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Huge Placental Myxoid Chorangioma Presenting with Severe Antepartum Hemorrhage

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Huge Placental Myxoid Chorangioma Presenting with Severe Antepartum Hemorrhage CASE REPORT J Nep Med Assoc 2006; 45: 366-369 HUGE PLACENTAL MYXOID CHORANGIOMA PRESENTING WITH SEVERE ANTEPARTUM HEMORRHAGE Bagga R*, Suri V*, Srinivasan R*, Chadha S*, Chopra S*, Gupta N* * Postgraduate Institute of Medical Education & Research, Sector 12, Chandigarh - 160012, India. ABSTRACT A 2nd gravida presented at 27 weeks with antepartum hemorrhage. Ultrasound showed a single live fetus (parameters ~23 weeks) and a huge placenta with multiple cystic areas extending into the lower uterine segment; some of which showed increased vascularity on colored doppler. A diagnosis of low lying placenta with a possibility of chorangioma or a partial mole was made. The next bout of antepartum hemorrhage was severe necessitating an emergency cesarean. Histopathology of the placenta (weighing 2240 grams) revealed a myxoid chorangioma. All features in this woman are uncommonly reported in literature (large size, myxoid degeneration and severe antepartum hemorrhage necessitating a cesarean). Key Words: Placental Chorangioma; Placental Chorioangioma, Antepartum Hemorrhage, Myxoid. INTRODUCTION associated with both maternal and fetal complications. Chorangiomas consist of blood vessels and stroma which Placental chorangioma (also known as chorioangioma) proliferate beyond normally developing chorionic villi. is the commonest benign tumor of the placenta with an Marchetti2 described three histological tumor types which incidence of 1% 1. These tumors may be single or multiple are believed to represent various phases of development. and are rounded, encapsulated and well demarcated This tumor is considered to be a hamartoma of primitive within the placental parenchyma. However, most tumors chorionic mesenchyme and may be of angiomatous, are small and go unnoticed because these are not visible cellular or degenerate varieties. Among the degenerate to the naked eye and all placentas are not examined by variety, the myxoid degeneration is very rare.2 pathologists. Large placental chorangiomas (>5 cm) are rare and are reported to occur among 0.2-4/10,000 live We report a case of huge myxoid chorangioma (weighing births.1 Recurrence is mostly unknown. Large tumors are 2240 g) presenting with antepartum hemorrhage. All Address for correspondence : Dr. Rashmi Bagga Postgraduate Institute of Medical Education & Research, Sector 12, Chandigarh - 160012, India Email: [email protected] Received Date : 17th Jan, 2006 Accepted Date : 12th Aug, 2006 JNMA, Oct - Dec, 2006, 45 367 Bagga et al. Huge Placental Chorangioma and APH features in this woman are uncommonly reported in episode of bleeding necessitating an emergency cesarean. literature (large size, myxoid degeneration and severe Her blood pressure was 100/70, pulse 120/mimute and antepartum hemorrhage necessitating a cesarean). hemoglobin was 9.0G/dl. A female baby weighing 600 grams with an assessed gestational age of 26 weeks, Apgar CASE REPORT score of 5 and 7 and no obvious congenital malformation was delivered. The placenta was huge (weight 2240 A 24 years 2nd gravida presented at 27 weeks with painless grams), friable and removed piece meal (Figure 1). She vaginal bleeding (~50cc). She was normotensive and there received 2 units of blood transfusion. Her post-operative was no history of chronic or gestational hypertension. period was uneventful. The baby’s abdominal ultrasound Ultrasound revealed a single live fetus (parameters ~23 showed two focal hemangiomas in the liver measuring weeks) and a huge placenta starting from the fundus and 0.8×0.9×0.5 cms and 2.3×1.3×1.6 cms and cranial reaching up to the internal os with multiple cystic areas; ultrasound revealed multiple small cystic lesions in the the largest of which measured 7.1x 6.7cm. These cystic frontal region suggestive of encephalomalacia. Despite areas showed increased vascularity on doppler. A diagnosis intensive neonatal care, the baby expired on day 3 due to of low lying placenta with a possibility of chorangioma extreme prematurity. or a partial mole was made. Next day she had a severe The placenta showed an admixture of hydropic villi and normal architecture. In addition there were multiple solid areas ranging from 2-6 cms which on cut surface showed a gelatinous appearance. On microscopy there was admixture of normal 3rd trimester villi and hydropic villi. The hydropic villi showed presence of vessels within them and the stroma showed edema or was fibrous (Figure 2). Sections from the solid gelatinous areas showed typical features of a chorangioma with numerous proliferating blood vessels. The intervening stroma showed extensive myxoid change compressing the vessels which appeared to have slit-like lumen (Figure 3). Fig. 1: Gross Photograph of the Placenta These areas were positive on Alcian blue staining. CD34 immunostain confirmed the vascular nature of the tumor Fig. 2: Microphotograph showing an admixture Fig. 3: Extensive myxoid areas in the chorangioma of hydropic and normal sized chorionic villi (original magnification X 200) (original magnification X 200) JNMA, Oct - Dec, 2006, 45 Bagga et al. Huge Placental Chorangioma and APH 368 in the myxoid areas. Overall features were those of a Management options are limited. An ultrasound to exclude multifocal myxoid chorangioma in a 3rd trimester placenta any congenital anomaly and fetal karyotyping are necessary with partial hydropic change. followed by serial ultrasounds to assess tumor growth, fetal growth and development of hydrops. Pregnancy should DISCUSSION be terminated as soon as fetal viability is achieved. In the presence of fetal anemia (not nearing viability) intrauterine Large chorangiomas (>5cm) may be associated with transfusion may be considered. Successful ablation of pregnancy complications like hydramnios (18 to 35%), pre- blood supply of chorangioma by operative fetoscopy has eclampsia (4.8% to 16.4%), preterm labor and increased been reported.10 Laser coagulation of the feeding vessels occurrence of antepartum and postpartum hemorrhage. of a large chorangioma at 25 weeks has been followed by Antepartum hemorrhage is due to premature separation delivery of a healthy baby.11 If karyotype is normal and of the placenta which was further aggravated by the low- hydrops is absent, fetal salvage may be possible. Although lying placenta in the present case. Associated theca lutein aggressive prenatal management has been reported using cysts due to high beta-HCG levels may be present.3,4 amniodrainage, intrauterine transfusions, embolization, Though antepartum hemorrhage (usually due to placental or ligation of the vessels, fetal prognosis remains poor for abruption)5 is associated with large chorioangiomas, the large tumors and largely depends on fetal hemodynamic exact incidence is not reported in literature. tolerance.12 Fetal complications associated with large chorangiomas The management in the present case was of an emergency are growth restriction, anemia, thrombocytopenia, nature as severe antepartum hemorrhage due to the huge disseminated intravascular coagulation, hydrops, stillbirth, placenta (2240g) occupying most of the uterine cavity congenital malformations, and chromosomal anomalies.6,7 including the lower segment needed immediate delivery Rarely, periventricular leukomalacia and hepatic by emergency cesarean in order to prevent complications lymphangioma may occur which were also seen in this to the mother. In cases when the chorangioma is small and case.8,9 Large chorangiomas have the potential to become diagnosed early, other management options as mentioned arteriovenous shunts which compromise fetal circulation above can be attempted in order to achieve a better perinatal by increasing the venous return to the fetal heart causing outcome. An outcome similar to the present case has been cardiac failure, hydrops and stillbirth. Thrombocytopenia, reported recently,12 though in that case the chorangioma anemia and disseminated intravascular coagulation occur was diagnosed at 20 weeks gestation. At 24 weeks, the probably because blood circulates through tortuous patient had preterm labor with ruptured membranes and channels causing sequestration of blood elements. profuse vaginal bleeding. An emergency cesarean section delivered a female newborn of 670 g who died after a few Antenatal diagnosis by ultrasound is possible in larger minutes. A giant (12x12cm) placental chorioangioma was tumors. Findings include well defined circumscript confirmed on histology. The authors concluded that the complex tumors whose echogenicity is different from large volume of the placental tumor may have caused labor rest of placenta. These may be single or multiple and may to start in the second trimester of gestation as well as severe protrude in the amniotic cavity most commonly near the vaginal bleeding that culminated with the interruption of cord insertion. Color doppler shows a pulsatile flow in the pregnancy and neonatal death. The situation in the present vascular channels which is similar to that of the umbilical case is similar to this report. Hence, the perinatal outcome cord. The tumor vascular channels are continuous with the in huge chorangiomas may be different from cases where fetal circulation and thus differentiate chorangioma from smaller tumors are diagnosed early in pregnancy and other pathologies with similar ultrasound findings like various management options which may improve fetal placental teratoma, partial mole and degenerated fibroid. outcome can be undertaken. JNMA, Oct - Dec, 2006, 45 369 Bagga et al. Huge Placental Chorangioma
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