Sacrococcygeal teratoma pathophysiology

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

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.

• 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.^[1]

• 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]

Following conditions are associated with sacrococcygeal teratoma:

• Myelomeningocoele^[4]
• Vertebral anomalies
• Bladder outlet obstruction
• Hydronephrosis
• Rectal stenosis or atresia
• Intraocular teratoma^[5]
• Hydrops fetalis
• Cardiomegaly due to vascular shunting and high output cardiac failure
• Tethered spinal cord^[6]

• 
  Gross Image of Sacrococcygeal teratoma [Case courtesy of Dr Saeed Soltany Hosn, Radiopaedia.org, rID: 33220]
• 
  Gross Image of Sacrococcygeal teratoma [Image courtesy of Sinha S, Sarin YK, Deshpande VP]
• 
  Gross Image of Excised Sacrococcygeal teratoma[Image courtesy of Sinha S, Sarin YK, Deshpande VP]

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

• Benign
• Consists of fully differentiated somatic tissue

• Malignant
• Consists of small fraction of incompletely differentiated tissue
• They have elevated tumor markers including yolk sac component secreting alpha fetoprotein or primitive neuroectodermal tumor (PNET).

Template:WH Template:WS

Template:WikiDoc Sources