Sacrococcygeal Teratoma : Experience with 36 Patients in a Teritiary Care Hospital

Introduction: Sacrococcygeal teratomas (SCT) are the most common solid tumours in the neonatal period, originate from embryonic totipotent cells. Most of them are benign however some that present later in life and have major intra-pelvic component have greater malignant potential. Complete surgical excision provides an excellent prognosis. The aim of this study was to describe the clinical profile, age of presentation, sex distribution, diagnostic evaluation and management of children with a histological confirmed diagnosis of sacrococcygeal teratoma. Material and Methods : Patients with histological diagnosis of sacrococcygeal teratoma during a period from January 2011 to December 2012 were included in this retrospective study. Data collected included age of presentation, sex distribution, symptoms, associated anomalies, diagnostic evaluation, operative approach technique employed, tumour histology, operative complications and outcome. Results : Thirty six patients with diagnosis of sacrococcygeal teratoma were included in the study. Of these children, 58.33% were females and 41.66% were males. The median age at presentation was four days. 69.44% of patients presented during the neonatal period. 77.77% of patients presented with sacrococcygeal mass followed by lower abdominal mass in 11.11%. The tumour was resected by sacral approach in 27 patients and abdomino sacral approach in nine patients. Tumour was resectable in 34 patients and two had incomplete resection. Follow up duration varied from six months to 24 months. Conclusion : Sacrococcygeal teratomas represent a group of benign and malignant lesions of children. Neonates present with benign disease and aggressive lesions are seen as age progresses. Overall survival of SCT is high. J Nepal Paediatr Soc 2015;35(1):89-93


Introduction
S acrococcygeal teratomas (SCT) are the most common solid tumours in the neonatal period with a reported incidence of 1:35,000 to 1:40,000 live births 1,2 .These tumours arise from the Henson's node, believed to originate from embryonic to potent primi ve cells 3 .It is more common in females with male: female ra o of 1: 3-4 4 .These tumours are composed of two or three germ cell layers and can present in varying sizes and shapes 5 .Most germ cell tumours in neonates are benign and are divided into mature and immature teratomas 6 .However, fewer tumours that present later in life and have major intra-pelvic component have greater malignant poten al. Complete surgical excision of these tumours provides an excellent prognosis 7 .
The aim of this study was to study the clinical profi le, age of presenta on, sex distribu on, diagnos c evalua on and management of children admi ed in our hospital with a histological confi rmed diagnosis of sacrococcygeal teratoma.

Material and Methods
A total of 36 pa ents were received during the period from January 2011 to December 2012 in the Department of paediatric surgery, Sawai Man Singh medical college and hospital, Jaipur, India.The medical records of the pa ents regarding age of presenta on, sex distribu on, presen ng symptoms, associated anomalies, various blood and radiological inves ga ons done, type of opera ve approach and tumour histopathology and complica ons associated with opera ve were reviewed retrospec vely.Only histopathological confi rmed cases of sacrococcygeal teratomas were included in the study.Lumbosacral meningomyelocoeles who were ini ally misdiagnosed as teratomas were not included in the study.

Results
Thirty six pa ents with histopathological diagnosis of sacrococcygeal teratoma were included in the retrospec ve study.The study included 21 (58.33%)females and 15 (41.66%) males.The median age at presenta on was 4 days, with ranges between day one to fi ve years.25 (69.44%)pa ents presented during the neonatal period and 9 (25%) pa ents presented during the period of one year excluding neonatal period.Only two (5%) pa ents presented a er the period of one year (Table 1).Out of 36 pa ents, 19 pa ents had undergone antenatal ultrasonography and only six pa ents were diagnosed with large sacrococcygeal mass.Clinical presenta ons of all 36 pa ents included in the study are described (Table 2).Most of the pa ents presented with sacrococcygeal mass (77.77%) followed by lower abdominal mass (11.11%), urinary tract and gastrointes nal tract obstruc on.Only three pa ents presented with associated anomalies.One pa ent had polydactyly, other had anorectal malforma on & clubfoot and the 3 rd one had occipital encephalocoele.All pa ents were inves gated by baseline blood inves ga ons, X-ray and Ultrasonography abdomen and pelvis, Serum Alfa-feto protein (AFP) contrast enhanced computed tomography (CECT) abdomen and pelvis preopera vely.Serum Alfa-fetoprotein is normally elevated in the fi rst eight months, therefore such high levels should be interpreted with cau on in infants.Beyond this age, serum Alfa feto-protein has signifi cant oncologic relevance.Serum Alfa-feto protein was raised in four pa ents of 11, 12, 18, 24 months age group.These levels normalized six to nine months a er surgery.Altman's classifi ca on in 36 pa ents was type I(n=15), type II(n=12), type III(n=5) and type IV(n=4).The tumour was resected by sacral approach in 27 pa ents and abdomino-sacral approach was employed in nine pa ents.The tumour was resectable in 34 pa ents and had two pa ents had incomplete resec on, biopsy followed by chemotherapy.Standard cispla n based chemotherapy was given in our pa ents.Coccygectomy was done in all pa ents.The mass was purely cys c in 11 pa ents and having heterogenous consistency in 18 pa ents and only seven pa ents had pure solid component.Gonzalez-Crussi grading of teratoma in our 36 pa ents is as, grade 0 (n=27), grade I (n=4), grade II (n=3) and grade III (n=2).In a follow up dura on, that varied from 6 month to 24 months, two pa ents had recurrence who were managed by re-do surgery and chemotherapy.Two pa ents of grade 3 immature teratoma were also placed on cispla n-based chemotherapy.Peri-opera ve complica ons occurred in seven pa ents that included surgical site infec on in two pa ents, one of which required sigmoid colostomy and other was managed with regular dressings and prone posi on.Massive Intraopera ve bleeding occurred in one pa ent, managed by liga on of sacral vessels and blood transfusion.Recurrence was seen in two pa ents (Gonzalez-Crussi grade 3) and managed by re-excision and chemotherapy.One pa ent presented with postopera ve faecal soiling which improved over a period of 3 month follow up.One pa ent died of sep caemia on 10 th post-opera ve day. to the 33 pa ents and 38 pa ents in 25 years Canadian and 18 years Nigerian study respec vely 10,11 .In recent years, antenatal detec on of the Sacrococcygeal mass has increased manifold in western world due to be er imaging modali es and with ter ary care services at doorsteps, antenatal detec on has enabled be er outcome.But in developing countries, poverty, poor perinatal care, low educa on jus fy the delayed ming of presenta on of disease.Antenatal ultrasonography (USG) has increased the detec on of disease and has enabled possible antenatal interven on and planned caesarean deliveries resul ng in be er outcome.More than half of our pa ents presented during neonatal period.In our series of 36 pa ents, 11 pa ents had antenatal USG done and only fi ve pa ents could be detected as having sacrococcygeal mass.19 pa ents had undergone antenatal ultrasonography and only six pa ents were diagnosed with large sacrococcygeal mass.69.44% of our pa ents presented during neonatal period, probably because of external lesions and rest presented later.Timing of presenta on also relates to the low socio-economic status and scarcity of ter ary care ins tu ons in developing countries like India.
As per Rescorla et al, all pa ents presen ng a er the age of one year had malignant tumours 12 .Gabra et al also reported that 71% of their pa ents presen ng beyond neonatal period were malignant 13.These interpreta ons are consistent with our results, two pa ents who presented beyond 1 year of age had >50% immature ssue (malignant component) on histology.Altman classifi ca on divides SCT according to its anatomical loca on and intrapelvic extension 1 .As per published reports, Altman type I,II,III,IV is seen in 47%, 35%, 8% and 10% cases respec vely.In our series of 36 pa ents, incidence of four types was 41.66%, 33.33%, 13.88% and 11.11% respec vely, which is comparable to the American Academy of Pediatrics data.Commonest mode of presenta on is sacral mass followed by pelvic tumour.Other less common modes of presenta ons are bowel obstruc on, urinary obstruc on, and ulcera on over the swelling.Ascra and Holder in 1965 reported the associa on of sacral mass with sacral defect and anal stenosis 14,15 .Associa on of SCT along with anorectal malforma on and sacral agenesis is called as Currarino triad 16 .In our study, one pa ent had Currarino triad with bladder and bowel symptoms.
The diagnos c evalua on of SCT involves, blood inves ga ons, USG abdomen, pelvis and sacral region.Serum alfa-fetoprotein is an important tumour marker used to monitor recurrence of disease and malignant

Discussion
Sacrococcygeal teratomas (SCT) are well known germ cell tumours aff ec ng neonates, infants and children.These tumours have bimodal age of distribu on; those presen ng in the neonatal period predominantly having benign course and older infants and children with intrapelvic tumour having malignant histology 8 .The disease is predominantly more common in females worldwide, but in our series, there is only slight increased female preponderance.In Wakhlu et al study from India shows equal sex distribu on 4,9 .The incidence of SCT is 1:35,000-40,000 live births 2 .In our series, 36 pa ents were seen over a period of two years.The incidence is much more as compared change.Although computed tomography and Magne c resonance imaging are considered be er imaging modali es, as they give be er tumour characteriza on and its rela onship to adjacent structures.Current management strategy in the western world is antenatal diagnosis with a planned caesarean delivery and immediate excision of the tumour 17,18 .
The treatment of SCT involves complete surgical excision with coccygectomy as in our cases.In case of benign lesions, excision is suffi cient.Failure to remove the coccyx results in recurrence as high as 37% 19,20 .In malignant lesions (immature components), cispla num-based chemotherapy and radiotherapy is indicated 21 .Surgical approach depends upon the size and anatomical loca on of the tumour.Altman type I and II can be removed by sacral route and type III and IV lesions can be approached by combined abdominosacral route.In our series type I,II and even some cases of type III were approached by sacral route and type IV were operated by combined abdomino-sacral approach.
Postopera ve complica ons a er SCT excision include surgical site infec ons (SSI), haemorrhage, rectal dysfunc on, urinary incon nence.SSIs are considered to be due to proximity to anal opening and con nuous faecal soiling of wound.Ein SH et al reports an infec on rate of 18% in surgical wound 10 .In another study by Wakhlu et al surgical site infec on was seen in only two pa ents 9 .Our series also reported SSI in two pa ents, haemorrhage in one pa ent, recurrence in two pa ents (grade 3), faecal soiling in one pa ent which improved with follow up and one pa ent expired of sep caemia.We feel that long term follow up is required to deal with bowel and urinary symptoms and delayed complica ons.
Teratomas are graded according to the Gonzalez-Crussi classifi ca on on the basis of percentage of immature and neuroepithelial element 22 .It includes grade I, II, III having, <10%, 10%-50%, >50% immature component respec vely.Our results reveal 75% of pa ents had benign disease (no immature element) and only 5.5% pa ents revealed grade III disease.

Conclusion
We conclude that most cases of sacrococcygeal teratomas present in neonatal period as sacrococcygeal mass.Neonates present with benign disease and aggressive malignant lesions are seen in age group beyond the neonatal period and young children.Diagnos c evalua on includes clinical examina on, USG and CT abdomen and pelvis and serum Alfa-fetoprotein levels.Complete removal of the tumour along with coccygectomy and avoidance of intra-opera ve spillage is associated with excellent prognosis.Late age of presenta on and presence of immature components are associated with poor prognosis.Post opera ve complica ons a er tumour excision are known.Compliance with follow up visits requires good parental counselling and super speciality services at the doorsteps.

Fig 1 :
Fig 1: showing large sacrococcygeal mass in a neonate

Table 1 :
Showing age of presenta on of sacrococcygeal teratomas

Table 2 :
Showing clinical manifesta ons

Table 3 :
Altman classifi ca on of Sacrococcygeal teratoma in 36 pa ents