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CLINICAL LETTERS
Massive Necrosis of a Complete Hydatidiform Mole in a Twin Pregnancy With a Surviving Coexistent Fetus We describe a case of a twin pregnancy with a complete hydatidiform mole and a coexistent fetus diagnosed at 15 weeks’ gestation that resulted in a surviving fetus and massively necrotic molar tissue at 32 weeks 2 days’ gestation. Sonographic examination of a 27-year-old woman, gravida 2, para 1, showed a 15-week-old live fetus with a normal placenta and a mass with multiple cysts in the right lateral portion of the uterus (Figure 1A). Both ovaries were enlarged and multicystic. The serum β-human chorionic gonadotropin (β-hCG) value was greater than 200,000
mIU/mL. Amniocentesis revealed a normal 46,XY karyotype at 16 weeks, and a diagnosis of a complete hydatidiform mole with a coexistent fetus was made. On subsequent sonographic examinations the molar tissue was seen to have changed in appearance; at 26 weeks, it showed a heterogeneous texture with irregular cystic areas (Figure 1B), and at 32 weeks it was indistinguishable from a normallooking placenta with few anechoic images (Figure 1C). Fetal growth restriction was detected. At 32 weeks, a rise in blood pressure to 150/90 mm Hg and proteinuria of 1.8 g/24 h were observed after a decrease in the platelet count to 57,000 cells/mm3 had occurred 2 days earlier. Other laboratory test results for HELLP syndrome (hemolysis, elevated liver enzyme lev-
Figure 1. Complete hydatidiform mole and coexistent fetus. A, Transverse sonogram at 15 weeks’ gestation showing a sonographically normal fetus and associated normal placenta (P). There was an adjacent large mass (M) with multiple anechoic areas, suspected of being molar tissue in the right lateral portion of the uterus. B, At 26 weeks’ gestation the sonographic appearance of the molar tissue had changed, showing a different heterogeneous texture with irregular cystic areas. C indicates theca lutein cyst. C, At 32 weeks’ gestation, the molar tissue was indistinguishable from a normal-looking placenta with few anechoic images. D, Photomicrograph of necrotic molar villi. Some viable trophoblastic cells with nuclear atypia are shown in the top right corner. A
B
C
D
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els, and low platelet count) were normal. Preeclampsia associated with thrombocytopenia was diagnosed, and a cesarean delivery was performed at 32 weeks 2 days. After the delivery of a 1240-g male neonate and its normallooking placenta, a brown amorphous sticky necrotic tissue was found in the right portion of the uterine cavity. Some vesicles of the hydatidiform mole were identified. It was not possible to remove that tissue completely because attempts to do so resulted in a friable bloody surface in the uterine cavity with substantial bleeding. In view of the patient’s low platelet count and the risk of infection in the remaining necrotic tissue, hysterectomy was performed. Pathologic examination revealed massively necrotic complete hydatidiform molar tissue (Figure 1D) adhering to the endometrium, but there were no signs of persistent trophoblastic disease. In spite of its prematurity and growth restriction, the neonate had an uneventful neonatal course and was discharged in good condition. β-Human chorionic gonadotropin surveillance showed that its level fell from greater than 200,000 mIU/mL at 15 weeks to 28,764 mIU/mL at 30 weeks, and this decrease may have been attributed to the necrosis of the molar tissue. The blood pressure normalized on the second postoperative day, and the β-hCG level and platelet count normalized after 33 days. The coexistence of a healthy fetus with a hydatidiform mole is a rare event, occurring in 1 per 20,000 to 100,000 pregnancies.1 Morphologically, these twin pregnancies consist of two clearly distinct regions: a normal placenta connected to a healthy fetus and a second placenta with hydatidiform changes. The chances of a live birth are diminished because of the high frequencies of spontaneous abortion and intrauterine death and also because of required pregnancy terminations in cases with severe maternal complications, mainly hemorrhage and preeclampsia. There is also a risk of persistent trophoblastic disease.1–4 In the literature, despite there being no increased risk of persistent trophoblastic disease with the continuance of pregnancy,2,3 only about 33%2 and 40%3 of these pregnancies had a live outcome, excluding those 55%2 and 31%3 of women that presented with spontaneous abortion or opted to terminate the pregnancy, respectively. Moreover, in the past, most gestations with a complete hydatidiform mole and a coexistent fetus were terminated immediately after diagnosis, resulting in a paucity of information concerning its natural history.5 The larger part of the literature relating to this entity consists of case reports, small case series, and reviews of case reports. About 70% of cases of complete hydatidiform moles with coexistent fetuses were suspected on sonography at the end of the first or during the second trimester of pregnancy.1,6,7 In a case
178
of complete hydatidiform mole and a surviving coexistent fetus, Lee et al7 described a shrinking in the molar portion of the placenta and a concomitant decrease in the serum β-hCG level. Our case is unique in demonstrating that the typical image of the mole seen in a complete hydatidiform mole with a coexistent fetus may change considerably during the course of pregnancy because of necrosis, converting to a normal placental pattern and eventually making its diagnosis impossible. If our patient had presented for sonography for the first time at 32 weeks with the onset of preeclampsia, the diagnosis of a coexistent molar pregnancy would not have been made prenatally. We suggest that this possibility should be considered in patients who present with preeclampsia and who have not had prior sonograms, and a careful placental pathologic examination should be recommended. This change to an almost normal placental appearance may explain some reports in which sonography failed to show complete hydatidiform moles with coexistent fetuses, which could only be suspected and confirmed on examination of the placenta after delivery.1,8,9 The fall in the serum β-hCG level associated with shrinking7 or massive necrosis of the molar tissue, as observed in our case, may be indicative of a successful pregnancy outcome with a viable fetus, as has been suggested in the literature.7,10 Maria Okumura, MD, Koji Fushida, MD, Rossana Pulcineli Vieira Francisco, PhD, Regina Schultz, MD, Marcelo Zugaib, PhD Department of Obstetrics (M.O., K.F., R.P.V.F., M.Z.) Division of Pathology (R.S.) Hospital das Clínicas da Faculdade de Medicina University of Sao Paulo Sao Paulo, Brazil doi:10.7863/ultra.33.1.177
References 1.
2.
3.
4.
Steller MA, Genest DR, Bernstein MR, Lage JM, Goldstein DP, Berkowitz RS. Natural history of twin pregnancy with complete hydatidiform mole and coexisting fetus. Obstet Gynecol 1994; 84:35–42. Matsui H, Sekiya S, Hando T, Wake N, Tomoda Y. Hydatidiform mole coexistent with twin live fetus: a national collaborative study in Japan. Hum Reprod 2000; 15:608–611. Sebire NJ, Foskett M, Paradinas FJ, et al. Outcome of twin pregnancies with complete hydatidiform mole and healthy co-twin. Lancet 2002; 359:2165–2166. Niemann I, Sunde L, Petersen LK. Evaluation of the risk of persistent trophoblastic disease after twin pregnancy with diploid hydatidiform mole and coexisting normal fetus. Am J Obstet Gynecol 2007; 197:45.e1–45.e5.
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5.
Vaisbuch E, Ben-Arie A, Dgani R, Perlman S, Sokolovsky N, Hagay Z. Twin pregnancy consisting of a complete hydatidiform mole and coexistent fetus: report of two cases and review of literature. Gynecol Oncol 2005; 98:19–23. 6. Massardier J, Golfier F, Journet D, et al. Twin pregnancy with complete hydatidiform mole and coexistent fetus: obstetrical and oncological outcomes in a series of 14 cases. Eur J Obstet Gynecol Reprod Biol 2009; 143:84–87. 7. Lee SW, Kim MY, Chung JH, Yang JH, Lee YH, Chun YM. Clinical findings of multiple pregnancy with a complete hydatidiform mole and coexisting fetus. J Ultrasound Med 2010; 29:271–280. 8. Soper JT. Gestational trophoblastic disease. Obstet Gynecol 2006; 108:176–187. 9. Chesnais AL, Le Breton F, Devouassoux-Shisheboran M, et al. Twin pregnancy with both complete hydatiform mole and coexistent alive fetus: report of a non-antenatal diagnosed case [in French]. Ann Pathol 2011; 31:299–302. 10. Kihara M, Usui H, Tanaka H, Inoue H, Matsui H, Shozu M. Complicating preeclampsia as a predictor of survival of the fetus in complete hydatidiform mole coexistent with twin fetus. J Reprod Med 2012; 57:325–328.
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CLINICAL LETTERS
Massive Necrosis of a Complete Hydatidiform Mole in a Twin Pregnancy With a Surviving Coexistent Fetus We describe a case of a twin pregnancy with a complete hydatidiform mole and a coexistent fetus diagnosed at 15 weeks’ gestation that resulted in a surviving fetus and massively necrotic molar tissue at 32 weeks 2 days’ gestation. Sonographic examination of a 27-year-old woman, gravida 2, para 1, showed a 15-week-old live fetus with a normal placenta and a mass with multiple cysts in the right lateral portion of the uterus (Figure 1A). Both ovaries were enlarged and multicystic. The serum β-human chorionic gonadotropin (β-hCG) value was greater than 200,000
mIU/mL. Amniocentesis revealed a normal 46,XY karyotype at 16 weeks, and a diagnosis of a complete hydatidiform mole with a coexistent fetus was made. On subsequent sonographic examinations the molar tissue was seen to have changed in appearance; at 26 weeks, it showed a heterogeneous texture with irregular cystic areas (Figure 1B), and at 32 weeks it was indistinguishable from a normallooking placenta with few anechoic images (Figure 1C). Fetal growth restriction was detected. At 32 weeks, a rise in blood pressure to 150/90 mm Hg and proteinuria of 1.8 g/24 h were observed after a decrease in the platelet count to 57,000 cells/mm3 had occurred 2 days earlier. Other laboratory test results for HELLP syndrome (hemolysis, elevated liver enzyme lev-
Figure 1. Complete hydatidiform mole and coexistent fetus. A, Transverse sonogram at 15 weeks’ gestation showing a sonographically normal fetus and associated normal placenta (P). There was an adjacent large mass (M) with multiple anechoic areas, suspected of being molar tissue in the right lateral portion of the uterus. B, At 26 weeks’ gestation the sonographic appearance of the molar tissue had changed, showing a different heterogeneous texture with irregular cystic areas. C indicates theca lutein cyst. C, At 32 weeks’ gestation, the molar tissue was indistinguishable from a normal-looking placenta with few anechoic images. D, Photomicrograph of necrotic molar villi. Some viable trophoblastic cells with nuclear atypia are shown in the top right corner. A
B
C
D
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els, and low platelet count) were normal. Preeclampsia associated with thrombocytopenia was diagnosed, and a cesarean delivery was performed at 32 weeks 2 days. After the delivery of a 1240-g male neonate and its normallooking placenta, a brown amorphous sticky necrotic tissue was found in the right portion of the uterine cavity. Some vesicles of the hydatidiform mole were identified. It was not possible to remove that tissue completely because attempts to do so resulted in a friable bloody surface in the uterine cavity with substantial bleeding. In view of the patient’s low platelet count and the risk of infection in the remaining necrotic tissue, hysterectomy was performed. Pathologic examination revealed massively necrotic complete hydatidiform molar tissue (Figure 1D) adhering to the endometrium, but there were no signs of persistent trophoblastic disease. In spite of its prematurity and growth restriction, the neonate had an uneventful neonatal course and was discharged in good condition. β-Human chorionic gonadotropin surveillance showed that its level fell from greater than 200,000 mIU/mL at 15 weeks to 28,764 mIU/mL at 30 weeks, and this decrease may have been attributed to the necrosis of the molar tissue. The blood pressure normalized on the second postoperative day, and the β-hCG level and platelet count normalized after 33 days. The coexistence of a healthy fetus with a hydatidiform mole is a rare event, occurring in 1 per 20,000 to 100,000 pregnancies.1 Morphologically, these twin pregnancies consist of two clearly distinct regions: a normal placenta connected to a healthy fetus and a second placenta with hydatidiform changes. The chances of a live birth are diminished because of the high frequencies of spontaneous abortion and intrauterine death and also because of required pregnancy terminations in cases with severe maternal complications, mainly hemorrhage and preeclampsia. There is also a risk of persistent trophoblastic disease.1–4 In the literature, despite there being no increased risk of persistent trophoblastic disease with the continuance of pregnancy,2,3 only about 33%2 and 40%3 of these pregnancies had a live outcome, excluding those 55%2 and 31%3 of women that presented with spontaneous abortion or opted to terminate the pregnancy, respectively. Moreover, in the past, most gestations with a complete hydatidiform mole and a coexistent fetus were terminated immediately after diagnosis, resulting in a paucity of information concerning its natural history.5 The larger part of the literature relating to this entity consists of case reports, small case series, and reviews of case reports. About 70% of cases of complete hydatidiform moles with coexistent fetuses were suspected on sonography at the end of the first or during the second trimester of pregnancy.1,6,7 In a case
178
of complete hydatidiform mole and a surviving coexistent fetus, Lee et al7 described a shrinking in the molar portion of the placenta and a concomitant decrease in the serum β-hCG level. Our case is unique in demonstrating that the typical image of the mole seen in a complete hydatidiform mole with a coexistent fetus may change considerably during the course of pregnancy because of necrosis, converting to a normal placental pattern and eventually making its diagnosis impossible. If our patient had presented for sonography for the first time at 32 weeks with the onset of preeclampsia, the diagnosis of a coexistent molar pregnancy would not have been made prenatally. We suggest that this possibility should be considered in patients who present with preeclampsia and who have not had prior sonograms, and a careful placental pathologic examination should be recommended. This change to an almost normal placental appearance may explain some reports in which sonography failed to show complete hydatidiform moles with coexistent fetuses, which could only be suspected and confirmed on examination of the placenta after delivery.1,8,9 The fall in the serum β-hCG level associated with shrinking7 or massive necrosis of the molar tissue, as observed in our case, may be indicative of a successful pregnancy outcome with a viable fetus, as has been suggested in the literature.7,10 Maria Okumura, MD, Koji Fushida, MD, Rossana Pulcineli Vieira Francisco, PhD, Regina Schultz, MD, Marcelo Zugaib, PhD Department of Obstetrics (M.O., K.F., R.P.V.F., M.Z.) Division of Pathology (R.S.) Hospital das Clínicas da Faculdade de Medicina University of Sao Paulo Sao Paulo, Brazil doi:10.7863/ultra.33.1.177
References 1.
2.
3.
4.
Steller MA, Genest DR, Bernstein MR, Lage JM, Goldstein DP, Berkowitz RS. Natural history of twin pregnancy with complete hydatidiform mole and coexisting fetus. Obstet Gynecol 1994; 84:35–42. Matsui H, Sekiya S, Hando T, Wake N, Tomoda Y. Hydatidiform mole coexistent with twin live fetus: a national collaborative study in Japan. Hum Reprod 2000; 15:608–611. Sebire NJ, Foskett M, Paradinas FJ, et al. Outcome of twin pregnancies with complete hydatidiform mole and healthy co-twin. Lancet 2002; 359:2165–2166. Niemann I, Sunde L, Petersen LK. Evaluation of the risk of persistent trophoblastic disease after twin pregnancy with diploid hydatidiform mole and coexisting normal fetus. Am J Obstet Gynecol 2007; 197:45.e1–45.e5.
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5.
Vaisbuch E, Ben-Arie A, Dgani R, Perlman S, Sokolovsky N, Hagay Z. Twin pregnancy consisting of a complete hydatidiform mole and coexistent fetus: report of two cases and review of literature. Gynecol Oncol 2005; 98:19–23. 6. Massardier J, Golfier F, Journet D, et al. Twin pregnancy with complete hydatidiform mole and coexistent fetus: obstetrical and oncological outcomes in a series of 14 cases. Eur J Obstet Gynecol Reprod Biol 2009; 143:84–87. 7. Lee SW, Kim MY, Chung JH, Yang JH, Lee YH, Chun YM. Clinical findings of multiple pregnancy with a complete hydatidiform mole and coexisting fetus. J Ultrasound Med 2010; 29:271–280. 8. Soper JT. Gestational trophoblastic disease. Obstet Gynecol 2006; 108:176–187. 9. Chesnais AL, Le Breton F, Devouassoux-Shisheboran M, et al. Twin pregnancy with both complete hydatiform mole and coexistent alive fetus: report of a non-antenatal diagnosed case [in French]. Ann Pathol 2011; 31:299–302. 10. Kihara M, Usui H, Tanaka H, Inoue H, Matsui H, Shozu M. Complicating preeclampsia as a predictor of survival of the fetus in complete hydatidiform mole coexistent with twin fetus. J Reprod Med 2012; 57:325–328.
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