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Teratoma in Infants and Children

Ahmed H. Al-Salem¹, Mustafa Hamchou², Akhter Nawaz², Hilal Matta²
¹Department of Pediatric Surgery, Maternity and Children Hospital, Dammam, Saudi Arabia
²Division of Pediatric Surgery, Tawam Hospital, Al-Ain, Abu-Dhabi, United Arab Emirates

 

Abstract

Background: Teratomas are a unique group of tumors with variable behavior depending on the site, size, histology and age at diagnosis. This paper aims to highlight the clinical features, investigations and treatment of teratomas at different parts of the body, in our settings.

Patients and Methods: The medical records of all infants and children with the diagnosis of teratoma treated between 1989 and 2007 were retrospectively reviewed for: age at diagnosis, sex, presenting symptoms, site of tumor, treatment, histology and outcome.

Results: Over a period of 18 years, we treated 29 infants and children with teratomas at various sites of the body. These included sacrococcygeal (14), ovarian (4), cervical (5), retroperitoneal (4), gastric (1) and buttock (1). There were 14 sacroccocygeal teratomas, all were females except two. The age at presentation ranged from birth to 18 months, but the majority (78.6%) presented within the first week of life. According to Altman’s classification, 10 were type 1, 2 type 2, and 1 type 3. In one patient, it was difficult to classify. The majority (77%) were benign. Two of the three with malignant sacrococcygeal teratoma presented late. Five had cervical teratoma. All were left sided. In all, the swelling was large to the extent that 3 presented with acute respiratory distress necessitating emergency intubation and one developed brain hypoxia. All were benign except one who had immature grade I teratoma. Four had ovarian teratoma, one of them presented as an emergency. Two were malignant. Four had retroperitoneal teratoma, one of them was malignant. A 4-days old male with a very large abdominal mass and calcifications was found to have a large gastric teratoma which was immature grade I. A 6-months old female was found to have a benign teratoma of the buttock.

Discussions: Teratomas are an interesting group of tumors with similar histological picture but variable behavior. Sacrococcygeal teratoma is the commonest and the majorities are benign but the risk of malignant transformation increases with age. The majority of cervical teratomas are benign, but they can cause significant morbidity and mortality as a result of respiratory compromise. Surgical excision of these large tumors needs to be planned. We found staged excision in some of these patients is beneficial. Retroperitoneal teratomas are rare and the majority present with an abdominal mass which can attain a large size. Careful attention to adjacent major blood vessels in the vicinity of the tumor at the time of resection is very important as these tumors tend to distort major blood vessels including the renal vessels. Ovarian teratomas are the second most common teratoma in infants and children. The majorities are easily resectable but they can attain a large size and present as an emergency because of a twist. Gastric teratoma is very rare and resection is curative.

Key words: teratoma, sacrococcygeal, cervical, ovarian, retroperitoneal, gastric, buttock teratoma, staged excision, benign tumors, significant morbidity

 

Correspondence

Dr. Ahmed H. Al-Salem
P.O. BOX 61015
Qatif 31911
Saudi Arabia
E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Introduction

Teratoma is derived from the Greek word teraton meaning monster and by definition, teratomas arise from totipotential cells containing multiple tissues foreign to the part in which they arise and so are comprised of tissues from all three germ cell layers, the ectoderm, endoderm and mesoderm [1,2]. As a result of this, teratoms display various degree of differentiation and immaturity and can be benign, malignant or in between. The most common sites for teratoma occurrence in children are saccrococygeal region (57%), gonads (29%), mediastinum (7%), retroperitoneal (4%), cervical (3%) and intracranial (3%). Teratomas have also been reported to arise at other rare sites including the stomach, thyroid gland, nose, liver and spinal cord [2,3,4,5,6,7,8]. The prognosis of teratoma is however variable and depends on several factors including site, age at operation, resectability, histological features and stage. The importance of early diagnosis and treatment is of great importance in this type of tumors as there is a potential for malignant transformation and invasion by time. This report is an analysis of our experience with teratomas at various sites of the body.

Patients and Methods

The medical records of all infants and children with the diagnosis of teratoma treated between 1989 and 2007 were retrospectively reviewed and the following information was obtained: age at diagnosis, sex, presenting symptoms, site of tumor, treatment, histology and outcome.

Results

Over a period of 18 years, we treated 29 infants and children with teratomas at various sites of the body (table 1). Sacrococcygeal teratoma (SCT) was the commonest (48.3%). The clinical features of those with SCT are summarized in table 2.

Table I: Types of teratoma. Total no. of patients: 29

TYPEOF TERATOMA

NO.OFPATIENTS

%

SACROCOCCYGEAL

14

48.3

CERVICAL

5

17.3

OVARIAN

4

13.8

RETROPERITONEAL

4

13.8

GASTRIC

1

3.45

BUTTOCK

1

3.45

Table 2: Clinical features of patients with sacrococcygeal teratoma

NO.

AGE

SEX

SIZE

TYPE

HISTOLOGY

1

1DAY

F

6x7CM

I

BENIGN

2

18 MONTHS

F

3x2CM

II

MALIGNANT

3

18 MONTHS

M

6x2CM

II

MALIGNANT

4

DAYS

F

8x7CM

I

MALIGNANT

5

DAY

F

10x9CM

I

BENIGN

6

28 HOURS

F

6x5CM

I

BENIGN

7

2DAYS

F

3.5x3CM

I

BENIGN

8

14 MONTHS

F

7X4CM

III

REFUSEDSURGERY

9

DAYS

F

10x8CM

II

BENIGN

10

DAYS

F

7x8CM

I

BENIGN

11

DAYS

F

6x8CM

I

BENIGN

12

DAYS

M

10x7CM

I

BENIGN

13

DAYS

F

6x7CM

I

BENIGN

14

1 WEEK

F

5x6CM

I

BENIGN

There were 14 patients with SCT, all were females except two. The age at presentation was variable ranging from birth to 18 months, but the majority (78.6%) presented within the first week of life. The size of the tumor was also variable (table 2). One of our patients refused surgery, but the majority (77%) were benign. This patient presented late at the age of 14 months with a SCT that was present since birth but had increased in size. Her alpha fetoprotein was elevated (980ug/dl) and her MRI showed a large intrapelvic extension. According to Altman’s classification, 10 were type 1, 2 type 2, and 1 type 3. In one patient, it was difficult to classify as there was as much extension inside the abdomen as outside (fig. 1).

Figure1: Clinical photograph and CT-scan showing a large sacrococcygeal teratoma. Note the intra-abdominal extension which is as much as the external extension.

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Two of the three patients with malignant SCT presented late at 18 months of age while the third one presented at the age of 4 days.

One of them (patient no. 2) was diagnosed at birth but the family refused surgery. She was operated several months later in another hospital and was referred to us because of recurrence. She had a local recurrence measuring about 3x2 cm with an elevated alpha-fetoprotein. This was excised and the histology showed a poorly differentiated invasive germ cell tumor. She was treated with chemotherapy and responded well. Her alpha-fetoprotein became normal and 5 years later, she was well with no recurrence. The other patient (patient no. 3) was referred to our hospital at the age of 18 months. She had incision of the tumor in another hospital mistakenly diagnosed as an abscess. The tumor was excised and histology showed malignant teratoma. She was treated with chemotherapy and was well 12 years later. Five of our patients had cervical teratoma (table 3). Three were males and 2 were females. Their age at presentation ranged from 1 day to 4 months (mean 44.4 days). All were left sided. In all, the swelling was large to the extent that 3 of them presented with acute respiratory distress necessitating emergency intubation.

Table 3: Clinical features of patients with cervical teratoma

NO.

AGE

SEX

SIDE

HISTOLOGY

1.

4 MONTHS

F

LEFT

BENIGN

2.

DAY

M

LEFT

BENIGN

3.

16 DAYS

F

LEFT

BENIGN

4.

25 DAYS

M

LEFT

BENIGN

5.

2 MONTHS

M

LEFT

IMMATURE GRADE  I

One of them (patient no. 3) was referred to us from another hospital. She presented with acute respiratory distress and her intubation was difficult and delayed with subsequent hypoxic brain damage. She had partial excision initially and subsequently she had complete resection at our hospital. Postoperatively, she did well but she died because of associated severe brain damage. In two of them, complete resection was not possible in one stage. Histologically, all were benign except one who had immature grade I teratoma. This was in a 2 monthold male who presented with a large left sided teratoma (fig. 2). Four of our patients had ovarian teratoma (table 4).

Figure 2: MRI showing cervical teratoma

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Table 4: Clinical features of patients with ovarian teratoma

NO.

AGE

SEX

SIZE

SIDE

HISTOLOGY

1.

3.5 YEARS

F

7x6CM

LEFT

BENIGN

2.

6 YEARS

F

10x6CM

RIGHT+LEFTSTREAKOVARY

MALIGNANT

3.

6 YEARS

F

3X5CM

RIGHT+CHOLE-CYSTECTOMY

BENIGN

4.

5 YEARS

F

20x15CM

RIGHT

MALIGNANT 

Their age at presentation ranged from 3.5 years to 6 years (mean 5.13 years). One of them (patient no. 2) presented with abdominal pain and a right sided lower abdominal mass. Abdominal ultrasound revealed a large 10 x 6 cm solid mass arising from the pelvis. At exploration, she was found to have a twisted right ovarian tumor and a streak left ovary. She had bilateral salpingo-oophorectomy and histology showed a malignant teratoma. She was treated with chemotherapy and on follow-up 5 years later, she was well. Another patient (patient no. 4) was 5 years old when she was referred to our hospital with a huge abdominal mass filling the whole abdominal and pelvic cavities (fig. 3). At the time of surgery, she was found to have a very large mass arising from the right ovary (fig. 4).

Figure 3: Clinical photograph showing a very large ovarian teratoma

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Figure 4: CT-scan and intraoperative photograph of the patient in figure 4 showing a very large ovarian teratoma filing the whole of the abdominal cavity

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This was excised and histology showed a malignant teratoma. She was treated with chemotherapy and 6 years later, she was well. One of our patients was a 6-year old girl with sickle cell disease (patient no. 3). She was found to have gallstones and on routine abdominal x-ray was found to have calcification in the pelvis. Pelvic ultrasound showed a 5X3 cm mass in the right ovary with calcification. Her alpha fetoprotein was normal. She was operated on and underwent cholecystectomy and right oophorectomy. This revealed a benign teratoma of the right ovary. Four patients had retroperitoneal teratoma (table 5).

 Table 5. Clinical features of patients with retroperitoneal teratoma

NO.

AGE

SEX

SIDE

HISTOLOGY

1.

3.5 YEARS

M

RIGHT

BENIGN

2.

2 WEEKS

F

LEFT

BENIGN

3.

2 YEARS

M

LEFT

MALIGNANT

4.

5 MONTHS

M

LEFT

BENIGN

Their age ranged from 2 weeks to 3.5 years (mean 1.5 years). A 2-year old male child presented with abdominal mass, fever and abdominal pain. His abdominal x-ray showed a soft tissue mass with calcifications. This was confirmed with an ultrasound and a CT-scan which showed a large (17.5x10 cm) mass with calcifications. His alpha fetoprotein was 2030 µg/dl. He underwent excision and histology showed it to be a malignant teratoma. He received chemotherapy and 7 years later, he was well. Another patient was 5 monthold when he was referred to us with a large left-sided abdominal mass which on CT-scan was found to extend from the anterior abdominal wall to the spine, pushing the liver to right and upwards. There were no calcifications. His alpha fetoprotein was normal. Intraoperatively, the mass was adherent to the stomach and esophagus. It was excised totally together with part of the lower esophagus and stomach which were repaired. Histology showed it to be a benign teratoma. The other two patients had benign retroperitoneal teratomas that were excised totally (fig. 5).

Figure 5: CT-scan and intraoperative photograph showing a large retroperitoneal teratoma with calcification

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Figure 6: CT-scan and clinical photograph showing a large gastric teratoma with calcification

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A 4-days old male child was referred to us with a very large abdominal mass with calcifications (fig. 6). His alpha fetoprotein was high. The mass was growing day by day and intraoperatively was found to have a large mass arising from the wall of the stomach. This was excised together with part of the stomach and histology showed an immature grade I gastric teratoma. Postoperatively, he did well and was discharged home in a good general condition. He is now 1.5 years postoperatively, doing well and his alpha fetoprotein became normal.

A 6-months old female was referred because of right buttock swelling since birth (fig. 7). The swelling was increasing gradually and her alpha fetoprotein was normal. She had excision of the swelling and histology showed a benign teratoma.

Figure 7: Clinical photograph showing a right buttock teratoma

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Discussion

Teratomas are an interesting group of tumors with similar histological picture containing multiple tissues foreign to the part in which they are located but of different behavior. In general, they are considered to be rare tumors and although there are reports of teratomas arising at unusual sites, commonly they are seen in the sacrococcygeal area, ovary, neck, retroperitoneum and testes [2, 3, 4, 5, 6, 7, 8].

CT is the commonest of all of them and considered the most common tumor in newborns but the incidence is reported to be 1 in 35,000 – 40,000 live births [9]. Most SCT are diagnosed in newborns but the increasingly routine use of prenatal ultrasound has increased the number of SCT diagnosed in utero. According to Altman’s classification, type IV SCT is completely intrapelvic and difficult to diagnose. Usually these patients present late and are more likely to be malignant. The majority of SCT are benign but the risk of malignant transformation increases with age. There is a 2% to 5% risk of malignancy in neonates with SCT, and this risk increases exponentially to 50% by 1 year of age [9,10,11].

Gabra et al. in a review of 25-year single-center experience found thirty-three patients with SCT, 7 of them presented beyond the newborn period which was associated with malignant histology and poorer outcome [9]. The importance of this need to be emphasized. This is specially so in our settings where some of these patients are born in peripheral hospitals and if this is not recognized and referred to a specialized center, the treatment will be delayed with increased risk of malignant transformation. This is even more for Type III and IV of Altman’s classification. This was clearly shown in three of our patients who presented late; two of them were proven to be malignant. One of them had incision and drainage of a SCT that was mistaken for an abscess. The importance of total excision of SCT together with the coccyx needs to be also emphasized.

Derikx et al. in a retrospective review of 173 children with SCT treated between January 1970 and February 2003 found immature and malignant histology or incomplete resection as risk factors for recurrence and metastasis [11]. It is also important to closely follow-up patients with SCT postoperatively. Poor cosmetic results in the buttock region were the most common long-term complication after surgery for SCT, which may need further corrective surgery in later life [12]. Not only this, but there is a significant risk of recurrence in these patients of about 11% within 3-years [10, 11, 13 14].

The risk is more in those with immature or malignant histology and those incompletely resected. Neuropathic bladder or bowel disturbance are also long term complications following resection of SCT [15, 16]. This was reported in as much as one third of the patients on long-term follow-up. There is no correlation between tumor grade and the incidence of urological complications. The most common urological complications were neurogenic bladder in 12% of the patients, ureteral obstruction in 10% and vesicoureteral reflux in 7% [15]. The highest incidence of urological complications (81%) was seen in patients with type IV SCT [15]. Parental counseling should include the continence problems that may follow removal of even benign tumors and these patients should undergo radiological and neurodynamic evaluation on follow-up.

Cervical teratomas are very rare tumors. In a series of 42 patients with teratoma, Partlow et al. found only 2 cervical teratomas [4]. In a large series of 354 teratomas seen over a 54-year period, Tapper and Lack reported only six (2.4%) cervical teratoma [2]. Five of our patients had cervical teratoma. Although the majority of cervical teratomas are benign, they can cause significant morbidity and mortality. One of our patients had grade one immature teratoma and for unknown reason, all our patients had leftsided cervical teratomas. Cervical teratomas tend to grow and attain a large size causing distortion of the normal anatomy with life-threatening upper airway obstruction. This makes postpartum intubation very difficult and sometimes impossible. In one of our patients who delivered in another hospital, there was difficulty and delay in intubating her leading to severe brain hypoxia. One way to overcome this is the EXIT (ex-utero intrapartum treatment) procedure [17]. This allows partial fetal delivery via caesarean section with establishment of a safe fetal airway by intubation, bronchoscopy, or tracheostomy while fetal oxygenation is maintained through utero-placental circulation. The antenatal diagnosis of large congenital cervical teratomas calls for early referral of these patients to specialized centers which allows for planned intervention by experienced personnel. Complete local excision of these tumors is the treatment of choice. This however may not be feasible always as these tumors may be extensive. To obviate postoperative morbidity and mortality, surgical excision of these large tumors needs to be planned also. We found staged excision in some of these patients beneficial. Two of our patients with large cervical teratomas were managed by staged resection with a good outcome.

Retroperitoneal teratomas are rare and comprise 3.5%- 4% of all germ cell tumors in children [3]. The majority present with an abdominal mass which can attain a large size. The presence of calcification on plain x-ray should raise the possibility of a teratoma. We found CT-scan valuable in delineating the extent of the tumor for proper planning and surgical excision. We also found that in spite of the large size of these tumors, they are amenable to complete surgical resection. Careful attention to adjacent major blood vessels in the vicinity of the tumor is very important as these tumors tend to distort major blood vessels including the renal vessels [18, 19, 20]. The majority of these tumors are benign. One of our patients who presented at the age of 2 years with an abdominal mass was found to have malignant retroperitoneal teratoma. He had complete excision followed by chemotherapy and was well 7 years postoperatively.

Ovarian teratomas are the second most common teratoma in infants and children. They make up about 37% of all teratomas in infants and children and in some series they are considered the commonest teratoma in infants and children [2, 3, 4]. We saw only 4 patients with ovarian teratoma which is a relatively small number. The reason for this low incidence is not known. The majority of ovarian teratomas are benign and only 6%-10% are malignant [2]. Two of our patients had malignant teratoma. In one of them, the contralateral ovary was a streak ovary. This raises the possibility that the tumor on the other side most likely originated in a streak ovary also. The other patient with malignant teratoma presented with a huge abdominal swelling occupying almost the whole of her abdomen which after excision was reported as malignant teratoma but in spite of the large size, there was no evidence of metastasis. In both, preoperative ultrasound revealed solid tumors which were more likely to be malignant. In one of our patients, the teratoma was discovered incidentally on plain abdominal x-ray in a sickle cell disease patient going for cholecystectomy. Serum alpha feto-protein is a useful marker in these patients for evidence of recurrence or the presence of residual tumor.

One of our patients had gastric teratoma. He presented immediately after birth with a large abdominal mass that was increasing in size. Gastric teratomas are very rare accounting for less than 1% of all teratomas in infants and children [6]. For unknown reasons, there is a male preponderance with 90% of cases reported in boys and mostly seen in infants. Clinically, the majority of them present with abdominal distension and a palpable abdominal mass, but there are reports of gastric teratomas presenting with upper gastrointestinal bleeding [21]. This is seen in gastric teratoma with intraluminal growth of the tumor and mucosal ulceration. The management of gastric teratoma is surgical excision of the tumor and part of its attachment to the stomach. This is usually curative and rarely partial or total gastrectomy is necessary depending on the extent of the tumor [22]. The majority of gastric teratomas however are benign and in the presence of immature neuroepithelium tissue these tumors are considered malignant, but even than the prognosis is excellent after total excision [6, 23, 24].

 

 

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