Natalucci, Giancarlo

Stallmach, Thomas

Ulrich Bucher, Hans

Weil, Robert

Wisser, Josef

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YOUR DIAGNOSISYour diagnosis? Umbilical cord tumorGiancarlo Natalucci & Josef Wisser & Robert Weil &Thomas Stallmach & Hans Ulrich BucherReceived: 23 May 2006 /Accepted: 29 August 2006 / Published online: 17 November 2006# Springer-Verlag 2006Clinical informationA 35-year-old woman, gravida 3, para 3, at 33 1/7 weeks ofgestation was referred to the obstetric clinic for prenatalassessment of an echogenic mass of the umbilical corddetected during a routine ultrasound examination. Chori-onic villus biopsy for advanced maternal age at 13-weekgestation revealed a normal 46 XX karyotype. Sonographicassessment of the fetus demonstrated growth parametersappropriate for the gestational age. The abdominal wall wasclosed, with an inhomogeneous mass measuring 43×32×28 mm near the insertion of the umbilical cord (Fig. 1). Thecardiotocogram showed no fetal distress. Doppler flowstudies of the umbilical cord showed three vessels,displaced by a multilobulated echogenic tumor. Maternalserum screening showed an elevated level of alpha-fetoprotein (326 μg/l), but no signs of congenital infection.At 38 5/7 weeks of gestation, a girl was deliveredvaginally, weighing 2,840 g (10th–25th centile), with alength of 47 cm (10th–25th centile) and head circumfer-ence of 33 cm (10th–25th centile). She adapted with Apgarscores of 8/9/9, and the arterial cord pH was 7.31. Theumbilical cord showed a nodular bulge proximal to itsinsertion into the abdominal wall. It measured 4 cm indiameter, had a smooth, translucent, pearly colored surface,without superficial vessels or pulsations, and was of firm,elastic consistency. A blue core was discernible within(Fig. 2). The findings were associated with markedthickening and edema of the Wharton’s jelly, and one veinand two arteries were present within the cord. A 1-cmsegment of normal umbilical cord separated the tumor fromthe intact abdominal wall. Abdominal ultrasound examina-tion showed no visceral abnormalities. The umbilical cordwas clamped distal to the lesion, and the infant recovered ina puerperal care unit. The macro- and microscopic placentaexamination (387 g, 19×15×3 cm) revealed no abnormal-ities. At 2 days of age, the infant was transferred to apediatric surgery unit for surgical revision.Eur J Pediatr (2007) 166:753–756DOI 10.1007/s00431-006-0301-2G. Natalucci (*) :H. Ulrich BucherClinic of Neonatology, University Hospital,Frauenklinikstrasse 10,8091 Zürich, Switzerlande-mail: Giancarlo.Natalucci@kispi.unizh.chJ. WisserDepartment of Obstetrics and Gynaecology, University Hospital,Zurich, SwitzerlandR. WeilClinic of Surgery, Children_s Hospital,Zurich, SwitzerlandT. StallmachInstitute of Pathology, University Hospital,Zurich, SwitzerlandWhat is your diagnosis?Diagnosis: umbilical cord hemangiomaSurgical revision was undertaken for the suspected diagno-sis of a hernia into the cord. However, no defect of theabdominal wall was found. Surgical section of the umbilicalcord was performed with an umbilicoplasty. Postoperativerecovery was uneventful. Concerning the umbilical cordpathology, the maximum diameter of the surgicallyremoved 8.5-cm long umbilical cord segment was 3.6 cm.Macroscopically, the cross section revealed three vessels.Only on microscopic examination did it become clear thatthe Wharton’s jelly was mostly replaced by abundantaggregates of thin-walled capillaries. The capillary heman-gioma showed a diffuse growth around the vessels, withfocal dissection of the muscular coat of the umbilical vein.In addition, nodular aggregates were seen, some freshlythrombosed (Fig. 3). Distal to the removed lesion, theremaining umbilical cord was unremarkable.Fig. 3 Cross-section of umbilical cord showing an umbilical artery(a). Note the abundance of tiny capillaries around the artery (thickarrow). Some capillary growth is nodular (thin arrow) with occasionalfresh thromboses (dotted arrow). Most of the Wharton’s jelly isreplaced by capillaries. Hematoxylin-eosin, magnification ×100Fig. 2 Appearance of the umbilical cord shortly after birth. Note thenodular bulging and the distal edemaFig. 1 Fetal abdominal wallwith umbilicus at 33+1 weeksof gestation. The arrow marksthe umbilical vein passingthrough the abdominal wall. Thedimensions of the umbilical cordtumor are shown754 Eur J Pediatr (2007) 166:753–756DiscussionDifferenial diagnosis of an umbilical cord tumorNodular bulges of the umbilical cord are rare entities ofpolymorphous presentation that can be detected prenatallyby ultrasound examination. Their differential diagnosis andsome of the respective characteristics are listed in Table 1, amore detailed review of which is beyond the this report’sscope. The clinical significance common to all of theseanomalies is determined by their size, which can potentiallycause vascular compromise and affect fetal growth. Afterbirth, umbilical cord clamping should be distal to the lesionto avoid intestinal strangulation. An ultrasound investiga-tion of the lesion is recommended, and referral of thenewborn to a pediatric surgery clinic for revision andcorrection is mandatory, but not an emergency.Umbilical cord teratomaUmbilical cord teratoma is a polymorphous, congenitalnodular-cystic neoplasm having different cellular andorganoid components deriving from more than one germlayer. Its texture varies from soft adipous areas to firm/solidcartilaginous or bony structures characterized by distinctcalcification. Teratomas are observed all along the umbil-ical cord, the origin of which is of normal aspect. Usuallyconsidered benign, about one half of all reported cases areassociated with other malformations [10].Hemangioma of the umbilical cordHemangioma of the umbilical cord is characterized by well-defined aggregates of closely packed, thin-walled capillaryproliferation originating from the umbilical arteries, theumbilical vein or vitelline capillaries [7]. The etiology isstill not clear; it may represent a true neoplasm or it maybe a developmental abnormality (hamartoma). Eventhough it lacks circumscription or encapsulation, it nevermetastasizes [3], although it has been reported to beassociated with additional skin, liver, intra-abdominal andplacental hemangiomas. Most often hemangioma is anisolated anomaly, but large lesions have been described inassociation with polyhydramnios [2], intrauterine growthretardation, elevated maternal serum alpha-fetoprotein [9]and fetal malformations [2]. Some authors have alsoreported its association with premature delivery and evenwith fetal death caused by impaired umbilical circulationresulting in nonimmune hydrops fetalis [4], torsion,compression, or stenosis of the umbilical vessels, fetalhemorrhage [7], thrombosis of an umbilical vessel andhematoma of the umbilical cord [11]. We reviewed 37reports in the literature from 1951 through 2005, in mostof which the lesion was referred to as hemangioma, andrarely as angiomyxoma, myangioma or hemangiofibro-myxoma because of the myxoid appearance of thecommonly associated edematous degeneration of theWharton’s jelly. A possible hereditary predisposition tothis vascular anomaly is still under discussion.Hernia into the cordHernia into the cord is a rare, peculiar form of abdominalwall defect differing from the omphalocele by its smallersize and its structure, consisting in a protruding peritonealsegment in the umbilical cord containing no intestinal loop.It develops proximally to the fetal abdominal wall and,typically, the origin of the umbilical cord is of normal size(diameter about 1 cm) and aspect. In the majority of cases itis an isolated finding.Table 1 Differential diagnosis of an umbilical cord tumorDiagnosis Macroscopic aspect Association with other findings1) Umbilical cordteratomaNodular or cystic; maximum 10 cm Ø; normal umbilicalcord origin; all along the umbilical cord• 50% associated with other malformations2) Hemangioma of theumbilical cordNodular 0.2 to 18 cm Ø; normal umbilical cord origin;proximal to the placenta•Mostly isolated finding; • edema of Wharton’s jelly;• complication: intrauterine bleeding3.1) Hernia into the cord Nodular <4 cm Ø; normal umbilical cord origin;proximal to the abdomen; elastic consistence• Mostly isolated finding; • associated with Meckel‘sdiverticulum3.2) Omphalocele Abdominal organ prolapse >4 cm Ø; large umbilical cordorigin• Associated with other malformations, syndromes orchromosomal abnormalities4) Umbilical cord cyst Nodular or cystic; most often single; normal umbilicalcord origin; all along the umbilical cord• Mostly isolated finding5) Umbilical cord polyp Nodular, solid, reddish, exudative, sometimes ulceratedand bleeding• Meckel’s diverticulum and umbilical enteric fistula6) Umbilical cordhematoma1 andaneurysm21Nodular, livid; 2cystic in US, turbulent, non-pulsatileblood flow• 1Fetal distress; • 2trisomy 18, single umbilicalartery, arteriovenous fistulaEur J Pediatr (2007) 166:753–756 755OmphaloceleOmphalocele is the most frequent pathologic entity of thosecited above, with an estimated incidence of 1 in 3,000 to4,000 births. It is a form of abdominal wall defect that is, bydefinition, between 4 and 12 cm in diameter. It consists of aprotrusion of intestinal loops into the base of the umbilicalcord, occasionally accompanied by other visceral organssuch as the liver, covered by peritoneum without overlyingskin. Characteristically, the origin of the umbilical cord isabnormally large. In the majority of cases, omphalocele isassociated with other congenital anomalies of the gastroin-testinal tract or cardiovascular system, syndromes orchromosomal aberrations.Cystic lesionsCystic lesions originate from embryonic vestiges remainingas a consequence of the incomplete obliteration of theurachus and omphalomesenteric duct. They are more oftensingle than multiple findings and are usually isolated, eventhough their association with other congenital anomalies,usually of the gastrointestinal tract, and other syndromesand sequences has been described. The presence of multipleumbilical cord cysts is associated with an increased risk ofmiscarriage, aneuploidy and congenital anomalies [5].Additionally, pseudocysts have also been described, origi-nating as a result of myxoid degeneration of the Wharton’sjelly secondary to persistent patent urachus, urachus cystsor abdominal wall defects [1].Umbilical cord polypUmbilical cord polyp results from the persistence ofprotruding intestinal tissue within the umbilical cord,remnants of the omphalomesenteric duct, which differ-entiates into proliferating gastrointestinal cells. Communi-cation with the intestinal canal is not always lost. As anisolated finding, it can be a harmless lesion, but its presenceshould lead to a careful investigation of the possiblepresence of other omphalomesenteric duct anomalies, suchas Meckel’s diverticulum and umbilical enteric fistula. Thelesion can be exudative and complicated by ulceration andbleeding [6]. There is a report in the literature of anumbilical cord polyp originating from the urachus [8].Hematoma and aneurysm of the umbilical cordHematoma of the umbilical cord is usually an accidentalevent secondary to cordocentesis done for diagnosticpurposes, while spontaneous hematoma remains very rare[11, 12]. Umbilical artery aneurysm is an extremely rarevascular anomaly associated with poor fetal outcome,comprehending chromosomal aberration, progressive oligo-hydramnios and fetal death due to acute umbilical venouscompression [13].ConclusionWe report the case of an infant with an umbilical cordtumor that had twice been diagnosed previously: antenatal-ly, by ultrasound, as a teratoma, based on its inhomoge-neous character and the absence of an intratumoral flow,and postnatally as a hernia into the cord. The infant wasborn at term weighing 2,840 g, with a 4-cm diameternodular bulge in the umbilical cord. The definitivediagnosis was made by histopathology as hemangioma ofthe umbilical cord.References1. Antonelli E, Wildhaber BE, Pfister RE (2005) Giant umbilicalcord. In: Case of the Month: January 2005. Swiss Society ofNeonatology. http://www.neonet.ch2. Armes JE, Billson VR (1994) Umbilical cord hemangiomaassociated with polyhydramnios, congenital abnormalities andperinatal death in a twin pregnancy. Pathology 26:218–2203. Caldarella A, Buccoliero AM, Taddei A, Savino L, Taddei GL(2003) Hemangioma of the umbilical cord: report of case. PatholRes Pract 199:51–554. Carles D, Maugey-Laulom B, Roux D, Jimenez M, Saudubray F,Alberti EM (1994) Lethal hydrops fetalis secondary to anumbilical cord hemangioma. Ann Pathol 14:244–2475. Grezzi F, Raio L, Di Naro E, Franci M, Cromi A, Durig P (2003)Single and multiple umbilical cord cysts in early gestation: twodifferent entities. Ultrasound Obstet Gynecol 21:215–2196. Guschmann M, Janda J, Wenzelides K, Vogel M (2002) Intestinalpolyp of the umbilical cord. Zentralbl Gynakol 124:132–1347. Heifetz SA, Rueda-Pedraza ME (1983) Hemangiomas of theumbilical cord. Pediatr Pathol 1:385–3988. Oguzkurt P, Kotiloglu E, Tanyel FC, Hicsonmez A (1996)Umbilical polyp originating from urachal remnants. Turk J Pediatr38:371–3749. Resta RG, Luthy DA, Mahony BS (1988) Umbilical cordhemangioma associated with extremely high alpha-fetoproteinlevels. Obstet Gynecol 72:488–49110. Satgé DCL, Laumond M-A, Desfarges F, Chenard M-P (2001) Anumbilical cord teratoma in a 17-week-old fetus. Prenat Diagn21:284–28811. Schlaeder G, Irrmann M, Philippe E (1964) A case of hemangi-oma of the cord with hematoma. Bull Fed Soc Gynecol ObstetLang Fr 16:208–21012. Seoud M, Aboul-Hosn L, Nassar A, Khalil A, Usta I (2001)Spontaneous umbilical cord hematoma: a rare cause of acute fetaldistress. Am J Perinatol 18:99–10213. Sepulveda W, Corral E, Kottmann C, Illanes S, Vasquez P,Monckeberg MJ (2003) Umbilical artery aneurysm: prenatalidentification in three fetuses with trisomy 18. Ultrasound ObstetGynecol 21:292–296756 Eur J Pediatr (2007) 166:753–756

Your diagnosis? Umbilical cord tumor

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Your diagnosis? Umbilical cord tumor

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