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

Introduction

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

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:

• Grade 0: Tumor shows only mature tissue

• Grade 1: Tumor shows rare foci of immature tissue <1>

• Grade 2: Tumor shows moderate quantities of immature tissues, 1?3 lpf/slide

• Grade 3: Tumor shows large quantities of immature tissue. >3 lpf/slide; with or without malignant yolk sac elements.

  
  

Result

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].

References

1. zant RJJr, Filston HC.?Sacrococcygeal teratomas. Analysis of forty-three cases. Am J Surg 1975; 130: 617-21
2. akhoo K.?Neonatal teratomas. Early Hum Dev 2010; 86: 643-7
3. escorla 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
4. escorla FJ.?Pediatric germ cell tumors. Semin Surg Oncol 1999; 16: 144-58
5. in SH, Adeyemi SD, Mancer K.?Benign sacrococcygeal teratomas in infants and children: A 25 year review. Ann Surg 1980; 191: 382-4
6. aldiserri RO, Yunis EJ.?Sacrococcygeal teratomas: A review of 68 cases. Cancer 1981; 48: 217-21
7. eslar PJ, Buck JL, Suarez ES.?Germ cell tumors of the sacrococcygeal region: Radiologic-pathologic correlation. Radiographics 1994; 14: 607-20
8. ltman RP, Randolph JG, Lilly JR.?Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg 1974 1974; 9: 389-98
9. illiams AO, Lagundoye SB, Bankole MA.?Sacrococcygeal teratoma in Nigerian children. Arch Dis Child 1970; 45: 110-3
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
11. Aly KA, Shoier M, Badrawy T.?Sacrococcygeal teratoma: A neonatal surgical problem. Ann Pediatr Surg 2006; 2: 106-11
12. Ramani M, Husain KW, Geetha K, Radhika KrishnaOH, Ramesh ReddyK, Sreenivasa ReddyP.?et al.?In children-A pathologists overview. J Evol Med Dent Sci 2013; 2: 5932-42
13. Khanna S, Arya NC, Singhal GD.?Sacrococcygeal tumours in children. J Postgrad Med 1987; 33: 109-14
14. Amel HashishA, Fayad H, Ashraf El-attarA, Moursi RadwanM, Ismael K, Mohamed HM, Elhalaby E.?Sacrococcygeal teratoma: Management and outcomes. Ann Pediatr Surg 2009; 5: 119-25
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
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
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: 684-92
18. Havr?nek P, Hedlund H, Rubenson A, G?th 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
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
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