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

• Germ Cell Tumor of Coccyx
• Sacrococcygeal Germ Cell Tumor
• Teratoma of Tailbone

What is Sacrococcygeal Teratoma? (Definition/Background Information)

• Sacrococcygeal Teratoma (SCT) is a common form of teratoma that arises from the tailbone or coccyx region. It is typically diagnosed prenatally (before birth), or in some cases, shortly thereafter. It is amongst the most common tumor diagnosed in a newborn infant
• 3 in 4 Sacrococcygeal Teratomas are benign; 12-15% tumors are malignant, while 12% have atypical features and can be both. Nearly 80% of the tumors are diagnosed in girl babies. Very rarely, the condition has been reported in adults too
• 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. It is reported that almost 40% of the teratomas arise in the sacrococcygeal region
• The cause of formation of Sacrococcygeal Teratoma is unknown; but, they are known to contain both mature and/or immature tissue. Presently, the risk factors for the development of the tumor are not well-established
• The signs and symptoms of Sacrococcygeal Teratomas depend on the size and nature of the tumor (whether benign or malignant). Some tumors may grow to large sizes and cause obstructive signs and symptoms. Also, malignant tumors may metastasize to distant organs. Fetal teratoma may cause additional complications for both the mother and baby
• The treatment of Sacrococcygeal Teratoma involves surgery in most cases. Malignant teratomas may be additionally treated using chemotherapy and/or radiation therapy. In many expectant mothers, an elective C-section may be considered based on the healthcare provider’s assessment
• The prognosis of mature and benign tumors is typically good with early appropriate treatment. However, the prognosis of immature and malignant teratoma depends upon numerous factors and can be only assessed on a case-by-case basis

Who gets Sacrococcygeal Teratoma? (Age and Sex Distribution)

• Sacrococcygeal Teratoma is mostly observed during the fetal stage (fetal teratoma) or right after birth of the baby (congenital teratoma). The incidence of the tumor is approximately 1 in 35,000 to 40,000 births
• Adults are also known to be affected, though it is a highly-infrequent observance
• Both males and females are affected with a female predominance (female-male ratio is between 3:1 to 4:1)
• No specific racial or ethnic group predilection is noted

What are the Risk Factors for Sacrococcygeal Teratoma? (Predisposing Factors)

• Currently, no definite risk factors have been clearly identified for Sacrococcygeal Teratoma
• Studies indicate that the tumors may be occasionally associated with a positive family history of other congenital pelvic deformities

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 Sacrococcygeal Teratoma? (Etiology)

The cause of development of Sacrococcygeal Teratoma is generally 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
• These tumors can invade nearby tissues and adjoining body organs, and even metastasize and spread to other regions of the body

In some cases, Sacrococcygeal Teratomas can be associated with familial conditions such as Currano syndrome.

What are the Signs and Symptoms of Sacrococcygeal Teratoma?

The signs and symptoms of Sacrococcygeal Teratoma may include the following:

• Presence of a visible tumor mass in the affected region (pelvis, coccyx, or tailbone region)
• 75% of the teratomas are generally benign, while about 15% of the teratomas may be malignant and locally invasive
• When in adults, majority of the teratomas are benign
• Fetal teratomas may affect the growth and development of the fetus, in some cases
• The tumors may range in size from a few cm to over 10 cm
• Large tumors can compress the surrounding structures or organs
• Asymmetrical back portion including the buttocks
• Pelvic discomfort and pain; lower back pain
• Frequent urination due to compression/pressure of the tumor
• When metastasis is noted, it may result in weight loss, fatigue, and other systemic symptoms

It is important to note that in some cases, no visible sacrococcygeal tumor mass may be noted.

How is Sacrococcygeal Teratoma Diagnosed?

There are a variety of tests the healthcare provider may employ to diagnose Sacrococcygeal Teratoma, which may include:

• Physical examination and complete medical history screening
• Assessment of the symptoms observed
• Blood tests for various markers
• Prenatal and postnatal diagnostic tests including:
  • Ultrasound scan of the abdomen
  • Fetal MR scan
  • CT scan with intravenous contrast to study the extent of bone/spine involvement by the teratoma

Tissue biopsy from the affected region:

• 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, flow cytometric analysis and very rarely, electron microscopic studies, to assist in the diagnosis

A differential diagnosis to eliminate other tumor types may be necessary prior to establishing a definite diagnosis. The following conditions and tumors may be excluded:

• Dermoid tumor
• Chordoma
• Rhabdomyosarcoma, exophytic type
• Birth defects involving the spine including sacral meningocele and meningomyelocele

Only around 10% of the tumors are diagnosed after age 2 years; nearly 80% of the tumors are diagnosed within the first 6 months of life (60-70% are diagnosed during the fetal stage itself).

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 Sacrococcygeal Teratoma?

The complications of Sacrococcygeal Teratoma may depend on the size of the tumor and whether it is benign or malignant. The complications may include:

• Fetal growth and development can be severely affected; spinal cord injury may take place leading to long-term neurological damage
• In the womb, the following complications may take place:
  • Hydrops leading to heart failure
  • Enlarged liver (hepatomegaly)
  • Enlarged placenta (placentomegaly)
  • Excess fluid in the amniotic sac (polyhydramnios)
• Large tumors pressing on the urinary bladder can cause bladder dysfunction and urinary retention; in rectum, it can lead to fecal incontinence
• Some teratomas can rupture and cause associated complications
• Metastasis of the malignant teratoma to distant sites; malignant teratoma can infiltrate into the bladder and rectum
• Recurrence of the tumor following its incomplete surgical removal
• Severe emotional stress for the mother/parents
• Complications related to the methods used in treating the condition

How is Sacrococcygeal Teratoma Treated?

The treatment of Sacrococcygeal Teratoma may involve the following:

• Fetal teratoma 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
  • Radiofrequency ablation (RFA) may be considered following birth of the child
  • Surgical removal of the entire teratoma tumor and the coccyx can result in a cure, if the tumor is benign. This can lower the risk for tumor recurrence
• Generally, the treatment of choice is complete surgical excision for all teratomas (mature or immature)
• Additionally, chemotherapy and/or radiation therapy may be necessary for malignant teratomas
• Follow-up care with regular screening and check-ups are important and encouraged

How can Sacrococcygeal Teratoma be Prevented?

Currently, there are no known methods to prevent the development of Sacrococcygeal Teratoma.

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

The prognosis of Sacrococcygeal Teratoma depends upon several factors including the age of the child at diagnosis and whether the tumor is benign or malignant.

• The prognosis is typically good in case of mature and cystic tumors that are benign, following surgery
• Immature teratomas may be aggressive, difficult to treat (complete tumor resection may not be possible), and can spread to other sites. The prognosis of these tumors is usually adjudged on a case-by-case basis
• Tumors that are malignant, diagnosed after the child reaches 12 months of age, and is located internally (no visible mass noted), generally have poor prognoses
• Complications involving the child in the womb, such as hydrops fetalis, tumor hemorrhage, and cardiac failure, can result in fetal mortality

Additional and Relevant Useful Information for Sacrococcygeal Teratoma:

The following article link will help you understand other cancers and benign tumors:

http://www.dovemed.com/diseases-conditions/cancer/