Abstract
Introduction
Ventriculoperitoneal shunts (VPS) have been implicated as a source of the extraneural spread of a wide variety of central nervous system tumors. The purpose is to review the literature on peritoneal seeding of central nervous system tumors from VPS in the context of a case report.
Methods
Medline was searched using the phrase ‘peritoneal seeding ventriculoperitoneal shunt’. Inclusion criteria included patients (<18 years) with evidence of peritoneal seeding from VPS.
Results
Search of the literature revealed a final total of 22 articles and a total of 28 patients.
Case report
A 7-year-old boy presented with intermittent vomiting, headaches, photophobia; a 4.4 cm left thalamic mass (glioblastoma multiforme) was found. Occipital VPS catheters were placed for increasing hydrocephalus and the patient developed increased abdominal distention and pain. Computed tomography revealed diffuse ascites with carcinomatosis and the patient was diagnosed clinically with peritoneal metastases.
Discussion
Our case report and literature review revealed 28 cases of central nervous system tumors demonstrating evidence of extraneural spread associated with VPS in children in a wide variety of tumors. Larger studies are required to evaluate VPS as potential risk factors for peritoneal seeding and familiarity with potential VPS-related peritoneal seeding is important for diagnostic consideration.
Keywords: Ventriculoperitoneal shunt, glioblastoma, peritoneal seeding
Introduction
Ventriculoperitoneal shunts (VPS) have been implicated as a source of the extraneural spread of a wide variety of central nervous system tumors; however, there have been few reports focusing on VPS in pediatric patients. The purpose of this report is to review the literature on peritoneal seeding of pediatric central nervous system tumors from VPS in the context of a case of peritoneal seeding of a glioblastoma multiforme probably from a VPS.
Materials and methods
The Medline database was searched using the phrase ‘peritoneal seeding ventriculoperitoneal shunt’ on 1 November 2014. Inclusion criteria included reports of patients younger than 18 years with evidence of peritoneal seeding from VPS. Articles were selected for inclusion in the report by the first author (AN) with experience performing systematic reviews. Additional articles were derived from reference lists of included articles.
Case report
The patient was a previously healthy 7-year-old boy who was transferred to our hospital in December 2013 after presenting with three weeks of intermittent vomiting, headaches, photophobia, eye pain and gait disturbances. Initial head computed tomography (CT) revealed a 4.4 cm mass lesion centered in the left thalamus with internal hemorrhage, mass effect and evidence of extension into the left lateral ventricle with mild left-sided hydrocephalus, particularly of the atria and occipital horn of the lateral ventricle. A subsequent magnetic resonance imaging (MRI) evaluation of the spine was negative for tumor seeding. The child was started on dexamethasone and acetazolamide for treatment of the mass effect and edema. The boy then underwent partial resection of the left thalamic lesion with placement of an external ventricular drain on 20 December 2013. Follow-up imaging revealed stability of the ventricular size so the external ventricular drain was removed on 25 December. Final pathology revealed glioblastoma multiforme grade IV.
The child subsequently received one fraction of radiation in February 2014 and was initially planned to be treated with daily temozolomide with the inclusion of bevacizumab; however, the family elected to pursue homeopathic treatments. In March 2014, he presented with vision changes including blurring and ‘horizontal double vision’. Brain MRI revealed markedly increased size of the left thalamic mass and mass effect on ventricular system resulting in moderate bilateral hydrocephalus. At the time, the patient had no known metastases. He patient had a right-sided occipital VPS catheter placed at the end of March 2014. Additional subtotal tumor resection was performed in early April 2014 followed by chemotherapy and radiation. On follow-up imaging in July 2014, areas of new tumor extension were noted involving the left cerebral peduncle; treatment with temozolomide and valproic acid was started per protocol. In early October 2014, he presented with multiple episodes of vomiting and increasing headaches. Repeat MRI revealed a trapped left ventricular system for which a left occipital approach VPS was installed on 9 October (Figure 1). At the time, the patient had no known metastases. Approximately 2 weeks afterwards, the patient noted increased lethargy, emesis, increased abdominal distention and vague complaints of abdominal pain. CT scan of the abdomen revealed diffuse ascites with evidence of peritoneal carcinomatosis, most likely secondary to tumor seeding by the VPS (Figure 1). He patient underwent therapeutic paracenteses, which revealed atypical cells in the first paracentesis and markedly increased cellularity in the second paracentesis. The patient was assumed clinically to have peritoneal metastases and additional work-up revealed extensive leptomeningeal carcinomatosis involving the cord and cauda equina with areas of near complete replacement of cord by tumor. The patient was sent to hospice care, where he died within a month. Autopsy was not performed to confirm or reveal new sites of metastatic involvement.
Figure 1.
The top two images are axial T2 images of the brain demonstrating the large left thalamic glioblastoma with adjacent ventriculoperitoneal shunt catheter tips. The bottom two images demonstrate axial and coronal contrast enhanced computed tomography images of the abdomen with peritoneal nodularity with associated ascites.
Literature search
Search of the literature revealed 27 articles, of which 16 fulfilled the inclusion criteria. Search of reference lists revealed six additional articles for a final total of 22 articles (Table 1).
Table 1.
Literature review.
| Sample size | Age (years) | Sex | Tumor type | Reference |
|---|---|---|---|---|
| 1 | 7 | M | Glioblastoma (grade IV) | Current |
| 1 | 9 | F | Atypical teratoid/rhabdoid tumor | 1 |
| 1 | 5 | M | Medulloblastoma | 2 |
| 1 | 13 | F | Pineal germinoma | 3 |
| 1 | 0.5 | M | Pilocytic astrocytoma | 4 |
| 1 | 8 | M | Medulloblastoma | 5 |
| 1 | 13 | M | Glioblastoma (grade IV) | 6 |
| 1 | 1 | F | Medulloblastoma | 7 |
| 1 | 4 | F | Pilocytic astrocytoma | 8 |
| 1 | 15 | M | Pineal germinoma | 9 |
| 1 | 1 | M | Primitive neuroectodermal tumor | 10 |
| 1 | 15 | M | Pineal germinoma | 11 |
| 1 | 17 | M | Endodermal sinus tumor | 12 |
| 1 | 9 | M | Ependymoblastoma | 13 |
| 1 | 7 | M | Mixed malignant germ cell tumor | 14 |
| 1 | 12 | F | Pineoblastoma | 15 |
| 3 | 11, 13, 15 | M, F, M | Pineal dysgerminoma | 16 |
| 1 | Not reported | Not reported | Medulloblastoma | 18 |
| 1 | 13 | F | Pilomyxoid astrocytoma | 19 |
| 5 | 11, 7, 6, 5, 3 | M, M, M, M, F | Medulloblastoma | 20 |
| 1 | 2 | F | Medulloblastoma | 21 |
| 1 | 6 | M | Medulloblastoma | 22 |
Discussion
Our case report and review of the literature revealed 28 cases1–22 of central nervous system tumors demonstrating evidence of extraneural spread associated with VPS in children. In patients of widely varying ages and similar gender distributions a wide variety of tumors were reported, with medulloblastomas and pineal tumors cited most commonly.
Although several reports have implicated VPS as sources of the extraneural spread of central nervous system malignancies, there are limited data evaluating VPS as risk factors for peritoneal seeding. In one report comparing patients with central nervous system malignancies, similar numbers of patients were found with extraneural involvement in patients with and without VPS; however, the study was limited by small numbers of patients who developed the outcome of interest.23 In the absence of such data, VPS should be considered as potential causes for increasing abdominal distention and ascites in patients with histories of intracranial malignancies. Larger retrospective and/or prospective cohorts are required to evaluate VPS as potential risk factors for peritoneal seeding. Familiarity with potential VPS-related peritoneal seeding is, however, of importance for each physician in order to prevent misdiagnosis. Review of the literature showed that VPS-related peritoneal seeding occurred primarily with high grade primary brain neoplasms.
Funding
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflict of interest
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
References
- 1.Han YP, Zhao Y, He XG, et al. Peritoneal metastasis of third ventricular atypical teratoid/rhabdoid tumor after VP shunt implantation for unexplained hydrocephalus. World J Pediatr 2012; 8(4): 367–370. doi: 10.1007/s12519-012-0384-y. [DOI] [PubMed] [Google Scholar]
- 2.Pettersson D, Schmitz KR, Pollock JM, et al. Medulloblastoma: seeding of VP shunt tract and peritoneum. Clin Pract 2012; 2(2): e37.. doi: 10.4081/cp.2012.e37. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 3.Murray MJ, Metayer LE, Mallucci CL, et al. Intra-abdominal metastasis of an intracranial germinoma viaventriculo-peritoneal shunt in a 13-year-old female. Br J Neurosurg 2011; 25(6): 747–749. doi: 10.3109/02688697.2011.566383. [DOI] [PubMed] [Google Scholar]
- 4.Pollack IF, Hurtt M, Pang D, et al. Dissemination of low grade intracranial astrocytomas in children. Cancer 1994; 73(11): 2869–2878. [DOI] [PubMed] [Google Scholar]
- 5.Coley BD, Fischbein NJ. Abdominal masses in an eight-year-old boy. Invest Radiol 1994; 29(6): 656–658. [DOI] [PubMed] [Google Scholar]
- 6.Newton HB, Rosenblum MK, Walker RW. Extraneural metastases of infratentorial glioblastoma multiforme to the peritoneal cavity. Cancer 1992; 69(8): 2149–2153. [DOI] [PubMed] [Google Scholar]
- 7.Oemus K, Gerlach H, Rath FW. [A rare complication of shunt therapy. Metastasis of brain tumors by cerebrospinal fluid drainage]. Zentralbl Neurochir 1992; 53(1): 25–32. [PubMed] [Google Scholar]
- 8.Jiménez-Jiménez FJ, Garzo-Fernández C, De Inovencio-Arocena J, et al. Extraneural metastases from brainstem astrocytoma through ventriculoperitoneal shunt. J Neurol Neurosurg Psychiatry 1991; 54(3): 281–282. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 9.Pallini R, Bozzini V, Scerrati M, et al. Bone metastasis associated with shunt-related peritoneal deposits from a pineal germinoma. Case report and review of the literature. Acta Neurochir (Wien) 1991; 109(1–2): 78–83. [DOI] [PubMed] [Google Scholar]
- 10.Yamamoto Y, Kunishio K, Suga M, et al. [Primitive neuroectodermal tumor with peritoneal metastasis through a ventriculoperitoneal shunt. Case report]. Neurol Med Chir (Tokyo) 1989; 29(12): 1137–1143. [DOI] [PubMed] [Google Scholar]
- 11.Triolo PJ, Schulz EE. Metastatic germinoma (pinealoma) via a ventriculoperitoneal shunt. AJR Am J Roentgenol 1980; 135(4): 854–855. [DOI] [PubMed] [Google Scholar]
- 12.Kimura N, Namiki T, Wada T, et al. Peritoneal implantation of endodermal sinus tumor of the pineal region via a ventriculoperitoneal shunt. Cytodiagnosis with immunocytochemical demonstration of alpha-fetoprotein. Acta Cytol 1984; 28(2): 143–147. [PubMed] [Google Scholar]
- 13.Shibasaki T, Takeda F, Kawafuchi J. [Extraneural metastases of malignant brain tumors through ventriculoperitoneal shunt – report of two autopsy cases and a review of the literature (author’s transl.)]. No Shinkei Geka 1977; 5(1): 71–79. [PubMed] [Google Scholar]
- 14.Belongia M, Jogal S. Extraneural metastasis of a nongerminomatous germ cell tumor of the central nervous system in a pediatric patient with a ventriculoperitoneal shunt: a case report and review of the literature. J Pediatr Hematol Oncol 2012; 34(1): e12–16. doi: 10.1097/MPH.0b013e31823dd370. [DOI] [PubMed] [Google Scholar]
- 15.Cranston PE, Hatten MT, Smith EE. Metastatic pineoblastoma via a ventriculoperitoneal shunt: CT demonstration. Comput Med Imaging Graph 1992; 16(5): 349–351. [DOI] [PubMed] [Google Scholar]
- 16.Wood BP, Haller JO, Berdon WE, et al. Shunt metastases of pineal tumors presenting as a pelvic mass. Pediatr Radiol 1979; 8(2): 108–109. [DOI] [PubMed] [Google Scholar]
- 17.Berger MS, Baumeister B, Geyer JR, et al. The risks of metastases from shunting in children with primary central nervous system tumors. J Neurosurg 1991; 74(6): 872–877. [DOI] [PubMed] [Google Scholar]
- 18.Arulrajah S, Huisman TA. Pilomyxoid astrocytoma of the spinal cord with cerebrospinal fluid and peritoneal metastasis. Neuropediatrics 2008; 39(4): 243–245. doi: 10.1055/s-0028-1103273. [DOI] [PubMed] [Google Scholar]
- 19.Campbell AN, Chan HS, Beker LE, et al. Extracranial metastases in childhood primary intracranial tumors. A report of 21 cases and review of the literature. Cancer 1984; 53: 974–981. [DOI] [PubMed] [Google Scholar]
- 20.Mori T, Kayama T, Katakura R, et al. Medulloblastoma with intractable ascites treated by carboquone. A complication of ventriculoperitoneal shunt. Neurol Surg Qpn 1977; 5: 1299–1303. [PubMed] [Google Scholar]
- 21.Jamjoom ZA, Jamjoom AB, Sulaiman AH, et al. Systemic metastasis of medulloblastoma through ventriculoperitoneal shunt: report of a case and critical analysis of the literature. Surg Neurol 1993; 40(5): 403–410. [DOI] [PubMed] [Google Scholar]
- 22.Kessler LA, Dugan P, Concannon JP. Systemic metastases of medulloblastoma promoted by shunting. Surg Neurol 1975; 3(3): 147–152. [PubMed] [Google Scholar]
- 23.Berger MS, Baumeister B, Geyer JR, et al. The risks of metastases from shunting in children with primary central nervous system tumors. J Neurosurg 1991; 74(6): 872–877. [DOI] [PubMed] [Google Scholar]