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INTRODUCTION

A teratoma is a rare type of tumor that can contain fully developed tissues and organs, including hair, teeth, muscle,fat, skin, bone and endocrine tissue.
Teratomas are germ cell tumors commonly composed of multiple cell types derived from one or more of the 3 germ layers.
By definition, they contain elements from all three embryological layers: endoderm, mesoderm and ectoderm although frequently, elements from only two layers are evident.
Teratomas are most common in the tailbone, ovaries, and testicles, but can occur elsewhere in the body.
Teratomas can appear in newborns, children, or adults. They’re more common in females. Teratomas are usually benign in newborns, but may still require surgical removal.

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LOCATION�

They are found in a variety of locations, including:

Ovary: see ovarian teratoma (also known as ovarian dermoid cyst)
Testis: testicular teratoma
Mediastinum: see mediastinal teratoma

account for 27% of all teratomas in adults
account for 4-13% of all teratomas in children ³

Intracranial: see intracranial teratoma
Sacrococcygeal region: see sacrococcygeal teratoma

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TYPES

Teratomas are generally described as either mature or immature.
Mature teratomas are usually benign (not cancerous). But they may grow back after being surgically removed.
Immature teratomas are more likely to develop into a malignant cancer.

Mature teratomas are further classified as:
cystic: enclosed in its own fluid-containing sac e.g. Dermoid cyst
solid: made up of tissue, but not self-enclosed
mixed: containing both solid and cystic parts

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EPIDEMIOLOGY�

Sacrococcygeal teratomas (SCT) are the most common tumors in newborns, occurring in 1 per 20,000-40,000 births.
Mature cystic teratomas account for 10-20% of all ovarian neoplasms. They are the most common ovarian germ cell tumor and also the most common ovarian neoplasm in patients younger than 20 years. They are bilateral in 8-14% of cases.
Germ cell tumors represent 95% of testicular tumors after puberty, but pure benign teratomas of the testis are rare.
Benign teratomas of the mediastinum are rare, representing 8% of all tumors of this region.
Sacrococcygeal teratomas are much more common in females than in males.
Cystic teratomas of the ovary can occur in persons of any age, although they are diagnosed most frequently during the reproductive years (20-40 years)

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Testicular teratomas may occur at any age but are more common in infants and children. In adults, pure testicular teratomas are rare, constituting 2-3% of germ cell tumors.
Mediastinal teratomas can be found in persons of any age but occur most commonly in adults aged 20-40 years.

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AETIOLOGY/RISK FACTORS

The cause of germ cell tumors isn't completely understood.
A number of inherited defects have also been associated with an increased risk for developing germ cell tumors including the CNS and genitourinary tract malformations and major malformations of the lower spine.
Specifically, males with cryptorchidism (failure of the testes to descend into the scrotal sac) have an increased risk to develop testicular germ cell tumors. Cryptorchidism can occur alone, however, and is also present in some genetic syndromes.
Some genetic syndromes caused by extra or missing sex chromosomes can cause incomplete or abnormal development of the reproductive system.

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SYMPTOMS OF A TERATOMA

Teratomas may have no symptoms at first. When symptoms develop, they can be different depending on where the teratoma is located. The most common locations for teratomas are the tailbone (coccyx), ovaries, and testicles.
Signs and symptoms common to many teratomas include:
Pain
swelling and bleeding
mildly elevated levels of alpha-feroprotein (AFP), a marker for tumors
mildly elevated levels of the hormone beta-human chorionic gonadotropin (BhCG)

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SACROCOCCYGEAL TERATOMA

It is a congenital condition affecting the sacrococcygeal region in the coccyx or tailbone. It’s the most common tumor found in newborns and children, but it’s still rare overall.
20% of the cases are stillborn babies. It is common in a female child.
Retention of large amount of primitive toti-potential cell in this region may be the reason for this tumour.
It occurs between coccyx and rectum, attached to coccyx, extends commonly downwards as a huge mass, occasionally upwards into the pelvis.

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CLINICAL FEATURES

A sacrococcygeal teratoma can grow outside or inside the body in the tailbone area. Aside from a visible mass, symptoms include:
Constipation
Abdominal pain
Painful urination
Swelling in the pubic or sacrococcygeal region pushing the rectum anteriorly.
Leg weakness
The most common pattern is in neonates, who present with a large, predominantly benign tumour protruding from the sacral area that is noted prenatally or at the time of delivery.
The swelling is fixed to the sacrum and coccyx from which it is impossible to separate/isolate.

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DIFFERENTIAL DIAGNOSES�

Rectal prolapse
Imperforate anus
Rectal abscesses
Anal duct or pilonidal cysts
Lymphangiomas

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INVESTIGATION

Sacrococcygeal teratomas may be diagnosed antenatally during routine ultrasounds, fetal anomaly scans, or when the mother presents with clinical symptoms such as size greater than dates or polyhydramnios.
A common symptom is a swelling at the tailbone, X-ray of the pelvis, ultrasound, and CT scans to help diagnose a teratoma.

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TREATMENT

Excision of the teratoma with part of sacrum and coccyx.
If the teratoma is malignant, chemotherapy is used along with the surgery.
Survival rates are excellent with modern chemotherapy.
In fetuses with larger tumors in utero, cesarean delivery should be considered to prevent dystocia or tumor rupture.

COMPLICATIONS

Ulceration
Secondary infection
Haemorrhage
Teratocarcinomatous change occurs by one year of age.

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OVARIAN TERATOMA�

About 1 to 3% of mature ovarian teratomas are cancerous. They’re usually found in women during their reproductive years.
Immature (malignant) ovarian teratomas are rare. They’re usually found in girls and young women up to the age of 20.
Mature cystic teratomas (dermoid cysts) usually present no symptoms discovered as incidental findings on physical examination, during radiographic studies, or during abdominal surgery performed for other indications.
Sometimes large dermoid cysts cause twisting of the ovary (ovarian torsion), which can result in abdominal or pelvic pain.
In 25% of cases, dermoid cysts are found in both ovaries. This increases risk of infertility.

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CLINICAL FEATURES

Mature ovarian teratomas (dermoid cysts) usually present no symptoms.
Asymptomatic mature cystic teratomas of the ovaries have been reported at rates of 6-65%.

When symptoms are present, they may include:
abdominal pain (Torsion or acute rupture) from slight to moderate in intensity to severe pain.
mass or swelling, and
abnormal uterine bleeding (due to Hormonal production)
Bladder symptoms, gastrointestinal disturbances, and back pain are less frequent.

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DIFFERENTIAL DIAGNOSES�

Tubo-ovarian abscesses
Ectopic pregnancy
Pedunculated uterine fibroids
Hydrosalpinx
Endometriomas
Endometrial Carcinoma
Endometriosis
Other benign or malignant ovarian neoplasms

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INVESTIGATIONS

Elevated serum alpha-fetoprotein (AFP) and beta human chorionic gonadotropin (HCG) levels may be indicative of malignancy, as these values are within reference ranges in most patients with benign teratomas.

The workup for cystic teratomas is largely radiographic, and their appearance is similar despite varying locations. So, ultrasound, CT scanning and MRI.
(MRI) likely provides the most accurate assessment of anatomical extent and impact

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TREATMENT

Mature cystic teratomas of the ovaries may be removed by simple cystectomy rather than salpingo-oophorectomy.
Although malignant degeneration is quite rare, the cyst should be removed in its entirety

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COMPLICATIONS

Complications of ovarian teratomas include the following:

Torsion is by far the most significant cause of morbidity, occurring in –3-11% of cases.(tumor size correlates with increased risk of torsion).
Rupture of a cystic teratoma is rare and may be spontaneous or associated with torsion.
Infection is uncommon and Coliform bacteria are the organisms most commonly implicated.
Hemolytic anemia - Autoimmune hemolytic anemia has been associated with mature cystic teratomas in rare cases.
Malignant degeneration. In its pure form, mature cystic teratoma of the ovary is always benign, but may undergo malignant transformation in approximately 0.2-2% of cases

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MEDIASTINAL TERATOMAS��

Mediastinal teratomas are often asymptomatic. When symptoms are present, they relate to mechanical effects and include:
Chest pain,
Cough,
Dyspnea, or
Symptoms related to recurrent pneumonitis.
Many patients present with respiratory findings, and the pathognomonic finding of trichoptysis (cough productive of hair or sebaceous material) may result if a communication develops between the mass and the tracheobronchial tree.
Mediastinal teratomas are occasionally discovered incidentally on chest radiographs.

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Fine-needle aspiration or core biopsy can be used to differentiate benign from malignant mediastinal masses in 90% of cases.

TREATMENT

Mature teratomas of the mediastinum should be completely surgically resected.
The tumor may be adherent to surrounding structures, necessitating resection of the pericardium, pleura, or lung.

PROGNOSIS

When complete resection is achieved, it results in excellent long-term cure rates with little chance of recurrence.
When complete resection is impossible, partial resection often leads to symptom relief, frequently without relapse.

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DIFFERENTIAL DIAGNOSES�

Thymomas (21%)
Lymphomas (13%)
Pericardial cysts (7%)
Neurogenic tumors (23% of mediastinal masses)
Enterogenous cysts (10%)

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TESTICULAR TERATOMAS�

Is one the Testicular tumour most common between the ages of 20 to 30, though it can occur at any age.
It arises from totipotent cells, i.e. ecto, meso, endoderm.

Classification of Testicular Tumours

1. Seminoma—40%

2. Teratoma—32%

3. Seminoma + teratoma—14%

4. Interstitial tumours—1.5% (Leydig cell tumour - musculinises, Sertoli cell tumour-feminises)

5. Lymphomas—7%

6. Others

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INTRODUCTION

There are two broad types of testicular teratoma: pre- and post-puberty. Pre-puberty or pediatric teratomas are usually mature and noncancerous.
Post-puberty (adult) testicular teratomas are malignant. About two-thirds of men diagnosed with adult teratoma show an advanced state of metastasis (spread) of the cancer.
Both benign and malignant testicular teratoma usually cause testicular pain.

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TYPES OF TERATOMA

1. Malignant teratoma differentiated: It is the least common variety (1 %).

It is almost a benign tumour-Dermoid cyst. Orchidectomy cures the disease.

2. Malignant teratoma intermediate: This is the most common variety (30%) of teratoma containing malignant and incompletely differentiated components.

3. Malignant teratoma anaplastic: Highly malignant tumour. Secretes alfa fetoprotein (AFP). Cells are presumed to be from yolk sac.

4. Malignant teratoma trophoblastic: This is an uncommon tumour (I%). This secretes very high levels of β-Human chorionic gonadotropins (β-hCG).

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CLINICAL FEATURES

Typical presentation age: Teratoma 20-30 years, seminoma 30-40 years.
Testicular teratomas most often present as a painless scrotal mass, except in the case of torsion
Fullness and heaviness in the scrotum.
In most cases, the masses are firm or hard, non-tender, and do not transilluminate.
Testicular pain and scrotal swelling are occasionally reported with teratomas, but this is non-specific and simply indicates torsion until proven otherwise.
Hydrocele is frequently associated with teratoma in childhood.
On examination, the testis is diffusely enlarged, rather than nodular, although a discreet nodule in the upper or lower pole sometimes can be appreciated.

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