A. Gurrera

A. leni

A. Santoro

D. Arciuolo

F. Inzani

G. Angelico

G. F. Zannoni

M. G. Arena

M. Valente

S. G. Vitale

S. Spadola

Undetermined

Umbilical cord hemangioma is an uncommon benign vascular neoplasm arising from the free segment of the umbilical cord, distinct from placental
and fetal insertion, and is thought to originate from endothelial cells of the umbilical vessels. Cystic changes in the umbilical cord rarely
occur as a consequence of the damage to the amnionic surface of the cord caused by the presence of the hemangioma. Until now, a total of 8
cases of umbilical cord hemangioma associated with cystic changes in the umbilical cord have been reported in the literature, however, among
these cases, only one showed an associated cyst derived from inclusion of the amniotic epithelium, and the remaining seven cases consisted
of hemangiomas with associated pseudocyst of the umbilical cord. We herein report a case of umbilical cord hemangioma with an associated
amnionic epithelial inclusion cyst. Clinicopathological features and differential diagnostic considerations are also discussed.Umbilical cord hemangioma is an uncommon benign vascular neoplasm arising from the free segment of the umbilical cord, distinct from placental
and fetal insertion, and is thought to originate from endothelial cells of the umbilical vessels. Cystic changes in the umbilical cord rarely
occur as a consequence of the damage to the amnionic surface of the cord caused by the presence of the hemangioma. Until now, a total of 8
cases of umbilical cord hemangioma associated with cystic changes in the umbilical cord have been reported in the literature, however, among
these cases, only one showed an associated cyst derived from inclusion of the amniotic epithelium, and the remaining seven cases consisted
of hemangiomas with associated pseudocyst of the umbilical cord. We herein report a case of umbilical cord hemangioma with an associated
amnionic epithelial inclusion cyst. Clinicopathological features and differential diagnostic considerations are also discussed

Valente, M

Inzani, F

Zannoni, G F (ORCID:0000-0003-1809-129X)

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Hemangioma of the umbilical cord with associated amnionic inclusion cyst: two uncommon entities occurring simultaneously

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SummaryUmbilical cord hemangioma is an uncommon benign vascular neoplasm arising from the free segment of the umbilical cord, distinct from pla-cental and fetal insertion, and is thought to originate from endothelial cells of the umbilical vessels. Cystic changes in the umbilical cord rarely occur as a consequence of the damage to the amnionic surface of the cord caused by the presence of the hemangioma. Until now, a total of 8 cases of umbilical cord hemangioma associated with cystic changes in the umbilical cord have been reported in the literature, however, among these cases, only one showed an associated cyst derived from inclusion of the amniotic epithelium, and the remaining seven cases consisted of hemangiomas with associated pseudocyst of the umbilical cord. We herein report a case of umbilical cord hemangioma with an associated amnionic epithelial inclusion cyst. Clinicopathological features and differential diagnostic considerations are also discussed.Key wordsHemangioma • Plancenta • Umbilical cord • Cyst • Amnionic epitheliumHow to cite this article: Angelico G, Spadola S, Ieni A, et al. Hemangioma of the umbilical cord with associated amnionic inclusion cyst: two uncom-mon entities occurring simultaneously. Pathologica 2019;111:13-17. https://doi.org/10.32074/1591-951X-26-17Correspondence: Giuseppe Angelico, Department of Gynecologic Pathology, Fondazione Policlinico Universitario A. Gemelli, IRCCS, largo Agostino Gemelli 1, 00168, Rome, Italy - Tel. +39 095 378 2022 - Fax +39 095 378 2023 - E-mail: giuangel86@hotmail.itOriginal ArticleHemangioma of the umbilical cord with associated amnionic inclusion cyst: two uncommon entities occurring simultaneouslyG. Angelico1, S. Spadola1, A. Ieni3, A. Gurrera4, M.G. Arena5, S.G. Vitale6, D. Arciuolo1, M. Valente1, A. Santoro1, F. Inzani1, G.F. Zannoni1,21 Department of Gynecologic Pathology, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Rome, Italy; 2 Department of Gynecologic Pathology, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy; 3 Department of Human Pathology in Adult and Developmental Age “Gaetano Barresi”, Unit of Pathological Anatomy, University of Messina, AOU Policlinico G. Martino, Messina, Italy; 4 Division of Pathology, Vittorio Emanuele, University Hospital “Policlinico-Vittorio Emanuele”, University of Catania, Italy; 5 Department of Obstetrics & Gynecology, University of Catania, Italy; 6 Unit of Gynecology and Obstetrics, Department of Human Pathology in Adulthood and Childhood “G. Barresi”, University of Messina, Italy OPEN ACCESS© Copyright by Società Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of PathologyPATHOLOGICA 2019;111:13-17; doi: 10.32074/1591-951X-26-17 IntroductionHemangiomas are benign mesenchymal neoplasms composed of blood vessels and are the most common tumors during infancy and childhood. Most hemangi-omas are superficial lesions that have a predilection for the skin and soft tissues, especially in the head and neck region, but they may affect almost any or-gan 1. On histological examination, hemangiomas are usually composed of a proliferation of capillary-sized vessels lined by a single layer of flattened endothe-lial cells. A characteristic histological finding in most hemangiomas is the presence of neoplastic vascular channels arranged around larger, dilated “feeder” ves-sels. Several cases of placental hemangioma have been reported in the literature, however the umbilical cord is an extremely rare site of occurrence 2. Umbil-ical cord hemangioma is regarded as a vascular pro-liferation placed on the free segment of the umbilical cord, distinct from placental and fetal insertion and is thought to originate from endothelial cells of the um-bilical vessels 3. On gross examination, the tumor pre-sents as a fusiform swelling in the cord surrounded by edema of the Wharton jelly. Umbilical cord cysts are usually classified as true cysts, which are epithe-lial-lined cysts derived from embryological remnants of the allantois or omphalomesenteric duct and pseu-docysts, which arise from degeneration of Wharton’s jelly surrounding the cord vessels 4. Until now, a total of 8 cases of umbilical cord hemangioma associated with cystic changes in the umbilical cord have been reported in the literature, and only one case showed G. Angelico et al.14an associated cyst derived from inclusion of the am-niotic epithelium 5-13. We herein report a singular case of umbilical cord hemangioma with an associated amnionic epithelial inclusion cyst. Clinicopathological features and differential diagnostic considerations are also discussed.Materials and methodsThe placenta and umbilical cord samples were sub-mitted for histological examination in neutral-buffered 10% formalin. They were dehydrated according to the standard techniques, embedded in paraffin, cut to 5-mm, and stained with hematoxylin and eosin (H&E). Immunohistochemical analysis was performed with the labeled streptavidin–biotin peroxidase detection system using the Ventana automated immunostainer (Ventana Medical systems, Tucson, AZ). The following antibodies were tested: CD31 (dilution 1:50), CD34 (dilution 1:50), cytokeratins (AE1/AE3 clone; dilution 1:50); all from Dako, Glostrup, Denmark. Endothelial cells of the umbilical vessels and amnion epithelium of the umbilical cord were used as internal controls for pan-cytokeratin and CD31 antibody respectively. Clinical summaryA 28-year old woman came into our observation at 34 weeks of gestational age for pelvic pain. US of the um-bilical cord showed a solid hypoechoic mass meas-uring 7 x 5 cm consistent with an umbilical cord cyst. Cardiotocography and ECG showed no abnormalities. The patient came again to our observation after 2 weeks, at 36° week of pregnancy, for pelvic pain and reduced fetal movement. Cardiotocography showed a basic frequency of 120 bpm with unregular contractile activity. US of the umbilical cord showed an increase in size of the solid hypoechoic mass previously detect-ed (10 x 9 cm). For these reasons, in agreement with the patient, a caesarean section was performed. The alive newborn was transferred to neonatal intensive care unit for low birth weight and respiratory distress, after 7 days the infant was resigned in good general condition.Pathological findings The placenta and its umbilical cord were submit-ted for pathologic examination. The placenta was discoid in shape, weighed 630  g and measured 17 x 15.5 x 5 cm. The chorionic blood vessels were normally distributed over the fetal surface. The umbil-ical cord showed three vessels, measured 40 cm in length and was inserted centrally. Twenty cm from the placental insertion, a large unilocular cystic mass of 10 cm in greatest diameter, filled with clear fluid was detected (Fig.  1). Cut sectioning through the wall of the cyst revealed a brown nodular mass of 6  cm in diameter originating from the umbilical cord. On his-tological examination, the mass consisted of a heter-ogeneous proliferation of multiple dilated thin-walled vascular channels, containing an admixture of capil-lary, cavernous and mixed vessel types. The neoplas-tic vessels were circumpherentially arranged around the umbilical artery and at higher magnification, an evident origin from endothelial cells of the umbilical vessel was noted. In fact, cross-sections of the um-bilical artery, revealed endothelial lined channels transgressing the vessel wall and communicating with the surrounding vascular proliferation (Fig. 2). No ap-parent thrombus formation was seen. The neoplas-tic vessels were composed of a single layer of flat, non-atypical endothelial cells, showing no mitotic ac-tivity. Immunohistochemically, the vascular channels were positive for CD31 and CD34. The accompanying stroma showed marked myxo-edematous changes; no inflammatory infiltration was noted. The associated cyst was lined by a single layer of flattened to cuboidal cells, which showed positive immunostain for pan-cy-tokeratin antibody (clone AE1/AE3) (Fig. 1), consist-ent with an amnionic epithelial inclusion cyst. On the basis of the above-mentioned findings a diagnosis of umbilical cord hemangioma with associated amnionic epithelial inclusion cyst was rendered. Placenta exam-ination showed chorangiosis, but no additional signifi-cant pathologic abnormalities were identified.Discussion The association between umbilical cord hemangi-oma and cystic degeneration of umbilical cord is a rare evenience with only 8 cases reported in the lit-erature so far. In all reported cases except one, the cystic mass of the umbilical cord revealed to be a pseudocyst covered by amnion with no epithelial or endothelial lining 5-12. These lesions were located near the placental side or at the end of the umbilical cord and ranged in size from 8 to 25 cm. Regarding their or-igin, it has been suggested that pseudocystic changes of the umbilical cord might result from liquefaction of the mucopolysaccharide-rich membrane surrounding the cord vessels  12. S.M. Jacques et al. in 2013 re-ported a unique case of hemangioma of the umbilical cord associated with a true cyst covered and lined by HEMANGIOMA OF THE UMBILICAL CORD WITH ASSOCIATED AMNIONIC INCLUSION CYST 15a layer of amnionic epithelium. The author suggest-ed that the cyst formation was a consequence of the damage to the amnionic surface of the cord caused by the presence of the hemangioma 13. Amnionic epi-thelial inclusion cysts are very uncommon lesions that arise from inclusions or infoldings of the amnionic sur-face 13. These are simple cysts lined by a single layer of amnionic epithelium and usually contain clear fluid. The present case showed overlapping macroscopic and histological features with the above-mentioned case. In fact, in proximity of the brown, nodular lesion that we diagnosed as hemangioma, a 10  cm cystic lesion, lined by a single layer of flattened to cuboidal cells identical to that seen on the outer surface of the cord was observed. On the basis of the clinical and histological findings, a diagnosis of hemangioma of the umbilical cord with amnionic epithelial inclusion cyst was made. To the best of our knowledge this is the second case in the literature which reports the un-usual association between umbilical cord hemangio-ma and amnionic epithelial inclusion cyst. Differently from the case reported by Jacques et al. where these lesion lead to intrauterine fetal demise, in our case the child was born alive showing low birth weight and respiratory distress due to obstruction of blood flow from the umbilical cord. In conclusion we provide a de-tailed clinical morphological and immunohistochemi-cal characterization of two extremely uncommon condition that presented simultaneously in the same patient: umbilical cord hemangioma and amnionic epi-thelial inclusion cyst. Further studies on larger cohorts are needed in order to establish their clinical signif-icance as well as their etiopathogenetic correlation.Fig. 1. A) Macroscopic appearance the placenta and umbilical cord. A large cystic mass of 10 cm in greatest diameter can be appreciated in the umbilical cord close to the placental insertion. B) On histological examination, the cystic mass consisted of a single layer of eosinophilic cells. Myxoid degeneration of the cystic wall can also be appreciated. C) Higher magnification of the cyst reveals a single layer of amnionic epithelial cells with flattened or cuboidal morphology (arrow). The epithelial origin of the cyst is confirmed by positive immunostain for pan-cytokeratin antibody (clone AE1/AE3) (inset; D). A BC DG. Angelico et al.16ConfliCt of interest statementNone declared.References1 George A, Mani V, Noufal A. Update on the classification of hem-angioma. J Oral Maxillofac Pathol 2014;18:117-20. 2 Ghidini A, Romero R, Eisen RN, et al. Umbilical cord heman-gioma. Prenatal identification and review of the literature. J Ul-trasound Med 1990;9:297-300.3 Heifetz SA, Rueda-Pedraza ME. Hemangiomas of the umbilical cord. Pediatr Pathol 1983;1:385-98.4 Nguyen M, Addicott B, Chu J, et al. Congenital cyst of the umbili-cal cord. Fetal Pediatr Pathol 2016;5:1-4.5 Yavner DL, Redline RW. Angiomyxoma of the umbilical cord with massive cystic degeneration of Wharton’s jelly. Arch Pathol Lab Med 1989;113:935-7.6 Jauniaux E, Moscoso G, Chitty L, et al. An angiomyxoma involv-ing the whole length of the umbilical cord. Prenatal diagnosis by ultrasonography. J Ultrasound Med 1990;9:419-22.7 Wilson RD, Magee JF, Sorensen PH, et al. In utero decompres-sion of umbilical cord angiomyxoma followed by vaginal deliv-ery. Am J Obstet Gynecol 1994;171:1383-5.8 Søndergaard G. Hemangioma of the umbilical cord. Acta Obstet Gynecol Scand 1994;73:434-6.Fig. 2. A) Photomicrograph of the hemagioma showing multiple round or elongated, vascular channel surrounding the um-bilical artery (UA) (Stain: Haematoxylin and Eosin). B) The neoplastic vessels seem to originate and radiate from the umbilical artery (arrow). C) The neoplastic vascular channels are embedded in a dense myxoid stroma and show diffuse immunohis-tochemical staining for CD31 antibody (D).A BC DHEMANGIOMA OF THE UMBILICAL CORD WITH ASSOCIATED AMNIONIC INCLUSION CYST 179 Tennstedt C, Chaoui R, Bollmann R, et al. Angiomyxoma of the umbilical cord in one twin with cystic degeneration of Wharton’s jelly. A case report. Pathol Res Pract 1998;194:55-8.10 Iyoob SD, Tsai A, Ruchelli ED, et al. Large umbilical cord hem-angioma: sonographic features with surgical pathologic correla-tion. J Ultrasound Med 2006;25:1495-8.11 Daniel-Spiegel E, Weiner E, Gimburg G, et al. The association of umbilical cord hemangioma with fetal vascular birthmarks. Pre-nat Diagn 2005;25:300-3.12 Matsuda S, Sato Y, Marutsuka K, et al. Hemangioma of the um-bilical cord with pseudocyst. Fetal Pediatr Pathol 2011;30:16-21.13 Jacques SM, Qureshi F. Hemangioma of the umbilical cord with amnionic epithelial inclusion cyst. Fetal Pediatr Pathol 2013; 32:235-9.Received: October 17, 2017 - Accepted: February 7, 2019

hemangioma of the umbilical cord with associated amnionic inclusion cyst two uncommon entities occurring simultaneously

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hemangioma of the umbilical cord with associated amnionic inclusion cyst two uncommon entities occurring simultaneously

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