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Liver metastasis in an adolescent treated for third ventricle germ cell tumor

CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2014; 35(02): 184-186

DOI: DOI: 10.4103/0971-5851.138999

Abstract

Systemic dissemination of intracranial germ cell tumors (GCTs) occur only in 3% of cases and the common sites are bone, lungs, and lymph nodes. Metastasis to the liver is rare. As far as we could find, only six cases of liver metastasis of intracranial GCTs have been reported so far. We report an adolescent girl who presented with hepatic relapse 2½ years after successful completion of treatment of intracranial GCT. She was treated with chemotherapy and right hepatectomy and is doing well 30 months after treatment for the metastatic disease.

Publication History

Article published online:
19 July 2021

© 2014. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

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Abstract

Systemic dissemination of intracranial germ cell tumors (GCTs) occur only in 3% of cases and the common sites are bone, lungs, and lymph nodes. Metastasis to the liver is rare. As far as we could find, only six cases of liver metastasis of intracranial GCTs have been reported so far. We report an adolescent girl who presented with hepatic relapse 2½ years after successful completion of treatment of intracranial GCT. She was treated with chemotherapy and right hepatectomy and is doing well 30 months after treatment for the metastatic disease.

Keywords: Intracranial germ cell tumorliver metastasisextra cranial metastasis

INTRODUCTION

Intracranial germ cell tumors (GCTs) account for about 2% of brain tumors in children in India.[] Spread of tumor outside the primary site occurs in 10-30% of cases,[] however, metastasis outside the central nervous system is rare. We report a case of liver metastasis in an adolescent, 2½ years after treatment for third ventricular GCT.

CASE REPORT

Initial presentation

A 14-year-old girl presented to us in 2008 with the complaints of headache, vomiting, and giddiness of 3 months duration. On examination, she had left hemiparesis and features of raised intracranial pressure. Magnetic resonance imaging (MRI) scan revealed an ill-defined nonenhancing, heterogeneous mass in the third ventricle measuring 4 cm × 3.5 cm × 3.5 cm. Both lateral ventricles were dilated, fourth ventricle was normal in size and the third ventricle not seen separately seen from mass. The sella and pituitary were normal.

A ventriculo-peritoneal (VP) shunt was placed as an emergency procedure. Serum and cerebrospinal fluid (CSF) alpha fetoprotein (AFP) were normal, however beta human chorionic gonadotrophin (HCG) levels were raised (13.0 mIU/mL in serum and 127 mIU/mL in CSF - normal <5 xss=removed>2 on day 1), etoposide (100 mg/m2 days 1-3 and days 22-24) and, ifosfamide (1800 mg/m2 on days 22-26), followed by radiotherapy, 5040 cGy in 28 fractions.

Serum beta HCG levels came down to <1>

Hepatic relapse

Two and a half years after completion of treatment, she presented with progressive abdominal distension of 1-month duration. She was deeply icteric; a hard nodular mass occupied the entire right side of the abdomen. Liver function tests were deranged with total bilirubin-22.7 mg%, direct bilirubin-21.5 mg%, total protein-7.0 g%, albumin-3.1 g%, serum glutamic oxaloacetic transaminase-241U/L, serum glutamic-pyruvic transaminase - 44U/L, alkaline phosphatase-615U/L prothrombin time international normalized ratio 1.98, beta HCG-199.3 mIU/mL, AFP-1.04 IU/mL, hepatitis B and C screen was negative. CT scan of abdomen showed a large predominantly cystic lesion with foci of solid enhancing areas occupying the right lobe of liver (segments 5, 6 and 7) with extension to segment 4B of left lobe, measuring approximately 12 cm × 8 cm × 9 cm. The mass was infiltrating the primary hilar confluence with intrahepatic biliary radicular dilatation and was encasing the gall bladder. There was significant periportal adenopathy. There was only mild ascites and the bowel, mesentery and omentum were normal. VP shunt was in situ. MRI brain did not show any recurrence and MRI of spine was normal.

She received a total of five courses of chemotherapy using carboplatin 600 mg/m2 and bleomycin 15 mg/m2. Carboplatin alone was given in the first course in view of her poor clinical condition. Following this her general condition improved, jaundice reduced and beta HCG dropped to 15.7 mIU/mL. After the third course of chemotherapy beta HCG level normalized and has remained normal since then. She underwent right hepatectomy with excision of segment 4B after four courses of chemotherapy. The tumor was found to occupy the whole of the right lobe except a small portion of segment 8. There were two periportal lymph nodes which were also excised and the peritoneal end of the VP shunt was removed. No ascites or peritoneal deposits were seen. Histopathology revealed a well-circumscribed tumor with extensive necrosis, foci of recent and old hemorrhage, chronic inflammation and fibrosis with areas of hyalinization. Immunohistochemistry was positive for placental alkaline phosphatase and CD30 which was consistent with GCT. The postoperative period was uneventful. After she recovered from surgery, she received the fifth course of chemotherapy.

She has been on regular follow-up and is well 30 months after the completion of relapse treatment.

DISCUSSION

Extracranial metastases of primary brain tumors are uncommon and those from GCT tumors are rare. Absence of lymphatic vessels in the brain and enclosure of the intracranial sinuses in a dense dural membrane may be factors, which are responsible for the rarity of systemic metastasis in brain tumors. Abdominal metastases have been associated with VP shunts[] and neurosurgical procedures have been regarded as possible factors for systemic dissemination.[]

Campbell et al.[] did a clinical and pathological review of 917 cases of pediatric primary brain tumors and found only 21 cases with systemic metastasis (2.3%), 15 of which were medulloblastomas, and only two were GCT (teratoma and endodermal sinus tumor). Varan et al.[] had reported extra-neural metastasis in only 10 out of 1,011 patients (0.98%) with pediatric brain tumors, of which two had GCT, one with lung and the other with liver metastasis.

Jennings et al.[] reviewed 389 published cases of intracranial GCT. They found peritoneal and pelvic metastases in 10% of cases with VP shunt in situ, however only 3% had systemic dissemination which was mainly to the lung and bones.

Common sites for hematogeneous metastases of intracranial GCT are bone, lung, lymph nodes and soft tissue.[,] Metastasis to the liver, like our patient had, seems to be rare. We were able to find 6 reports of liver metastasis in patients with intracranial GCT, three were alive at the time of reporting and three[,,] were autopsy findings with liver metastasis noted along with multiple other metastases. Of the three that were surviving at time of report two,[,] were doing well and one[] had progressive disease and was on treatment.

Platinum-based chemotherapy seems to be the choice of treatment for intracranial GCT that has systemic dissemination.[] Our patient was treated with carboplatin and bleomycin and required surgical excision as well. She is doing well 30 months after completion of treatment for metastatic disease

CONCLUSION

Liver is a rare site of extra-neural relapse of intracranial GCT. Platinum-based chemotherapy and complete resection may lead to long-term survival in these patients.

ACKNOWLEDGEMENTS

We acknowledge Dr. Ari Chacko, Professor of Neurosurgery, Christian Medical College and Dr. Narendra Chaudhury, Assistant professor, Paediatric Hematology Oncology, Christian Medical College, Vellore for their contribution.

Footnotes

Source of Support: Nil

Conflict of Interest: None declared.

References

  1. Jain A, Sharma MC, Suri V, Kale SS, Mahapatra AK, Tatke M, et al. Spectrum of pediatric brain tumors in India : a0 multi-institutional study. Neurol India 2011;59:208-11.
  2. Jennings MT, Gelman R, Hochberg F. Intracranial germ-cell tumors : n0 atural history and pathogenesis. J Neurosurg 1985;63:155-67.
  3. Asanuma M, Aoyama T, Sakai K, Asano K, Uehara T, Hongo K. Hematogenous extraneural metastasis of the germinomatous component of a pineal mixed germ cell tumor. Brain Tumor Pathol 2012;29:245-50.
  4. Campbell AN, Chan HS, Becker LE, Daneman A, Park TS, Hoffman HJ. Extracranial metastases in childhood primary intracranial tumors. A report of 21 cases and review of the literature. Cancer 1984;53:974-81.
  5. Varan A, Sari N, Akalan N, Söylemezoglu F, Akyüz C, Kutluk T, et al. Extraneural metastasis in intracranial tumors in children : t0 he experience of a single center. J Neurooncol 2006;79:187-90.
  6. Galassi E, Tognetti F, Frank F, Gaist G. Extraneural metastases from primary pineal tumors. Review of the literature. Surg Neurol 1984;21:497-504.
  7. Shinmura F, Takayasu K, Sakata R, Ariwa R, Takagi S. Autopsy case of extra-neural metastasis of suprasellar pineal germinoma. Gan No Rinsho 1983;29:832-7.
  8. Motomochi M, Makita Y, Nabeshima S, Aoyama I, Ichijima K, Yamabe H, et al. A case of intracranial germinoma with multiple remote metastases (author′s transl). No Shinkei Geka 1980;8:563-70.
  9. Jennings CD, Powell DE, Walsh JW, Mortara RH. Suprasellar germ cell tumor with extracranial metastases. Neurosurgery 1985;16:9-12.
  10. Adachi J, Ono N, Misumi S, Tamura M, Ohye C. Multiple liver metastases of a suprasellar germ cell tumor treated with combined chemotherapy of cisplatin and etoposide. No Shinkei Geka 1991;19:671-5.

References

  1. Jain A, Sharma MC, Suri V, Kale SS, Mahapatra AK, Tatke M, et al. Spectrum of pediatric brain tumors in India : a0 multi-institutional study. Neurol India 2011;59:208-11.
  2. Jennings MT, Gelman R, Hochberg F. Intracranial germ-cell tumors : n0 atural history and pathogenesis. J Neurosurg 1985;63:155-67.
  3. Asanuma M, Aoyama T, Sakai K, Asano K, Uehara T, Hongo K. Hematogenous extraneural metastasis of the germinomatous component of a pineal mixed germ cell tumor. Brain Tumor Pathol 2012;29:245-50.
  4. Campbell AN, Chan HS, Becker LE, Daneman A, Park TS, Hoffman HJ. Extracranial metastases in childhood primary intracranial tumors. A report of 21 cases and review of the literature. Cancer 1984;53:974-81.
  5. Varan A, Sari N, Akalan N, Söylemezoglu F, Akyüz C, Kutluk T, et al. Extraneural metastasis in intracranial tumors in children : t0 he experience of a single center. J Neurooncol 2006;79:187-90.
  6. Galassi E, Tognetti F, Frank F, Gaist G. Extraneural metastases from primary pineal tumors. Review of the literature. Surg Neurol 1984;21:497-504.
  7. Shinmura F, Takayasu K, Sakata R, Ariwa R, Takagi S. Autopsy case of extra-neural metastasis of suprasellar pineal germinoma. Gan No Rinsho 1983;29:832-7.
  8. Motomochi M, Makita Y, Nabeshima S, Aoyama I, Ichijima K, Yamabe H, et al. A case of intracranial germinoma with multiple remote metastases (author′s transl). No Shinkei Geka 1980;8:563-70.
  9. Jennings CD, Powell DE, Walsh JW, Mortara RH. Suprasellar germ cell tumor with extracranial metastases. Neurosurgery 1985;16:9-12.
  10. Adachi J, Ono N, Misumi S, Tamura M, Ohye C. Multiple liver metastases of a suprasellar germ cell tumor treated with combined chemotherapy of cisplatin and etoposide. No Shinkei Geka 1991;19:671-5.