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Sacrococcygeal Teratoma

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Sacrococcygeal Teratomas
 
Sacrococcygeal teratomas (SCTs) are large tumors of germ cell origin arising from the coccyx. These tumors occur in approximately 1 per 35,000 live births and are often diagnosed prenatally (fetal diagnosis). The surgeons at Pediatric Surgeons of Phoenix manage these often massive tumors in conjunction with neonatologists, pediatric anesthesiologists, and pediatric oncologists. This multidisciplinary approach allows us to offer the families the best care available. A small number of fetuses develop "hydrops fetalis" which may result from the tumor stealing too much of the circulating blood for the baby to do well. There patients may be candidates for fetal intervention since the loss rate in this subset of fetuses is so high. All other infants are treated after birth.
 
The tumors may be benign, malignant, or "immature." All patients with sacrococcygeal teratomas diagnosed prenatally are seen in our office for a detailed discussion of the condition and treatment options. Please see Prenatal Consultations sections for a detailed presentation on Sacrococcygeal Teratomas.
 
 
 
Data source for chance of survival charts: Usui N, Kitano Y, Sago H, KanamoriY, YonedaA, Nakamura T, NosakaS, Saito M, Taguchi T. Outcomes of prenatally diagnosed sacrococcygealteratomas: the results of a Japanese nationwide survey. J PediatrSurg. 2012 Mar;47(3):441-7.
 
Warning: Extremely graphic content related to the type of surgery, organs, procedures or trauma depicted
 
 
External appearance of large Sacrococcygeal Teratoma prior to surgery.
 
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Pre-Op photo of an infant sacrococcygeal teratoma
 
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MRI showing intra abdominal extent of large sacrococcygeal teratoma
 
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Post-Op appearance after resection of massive tumor
 
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Pediatric Surgeons of Phoenix - Phoenix, Arizona

Sacrococcygeal Teratomas
David M Notrica, MD FACS FAAP
Pediatric Surgeons of Phoenix
Phoenix, Arizona
 
 
Basics
“A teratoma is a true tumor or neoplasm composed of multiple tissues of kinds foreign to the parts in which it arises.”
Where do they come from? Several theories abound. One suggests wandering Germ Cells are left behind during the migration of embryonic germ cells from yolk sac to gonad during fetal development.
The tumor must (by definition) contain elements derived from more than one embryonic germ layer, must be foreign to the anatomic site and cannot have resulted from metaplasia. It must also show a high degree of disorganization
 
The tumor may be either: (1) Benign (2) Malignant (3) Benign with Immature Elements (kind of benign, but worrisome).
Benign Teratomas contain only recognizable adult mature tissues such as epithelium, bone, cartilage, epithelium
Malignant Teratomas contain a malignant tumor among the benign parts. Examples include Embryonal carcinoma, Yolk Sac Tumors, endodermal sinus tumor, Choriocarcinoma, Germinomas, seminomas, dysgerminomas, Mixtures of Malignant Cells
Immature Teratoma contain cells that are not malignant, but are fetal and should not be present in a newborn. These immature elements may or may not represent cancer.
 
“Because benign and malignant, mature and immature elements may exist side by side in the same teratoma, malignant areas may escape being sectioned.”
 
Treatment
-Surgical Excision in All Cases
-Sacral Incision
-Inverted -V Incision
-May Need Abdominal Incision if tumor extends considerably to the pelvis and retroperitoneum
-Malignant invasion may preclude complete excision in occasional patients

 

Sacrococcygeal Teratomas
Technical Aspects
-Blood Supply comes from the Middle Sacral Artery
-Consider ligation of middle sacral artery through abdomen if high output heart failure
-Coccyx should be removed with the tumor
-Reconstruction of the flap is important to avoid a flat shapeless bottom

 

After the Surgery
-Benign (majority of SCTs)
-No Further Treatment Necessary
-Perineal and Rectal Exams at 2, 4, and then 6 month intervals for at least 3 years
-Any Recurrences should be promptly re-excised
-Rarely, a Malignant Recurrence may occur after a benign primary

 

Postoperative Therapy for malignant tumors, chemotherapy is recommended

Chemotherapy may deter recurrence

Recurrent malignant tumors are at risk for death from tumor progression
 
For immature, but benign tissue, the decision to treat is more difficult
-Alpha-fetoprotein may be of predictive value
 
Recommendation:
Many oncologists recommend chemotherapy for patients with Immature Elements and elevated alpha-Fetoprotein or other markers. Consult with an experienced pediatric oncologist who is a member of the Children's Oncology Group and works at a COG-affiliated hospital.
 

 

 
See www.curesearch.org and search "teratoma" for even more info.

 

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