomatosis and benign metastasizing leiomyoma [3,6]. Clinical distinction at the time of surgery could be important since it may influence the extent of surgery. A frozen section will be of help. There is no doubt however, that surgical excision is required and that this can be successfully done even with intracardiac extension [ 10,121. Extrauterine extension occurs in about 50% of cases of which 25% go beyond the broad ligament to involve the iliac veins, inferior vena cava and the right side of the heart [2]. It is therefore
Inrravenous leiomyomalosi.~ with as&es
Fig. 2.
Low magnification
of a typical
intravenous
growth.
H&E
important to palpate the vessels in and around the site of the tumor. The histogenesis of this tumor is uncertain. Evans et al. [3] stated that Knauer in 1903 postulated an origin from the vessel wall and Sitzenfrey, in 1911 that they are primary uterine leiomyomas. Evidence collected since then supports both theories. Marshall and Morris [5] did not see any typical leiomyoma in 17 cases, but others [1,7,8,1 l] all reported IVL extending from preexisting leiomyoma. Norris and Parmley [8] further concluded from their report that both theories were correct. It appears that when IVL results from vascular invasion the bulk of the tumor is ex-
281
x 38.
travascular with no site of origin from the vessel wall. Where venous smooth muscle is the origin, it is predominantly or entirely intravenous with many sites of attachment. That estrogen can induce proliferation of endothelial cells which can differentiate into smooth muscle has been suggested by some authors [4,6,12]. Testing for estrogen receptors has been used to try and substantiate this theory. The treatment is primarily surgical excision. Recurrence after excision is rare but amenable to repeated excision. At present neither chemo- nor radiotherapy has proven to be of any benefit [6]. Case Report
288
Mullings
Fig. 3.
et al.
Microphotograph
of the tumor showing smooth muscle libres arranged in interlacing bundles. H&E x 350.
References Bahary GM, Gordeski IG, Nilly M, Neri A, Avidor I, Garti IJ: Intravascular leiomyomatosis. Obstet Gynecol 59(6 Suppl): 13S, 1982. Bresica RI, Tazecar HD, Hobbs I, Miller AW: Intravascular leiomyomatosis: a report of two cases. Human Path 20: 252, 1989. Evans AT, Symmonds RE, Gaffey TA: Recurrent pelvic intravenous leiomyomatosis. Obstet Gynecol 57: 260, 1981. Ivey NS, Norris IJ: Intimal vascular lesions associated with female reproductive steroids. Arch Pathol 96: 227, 1973. Marshall JF, Morris DS: Intravenous leiomyomatosis of the pelvis: case report. Ann Surg 149: 126, 1959. Mukherjee SK: Intravenous leiomyomatosis diagnosis and management. NY State J Med 79: 1905, 1979. Nogales FF, Navarro N, Martinez de VJM, Contreras F, Redondo C, Herraiz MA, Seco MA, Velasco A: Uterine intravascular leiomyomatosis: an update and report of seven cases. Int J Gynecol Pathol 6: 331, 1987. In1 J Gynecol Obsret 37
Norris HJ, Parmley T: Mesenchymal tumors of the uterus v intravenous leiomyomatosis: a clinical and pathological study of I4 cases. Cancer 36: 2164, 1975. Novak ER, Woodruff JD: Novak’s Gynecologic and Obstetric Pathology, pp 248, 279. WB Saunders, Washington, DC, 1974. Roman DD, Mirchandani H: Intravenous leiomyoma with intracardiac extension causing sudden death. Arch Pathol Lab Med 3: 1176, 1987. Steiner G, Warren JW, Judd AS: Intravenous leiomyomatosis. Am J Obstet Gynecol 87: 166, 1963. Suginani H, Kaura R, Ckhi H, Matsuura S: Intravenous leiomyomatosis with cardiac extension: successful surgical management and histopathologic study. Obstet Gynecol 76: 521, 1990. Address for reprints: A.M.A. Mullings Department of Obstetrics and Gynaecology University Hospital of The West Indies Mona, Kingston 7 Jamaica
285
International Federation of Gynecology and Obstetrics
Intravenous A.M.A.
Mullings,
leiomyomatosis
with massive ascites
G. Char”, M.P. Martina,
J. Frederick,
N. Walton
and K. Pickering
Departments of Obstetrics and Gynaecology and ‘Pathology, University of The West Indies. Kingston (Jamaicu)
(Received July 14th, 1991) (Revised and accepted August 22nd. 1991)
Abstract
A case of intravenous leiomyomatosis with massive ascites is reported, This is the first such recorded case. The patient was treated with a subtotal abdominal hysterectomy and bilateral salpingo-oophorectomy. Pathological examination established a vessel wall origin. There is no evidence of recurrence up to 20 months after initial treatment. Keywords: Intravenous leiomyomatosis; Ascites; Clinico-pathological. Introduction
A rare gynecological tumor, the intravenous leiomyoma (IVL), is a benign smooth muscle tumor growing within veins [9]. It may arise primarily from the blood vessel or from the uterus itself with extension into the venous system. Though slow growing and benign it may be fatal largely due to its location [2,3,10]. A review of the literature reveals 60 reported cases including a recent report [2,12]. This is the first with ascites and the first from the Caribbean. Case report
A 46-year-old hypertensive unmarried negro woman was admitted to the University 0020-7292/92/$05.00 0 1992 International Federation of Gynecology and Obstetrics Printed and Published in Ireland
Hospital in April 1989 giving a 4-month history of swollen abdomen with a rapid increase in size over the previous 7 days. Of significance in the history was weight loss, anorexia, weakness and abdominal pain but no menstrual or reproductive organ dysfunction. Examination revealed a moderately distressed middle-aged lady with a grossly distended abdomen. No masses were palpable and a fluid thrill was elicited. Abdominal ultrasound showed a predominantly solid pelvic mass with cystic areas and the ascites. Liver architecture was normal. Abdominal paracentesis was performed and 5.5 1 of straw colored fluid drained. Cytological studies of this fluid were negative for malignancy showing only polymorphs, lymphocytes and mesothelial cells. A barium series was normal. At surgery a further 10 1 of ascitic fluid was drained and a vascular cystic tumor measuring approximately 12 x 18 cm involving the right side of the uterus, round ligament, parietal peritoneum and dome of bladder was seen. There were no palpable nodes. A subtotal hysterectomy with bilateral salpingooophorectomy was performed. On gross pathological examination of the uterus it was found to be markedly distorted on the right by a large lobulated mass extending laterally from the uterine wall. The cut surface showed coiled, nodular, well demarcated firm masses in dilated myometrial veins within the right broad ligament from the Case Report
286
Mullings ef al.
mesosalpinx superiorly to the vault at the lower margin. Laterally the intravenous worm-like masses were expanding to form a large mass which was partly cystic, partly solid with areas of infarction and recent and old hemorrhages (Fig. 1). Microscopically the tumor was present in multiple venous channels lined by endotheliurn and grew within these channels (Fig. 2). In places these were attached to the wall as if arising from it. All the intravenous masses and the largest were composed of spindle shaped smooth muscle fibers arranged in whorls and interlacing bundles (Fig. 3). In places they appeared epitheloid. Stromal fibrosis, hyalinization and presence of
Fig. 1. venous
Cut surface of the uterus showing coiled nodular masses with parametrial
Int J Gynecol Obsret 37
extension.
intra-
numerous thick walled blood vessels were prominent features. Nuclear atypia and mitotic activity were absent. The overall histological picture was that of intravenous leiomyomatosis. Discussion The condition of intravenous leiomyomatosis (IVL) was first described by Birch Hirschfield in 1896 and the first case of intracardiac spread by Durek in 1907 [lo]. Reference in English language textbooks to a similar condition was found as early as 1922 [6]. Mukherjee [6] in his report agrees with others that the clinical features are essentially the same as for fibromyomata and that the surprise usually comes with the surgery. Roman and Mirchandani [lo], in their report, attempted to give a clinical profile. From their review of 50 cases 48 or 96% of cases were in women, possibly implying that it may occur in males. The age range was 28-76 with a median of 46 years, the age of this patient, and a mean of 46.4 years. No specific data are given as to race or parity, but from the published data reviewed parity is variable’. None of the reports reviewed mentioned ascites as a feature of the disease and this was the most striking feature in our patient. The nonrecurrence of ascites up to 20 months after treatment is strong evidence to suggest a direct relationship to IVL. At laparotomy the differential diagnosis would include endometrial stromatosis, uterine sarcoma, disseminated peritoneal leiomyomatosis and benign metastasizing leiomyoma [3,6]. Clinical distinction at the time of surgery could be important since it may influence the extent of surgery. A frozen section will be of help. There is no doubt however, that surgical excision is required and that this can be successfully done even with intracardiac extension [ 10,121. Extrauterine extension occurs in about 50% of cases of which 25% go beyond the broad ligament to involve the iliac veins, inferior vena cava and the right side of the heart [2]. It is therefore
Inrravenous leiomyomalosi.~ with as&es
Fig. 2.
Low magnification
of a typical
intravenous
growth.
H&E
important to palpate the vessels in and around the site of the tumor. The histogenesis of this tumor is uncertain. Evans et al. [3] stated that Knauer in 1903 postulated an origin from the vessel wall and Sitzenfrey, in 1911 that they are primary uterine leiomyomas. Evidence collected since then supports both theories. Marshall and Morris [5] did not see any typical leiomyoma in 17 cases, but others [1,7,8,1 l] all reported IVL extending from preexisting leiomyoma. Norris and Parmley [8] further concluded from their report that both theories were correct. It appears that when IVL results from vascular invasion the bulk of the tumor is ex-
281
x 38.
travascular with no site of origin from the vessel wall. Where venous smooth muscle is the origin, it is predominantly or entirely intravenous with many sites of attachment. That estrogen can induce proliferation of endothelial cells which can differentiate into smooth muscle has been suggested by some authors [4,6,12]. Testing for estrogen receptors has been used to try and substantiate this theory. The treatment is primarily surgical excision. Recurrence after excision is rare but amenable to repeated excision. At present neither chemo- nor radiotherapy has proven to be of any benefit [6]. Case Report
288
Mullings
Fig. 3.
et al.
Microphotograph
of the tumor showing smooth muscle libres arranged in interlacing bundles. H&E x 350.
References Bahary GM, Gordeski IG, Nilly M, Neri A, Avidor I, Garti IJ: Intravascular leiomyomatosis. Obstet Gynecol 59(6 Suppl): 13S, 1982. Bresica RI, Tazecar HD, Hobbs I, Miller AW: Intravascular leiomyomatosis: a report of two cases. Human Path 20: 252, 1989. Evans AT, Symmonds RE, Gaffey TA: Recurrent pelvic intravenous leiomyomatosis. Obstet Gynecol 57: 260, 1981. Ivey NS, Norris IJ: Intimal vascular lesions associated with female reproductive steroids. Arch Pathol 96: 227, 1973. Marshall JF, Morris DS: Intravenous leiomyomatosis of the pelvis: case report. Ann Surg 149: 126, 1959. Mukherjee SK: Intravenous leiomyomatosis diagnosis and management. NY State J Med 79: 1905, 1979. Nogales FF, Navarro N, Martinez de VJM, Contreras F, Redondo C, Herraiz MA, Seco MA, Velasco A: Uterine intravascular leiomyomatosis: an update and report of seven cases. Int J Gynecol Pathol 6: 331, 1987. In1 J Gynecol Obsret 37
Norris HJ, Parmley T: Mesenchymal tumors of the uterus v intravenous leiomyomatosis: a clinical and pathological study of I4 cases. Cancer 36: 2164, 1975. Novak ER, Woodruff JD: Novak’s Gynecologic and Obstetric Pathology, pp 248, 279. WB Saunders, Washington, DC, 1974. Roman DD, Mirchandani H: Intravenous leiomyoma with intracardiac extension causing sudden death. Arch Pathol Lab Med 3: 1176, 1987. Steiner G, Warren JW, Judd AS: Intravenous leiomyomatosis. Am J Obstet Gynecol 87: 166, 1963. Suginani H, Kaura R, Ckhi H, Matsuura S: Intravenous leiomyomatosis with cardiac extension: successful surgical management and histopathologic study. Obstet Gynecol 76: 521, 1990. Address for reprints: A.M.A. Mullings Department of Obstetrics and Gynaecology University Hospital of The West Indies Mona, Kingston 7 Jamaica
