|Year : 2015 | Volume
| Issue : 4 | Page : 364-366
Rare presentation of sacrococcygeal teratoma in a prepubertal girl
Minakshi Bhosale, Dasmit Singh, Amit Gupta
Department of Pediatric Surgery, B J Government Medical College, Pune, Maharashtra, India
|Date of Web Publication||18-Sep-2015|
G/101, Sudarshan Apartments, Behind Spencer's Daily, Karvenagar, Pune - 411 052, Maharashtra
Source of Support: Nil., Conflict of Interest: NO
Eighty-five percent of sacrococcygeal teratomas, the most common neonatal tumor present in infancy with externally visible swelling. Because of associated risk of malignancy, which increases exponentially with age, majority of the tumors undergo operative intervention. Hence, presentation in an adult that too of a benign mass is extremely rare, with less than 10 reported cases in world literature. We report case of a 9-year-old girl who presented with swelling in the gluteal cleft. Excision of the swelling along with the coccyx was done. Benign cystic sacrococcygeal teratoma was diagnosed on histopathological evaluation of the excised tumor mass. This case is reported for a very unusual presentation of sacrococcygeal teratoma and also brings forth the difficulties encountered during excision of the tumor.
Keywords: Benign, delayed presentation, gluteal cleft, prepubertal girl, sacrococcygeal teratoma
|How to cite this article:|
Bhosale M, Singh D, Gupta A. Rare presentation of sacrococcygeal teratoma in a prepubertal girl. CHRISMED J Health Res 2015;2:364-6
|How to cite this URL:|
Bhosale M, Singh D, Gupta A. Rare presentation of sacrococcygeal teratoma in a prepubertal girl. CHRISMED J Health Res [serial online] 2015 [cited 2019 Sep 23];2:364-6. Available from: http://www.cjhr.org/text.asp?2015/2/4/364/165734
| Introduction|| |
Sacrococcygeal teratomas (SCTs) are tumors derived from more than one embryonic germ cell layer and are usually diagnosed in infancy. The estimated incidence of presacral teratomas in children ranges from 1 in 30,000 to 1 in 43,000 live births. Our patient presented at the age of 9-years with a benign mass. This kind of presentation is extremely rare.
| Case Report|| |
A 9-year-old girl presented with cystic swelling in the gluteal cleft since 2–3 months. There was recent increase in size of the swelling. There was no history of constipation, urinary frequency or lower limb weakness. The girl had not attained menarche.
On external examination, a mass of 5 cm × 5 cm × 4 cm was found in the gluteal cleft overlying the coccyx [Figure 1]a and [Figure 1]b. It was cystic in consistency. The overlying skin was shiny and contained hair follicles. The tumor was not palpable by abdominal palpation. However, on per rectal examination, a firm cystic mass of about 8 cm × 4 cm size was palpable. There was no evidence of any other anomalies. Her gait and lower limb power were normal.
Hematological investigations, liver function tests, and coagulation profile were normal. Serum α-feto protein (AFP) and β-human chorionic gonadotropin levels were normal. The mass was nonradio-opaque on plain X-rays. Magnetic resonance imaging scan revealed a well-defined, multiloculated, heterogenous, predominantly hyperintense lesion of 11 cm × 5.6 cm × 4.5 cm in presacral region on T2 weighed images (isointense on T1) with fat component of 5.6 cm × 1 cm within [Figure 2]. The mass was abutting the urinary bladder anteriosuperiorly and the rectum anterioinferiorly. Superiorly, it was extending up to thefirst sacral vertebra. However, the fat planes were well maintained. Both ovaries, liver, kidneys and the lung fields were normal. Complete excision of the mass, along with excision of the coccyx was planned. It was difficult to approach the tumor via Pfannenstiel incision. Hence, she was catheterized, placed prone as for PSARP and further excision was completed. The tumor was gently shaved off from the rectum (identified by a Hegar dilator within its lumen) [Figure 3]. The tumor had infiltrated into the subcutaneous fat as well. This dissection was difficult and time-consuming. En mass excision of the tumor with coccygectomy was done. A small bore suction drain was kept in situ. The excised tumor weighed 300 g. It was 7 cm × 3 cm × 2 cm in size, filled with whitish pultaceous material. It contained small cysts of 2 cm × 1.5 cm × 1.5 cm and 1 cm × 1 cm in size within. It also had a ball of hair, muscle, respiratory epithelium, glandular intestine like material infiltrated by lymphocytes and polymorphonuclear cells, suggestive of a benign cystic SCT [Figure 4].
|Figure 2: Magnetic resonance imaging showing large tumor in the presacral region|
Click here to view
|Figure 3: Intra-operative photograph showing near complete separation of the tumor. Also seen is Hegar's dilator within the rectum|
Click here to view
|Figure 4: Histopathology slide showing muscle, respiratory epithelium, glandular intestine infiltrated by lymphocytes and polymorphonuclear cells (HP, ×—40)|
Click here to view
Postoperatively, to prevent fecal contamination of the wound, the child was kept nil per orally for 5 days. Since postoperative day six, oral feeds were started and gradually advanced. However she was given constipating diet. Drain was removed on postoperative day six. Postoperative course was uneventful, except for minor superficial wound infection. However, wound healing occurred eventually. Presently, at a follow-up of 2 years, the child is totally asymptomatic. There is no radiological evidence of tumor recurrence. Her follow-up AFP levels are normal. She is on the regular follow-up to assess tumor recurrence in the long run.
| Discussion|| |
Most SCTs are discovered in the newborn period as an obvious mass. They are either both cystic and solid, predominantly cystic or rarely solid. Over 50% have calcification or ossification. Female:Male ratio is 4:1. Though, multiple theories have been postulated about the histiogenesis of teratomas, the most widely accepted concept is the "germ cell theory". Many well-differentiated teratomas may include derivatives from all three embryonic germ cell layers.
The tumor is known to have high malignant potential. The risk of developing malignancy being 30% at 7 days, 50% at 1-month and 70% at 2 years, early surgical excision is therefore advocated. Additional coccygectomy forms integral part of the surgery. Complete surgical excision (with coccygectomy) is adequate if the tumor is benign; however, chemotherapy and radiotherapy are indicated in malignant cases and in cases of recurrence after previous excision.
About 70–80% of the teratomas in literature have been reported in girls., Schropp et al. have reported Female:Male ratio of 4.2:1 in their experience of SCTs over four decades. Syzllo et al. have reported SCT in a 26-year-old patient, presenting with abdominal pain. This patient had a 5 cm sized presacral cyst diagnosed on radiological evaluation. It was removed laparoscopically and turned out to be a mature teratoma on histopathological evaluation. Aniba et al. have reported benign SCT in an 8-year-old girl, in whom the tumor presented as progressive sacral tumefaction, since the age of 2 years. Bull et al. have reported mature presacral teratoma in an adult male, who presented with recurrent infected pilonidal cysts.
Our patient, a 9-year-old girl, probably had a small dormant tumor in situ for a long period. However, because of small size, it went unnoticed. At the age of 9 years, she presented with overt sacrococcygeal mass. Gradual increase in the size of the swelling had brought the child to medical attention. There are very few reports of delayed presentation of the tumor that too of a benign teratoma, in a child aged 9 years. This report highlights the importance of thorough preoperative evaluation of any mass in the vicinity of sacrum keeping in mind the possibility of SCT. Careful complete excision of the tumor along with all invading tails should be done. Excision of coccyx (radical resection) should be integral part of the surgery since majority of the recurrences have been reported in cases of spillage of tumor and absence of removal of coccyx.,
| References|| |
Bull J Jr, Yeh KA, McDonnell D, Caudell P, Davis J. Mature presacral teratoma in an adult male: A case report. Am Surg 1999;65:586-91.
Keslar PJ, Buck JL, Suarez ES. Germ cell tumors of the sacrococcygeal region: Radiologic-pathologic correlation. Radiographics 1994;14:607-20.
Tapper D, Sawin R. Teratomas and other germ cell tumors. In: O'Neill JA, Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG, editors. Pediatric Surgery. 5th
ed. St. Louis: Mosby; 1998. p. 447-60.
Shonubi AM, Musa AA, Akiode O, Salami BA, Kingu HJ, Adnan SM. Mature sacrococcygeal teratoma: A case report and literature review. West Afr J Med 2004;23:176-9.
Abubakar AM, Nggada HA, Chinda JY. Sacrococcygeal teratoma in Northeastern Nigeria: 18-years experience. Pediatr Surg Int 2005;21:645-8.
Schropp KP, Lobe TE, Rao B, Mutabagani K, Kay GA, Gilchrist BF, et al.
Sacrococcygeal teratoma: The experience of four decades. J Pediatr Surg 1992;27:1075-8.
Szyllo K, Lesnik N. Sacrococcygeal teratoma-Case report and review of the literature. Am J Case Rep 2013;14:1-5.
Aniba K, Ghannane H, Lmejjati M, Ouali M, Jalal H, Ousehal A, et al.
Benign sacrococcygeal teratoma in a child: A case report with a review of the literature. Arch Pediatr 2009;16:1467-9.
De Backer A, Madern GC, Hakvoort-Cammel FG, Haentjens P, Oosterhuis JW, Hazebroek FW. Study of the factors associated with recurrence in children with sacrococcygeal teratoma. J Pediatr Surg 2006;41:173-81.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]