sign in sign up
<<
Home , Carcinoma, Neoplasm, Sacrococcygeal teratoma

Published online: 2021-06-23

Original Article

An Epidemiological Review of Sacrococcygeal over Five Years in a Tertiary Care Hospital

Abstract Mukhopadhyay Background: Teratoma refers to containing at least two germ layers derivatives Bedabrata, foreign to the site of origin. is most common congenital tumor commonly Das Chhanda1, present as an exophytic mass of varying size at . Aims and Objectives: (1) The aim is to 1 find age, sex, and clinical presentation of cases of sacrococcygeal teratoma. (2) Application of Sengupta Moumita , 2 American Academy of Pediatrics’ surgical section classification and histological in all cases. Saha Ashis Kumar , Materials and Methods: A retrospective, observational study was conducted in the Department Mukhopadhyay of in collaboration with the Department of from February 2009 to Madhumita1, January 2014. A total 13 cases of sacrococcygeal teratoma were included in the present study. The Mukhopadhyay records of these patients were reviewed, and clinical profiles were noted. An average 12 slides 3 were examined in each case to evaluate histological type and grades. Results: A retrospective Biswanath study was conducted including thirteen cases of histologically confirmed sacrococcygeal teratoma Department of Biochemistry, over 5 years period. Male to female ratio was 1:2. As per as age distribution is concerned, 3 Banaras Hindu University, Varanasi, Uttar Pradesh, neonates (23%) presented with sacrococcygeal mass. Associated congenital malformation was seen 1Department of Pathology, in 2 cases (15.4%). According to the American Academy of Pediatrics (Altman’s) classification, 23% Institute of Post Graduate of cases were Type I, 31% of cases patients Type II, 31% of cases Type III and 15% were for Medical Education and Type IV. The significant presacral component was noted in all malignant tumors. Conclusion: Two Research, 2Department of clinical patterns were observed in sacrococcygeal teratoma related to the age of presentation. As Surgery, College of sacrococcygeal teratoma has potential to become malignant, meticulous search for the malignant and Sagore Dutta Hospital, component is required for histopathological categorization. 3Department of Pediatric Surgery, Nil Ratan Sircar Keywords: Neuroepithelium, sacrococcygeal teratoma, Totipotential cell Medical College and Hospital, Kolkata, West Bengal, India

Introduction are diagnosed by prenatal ultrasound. Association with other congenital anomaly are composed of tissues derived such as myelomeningocele and vertebral from , , and . malformation were reported.[5] Sacrococcygeal teratomas are developed from the totipotential cells of primitive knot As per as the is concerned, which is a remnant of the primitive streak in sacrococcygeal teratomas are classified into the coccygeal region.[1] With the incidence of mature, immature and malignant category.[6] 1/35000–1/40000 live birth, sacrococcygeal Biological behaviors are affected by age teratoma is considered as the most common and sex. Mature teratomas are common in in the neonatal period and neonates.[7] infancy.[2] Sacrococcygeal teratoma shows Clinical and pathological variables of cases female preponderance with male to female of sacrococcygeal teratoma were evaluated ratio 1:4.[3] Address for correspondence: in our study. Dr. Das Chhanda, Clinical presentation varies depending 31 Eastern Park, First Road, on the age of the patient and location Materials and Methods Santoshpur, Kolkata ‑ 700 075, of the tumor. In the neonatal period, West Bengal, India. A retrospective, observational study was E‑mail: [email protected] tumor presents as exophytic mass at the conducted in the Department of Pathology sacral region with occasional surface in collaboration with the Department of ulceration and hemorrhage, but younger Pediatric Surgery from February 2009 Access this article online child presents with mass effect due to January 2014. A total 13 cases of Website: www.ijmpo.org to its enormous size and proximity to sacrococcygeal teratoma were included intra‑abdominal organs.[4] Fetal tumors DOI: 10.4103/ijmpo.ijmpo_239_14 in the study. The records of these patients Quick Response Code:

This is an open access article distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 3.0 How to cite this article: Bedabrata M, Chhanda D, License, which allows others to remix, tweak, and build upon the Moumita S, Kumar SA, Madhumita M, Biswanath M. work non‑commercially, as long as the author is credited and the An epidemiological review of sacrococcygeal teratoma new creations are licensed under the identical terms. over five years in a tertiary care hospital. Indian J Med For reprints contact: [email protected] Paediatr Oncol 2018;39:4-7.

4 © 2018 Indian Journal of Medical and Paediatric | Published by Wolters Kluwer ‑ Medknow Bedabrata, et al.: An epidemiological review of sacrococcygeal teratoma with pediatric sacrococcygeal teratoma were reviewed for According to the American Academy of Pediatrics age, sex distribution, clinical presentation, preoperative (Altman’s) classification, 23% of cases were Type I, 31% investigation, mode of treatment, and follow‑up. of cases patients Type II, 31% of cases Type III and 15% Pre‑operative hematological and radiological evaluation were for Type IV. The significant presacral component was were noted in all cases. noted in all malignant tumors. Depending on the radiological findings, the extent of Majority of cases on histopathological evaluation were the tumors was analyzed and categorized according to documented as mature teratoma, about 53.4% (7/13) the American Academy of Pediatrics’ surgical section followed by immature teratoma with 30.8% (4/13). Grossly, classification which includes four categories: mature teratoma was predominantly cystic (75%), whereas 1. Type I: Predominantly external with minimal presacral the remaining 25% were solid and cystic. The immature component; teratoma was predominantly solid. Immature teratoma 2. Type II: Present externally but with significant showed immature neuroepithelial in the form of intrapelvic extension neuroepithelial rosette. Two cases of were 3. Type III: Apparent externally but predominantly a reported in our study. Malignant yolk sac component was pelvic mass extending into the noted in both cases [Table 1]. 4. Type IV: Presacral with no external presentation.[8] Mature sacrococcygeal teratoma showed the components Both size and appearance of the tumors were recorded derived from all the three germ cell layers with complete thoroughly. Depending on the size, tumors were differentiation. Ectodermal and endoderm derived tissues classified into small (2–5 cm diameter); moderate were seen in all the cases of mature teratoma. Neural (5–10 cm diameter) and large (>10 cm diameter) elements, glial tissue, and choroid plexus were seen in tumors. Hematoxylin and eosin stained slides were 69.2%. Mesodermal elements such as adipose tissue, reviewed in each case, and additional sections were , smooth, and skeletal muscle were noted. from taken from paraffin block if needed. An average Organoid elements such as pancreatic and salivary 12 slides were examined in each case. Depending on tissue in 15.4% [Table 2]. histological findings, tumors were classified broadly into mature, immature, and malignant tumors. Tumor Discussion showing differentiated tissues and tumor having Sacrococcygeal teratoma at birth usually presents as a immature neuroepithelium were considered to be mature visible mass in the sacrococcygeal region. Most of the and immature teratoma accordingly. Malignant tumors neonates do not have any symptoms. Some may have showed yolk sac tumor, , or embryonal cardiac failure, disseminated intravascular coagulation, [9] along with differentiated tissues. and rupture or bleeding within the tumor. Those neonates Histological grading was done: having with an intrapelvic component may i. Grade 0: Tumor shows only mature tissue present with urinary obstruction. Children present with ii. Grade 1: Tumor shows rare foci of immature constipation, , an or [2] tissue <1 lpf/slide symptoms of malignancy, like failure to thrive. The iii. Grade 2: Tumor shows moderate quantities of immature present study mainly had neonates who presented with a tissues, 1–3 lpf/slide iv. Grade 3: Tumor shows large quantities of immature Table 1: Histopathological diagnosis and grades of tissue. >3 lpf/slide; with or without malignant yolk sac sacrococcygeal teratomas elements. Tumor type Tumor grade Number of cases Mature 0 7 Result Immature 1 1 In the present study, a total of 13 cases diagnosed as 2 3 congenital sacrococcygeal teratoma were evaluated. Malignant 3 2 The tumors presenting to us were mainly in the age Total 13 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: Table 2: Components of sacrococcygeal teratomas female ratio of 1:2. Component Total cases it is present (%) Ectodermal elements 13 (100) The most common clinical presentation of sacrococcygeal Endodermal elements 13 (100) teratoma in our study was sacrococcygeal mass. All the Neural and CNS elements 9 (69.2) cases of mature teratoma presented with a prominent Mesodermal elements 8 (61.5) mass at sacrococcyx. 2 cases showed associated Organoid elements 2 (15.4) malformations (anorectal malformations). CNS – Central

Indian Journal of Medical and Paediatric Oncology | Volume 39 | Issue 1 | January-March 2018 5 Bedabrata, et al.: An epidemiological review of sacrococcygeal teratoma

Table 3: Comparison of different studies with the present study Parameter William et al. Valdiserri et al. Khanna et al. Ramani et al. Our study Total cases 103 68 41 25 13 Age incidence (<2 month), (%) 79 (76) 54 (79) 23 (56) 18 (72) 9 (69) Male:female 1:4 1:4 2:1 1:4 1:2 Clinical presentation Sacrococcygeal Sacrococcygeal Sacrococcygeal Sacrococcygeal Sacrococcygeal mass mass mass mass mass Tumour maturity (%) Mature: 73 (70) Mature: 51 (75) Mature: 31 (76) Mature: 20 (80) Mature: 7 (53.4) Immature: 30 (29) Immature: 8 (12) Immature: 5 (12) Immature: 5 (20) Immature: 4 (30.8) Malignant: 9 (13) Malignant: 5 (12) Malignant: 2 (15.4) mass at sacrococcyx. The older children presented with sacrococcygeal teratoma were classified as Grade 0. Among mass, , and constipation. 8 immature teratomas, 2 were Grade 2 and 6 were Grade 3.[6] In the study, the major components of mature teratoma were The grading of sacrococcygeal teratoma is done according to the presence of immature tissues. Grading ectodermal and endodermal tissues followed by mesodermal of sacrococcygeal teratoma is done similar to the grading and organoid elements. All the immature teratomas in our of an ovarian teratoma.[6] According to this grading, the study were composed of neuroepithelial elements. Two cases are classified into Grade 0, 1, 2 and 3. Grading of cases of malignancy have been reported in our study where, sacrococcygeal teratoma does not correlate directly with yolk sac tumor is the malignant component. Some say size prognosis, unlike that of ovarian teratoma where grading is independent of biological behavior [Table 3]. has direct correlation with prognosis.[6] However, larger tumors are more likely to have In our study, the most common age of presentation was immature histology and may have greater intraoperative [16] above 2 months of age. The male: female ratio was 1:2, complications. Mature teratoma should not recur if indicating female preponderance, as in other studies.[6,8,10‑12] complete surgical excision and are done. Persistently, elevated alpha fetoprotein levels may indicate Clinical manifestations of sacrococcygeal teratoma in a residual or recurrent tumor. our series included sacrococcygeal mass in the majority of cases. Immature teratoma presented with a visible to Conclusion ill‑defined mass at sacrococcyx. Our findings were similar to other studies.[6,8,13] In ours study, mass with congenital Sacrococcygeal teratoma is the most common congenital anomalies seen in 15%. This was 25% in a study by neoplasm presenting at birth. Sacrococcygeal teratomas Billman et al. Children <2 month age have a better when diagnosed at birth usually reveal fully differentiated prognosis. Malignant risk rises with age (Herman et al., tissues and are benign. Meticulous search for the 2002). Rescorla et al. and Gabra et al. reported that mostly, immature or malignant component should be instituted children >1 year age had malignant tumors.[3] In our study, as it helps in therapeutic decisions. Immature tissue in 16% were malignant and they all presented after 1 month. sacrococcygeal teratoma is predominantly neuroepithelial. In our study, the incidence of Altman’s types II and III Histopathological grading of immature tissue in were maximum. Amel Hashish et al. studied (Type I >Type sacrococcygeal teratomas does not correlate directly with II) in their study.[14] prognosis. Early diagnosis influences clinical decision and management, providing a better outcome. Continued A study by Bilik et al. reported that grossly 40% of the follow‑up (with alpha‑fetoprotein and ) to rule out sacrococcygeal teratoma were solid, 20% were cystic, recurrence. and 40% were mixed.[15] Another series by Khanna et al., reported that mature teratoma were predominantly cystic More effective antenatal screening, for early diagnosis and and immature teratoma was partly solid and partly cystic.[13] management, to reduce the risk of malignant transformation, According to a review, majority of benign sacrococcygeal hence better prognosis. teratoma were cystic in nature.[15] In our study, majority Financial support and sponsorship of the mature teratoma were predominantly cystic while immature teratoma was mostly solid. Nil. On , the majority of sacrococcygeal teratoma Conflicts of interest are mature followed by immature teratoma. In our There are no conflicts of interest. study, Grade 0 or mature teratomas are most commonly seen (53%). References [6,11,16‑20] Our findings confirm the results by other studies. 1. Izant RJ Jr., Filston HC. Sacrococcygeal teratomas. Analysis of According to Valdiserri and Yunis, 51 cases of mature forty‑three cases. Am J Surg 1975;130:617‑21.

6 Indian Journal of Medical and Paediatric Oncology | Volume 39 | Issue 1 | January-March 2018 Bedabrata, et al.: An epidemiological review of sacrococcygeal teratoma

2. Lakhoo K. Neonatal teratomas. Early Hum Dev 2010;86:643‑7. Ramesh Reddy K, Sreenivasa Reddy P, et al. In children-A 3. Rescorla FJ, Sawin RS, Coran AG, Dillon PW, Azizkhan RG. pathologists overview. J Evol Med Dent Sci 2013;2:5932-42. Long‑term outcome for and children with sacrococcygeal 13. Khanna S, Arya NC, Singhal GD. Sacrococcygeal tumours in teratoma: A report from the childrens group. J Pediatr children. J Postgrad Med 1987;33:109‑14. Surg 1998;33:171‑6. 14. Amel Hashish A, Fayad H, Ashraf El‑attar A, Moursi Radwan M, 4. Rescorla FJ. Pediatric germ cell tumors. Semin Surg Oncol Ismael K, Mohamed HM, Elhalaby E. Sacrococcygeal teratoma: 1999;16:144‑58. Management and outcomes. Ann Pediatr Surg 2009;5:119‑25. 5. Ein SH, Adeyemi SD, Mancer K. Benign sacrococcygeal 15. Bilik R, Shandling B, Pope M, Thorner P, Weitzman S, Ein SH, teratomas in infants and children: A 25 year review. Ann Surg et al. Malignant benign neonatal sacrococcygeal teratoma. 1980;191:382‑4. J Pediatr Surg 1993;28:1158‑60. 6. Valdiserri RO, Yunis EJ. Sacrococcygeal teratomas: A review of 16. Noseworthy J, Lack EE, Kozakewich HP, Vawter GF, Welch KJ. 68 cases. Cancer 1981;48:217‑21. Sacrococcygeal germ cell tumors in childhood: An updated 7. Keslar PJ, Buck JL, Suarez ES. Germ cell tumors of the experience with 118 patients. J Pediatr Surg 1981;16:358‑64. sacrococcygeal region: Radiologic‑pathologic correlation. 17. Elesha SO, Aina AO, Odunjo EO. Sacrococcygeal teratomas Radiographics 1994;14:607‑20. in Lagos, Nigeria: Relationship of age, sex, clinical type 8. Altman RP, Randolph JG, Lilly JR. Sacrococcygeal teratoma: and histopathology to prognosis in 30 cases. East Afr Med J American Academy of Pediatrics Surgical Section Survey‑1973. 1989;66:685‑92. J Pediatr Surg 1974;9:389‑98. 18. Havránek P, Hedlund H, Rubenson A, Güth D, Husberg M, 9. Williams AO, Lagundoye SB, Bankole MA. Sacrococcygeal Frykberg T, et al. Sacrococcygeal teratoma in Sweden between 1978 teratoma in Nigerian children. Arch Dis Child 1970;45:110‑3. and 1989: Long‑term functional results. J Pediatr Surg 1992;27:916‑8. 10. Holterman AX, Filiatrault D, Lallier M, Youssef S. The natural 19. Chirdan LB, Uba AF, Pam SD, Edino ST, Mandong BM, history of sacrococcygeal teratomas diagnosed through routine Chirdan OO, et al. Sacrococcygeal teratoma: Clinical obstetric sonogram: A single institution experience. J Pediatr characteristics and long‑term outcome in Nigerian children. Ann Surg 1998;33:899‑903. Afr Med 2009;8:105‑9. 11. Aly KA, Shoier M, Badrawy T. Sacrococcygeal teratoma: 20. Ho KO, Soundappan SV, Walker K, Badawi N. Sacrococcygeal A neonatal surgical problem. Ann Pediatr Surg 2006;2:106‑11. teratoma: The 13‑year experience of a tertiary paediatric centre. 12. Ramani M, Husain KW, Geetha K, Radhika Krishna OH, J Paediatr Child Health 2011;47:287‑91.

Indian Journal of Medical and Paediatric Oncology | Volume 39 | Issue 1 | January-March 2018 7