A DNA INDEX STUDY OF TWELVE NEUROBLASTOMA ASSOCIATED WITH

OPSOCLONUS-MIOCLONUS SYNDROM Benavent Garciacutea N 1 Tadeo Cervera I2 and Noguera Salvaacute R3

Medical student1 INCLIVA2 and Department of Pathology (DP-UV) 3 Medical School of Valencia University of Valencia Avda Blasco Ibantildeez 15 46010 Valencia Valencia Spain

nubegaralumniuves irenetadeohotmailcom Rosanoguerauves

NEU

RO

BLA

STIC

TU

MO

RS

METHODS BACKGROUND AND OBJETIVES

Loca

lizat

ion

Sy

mp

tom

s

2012 SOM

Age Total Age Total

le18 months 32 le18 months 5

gt 18 months 22 gt 18 months 7

Total 54 Total 12 Table 1 Number of tumors that

were registrated in DP-UV during

the first semester 2012 classified

according to patients age

According to DNA INDEX (ID)8

DIPLOIDY ID 1- 119

TRIPLOIDY ID 120-174

TETRAPLOIDY ID 175-219

2012 Control

Age Total D D Tr Tr Te Te

le18 months 32 9 29 21 65 2 6

gt 18 months 22 6 27 16 73 0 0

OMS Cases

Age Total D Tr Te

le 18 months 5 0 0 5 100 0

gt 18 months 7 0 0 7 100 0 Table 4Number of control cases place in groups according to age and ploidy It is shown absolute number and percentaje in each age group

D Diploidy (2n) Tr Triploidy (3n) Te Tetraploidy (4n)

Table 3Number of control cases place in groups according to age and ploidy It is shown absolute number and percentaje in each age group

D Diploidy (2n) Tr Triploidy (3n) Te Tetraploidy (4n)

In order to obtain images ldquoOlympus ProgRes C10plus of Jenoptik Laser Microscoperdquo

(above) and informatic programme ldquoProgres Capture Pro

21rdquo (below) were used

CONCLUSION RESULTS

Tumor ploidy in our cohort of patients with

neuroblastic tumors and OMS are

homogeneously triploid (3n)

OPSOCLONUS ATAXIA

Opsoclonus myoclonus

syndrome(OMS) is a rare

immune-mediated

paraneoplastic syndrome which

occurs in 18 to 3 of patients

with neuroblastoma25 The

tumors seen in conjunction with

OMS are typically classified as

stage I or II without metastases

so patients with OMS and

neuroblastoma have a uniformly

good prognosis6

Neuroblastoma are embryonal tumors of the sympathetic nervous

system and the most common extracerebral solid tumors of infancy and

childhood about 7 of all paediatric tumors

Diploid and tetraploid are asociated with

poor prognosis in lt18 months old-children

and whereas triploid neuroblastoma tend to

be associated with favorable prognosis

The aim of this study is compare ploidy between tumors related with

OMS and tumors that were registrated as primary tumors in DP-UV

during the first semester in 2012

MIOCLONUS

OMS

DNA index has been studied by static cytometry based on Feulgen reaction Then Integrated Optical Density (IOD) has been quantified with ldquoImage-Pro Plus 60rdquo programme IOD is proportional to DNA amount Each tumor DNA index has been obtained with the relation between 500 neuroblastic cell nucleus IOD average and at least 50 lymphocyte (diploid cells ) nucleus IOD average

1 Cohn SL Et al The International Neuroblastoma Risk Group (INRG) Classification System An INRG Task Force Report JCO 2009 27(2)289-297 2 Lanzkowsky P Manual of Padiatric Hematology and Oncology 5th edition 2011 Elsevier Chapter 22 3 Monclair T et al The International Neuroblastoma Risk Group (INRG) staging system an INRG Task Force report J Clin Oncol 2009 27(2)298-303 4 Schleiermacher G et al Segmental chromosomal alterations have prognostic impact in neuroblastoma a report from the INRG project 2012

9107(8)1418-22 5 Samin Alav Et al Clinical Responses to Rituximab in a Case of Neuroblastoma with Refractory OpsoclonusMyoclonus Ataxia Syndrome Case

Reports in Oncological Medicine Volume 2012 Article ID 164082 4 pages 6 Alexis B et al The association between neuroblastoma and opsoclonus-myoclonus syndrome a historical review Pediatr Radiol (2009) 39723ndash

726 7 Villamoacuten E et al NeuPAT An intranet database supporting translational research in neuroblastic tumors Comput Biol Med 2013 Mar 143(3)219-

28 8 Look AT Hayes FA Nitschke R McWilliams NB Green AA Cellular DNA content as a predictor of response to chemotherapy in infants with

unresectable neuroblastoma N Engl J Med 1984 311 231-5 9 Hugo A Arroyo et al Sindrome de Opsoclonus-Mioclonus Medicina (Buenos Aires) 2009 69 (11) 64-70 10 Ambros I M Et al Tyrrole of ploidy chromosome 1p and Schwann Cells in the Maturation of Neuroblastoma N Engl J Med 1996 3341505-11 11 Maris JM Recent Advances in Neuroblastoma N Engl J Med 20103622202-11

BIBLIOGRAPHY

Symptoms

All the dates have been taken from Neupat Database 7

Table 2 Number of tumors that were

registrated in DP-UV during 10 years

related with SOM classified according to

patients age

This project has been financed by FIS (PI1015) and RD0600200102 RD1200360020 Carlos III Institute Madrid amp ERDF