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2012-01-09-13 Chorioangioma @Tihonenko www.thefetus.net


Chorioangioma

Irina Tihonenko, MD.


Minsk Prenatal Ultrasound Center, 1st Minsk Clinic Hospital, Minsk, Belarus.  




Case report:

A 30-year-old G0P1 woman was referred to our center for routine ultrasound examination at 34 weeks of gestation. She had colpitis at 15 weeks. Ultrasound examinations at 11 and 21 weeks were normal. 
Fetal morphology and biometry were normal and consistent with dates. Ultrasound examination revealed multiple well-circumscribed heterogeneous hypoechoic masses in the placenta (Image 1). Masses were highly vascularized (Image 2). Doppler indices in uterine and fetal arteries were normal. The diagnosis was multiple diffuse chorioangiomas of the placenta. 

Image 1,2:

 
  
The same pictures were seen at 36 weeks. 

A follow-up ultrasound at 37 weeks 6 days found fetal congestive heart failure: cardiomegaly, distension of the right heart (Image 3), vena cave inferior, tricuspid regurgitation, fluid in pericardium. Asymmetric neck skin edema was seen (mostly on the left side) (Image 4). Scalp edema (Image 5) and edema of the thorax and abdominal wall were apparent. 

The placenta was enlarged up to 50 mm. There were multiple, mostly well-defined hypoechoic masses of heterogeneous structure from 10 to 25 mm in diameter with the blood flow pattern similar to umbilical artery with fetal heart rate (Image 6).  

Image3 ,4, 5 ,6: Pericardial fluid, edema, prefrontal edema, Doppler of the chorioangioma

     

 
 
Feto-placental Doppler indices still remain within normal range. However, systolic velocity in the middle cerebral artery was increased up to 89 cm/sec ( Image 7), what was considered as the evidence of fetal anemia.

Image 7: Increased value in the middle cerebral artery velocity Doppler.



Because of the developing fetal hydrops and heart failure a cesarean delivery was performed. On gross examination fetal surface of the placenta was noticeable uneven with multiple well-defined soft lobulated grayish masses up to 3 cm in diameter (image 8).
 
The newborn girl had a weight of 2750 g, Apgar score 5/ventilation. There was tumor-like buldging on the left neck side (image 9). The girl had ascitis, hepatomegaly, mitral and tricuspid incompetence and vena cava inferior distension. The neonatal period was complicated by lung-cardiac failure, edema, lung hemorrhage, hemorrhagic syndrome, anemia and cerebral ischemia. She was infection-free. 
Morphological examination of the placenta revealed delayed maturation of villi – there were mature intermediate and immature terminal villi lacking syncytio-capillary membranes; multiple hemangiomas (chorioangiomas) with stromal fibrosis and myxomatosis. 

Image 8,9: The surface of the placenta, and the edematous newborn.

 
 

Definition. A chorioangioma (haemangioma) is a benign tumor of the placenta, frequently referred to as placental hemangioma or haemangioblastoma. Chorioangioma consists of blood vessels and stroma that proliferates beyond normally developing chorionic villi. 

The pathogenesis of infantile hemangioma is still unknown [1]. In recent literature some attempts of identifying the cell types responsible for tumor initiation have been made. New hypothesis suggests an involvement of progenitor cells in the pathogenesis of this vascular tumor [2].

Incidence. Chorangioma of the placenta is considered to be a rare tumor of placenta with reported frequency is about 1% [3]. Ogino S. and Redline R.W. (2000) found chorangiomas in 36 (0.51%) of examined placentas [4]. Their frequency rises in women over 30 years old. They are often found in primipara and twin pregnancies. Hypertension and diabetes are found more often in combination with chorangiomas than they are in otherwise normal pregnancies [5]. The recurrence risk is not yet known, but appears to be very small [6].

Histological findings: On gross examination, chorioangioma is of heterogeneous structure. It has capsule with some vessels[7]. It is microscopically composed of numerous proliferative blood vessels in various stages of differentiation – endotheliomatous, capillary, cavernous – in fibrous stroma[8]. Immunohistochemicaly, the tumor cells show focal staining for cytokeratin [8], a finding that suggests origin from blood vessels of the chorionic plate and anchoring villi [9]. 

Ultrasound findings. Chorioangiomas vary in size from a few millimeters to several centimeters in diameter. Usually chorioangiomas present as a single mass, but multiple masses can be seen. They are usually located on the fetal surface of the placenta, though these tumors may occur inside of the placental tissue or can buldge from the maternal side. Rarely masses can be located in the membranes and attached to the placenta by a vascular pedicle; chorioangiomas on the umbilical cord have been described as well as variation of these findings [10]. Sonographycally it looks like well-circumscribed placental mass of homogeneous or heterogeneous structure with echogenicity different from that of the placental tissue. Multilocular masses can be found. 

Caldwell et al. described the ultrasound appearance of a diffuse chorioangioma in 1977 [11] as an enlarged placenta. Luis A. Bracero et al. (1993) described a diffuse angiomatous lesion of the placenta which differs from the usual sonographic characteristics of chorioangiomas: the enlarged placenta contained multiple hypoechoic areas. 

Flow patterns of chorioangioma are similar to that of the umbilical cord [12], and this fact confirmes that the vascular channels in the tumor are involved with the fetal circulation. Color Doppler may help to diagnose the choriangioma. Data of fetal umbilical artery Doppler indices are rather contradictory: normal Doppler indices have been recorded in both normal fetal condition and in case of fetal compromise [13],[14]. Unfortunately, there are no studies showing impact of chorioangioma, especially diffuse form, with arteriovenous shunting, on the umbilical circulation. 

Associated anomalies. Association of chorioangioma with congenital anomalies such as hip deformity, inguinal hernia, talipes, ear deformity, metatarsus deformity, and pigmented nevus has been described, but there is no apparent connection between placental tumor and malformations. The incidence of single umbilical artery, skin hemangioma and velamentous insertion of the cord in case of chorioangioma is slightly higher compared to uncomplicated pregnancies [15]. There have been some reports of chromosome abnormalities associated with chorioangiomas [15],[16]. 

Complications. Small chorioangiomas (less than 4 cm in diameter) are though to be inconsequential. Large placental chorangioma may cause a variety of complications affecting the mother, the developing fetus or the neonate [17],[18] and can be associated with fetal heart failure, hydrops fetalis and sudden intrauterine fetal death

Chorioangiomas are often associated with polyhydramnios, but oligohydramnios has also been reported [19],[20]. Increased risk of pre-eclampsia, ante- and postnatal haemorrhage [10], abruptio placentae [15] are described as well as ovarian theca lutein cysts and high levels of hCG [21] and high levels of AFP being associated with chorioangioma [22]. 

Arteriovenous shunts in large chorioangiomas can impair the fetal circulation by increasing the venous return to the heart, causing tachycardia, cardiomegaly and hypervolemia [23],[24]. As a result, there is the possibility of high output cardiac failure, edema, hydrops, and stillbirth [25],[26],[27],[28]. Diffuse placental hemangioma may lead to intrauterine fetal death due to the severe hypoxemic state and the difficulty of venous drainage from the fetus to the mother owing to increased materno-fetal placental shunts [29].

Fetal hemolytic anemia and disseminated intravascular coagulation may occur because of red cell destruction and platelet sequestration. Fetal anemia can also result in hydrops. Feto-maternal hemorrhage may also cause fetal anemia [30]. The abnormal vascular channels reducing placental function lead to intrauterine growth retardation (IUGR) [14].

Outcome depends on the tumor's size. Large and diffuse tumors have been associated with pregnancy complications. A giant haemangioma can endanger the life of mother and fetus [31].

Management. A careful ultrasound examination of the fetus is mandatory to exclude fetal anomaly. Serial ultrasound examinations should be performed to estimate the condition of the tumor, the condition of the fetus and to determine the timing of complications. Doppler should be used for estimation of the fetal well-being. 

Differential diagnosis

Differential diagnosis of chorangioma includes chorangiosis and chorangiomatosis, that presents a diffuse or more often a focal proliferation of villous angioblastema with villi that are not present in chorangioma. They have overlapping similarities with chorangioma, and have similar clinical implications. 

Normal chorionic villi should contain no more than 5 vascular channels, even when the same vessel is present in more than 1 plane of section. 

Chorangiosis has been defined as a process involving terminal villi. Chorangiosis is associated with maternal diabetes mellitus (placentomegaly is especially frequent among placentas with chorangiosis )[32] as well as syphilis, pre-eclampsia etc[33]. Other associations, reported with chorangiosis, include maternal infections, amnion nodosum and drug ingestion (Benirschke and Franciosi, 1999). Placental conditions that have been associated with chorangiosis include umbilical cord anomalies, single umbilical artery, abruptio placentae, placenta previa, chorangioma, amnion nodosum, and villitis (rubella virus, cytomegalovirus, syphilis, and Bartonella sp are known to infect and induce proliferation of the endothelial cells). When these diseases and viruses are present, an abnormal placenta could decompensate acutely, leading to a catastrophic outcome [34]. The fetal factors most commonly associated with chorangiosis are the presence of major congenital anomalies and Apgar scores of less than 5.

Chorangiosis is believed to result from long-standing placental hypoperfusion or low-grade tissue hypoxemia. This occurs in a condition in which placental tissue hypoxia causes villous capillary endothelial cell proliferation and capillary hypervascularity[35]. This diagnosis should be considered in case of fetal compromise especially when no other significant obstetric incident is identified [36].

The diagnostic criteria of chorangiosis were established by Altshuler [37] in 1984 as the presence of a minimum of 10 villi, each with 10 or more vascular channels, in 10 or more areas of 3 or more random, noninfarcted placental areas when using a X10 ocular. The criteria to evaluate the severity of this process include determining the number of vessels within each villus and the placental area throughout which the vasculature is seen37.

Chorangiomatosis is a lesion intermediate between chorangiosis and  chorioangioma. It has similar histological characteristics as chorioangioma; however, chorangiomatosis shows permeated normal villi instead of an expansile lesion (Ogino and Redline, 2000). Chorangiomatosis and chorioangioma are differentiated from chorangiosis histologically by the presence of perivascular cells (positive for muscle-specific actin) and a background of increased stromal collagenization and cellularity in the former two conditions. Chorangiosis is 10-fold commoner than chorioangioma or chorioangiomatosis [4]. Chorangiomatosis has been associated with negative fetal outcomes such as intrauterine growth retardation and preeclampsia. 

Diffuse multifocal chorangiomatosis is often complicated by hydrops fetalis, with extensive subcutaneous edema, and pleural and pericardial effusions and ascites. This condition have shown associations with extreme prematurity (<32 weeks), congenital malformations, IUGR, delayed villous maturation, avascular villi, and placentomegaly [38]. Miscarriage or antenatal death are possible [7].

Ogino S, Redline RW. (2000) concluded that chorioangioma and localized chorangiomatosis are clinically and morphologically similar lesions distinct from chorangiosis. Diffuse multifocal chorangiomatosis is morphologically similar to chorioangioma and localized chorangiomatosis, but has a distinct clinicopathologic profile [4].

It has been reported that both chorangiosis and chorangioma are increased in placentas at high altitudes[39].

Chorioangioma and chorangiomatosis share associations with preeclampia, multiple gestation, and premature delivery at 32 to 26 weeks. Diffuse multifocal chorioangiomatosis  showed associations with extreme prematurity (<32 weeks), congenital malformations, IUGR, delayed villous maturation, avascular villi, and placentomegaly.

Chorioangiomas, chorangiosis and chorioangiomatosis are extremely similar. Morphologically, the differentiation is possible. But there is no clear ultrasound description which enables prenatal differential diagnosis.

Gestational age distribution is different for chorangioma, chorangiosis, and chorangiomatosis [40]. Chorangiosis is more common over 37 weeks and most of deliveries happen in term. Chorangioma and chorangiomatosis are seen from 32 weeks of pregnancy onward [41] and they are associated with pre-term birth. However, other complications are similar for all these conditions. It seems to be that chorioangioma is more focal lesion that chorangiosis and chorioangiomatosis.

Placental chorangioma can often grossly be confused with infarct or intervillous thrombus [42]. Ultrasound pictures of infarct and intervillous thrombus change during pregnancy compared to chorioangiomas.

Angiomatous lesions should be differentiated from submucous myomas. Myomas are closely related to myometrium and they are found near the maternal surface of the placenta.

Partial hydatidiform mole sonographically looks like multiple diffuse sonoluscent areas inside of the placenta. 

Treatment. Treatment modalities of chorangioma include endoscopic devascularization, alcoholic ablation, and interstitial laser coagulation [43].


References

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