Sacrococcygeal teratoma (SCT) is a type of tumor known as a

teratoma A teratoma is a tumor made up of several different types of tissue, such as hair, muscle, teeth, or bone. Teratomata typically form in the ovary, testicle, or coccyx. Symptoms Symptoms may be minimal if the tumor is small. A testicular terato ...

that develops at the base of the

coccyx The coccyx ( : coccyges or coccyxes), commonly referred to as the tailbone, is the final segment of the vertebral column in all apes, and analogous structures in certain other mammals such as horses. In tailless primates (e.g. humans and othe ...

(tailbone) and is thought to be primarily derived from remnants of the

primitive streak The primitive streak is a structure that forms in the early embryo in amniotes. In amphibians the equivalent structure is the blastopore. During early embryonic development, the embryonic disc becomes oval shaped, and then pear-shaped with the ...

. Sacrococcygeal teratomas are

benign Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malign ...

75% of the time,

malignant Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

12% of the time, and the remainder are considered "immature teratomas" that share benign and malignant features. Benign sacrococcygeal teratomas are more likely to develop in younger children who are less than 5 months old, and older children are more likely to develop malignant sacrococcygeal teratomas. The

Currarino syndrome Currarino syndrome is an inherited congenital disorder where either the sacrum (the fused vertebrae forming the back of the pelvis) is not formed properly, or there is a mass in the presacral space in front of the sacrum, and there are malformatio ...

, due to an

autosomal dominant In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the effect of a different variant of the same gene on the other copy of the chromosome. The first variant is termed dominant and t ...

mutation in the

MNX1 Motor neuron and pancreas homeobox 1 (MNX1), also known as Homeobox HB9 (HLXB9), is a human protein encoded by the ''MNX1'' gene In biology, the word gene (from , ; "... Wilhelm Johannsen coined the word gene to describe the Mendelian unit ...

gene, consists of a presacral mass (usually a mature teratoma or anterior meningocele), anorectal malformation and sacral dysgenesis.

Presentation

Complications

Maternal complications of pregnancy may include mirror syndrome. Maternal complications of delivery may include a

Cesarean section Caesarean section, also known as C-section or caesarean delivery, is the surgical procedure by which one or more babies are delivered through an incision in the mother's abdomen, often performed because vaginal delivery would put the baby or mo ...

or, alternatively, a vaginal delivery with mechanical

dystocia Obstructed labour, also known as labour dystocia, is the baby not exiting the pelvis because it is physically block during childbirth although the uterus contracts normally. Complications for the baby include not getting enough oxygen which may ...

. Complications of the mass effect of a teratoma in general are addressed on the

page. Complications of the mass effect of a large SCT may include

hip dysplasia Hip dysplasia is an abnormality of the hip joint where the socket portion does not fully cover the ball portion, resulting in an increased risk for joint dislocation. Hip dysplasia may occur at birth or develop in early life. Regardless, it doe ...

bowel obstruction Bowel obstruction, also known as intestinal obstruction, is a mechanical or Ileus, functional obstruction of the Gastrointestinal tract#Lower gastrointestinal tract, intestines which prevents the normal movement of the products of digestion. Eith ...

urinary obstruction Urinary retention is an inability to completely empty the bladder. Onset can be sudden or gradual. When of sudden onset, symptoms include an inability to urinate and lower abdominal pain. When of gradual onset, symptoms may include urinary incont ...

hydronephrosis Hydronephrosis describes hydrostatic dilation of the renal pelvis and calyces as a result of obstruction to urine flow downstream. Alternatively, hydroureter describes the dilation of the ureter, and hydronephroureter describes the dilation of t ...

and

hydrops fetalis Hydrops foetalis or hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in ...

. Even a small SCT can produce complications of mass effect, if it is presacral (Altman Type IV). In the fetus, severe hydronephrosis may contribute to inadequate lung development. Also in the fetus and newborn, the

anus The anus (Latin, 'ring' or 'circle') is an opening at the opposite end of an animal's digestive tract from the mouth. Its function is to control the expulsion of feces, the residual semi-solid waste that remains after food digestion, which, d ...

may be

imperforate For postage stamps, separation is the means by which individual stamps are made easily detachable from each other. Methods of separation include: # perforation: cutting rows and columns of small holes # rouletting: small horizontal and vert ...

. Later complications of the mass effect and/or surgery may include

neurogenic bladder Neurogenic bladder dysfunction, or neurogenic bladder, refers to urinary bladder problems due to disease or injury of the central nervous system or peripheral nerves involved in the control of urination. There are multiple types of neurogenic bladde ...

, other forms of

urinary incontinence Urinary incontinence (UI), also known as involuntary urination, is any uncontrolled leakage of urine. It is a common and distressing problem, which may have a large impact on quality of life. It has been identified as an important issue in geria ...

fecal incontinence Fecal incontinence (FI), or in some forms encopresis, is a lack of control over defecation, leading to involuntary loss of bowel contents, both liquid stool elements and mucus, or solid feces. When this loss includes flatus (gas), it is referred ...

, and other chronic problems resulting from accidental damage to or sacrifice of nerves and muscles within the pelvis. Removal of the coccyx may include additional complications. In one review of 25 patients, however, the most frequent complication was an unsatisfactory appearance of the surgical scar.

Late effects

Late effects are of two kinds: consequences of the tumor itself, and consequences of surgery and other treatments for the tumor. Complications of not removing the coccyx may include both recurrence of the teratoma Synopsis: 45 survivors of infant SCT were followed up. Two reported recurrent benign teratoma and one reported metastatic adenocarcinoma originating from the residual coccyx. They were aged 21–43 at diagnosis. and

metastatic cancer Metastasis is a pathogenic agent's spread from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, the ...

. Synopsis: A 40 year old man has widely metastatic

adenocarcinoma Adenocarcinoma (; plural adenocarcinomas or adenocarcinomata ) (AC) is a type of cancerous tumor that can occur in several parts of the body. It is defined as neoplasia of epithelial tissue that has glandular origin, glandular characteristics, or ...

arising from the residual coccyx remaining after surgical removal of an apparently benign SCT at age 2 months. Late malignancies usually involve incomplete excision of the coccyx and are

.Although functional disability in survivors is common, a small comparative study found a nonsignificant difference between SCT survivors and a matched control group. In rare cases, pelvic scarring may necessitate that a pregnant woman who is a SCT survivor deliver her baby by

Cause

SCT is seen in 1 in every 35,000 live births, and is the most common tumor

presenting In medicine, a presentation is the appearance in a patient of illness or disease—or signs or symptoms thereof—before a medical professional. In practice, one usually speaks of a patient as ''presenting'' with this or that. Examples include: ...

in newborn humans. Most SCTs are found in babies and children, but SCTs have been reported in adults and the increasingly routine use of prenatal ultrasound exams has dramatically increased the number of diagnosed SCTs presenting in fetuses. Like other teratomas, an SCT can grow very large. Unlike other teratomas, an SCT sometimes grows larger than the rest of the fetus. Sacrococcygeal teratomas are the most common type of germ cell tumors (both

and

) diagnosed in

neonates An infant or baby is the very young offspring of human beings. ''Infant'' (from the Latin word ''infans'', meaning 'unable to speak' or 'speechless') is a formal or specialised synonym for the common term ''baby''. The terms may also be used to ...

infants An infant or baby is the very young offspring of human beings. ''Infant'' (from the Latin word ''infans'', meaning 'unable to speak' or 'speechless') is a formal or specialised synonym for the common term ''baby''. The terms may also be used to ...

, and

children A child ( : children) is a human being between the stages of birth and puberty, or between the developmental period of infancy and puberty. The legal definition of ''child'' generally refers to a minor, otherwise known as a person younger ...

younger than 4 years. SCTs occur more often in girls than in boys; ratios of 3:1 to 4:1 have been reported. Historically, sacrococcygeal teratomas present in 2 clinical patterns related to the child's age, tumor location, and likelihood of tumor malignancy. With the advent of routine prenatal ultrasound examinations, a third clinical pattern is emerging. * Fetal tumors present during prenatal ultrasound exams, with or without maternal symptoms. SCTs found during routine exams tend to be small and partly or entirely external. The internal SCTs are not easily seen via ultrasound, unless they are large enough to reveal their presence by the abnormal position of the fetal

urinary bladder The urinary bladder, or simply bladder, is a hollow organ in humans and other vertebrates that stores urine from the kidneys before disposal by urination. In humans the bladder is a distensible organ that sits on the pelvic floor. Urine enters ...

and other organs, but large fetal SCTs frequently produce maternal complications which necessitate non-routine, investigative ultrasounds. * Neonatal tumors present at birth protruding from the sacral site and are usually mature or immature teratomas. *Among infants and young children, the tumor presents as a palpable mass in the sacropelvic region compressing the bladder or rectum. These pelvic tumors have a greater likelihood of being malignant. An early survey found that the rate of tumor malignancy was 48% for girls and 67% for boys older than 2 months at the time of sacrococcygeal tumor diagnosis, compared with a

malignant tumor Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal ble ...

incidence of 7% for girls and 10% for boys younger than 2 months at the time of diagnosis. The pelvic site of the

primary tumor A primary tumor is a tumor growing at the anatomical site where tumor progression began and proceeded to yield a cancerous mass. Most cancers develop at their primary site but then go on to metastasize or spread to other parts of the body. These fur ...

has been reported to be an adverse prognostic factor, most likely caused by a higher rate of incomplete resection. *In older children and adults, the tumor may be mistaken for a

pilonidal sinus Pilonidal disease is a type of skin infection which typically occurs as a cyst Intergluteal cleft, between the cheeks of the buttocks and often at the upper end. Symptoms may include pain, swelling, and redness. There may also be drainage of flui ...

, or it may be found during a rectal exam or other evaluation.

Diagnosis

During

prenatal ultrasound Obstetric ultrasonography, or prenatal ultrasound, is the use of medical ultrasonography in pregnancy, in which sound waves are used to create real-time visual images of the developing embryo or fetus in the uterus (womb). The procedure is a stan ...

, an SCT having an external component may appear as a fluid-filled cyst or a solid mass sticking out from the fetus' body. Fetal SCTs that are entirely internal may be undetected if they are small; detection (or at least suspicion) is possible when the fetal bladder is seen in an abnormal position, due to the SCT pushing other organs out of place. At

birth Birth is the act or process of bearing or bringing forth offspring, also referred to in technical contexts as parturition. In mammals, the process is initiated by hormones which cause the muscular walls of the uterus to contract, expelling the f ...

, the usual presentation is a visible lump or mass under the skin at the top of the buttocks crease. If not visible, it can sometimes be felt; gently prodded, it feels somewhat like a hardboiled egg. A small SCT, if it is entirely inside the body, may not present for years, until it grows large enough to cause pain, constipation and other symptoms of a large mass inside the

pelvis The pelvis (plural pelves or pelvises) is the lower part of the trunk, between the abdomen and the thighs (sometimes also called pelvic region), together with its embedded skeleton (sometimes also called bony pelvis, or pelvic skeleton). The ...

, or until it begins to extend out of the pelvis. Even a relatively large SCT may be missed, if it is internal, because the bony

conceals and protects it.

Mediastinal tumor A mediastinal tumor is a tumor in the mediastinum, the cavity that separates the lungs from the rest of the chest. It contains the heart, esophagus, trachea, thymus, and aorta. The most common mediastinal masses are neurogenic tumors (20% of medias ...

s, including teratomas, are similarly concealed and protected by the

rib cage The rib cage, as an enclosure that comprises the ribs, vertebral column and sternum in the thorax of most vertebrates, protects vital organs such as the heart, lungs and great vessels. The sternum, together known as the thoracic cage, is a semi- ...

. Some SCTs are discovered when a child begins to talk at about age 2 years and complains of their bottom hurting or feeling "poopy" when they ride in a car seat. Other tumors can occur in the sacrococcygeal and/or presacral regions and hence must be ruled out to obtain a

differential diagnosis In healthcare, a differential diagnosis (abbreviated DDx) is a method of analysis of a patient's history and physical examination to arrive at the correct diagnosis. It involves distinguishing a particular disease or condition from others that p ...

. These include extraspinal ependymoma,

ependymoblastoma Primitive neuroectodermal tumor is a malignant (cancerous) neural crest tumor. It is a rare tumor, usually occurring in children and young adults under 25 years of age. The overall 5 year survival rate is about 53%. It gets its name because the m ...

neuroblastoma Neuroblastoma (NB) is a type of cancer that forms in certain types of nerve tissue. It most frequently starts from one of the adrenal glands but can also develop in the neck, chest, abdomen, or spine. Symptoms may include bone pain, a lump in the ...

and

rhabdomyosarcoma Rhabdomyosarcoma (RMS) is a highly aggressive form of cancer that develops from mesenchymal cells that have failed to fully differentiate into myocytes of skeletal muscle. Cells of the tumor are identified as rhabdomyoblasts. There are four subt ...

. Smaller SCTs with an external component, seen in prenatal ultrasounds or at birth, often are mistaken for

spina bifida Spina bifida (Latin for 'split spine'; SB) is a birth defect in which there is incomplete closing of the spine and the membranes around the spinal cord during early development in pregnancy. There are three main types: spina bifida occulta, me ...

. Cystic SCT and terminal myelocystocele are especially difficult to distinguish; for more accurate diagnosis, MRI has been recommended.

Treatment

The preferred first treatment for SCT is complete surgical removal (i.e., complete resection). The preferred approach to a small SCT is through the

perineum The perineum in humans is the space between the anus and scrotum in the male, or between the anus and the vulva in the female. The perineum is the region of the body between the pubic symphysis (pubic arch) and the coccyx (tail bone), includi ...

; a large SCT may require an additional approach through the

abdomen The abdomen (colloquially called the belly, tummy, midriff, tucky or stomach) is the part of the body between the thorax (chest) and pelvis, in humans and in other vertebrates. The abdomen is the front part of the abdominal segment of the torso. ...

. Resection should include the coccyx and may also include portions of the

sacrum The sacrum (plural: ''sacra'' or ''sacrums''), in human anatomy, is a large, triangular bone at the base of the spine that forms by the fusing of the sacral vertebrae (S1S5) between ages 18 and 30. The sacrum situates at the upper, back part ...

. The surgery should include reattachment of the small muscles and ligaments formerly attached to the coccyx, in effect reconstructing the posterior perineum. If not, there is an increased risk of

perineal hernia Perineal hernia is a hernia involving the perineum ( pelvic floor). The hernia may contain fluid, fat, any part of the intestine, the rectum, or the bladder. It is known to occur in humans, dogs, and other mammals, and often appears as a sudden ...

later in life. SCTs are classified morphologically according to their relative extent outside and inside the body: *Altman type I — entirely outside, sometimes attached to the body only by a narrow stalk *Altman type II — mostly outside *Altman type III — mostly inside *Altman type IV — entirely inside; this is also known as a presacral teratoma or retrorectal teratoma The Altman type is significant in the contexts of management of labor and delivery, surgical approach, and complications of SCT. Serial ultrasound and MRI monitoring of SCTs in fetuses

in utero ''In Utero'' is the third and final studio album by American rock band Nirvana. It was released on September 21, 1993, by DGC Records. After breaking into the mainstream with their second album, ''Nevermind'' (1991), Nirvana hired Steve Albini t ...

has demonstrated that the Altman type can change over time. As the tumor grows, it can push between other organs and through the perineum to the body surface where the tumor appears as a bulge covered only by skin. Sometimes, the tumor bulge later slips back inside the perineum. Like all

s, a sacrococcygeal teratoma has the potential to be

, and the standard of care requires long-term followup by an

oncologist Oncology is a branch of medicine that deals with the study, treatment, diagnosis and prevention of cancer. A medical professional who practices oncology is an ''oncologist''. The name's etymological origin is the Greek word ὄγκος (''ó ...

Management of fetal SCTs

Management of most fetal SCTs involves

watchful waiting Watchful waiting (also watch and wait or WAW) is an approach to a medical problem in which time is allowed to pass before medical intervention or therapy is used. During this time, repeated testing may be performed. Related terms include ''expec ...

prior to any treatment. An often used

decision tree A decision tree is a decision support tool that uses a tree-like model of decisions and their possible consequences, including chance event outcomes, resource costs, and utility. It is one way to display an algorithm that only contains condit ...

is as follows: *Perform detailed ultrasound exam including fetal

echocardiogram An echocardiography, echocardiogram, cardiac echo or simply an echo, is an ultrasound of the heart. It is a type of medical imaging of the heart, using standard ultrasound or Doppler ultrasound. Echocardiography has become routinely used in th ...

and Doppler

flow analysis Flow may refer to: Science and technology * Fluid flow, the motion of a gas or liquid * Flow (geomorphology), a type of mass wasting or slope movement in geomorphology * Flow (mathematics), a group action of the real numbers on a set * Flow (psy ...

**If fetal high output failure, placentomegaly, or hydrops ***If fetus not mature, perform

pregnancy termination Abortion is the termination of a pregnancy by removal or expulsion of an embryo or fetus. An abortion that occurs without intervention is known as a miscarriage or "spontaneous abortion"; these occur in approximately 30% to 40% of pregn ...

fetal intervention Fetal surgery also known as antenatal surgery, prenatal surgery, is a growing branch of maternal-fetal medicine that covers any of a broad range of surgical techniques that are used to treat congenital abnormalities in fetuses who are still in ...

***Else fetus mature, perform emergency

**Else no emergent problems, perform serial

non-stress test A nonstress test (NST) is a screening test used in pregnancy to assess fetal status by means of the fetal heart rate and its responsiveness. A cardiotocograph is used to monitor the fetal heart rate and presence or absence of uterine contractions. ...

s and ultrasound

biophysical profile A biophysical profile (BPP) is a prenatal ultrasound evaluation of fetal well-being involving a scoring system, with the score being termed Manning's score.

s and plan delivery, as follows ***If emergent problems develop, return to top of decision tree ***Else if SCT over 5–10 cm or

polyhydramnios Polyhydramnios is a medical condition describing an excess of amniotic fluid in the amniotic sac. It is seen in about 1% of pregnancies. It is typically diagnosed when the amniotic fluid index (AFI) is greater than 24 cm. There are two clin ...

, perform early (37 weeks gestation) elective Cesarean section ***Else SCT small and no complications, permit term

spontaneous vaginal delivery A vaginal delivery is the birth of offspring in mammals (babies in humans) through the vagina (also called the "birth canal"). It is the most common method of childbirth worldwide. It is considered the preferred method of delivery, with lower m ...

Emergent problems include maternal mirror syndrome,

, and

preterm labor Preterm birth, also known as premature birth, is the birth of a baby at fewer than 37 weeks gestational age, as opposed to full-term delivery at approximately 40 weeks. Extreme preterm is less than 28 weeks, very early preterm birth is between 2 ...

. Poor management decisions, including interventions that are either premature or delayed, can have dire consequences. A very small retrospective study of 9 babies with SCTs greater than 10 cm diameter reported slightly higher survivorship in babies remaining in utero slightly longer. In many cases, a fetus with a small SCT (under 5 or 10 cm) may be delivered vaginally. Prior to the advent of prenatal detection and hence scheduled C-section, 90% of babies diagnosed with SCT were born full term.

Management of adult SCTs

SCTs are very rare in adults, and as a rule these tumors are benign and have extremely low potential for malignancy. This estimation of potential is based on the idea that because the tumor existed for decades prior to diagnosis, without becoming malignant, it has little or no potential to ever become malignant. For this reason, and because coccygectomy in adults has greater risks than in babies, some surgeons prefer not to remove the coccyx of adult survivors of SCT. There are case reports of good outcomes.

References

External links

{{Joints of torso, state=collapsed Congenital disorders Obstetrics Rare cancers Pediatric cancers