Evaluation of Different Presentations of

The New Iraqi Journal of Medicine

Case Series

Evaluation of Different Presentations of Sacrococcygeal Teratoma in Single Pediatric Surgery Unit: A Case Series Mohammed Joudi Aboud

Abstract Introduction: Sacrococcygeal teratoma (SCT) is a well-known tumor of the newborn which carries an excellent prognosis provided adequate surgical treatment. The aim of this study were to the study the age distribution at presentation, the sex ratio, variation of the morphological presentations, and the incidence of postoperative complications, recurrence rate and the malignancy at the time of diagnosis. Material and Method: Retrospective analysis of clinical and epidemiological data of seven cases of sacrococcygeal teratomas operated in Pediatric Surgery Unit at the Maternity and Child Teaching Hospital / Al-Qadisiya -Iraq from the period 1 st of January 2005 to 31 st of December 2008 the data used being extracted of the surgical protocols and histopathological exams. Results: Of 7 patients, 6 (86 %) were female. Sacrococcygeal teratoma contained only cystic component in four patients (57 %), both cystic and solid components in one patient (14 %) and predominantly solid components in two patients (29 %). Four patients (57 %) were delivered by caesarean section. The follow up ranges between 3 months and 2 years in only 5 patients. Follow-up sonogram and magnetic resonance imaging MRI was done for all patients. Five patients (71 %) were diagnosed and treated before the age of two months. One patient (14 %) died postoperatively and one (14 %) developed a recurrence. Conclusion: We suggest a national survey for large group of patients with sacrococcygeal teratoma in other centers for reliable identification of different presentation and risk factors. Whenever histopathological examination of the resected specimen shows mature teratoma, a careful search for areas of malignancy in the specimen should be performed especially in delayed presentation (later than the age of 2 months). Keywords: The N Iraqi J Med August 2010; 6(2): 86-91

1 INTRODUCTION

eratoma is a type of neoplasm. The word teratoma comes from Greek and means roughly "monstrous tumor". Sacrococcygeal teratoma (SCT) is a well-known tumor of the newborn, which carries an excellent prognosis provided adequate surgical treatment is prompt and excision complete [1]. Although generally a rare condition, it is said to be the most common tumor in the newborn period, with a reported incidence of approximately one in 35,000 40,000 live births [13]. It is a more common tumor in females [4], with a male: female ratio of about 1: 3 4 (4 , 5) . There are many conflicting theories as to the origin of SCT (3) . Historically, teratomas were attributed to demons, sexual misconduct and abnormal fertilization (6) . They are composed of two or three germ cell layers; *

Pediatric Surgeon FICMS/Pediatrics surgery unit The Maternity and Child Teaching Hospital / Al-Qadisiya/ Iraq. E-mail : [email protected]

have multiple tissue types, and can present in various sizes and shapes (6 , 7 ) . Believed to have arisen from the totipotent somatic cells that originate from the primitive knot (Hensen's node), they are usually attached to the coccyx (7, 8) . Calbert, as quoted by Hundling, (10) has given an indication of the rarity of these teratomas. He found an incidence of 1 sacral tumor in 34, 582 births, and only a small proportion of these were teratomas. Two large series have been reported in recent years: Gross et al (9) described 40 cases, and Gwinn et aI (11) reported 18 children with this tumor seen at the Mayo Clinic between the years 1907 and 1953. In contrast to the newborn with SCT, the fetus with SCT remains at high risk of perinatal complications and death (12) . Fetuses with SCT detected antenatally have a mortality rate that is three times higher than the postnatally diagnosed neonates (13) . Neonatal death may result from maternal obstetric complications of tumor rupture, preterm labor, or dystocia (12, 14) . The fetus is also at

Evaluation of Different Presentations of Sacrococcygeal Teratoma in Single Pediatric Surgery Unit

risk of high output cardiac failure, placentomegally and hydrops with subsequent fetal demise secondary to the metabolic demands and vascular steal of a rapidly growing solid tumor (7, 1 2) . Modern technology using the three-dimensional (3D) sonography now allows prenatal diagnosis of SCT even in the first trimester (15) . Surgical resection remains the mainstay of therapy, and recurrence following complete excision is rare. Only a few cases of SCT with a complete limb have been reported worldwide (7, 16) .

. Mohammed Joudi Aboud

classification is shown in Table 1. The distribution according to the age group is given in Table 2. Five patients (71 %) were diagnosed and treated before the age of two months, whereas the remaining two were diagnosed and treated after the age of two months. SCT showed different morphological presentation: SCT appeared as multicystic tumor in 3 patients (Figure 1-4), rupture cyst/ tumor in one patient (Figure 5), limbs with well prominent bones, fingers in one patient (Figure 6,7), and in one patient the tumor was similar to meningocele (Figure 8).

The aim of this study were to the study the age distribution at presentation, the sex ratio, variation of the morphological presentations, and the incidence of the postoperative complications, recurrence rate and malignancy at the time of diagnosis.

Table (1): Altman Distribution of SCT Altman No of patients classification Stage 1 6 Stage 2 1 Stage 3 0 Stage 4 0

PATIENTS AND METHODS

% 85 % 14 % 0 0

Table (2): The Distribution According to The Age Group. Age at presentation Case % Below 2 months 5 71 % 2-6 months 1 14.5 % 6-12 0 0 More than 12 months 1 14.5 %

We present a clinical and epidemiological analysis of the cases of sacrococcygeal teratomas operated in Pediatric Surgery Unit at the Maternity and Child Teaching Hospital \ Al-Qadisiya -Iraq from the period 1 st of January 2005 to 31 st of December 2008 the data used being extracted of the surgical protocols and histopathological exams. A retrospective review was carried out on the records of 7 children with the diagnosis of teratoma treated and operated at this single unit. Regarding the neonates with tumors detected within the first two months of life (n=5), four patients were operated during the first two weeks of life and one was operated at the second day of life because of tumor rupture with hemorrhagic shock at first day of age. Each patient was operated by inverted Chevron incision in prone jack-knife position with its apex directed superiorly. Skin flaps at the superior margin of the tumor were elevated first and the tumor mass drop caudal. The mass was mobilized close to its capsule, widely displaced retrorectally. Muscles were carefully identified and preserved. The tumor was dissected free from rectal wall and removed with coccyx. The anorectal muscles were reconstructed. All excised tissues were histologically examined. Multiple sections were routinely cut. This was done at the time of the original resection. We have faced difficulties with regards to the follow up of those cases; records are not available and some patients were lost to follow up. Long term outcome could not be ascertained.

Postoperative recovery was uneventful in 4 cases. One patient developed urinary retention, 1 developed urinary tract infection. In 1 case, wound dehiscence occurred, this was treated conservatively, and healing by secondary intention was allowed. The mean period of follow up was from 3 months to 2 years in only 5 patients. Follow-up sonogram and magnetic resonance imaging MRI were done for all patients. One children had tumor recurrence (14 %), that were detected by physical examination, laboratory tests by means of clinical evaluation or urinary or anorectal functional impairment. One child with a patulous anus presented with fecal soiling and two reported nocturnal enuresis. One postoperative death occurred in our series because of circulatory failure and/ or hemorrhage. SCTs contained only cystic component in four patients (57 %), both cystic and solid components in one patient (14 %) and predominantly solid components in two patients (29 %). The histological diagnosis was mature teratoma in 4 children (57 %), immature teratoma in one (14 %), and malignant germ cell tumor in 2 (29 % per cent) both were a yolk sac carcinoma. In 5 children the resection margins were microscopically free of tumor on histopathological examination; the resection was incomplete in 2 children .The two malignancies were treated with a combination of chemotherapy in other center. At two years, one was alive, while the other had started therapy a year later, she developed tumor recurrence and we lost during our study. The different characteristics of the study population are shown in Table 3.

RESULTS Of 7 patients, 6 (86 %) were female. Four (57 %) out of 7 patients delivered by caesarean section. The distribution of the tumor according to Altman s

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Evaluation of Different Presentations of Sacrococcygeal Teratoma in Single Pediatric Surgery Unit

Figure (1): Multicystic mass presentation of SCT

Figure (2): Multicystic mass presentation of SCT. Post operative gross pathology

Figure ( 3): Multicystic mass presentation of SCT bulging from the rectum

Figure (5): Ruptured cyst/ tumor

. Mohammed Joudi Aboud

Figure(4): Postoperative view for the same case presented in Figure 3

Figure (6): A patient with tumor presented as limbs with well prominent fingers

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Figure (7): A patient with cyst/tumor presented as limbs

Figure (8): In one patient SCT was similar to meningocele

Table 3. The different characteristics of the study population No. (%)

care. The surgical approach to SCT consists of complete removal of the tumor through the sacral area or a combined abdominosacral approach with the coccyx always being removed. The importance of removing the coccyx with the tumor was emphasized first by Gross (9) and then by others (1820) . High recurrence rates of up to 37% were registered if the coccyx had not been excised at the initial procedure (9, 21, 22) . In all our cases the coccyx was removed. Both immature and malignant histology of the primary tumor were also positive predictors of recurrence. Bilik et al(23) identified no factors that influenced the recurrence of SCT in 28 children .Whenever histopathological examination of the resected specimen shows mature teratoma, a careful search for areas of malignancy in the specimen may be beneficial [23,2 4]. The mortality rate soon after birth and around the time of operation was relatively high (4·6 %). These deaths were caused by high-output cardiac failure, hemorrhage into the tumor or perioperative bleeding. To minimize both the risk of operative cardiac failure and hemorrhage, laparoscopic ligation of the median sacral artery has been advocated before resection of the SCT (25-27) . These facilities are still not valid in our institution. One postoperative death occurred in our series regardless of the size of the primary SCT. The recurrent SCTs in our series occurred in patient whose primary tumor contained both cystic and solid elements, and no recurrent SCT occurred in the totally cystic SCT group in our series. Four patients in our study had been diagnosed benign as having mature SCT at the primary tumor resection but malignant components were documented in the recurrences in one patient .This phenomenon has been reported also by other authors (9,21,28) . Attempts to explain this malignant transformation of mature and benign SCT were made by Willis who believed

Age at presentation: < 2 months > 2 months Gender: Male/Female Postoperative complications Postoperative mortality Rate of malignancy at presentation Recurrence rate

. Mohammed Joudi Aboud

5 (71 %) 2 (29 %) 5/2 (71% /29 %) 3 (43 %) 1 (14 %) 2 (29 %) 1 (14 %)

DISCUSSION Teratomas in infants and children most commonly appear in the sacrococcygeal region. Less common sites are the mediastinum (5) , testes, retro peritoneum, neck and stomach, as reported in the literature. The majority of SCT cases are apparent at birth. Most of the infants are female and more than 60% are present at birth (9 , 17) . Regarding the SCT our series correlate with these published data. Only one of our seven SCT patients was a male. The reason for this female preponderance is not known (4, 5) . We were quite limited in our data collection due to non availability of proper records, but nevertheless some significant points emerged: foremost of which was the late presentation of these patients in our series (2 of 7 patients). With 4 of the early presenting cases being from our hospital record and 3 from the urban areas and referred from minor primary health clinics. The rural cases were inevitably sent late, this is due to the dual problems of illiterate parents and failure of recognition by the general practitioners. Although prenatal diagnosis is possible in some cases, this was not possible in our patients since the mothers did not attend antenatal

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that late malignancy might be due to the retained capacity for continued growth at the embryonic level (29) . The differential diagnosis for perinatal sacrococcygeal masses include meningocele, myelomeningocele, myelocystocele, teratoma, lipoma, hamartoma, lymphangioma, hemangioma, chordoma, and ependymoma. There are case reports of SCT that are misdiagnosis as myelomeningocele (4, 30) . Other studies showed an accurate anatomical extent of the mass and vertebral anomaly was detected unexpectedly during an operation in three babies (31, 32) . In a fourth case of extensive involvement of the spinal canal diagnosed preoperatively was presented by Ribeiro et al. (33) . Sutton suggested that the tumor might have originated from the occult dysraphism and secondarily formed the predominant exterior sacrococcygeal mass [33]. The etiology of sacrococcygeal teratomas has been the subject of great controversy (34) . One school of thought says that these tumors arise from a primitive knot of cells which are found in the coccygeal area. These cells retain their totipluratiry for the longest time aside from the gonadal tissue. Other authors suggest that this is an attempt at twinning and the term Fetus -in Fetu came about. Now this term is reserved for those cases in which there is axial differentiation of limbs and organs as in one of our cases (4, 34) . In our study, the Altman (4) staging of the primary SCT was not found to be helpful in predicting tumor recurrence in terms of benign or malignant status because of limited data in our study and the poor follow up due

. Mohammed Joudi Aboud

to the large distances involved in traveling to our hospital and also duo to ignorance of the parents. All seven cases that recurred were initially staged as Altman stage II and I.

CONCLUSION In most cases, surgery is the principle mode of treatment of SCT. From this study with the limited cases we aimed to encourage more advances in accurate prenatal diagnosis, which may be useful in prospective perinatal management as well in parental counseling to help us for initiating a precise surgical interference with availability of more facilities such as estimation of alpha-fetoprotien, 3D sonography and MRI. More detailed diagnostic imaging should be without doubt indicated in babies affected by SCT, regardless of anatomic type, in whom preoperative radiograph suggests any vertebral anomaly. We suggest a national survey for large group of patients with SCT in other centers for reliable identification of different presentation and risk factors. Whenever histopathological examination of the resected specimen shows mature teratoma, a careful search for areas of malignancy in the specimen should be peformed especially in delayed presentation (more than 2 months).

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Mohammed Joudi Aboud Evaluation of Different Presentations of Sacrococcygeal Teratoma in Single Pediatric Surgery Unit: A Case Series

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