Radiologia Brasileira - Publicação Científica Oficial do Colégio Brasileiro de Radiologia

Dear Editor,

A 27-year-old male presented with polydipsia, polyuria, xerostomia, and mild bone pain, being diagnosed with and treated for diabetes insipidus. Thereafter, he presented with diffuse and severe bone pain, xanthomas, xanthelasmas, exophthalmia, and cholelithiasis. After a complete medical investigation, Erdheim-Chester disease (non-Langerhans cell histiocytosis) was considered the most probable clinical diagnosis. Among the imaging exams performed, he was referred for ¹⁸F-FDG PET/CT and ¹⁸F-NaF PET/CT.

The initial ¹⁸F-NaF PET/CT showed that ¹⁸F-NaF uptake was more intense in the distal femora and throughout the tibiae, as well as in the fibulae (proximal and distal), tarsi, and maxillas, than in the other bones (Figure 1A). The ¹⁸F-FDG PET/CT study revealed increased glycolytic metabolism in the pituitary stalk, proximal left femur, proximal fibulae, ankle, and feet, less intense uptake being observed in other areas (Figures 1B and 1C). It is of note that the ¹⁸F-FDG PET/CT was performed 9 months after the ¹⁸F-NaF PET/CT, showing a heterogeneous response of the lesions to the various treatment modalities the patient underwent, and that, over the course of the follow-up, he alternated between periods of clinical stability and disease progression.




Erdheim-Chester disease is systemic, although variable in extent, and bone involvement is quite typical. Classical radiological findings include sclerotic and osteolytic lesions in the cortical layer of long bones, occurring bilaterally and symmetrically in their metaphysis and diaphysis, sparing the epiphysis and the axial skeleton. Approximately 50% of patients with Erdheim-Chester disease present extraosseous impairment, including changes in the hypothalamus, posterior hypophysis, eyes, retroperitoneum, skin, lungs, and heart⁽¹⁾.

¹⁸F-NaF PET/CT has the advantage of being a whole-body study with high sensitivity, thereby detecting bone impairment in Erdheim-Chester disease. The use of imaging methods enables clinical suspicion for early diagnosis and patient follow-up, including therapy response assessment⁽²⁾. In comparison with ^99mTc-MDP, ¹⁸F-NaF shows better pharmacokinetic characteristics, including faster blood clearance and two-fold higher uptake in bone⁽³⁾. Data from a number of studies, all involving small patient samples, have shown that ¹⁸F-NaF PET has higher sensitivity and specificity than do conventional ^99mTc-based bone scans^(4-7). In the present study, ¹⁸F-NaF PET/CT revealed some bone lesions in the ribs and arms that were not detected by ¹⁸F-FDG PET/CT, indicating that the former has greater sensitivity for detecting bone lesions.

In Erdheim-Chester disease, extraosseous impairment can occur in almost every organ, which suggests that ¹⁸F-FDG PET/CT has potential value as a diagnostic tool. However, its main advantage is probably therapy response assessment, although that has not been well established⁽⁸⁾. This imaging modality also allows guided percutaneous biopsies (by identifying areas of high metabolic activity). Therefore, the role of ¹⁸F-FDG PET/CT in the initial diagnosis of Erdheim-Chester disease remains unclear, especially because the systemic presentation patterns of the disease are extremely variable, and it is likely to prove much more valuable for patient follow-up^(8,9).


REFERENCES

1. Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, et al. Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases. Medicine (Baltimore). 1996;75:157–69.

2. Caoduro C, Ungureanu CM, Rudenko B, et al. 18F-fluoride PET/CT aspect of an unusual case of Erdheim-Chester disease with histologic features of Langerhans cell histiocytosis. Clin Nucl Med. 2013;38:541–2.

3. Segall G, Delbeke D, Stabin MG, et al. SNM practice guideline for sodium 18F-fluoride PET/CT bone scans 1.0. J Nucl Med. 2010;51:1813–20.

4. Hetzel M, Arslandemir C, König HH, et al. ¹⁸F-NaF PET for detection of bone metastases in lung cancer: accuracy, cost-effectiveness, and impact on patient management. J Bone Miner Res. 2003;18:2206–14.

5. Hoh CK, Hawkins RA, Dahlbom M, et al. Whole body skeletal imaging with [18F]fluoride ion and PET. J Comput Assist Tomogr. 1993;17:34–41.

6. Langsteger W, Heinisch M, Fogelman I. The role of fluorodeoxyglucose, 18F-dihydroxyphenylalanine, 18F-choline, and 18F-fluoride in bone imaging with emphasis on prostate and breast. Semin Nucl Med. 2006;36:73–92.

7. Schirrmeister H, Guhlmann A, Kotzerke J, et al. Early detection and accurate description of extent of metastatic bone disease in breast cancer with fluoride ion and positron emission tomography. J Clin Oncol. 1999;17:2381–9.

8. Arnaud L, Malek Z, Archambaud F, et al. 18F-fluorodeoxyglucose-positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim-Chester disease. Arthritis Rheum. 2009;60:3128–38.

9. Campochiaro C, Tomelleri A, Cavalli G, et al. Erdheim-Chester disease. Eur J Intern Med. 2015;26:223–9.










1. Instituto do Câncer do Estado de São Paulo (Icesp), São Paulo, SP, Brazil
2. Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo, SP, Brazil

Mailing address:
Dr. Romulo Hermeto Bueno do Vale
Universidade de São Paulo - Medicina Nuclear
Rua Doutor Ovidio Pires de Campos, 75, Cerqueira César
São Paulo, SP, Brazil, 05403-010
E-mail: romulohermeto@hotmail.com