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Sacrococcygeal teratoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]Sabawoon Mirwais, M.B.B.S, M.D.[3]

Synonyms and keywords: Fetiform Teratoma, Fetus in fetu

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]

Overview

Sacrococcygel teratoma was first described by Stanley in 1842. In 1863, first successful surgery was performed on Sacrococcygeal teratoma by Dr. W. Blizard. Between 1950s and 1960s, germ cell theory of the origin of Sacrococcygeal teratoma was introduced.[1] Sacrococcygeal teratoma is one of the most common congenital tumors.[2] The incidence of Sacrococcygeal teratoma is estimated to be 1 in 35,000 births.[2] Sacrococcygeal teratoma is classified into four different categories according to American Academy of Pediatrics Surgery classification based upon the anatomical location of the tumor.[3][4] Subtypes of sacrococcygeal teratoma have different staging systems based on the size and growth of the tumor, lymph node involvement, and the presence of metastasis.[5] Sacrococcygeal teratoma originates from the pluripotent cells in primitive knot or Hensen’s node, which is the primary organizer of embryonic development, located on the anterior surface of the sacrum or coccyx by 2rd or 3rd gestational week.[6] Development of Sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and losses of the 6q24-qter and 18q21-qter regions.[7][8] The pathophysiology of sacrococcygeal teratoma depends on the histological subtype. Development of sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and losses of the 6q24-qter and 18q21-qter regions.[7][8] Sacrococcygeal teratoma must be differentiated from endodermal sinus tumor, ependymoma, fibromatosis, ganglioneuroma, giant cell tumor of the sacrum, leiomyoma, meningomyelocele, ovarian teratoma, neuroblastoma, retrorectal hamartoma, intracanalicular epidermoid tumor, rhabdomyosarcoma, paraganglioma, dermal sinus stalk ascending towards the conus modulars, and hydromelia.[9] According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for sacrococcygela teratoma.[10] The hallmark of sacrococcygeal teratoma is a protruding mass at the sacrococcygeal region. It is important to obtain the history about the onset, and the progression of the symptoms. Most common symptoms of sacrococcygeal teratoma are due to compression caused by the large tumor mass.[11] Patient with sacrococcygeal teratoma usually are generally well-appearanceing. Physical examination of patients with sacrococcygeal teratoma is usually remarkable for a protruding pre sacral mass.[12] Alpha-Fetoprotein and beta-hCG should be evaluated in patients with sacrococcygeal teratoma to determine the presence of malignant component of the tumor.[13] On x-ray, sacrococcygeal teratoma is characterized by a large mass projecting from the lower pelvic region or within the abdominopelvic cavity.[14] CT scan is not part of the routine investigation of sacrococcygeal teratoma. On CT scan, sacrococcygeal teratoma is characterized by bone, fat, and cystic components.[15] Ultrasound is used to diagnose Sacrococcygeal teratoma in second trimester.[16] Mature sacrococcygeal teratomas tend to be cystic, showing anechoic component. Immature sacrococcygeal teratomas are much rare and solid type, showing echogenic mass within the pelvis.[17] Echocardiography identifies high output cardiac state preceding hydrops fetalis.[18] Colour Doppler studies in sacrococcygeal teratoma may show marked hypervascularity with arterio-venous (AV) shunting.[19] There is no medical treatment for sacrococcygeal teratoma; the mainstay of therapy is surgical.[20] Perinatal surgical intervention is used to decrease cardiovascular complications caused by the large sacrococcygeal teratoma.[21] Early complete resection is the mainstay of management of benign tumor. Complete surgical excision in malignant sacrococcygeal teratoma is followed by platinum based chemotherapy.[22] Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. Most of the sacrococcygeal teratomas are benign in nature and prognosis is generally regarded as good after resection.[23] The sacrococcygeal teratoma with cystic morphology is associated with the most favorable prognosis.[24]

Historical Perspective

Sacrococcygel teratoma was first described by Stanley in 1842. In 1863, first successful surgery was performed on Sacrococcygeal teratoma by Dr. W. Blizard. Between 1950s and 1960s, germ cell theory of the origin of Sacrococcygeal teratoma was introduced.[1]

Classification

Sacrococcygeal teratoma is classified into four different categories according to American Academy of Pediatrics Surgery classification based upon the anatomical location of the tumor.[3][4] Subtypes of sacrococcygeal teratoma have different staging systems based on the size and growth of the tumor, lymph node involvement, and the presence of metastasis.[5]

Pathophysiology

Sacrococcygeal teratoma originates from the pluripotent cells in primitive knot or Hensen’s node, which is the primary organizer of embryonic development, located on the anterior surface of the sacrum or coccyx by 2rd or 3rd gestational week.[25] Development of Sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and losses of the 6q24-qter and 18q21-qter regions.[7][8] The pathophysiology of sacrococcygeal teratoma depends on the histological subtype.

Causes

Development of sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and losses of the 6q24-qter and 18q21-qter regions.[7][8]

Differential Diagnosis

Sacrococcygeal teratoma must be differentiated from endodermal sinus tumor, ependymoma, fibromatosis, ganglioneuroma, giant cell tumor of the sacrum, leiomyoma, meningomyelocele, ovarian teratoma, neuroblastoma, retrorectal hamartoma, intracanalicular epidermoid tumor, rhabdomyosarcoma, paraganglioma, dermal sinus stalk ascending towards the conus modulars, and hydromelia.[26]

Epidemiology & Demographics

Sacrococcygeal teratoma is one of the most common congenital tumors.[2] The incidence of Sacrococcygeal teratoma is estimated to be 1 in 35,000 births.[2]

Risk Factors

There are no established risk factors for sacrococcygeal teratoma.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for sacrococcygela teratoma.[10]

Natural History, Complications, and Prognosis

Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. Most of the sacrococcygeal teratomas are benign in nature and prognosis is generally regarded as good after resection.[27] The sacrococcygeal teratoma with cystic morphology is associated with the most favorable prognosis.[24]

History and Symptoms

The hallmark of sacrococcygeal teratoma is a protruding mass at the sacrococcygeal region. It is important to obtain the history about the onset, and the progression of the symptoms. Most common symptoms of sacrococcygeal teratoma are due to compression caused by the large tumor mass.[28]

Physical Examination

Patient with sacrococcygeal teratoma usually are generally well-appearanceing. Physical examination of patients with sacrococcygeal teratoma is usually remarkable for a protruding pre sacral mass.[29]

Laboratory tests

Alpha-Fetoprotein and beta-hCG should be evaluated in patients with sacrococcygeal teratoma to determine the presence of malignant component of the tumor.[13]

Pelvic X-Ray Findings in Sacrococcygeal Teratoma

On x-ray, sacrococcygeal teratoma is characterized by a large mass projecting from the lower pelvic region or within the abdominopelvic cavity.[30]

CT Findings in Sacrococcygeal Teratoma

CT scan is not part of the routine investigation of sacrococcygeal teratoma. On CT scan, sacrococcygeal teratoma is characterized by bone, fat, and cystic components.[31]

Ultrasound Findings in Sacrococcygeal Teratoma

Ultrasound is used to diagnose Sacrococcygeal teratoma in second trimester.[16] Mature sacrococcygeal teratomas tend to be cystic, showing anechoic component. Immature sacrococcygeal teratomas are much rare and solid type, showing echogenic mass within the pelvis.[32] Echocardiography identifies high output cardiac state preceding hydrops fetalis.[18]

Other Imaging Findings

Colour Doppler studies in sacrococcygeal teratoma may show marked hypervascularity with arterio-venous (AV) shunting.[33]

Medical Therapy

There is no medical treatment for sacrococcygeal teratoma; the mainstay of therapy is surgical.[34]

Surgery

Perinatal surgical intervention is used to decrease cardiovascular complications caused by the large sacrococcygeal teratoma.[35] Early complete resection is the mainstay of management of benign tumor. Complete surgical excision in malignant sacrococcygeal teratoma is followed by platinum based chemotherapy.[36]

References

  1. ↑ 1.0 1.1 Olson JS. The History of Cancer, An Annotated Bibliography. ABC-CLIO; 1989.
  2. ↑ 2.0 2.1 2.2 2.3 Sacrococcygeal Teratoma. Cincinnati Children’s (2015)http://www.cincinnatichildrens.org/service/f/fetal-care/conditions/sct/default/ Accessed on November 24, 2015
  3. ↑ 3.0 3.1 Puri P, Höllwarth ME. Pediatric Surgery, Diagnosis and Management. Springer Science & Business Media; 2009.
  4. ↑ 4.0 4.1 Myers LB, Bulich LA. Anesthesia for Fetal Intervention and Surgery. PMPH-USA; 2005.
  5. ↑ 5.0 5.1 DeVita VT, Lawrence TS, Rosenberg SA. DeVita, Hellman, and Rosenberg’s Cancer, Principles & Practice of Oncology. Lippincott Williams & Wilkins; 2008.
  6. ↑ http://www.hindawi.com/journals/criog/2012/131369/
  7. ↑ 7.0 7.1 7.2 7.3 Harms D, Zahn S, Göbel U, Schneider DT (2006). “Pathology and molecular biology of teratomas in childhood and adolescence”. Klin Padiatr. 218 (6): 296–302. doi:10.1055/s-2006-942271. PMID 17080330.
  8. ↑ 8.0 8.1 8.2 8.3 Veltman I, Veltman J, Janssen I, Hulsbergen-van de Kaa C, Oosterhuis W, Schneider D, Stoop H, Gillis A, Zahn S, Looijenga L, Göbel U, van Kessel AG (2005). “Identification of recurrent chromosomal aberrations in germ cell tumors of neonates and infants using genomewide array-based comparative genomic hybridization”. Genes Chromosomes Cancer. 43 (4): 367–76. doi:10.1002/gcc.20208. PMID 15880464.
  9. ↑ Myers LB, Bulich LA. Anesthesia for Fetal Intervention and Surgery. PMPH-USA; 2005.
  10. ↑ 10.0 10.1 Recommendations. US preventive services task force(2015) http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=sacrococcygeal+teratoma Accessed on November 25, 2015
  11. ↑ https://rarediseases.org/rare-diseases/sacrococcygeal-teratoma/
  12. ↑ Mahour GH (1988). “Sacrococcygeal teratomas”. CA Cancer J Clin. 38 (6): 362–7. PMID 3141009.
  13. ↑ 13.0 13.1 Wu JT, Book L, Sudar K (1981). “Serum alpha fetoprotein (AFP) levels in normal infants”. Pediatr. Res. 15 (1): 50–2. PMID 6163129.
  14. ↑ http://radiopaedia.org/articles/sacrococcygeal-teratoma
  15. ↑ http://radiopaedia.org/articles/sacrococcygeal-teratoma
  16. ↑ 16.0 16.1 Wilson RD, Hedrick H, Flake AW, Johnson MP, Bebbington MW, Mann S, Rychik J, Liechty K, Adzick NS (2009). “Sacrococcygeal teratomas: prenatal surveillance, growth and pregnancy outcome”. Fetal. Diagn. Ther. 25 (1): 15–20. doi:10.1159/000188056.
  17. ↑ http://radiopaedia.org/articles/sacrococcygeal-teratoma
  18. ↑ 18.0 18.1 Adzick NS (2010). “Open fetal surgery for life-threatening fetal anomalies”. Semin Fetal Neonatal Med. 15 (1): 1–8. doi:10.1016/j.siny.2009.05.003. PMID 19540178.
  19. ↑ http://radiopaedia.org/articles/sacrococcygeal-teratoma
  20. ↑ https://rarediseases.org/rare-diseases/sacrococcygeal-teratoma/
  21. ↑ Roybal JL, Moldenhauer JS, Khalek N, Bebbington MW, Johnson MP, Hedrick HL, Adzick NS, Flake AW (2011). “Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas”. J. Pediatr. Surg. 46 (7): 1325–32. doi:10.1016/j.jpedsurg.2010.10.020. PMID 21763829.
  22. ↑ Marina NM, Cushing B, Giller R, Cohen L, Lauer SJ, Ablin A, Weetman R, Cullen J, Rogers P, Vinocur C, Stolar C, Rescorla F, Hawkins E, Heifetz S, Rao PV, Krailo M, Castleberry RP (1999). “Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children’s Cancer Group Intergroup Study”. J. Clin. Oncol. 17 (7): 2137–43. PMID 10561269.
  23. ↑ http://www.chop.edu/conditions-diseases/sacrococcygeal-teratoma-sct/about#.VnhH0bRYZFI
  24. ↑ 24.0 24.1 Shue E, Bolouri M, Jelin EB, Vu L, Bratton B, Cedars E, Yoke L, Byrne F, Hirose S, Feldstein V, Miniati D, Lee H (2013). “Tumor metrics and morphology predict poor prognosis in prenatally diagnosed sacrococcygeal teratoma: a 25-year experience at a single institution”. J. Pediatr. Surg. 48 (6): 1225–31. doi:10.1016/j.jpedsurg.2013.03.016. PMID 23845611.
  25. ↑ http://www.hindawi.com/journals/criog/2012/131369/
  26. ↑ Myers LB, Bulich LA. Anesthesia for Fetal Intervention and Surgery. PMPH-USA; 2005.
  27. ↑ http://www.chop.edu/conditions-diseases/sacrococcygeal-teratoma-sct/about#.VnhH0bRYZFI
  28. ↑ https://rarediseases.org/rare-diseases/sacrococcygeal-teratoma/
  29. ↑ Mahour GH (1988). “Sacrococcygeal teratomas”. CA Cancer J Clin. 38 (6): 362–7. PMID 3141009.
  30. ↑ http://radiopaedia.org/articles/sacrococcygeal-teratoma
  31. ↑ http://radiopaedia.org/articles/sacrococcygeal-teratoma
  32. ↑ http://radiopaedia.org/articles/sacrococcygeal-teratoma
  33. ↑ http://radiopaedia.org/articles/sacrococcygeal-teratoma
  34. ↑ https://rarediseases.org/rare-diseases/sacrococcygeal-teratoma/
  35. ↑ Roybal JL, Moldenhauer JS, Khalek N, Bebbington MW, Johnson MP, Hedrick HL, Adzick NS, Flake AW (2011). “Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas”. J. Pediatr. Surg. 46 (7): 1325–32. doi:10.1016/j.jpedsurg.2010.10.020. PMID 21763829.
  36. ↑ Marina NM, Cushing B, Giller R, Cohen L, Lauer SJ, Ablin A, Weetman R, Cullen J, Rogers P, Vinocur C, Stolar C, Rescorla F, Hawkins E, Heifetz S, Rao PV, Krailo M, Castleberry RP (1999). “Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children’s Cancer Group Intergroup Study”. J. Clin. Oncol. 17 (7): 2137–43. PMID 10561269.


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Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]

Overview

Sacrococcygel teratoma was first described by Stanley in 1842. In 1863, first successful surgery was performed on sacrococcygeal teratoma by Dr. W. Blizard. Between 1950s and 1960s, germ cell theory of the origin of sacrococcygeal teratoma was introduced.[1]

Historical Perspective

Discovery

  • Teratoma is one of the oldest known tumor.[1]
  • A 4000 year old Babylonian document mentions the first case of sacrococcygeal teratoma.
  • 17th century: French obstetrician reported the first unquestionable teratoma.
  • 1841: Typical sacrococcygeal teratoma was described in detail by Stanley.[1]
  • 1863: The term “teratoma” was introduced by Rudolf Virchow.
  • 1863: Dr. W. Blizard became the first surgeon to successfully operate on sacrococcygeal teratoma.
  • 1884: Virchow referred to external sacrococcygeal teratoma growth as a “soft tail”.
  • 1920: M.A. Perlstein, E. R. Le Count, and J. Bland-Sutton suggested sacrococcygeal teratoma as a suppressed twins or parasitic fetuses since teratomas have many well developed features as finger, nail, hair.
  • 1920s: Paul Kraske perfected the surgical technique for removal of the sacrococcygeal teratoma.
  • 1922: A. A. Law mentioned malignant degeneration of sacrococcygeal teratoma and called for the removal of the tumor.
  • 1950s and 1960s: R.E. Gross, H. W. Clathworthy, I.A. Meeker, and G. V. Brindley hypothesized that teratoma originated from totipotential cells of Henson’s node.
  • 1950s and 1960s: Germ cell theory of the sacrococcygeal teratoma origin was introduced.

References

  1. ↑ 1.0 1.1 1.2 Olson JS. The History of Cancer, An Annotated Bibliography. ABC-CLIO; 1989.

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Classification

Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]

Overview

Sacrococcygeal teratoma is classified into four different categories according to the American academy of pediatrics surgery classification based upon the anatomical location of the tumor. Sub-types of sacrococcygeal teratoma have different staging systems based on the size and growth of the tumor, lymph node involvement, and the presence of metastasis.

Classification

Classification Based Upon Anatomical Location

American academy of pediatrics surgery section system classifies sacrococcygeal teratoma depending on the anatomical location of the tumor. [1][2]

Stage Features

Type I

  • Predominantly external; with minimal pelvic involvement

Type II

  • Predominantly external; with significant intrapelvic growth giving “hour-glass” appearance

Type III

Type IV

  • Entirely pre-sacral; without external growth

Classification Based Upon Pathology

  • Benign : Mature[1]
  • Malignant : Immature

References

  1. ↑ 1.0 1.1 Puri P, Höllwarth ME. Pediatric Surgery, Diagnosis and Management. Springer Science & Business Media; 2009.
  2. ↑ Myers LB, Bulich LA. Anesthesia for Fetal Intervention and Surgery. PMPH-USA; 2005.

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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]

Overview

Sacrococcygeal teratoma originates from the pluripotent cells in primitive knot or Hensen’s node, which is the primary organizer of embryonic development, located on the anterior surface of the sacrum or coccyx by the 2nd or 3rd gestational week. Development of sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and loss of the 6q24-qter and 18q21-qter regions. The pathophysiology of sacrococcygeal teratoma depends on the histological subtype.

Pathophysiology

Pathogenesis

Genetics

  • Development of Sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and loss of the 6q24-qter and 18q21-qter regions.[2][3]

Associated Conditions

Following conditions are associated with sacrococcygeal teratoma:

Gross Pathology

Microscopic Pathology

Sacrococcygeal teratoma can be divided into following two types depending on the microscopic pathology: [7]

Mature Teratoma

Immature Teratoma

References

  1. ↑ Sacrococcygeal teratoma. Hindawi (2015)http://www.hindawi.com/journals/criog/2012/131369/ Accessed on December 15th, 2015
  2. ↑ Harms D, Zahn S, Göbel U, Schneider DT (2006). “Pathology and molecular biology of teratomas in childhood and adolescence”. Klin Padiatr. 218 (6): 296–302. doi:10.1055/s-2006-942271. PMID 17080330.
  3. ↑ Veltman I, Veltman J, Janssen I, Hulsbergen-van de Kaa C, Oosterhuis W, Schneider D, Stoop H, Gillis A, Zahn S, Looijenga L, Göbel U, van Kessel AG (2005). “Identification of recurrent chromosomal aberrations in germ cell tumors of neonates and infants using genomewide array-based comparative genomic hybridization”. Genes Chromosomes Cancer. 43 (4): 367–76. doi:10.1002/gcc.20208. PMID 15880464.
  4. ↑ Sacrococcygel Teratoma. Radiopedia (2015) http://radiopaedia.org/articles/sacrococcygeal-teratoma Accessed on December 15, 2015
  5. ↑ Kristen E. Zhelnin, Grant M. Gebhard, David M. Mirsky, Scott Cn Oliver, Mark A. Lovell, Csaba Galambos, Timothy M. Crombleholme & Emily A. McCourt (2017). “Pediatric Intraocular Immature Teratoma Associated With Sacrococcygeal Teratoma”. Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society. 20 (3): 240–244. doi:10.1177/1093526616686233. PMID 28521629. Unknown parameter |month= ignored (help)
  6. ↑ Tugba Sarac Sivrikoz, Recep Has, Aytul Corbacioglu Esmer, Ibrahim Kalelioglu, Atil Yuksel & Orhun Cig Taskin (2016). “Prenatal diagnosis of tethered spinal cord associated with sacrococcygeal teratoma”. Journal of clinical ultrasound : JCU. 44 (8): 506–509. doi:10.1002/jcu.22344. PMID 26892808. Unknown parameter |month= ignored (help)
  7. ↑ Calaminus G, Schneider DT, Bökkerink JP, Gadner H, Harms D, Willers R, Göbel U (2003). “Prognostic value of tumor size, metastases, extension into bone, and increased tumor marker in children with malignant sacrococcygeal germ cell tumors: a prospective evaluation of 71 patients treated in the German cooperative protocols Maligne Keimzelltumoren (MAKEI) 83/86 and MAKEI 89”. J. Clin. Oncol. 21 (5): 781–6. PMID 12610174.

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Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]

Overview

Development of sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and loss of the 6q24-qter and 18q21-qter regions.

Causes

Development of sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and loss of the 6q24-qter and 18q21-qter regions.[1][2]

References

  1. ↑ Harms D, Zahn S, Göbel U, Schneider DT (2006). “Pathology and molecular biology of teratomas in childhood and adolescence”. Klin Padiatr. 218 (6): 296–302. doi:10.1055/s-2006-942271. PMID 17080330.
  2. ↑ Veltman I, Veltman J, Janssen I, Hulsbergen-van de Kaa C, Oosterhuis W, Schneider D, Stoop H, Gillis A, Zahn S, Looijenga L, Göbel U, van Kessel AG (2005). “Identification of recurrent chromosomal aberrations in germ cell tumors of neonates and infants using genomewide array-based comparative genomic hybridization”. Genes Chromosomes Cancer. 43 (4): 367–76. doi:10.1002/gcc.20208. PMID 15880464.

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Differentiating Sacrococcygeal teratoma from other Diseases

Differentiating Sacrococcygeal teratoma from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]

Overview

Sacrococcygeal teratoma must be differentiated from endodermal sinus tumor, ependymoma, fibromatosis, ganglioneuroma, giant cell tumor of the sacrum, leiomyoma, meningomyelocele, ovarian teratoma, neuroblastoma, retrorectal hamartoma, intracanalicular epidermoid tumor, rhabdomyosarcoma, paraganglioma, dermal sinus stalk ascending towards the conus modullaris, and hydromelia.[1]

Differenting Sacrococcygeal Teratoma from other Diseases


Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Physical examination
Imaging
Vertebra column defect Protrusions, Dimples, Hair patch in the spine Motor and sensory abnormalities X-ray

(PA and Lateral view)

M.R.I.
Sacrococcygeal teratoma +/- ++ +/- Ultrasound
Spina bifida ++ ++ +/- Incomplete union of the posterior elements of vertebral levels MRI –
Terminal myelocystocele +/- Protrusions and dimples +/- Incomplete union of the posterior elements of vertebral levels MRI
Spine segmental dysgenesis + – +/- Defects in the segments of the spine and spinal cord
  • Spinal cord at the level of the abnormality is thinned or even indiscernible
  • a bulky, low-lying cord segment may be present caudad to the focal abnormality in most cases
 MRI
  • The spinal column distal to the abnormality may be partially bifid
    Caudal regression syndrome (sacral agenesis) + +/- +/- Agenesis of the sacrum and lumbar spine
    • Lumbosacral vertebral body dysgenesis/hypogenesis
    • The level of atresia/dysgenesis is usually below L1 and often limited to sacrum
    		 MRI Associated with other developmental malformations (orthopedic, neurological, genito-urinary, gastrointestinal…)
      VACTERL association + +/- +/- Vertebral body dysgenesis/hypogenesis – MRI, Ct scan, X ray VACTERL stands for:
      • Vertebral defects
      • Anal atresia
      • Cardiac defects
      • Tracheo-Esophageal fistula
      • Renal anomalies
      • Limb abnormalities
        Arnold-chiari malformation + + + None MRI
        Syringomyelia + + + MRI
        • Impaired ambulation and loss of penile erection when syrinx involves lumbosacral area
        Scoliosis +/- +/- +/-
        • Abnormality that may be causing the deformity
        • Spinal cord abnormalities may be seen
        		 MRI
        Leg length discrepancy – – – None Orthoroentogram
        • Repeat standing x-rays with the patient standing on a block to account for the discrepancy demonstrates correction of the postural abnormality.

        References

        1. ↑ 1.0 1.1 Myers LB, Bulich LA. Anesthesia for Fetal Intervention and Surgery. PMPH-USA; 2005.
        2. ↑ Calloni SF, Huisman TA, Poretti A, Soares BP (2017). “Back pain and scoliosis in children: When to image, what to consider”. Neuroradiol J. 30 (5): 393–404. doi:10.1177/1971400917697503. PMC 5602330. PMID 28786774.
        3. ↑ Azar, F., Canale, S., Beaty, J. & Campbell, W. (2017). Campbell’s operative orthopaedics. Philadelphia, PA: Elsevier. Page: 1898-2028.
        4. ↑ Meyer SH, Morris GF, Pretorius DH, James HE (March 1998). “Terminal myelocystocele: important differential diagnosis in the prenatal assessment of spina bifida”. J Ultrasound Med. 17 (3): 193–7. PMID 9514174.
        5. ↑ Scott RM, Wolpert SM, Bartoshesky LE, Zimbler S, Karlin L (April 1988). “Segmental spinal dysgenesis”. Neurosurgery. 22 (4): 739–44. PMID 3374785.
        6. ↑ Kremser E, Mitchell GM (February 1971). “Treatment of primary dysmenorrhea with a combined type oral contraceptive–a double blind study”. J Am Coll Health Assoc. 19 (3): 195–6. PMID 4925436.
        7. ↑ Benedetti-Valentino F J, De Feo V, Pistolese GR, Fiorani P (September 1966). “[Nephroptosis and fibromuscular hyperplasia of the tunica media of the renal arteries]”. Minerva Cardioangiol (in Italian). 14 (9): 528–34. PMID 5991006. Vancouver style error: name (help)
        8. ↑ Naccarato R, Sturniolo GC, Martin A, D’Odorico A, Montino C (March 1988). “[Irritable bowel syndrome]”. G Clin Med (in Italian). 69 (3): 163–8. PMID 3169446.
        9. ↑ Sacrococcygel Teratoma. Radiopedia (2015) http://radiopaedia.org/articles/sacrococcygeal-teratoma Accessed on December 15, 2015
        10. ↑ Danzer E, Hubbard AM, Hedrick HL, Johnson MP, Wilson RD, Howell LJ, Flake AW, Adzick NS (2006). “Diagnosis and characterization of fetal sacrococcygeal teratoma with prenatal MRI”. AJR Am J Roentgenol. 187 (4): W350–6. doi:10.2214/AJR.05.0152. PMID 16985105.

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        Epidemiology and Demographics

        Epidemiology and Demographics

        Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]

        Overview

        Sacrococcygeal teratoma is one of the most common congenital tumors. The incidence of sacrococcygeal teratoma, based on multiple studies conducted around the world, ranges from 3.5 – 9.3 per 100, 000 individuals. The prevalence of sacrococcygeal teratoma ranges from 1.4 – 3.3 per 100, 000 individuals. In a retrospective cohort study conducted in southern Sweden from 2000 to 2013, the overall mortality rate of sacrococcygeal teratoma was 11%. Sacrococcygeal teratoma affects neonates. There is no racial predilection to sacrococcygeal teratoma. The female to male ratio is approximately 4 to 1.

        Epidemiology and Demographics

        Incidence

        Below given table compares different studies on the incidence of sacrococcygeal teratoma

        Author Study period Country Case number Incidence
        Derikx 2006[1] 1990 – 1999 The Netherlands 173 3.5/100,000
        *Forrester 2006[2] 1986 – 2001 USA, Hawaii 22 4.29/100,000
        *Swamy 2008[3] 1985 – 2006 England 28 3.6/100,000
        *Pauniaho 2013[4] 1987 – 2008 Finland 124 9.3/100,000
        *Hambraeus 2015 2000 – 2013 Sweden 19 7.1/100,000
        *Included spontaneous abortions, terminations of pregnancy, and stillbirths

        Prevalence

        The prevalence of sacrococcygeal teratoma ranges from 1.4 – 3.3 per 100, 000 individuals.[5]

        Mortality rate

        In a retrospective cohort study conducted in southern Sweden from 2000 to 2013, the overall mortality rate of sacrococcygeal teratoma was 11%.[6]

        Age

        Race

        There is no racial predilection to sacrococcygeal teratoma.

        Gender

        Women are more commonly affected by sacrococcygeal teratoma than men. The female to male ratio is approximately 4 to 1.[9]

        References

        1. ↑ Derikx, J. P. M.; De Backer, A.; van de Schoot, L.; Aronson, D. C.; de Langen, Z. J.; van den Hoonaard, T. L.; Bax, N. M. A.; van der Staak, F.; van Heurn, L. W. E. (2006). “Factors associated with recurrence and metastasis in sacrococcygeal teratoma”. British Journal of Surgery. 93 (12): 1543–1548. doi:10.1002/bjs.5379. ISSN 0007-1323.
        2. ↑ Forrester, Mathias B.; Merz, Ruth D. (2006). “Descriptive epidemiology of teratoma in infants, Hawaii, 1986-2001”. Paediatric and Perinatal Epidemiology. 20 (1): 54–58. doi:10.1111/j.1365-3016.2006.00678.x. ISSN 0269-5022.
        3. ↑ Swamy, Ravi; Embleton, Nicholas; Hale, Juliet (2008). “Sacrococcygeal teratoma over two decades: Birth prevalence, prenatal diagnosis and clinical outcomes”. Prenatal Diagnosis. 28 (11): 1048–1051. doi:10.1002/pd.2122. ISSN 0197-3851.
        4. ↑ Hambraeus, Mette; Arnbjörnsson, Einar; Börjesson, Anna; Salvesen, Kjell; Hagander, Lars (2016). “Sacrococcygeal teratoma: A population-based study of incidence and prenatal prognostic factors”. Journal of Pediatric Surgery. 51 (3): 481–485. doi:10.1016/j.jpedsurg.2015.09.007. ISSN 0022-3468.
        5. ↑ https://rarediseases.org/
        6. ↑ Mette Hambraeus, Einar Arnbjornsson, Anna Borjesson, Kjell Salvesen & Lars Hagander (2016). “Sacrococcygeal teratoma: A population-based study of incidence and prenatal prognostic factors”. Journal of pediatric surgery. 51 (3): 481–485. doi:10.1016/j.jpedsurg.2015.09.007. PMID 26454470. Unknown parameter |month= ignored (help)
        7. ↑ https://rarediseases.org/
        8. ↑ https://rarediseases.org/
        9. ↑ https://rarediseases.org/

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        Risk Factors

        Risk Factors

        Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]

        Overview

        There are no established risk factors for sacrococcygeal teratoma.

        Risk Factors

        There are no established risk factors for sacrococcygeal teratoma.

        References

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        Screening

        Screening

        Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]

        Overview

        According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for sacrococcygeal teratoma.[1]

        Screening

        According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for sacrococcygela teratoma.[1]

        References

        1. ↑ 1.0 1.1 Recommendations. US preventive services task force(2015) http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=sacrococcygeal+teratoma Accessed on November 25, 2015

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        Natural History, Complications and Prognosis

        Natural History, Complications and Prognosis

        Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]

        Overview

        Depending on the extent of the tumor invasion at the time of diagnosis, the prognosis may vary. Most of the sacrococcygeal teratomas are benign in nature and prognosis is generally regarded as good after resection. The sacrococcygeal teratoma with cystic morphology is associated with the most favorable prognosis.

        Natural History, Complications, and Prognosis

        Natural History

        Sacrococcygeal teratomas are the most common type of germ cell tumors (both benign and malignant) diagnosed in neonates, infants, and children younger than 4 years.[1]

        Fetal Sacrococcygeal Teratoma

        Neonatal Sacrococcygeal Teratoma

        • The tumor presents as a protruding mass from the sacral region and is usually mature or immature teratoma.

        Sacrococcygeal Teratoma in Infants and Young Children

        Sacrococcygeal Teratoma in Older Children and Adults

        Complications

        Maternal Complications

        Fetal Complications

        Prognosis

        Poor Prognostic Factors

        Favorable Prognostic Factors

        References

        1. ↑ 1.0 1.1 Sacrococcygeal teratoma. Cancer.gov (2015)(PDQ) Sacrococcygeal Tumors in Children Accessed on November 2th, 2015
        2. ↑ Rescorla FJ: Pediatric germ cell tumors. Semin Surg Oncol 16 (2): 144-58, 1999. PUBMED Abstract
        3. ↑ Finamore PS, Kontopoulos E, Price M, Giannina G, Smulian JC (2007). “Mirror syndrome associated with sacrococcygeal teratoma: a case report”. The Journal of reproductive medicine. 52 (3): 225–7. PMID 17465292.
        4. ↑ Nalbanski B, Markov D, Brankov O (2007). “[Sacrococcygeal teratoma–a case report and literature review]”. Akusherstvo i ginekologii͡a (in Bulgarian). 46 (2): 41–5. PMID 17469451.
        5. ↑ Galili O, Mogilner J (2005). “Type IV sacrococcygeal teratoma causing urinary retention: a rare presentation”. J. Pediatr. Surg. 40 (2): E18–20. doi:10.1016/j.jpedsurg.2004.10.003. PMID 15750911.
        6. ↑ Engelskirchen R, Holschneider AM, Rhein R, Hecker WC, Höpner F (1987). “[Sacral teratomas in childhood. An analysis of long-term results in 87 children]”. Zeitschrift für Kinderchirurgie : organ der Deutschen, der Schweizerischen und der Osterreichischen Gesellschaft für Kinderchirurgie = Surgery in infancy and childhood (in German). 42 (6): 358–61. PMID 3439358.
        7. ↑ Surgical experience and cosmetic outcomes in children with sacrococcygeal teratoma. PubMed (2006) 16373161&dopt=AbstractPlus PubMed Accessed on November 24th, 2015
        8. ↑ Lack EE, Glaun RS, Hefter LG, Seneca RP, Steigman C, Athari F (1993). “Late occurrence of malignancy following resection of a histologically mature sacrococcygeal teratoma. Report of a case and literature review”. Arch. Pathol. Lab. Med. 117 (7): 724–8. PMID 8323438. Synopsis: A 40 year old man has widely metastatic adenocarcinoma arising from the residual coccyx remaining after surgical removal of an apparently benign SCT at age 2 months.
        9. ↑ Partridge EA, Canning D, Long C, Peranteau WH, Hedrick HL, Adzick NS, Flake AW (2014). “Urologic and anorectal complications of sacrococcygeal teratomas: prenatal and postnatal predictors”. J. Pediatr. Surg. 49 (1): 139–42, discussion 142–3. doi:10.1016/j.jpedsurg.2013.09.042. PMID 24439598.
        10. ↑ Sacrococcygeal teratoma. Chop.Edu (2015) http://www.chop.edu/conditions-diseases/sacrococcygeal-teratoma-sct/about#.VnhH0bRYZFI Accessed on November 24th, 2015
        11. ↑ 11.0 11.1 Shue E, Bolouri M, Jelin EB, Vu L, Bratton B, Cedars E, Yoke L, Byrne F, Hirose S, Feldstein V, Miniati D, Lee H (2013). “Tumor metrics and morphology predict poor prognosis in prenatally diagnosed sacrococcygeal teratoma: a 25-year experience at a single institution”. J. Pediatr. Surg. 48 (6): 1225–31. doi:10.1016/j.jpedsurg.2013.03.016. PMID 23845611.
        12. ↑ Sy ED, Filly RA, Cheong ML, Clifton MS, Cortes RA, Ohashi S, Takifuji K, Ventura DE, Grethel EJ, Wagner AJ, Gress EA, Ball RH, Lee H, Harrison MR (2009). “Prognostic role of tumor-head volume ratio in fetal sacrococcygeal teratoma”. Fetal. Diagn. Ther. 26 (2): 75–80. doi:10.1159/000238113. PMID 19752518.
        13. ↑ Seung Mi Lee, Dong Hoon Suh, So Yeon Kim, Min Kyoung Kim, Sohee Oh, Sang Hoon Song, Hyun-Young Kim, Chan-Wook Park, Joong Shin Park & Jong Kwan Jun (2018). “Antenatal Prediction of Neonatal Survival in Sacrococcygeal Teratoma”. Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine. 37 (8): 2003–2009. doi:10.1002/jum.14553. PMID 29399854. Unknown parameter |month= ignored (help)


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        Diagnosis

        Diagnosis

        History & Symptoms | Physical Examination | Laboratory Tests | Pelvis X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

        Treatment

        Treatment

        Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

        Case Studies

        Case Studies

        Case #1

        See also

        See also

        References

        References

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