Langerhans Cell Histiocytosis of the Spine in a Child: A Rare Case and a Diagnostic Dilemma
Original Article | Volume 6 | Issue 3 | JBST September – December 2020 | Page 5-8 | Abigail R Tud, Jose Bayani O Aliling, Carlo Emmanuel J SumpaicoDOI: 10.13107/jbst.2020.v06i03.31
Author: Abigail R Tud, Jose Bayani O Aliling, Carlo Emmanuel J Sumpaico
Department of Orthopedics, The Medical City, Ortigas Avenue, Pasig City, Metro Manila 1605, Philippines.
Address of Correspondence
Dr. Abigail R Tud,
Unit 5F Tower 1, Sonata Private Residences, Lourdes corner St. Francis Avenues, East Wack Wack, Mandaluyong City, Metro Manila 1505, Philippines.
E-mail address: firstname.lastname@example.org
Introduction: Langerhans cell histiocytosis (LCH) comprises a rare spectrum of disorders characterized by abnormal proliferation of histiocytes. Lesions may be limited to a single system, or present as disseminated disease, with subsequent worse prognosis. Radiologic findings for spinal LCH are non-specific however and must be carefully differentiated from Pott’s disease (spinal tuberculosis, or [TB]) in endemic countries.
Case Report: An 8-year-old female was brought for consult due to back pain and compression deformity on plain radiographs, unaccompanied by constitutional symptoms. Computed tomography (CT) scan of the thoracic spine was done, which showed osteolysis of the T11 vertebra and paravertebral soft-tissue extension. Findings were deemed suggestive of tuberculous spondylitis, and CT-guided biopsy was performed for confirmation. Acid-fast Bacilli smears as well as Gram stains were negative. While waiting for definitive results, the patient was started on an empiric treatment regimen for TB. Final tissue and fluid cultures were negative for Mycobacterium tuberculosis, as were Gene Xpert and TB polymerase chain reaction studies. Histopathologic analysis showed atypical mononuclear histiocytes surrounded by inflammatory cells, suggestive of LCH. At present, the patient is 2 years post-biopsy, with stable lesions and no evidence of multi-system involvement.
Conclusion: In the setting of a spinal lesion in a child with a benign clinical history and no other pertinent laboratory findings, histopathologic analysis constitutes the gold standard in differentiating non-specific features of LCH from spinal TB. Both conditions necessitate long-term follow-up, due to the risk of progression, deformities, and neurologic sequelae.
Keywords: Langerhans cell histiocytosis, pediatric spine, Pott’s disease, tuberculosis, vertebra plana.
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|How to Cite this article: Tud AR, Aliling JBO, Sumpaico CEJ | Langerhans Cell Histiocytosis of the Spine in a Child: A Rare Case and a Diagnostic Dilemma. | Journal of Bone and Soft Tissue Tumors | September-December 2020; 6(3): 5-8.|