Case of the Week #579

Parker S. Sheldon; Roxanna Twedt; Garrett Lam; David N. Jackson
High Risk Pregnancy Center, Hera Women's Health (Las Vegas and Reno, Nevada USA)

Posting Dates: Mar 31, 2023 - Apr 14, 2023

Case report: A 35-year-old multiparous patient was referred for a consultative ultrasound. Serial ultrasounds were performed between 23 and 38 weeks gestation. The following images were obtained.

23 weeks gestation
Image 1 23 weeks gestation
Video 1 23 weeks gestation © 2023 David Jackson
26 6/7 weeks gestation
Image 2 26 6/7 weeks gestation
26 6/7 weeks gestation
Image 3 26 6/7 weeks gestation
26 6/7 weeks gestation
Image 4 26 6/7 weeks gestation
26 6/7 weeks gestation
Image 5 26 6/7 weeks gestation
26 6/7 weeks gestation
Image 6 26 6/7 weeks gestation
Video 2 26 6/7 weeks gestation © 2023 David Jackson
26 6/7 weeks gestation
Image 7 26 6/7 weeks gestation
26 6/7 weeks gestation
Image 8 26 6/7 weeks gestation
27 weeks gestation
Image 9 27 weeks gestation
27 weeks gestation
Image 10 27 weeks gestation
27 weeks gestation
Image 11 27 weeks gestation
27 weeks gestation
Image 12 27 weeks gestation
28 weeks gestation
Image 13 28 weeks gestation
28 weeks gestation
Image 14 28 weeks gestation
33 weeks gestation
Image 15 33 weeks gestation
38 weeks gestation
Image 16 38 weeks gestation

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Answer

We present a case of Umbilical Cord Hemangioma

Ultrasound identified an echogenic mass at the umbilical cord insertion, extending up the cord toward the fetus measuring 7.6cm x 7.1cm x 4cm in diameter. Three-dimensional imaging confirmed a central solid tissue nodule and multiple pseudocysts. Doppler indices were without increased resistance. A small umbilical varix was seen. Serial weekly imaging (with biophysical profile and Doppler of the umbilical artery) identified a progressive mass expansion and polyhydramnios development. At 31 weeks, the fetus developed moderate cardiomegaly, mild pericardial effusion, and an altered E/A ratio. At that point, the mass diameter was 12.9cm x 9.6cm x 5cm. Monitoring was increased to twice a week. The mass and cardiac findings stabilized. There were no abnormalities on monitoring or Doppler. She was delivered vaginally at 38 weeks.  Mother and baby did well. The child did not require any surgical interventions.

On pathologic examination, the placenta weighed 1187g with a 15.0 x 10.0 x 5.0cm tumor near the umbilical cord insertion at the placenta. The gross pathologic evaluation revealed a dense mass with rubber-like consistency and pseudocyst formation. Histologically there were thin-walled, dilated vascular channels distributed in a myxoid matrix, characteristic of a true hemangioma of the umbilical cord.

Cord insertion with pseudocyst and tumor with firm, rubber consistency at the base.
Image 1 Cord insertion with pseudocyst and tumor with firm, rubber consistency at the base.
Cord insertion with pseudocyst and tumor with firm, rubber consistency at the base.
Image 2 Cord insertion with pseudocyst and tumor with firm, rubber consistency at the base.
Central dense mass with thin-walled, dilated vascular channels distributed in a myxoid matrix on histology.
Image 3 Central dense mass with thin-walled, dilated vascular channels distributed in a myxoid matrix on histology.

Discussion

Umbilical cord hemangiomas are rare tumors of the umbilical cord that require serial evaluation and monitoring when detected on prenatal ultrasound. Since 1951, 56 cases of umbilical cord hemangiomas have been reported in the literature. While most cases result in a live birth, evidence suggests that at least a quarter end in demise and approximately 20% in preterm birth. Reports vary regarding the incidence of maternal complications, growth of the mass, integrity of cord and placental vasculature, amniotic fluid indices, as well as the presence of fetal cardiac dysfunction and other associated anomalies.

While hemangiomas of the placenta are well known, identified in an estimated 1% of pregnancies [1], hemangiomas arising within the umbilical cord are far less common, with only 40 cases published since 1990, 33 of which were identified prenatally. These vascular neoplasms typically originate from the umbilical artery. Histologically, they are characterized by endothelial proliferation and present with a distinct pattern of capillaries embedded within myxoid stroma [2,3]. On examination, the lesion presents as a fusiform swelling comprised of vascularized nodules and pseudocysts that cause expansion of Wharton’s jelly manifesting as cord edema [2,4,5]. Progressive cord edema and pseudocyst degeneration may cause mechanical compression of cord vessels, compromising fetal circulation, and leading to associated complications which include fetal cardiac dysfunction, non-immune fetal hydrops, intravascular thrombosis, fetal hemorrhage, and intrauterine fetal demise [3,5]. Furthermore, excessive, closely approximated, low resistance vasculature can lead to arteriovenous malformation, which can further contribute to the development of hydropic changes and cardiac dysfunction [3]. Umbilical cord hemangiomas have also been associated, to varying degrees, with increased maternal serum alpha-fetoprotein levels, other vascular anomalies, polyhydramnios, and preterm birth [6].

Umbilical cord hemangiomas should be suspected when antenatal sonography reveals a hyperechoic mass with or without cystic features, often containing branching, low-resistance vessels [2]. Differential diagnoses include hematomas, varicose veins, aneurysms, thrombosis, abdominal wall hernia, omphalomesenteric duct cysts, allantoic cysts, and tumors such as teratoma and metastatic neuroblastoma [7]. The presence of blood flow on color Doppler with associated uterine artery hypoplasia or the presence of calcifications within the mass can help differentiate hemangioma from teratomas [4,5]. Three-dimensional imaging may improve the spatial orientation of the size, location, and internal characteristics of cord swelling [8].

In our literature review, 33 cases were identified prenatally at an average of 23.7 weeks gestation (Table 1) [2,4,5,7-36]. In the 28 cases that specified tumor location, umbilical cord hemangiomas were found within 10cm of the placenta in 50% of cases, within 10cm of the fetal insertion in approximately 40% of cases, and the remainder were in the mid-cord. These tumors were found to enlarge in nearly 40% of cases, and cystic structures were present in almost half. Umbilical cord hemangiomas are usually an isolated lesion, without major structural anomalies, however, they have been associated with vascular malformations. In our literature review, nearly 25% of umbilical cord hemangiomas were found in patients with cutaneous hemangiomas, visceral hemangiomas, and even Klippel-Trenaunay-Weber syndrome [9,11,18,20,22,30,32,34]. While umbilical cord hemangiomas have been associated with alterations in amniotic fluid, this was not often documented in the reports, and we identified oligohydramnios in 9%, and polyhydramnios in 15%. In cases identified prenatally, 40% resulted in preterm birth and 18% were found to have cardiac failure or dysfunction. In all, fetal survival was approximately 80%.

Table 1Literature review includes 33 prenatally diagnosed cases of umbilical cord hemangioma since 1990.
Study
GA
Location
Growth
Cysts
AFP
Amniotic fluid
Preterm birth
Hydrops / heart failure
Survival
GA: gestational age at diagnosis; AFP: maternal alpha fetoprotein; Y: yes; N: no; NA: not available/applicable; oligo: oligohydramnios; poly: polyhydramnios; IUFD: intrauterine fetal demise; TOP: termination of pregnancy
Jauniaux 1990
20w
placenta
Y
Y
normal
N
none
Y
Ghidini 1990
34w
10cm from fetal
N
Y
normal
N
Y
Gramellini 1993
26w
placenta
N
poly
36w
none
Y
Weyerts 1993
33w
fetal
N
normal
N
Y
Wilson 1994
17w
NA
Y
Y
oligo
36w
Y
Armes 1994
32w
9cm from placenta
poly
34w
N (perinatal)
Shipp 1995
33w
placenta
oligo
36w
Y
Miller 1997
15w
fetal
N
Y
Tennsteadt 1998
18w
fetal
Y
Y
normal
36w
Y
Kamitomo 2000
16w
placenta
Y
NA
IUFD 17w
Sahinoglu 2003
24w
NA
normal
26w
N (perinatal)
Caldarella 2003
32w
placenta
normal
32w
hydrops, cardiomegaly
Y
Daniel-Spiegel 2005
27w
10cm from placenta
Y
Y
normal
oligo
N
Y
Cheng 2006
19w
placenta
Y
N
Y
Iyoob 2006
30w
placenta
Y
poly
30w
heart failure
Y
Natalucci 2006
33w
fetal
N
Y
Malliah 2007
19w
placenta
Y
poly
N
Y
Papadopoulos 2009
22w
22cm from placenta
Y
normal
37w
Y
Gouin 2009
25w
NA
normal
N
Y
Sathiyathasan 2010
22w
NA
NA
IUFD 26w
Matsuda 2011
18w
fetal
Y
Y
N
Y
Hara 2015
26w
placenta
Y
29w
heart failure
Y
Smulian 2016
25w
fetal
36w
hydrops
Y
Akiba 2017
19w
fetal
N
Y
N
Y
Rocha 2017
23w
NA
normal
N
Y
Schwickert 2018
38w
placenta
N
N
Y
Angelico 2019
34w
20cm from placenta
Y
Y
36w
Y
Yu 2020
17w
fetal
N
Y
normal
NA
pericardial effusion
TOP
Zhang 2021
22w
placenta
Y
Y
34w
Y
Hadravská 2021
22w
placenta
N
normal
poly
NA
hydrops
IUFD 28w
Kaur 2022
13w
fetal
Y
Y
33w
Y
Lok 2022
21w
fetal
Y
NA
IUFD 21w
Lisovaja 2022
24w
mid-cord
Y
Y
N
Y

The optimal management of a patient with cord swelling or mass is not clearly defined. Since 1990, serial surveillance has been suggested once a suspected umbilical cord hemangioma is detected on ultrasound [4]. Three decades later, there is no standard for frequency or type of antepartum assessment. Our patient underwent weekly ultrasound examinations and monitoring with biophysical profile. Despite progressive mass enlargement and development of polyhydramnios, there were no concerning findings in the cord vessel dynamics and no fetal distress. Upon detection of fetal cardiac changes at 31 weeks, we increased monitoring to twice weekly with non-stress testing and umbilical artery Doppler assessment. No flow abnormalities were detected and fetal cardiac findings stabilized. Despite a large mass confirmed at delivery, there were no variable decelerations in labor.

Umbilical cord hemangiomas should be suspected when a hyperechoic mass is found along the fetal umbilical cord. As a rare tumor with solid and pseudocystic changes, serious maternal and fetal complications may occur, including preterm birth and fetal demise. While labor may be tolerated, we recommend at least weekly ultrasound surveillance from the time of initial detection to monitor for enlargement, cord vessel compression, thrombus, hemorrhage, polyhydramnios, as well as fetal hydrops and cardiac dysfunction.

References

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[2]  Iyoob SD, Tsai A, Ruchelli ED, et al. Large Umbilical Cord Hemangioma: Sonographic Features With Surgical Pathologic Correlation. J Ultrasound Med. 2006;25(11):1495-1498. doi:10.7863/jum.2006.25.11.1495
[3]  Smulian JC, Sarno AP, Rochon ML, et al. The natural history of an umbilical cord hemangioma. J Clin Ultrasound. 2016;44(7):455-458. doi:10.1002/jcu.22346
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[8]  Akiba Y, Miyakoshi K, Ochiai D, Kawaida M, Matsumoto T, Tanaka M. Umbilical cord hemangioma: Sonographic features by HDlive Flow. Eur J Obstet Gynecol Reprod Biol. 2018;221:195-196. doi:10.1016/j.ejogrb.2017.12.011
[9]  Jauniaux E, Moscoso G, Chitty L, et al. An angiomyxoma involving the whole length of the umbilical cord. Prenatal diagnosis by ultrasonography. J Ultrasound Med. 1990;9(7):419-422. doi:10.7863/jum.1990.9.7.419
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[11]  Weyerts LK, Jones MC, Grafe M, S et al.  Umbilical cord haemangioma associated with an eruptive cutaneous haemangioma in a female infant. Prenat Diagn. 1993;13(1):61-64. doi:10.1002/pd.1970130109
[12]  Wilson RD, Magee JF, Sorensen PHB, et al. In utero decompression of umbilical cord angiomyxoma followed by vaginal delivery. Am J Obstet Gynecol. 1994;171(5):1383-1385. doi:10.1016/0002-9378(94)90167-8
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[14]  Shipp T, Bromley B, Benacerraf B. Sonographically detected abnormalities of the umbilical cord. Int J Gynecol Obstet. 1995;48(2):179-185. doi:10.1016/0020-7292(94)02297-C
[15]  Miller KA, Gauderer MWL. Hemangioma of the umbilical cord mimicking an omphalocele. J Pediatr Surg. 1997;32(6):810-812. doi:10.1016/S0022-3468(97)90625-4
[16]  Tennstedt C, Chaoui R, Bollmann R, D et al.  Angiomyxoma of the Umbilical Cord in One Twin with Cystic Degeneration of Wharton’s Jelly. A Case Report. Pathol - Res Pract. 1998;194(1):55-58. doi:10.1016/S0344-0338(98)80012-5
[17]  Kamitomo M, Sueyoshi K, Matsukita S, et al. Hemangioma of the Umbilical Cord: Stenotic Change of the Umbilical Vessels. Fetal Diagn Ther. 1999;14(6):328-331. doi:10.1159/000020951
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[20]  Daniel-Spiegel E, Weiner E, Gimburg G, et al. The association of umbilical cord hemangioma with fetal vascular birthmarks. Prenat Diagn. 2005;25(4):300-303. doi:10.1002/pd.1109
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[22]  Malliah R, Shah V, Heller D. Umbilical cord hemangioma associated with multiple cutaneous hemangiomas in a newborn. Int J Gynecol Obstet. 2007;99(1):58-58. doi:10.1016/j.ijgo.2007.02.016
[23]  Papadopoulos VG, Kourea HP, Adonakis GL, et al. A case of umbilical cord hemangioma: Doppler studies and review of the literature. Eur J Obstet Gynecol Reprod Biol. 2009;144(1):8-14. doi:10.1016/j.ejogrb.2009.01.011
[24]  Gouin K, Kingdom J, Keating S. A Giant Cord Hemangioma Diagnosed at 32 Weeks’ Gestation. J Obstet Gynaecol Can. 2009;31(8):695. doi:10.1016/S1701-2163(16)34268-2
[25]   Sathiyathasan S, Jeyanthan K, Hamid R. Umbilical hemangioma: a case report. Arch Gynecol Obstet. 2011;283(1):15-17. doi:10.1007/s00404-010-1606-y
[26]  Matsuda S, Sato Y, Marutsuka K, et al. Hemangioma of the Umbilical Cord with Pseudocyst. Fetal Pediatr Pathol. 2011;30(1):16-21. doi:10.3109/15513811003796920
[27]  Hara K, Fukumura Y, Saito T, et al. A giant cord hemangioma with extramedullary hematopoiesis and elevated maternal serum human chorionic gonadotropin: a case report and review of the literature. Diagn Pathol. 2015;10(1):154. doi:10.1186/s13000-015-0385-y
[28]  Smulian JC, Sarno AP, Rochon ML, et al. The natural history of an umbilical cord hemangioma. J Clin Ultrasound. 2016;44(7):455-458. doi:10.1002/jcu.22346
[29]  Rocha A, Rodrigues M do C, Braga J. Umbilical Cord Hemangioma with Pseudocyst: An Exceptional Finding. Acta Médica Port. 2017;30(9):662-662. doi:10.20344/amp.9274
[30]  Schwickert A, Seeger KH, Rancourt RC, et al. Prenatally detected umbilical cord tumor as a sign of diffuse neonatal hemangiomatosis. J Clin Ultrasound JCU. 2019;47(6):366-368. doi:10.1002/jcu.22689
[31]  Angelico G, Spadola S, Ieni A, et al. Hemangioma of the umbilical cord with associated amnionic inclusion cyst: two uncommon entities occurring simultaneously. Pathologica. 2019;111(2):86. doi:10.32074/1591-951X-26-17-EC
[32]  Yu D, Sun L, Chen T. Prenatal ultrasound diagnosis of Klippel-Trenaunay-Weber syndrome associated with umbilical cord hemangioma. J Clin Ultrasound. 2021;49(3):254-256. doi:10.1002/jcu.22896
[33]  Zhang L, Delisle MF, Hendson G, et al. Umbilical cord hemangioma with significant cord edema. J Obstet Gynaecol Can. 2021 Jul 22;S1701-2163(21)00579-X.
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