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Pancreatic Teratoma in an Infant – A Case Report

Bhanumathi Lakshminarayanan¹, Kokila Lakhoo²

¹Department of Paediatric Surgery, Leeds General Infirmary, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom

²Department of Paediatric Surgery, John Radcliffe Hospital, Oxford, United Kingdom

 

Correspondence:

Bhanumathi Lakshminarayanan

Department of Paediatric Surgery

Leeds General Infirmary, Leeds Teaching Hospitals NHS Trust

Great George St, Leeds LS1 3EX, UK

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Teratoma arising from the pancreas is very rare. We report a case of immature cystic teratoma of the head of the pancreas in an infant. A literature search of presentation and management is discussed.

Keywords: immature teratoma, head of the pancreas

 

Case Report

A 5-month-old infant presented with abdominal distension and feed intolerance since birth. There was a sudden increase in abdominal distention 2 weeks prior to presentation to hospital. The child was born at term and had a normal antenatal course. A palpable abdominal mass was the prominent feature. This prompted further investigations including urinary catecholamines, which was normal and tumour markers such as alfa-feto protein which was elevated to 1600 and a normal beta human chorionic gonadotropin. A chest radiograph was normal but had an indication of some intraabdominal calcification below the diaphragm with an elevated diaphragm (Fig 1).

Figure 1. Chest radiograph showing elevated diaphragms but otherwise normal lung fields. Below the diaphragm there are dilated bowel loops with the rim of calcification (black arrow) seen at L1- L3 vertebrae.

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An ultrasound abdomen showed a multicystic intraabdominal mass. A computerised tomography was done to further clarify the diagnosis and radiological diagnosis of teratoma was made. However, the tumour was large and occupying the entire peritoneal cavity that the point of origin could not be identified (Fig 2). The child proceeded to a laparotomy done through an upper transverse incision. It was immediately apparent that the cyst measuring 16 x 13 x 7 cms was in close proximity to the head of the pancreas and stretching the duodenum (Fig 3). The cyst wall was adherent to the common bile duct, superior mesenteric vessels, portal vein and celiac trunk (Fig 4). The only visible part of the pancreas was the tail with the head fully distorted or replaced by the teratoma. No pancreatic duct was seen draining this structure to the duodenum.

Figure 2. CT abdomen coronal section showing a large Intraabdominal mass with solid and cystic areas with area of calcification highly suggestive of a teratoma

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Figure 3. Intraoperative picture of cyst in close proximity to the medial side of the duodenum. (D - duodenum , T – teratoma, PT –portal triad).

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Figure 4. Intraoperative picture of cyst showing close adherence to major structures (T – teratoma, DP – distal pancreas, SMV – superior mesenteric vein, PV – portal vein)

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The teratoma was excised. A < 0.5 cm capsule was left behind as adherent to the common bile duct and portal vein. A pancreatico jejunostomy was performed to aid the drainage of the distal pancreas. Post-operatively the endocrine function of the pancreas was preserved. The histology confirmed a grade 3 immature teratoma of the head of the pancreas. The exocrine function was monitored and after an initial post-operative decline in the faecal elastase, this improved to normal levels within 6 weeks. Following a national multidisciplinary discussion, no chemotherapy was recommended. The child was regularly followed up with tumour markers and ultrasound of the abdomen. There has been no evidence of any recurrence at 4 years of follow-up.

Discussion

The commonest location for teratoma in the paediatric age group is sacrococcygeal. Retroperitoneal location of teratomas accounts for less than 5 % of all teratomas [1] of all these retroperitoneal teratomas the ones arising from the pancreas are even rarer. Knowledge of pancreatic teratomas is limited to case reports in the literature [2-12]. There are so far only 12 cases, including our case of paediatric pancreatic teratoma reported. The age range is from 4 months to 16 years and 5 of these teratomas are in male patients. In a majority of the paediatric cases, the teratoma seems to arise either from the head or the body of the pancreas, unlike, the adult pancreatic teratomas. The various case reports, their presentations, and management are presented in Table 1.

Table 1 - Paediatric pancreatic teratoma – presentation and management

Source, year            

Age, Sex

Symptoms

Procedure

Size

Histology

Follow-up

DeCourcy, 1943 [2]

2 F

Vomiting, dehydration, epigastric mass

Resection  with large denuded area of pancreas

Size of small orange

Body. Dermoid cyst

No f/u details

Bittner, 1970 [3] (Polish)

2 F

Liver failure, epigastric mass

Resection

Not known

Head

Well at 6 months

Iovchev 1972 [4]

(Russian)

8M

Vomiting, fever, LUQ mass

Ext drain

Not known

Body, Suppurative dermoid cyst

Well after 7 months

Pomosov 1973 [6]

(Russian)

6M

Abdo pain, fever, mass

Distal pancreatectomy

Not known

Tail, dermoid cyst

Well after 6 months

Komarov 1973 [5]

(Russian)

4F

Pain, vomiting, mass

Ext drain

Not known

?

Not known

Assawamatiyanont 1977 [7]

11F

Asymptomatic, mass

Resection

9 x 8 x 6

Body

Uneventful. ?duration of f/u

Das 1996 [8]

4mo F

Abdo mass

Resection

9.5 x 8.5cm

Body/tail. Mature cystic teratoma

No f/u details

Yu 2003 [9]

2 M

Asymptomatic, mass

Resection

11x 9 cm

Head/body . Mature cystic teratoma

No f/u details

Salimi 2004 [10]

16M

Jaundice, weight loss

Choledochoduodenostomy, resection

Not known

Head, cystic teratoma

Well after 4 years

Kela, 2008 [11]

5mo M

Abdominal pain, mass

Deroofing, cystectomy

8x7cm

head, immature cystic teratoma

Chemotherapy lost to f/u

Wang, 2013 [12]

12 mo F

Jaundice, vomiting

Panceatico-duodenectomy

4.6 x 4.1 x 3.3cm

Head, mixed germ cell tumour with endodermal sinus tumour and teratoma

Chemotherapy, possible recurrence in liver after 2 months

Our case

5 mo M

vomiting, abdominal distension

Cystectomy

16 x 13 x 7 cm

Head, immature teratoma Grade 3

No recurrence at 4 years f/u

As with most teratomas in this age group, they are mostly benign mature teratomas. However, Kela et al. report an immature cystic teratoma arising from the head of the pancreas in their patient who was 5 months of age at presentation [11]. This patient had deroofing followed by simple cystectomy. This patient received adjuvant chemotherapy due to the immature nature of the tumour. The case reported by Wang et al had malignant elements to the teratoma and was given chemotherapy [12]. This child had an early recurrence within 2 months of initial resection, even though a major pancreaticoduodenectomy was performed. Our patient had grade 3 immature teratoma and was discussed in a national meeting that concluded that chemotherapy would not be required and regular surveillance with tumour markers and ultrasound of the abdomen would suffice to look for recurrence.

The role of chemotherapy in immature teratoma remains controversial as the treatment has to be weighed against the risk of recurrence versus the long-term effect of chemotherapy. A report from the paediatric oncology group studying immature teratoma arising in the ovary has concluded that surgery alone is an effective treatment in children and young adolescents [13]. This appears to be the case not only for gonadal germ cell tumour but also extragonadal teratomas. The recurrence rate is 6.8% with 3 years event free survival of 93%; the recurrence occurring in-spite of chemotherapy [14].

Lo Curto et al. studied the risk of recurrence in teratomas and showed a 5% risk of recurrence in mature teratoma and 15% risk in immature teratoma [15]. The risk of recurrence is increased in incomplete resection and in the female gender. Incomplete resection is an independent risk factor for relapse, more so than immature histology [15]. Our patient had a complete resection except for 0.5 cm of the capsule that was adherent to major vessels, with no evidence of recurrence at 4 years follow-up. Our case is an addition to the literature of only the second case of immature teratoma arising from the head of the pancreas in a child. We have discussed the dilemma in the management of this rare tumour. It appears that in children these tumours can be treated with complete cystectomy without the need for an extensive procedure like Whipple's pancreaticoduodenectomy with a good outcome.

 

 

References:

1. Tapper D, Lack EE. Teratomas in infancy and childhood. A 54-year experience at the Children's Hospital Medical Center. Ann Surg. 1983;198(3):398-410.

2. DeCourcy JL. Dermoid cyst of the pancreas: case report. Ann Surg. 1943;118(3):394-5.

3. Bittner K, Sarrazin O. Pancreatic teratoma in a 2-year old girl. Wiad Lek. 1970;23(14):1251-2.

4. Iovchev II. Suppurative dermoid cyst of the pancreas. Vestn Khir Im I I Grek. 1972;107(3):124.

5. Komarov IF. Acute destructive pancreatitis in a child. Vestn Khir Im I I Grek. 1973;110(4):93-4.

6. Pomosov DV, Shpit'ko FS, Volianiuk MA. Dermoid cyst of the pancreas in a child. Vestn Khir Im I I Grek. 1973;110(4):92-3.

7. Assawamatiyanont S, King AD, Jr. Dermoid cysts of the pancreas. Am Surg. 1977;43(8):503-4.

8. Das PC, Radhakrishna K, Rao PLNG. Cystic teratoma of the pancreas. Pediatr Surg Int 1996;11:177-8.

9. Yu CW, Liu KL, Lin WC, Li YW. Mature cystic teratoma of the pancreas in a child. Pediatr Radiol. 2003;33(4):266-8.

10. Salimi J, Karbakhsh M, Dolatshahi S, Ahmadi SA. Cystic teratoma of the pancreas: a case report. Ann Saudi Med. 2004;24(3):206-9.

11. Kela M, Singh S, Lahoti B. Immature cystic teratoma of head of pancreas. J Indian Assoc Pediatr Surg. 2008;13(2):85-7.

12. Wang J, Zheng Z, Qiu Y, Tou J, Liu W, Xiong Q, et al. Primary mixed germ cell tumor arising in the pancreatic head. J Pediatr Surg. 2013;48(1):e21-4.

13. Cushing B, Giller R, Ablin A, Cohen L, Cullen J, Hawkins E, et al. Surgical resection alone is effective treatment for ovarian immature teratoma in children and adolescents: a report of the pediatric oncology group and the children's cancer group. Am J Obstet Gynecol. 1999;181(2):353-8.

14. Marina NM, Cushing B, Giller R, Cohen L, Lauer SJ, Ablin A, et al. Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Intergroup Study. J Clin Oncol. 1999;17(7):2137-43.

15. Lo Curto M, D'Angelo P, Cecchetto G, Klersy C, Dall'Igna P, Federico A, et al. Mature and immature teratomas: results of the first paediatric Italian study. Pediatr Surg Int. 2007;23(4):315-22.