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Xanthogranulomatous osteomyelitis of the humerus in a pediatric patient with Alagille syndrome: a case report and literature review

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Abstract

Xanthogranulomatous osteomyelitis (XO) is an exceedingly rare disease characterized by infiltration of histiocytes and foamy macrophages. Both on gross examination and on radiographs, XO can mimic malignancy. We describe the case of a 5-year-old female with Alagille syndrome who presented with a pathologic fracture of the right humerus. Initial radiographs revealed multiple osteolytic lesions in the distal humerus while MRI showed a large soft tissue mass. Biopsy confirmed the diagnosis of XO, which has hitherto not been described in a patient with Alagille syndrome. The patient was admitted for IV antibiotics and taken to the operating room for an incision and debridement. Tissue cultures were obtained and grew Salmonella. Antibiotic therapy was tapered, and the patient responded to treatment. She was doing well at her 6-month follow-up visit. In the discussion section, we explore how osteopenia and immune dysregulation caused by Alagille syndrome can affect the development of XO. We summarize all previously reported cases of XO and conclude that XO presents as an osteolytic lesion that expands rapidly over the course of a few weeks. We highlight that XO can mimic sarcoma because of its mass effect but can be distinguished radiographically by the presence of surrounding sclerosis. Given the rapid onset of XO, we classify it as an acute osteomyelitis. We discuss how leukemia and Ewing sarcoma can present similarly to acute osteomyelitis. We then emphasize key features that can be used to distinguish these malignancies from acute osteomyelitis.

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References

  1. Borjian A, Rezaei F, Eshaghi MA, Shemshaki H. Xanthogranulomatous osteomyelitis. J Orthop Traumatol. 2011;13(4):217–20.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Franco V, Aragona F, Genova G, Florena A, Stella M, Campesi G. Xanthogranulomatous cholecystitis. Pathology - Research and Practice. 1990;186(3):383–90.

    Article  CAS  Google Scholar 

  3. Antonakopoulos GN, Chapple CR, Newman J. Xanthogranulomatous pyelonephritis. A reappraisal and immunohistochemical study. Arch Pathol Lab Med. 1988;112:275–81.

    CAS  PubMed  Google Scholar 

  4. Maeda T, Shimada M, Matsumata T. Xanthogranulomatous cholecystitis masquerading as gallbladder carcinoma. Am J Gastroenterol. 1994;89:628–30.

    CAS  PubMed  Google Scholar 

  5. Sapra R, Jain P, Gupta S, Kumar R. Multifocal bilateral xanthogranulomatous osteomyelitis. Indian Journal of Orthopaedics. 2015;49(4):482.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Cozzutto C. Xanthogranulomatous osteomyelitis. Arch Pathol Lab Med. 1984;08(12):973–6.

    Google Scholar 

  7. Pati GK, Singh A, Nath P, Narayan J, Padhi PK, Parida PK, et al. A 10-year-old child presenting with syndromic paucity of bile ducts (Alagille syndrome): a case report. Journal of Medical Case Reports. 2016;10(1). doi:10.1186/s13256-016-1126-x.

  8. Berniczei-Royko A, Chałas R, Mitura I, Nagy K, Prussak E. Medical and dental management of Alagille syndrome: a review. Med Sci Monit. 2014;20:476–80.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Olivier AH. The inter-relationships between calcium absorption and bile. Experientia. 1970;26(8):854. doi:10.1007/bf02114219.

    Article  CAS  PubMed  Google Scholar 

  10. Loomes KM, Spino C, Goodrich NP, Hangartner TN, Marker AE, Heubi JE, et al. DXA bone density in Alagille syndrome correlates with fracture history and degree of cholestasis. Hepatology. 2015;62(s1):1694, 1034A–5A. http://corescholar.libraries.wright.edu/bie/114.

    Google Scholar 

  11. Tilib Shamoun S, Le Friec G, Spinner N, Kemper C, Baker A. Immune dysregulation in Alagille syndrome: a new feature of the evolving phenotype. Clinics and Research in Hepatology and Gastroenterology. 2015;39(5):566–9. doi:10.1016/j.clinre.2015.02.003.

    Article  CAS  PubMed  Google Scholar 

  12. Shailendra Singh MS, Sahil Batra MBBS, Lalit Maini MS, Virender Kumar Gautam MS. Xanthogranulomatous osteomyelitis of proximal femur masquerading as benign bone tumor. Am J Orthop. 2015;44(8):E272–4.

    PubMed  Google Scholar 

  13. Lee SH, Lee YH, Park H, Cho YJ, Song HT, Yang WI, et al. A case report of xanthogranulomatous osteomyelitis of the distal ulna mimicking a malignant neoplasm. Am J Case Rep. 2013;14:304–7. doi:10.12659/AJCR.889422.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Vankalakunti M, Saikia UN, Mathew M, Kang M. Xanthogranulomatous osteomyelitis of ulna mimicking neoplasm. World Journal of Surgical Oncology. 2007;5(1):46. doi:10.1186/1477-7819-5-46.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Kamat G, Gramapurohit V, Myageri A, Shettar C. Xanthogranulomatous osteomyelitis presenting as swelling in right tibia. Case Reports in Pathology. 2011;2011:1–2. doi:10.1155/2011/257458.

    Article  Google Scholar 

  16. Wang Y, Yu H, Zhang H, Cui X, Han J. Xanthogranulomatous osteomyelitis of rib mimicking malignant lesions in 18F-FDG PET/CT imaging. Clin Nucl Med. 2014;39(5):e315–7. doi:10.1097/rlu.0b013e3182a20d45.

    Article  PubMed  Google Scholar 

  17. Kaneuchi Y, Tajino T, Hakozaki M, Yamada H, Tasaki K, Hasegawa O, et al. Xanthogranulomatous osteomyelitis of the distal tibia: a case report. Int J Rheum Dis. 2015. doi:10.1111/1756-185x.12817.

  18. Cennimo DJ, Agag R, Fleegler E, Lardizabal A, Klein KM, Wenokor C, et al. Mycobacterium marinum Hand infection in a “sushi chef”. Eplasty. 2009;14(9):e43.

    Google Scholar 

  19. Kashani MM, Zakerian BZ, Shayan K, Riyasi F. Xanthogranulomatous osteomyelitis of femur. Iran J OrthopSurg. 2010;8(4):187–90.

    Google Scholar 

  20. Nalini G. Xanthogranulomatous osteomyelitis: a case report. Medicine Journal. 2014;1(6):45–7.

    Google Scholar 

  21. Baisakh M, Kar M, Agrawal A, Mohapatra N. Xanthogranulomatous osteomyelitis mimicking neoplasm: a rare entity. Indian J Pathol Microbiol. 2016;59(2):253. doi:10.4103/0377-4929.182016.

    Article  PubMed  Google Scholar 

  22. Stewart JR, Dahlin DC, Pugh DG. The pathology and radiology of solitary benign bone tumors. Semin Roentgenol. 1966;1(3):268–92. doi:10.1016/0037-198x(66)90003-4.

    Article  Google Scholar 

  23. Rogalsky RJ, Black GB, Reed MH. Orthopaedic manifestations of leukemia in children. The Journal of Bone & Joint Surgery. 1986;68(4):494–501. doi:10.2106/00004623-198668040-00003.

    Article  CAS  Google Scholar 

  24. Chiappini E, Mastrangelo G, Lazzeri S. A case of acute osteomyelitis: an update on diagnosis and treatment. Int J Environ Res Public Health. 2016;13(6):539. doi:10.3390/ijerph13060539.

    Article  PubMed Central  Google Scholar 

  25. Ozaki T. Diagnosis and treatment of Ewing sarcoma of the bone: a review article. J Orthop Sci. 2015;20(2):250–63. doi:10.1007/s00776-014-0687-z.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Oudjhane K, Azouz EM. Imaging of osteomyelitis in children. Radiol Clin N Am. 2001;39(2):251–66. doi:10.1016/s0033-8389(05)70276-1.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Orthopedic Surgery, University of Pennsylvania, 3737 Market St, Philadelphia, PA, 19104, USA

    Adnan Cheema

  2. Department of Orthopedic Surgery, Children’s Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA

    Alexandre Arkader

  3. Department of Pathology, Children’s Hospital of Philadelphia, 5NW11, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA

    Bruce Pawel

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  1. Adnan Cheema
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  2. Alexandre Arkader
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Correspondence to Adnan Cheema.

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Cheema, A., Arkader, A. & Pawel, B. Xanthogranulomatous osteomyelitis of the humerus in a pediatric patient with Alagille syndrome: a case report and literature review. Skeletal Radiol 46, 1447–1452 (2017). https://doi.org/10.1007/s00256-017-2707-5

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  • DOI: https://doi.org/10.1007/s00256-017-2707-5

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