Coccyx teratoma

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Classification according to ICD-10
D48.0 New formation of unsafe or unknown behavior in other and unspecified locations - bones and articular cartilage
O33.7 Care of the mother in the event of disproportion due to other deformities of the fetus
ICD-10 online (WHO version 2019)

The sacrococcygeal teratoma or coccygeal teratoma (lat. Teratoma sacrococcygeale ), a teratoma in the region of the coccyx , is the most common manifestation of germ cell tumors of infancy . It describes a malformation tumor in the lower part of the fetal spine that can grow into the pelvis of the unborn child.

pathology

A midline teratoma arises from a blastula cell (abortive twin). Histologically , it is lymphangiomatous lipomatous growths that contain descendants of all three germ layers.

The origin is assumed to be in cells of the primitive streak (Hensenscher knot) located anterior to the coccyx, with development around the 2nd – 3rd. Gestation week .

Usually the teratoma is mixed solid-cystic, purely cystic in about 15%

Occurrence and frequency

The incidence of tail teratomas is given as 1 in 40,000 live births, the female to male ratio is 4 to 1.

Classification

A classification can be based on pathological criteria :

  • benign, mature teratoma, most common form, about 60–70%
  • immature teratoma
  • embryonic teratoma
  • Teratocarcinoma in 10–30%

classification

The classification of the American Academy of Pediatric Surgery Section Survey is based on the localization and direction of growth :

  • Type I: "Deep medial form", growth "extra-fetal" behind the rectum in a caudal direction, most common form, about 47%
  • Type II: “extra-fetal” growth with expansion in front of the sacrum into the small pelvis
  • Type III: "extra-fetal" growth with expansion towards the abdomen
  • Type IV: growth exclusively within the pelvis

Clinical appearance

This tumor can, due to the incorrect development of the cotyledons, contain all kinds of tissue, including organs or other limbs. Coccyx teratomas are benign in over 90% of cases but tend to become malignant. They can be the size of a child's head by the time they are born. A subgroup of tumors is supplied with blood to such an extent that this can lead to severe fetal heart failure or even death of the unborn child.

diagnosis

The point in time at which the teratoma becomes apparent is essential for the diagnosis:

  • Fetal, already in the womb, by ultrasound during a routine examination or by fine ultrasound
  • Neonatal, in the newborn, mostly clinical
  • only in early childhood with a higher probability of a malignancy of 48% - 67% for girls or boys older than 2 months

In blood serum can Alpha-1-fetoprotein and beta HCG be increased.

X-ray of a newborn with a posterior teratoma
Sonogram showing the teratoma in relation to the tip of the coccyx, cauda equina and conus medullaris

For imaging , the tumor, its structure, extent and blood flow can be shown well in ultrasound and in magnetic resonance tomography , calcium and bone components can be seen in the X-ray image .

There is an association with meningomyelocele and vertebral body abnormalities.

The following complications can occur:

Differential diagnostics

The following are to be distinguished:

therapy

Before birth , fetal surgery between the 20th and 32nd week of pregnancy can reduce tumor blood flow and improve the circulatory situation. If there was no prenatal treatment, it will be surgically removed as soon as possible after the birth , including parts of the tailbone.

forecast

About 80% are benign. The likelihood of malignancy increases if it manifests after the newborn age.

literature

  • W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Springer 1996, Volume II, pp. 399 f., ISBN 3-540-60224-0 .

Web links

Individual evidence

  1. ↑ a b c d e f Radiopaedia
  2. ↑ a b c d e f g h i Marcel Bettex (ed.), Max Grob (introduction), D. Berger (arrangement), N. Genton, M. Stockmann: Pediatric Surgery. Diagnostics, indication, therapy, prognosis. 2nd, revised edition. Thieme, Stuttgart / New York 1982, pp. 10.26ff, ISBN 3-13-338102-4
  3. ^ RP Altman, JG Randolph, JR Lilly: Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. In: Journal of pediatric surgery. Volume 9, Number 3, June 1974, pp. 389-398, PMID 4843993 .
  4. ↑ HM Yoon, SJ Byeon, JY Hwang, JR Kim, AY Jung, JS Lee, HK Yoon, YA Cho: Sacrococcygeal teratomas in newborns: a comprehensive review for the radiologists. In: Acta radiologica. [Electronic publication before going to press] January 2017, doi: 10.1177 / 0284185117710680 , PMID 28530139 .
  5. ↑ P. Kohlberger, A. Schaller: The tail teratoma in obstetrics. In: Journal of Obstetrics and Neonatology. Volume 204, No. 3, 2000 May-Jun, pp. 106-113, doi: 10.1055 / s-2000-10205 , PMID 10909166 .