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Published in: BMC Medical Imaging 1/2019

Open Access 01-12-2019 | Magnetic Resonance Imaging | Case report

Diagnostic value of whole-body MRI in Opsoclonus-myoclonus syndrome: a clinical case series (3 case reports)

Authors: Corinna Storz, Roland Bares, Martin Ebinger, Rupert Handgretinger, Ilias Tsiflikas, Jürgen F. Schäfer

Published in: BMC Medical Imaging | Issue 1/2019

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Abstract

Background

Opsoclonus-myoclonus syndrome (OMS) is a rare clinical disorder and typically occurs in association with occult neuroblastic tumor in pediatric patients. I-123 metaiodobenzylguanidine (mIBG) scintigraphy is widely adopted as screening procedure in patients with suspected neuroblastic tumor. Also, contrast-enhanced magnetic resonance imaging (MRI) or computed tomography (CT) are involved in the imaging workup, primarily for the assessment of the primary tumor region. However, the diagnostic value of whole-body MRI (WB-MRI) for the detection of occult neuroblastic tumor in pediatric patients presenting with OMS remains unknown.

Case presentation

We present three cases of patients with OMS, in whom WB-MRI revealed occult neuroblastic tumor masses, whereas scintigraphy was inconclusive:
In a 17 months old girl with OMS, WB-MRI revealed a paravertebral mass. After thoracoscopic resection, histopathology revealed a ganglioneuroblastoma.
A 13 months old boy presenting with OMS WB-MRI detected a tumor of the left adrenal gland; histopathology demonstrated a ganglioneuroblastoma after adrenalectomy.
In a 2 year old boy with OMS, immunoscintigraphy at the time of diagnosis was inconclusive. At the age of 13 years, a WB-MRI was performed due to persistent neurological symptoms, revealing a paravertebral retroperitoneal mass, which was classified as ganglioneuroblastoma.

Conclusion

In OMS, particularly in the setting of inconclusive scintigraphy, WB-MRI may be considered as a valuable alternative in the early phase of diagnostic work-up.
Literature
1.
go back to reference Pang KK, de Sousa C, Lang B, Pike MG. A prospective study of the presentation and management of dancing eye syndrome/opsoclonus-myoclonus syndrome in the United Kingdom. Eur J Paediatr Neurol. 2010;14(2):156–61.CrossRef Pang KK, de Sousa C, Lang B, Pike MG. A prospective study of the presentation and management of dancing eye syndrome/opsoclonus-myoclonus syndrome in the United Kingdom. Eur J Paediatr Neurol. 2010;14(2):156–61.CrossRef
2.
go back to reference Matthay KK, Blaes F, Hero B, Plantaz D, De Alarcon P, Mitchell WG, et al. Opsoclonus myoclonus syndrome in neuroblastoma a report from a workshop on the dancing eyes syndrome at the advances in neuroblastoma meeting in Genoa, Italy, 2004. Cancer Lett. 2005;228(1–2):275–82.CrossRef Matthay KK, Blaes F, Hero B, Plantaz D, De Alarcon P, Mitchell WG, et al. Opsoclonus myoclonus syndrome in neuroblastoma a report from a workshop on the dancing eyes syndrome at the advances in neuroblastoma meeting in Genoa, Italy, 2004. Cancer Lett. 2005;228(1–2):275–82.CrossRef
3.
go back to reference Blaes F, Fuhlhuber V, Korfei M, Tschernatsch M, Behnisch W, Rostasy K, et al. Surface-binding autoantibodies to cerebellar neurons in opsoclonus syndrome. Ann Neurol. 2005;58(2):313–7.CrossRef Blaes F, Fuhlhuber V, Korfei M, Tschernatsch M, Behnisch W, Rostasy K, et al. Surface-binding autoantibodies to cerebellar neurons in opsoclonus syndrome. Ann Neurol. 2005;58(2):313–7.CrossRef
4.
go back to reference Connolly AM, Pestronk A, Mehta S, Pranzatelli MR 3rd, Noetzel MJ. Serum autoantibodies in childhood opsoclonus-myoclonus syndrome: an analysis of antigenic targets in neural tissues. J Pediatr. 1997;130(6):878–84.CrossRef Connolly AM, Pestronk A, Mehta S, Pranzatelli MR 3rd, Noetzel MJ. Serum autoantibodies in childhood opsoclonus-myoclonus syndrome: an analysis of antigenic targets in neural tissues. J Pediatr. 1997;130(6):878–84.CrossRef
5.
go back to reference Altman AJ, Baehner RL. Favorable prognosis for survival in children with coincident opso-myoclonus and neuroblastoma. Cancer. 1976;37(2):846–52.CrossRef Altman AJ, Baehner RL. Favorable prognosis for survival in children with coincident opso-myoclonus and neuroblastoma. Cancer. 1976;37(2):846–52.CrossRef
6.
go back to reference Rudnick E, Khakoo Y, Antunes NL, Seeger RC, Brodeur GM, Shimada H, et al. Opsoclonus-myoclonus-ataxia syndrome in neuroblastoma: clinical outcome and antineuronal antibodies-a report from the Children's Cancer group study. Med Pediatr Oncol. 2001;36(6):612–22.CrossRef Rudnick E, Khakoo Y, Antunes NL, Seeger RC, Brodeur GM, Shimada H, et al. Opsoclonus-myoclonus-ataxia syndrome in neuroblastoma: clinical outcome and antineuronal antibodies-a report from the Children's Cancer group study. Med Pediatr Oncol. 2001;36(6):612–22.CrossRef
7.
go back to reference De Grandis E, Parodi S, Conte M, Angelini P, Battaglia F, Gandolfo C, et al. Long-term follow-up of neuroblastoma-associated opsoclonus-myoclonus-ataxia syndrome. Neuropediatrics. 2009;40(3):103–11.CrossRef De Grandis E, Parodi S, Conte M, Angelini P, Battaglia F, Gandolfo C, et al. Long-term follow-up of neuroblastoma-associated opsoclonus-myoclonus-ataxia syndrome. Neuropediatrics. 2009;40(3):103–11.CrossRef
8.
go back to reference Brunklaus A, Pohl K, Zuberi SM, de Sousa C. Outcome and prognostic features in opsoclonus-myoclonus syndrome from infancy to adult life. Pediatrics. 2011;128(2):e388–94.CrossRef Brunklaus A, Pohl K, Zuberi SM, de Sousa C. Outcome and prognostic features in opsoclonus-myoclonus syndrome from infancy to adult life. Pediatrics. 2011;128(2):e388–94.CrossRef
9.
go back to reference Bleeker G, Tytgat GA, Adam JA, Caron HN, Kremer LC, Hooft L, et al. 123I-MIBG scintigraphy and 18F-FDG-PET imaging for diagnosing neuroblastoma. Cochrane Database Syst Rev. 2015;(9):CD009263. Bleeker G, Tytgat GA, Adam JA, Caron HN, Kremer LC, Hooft L, et al. 123I-MIBG scintigraphy and 18F-FDG-PET imaging for diagnosing neuroblastoma. Cochrane Database Syst Rev. 2015;(9):CD009263.
10.
go back to reference Parisi MT, Hattner RS, Matthay KK, Berg BO, Sandler ED. Optimized diagnostic strategy for neuroblastoma in opsoclonus-myoclonus. J Nucl Med. 1993;34(11):1922–6.PubMed Parisi MT, Hattner RS, Matthay KK, Berg BO, Sandler ED. Optimized diagnostic strategy for neuroblastoma in opsoclonus-myoclonus. J Nucl Med. 1993;34(11):1922–6.PubMed
11.
go back to reference Sharp SE, Trout AT, Weiss BD, Gelfand MJ. MIBG in neuroblastoma diagnostic imaging and therapy. Radiographics. 2016;36(1):258–78.CrossRef Sharp SE, Trout AT, Weiss BD, Gelfand MJ. MIBG in neuroblastoma diagnostic imaging and therapy. Radiographics. 2016;36(1):258–78.CrossRef
12.
go back to reference Hoefnagel CA, Voute PA, de Kraker J, Marcuse HR. Radionuclide diagnosis and therapy of neural crest tumors using iodine-131 metaiodobenzylguanidine. J Nucl Med. 1987;28(3):308–14.PubMed Hoefnagel CA, Voute PA, de Kraker J, Marcuse HR. Radionuclide diagnosis and therapy of neural crest tumors using iodine-131 metaiodobenzylguanidine. J Nucl Med. 1987;28(3):308–14.PubMed
13.
go back to reference Vik TA, Pfluger T, Kadota R, Castel V, Tulchinsky M, Farto JC, et al. (123) I-mIBG scintigraphy in patients with known or suspected neuroblastoma: results from a prospective multicenter trial. Pediatr Blood Cancer. 2009;52(7):784–90.CrossRef Vik TA, Pfluger T, Kadota R, Castel V, Tulchinsky M, Farto JC, et al. (123) I-mIBG scintigraphy in patients with known or suspected neuroblastoma: results from a prospective multicenter trial. Pediatr Blood Cancer. 2009;52(7):784–90.CrossRef
14.
go back to reference Greer MC, Voss SD, States LJ. Pediatric Cancer predisposition imaging: focus on whole-body MRI. Clin Cancer Res. 2017;23(11):e6–e13.CrossRef Greer MC, Voss SD, States LJ. Pediatric Cancer predisposition imaging: focus on whole-body MRI. Clin Cancer Res. 2017;23(11):e6–e13.CrossRef
15.
go back to reference Takama Y, Yoneda A, Nakamura T, Nakaoka T, Higashio A, Santo K, et al. Early detection and treatment of Neuroblastic tumor with Opsoclonus-myoclonus syndrome improve neurological outcome: a review of five cases at a single institution in Japan. Eur J Pediatr Surg. 2016;26(1):54–9.PubMed Takama Y, Yoneda A, Nakamura T, Nakaoka T, Higashio A, Santo K, et al. Early detection and treatment of Neuroblastic tumor with Opsoclonus-myoclonus syndrome improve neurological outcome: a review of five cases at a single institution in Japan. Eur J Pediatr Surg. 2016;26(1):54–9.PubMed
16.
go back to reference Pfluger T, Schmied C, Porn U, Leinsinger G, Vollmar C, Dresel S, et al. Integrated imaging using MRI and 123I metaiodobenzylguanidine scintigraphy to improve sensitivity and specificity in the diagnosis of pediatric neuroblastoma. AJR Am J Roentgenol. 2003;181(4):1115–24.CrossRef Pfluger T, Schmied C, Porn U, Leinsinger G, Vollmar C, Dresel S, et al. Integrated imaging using MRI and 123I metaiodobenzylguanidine scintigraphy to improve sensitivity and specificity in the diagnosis of pediatric neuroblastoma. AJR Am J Roentgenol. 2003;181(4):1115–24.CrossRef
17.
go back to reference Biasotti S, Garaventa A, Villavecchia GP, Cabria M, Nantron M, De Bernardi B. False-negative metaiodobenzylguanidine scintigraphy at diagnosis of neuroblastoma. Med Pediatr Oncol. 2000;35(2):153–5.CrossRef Biasotti S, Garaventa A, Villavecchia GP, Cabria M, Nantron M, De Bernardi B. False-negative metaiodobenzylguanidine scintigraphy at diagnosis of neuroblastoma. Med Pediatr Oncol. 2000;35(2):153–5.CrossRef
18.
go back to reference Reuland P, Geiger L, Thelen MH, Handgretinger R, Haase B, Muller-Schauenburg W, et al. Follow-up in neuroblastoma: comparison of metaiodobenzylguanidine and a chimeric anti-GD2 antibody for detection of tumor relapse and therapy response. J Pediatr Hematol Oncol. 2001;23(7):437–42.CrossRef Reuland P, Geiger L, Thelen MH, Handgretinger R, Haase B, Muller-Schauenburg W, et al. Follow-up in neuroblastoma: comparison of metaiodobenzylguanidine and a chimeric anti-GD2 antibody for detection of tumor relapse and therapy response. J Pediatr Hematol Oncol. 2001;23(7):437–42.CrossRef
19.
go back to reference Brunklaus A, Pohl K, Zuberi SM, de Sousa C. Investigating neuroblastoma in childhood opsoclonus-myoclonus syndrome. Arch Dis Child. 2012;97(5):461–3.CrossRef Brunklaus A, Pohl K, Zuberi SM, de Sousa C. Investigating neuroblastoma in childhood opsoclonus-myoclonus syndrome. Arch Dis Child. 2012;97(5):461–3.CrossRef
20.
go back to reference Brisse HJ, McCarville MB, Granata C, Krug KB, Wootton-Gorges SL, Kanegawa K, et al. Guidelines for imaging and staging of neuroblastic tumors: consensus report from the international neuroblastoma risk group project. Radiology. 2011;261(1):243–57.CrossRef Brisse HJ, McCarville MB, Granata C, Krug KB, Wootton-Gorges SL, Kanegawa K, et al. Guidelines for imaging and staging of neuroblastic tumors: consensus report from the international neuroblastoma risk group project. Radiology. 2011;261(1):243–57.CrossRef
21.
go back to reference Shapiro B, Gross MD. Radiochemistry, biochemistry, and kinetics of 131I-metaiodobenzylguanidine (MIBG) and 123I-MIBG: clinical implications of the use of 123I-MIBG. Med Pediatr Oncol. 1987;15(4):170–7.CrossRef Shapiro B, Gross MD. Radiochemistry, biochemistry, and kinetics of 131I-metaiodobenzylguanidine (MIBG) and 123I-MIBG: clinical implications of the use of 123I-MIBG. Med Pediatr Oncol. 1987;15(4):170–7.CrossRef
22.
go back to reference Razek AA, Ezzat A, Azmy E, Tharwat N. Role of whole-body 64-slice multidetector computed tomography in treatment planning for multiple myeloma. Radiol Med. 2013;118(5):799–805.CrossRef Razek AA, Ezzat A, Azmy E, Tharwat N. Role of whole-body 64-slice multidetector computed tomography in treatment planning for multiple myeloma. Radiol Med. 2013;118(5):799–805.CrossRef
23.
go back to reference Razek A, Ashmalla GA. Assessment of paraspinal neurogenic tumors with diffusion-weighted MR imaging. Eur Spine J. 2018;27(4):841–6.CrossRef Razek A, Ashmalla GA. Assessment of paraspinal neurogenic tumors with diffusion-weighted MR imaging. Eur Spine J. 2018;27(4):841–6.CrossRef
24.
go back to reference Uhl M, Altehoefer C, Kontny U, Il'yasov K, Buchert M, Langer M. MRI-diffusion imaging of neuroblastomas: first results and correlation to histology. Eur Radiol. 2002;12(9):2335–8.CrossRef Uhl M, Altehoefer C, Kontny U, Il'yasov K, Buchert M, Langer M. MRI-diffusion imaging of neuroblastomas: first results and correlation to histology. Eur Radiol. 2002;12(9):2335–8.CrossRef
25.
go back to reference Kong G, Hofman MS, Murray WK, Wilson S, Wood P, Downie P, et al. Initial experience with Gallium-68 DOTA-Octreotate PET/CT and peptide receptor radionuclide therapy for pediatric patients with refractory metastatic neuroblastoma. J Pediatr Hematol Oncol. 2016;38(2):87–96.CrossRef Kong G, Hofman MS, Murray WK, Wilson S, Wood P, Downie P, et al. Initial experience with Gallium-68 DOTA-Octreotate PET/CT and peptide receptor radionuclide therapy for pediatric patients with refractory metastatic neuroblastoma. J Pediatr Hematol Oncol. 2016;38(2):87–96.CrossRef
26.
go back to reference Schafer JF, Gatidis S, Schmidt H, Guckel B, Bezrukov I, Pfannenberg CA, et al. Simultaneous whole-body PET/MR imaging in comparison to PET/CT in pediatric oncology: initial results. Radiology. 2014;273(1):220–31.CrossRef Schafer JF, Gatidis S, Schmidt H, Guckel B, Bezrukov I, Pfannenberg CA, et al. Simultaneous whole-body PET/MR imaging in comparison to PET/CT in pediatric oncology: initial results. Radiology. 2014;273(1):220–31.CrossRef
27.
go back to reference Pandit-Taskar N, Zanzonico P, Staton KD, Carrasquillo JA, Reidy-Lagunes D, Lyashchenko S, et al. Biodistribution and dosimetry of (18) F-meta-Fluorobenzylguanidine: a first-in-human PET/CT imaging study of patients with neuroendocrine malignancies. J Nucl Med. 2018;59(1):147–53.CrossRef Pandit-Taskar N, Zanzonico P, Staton KD, Carrasquillo JA, Reidy-Lagunes D, Lyashchenko S, et al. Biodistribution and dosimetry of (18) F-meta-Fluorobenzylguanidine: a first-in-human PET/CT imaging study of patients with neuroendocrine malignancies. J Nucl Med. 2018;59(1):147–53.CrossRef
Metadata
Title
Diagnostic value of whole-body MRI in Opsoclonus-myoclonus syndrome: a clinical case series (3 case reports)
Authors
Corinna Storz
Roland Bares
Martin Ebinger
Rupert Handgretinger
Ilias Tsiflikas
Jürgen F. Schäfer
Publication date
01-12-2019
Publisher
BioMed Central
Published in
BMC Medical Imaging / Issue 1/2019
Electronic ISSN: 1471-2342
DOI
https://doi.org/10.1186/s12880-019-0372-y

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