Angiomyxoma of the umbilical cord is a rare tumor that can be detected by Ultrasound prenatally as a complex solid-cystic mass.

Abstract: Angiomyxoma of the umbilical cord is a rare tumor that can be detected by Ultrasound prenatally as a complex solid-cystic mass. Serial ultrasound and Doppler examinations are used to monitor the tumor’s size and the overall fetal well-being. Most umbilical cord Angiomyxomas are incidental findings and the prognosis is  dependent on size of he tumor, associate fetal anomalies and hemodynamic changes in the fetus. There is no guideline for management of these pregnanciesthis such rare condition, however, increase fetal surveillance is recommended. Overall, the mode of delivery depends on the size and location of the tumor.

Keywords: Angiomyxoma, umbilical cord, haemangioma, myxangioma, haemangiofibromyxoma, myxosarcoma, prenatal diagnostic.

Authors: MD Ija Lisovaja1,2, Prof. Natalija Vedmedovska 1,2, MD Ivanda Franckevica2,3

 1.Riga Maternity hospital;

2.Department of Obstetrics and Gynaecology, Riga Stradins university;

3. Department of Pathology, Children's Clinical University Hospital

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Angiomyxoma of umbilical cord

Definition

Angiomyxoma of the umbilical cord is a benign tumour arising from endothelial cells of the umbilical cord vessels2. Also known as haemangioma, myxangioma, haemangiofibromyxoma and myxosarcoma of the umbilical cord, it appears sonographically  as a homogenous solid echogenic mass of variable size, usually located near the placental insertion of the cord, but can be located in any part of the umbilical cord. Separate from, but close to, the haemangioma there may be cystic degeneration of Wharton's jelly1.

ICD code

O43.9 Placental disorder, unspecified

O69.9 Labour and delivery complicated by cord complication, unspecified

Incidence

The incidence is unknown. Because of its rarity, the prenatal diagnosis of angiomyxoma is based on findings described in isolated case reports2. Angiomyxoma is a rare tumor of the umbilical cord and is associated with increased perinatal morbidity and mortality3,4.

Pathogenesis

The formation of the cord is complex and involves several parts of the developing fetus, yolk sac, and mesenchyme. The umbilical cord is formed between the 6th and 8th week of gestation by the approximation of the omphalomesenteric duct/yolk sac and the allantoic duct with the body stalk of the developing embryo4. The umbilical cord is subsequently made up of two umbilical arteries and one umbilical vein derived from the allantoic vessels. The connective tissue or mesenchyme supporting these vascular structures, also called Wharton’s jelly, liquefies under the pressure. The impermeability of the covering amnion may predispose this gel to pseudocyst formation4.

Etiology

Angiomyxoma of the umbilical cord is a benign tumor arising from endothelial cells of the umbilical cord vessels2. It consists of an angiomatous nodule, ranging in size  from 0.2 to 18 cm, generally encompassed by edema and myxomatous degeneration of Wharton’s jelly, which is often cystic4. The edema is due to high permeability of angiomatous vessels that are placed in a soft, gelatinous tissue5.

Pathology

Angiomyxoma is a benign mesenchymal neoplasm. Microscopically it is composed of anastomosing vascular structures, delineated by endothelium. The vascular channels may be widely dilated, and a variable amount of smooth muscle is always present. Fibrin thrombi may be present and associated with endothelial necrosis and degeneration or calcification and acute inflammation. Sometimes, stromal calcification or osseous metaplasia may occur5. Cytologic features suggesting malignancy are absent; the proliferation index Ki67 is very low. Immunohistochemical examination confirms the vascular nature of the lesion with CD31 and CD34  detected in the thin walls of the vessels6.

Associated anomalies

Clinically, the most frequent presentation is that of an isolated umbilical cord anomaly without other fetal malformations. Cases of umbilical cord angiomyxomas seen in association with cutaneous or visceral hemangiomas , i.e. port wine skin lesions, and single umbilical artery have been reported. Some cases of multiple lesions of the newborn was discribed with multiple hemangiomas in the liver, intestines, skin, and brain7.

Impaired umbilical circulation with stenosis of umbilical vein and arteries is considered as the predisposing factor for fetal demise and fetal intrauterine growth restriction8,9.

Sometimes polyhydramnios and hydrops fetalis can be present10. Hydrops fetalis is associated with intrauterine and neonatal death. In one case, there was pulmonary hypoplasia in the newborn due to extensive accumulation of serous fluid in the fetal tissues and the body cavitie11,12.

Umbilical vessels can become stenotic due to the intravascular proliferation of the angiomyxoma9. Fetal haemorrhage due to rupture is rare but lethal condition and has been described in some cases1,4,5. Some rare presentation of reducing in size of angiomyxoma due to spontaneous rupture without bleeding was also reported, that followed uncomplicated vaginal delivery 13.

Recurrence risk

Unknown. There is no data on the recurrence rate.

Diagnosis

Ultrasound and maternal serum alphafetoprotein evaluation in the second trimester can be used to diagnose angiomyxoma of the umbilical cord in early gestation14.

Sonographically, an angiomyxoma appears as homogenous solid echogenic mass of variable size, usually located near the placental insertion of the cord. Separate from but close to the echogenic mass there may be cystic degeneration of Wharton's jelly, and thus the lesion may appear quite heterogeneous. In some cases, colour Doppler ultrasound can support the suspected diagnosis by demonstrating blood vessels with low flow within the echogenic component of the tumor that can be demonstrated with HDlive Flow15,16.

Differential diagnosis

The differential diagnosis must include hematomas and teratomas of the umbilical cord, abdominal wall defects and true umbilical cord cysts17.

Diagnosis

Macroscopic/US aspect

Association with other findings

Umbilical cord teratoma

Nodular or cystic; maximum 10 cm; can be all along the umbilical cord

Associated with other malformations in 50 %, tend to grow rapidly and may cause cardiac failure and fetal hydrops

Hernia into the cord

Nodular or cystic; maximum 10 cm; all along the umbilical cord

Mostly isolated finding; associated with Meckel’s diverticulum

Omphalocele

Abdominal organ prolapse >4 cm; large umbilical cord origin

Associated with other malformations, syndromes or chromosomal abnormalities

Umbilical cord cyst

Nodular or cystic; most often single; normal umbilical cord origin; all along the umbilical cord

Single- usually transient, no adverse effect; multiple- increased risk of miscarriage, trisomies 18 and 13, omphalocele.

Umbilical cord hematoma and aneurysm

Nodular, livid; cystic on US with turbulent, non-pulsatile blood flow on Dopler studies

Associated with fetal distress, trisomy 18, single umbilical artery, arteriovenous fistula, rate of mortality >50%

Implications for sonographic diagnosis and screening

After the diagnosis of an angiomyxoma, it is essential to start proper monitoring with serial assessments for fetal growth, angiomyxoma’s size, well-being and Doppler examinations. Other malformations should be excluded4.

Prognosis

Limited data exists concerning mortality, morbidity, outcome and prognosis of angiomyxoma of the umbilical cord. Less than 50 case have been reported in the literature. Most cord angiomyxomas are incidental findings and when small in size and isolated in nature, are usually of limited clinical significance. Size does matter and vascular compromise due to compression of the umbilical vein leading to cardiac failure has been reported in association with large tumours15. In addition to fetal growth issues, compression of the adjacent umbilical vessels may lead to  fetal hydrops, and torsion around the axis of the cord can occur and lead to fetal death4,15. Fetal death secondary to rupture of this vascular cord lesion has been rarely reported4. Neonates with angiomyxoma of the cord may also demonstrate skin hemangiomas18,19.

Management

Serial ultrasound and Doppler examinations are used to monitor  size of the angiomyxoma, look for evidence of cardiac compromise and evaluate  the overall fetal well-being. Mapping of the fetal vessels through the mass has been advised to assess the potential risk of rupture of the cystic portion of the mass leading to tearing of the umbilical vein, and fetal hemorrhage22. Prognosis is largely determined by associated anomalies of the fetus and the hemodynamic changes in the fetus. There are no stringent guidelines for management of these pregnancies. Serial ultrasound examinations, assessing amniotic fluid volume,  tumour size and Doppler studies at 4 weeks intervals up to 32-34 weeks and every 1-2 weeks thereafter, have been suggested, depending on the findings20. Offering amniocentesis on a routine basis is not warranted from the available data, since umbilical cord angiomyxomas have never been associated with chromosomal abnormalities8. In most cases  of large angiomyxomas Cesarean section has been the reported mode of delivery. Cases of ultrasound-guided in utero decompression following vaginal delivery and cases of spontaneous delivery were described. Concerns regarding intrapartum management without cystic decompression include possible risks of dystocia or sudden cystic rupture affecting blood flow through the cord. Controlled aspiration with ultrasonographic guidance may be used to decrease these potential risk factors3,21. Generally, the mode of delivery depends on the size and location of the tumour.

References

  1. Michael J Weston. Twining's Textbook of Fetal Abnormalities, Chapter 5, 100-121.
  2. Ghidini A, Romero R, Eisen RN, Smith GJ, Hobbins JC. Umbilical cord hemangioma. Prenatal identification and review of the literature. Journal of Ultrasound in Medicine. 1990;9:297-300.
  3. Cheng HP, Hsu CY, Chen CP, Su TH. Angiomyxoma of the umbilical cord. Taiwan J Obstet Gynecol. 2006 Dec;45(4):360-2.
  4. Vougiouklakis T, Mitselou A, Zikopoulos K, Dallas P, Charalabopoulos K. Ruptured hemangioma of the umbilical cord and intrauterine fetal death, with review data. Pathol Res Pract. 2006;202(7):537-40
  5. Caldarella A, Buccoliero AM, Taddei A, Savino L, Taddei GL. Hemangioma of the Umbilical Cord: Report of a Case. Pathology - Research and Practice. 2003; 199 (1):51-55.
  6. Angelico G, Spadola S, Ieni A, Gurrera A, Arena M, Vitale S, Arciuolo D, Valente M, Santoro A, Inzan M., Zannoni G. Hemangioma of the umbilical cord with associated amnionic inclusion cyst: two uncommon entities occurring simultaneously. Pathologica - Journal of the Italian Society of Anatomic Pathology and Diagnostic Cytopathology. 2019 Mar;111(1):13-17.
  7. Schwickert A, Seeger KH, Rancourt RC, Henrich W. Prenatally detected umbilical cord tumor as a sign of diffuse neonatal hemangiomatosis. J Clin Ultrasound. 2019 Jul;47(6):366-368.
  8. Papadopoulos VG, Kourea HP, Adonakis GL, Decavalas GO. A case of umbilical cord hemangioma: Doppler studies and review of the literature. European Journal of Obstetrics & Gynecology and Reproductive Biology. 2009;144(1):8-14.
  9. Kamitomo M, Sueyoshi K, Matsukita S, Matsuda Y, Hatae M, Ikenoue T. Hemangioma of the umbilical cord: stenotic change of the umbilical vessels. Fetal Diagn Ther. 1999 Nov-Dec;14(6):328-31.
  10. Heep J, Weidenkopf KL, Tschahargane C. Kapilläres Hämangiom der Nabelschnur als Ursache eines Polyhydramnions [Capillary hemangioma of the umbilical cord as a cause of polyhydramnios]. Geburtshilfe Frauenheilkd. 1988 Nov;48(11):819-21.
  11. Seifer DB, Ferguson JE, Behrens CM, Zemel S, Stevenson DK, Ross JC. Nonimmune hydrops fetalis in association with hemangioma of the umbilical cord. Obstet Gynecol. 1985 Aug;66(2):283-6.
  12. Carles D, Maugey-Laulom B, Roux D, Jimenez M, Saudubray F, Alberti EM. Anasarque foeto-placentaire létale secondaire à un hémangiome du cordon ombilical [Lethal hydrops fetalis secondary to an umbilical cord hemangioma]. Ann Pathol. 1994;14(4):244-7.
  13. Lisovaja I, Vedmedovska N, Franckevica I. ISUOG Virtual International Symposium 2021.
  14. Resta RG, Luthy DA, Mahony BS. Umbilical cord hemangioma associated with extremely high alpha-fetoprotein levels. Obstet Gynecol. 1988 Sep;72(3 Pt 2):488-91.
  15. Göksever H, Celilo─člu M, Küpelio─člu A. Angiomyxoma: a rare tumor of the umbilical cord. J Turk Ger Gynecol Assoc. 2010;11(1):58-60.
  16. Akiba Y, Miyakoshi K, Ochiai D, Kawaida M, Matsumoto T, Tanaka M. Umbilical cord hemangioma: Sonographic features by HDlive Flow. European Journal of Obstetrics & Gynecology and Reproductive Biology. 2018;221:195-196.
  17. Natalucci G, Wisser J, Weil R, Stallmach T, Bucher HU. Your diagnosis? Umbilical cord tumor. Eur J Pediatr. 2007 Jul;166(7):753-6.
  18. Daniel-Spiegel E, Weiner E, Gimburg G, Shalev E. The association of umbilical cord hemangioma with fetal vascular birthmarks. Prenat Diagn. 2005 Apr;25(4):300-3.
  19. Malliah R, Shah V, Heller DS. Umbilical cord hemangioma associated with multiple cutaneous hemangiomas in a newborn. International Journal of Gynecology & Obstetrics. 2007;99(1):58.
  20. Sathiyathasan S, Jeyanthan K, Hamid R. Umbilical hemangioma: a case report. Arch Gynecol Obstet. 2011 Mar;283 Suppl 1:15-7.
  21. Wilson RD, Magee JF, Sorensen PH, Johnson A. In utero decompression of umbilical cord angiomyxoma followed by vaginal delivery. Am J Obstet Gynecol. 1994 Nov;171(5):1383-5.
  22. Goldooz M, Draper M, Comstock J, Kennedy A, Cord Hemangioma Complicated by Umbilical Vein  Rupture; What We Missed on Color Doppler. Am J of Sonogr, 2018; 1(10):1-3.

Lisovaja I, Vedmedovska N, Franckevica I. Angiomyxoma of umbilical cord, Visual Encyclopedia of Ultrasound in Obstetrics and Gynecology, www.isuog.org, 25.08.21

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