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Mature cystic sacrococcygeal teratoma in a child- a case report.

INTRODUCTION: Sacrococcygeal Teratoma (SCT) is the most common congenital neoplasm (1). SCT have tissue derived from endoderm, ectoderm and mesoderm. But embryonic origin is still uncertain. They are believed to originate from early gestation from totipotential cells of Hansen's node, a remnant of these primitive streaks in the coccygeal origin (2). SCT is most the common tumour seen in newborns and children. It accounts for 1 in 35,000 live births, although it is also seen in adults (3). These tumours are predominantly seen in females in a ratio of 4:1, and approximately 80% of affected infants are females (4). They are clinically presented more commonly as large midline exophytic mass located posterior to the sacrum. The anus is usually displaced anteriorly. The differential diagnosis includes lipoma, meningocele, dermoid cyst and a host of other tumour malformations (5). Teratomas form and grow during intrauterine life, and can become quite large with the growth of most sacrococcygeal teratomas paralleling the growth of fetus itself. Inspite of being histologically benign, these tumours may recur either as benign or malignant tumour (6,7).

CASE REPORT: A 2 1/2 years female baby attended surgical OPD of AMC Hospital, Bijapur presenting with swelling in the sacrococcygeal region since birth. The baby was full term, normal delivered at home by traditional birth attenders and routine antenatal checkup were not done. The child was apparently normal, and there was no H/O any other birth complications. Initially the swelling was small in size and as the age advanced the mass increased gradually to present size. On examination the mass measured 8 X 5 cm, soft, globular and slightly movable (Fig 1), no tenderness. The skin over the swelling was normal, and transillumination test and cough impulse was negative. There was no H/O similar complaint in the family. Systemic examinations were normal. The growth and development of baby was within normal range. Routine investigations were normal.

Then to evaluate, first FNAC was done, which revealed features of benign SCT. Depending upon FNAC diagnosis the patient was prepared for elective surgery. The tumour was excised enblock via the sacral approach. The pelvic component of tumor was excised while the rectum and bladder were carefully protected, and a coccygectomy was performed and specimen was sent for histopathology.

PATHOLOGY: Smears studied showed moderate cellular material composed predominantly of anucleated squamous cells, arranged in loose cohesive clusters. Nucleated squamous cells, ciliated cells, detached ciliary tufts and mucinous cells were also seen. The background showed thick material, inflammatory cells & respiratory epithelial cells.

IMPRESSION: Suggestive of Benign cystic teratoma--Sacrococcygeal Region.

GROSS: (Fig: 1) The specimen was a soft tissue mass, along with coccyx measuring 7 X 6 X 4 cm, soft to firm in consistency and well capsulated. Cut section showed unilocular cyst. Wall of the cyst was thin, opaque and grey white in colour. Cystic areas filled with cheesy sebaceous material, along with areas of calcification were seen.

HISTOPATHOLOGY: (fig: 6-9) Multiple sections studied under Hematoxylin & Eosin (H&E) stain showed thick cystic wall lined by stratified squamous epithelium, with sebaceous glands, acrine glands, fatty tissue and muscle tissue, hyaline cartilage, and bone, other underlying adnexal structure. These findings were consistent with benign SCT. Hence the FNAC report was consistent with histopathology. No malignant tissue was observed.

IMPRESSION: Consistent with Benign Cystic teratoma--Sacrococcyx region

DISCUSSION: Teratoma accounts for 32% to 66% of non gonadal germ cell tumours. It is the most common tumour of newborn occurring in about 1 in 35,000 births (8). Generally teratomas are more common in females than males. There are three main histological types of teratomas which include mature, immature and monodermal (highly specialized) teratomas (9). In our case, it was (benign) mature SCT, in a female baby. In this case initial diagnosis was made accurately based on FNAC as benign SCT. Abdominal and pelvic radiography and USG both revealed cystic mass in front of the sacrum. Subsequently diagnosis was confirmed by histology as benign SCT, thereby corroborating the FNAC diagnosis. The other masses presenting in the sacrococcygeal region may mimic SCT clinically. In our hospital this had been mistakenly diagnosed clinically as anterior meningocele, extraspinal glioma, or lipoma of buttock. Other similar conditions that may be confused include: abscess, neurofibroma, cystic hygroma, and chordoma (10). These conditions were excluded by FNAC.

In our case the presenting complaint was mass in the sacrococcygeal region, but on digital examination there was presence of a bulge in the presacral region. There was no history of urinary retention, constipation, loss of appetite or failure to thrive.

Based on FNAC diagnosis of benign SCT, investigations like USG and Computerized Tomography scan (CT scan) can be used to know the extent of tumor in pelvis and abdomen and also its anatomic relation to adjacent structures. However CT scan was not done for this patient because of poor socioeconomic condition.

In most of the cases of benign SCT surgery is the principal mode of treatment. In children every attempt should be made to remove the mass completely along with the coccyx. Higher incidence of recurrence rates of up to 37% were registered if the coccyx had not been excised during the initial procedure (11, 12,13).

Sacrococcygeal Teratoma, although histologically benign, has an alarming

capacity to recur both as a benign or malignant tumor even after successful removal. Patient should be followed up for at least first 3 years after the surgery as recurrence is more likely (13). Follow up includes physical examination, alpha-fetoprotein and USG for all cases.

CONCLUSION: Sacrococcygeal Teratoma constitutes a significant neonatal surgical problem. It requires an astute and experienced approach with regards to the clinical evaluation and correlation of the same with investigations like FNAC, histopathology and USG.

We conclude that FNAC plays an important role in the diagnosis of SCT as an initial step, which further must be confirmed with other diagnostic modalities.

REFERENCES:

(1.) Heena-Mckenney A, Harison MR, Bratton B, Farrel J, Zaloudek C. Congenital teratoma: a clinicopathologic study of 22 fetal and neonatal tumours. Amj Surg pathol. 2005 Jan 29(1): 29-38.

(2.) Kelsar PJ, Buk JL, Suarez ES: Germ cell tumours of the sacrococcygeal region: radiographic correlation. Radiographic 14: 607-622, 1994).

(3.) Killen Da, Jackson LM (1964) sacrococcygeal teratoma in adults, Archives of Surgery 88(3): 425-428.

(4.) Winderl LM, Silverman RK: Prenatal identification of a complete cystic internal sacrococcygeal teratoma (type IV) ultrasound obset gynecol 9: 425-428, 1997.

(5.) Michael A. Skinner. Germ cell tumours. Surgery of infants and childrens; Scientific principle and practice lippincott. Raven publishers Philadelphia.

(6.) Izant RJ Jr, Filston HC. sacrococcygeal teratomas. Analysis of forty three cases. Amj Surg 1975; 130(5): 617-621.

(7.) Mozan F. Talbert JL. Congenital anorectal malformation. Harbingers of sacrococcygeal teratomas. Arch Surg. 1985 JUL; 120(7); 586-859.

(8.) Michael A. skinner. Germ cell tumours. Surgery of infants and practice Lippincott-Raven publishers, Philadelphia.

(9.) Cum Christopher P: From Germ cell tumours. In Robbins and Cotran Pathologic basis of disease, 7th edition. Edited by: kumar V, Abdul Ka, Nelson F. USA Elsevier saunder's publishers; 2005:1099-1101.

(10.) Killings. Sacrococcygeal teratoma. Can J Surg 1969: 12: 22-26,

(11.) Gross RW, Clatulorthy HWJR, Meeker IA JR. Sacrococcygeal teratomas in infants and childrens'; a report of 40 cases. Surg Gynecol Obset,. 1951 Mar; 92(3); 341-354.

(12.) Waldhausen JA, Koman, Vellois F, Battersby JS. Sacrococcygeal teratoma. Surgery. 1963 Dec; 54: 933-949.

(13.) Mathour GH, Wolley MM, Trivedi SN, Landing BH. Sacrococcygeal teratoma: a 33-year experience. J Pediatr Surg. 1975 Apr; 10 (2): 183-188.

Mohammed Arifulla. K [1], Vivek. P. Honakeri [2], Sayeed. M. Yendigeri [3], B. B. Sajjanar [4]. Nasheen Fathima [5].

[1.] Assistant Professor, Department of Pathology, Al-Ameen Medical College and Hospital, Athani Road, Bijapur

[2.] Assistant Professor, Department of Pathology, Al-Ameen Medical College and Hospital, Athani Road, Bijapur

[3.] Associate Professor, Department of Pathology, Al-Ameen Medical College and Hospital, Athani Road, Bijapur

[4.] Professor, Department of Pathology, Al-Ameen Medical College and Hospital, Athani Road, Bijapur

[5.] Assistant Professor, Department of Pathology, Al-Ameen Medical College and Hospital, Athani Road, Bijapur

CORRESPONDING AUTHOR

Dr. Mohammed Arifulla. K, Assistant Professor, Department of Pathology, Al-Ameen Medical College, Bijapur.

Email-arif10mohdk@gmail.com
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Title Annotation:CASE REPORT
Author:Arifulla. K., Mohammed; Honakeri, Vivek. P.; Yendigeri, Sayeed. M.; Sajjanar, B.B.; Fathima, Nasheen
Publication:Journal of Evolution of Medical and Dental Sciences
Article Type:Clinical report
Date:Jun 24, 2013
Words:1352
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