Hemangiomas of the Umbilical Cord

Abstract

Four cases of umbilical cord hemangioma are presented and analyzed with all previously reported cases. Umbilical cord hemangiomas consist of an angiomatous nodule containing and encompassed by edema and myxomatous degeneration of Wharton's jelly, often cystic. The angiomatous nodules range from 0.2 to 7 cm in largest dimension, whereas the associated edema may measure up to 15 cm. The tumors tend to occur at the placental end of the cord and presumably arise from one or more of the major umbilical vessels. The angiomatous nodules tend to be sharply demarcated from the surrounding stroma and are invariably cytologically benign. Although cord hemangiomas are strongly associated with fetal morbidity and mortality, a causal relationship has not been definitely established. There is no apparent association between cord hemangiomas and maternal age, race, or gravidity nor does one sex predominate. Unlike placental hemangiomas, cord hemangiomas are not associated with maternal hydramnios. Ultrastructural differences between umbilical cord and placental amnion explain both this differing clinical effect as well as the presence of marked edema and myxomatous degeneration of Wharton's jelly in cord tumors. Speculation concerning an underlying congenital predisposition to vascular neoplasm in these infants is presented.

An angiomyxoma involving the whole length of the umbilical cord. Prenatal diagnosis by ultrasonography.

“…4 Potential fetal long-term complications of cord angiomyxomas include vascular compression of the main cord vessels with abnormal growth and possible intrauterine death. 1 Raised maternal serum alpha-fetoprotein, as in the present case, can be the earliest prenatal clue to the development of a cord angiomyxoma. 4 When the fetus appears structurally normal, placental chorioangioma (which has a different prognosis 5 and is also associated with raised levels of alpha-fetoprotein 6 ) must be excluded.…”

“…1 We are not aware of another case in which the entire length of the cord was infiltrated by angiomatous tissue. Theoretically, cord angiomyxoma may arise from one or more umbilical vessels or from remnants of embryonic vessels.…”

“…Theoretically, cord angiomyxoma may arise from one or more umbilical vessels or from remnants of embryonic vessels. 1 .3 However, in an extended tumor, the origin of the angiomatous tissue may be difficult to establish after birth. In the present case ultrasonography allowed the early location of the tumor at the placental insertion of the cord and enabled follow-up of its extension in direction of the fetus.…”

Your diagnosis? Umbilical cord tumor

“…Hemangioma of the umbilical cord is characterized by welldefined aggregates of closely packed, thin-walled capillary proliferation originating from the umbilical arteries, the umbilical vein or vitelline capillaries [7]. The etiology is still not clear; it may represent a true neoplasm or it may be a developmental abnormality (hamartoma).…”

“…Most often hemangioma is an isolated anomaly, but large lesions have been described in association with polyhydramnios [2], intrauterine growth retardation, elevated maternal serum alpha-fetoprotein [9] and fetal malformations [2]. Some authors have also reported its association with premature delivery and even with fetal death caused by impaired umbilical circulation resulting in nonimmune hydrops fetalis [4], torsion, compression, or stenosis of the umbilical vessels, fetal hemorrhage [7], thrombosis of an umbilical vessel and hematoma of the umbilical cord [11]. We reviewed 37 reports in the literature from 1951 through 2005, in most of which the lesion was referred to as hemangioma, and rarely as angiomyxoma, myangioma or hemangiofibromyxoma because of the myxoid appearance of the commonly associated edematous degeneration of the Wharton's jelly.…”