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

• Teratoma of Abdominal Cavity
• Teratoma of Retroperitoneum

What is Retroperitoneal Teratoma? (Definition/Background Information)

• Retroperitoneal Teratoma is an uncommon form of tumor that arises from the germ cells in the abdominal cavity or retroperitoneum. The tumor is mostly observed in infants and children; and sometimes, in young adults
• Most teratoma tumors are either mature or immature. A majority of benign teratomas are mature, while a majority of malignant teratomas are immature
• The cause of formation of Retroperitoneal Teratoma is unknown. Also, presently the risk factors for the same are not well-established
• The signs and symptoms of Retroperitoneal Teratoma depend on the size and nature of the tumor (if benign or malignant). Some tumors may grow to large sizes and cause symptoms such as nausea, vomiting, and abdominal discomfort. Also, malignant tumors may metastasize to distant organs
• The treatment of teratoma involves surgery in most cases. The successful surgical removal of the tumor is an important prognostic indicator. Malignant Retroperitoneal Teratomas may be additionally treated using chemotherapy and/or radiation therapy
• In a majority of cases, the prognosis of benign teratoma is excellent with early appropriate treatment. However, the prognosis of malignant teratoma depends upon several factors and can be only assessed on a case-by-case basis

Who gets Retroperitoneal Teratoma? (Age and Sex Distribution)

• Between 1% to 11% of all primary teratomas are believed to be Retroperitoneal Teratomas
• The tumor is mostly diagnosed in infant children before the age of 6 months, or in young adults. Thus, a bimodal distribution of the tumor with two peak presentation periods is observed
• 50% of these tumors are observed in young children below the age of 10 years; about 20% are observed in young adults around/after 30 years of age
• Both males and females are affected; although, it is more commonly observed in females than males (female-male ratio is 2:1)
• No specific racial or ethnic group predilection is noted

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

• Currently, no definite risk factors have been identified for Retroperitoneal Teratomas

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 Retroperitoneal Teratoma? (Etiology)

The cause of development of Retroperitoneal Teratoma is generally unknown. It is believed that abnormal differentiation of germ cells gives rise 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

What are the Signs and Symptoms of Retroperitoneal Teratoma?

The signs and symptoms of Retroperitoneal Teratoma depend on subtype of the tumor (whether mature/immature and whether benign/malignant). It may vary from one individual to another. In general, small tumors may be asymptomatic and large tumors can cause signs and symptoms that may include the following:

• Presence of a well-circumscribed tumor in the abdominal cavity; most of the tumors are observed near the liver and kidneys, especially near the left kidney
• The tumors may range in size from a few cm to over 30 cm; they may be solid or cystic in nature
• Tumors are typically solitary, but in some cases, they may grow large enough to involve both kidneys
• Large tumors (size over 4 cm) can compress the surrounding structures or organs and give the sensation of an abdominal mass
• Abdominal pain from large-sized tumors
• Abdominal discomfort, heaviness, and tenderness
• Nausea and vomiting
• Frequent urination due to compression/pressure of the tumor

It is reported that approximately 75% of the tumors are benign, while 25% can turn malignant. When metastasis is noted, it may result in weight loss, fatigue, and other systemic symptoms.

How is Retroperitoneal Teratoma Diagnosed?

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

• Physical examination and complete medical history screening
• Assessment of the symptoms observed
• Blood tests for various markers
• Alpha fetoprotein (AFP) blood test
• Plain X-ray of the abdomen
• CT or CAT scan with contrast of the abdomen usually shows a well-defined mass, which may have calcifications. This radiological procedure creates detailed 3-dimensional images of structures inside the body
• MRI scans of the abdomen: Magnetic resonance imaging (MRI) uses a magnetic field to create high-quality pictures of certain parts of the body, such as tissues, muscles, nerves, and bones. These high-quality pictures may reveal the presence of the tumor
• Ultrasound scan of the abdomen
• Prenatal diagnostic tests, if necessary

Invasive diagnostic procedures such as:

• Laparoscopy: A special device is inserted through a small hole into the abdomen, to visually examine it. If necessary, a tissue sample is obtained for further analysis. Exploration of the abdomen using a laparoscope is called ‘exploratory laparoscopy’
• Laparotomy: The abdomen is opened through an incision for examination, and if required, a biopsy sample obtained. Exploration of the abdomen using laparotomy procedure is called ‘exploratory laparotomy’

Although the above modalities can be used to make an initial diagnosis, a tissue biopsy of the tumor is necessary to make a definitive diagnosis to begin treatment. The tissue for diagnosis can be procured in multiple different ways which include:

• Fine needle aspiration (FNA) biopsy of the tumor: A FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred. Also, Solitary Fibrous Tumor is a fibrous tumor and FNA usually does not reveal many cells on microscopic examination
• Core biopsy of the tumor
• Open biopsy of the tumor

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

Note: A differential diagnosis to eliminate other tumor types may be necessary prior to establishing a definite 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 Retroperitoneal Teratoma?

The complications of Retroperitoneal Teratoma may depend on the size of the tumor and the region of the body affected. It also depends on whether the tumor is benign or malignant. The complications may include the following:

• Stress and anxiety due to fear of cancer of the retroperitoneum
• Large tumor masses may get secondarily infected with bacteria or fungus
• Tumor rupture and torsion:
  • Rupture of tumor can cause peritonitis; it can also cause shock and internal bleeding
  • Usually, larger tumors are more prone for torsion
  • Torsion can result in acute abdominal pain and a surgical emergency
• Some immature teratomas are known to rupture and cause associated complications
• Recurrence of the tumor following its incomplete surgical removal: Immature forms are more often likely to recur than mature forms
• Some mature teratomas are known to transform to malignant teratomas, which are most commonly carcinomas, sarcomas, or adenocarcinomas
• Metastasis of malignant teratoma to distant sites
• It is reported that Retroperitoneal Teratomas in males may coexist with testicular cancer in about 50% of the cases. Hence, men who are diagnosed with the tumor, should be additionally screened for cancers affecting the testis

There may be complications related to the methods used in treating the condition and may include:

• Side effects of radiation therapy that may include sunburn-like rashes, where radiation was targeted, red or dry skin, heaviness of the breasts, and general fatigue
• Side effects of chemotherapy, which may include nausea, vomiting, hair loss, decreased appetite, mouth sores, fatigue, low blood cell counts, and a higher chance of developing infections
• The treatment can also cause infertility in men and women. Hence, measures to protect the individual’s fertility must be considered, before starting chemotherapy
• Damage to the muscles, vital nerves, and blood vessels, during surgery
• Post-surgical infection at the wound site is a potential complication

How is Retroperitoneal Teratoma Treated?

The treatment of Retroperitoneal Teratoma may involve the following:

• Generally, the treatment of choice is complete surgical excision for all Retroperitoneal Teratomas
• However, while it is usually possible to surgically remove a benign teratoma, the complete removal of a malignant teratoma may be challenging, if local infiltration of the tumor is noted
• 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 Retroperitoneal Teratoma be Prevented?

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

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

• The prognosis of Retroperitoneal Teratoma depends upon whether it is a mature or immature type (also, whether the tumor is benign or malignant)
  • Many of the tumors are mature teratomas that behave in a benign manner. With complete surgical removal of the tumor, the prognosis is typically excellent
  • 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
• If immature teratomas can be completely removed through surgery, then the prognosis is generally improved

Additional and Relevant Useful Information for Retroperitoneal Teratoma:

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

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