Chorangiocarcinoma of the placenta: a case report and clinical review.

OBJECTIVE: We describe a case of chorangiocarcinoma, a complex lesion consisting of a trophoblastic proliferation within a chorangioma, presenting in a term placenta. MATERIALS AND METHODS: The lesion was diagnosed by ultrasound at a second trimester check-up after amniocentesis, performed because of increased combined risk at first trimester screening for trisomy 21. After uncomplicated vaginal delivery, a healthy child was born and the placenta was expelled spontaneously. RESULTS: Gross examination of the placenta showed a well-demarcated mass, bulging paracentrally from the fetal surface. Histology revealed a trophoblastic proliferation inside a chorangioma, consisting of multiple nodules with characters of focal multinucleation and pleomorphic cell nuclei, extensive central necrosis and high mitotic activity. Immunohistochemical staining showed strong intensity for hCG; Ki-67 (MIB-1) demonstrated a high proliferation index. Histopathological and immunohistochemical profile was compatible with a malignant trophoblastic proliferation. CONCLUSIONS: This is only the fifth reported case of so-called "chorangiocarcinoma" of the placenta (Table 1). However, histopathologically only one reported case was identical to ours. A proliferation of atypical trophoblast was observed inside a chorangioma, which formed as it were a shield around the trophoblast. No extravascular stromal invasion was present. Follow-up revealed no metastases, either in the mother or the child, up to 3 months after birth.

Non-immune hydrops fetalis caused by beta-glucuronidase deficiency (mucopolysaccharidosis VII). Study of a family with 3 affected siblings.

Non-immune hydrops fetalis caused by beta-glucuronidase deficiency (mucopolysaccharidosis VII). Study of a family with 3 affected siblings. We describe a family case of beta-glucuronidase deficiency with 3 consecutively affected siblings. The three fetuses showed hydrops at a very early stage. In the first and second pregnancy the hydrops was visible on ultrasound scan in the first trimester. In the second pregnancy this was highly suggestive for recurrence. The diagnosis of mucopolysaccharidosis type VII was suggested after pathologic examination of the first fetus and placenta, and confirmed by deficient beta-glucuronidase activity in cultured skin fibroblasts. In the second, as well as in the third pregnancy a prenatal biochemical diagnosis was possible on cultured chorionic villus cells. The third pregnancy was terminated before hydrops was visible on ultrasound scan. Pathologic findings in the 3 fetuses were similar. Vacuolated macrophages were found in all tissues, but were most prominent in placenta, liver, lymph nodes and bone marrow.