What are the other Names for this Condition? (Also known as/Symptoms)

• Teratoma of Umbilical Cord

What is Umbilical Cord Teratoma? (Definition/Background Information)

• Tumors of the umbilical cord are generally rare and an Umbilical Cord Teratoma is an extremely rare tumor occurrence
• Teratoma is a tumor that arises from the germ cells at various body sites. It can originate from the ectoderm, mesoderm, and endoderm, which are 3 types of tissues of a developing embryo. Teratomas may be benign or malignant (cancerous)
• Presently, the risk factors for the development of the tumor are not well-established. The cause of formation of Umbilical Cord Teratoma is also unknown
• The teratoma is hypothesised to arise from ectopic germ cells which have migrated to the umbilical cord. They may contain both mature (benign)and/or immature (malignant) tissue
• Some of the reported cases of Umbilical Cord Teratoma have displayed prominence of associated congenital malformations such as omphalocele, congenital heart defects, and malformations involving the genitourinary, gastrointestinal, and central nervous systems
• The signs and symptoms of Umbilical Cord Teratomas depend on the size and nature of the tumor (whether it is benign or malignant). Some tumors may grow to large sizes and cause significant symptoms. They may also cause additional complications for both the mother and the developing baby
• The treatment of Umbilical Cord Teratoma is based on the presenting symptoms and complications. It may involve delivery via a C-section. The prognosis depends upon various factors and can be only assessed on a case-by-case basis. The prognosis is also dictated by the presence of any associated congenital abnormalities

Who gets Umbilical Cord Teratoma? (Age and Sex Distribution)

• There are about less than 20 cases of Umbilical Cord Teratomas reported worldwide making it a highly infrequent occurrence
• Both males and females are affected with a female predominance being noted among the reported cases
• Presently, no specific racial or ethnic group predilection is noted

What are the Risk Factors for Umbilical Cord Teratoma? (Predisposing Factors)

• Currently, no definite risk factors have been clearly identified for Umbilical Cord Teratoma
• The tumors are reportedly associated with omphalocele (most common association), followed by a few cases observed in the background of congenital heart defects, genitourinary malformations, gastrointestinal malformations, and central nervous system malformations

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Umbilical Cord Teratoma? (Etiology)

The cause of development of an Umbilical Cord Teratoma is unknown. It is believed that abnormal differentiation of germ cells gives rise to cancerous cells that lead to the formation of this tumor.

• In general, it is known that cancers form when normal, healthy cells begin transforming into abnormal cells - these cancer cells grow and divide uncontrollably (and lose their ability to die), resulting in the formation of a mass or a tumor.
• The transformation of normally healthy cells into cancerous cells may be the result of genetic mutations. Mutations allow the cancer cells to grow and multiply uncontrollably to form new cancer cells

What are the Signs and Symptoms of Umbilical Cord Teratoma?

The signs and symptoms of Umbilical Cord Teratoma may include the following:

• Presence of a visible tumor mass in the affected region of the umbilical cord
• Teratomas are generally benign, while few of them may be malignant leading to severe symptoms
• Umbilical Cord Teratomas may affect the growth and development of the fetus, in some cases
• Large tumors can compress the umbilical vessels and lead to fetal distress and other complications

How is Umbilical Cord Teratoma Diagnosed?

It is reportedly difficult to diagnose Umbilical Cord Teratomas prenatally on imaging scans; a tumor biopsy may be necessary to ensure an accurate diagnosis. Also, the presence of a fetal teratoma should prompt the healthcare provider to additionally assess for associated abnormalities or malformations.

There are a variety of tests the healthcare provider may employ in diagnosing Umbilical Cord Teratoma that may include:

• Physical examination and complete medical history screening
• Assessment of the symptoms observed
• Blood tests for various markers
• Amniocentesis to check for any fetal congenital abnormalities
• Prenatal and postnatal diagnostic tests including:
  • Ultrasound scan of the abdomen
  • Fetal MR scan

Tissue biopsy from the tumor, which can be performed either prenatally or postnatally during exam of the placenta and umbilical cord:

• A biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
• Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
• Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing and very rarely, electron microscopic studies, to assist in the diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Umbilical Cord Teratoma?

The complications of Umbilical Cord Teratoma may include:

• Fetal growth and development can be severely affected
• In the womb, the following complications may take place:
  • Fetal hydrops
  • Cardiac failure

• There may be rupture of the fetal cord and heavy bleeding, when the teratoma is associated with the umbilical vessels or omphalocele
• Severe emotional stress for the mother/parents
• The presence of associated fetal abnormalities may give rise to additional, and often, severe complications

• Complications may arise from the methods used in treating the tumor

How is Umbilical Cord Teratoma Treated?

The treatment of Umbilical Cord Teratoma may involve the following:

• The tumor is treated on a case-by-case basis and may involve:
  • Elective C-section may be performed in some cases, when the tumor does not affect the fetus
  • In case the tumor affects fetal growth and development, an emergency C-section may be considered

• Mature (benign) teratomas generally do not require any additional treatment, while immature teratoma management depends on the grade of the tumor (the percentage of immature elements)

• Follow-up care with regular screening and check-ups are important and encouraged

How can Umbilical Cord Teratoma be Prevented?

• Currently, there are no known methods to prevent the development of Umbilical Cord Teratoma
• A careful and periodic monitoring of the pregnancy is advised and recommended

What is the Prognosis of Umbilical Cord Teratoma? (Outcomes/Resolutions)

• The prognosis of Umbilical Cord Teratoma is mainly determined by the presence of any associated anomalies and the hemodynamic changes in the fetus
• In general, the prognosis of a teratoma also depends upon whether it is of a mature or immature type (and, whether the tumor is benign or malignant)
• The outcomes are also dependent upon the size of the teratoma; smaller-sized tumors usually present better outcomes than larger-sized tumors

Additional and Relevant Useful Information for Umbilical Cord Teratoma:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/diseases-conditions/pregnancy-related-disorders/