Volume 10 Supplement 1

2011 Pediatric Rheumatology Symposium: Abstracts

Open Access

Neuroblastoma versus systemic JIA – a diagnostic dilemma

  • Lisabeth Scalzi4,
  • Greg Hychko2,
  • Barbara E Ostrov1,
  • Catherine A Bingham3,
  • David Ungar1 and
  • Brandt P Groh1
Pediatric Rheumatology201210(Suppl 1):A33

DOI: 10.1186/1546-0096-10-S1-A33

Published: 13 July 2012

Purpose

Children with malignancies, including neuroblastoma (NB), may present to their primary physician with complaints that mimic those seen in systemic juvenile idiopathic arthritis (sJIA). The purpose of this investgation was to identify significant distinctions between patients with systemic JIA and neuroblastoma at the time of disease presentation.

Methods

A retrospective chart review was completed on all patients less than age 18, between 1990 and 2009 at Pennsylvania State University Children’s Hospital, with a diagnosis of either sJIA or NB. The following variables were identified at the time of presentation; gender, age, race, time to diagnosis, presenting signs and symptoms, neuroblastoma stage, LDH, uric acid, sedimentation rate, CRP, platelet count, hemoglobin, peripheral white blood cell count, percentage of neutrophils and lymphocytes, ferritin, and d-dimer. Other categorical information included whether or not the patients had gait disturbance or extremity pain, fever, rash, abdominal mass, arthritis, fatigue, or weight loss. Individual logistic regression models were evaluated for each variable, with sJIA or NB as the outcome. Given that patients with NB and extremity pain may be referred to orthopedics or rheumatology first, instead of a timely referral to hematology/oncology, a subgroup analysis was completed for those patients.

Results

There was a total of 65 sJIA and 89 NB patients. Demographically, sJIA patients were significantly older (7.2 vs. 3.0 years of age; p<0.0001). Symptomatically, the sJIA patients had fever, rash,arthritis, and extemity pain or gait disturbance (41% vs. 18%) more frequently (p<0.0001 for all). Laboratories were also significantly different between the groups. NB patients had higher LDH and uric acid levels, while sJIA patients had higher platelet counts, total peripheral WBC, had relative neutrophilia (p<0.0001 for all), and had higher ferritin levels than the NB patients (p=0.001), see Table 1. In the subgroup of 58 children with extremity pain or gait disturbance, 45 (78%) had sJIA and 13 (22%) had NB; see Table 2. NB patients had higher LDH (p=0.01) and lower hgb levels (p=0.003), while sJIA patients had a relative neutrophilia (76% versus 51%; p<0.0001).
Table 1

Presentation characteristics of SJIA versus NB patients

Variables

sJIA (65)

Neuroblastoma (89)

 

Age (years)

7.2 ± 4.7

3.0 ± 3.5

<0.0001

Gender

Famle 51%

Female 42%

0.26

 

Male 49%

Male 58%

 

Race

White 91%

White 85%

0.86

 

Black 6%

Black 7%

 
 

Hispanic 3%

Hispanic 3%

 
  

Other 5%

 

Fever present (0=no, 1=yes)

45/45 (100%)

12/71 (18%)

<0.0001

Time to diagnosis (months)

1.5 (65)

1.2 (78)

0.18

Arthritis

51/65 (78%)

1/71 (1%)

<0.0001

Rash

53/64 (83%)

2/71 (3%)

<0.0001

Abdominal mass

1/65 (2%)

29/71 (41%)

0.0001

Weight loss

11/65 (17%)

5/71 (7%)

0.07

Gait disturbance or extremity pain

45/65 (41%)

13/73 (18%)

<0.0001

Neuroblastoma stage

n/a

Stage 1 = 19/62 (31%)

n/a

  

Stage 2 = 3/62 (5%)

 
  

Stage 3 = 9/62(14%)

 
  

Stage 4 =31/62 (50%)

 

LDH (units/L)

637 ± 530 (35)

1842 ± 2410 (46)

0.005

Uric Acid (mg/dL)

3.3 ± 1.2 (22)

5.1 ± 2.9 (34)

0.005

Crp (mg/dL)

14.9 ± 25.3 (26)

10.2 ± 7.2 (4)

0.71

ESR (mm/hr)

81.6 ± 36.3 (58)

61.7 ± 46.0 (13)

0.09

PLT count

464,016 ± 173,148 (62)

327,419 ± 136,488 (62)

<0.00001

WBC

18,881 ± 11,449 (63)

10,484 ± 5,892 (68)

<0.00001

HGB (g.dL)

10.2 ± 1.80 (62)

10.3 ± 2.46 (67)

0.97

Neurtophil %

73 ± 14 (57)

0.49 ± 0.17 (64)

<0.00001

Lymphocyte %

17 ± 10 (47)

40 ± 17 (64)

<0.00001

Ferntin (ng/mL)

4,016 ± 6, 135 (50)

159 ± 205 (29)

0.001

D-Dimer elevated

34/36 (94%)

2/2 (100%)

0.73

Aldolase

15.6 ± 11.4 (28)

8.85 ± 1.01 (2)

0.42

Table 2

Laboratory values for sJIA and NB subjects with gait disturbance or extremity pain

Laboratory

sJIA

NB

p-value

LDH

625 ± 522 (28)

1365 ± 1073 (9)

0.01

Uric Acid

3.2 ± 1.1 (20)

4.0 ± 1.2 (8)

0.14

CRP

9.9 ± 7.8 (22)

10.2 ± 7.3 (4)

0.95

ESR

84 ± 32 (42)

100 ± 32 (6)

0.24

Hgb

10.4 ± 1.7 (43)

8.5 ± 2.4 (12)

0.003

Neutrophil %

76 ± 12 (40)

51 ± 10 (11)

<0.0001

Lympocyte %

15 ± 9 (40)

37 ± 12 (11)

<0.0001

Ferritin

4554 ± 6890 (37)

555 ± 135 (3)

0.34

d-dimer abnormal

27/20

1/1

0.79

PTT

35 ± 6 (30)

31 ± 4 (5)

0.15

Aldolase

16 ± 2 (24)

9 ± 8 (2)

0.44

Conclusion

There are significantly different demographic, presenting symptoms, and laboratories at the time of presentation of patients who are ultimately diagnosed with sJIA or NB. In particular, there are discerning laboratories that may help facilitate an appropriate and timely referal to oncology when extremity pain or gait disturbance are among the intial signs and symptoms.

Disclosure

Lisabeth Scalzi: None; Greg Hychko: None; Barbara E. Ostrov: None; Catherine A. Bingham: None; David Ungar: None; Brandt P. Groh: None.

Authors’ Affiliations

(1)
Hershey Medical Center
(2)
Indiana University
(3)
(4)
Pennsylvania State University/Hershey

Copyright

© Scalzi et al; licensee BioMed Central Ltd. 2012

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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