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Indian Journal of Medical and Paediatric Oncology
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ORIGINAL ARTICLE
Year : 2018  |  Volume : 39  |  Issue : 1  |  Page : 4-7  

An epidemiological review of sacrococcygeal teratoma over five years in a tertiary care hospital


1 Department of Biochemistry, Banaras Hindu University, Varanasi, Uttar Pradesh, India
2 Department of Pathology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
3 Department of Surgery, College of Medicine and Sagore Dutta Hospital, Kolkata, West Bengal, India
4 Department of Pediatric Surgery, Nil Ratan Sircar Medical College and Hospital, Kolkata, West Bengal, India

Date of Web Publication29-Jan-2018

Correspondence Address:
Dr. Das Chhanda
31 Eastern Park, First Road, Santoshpur, Kolkata - 700 075, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmpo.ijmpo_239_14

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  Abstract 

Background: Teratoma refers to neoplasm containing at least two germ cell layers derivatives foreign to the site of origin. Sacrococcygeal teratoma is most common congenital tumor commonly present as an exophytic mass of varying size at coccyx. Aims and Objectives: (1) The aim is to find age, sex, and clinical presentation of cases of sacrococcygeal teratoma. (2) Application of American Academy of Pediatrics' surgical section classification and histological grading in all cases. Materials and Methods: A retrospective, observational study was conducted in the Department of Pathology in collaboration with the Department of Pediatric Surgery from February 2009 to January 2014. A total 13 cases of sacrococcygeal teratoma were included in the present study. The records of these patients were reviewed, and clinical profiles were noted. An average 12 slides were examined in each case to evaluate histological type and grades. Results: A retrospective study was conducted including thirteen cases of histologically confirmed sacrococcygeal teratoma over 5 years period. Male to female ratio was 1:2. As per as age distribution is concerned, 3 neonates (23%) presented with sacrococcygeal mass. Associated congenital malformation was seen in 2 cases (15.4%). According to the American Academy of Pediatrics (Altman's) classification, 23% of cases were Type I, 31% of cases patients Type II, 31% of cases Type III and 15% were for Type IV. The significant presacral component was noted in all malignant tumors. Conclusion: Two clinical patterns were observed in sacrococcygeal teratoma related to the age of presentation. As sacrococcygeal teratoma has potential to become malignant, meticulous search for the malignant component is required for histopathological categorization.

Keywords: Neuroepithelium, sacrococcygeal teratoma, Totipotential cell


How to cite this article:
Bedabrata M, Chhanda D, Moumita S, Kumar SA, Madhumita M, Biswanath M. An epidemiological review of sacrococcygeal teratoma over five years in a tertiary care hospital. Indian J Med Paediatr Oncol 2018;39:4-7

How to cite this URL:
Bedabrata M, Chhanda D, Moumita S, Kumar SA, Madhumita M, Biswanath M. An epidemiological review of sacrococcygeal teratoma over five years in a tertiary care hospital. Indian J Med Paediatr Oncol [serial online] 2018 [cited 2018 Dec 15];39:4-7. Available from: http://www.ijmpo.org/text.asp?2018/39/1/4/224019




  Introduction Top


Teratomas are composed of tissues derived from ectoderm, mesoderm, and endoderm. Sacrococcygeal teratomas are developed from the totipotential cells of primitive knot which is a remnant of the primitive streak in the coccygeal region.[1] With the incidence of 1/35000–1/40000 live birth, sacrococcygeal teratoma is considered as the most common germ cell tumor in the neonatal period and infancy.[2] Sacrococcygeal teratoma shows female preponderance with male to female ratio 1:4.[3]

Clinical presentation varies depending on the age of the patient and location of the tumor. In the neonatal period, tumor presents as exophytic mass at the sacral region with occasional surface ulceration and hemorrhage, but younger child presents with mass effect due to its enormous size and proximity to intra-abdominal organs.[4] Fetal tumors are diagnosed by prenatal ultrasound. Association with other congenital anomaly such as myelomeningocele and vertebral malformation were reported.[5]

As per as the histology is concerned, sacrococcygeal teratomas are classified into mature, immature and malignant category.[6] Biological behaviors are affected by age and sex. Mature teratomas are common in neonates.[7]

Clinical and pathological variables of cases of sacrococcygeal teratoma were evaluated in our study.


  Materials and Methods Top


A retrospective, observational study was conducted in the Department of Pathology in collaboration with the Department of Pediatric Surgery from February 2009 to January 2014. A total 13 cases of sacrococcygeal teratoma were included in the study. The records of these patients with pediatric sacrococcygeal teratoma were reviewed for age, sex distribution, clinical presentation, preoperative investigation, mode of treatment, and follow-up. Pre-operative hematological and radiological evaluation were noted in all cases.

Depending on the radiological findings, the extent of the tumors was analyzed and categorized according to the American Academy of Pediatrics' surgical section classification which includes four categories:

  1. Type I: Predominantly external with minimal presacral component;
  2. Type II: Present externally but with significant intrapelvic extension
  3. Type III: Apparent externally but predominantly a pelvic mass extending into the abdomen
  4. Type IV: Presacral with no external presentation.[8]


Both size and appearance of the tumors were recorded thoroughly. Depending on the size, tumors were classified into small (2–5 cm diameter); moderate (5–10 cm diameter) and large (>10 cm diameter) tumors. Hematoxylin and eosin stained slides were reviewed in each case, and additional sections were from taken from paraffin block if needed. An average 12 slides were examined in each case. Depending on histological findings, tumors were classified broadly into mature, immature, and malignant tumors. Tumor showing differentiated tissues and tumor having immature neuroepithelium were considered to be mature and immature teratoma accordingly. Malignant tumors showed yolk sac tumor, choriocarcinoma, or embryonal carcinoma along with differentiated tissues.[9]

Histological grading was done:

  1. Grade 0: Tumor shows only mature tissue
  2. Grade 1: Tumor shows rare foci of immature tissue <1 lpf/slide
  3. Grade 2: Tumor shows moderate quantities of immature tissues, 1–3 lpf/slide
  4. Grade 3: Tumor shows large quantities of immature tissue. >3 lpf/slide; with or without malignant yolk sac elements.



  Result Top


In the present study, a total of 13 cases diagnosed as congenital sacrococcygeal teratoma were evaluated. The tumors presenting to us were mainly in the age group >2 months, with 10 out of 13 cases presenting in that age group. There were 9 female and 4 male children. Gender incidence showed female preponderance with male: female ratio of 1:2.

The most common clinical presentation of sacrococcygeal teratoma in our study was sacrococcygeal mass. All the cases of mature teratoma presented with a prominent mass at sacrococcyx. 2 cases showed associated malformations (anorectal malformations).

According to the American Academy of Pediatrics (Altman's) classification, 23% of cases were Type I, 31% of cases patients Type II, 31% of cases Type III and 15% were for Type IV. The significant presacral component was noted in all malignant tumors.

Majority of cases on histopathological evaluation were documented as mature teratoma, about 53.4% (7/13) followed by immature teratoma with 30.8% (4/13). Grossly, mature teratoma was predominantly cystic (75%), whereas the remaining 25% were solid and cystic. The immature teratoma was predominantly solid. Immature teratoma showed immature neuroepithelial tissue in the form of neuroepithelial rosette. Two cases of malignancy were reported in our study. Malignant yolk sac component was noted in both cases [Table 1].
Table 1: Histopathological diagnosis and grades of sacrococcygeal teratomas

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Mature sacrococcygeal teratoma showed the components derived from all the three germ cell layers with complete differentiation. Ectodermal and endoderm derived tissues were seen in all the cases of mature teratoma. Neural elements, glial tissue, and choroid plexus were seen in 69.2%. Mesodermal elements such as adipose tissue, cartilage, smooth, and skeletal muscle were noted. Organoid elements such as pancreatic and salivary gland tissue in 15.4% [Table 2].
Table 2: Components of sacrococcygeal teratomas

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  Discussion Top


Sacrococcygeal teratoma at birth usually presents as a visible mass in the sacrococcygeal region. Most of the neonates do not have any symptoms. Some may have cardiac failure, disseminated intravascular coagulation, and rupture or bleeding within the tumor. Those neonates having lesions with an intrapelvic component may present with urinary obstruction. Children present with constipation, urinary retention, an abdominal mass or symptoms of malignancy, like failure to thrive.[2] The present study mainly had neonates who presented with a mass at sacrococcyx. The older children presented with mass, pain, and constipation.

The grading of sacrococcygeal teratoma is done according to the presence of immature tissues. Grading of sacrococcygeal teratoma is done similar to the grading of an ovarian teratoma.[6] According to this grading, the cases are classified into Grade 0, 1, 2 and 3. Grading of sacrococcygeal teratoma does not correlate directly with prognosis, unlike that of ovarian teratoma where grading has direct correlation with prognosis.[6]

In our study, the most common age of presentation was above 2 months of age. The male: female ratio was 1:2, indicating female preponderance, as in other studies.[6],[8],[10],[11],[12]

Clinical manifestations of sacrococcygeal teratoma in our series included sacrococcygeal mass in the majority of cases. Immature teratoma presented with a visible to ill-defined mass at sacrococcyx. Our findings were similar to other studies.[6],[8],[13] In ours study, mass with congenital anomalies seen in 15%. This was 25% in a study by Billman et al. Children <2 month age have a better prognosis. Malignant risk rises with age (Herman et al., 2002). Rescorla et al. and Gabra et al. reported that mostly, children >1 year age had malignant tumors.[3] In our study, 16% were malignant and they all presented after 1 month. In our study, the incidence of Altman's types II and III were maximum. Amel Hashish et al. studied (Type I >Type II) in their study.[14]

A study by Bilik et al. reported that grossly 40% of the sacrococcygeal teratoma were solid, 20% were cystic, and 40% were mixed.[15] Another series by Khanna et al., reported that mature teratoma were predominantly cystic and immature teratoma was partly solid and partly cystic.[13] According to a review, majority of benign sacrococcygeal teratoma were cystic in nature.[15] In our study, majority of the mature teratoma were predominantly cystic while immature teratoma was mostly solid.

On histopathology, the majority of sacrococcygeal teratoma are mature followed by immature teratoma. In our study, Grade 0 or mature teratomas are most commonly seen (53%).

Our findings confirm the results by other studies.[6],[11],[16],[17],[18],[19],[20] According to Valdiserri and Yunis, 51 cases of mature sacrococcygeal teratoma were classified as Grade 0. Among 8 immature teratomas, 2 were Grade 2 and 6 were Grade 3.[6] In the study, the major components of mature teratoma were ectodermal and endodermal tissues followed by mesodermal and organoid elements. All the immature teratomas in our study were composed of neuroepithelial elements. Two cases of malignancy have been reported in our study where, yolk sac tumor is the malignant component. Some say size is independent of biological behavior [Table 3].
Table 3: Comparison of different studies with the present study

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However, larger tumors are more likely to have immature histology and may have greater intraoperative complications.[16] Mature teratoma should not recur if complete surgical excision and coccygectomy are done. Persistently, elevated alpha fetoprotein levels may indicate a residual or recurrent tumor.


  Conclusion Top


Sacrococcygeal teratoma is the most common congenital neoplasm presenting at birth. Sacrococcygeal teratomas when diagnosed at birth usually reveal fully differentiated tissues and are benign. Meticulous search for the immature or malignant component should be instituted as it helps in therapeutic decisions. Immature tissue in sacrococcygeal teratoma is predominantly neuroepithelial. Histopathological grading of immature tissue in sacrococcygeal teratomas does not correlate directly with prognosis. Early diagnosis influences clinical decision and management, providing a better outcome. Continued follow-up (with alpha-fetoprotein and radiology) to rule out recurrence.

More effective antenatal screening, for early diagnosis and management, to reduce the risk of malignant transformation, hence better prognosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Izant RJ Jr., Filston HC. Sacrococcygeal teratomas. Analysis of forty-three cases. Am J Surg 1975;130:617-21.  Back to cited text no. 1
    
2.
Lakhoo K. Neonatal teratomas. Early Hum Dev 2010;86:643-7.  Back to cited text no. 2
[PUBMED]    
3.
Rescorla FJ, Sawin RS, Coran AG, Dillon PW, Azizkhan RG. Long-term outcome for infants and children with sacrococcygeal teratoma: A report from the childrens cancer group. J Pediatr Surg 1998;33:171-6.  Back to cited text no. 3
[PUBMED]    
4.
Rescorla FJ. Pediatric germ cell tumors. Semin Surg Oncol 1999;16:144-58.  Back to cited text no. 4
[PUBMED]    
5.
Ein SH, Adeyemi SD, Mancer K. Benign sacrococcygeal teratomas in infants and children: A 25 year review. Ann Surg 1980;191:382-4.  Back to cited text no. 5
[PUBMED]    
6.
Valdiserri RO, Yunis EJ. Sacrococcygeal teratomas: A review of 68 cases. Cancer 1981;48:217-21.  Back to cited text no. 6
[PUBMED]    
7.
Keslar PJ, Buck JL, Suarez ES. Germ cell tumors of the sacrococcygeal region: Radiologic-pathologic correlation. Radiographics 1994;14:607-20.  Back to cited text no. 7
[PUBMED]    
8.
Altman RP, Randolph JG, Lilly JR. Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg 1974;9:389-98.  Back to cited text no. 8
[PUBMED]    
9.
Williams AO, Lagundoye SB, Bankole MA. Sacrococcygeal teratoma in Nigerian children. Arch Dis Child 1970;45:110-3.  Back to cited text no. 9
[PUBMED]    
10.
Holterman AX, Filiatrault D, Lallier M, Youssef S. The natural history of sacrococcygeal teratomas diagnosed through routine obstetric sonogram: A single institution experience. J Pediatr Surg 1998;33:899-903.  Back to cited text no. 10
[PUBMED]    
11.
Aly KA, Shoier M, Badrawy T. Sacrococcygeal teratoma: A neonatal surgical problem. Ann Pediatr Surg 2006;2:106-11.  Back to cited text no. 11
    
12.
Ramani M, Husain KW, Geetha K, Radhika Krishna OH, Ramesh Reddy K, Sreenivasa Reddy P, et al. In children-A pathologists overview. J Evol Med Dent Sci 2013;2:5932-42.  Back to cited text no. 12
    
13.
Khanna S, Arya NC, Singhal GD. Sacrococcygeal tumours in children. J Postgrad Med 1987;33:109-14.  Back to cited text no. 13
[PUBMED]  [Full text]  
14.
Amel Hashish A, Fayad H, Ashraf El-attar A, Moursi Radwan M, Ismael K, Mohamed HM, Elhalaby E. Sacrococcygeal teratoma: Management and outcomes. Ann Pediatr Surg 2009;5:119-25.  Back to cited text no. 14
    
15.
Bilik R, Shandling B, Pope M, Thorner P, Weitzman S, Ein SH, et al. Malignant benign neonatal sacrococcygeal teratoma. J Pediatr Surg 1993;28:1158-60.  Back to cited text no. 15
    
16.
Noseworthy J, Lack EE, Kozakewich HP, Vawter GF, Welch KJ. Sacrococcygeal germ cell tumors in childhood: An updated experience with 118 patients. J Pediatr Surg 1981;16:358-64.  Back to cited text no. 16
[PUBMED]    
17.
Elesha SO, Aina AO, Odunjo EO. Sacrococcygeal teratomas in Lagos, Nigeria: Relationship of age, sex, clinical type and histopathology to prognosis in 30 cases. East Afr Med J 1989;66:685-92.  Back to cited text no. 17
[PUBMED]    
18.
Havránek P, Hedlund H, Rubenson A, Güth D, Husberg M, Frykberg T, et al. Sacrococcygeal teratoma in Sweden between 1978 and 1989: Long-term functional results. J Pediatr Surg 1992;27:916-8.  Back to cited text no. 18
    
19.
Chirdan LB, Uba AF, Pam SD, Edino ST, Mandong BM, Chirdan OO, et al. Sacrococcygeal teratoma: Clinical characteristics and long-term outcome in Nigerian children. Ann Afr Med 2009;8:105-9.  Back to cited text no. 19
[PUBMED]  [Full text]  
20.
Ho KO, Soundappan SV, Walker K, Badawi N. Sacrococcygeal teratoma: The 13-year experience of a tertiary paediatric centre. J Paediatr Child Health 2011;47:287-91.  Back to cited text no. 20
[PUBMED]    



 
 
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