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Primary yolk sac tumor of the common bile duct
Journal of Pediatric Surgery (2011) 46, 1271–1273
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Primary yolk sac tumor of the common bile duct Gayatri S. Munghate, Sandeep Agarwala, Veereshwar Bhatnagar ⁎ The Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi-110029, India Received 3 October 2010; revised 3 February 2011; accepted 4 February 2011
Key words: Yolk sac tumor; Endodermal sinus tumor; Extragonadal yolk sac tumor; Common bile duct; Obstructive jaundice
Abstract Yolk sac tumor (YST) is a germ cell neoplasm usually arising from the gonads. Extragonadal YST has been reported in the mediastinum, retroperitoneum, liver, and pelvis. Primary YST of the common bile duct (CBD) has not been previously reported. We describe an 11-month-old girl with a YST arising from the CBD who presented with obstructive jaundice. Imaging studies revealed an intraluminal CBD mass with intrahepatic biliary dilatation and extraluminal infiltration of the head of the pancreas. A complete resection was carried out, and postoperative chemotherapy with cisplatin, etoposide and bleomycin was administered. © 2011 Elsevier Inc. All rights reserved.
Yolk sac tumors (YSTs; endodermal sinus tumor) are the most common pediatric germ cell tumors, accounting for 20% of cases, occurring primarily in the gonads [1,2]. Extragonadal YSTs occur in 10% to 15% of the patients. The common sites for extragonadal YST are mediastinum, vagina, brain, and retroperitoneum [3]. Sporadic cases of YST at rare sites e.g. orbit, nasal cavity and lung have been reported [4-6]. This report describes a primary YST of the common bile duct (CBD), possibly the first of its kind.
1. Case report An 11-month-old girl presented with increasing jaundice, dark urine, clay colored stools, and abdominal distension of 1.5-month duration. During this time, she also had intermittent low-grade fever. The perinatal history was unremarkable. Physical examination showed an obviously jaundiced infant with a firm and nontender liver edge palpated 4.0 cm below the right costal margin. Serum ⁎ Corresponding author. Fax: +91 11 26588663. E-mail address: [email protected] (V. Bhatnagar). 0022-3468/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2011.02.001
bilirubin and serum alkaline phosphatase were 11.7 mg% and 3099 IU, respectively. Serum glutamic oxaloacetic transaminase and serum glutamic pyruvic transaminase were 330 and 257 IU, respectively. Ultrasonography revealed a 4 × 3-cm solid intraluminal CBD mass that was lobulated and metacentric; the gall bladder and intrahepatic bile ducts were dilated. Magnetic resonance cholangiopancreatography showed an intraluminal CBD mass extending from the confluence of the right and left hepatic ducts to the lower end of the CBD with a large extraluminal component infiltrating the head of the pancreas (Fig. 1). Facilities for endoscopic retrograde cholangiopancreaticography in infants were not available. Ultrasonography-guided fine-needle aspiration cytology of the mass showed a malignant lesion; further characterization was not possible. Preoperative serum αfetoprotein (AFP) level was 1485 ng/mL. Because the imaging studies had suggested a predominantly intraluminal mass with infiltration into the head of pancreas, a differential diagnosis of rhabdomyosarcoma was considered. An exploratory laparotomy was planned because a definitive histologic diagnosis could not be obtained. At exploratory laparotomy, the intraluminal CBD mass was palpated, and the large extraluminal component seemed
1272
Fig. 1 Magnetic resonance imaging showing an intraluminal mass in the CBD (black arrow) with a large extraluminal component infiltrating into the head of the pancreas (white arrow).
to involve the pancreatic head and infiltrate the duodenum. There was no evidence of ascites or intraperitoneal spread. All the other organs were normal. The entire CBD distal to the biliary confluence, gall bladder, head of the pancreas, gastric antrum, and duodenum were resected en bloc. Gastrointestinal continuity was restored by an end-to-end gastrojejunostomy. Bilioenteric and pancreaticoenteric continuity was restored using a Roux-en-Y loop. The pylorus could not be preserved because a safe margin of resection was not possible in the duodenum. Postoperatively, the patient recovered well, and the jaundice disappeared over a period of 7 days. Histopathologic examination showed that tumor cells were positive for pancytokeratin and AFP. The tumor was infiltrating into the duodenum and pancreas. Resected margins were free of tumor. A histologic diagnosis of primary yolk sac tumor of the CBD infiltrating the pancreas and duodenum was established. Subsequent to the histopathology report, the patient received six 3 weekly cycles of chemotherapy with cisplatin (35 mg/m2 per day × 3 days), Etoposide (120 mg/m2 per day × 3 days) and bleomycin (15 mg/m2 per day × 3 days). Her post chemotherapy serum AFP level was 1.04 ng/mL. At 1-year follow-up after surgery, she is asymptomatic, feeding well, and gaining weight, and the serum AFP is 1.23 ng/mL.
2. Discussion Primary solid tumors arising in the CBD in children are rare. Although, rhabdomyosarcoma has been reported, extraluminal tumors, for example, pancreatoblastoma causing compression of the CBD, can also be considered in the differential diagnosis. Choledochal cysts, the most common pathologic lesions of the CBD in small children, are easily excluded by ultrasonography and magnetic resonance imaging. Yolk sac tumor is the most common malignant pediatric germ cell tumor in children [1]. The term “germ cell tumor”
G.S. Munghate et al. refers to the tumors arising from immature germ cells. Germ cell tumors can be divided into gonadal and extragonadal types; extragonadal germ cell tumors develop from primordial germ cells that have been misplaced or arrested during their migration into the gonads. Extragonadal germ cell tumors often arise along the midline of the body, that is, at the cranium near the pineal body, the neck, the mediastinum, retroperitoneum, and sacrum [7]. Primary YSTs have been reported in the retroperitoneum, brain, pineal body, pelvis, and lung [3-6,8]. As far as we can determine, primary YST of the CBD has not been previously reported in the English literature. In the present case, there was no evidence of tumor arising from the ovaries. Hence, the lesion is considered a primary tumor arising from the CBD and infiltrating the pancreas and duodenum. This was initially suggested by the imaging studies and later confirmed on histopathologic examination. There were no calcifications noted on the imaging studies. Surgical management of malignant lesions can be variable. Malignant lesions that are large and have a definitive histopathologic diagnosis can be treated with neoadjuvant chemotherapy. The reduction in tumor size makes surgical resection easier and less extensive. In cases that do not have a histopathologic diagnosis but are resectable, complete surgical excision is indicated. In unresectable lesions, initial debulking or biopsy followed by chemotherapy and a second-look surgical procedure to achieve complete excision is recommended. In this case, preoperative ultrasonography-guided fine-needle aspiration cytology revealed malignant cells but was not sufficient to clearly determine the tumor type. Moreover, a complete resection of the tumor was feasible. Hence, surgery was selected as the primary modality of treatment and was conducted safely and effectively. In retrospect, this case may have been treated with a less extensive surgical resection if a repeat biopsy could have given a more accurate tissue diagnosis, as the tumors considered in the differential diagnosis are known to respond well to chemotherapy. Over the past 2 decades, the cure rate for germ cell tumors has markedly improved. This is largely due to advances in chemotherapy [9,10]. Currently, cisplatin, etoposide, and bleomycin are generally administered as a standard chemotherapy regimen given in 4 cycles for gonadal tumors in accordance with the Pediatric Oncology Group/Childrens Cancer Group (POG/CCG) guidelines [11-13]. Because the tumor was extragonadal and highly infiltrative in this case, it was decided to administer 6 cycles of chemotherapy instead of 4. The serum AFP levels normalized after resection and postoperative chemotherapy, thus signifying complete removal of the tumor.
References [1] Kanto S, Saito H, Ito A, et al. Clinical features of testicular tumors in children. Int J Urol 2004;11:890-3. [2] Nerli RB, Ajay G, Shivangouda P, et al. Prepubertal testicular tumors: our 10 years experience. Indian J Cancer 2010;47:292-5.
Primary yolk sac tumor of CBD [3] O'Sullivan P, Daneman A, Chan HS, et al. Extragonadal endodermal sinus tumors in children: a review of 24 cases. Pediatr Radiol 1983;13:249-57. [4] Kiratli H, Erkan Balci K, Güler G, et al. Primary orbital endodermal sinus tumor (yolk sac tumor). J AAPOS 2008;12:623-5. [5] Filho BC, McHugh JB, Carrau RL, et al. Yolk sac tumor in the nasal cavity. Am J Otolaryngol 2008;29:250-4. [6] Abdel Rahman AR, Ebied EN, Nouh MA, et al. Primary yolk sac tumor of the lung. Ann Thorac Surg 2009;87:1925-6. [7] Nakazawa R, Soneda S, Kinoshita A, et al. A case report: primary extragonadal yolk sac tumor of penile shaft in a 2-year-old child. Int J Urol 2009;16:413-5. [8] Pasternack T, Shaco-Levy R, Wiznitzer A, et al. Extraovarian pelvic yolk sac tumor: case report and review of published work. J Obstet Gynaecol Res 2008;34:739-44. [9] Cushing B, Giller R, Cullen JW, et al. Randomized comparison of combination chemotherapy with etoposide, bleomycin, and either high-dose or standard-dose cisplatin in children and adolescents with
1273
[10]
[11]
[12]
[13]
high-risk malignant germ cell tumors: a pediatric intergroup study— Pediatric Oncology Group 9049 and Children's Cancer Group 8882. J Clin Oncol 2004;22:2691-700. Rogers PC, Olson TA, Cullen JW, et al. Treatment of children and adolescents with stage II testicular and stages I and II ovarian malignant germ cell tumors: a pediatric intergroup study—Pediatric Oncology Group 9048 and Children's Cancer Group 8891. J Clin Oncol 2004;22:3563-9. Rescorla F, Billmire D, Stolar C, et al. The effect of cisplatin dose and surgical resection in children with malignant germ cell tumors at the sacrococcygeal region: a pediatric intergroup trial (POG 9049/CCG 8882). J Pediatr Surg 2003;38:12-7. Billmire D, Vinocur C, Rescorla F, et al. Malignant retroperitoneal and abdominal germ cell tumors: an intergroup study. J Pediatr Surg 2003;38:315-8. Billmire D, Vinocur C, Rescorla F, et al. Malignant mediastinal germ cell tumors: an intergroup study. J Pediatr Surg 2003;36:18-24.
www.elsevier.com/locate/jpedsurg
Primary yolk sac tumor of the common bile duct Gayatri S. Munghate, Sandeep Agarwala, Veereshwar Bhatnagar ⁎ The Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi-110029, India Received 3 October 2010; revised 3 February 2011; accepted 4 February 2011
Key words: Yolk sac tumor; Endodermal sinus tumor; Extragonadal yolk sac tumor; Common bile duct; Obstructive jaundice
Abstract Yolk sac tumor (YST) is a germ cell neoplasm usually arising from the gonads. Extragonadal YST has been reported in the mediastinum, retroperitoneum, liver, and pelvis. Primary YST of the common bile duct (CBD) has not been previously reported. We describe an 11-month-old girl with a YST arising from the CBD who presented with obstructive jaundice. Imaging studies revealed an intraluminal CBD mass with intrahepatic biliary dilatation and extraluminal infiltration of the head of the pancreas. A complete resection was carried out, and postoperative chemotherapy with cisplatin, etoposide and bleomycin was administered. © 2011 Elsevier Inc. All rights reserved.
Yolk sac tumors (YSTs; endodermal sinus tumor) are the most common pediatric germ cell tumors, accounting for 20% of cases, occurring primarily in the gonads [1,2]. Extragonadal YSTs occur in 10% to 15% of the patients. The common sites for extragonadal YST are mediastinum, vagina, brain, and retroperitoneum [3]. Sporadic cases of YST at rare sites e.g. orbit, nasal cavity and lung have been reported [4-6]. This report describes a primary YST of the common bile duct (CBD), possibly the first of its kind.
1. Case report An 11-month-old girl presented with increasing jaundice, dark urine, clay colored stools, and abdominal distension of 1.5-month duration. During this time, she also had intermittent low-grade fever. The perinatal history was unremarkable. Physical examination showed an obviously jaundiced infant with a firm and nontender liver edge palpated 4.0 cm below the right costal margin. Serum ⁎ Corresponding author. Fax: +91 11 26588663. E-mail address: [email protected] (V. Bhatnagar). 0022-3468/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2011.02.001
bilirubin and serum alkaline phosphatase were 11.7 mg% and 3099 IU, respectively. Serum glutamic oxaloacetic transaminase and serum glutamic pyruvic transaminase were 330 and 257 IU, respectively. Ultrasonography revealed a 4 × 3-cm solid intraluminal CBD mass that was lobulated and metacentric; the gall bladder and intrahepatic bile ducts were dilated. Magnetic resonance cholangiopancreatography showed an intraluminal CBD mass extending from the confluence of the right and left hepatic ducts to the lower end of the CBD with a large extraluminal component infiltrating the head of the pancreas (Fig. 1). Facilities for endoscopic retrograde cholangiopancreaticography in infants were not available. Ultrasonography-guided fine-needle aspiration cytology of the mass showed a malignant lesion; further characterization was not possible. Preoperative serum αfetoprotein (AFP) level was 1485 ng/mL. Because the imaging studies had suggested a predominantly intraluminal mass with infiltration into the head of pancreas, a differential diagnosis of rhabdomyosarcoma was considered. An exploratory laparotomy was planned because a definitive histologic diagnosis could not be obtained. At exploratory laparotomy, the intraluminal CBD mass was palpated, and the large extraluminal component seemed
1272
Fig. 1 Magnetic resonance imaging showing an intraluminal mass in the CBD (black arrow) with a large extraluminal component infiltrating into the head of the pancreas (white arrow).
to involve the pancreatic head and infiltrate the duodenum. There was no evidence of ascites or intraperitoneal spread. All the other organs were normal. The entire CBD distal to the biliary confluence, gall bladder, head of the pancreas, gastric antrum, and duodenum were resected en bloc. Gastrointestinal continuity was restored by an end-to-end gastrojejunostomy. Bilioenteric and pancreaticoenteric continuity was restored using a Roux-en-Y loop. The pylorus could not be preserved because a safe margin of resection was not possible in the duodenum. Postoperatively, the patient recovered well, and the jaundice disappeared over a period of 7 days. Histopathologic examination showed that tumor cells were positive for pancytokeratin and AFP. The tumor was infiltrating into the duodenum and pancreas. Resected margins were free of tumor. A histologic diagnosis of primary yolk sac tumor of the CBD infiltrating the pancreas and duodenum was established. Subsequent to the histopathology report, the patient received six 3 weekly cycles of chemotherapy with cisplatin (35 mg/m2 per day × 3 days), Etoposide (120 mg/m2 per day × 3 days) and bleomycin (15 mg/m2 per day × 3 days). Her post chemotherapy serum AFP level was 1.04 ng/mL. At 1-year follow-up after surgery, she is asymptomatic, feeding well, and gaining weight, and the serum AFP is 1.23 ng/mL.
2. Discussion Primary solid tumors arising in the CBD in children are rare. Although, rhabdomyosarcoma has been reported, extraluminal tumors, for example, pancreatoblastoma causing compression of the CBD, can also be considered in the differential diagnosis. Choledochal cysts, the most common pathologic lesions of the CBD in small children, are easily excluded by ultrasonography and magnetic resonance imaging. Yolk sac tumor is the most common malignant pediatric germ cell tumor in children [1]. The term “germ cell tumor”
G.S. Munghate et al. refers to the tumors arising from immature germ cells. Germ cell tumors can be divided into gonadal and extragonadal types; extragonadal germ cell tumors develop from primordial germ cells that have been misplaced or arrested during their migration into the gonads. Extragonadal germ cell tumors often arise along the midline of the body, that is, at the cranium near the pineal body, the neck, the mediastinum, retroperitoneum, and sacrum [7]. Primary YSTs have been reported in the retroperitoneum, brain, pineal body, pelvis, and lung [3-6,8]. As far as we can determine, primary YST of the CBD has not been previously reported in the English literature. In the present case, there was no evidence of tumor arising from the ovaries. Hence, the lesion is considered a primary tumor arising from the CBD and infiltrating the pancreas and duodenum. This was initially suggested by the imaging studies and later confirmed on histopathologic examination. There were no calcifications noted on the imaging studies. Surgical management of malignant lesions can be variable. Malignant lesions that are large and have a definitive histopathologic diagnosis can be treated with neoadjuvant chemotherapy. The reduction in tumor size makes surgical resection easier and less extensive. In cases that do not have a histopathologic diagnosis but are resectable, complete surgical excision is indicated. In unresectable lesions, initial debulking or biopsy followed by chemotherapy and a second-look surgical procedure to achieve complete excision is recommended. In this case, preoperative ultrasonography-guided fine-needle aspiration cytology revealed malignant cells but was not sufficient to clearly determine the tumor type. Moreover, a complete resection of the tumor was feasible. Hence, surgery was selected as the primary modality of treatment and was conducted safely and effectively. In retrospect, this case may have been treated with a less extensive surgical resection if a repeat biopsy could have given a more accurate tissue diagnosis, as the tumors considered in the differential diagnosis are known to respond well to chemotherapy. Over the past 2 decades, the cure rate for germ cell tumors has markedly improved. This is largely due to advances in chemotherapy [9,10]. Currently, cisplatin, etoposide, and bleomycin are generally administered as a standard chemotherapy regimen given in 4 cycles for gonadal tumors in accordance with the Pediatric Oncology Group/Childrens Cancer Group (POG/CCG) guidelines [11-13]. Because the tumor was extragonadal and highly infiltrative in this case, it was decided to administer 6 cycles of chemotherapy instead of 4. The serum AFP levels normalized after resection and postoperative chemotherapy, thus signifying complete removal of the tumor.
References [1] Kanto S, Saito H, Ito A, et al. Clinical features of testicular tumors in children. Int J Urol 2004;11:890-3. [2] Nerli RB, Ajay G, Shivangouda P, et al. Prepubertal testicular tumors: our 10 years experience. Indian J Cancer 2010;47:292-5.
Primary yolk sac tumor of CBD [3] O'Sullivan P, Daneman A, Chan HS, et al. Extragonadal endodermal sinus tumors in children: a review of 24 cases. Pediatr Radiol 1983;13:249-57. [4] Kiratli H, Erkan Balci K, Güler G, et al. Primary orbital endodermal sinus tumor (yolk sac tumor). J AAPOS 2008;12:623-5. [5] Filho BC, McHugh JB, Carrau RL, et al. Yolk sac tumor in the nasal cavity. Am J Otolaryngol 2008;29:250-4. [6] Abdel Rahman AR, Ebied EN, Nouh MA, et al. Primary yolk sac tumor of the lung. Ann Thorac Surg 2009;87:1925-6. [7] Nakazawa R, Soneda S, Kinoshita A, et al. A case report: primary extragonadal yolk sac tumor of penile shaft in a 2-year-old child. Int J Urol 2009;16:413-5. [8] Pasternack T, Shaco-Levy R, Wiznitzer A, et al. Extraovarian pelvic yolk sac tumor: case report and review of published work. J Obstet Gynaecol Res 2008;34:739-44. [9] Cushing B, Giller R, Cullen JW, et al. Randomized comparison of combination chemotherapy with etoposide, bleomycin, and either high-dose or standard-dose cisplatin in children and adolescents with
1273
[10]
[11]
[12]
[13]
high-risk malignant germ cell tumors: a pediatric intergroup study— Pediatric Oncology Group 9049 and Children's Cancer Group 8882. J Clin Oncol 2004;22:2691-700. Rogers PC, Olson TA, Cullen JW, et al. Treatment of children and adolescents with stage II testicular and stages I and II ovarian malignant germ cell tumors: a pediatric intergroup study—Pediatric Oncology Group 9048 and Children's Cancer Group 8891. J Clin Oncol 2004;22:3563-9. Rescorla F, Billmire D, Stolar C, et al. The effect of cisplatin dose and surgical resection in children with malignant germ cell tumors at the sacrococcygeal region: a pediatric intergroup trial (POG 9049/CCG 8882). J Pediatr Surg 2003;38:12-7. Billmire D, Vinocur C, Rescorla F, et al. Malignant retroperitoneal and abdominal germ cell tumors: an intergroup study. J Pediatr Surg 2003;38:315-8. Billmire D, Vinocur C, Rescorla F, et al. Malignant mediastinal germ cell tumors: an intergroup study. J Pediatr Surg 2003;36:18-24.