Recurrent Langerhans cell histiocytosis at the site of prior craniotomy: case report

M. Omar Iqbal MD1, Ashirwad Merve PhD, FRCPath2, Nathalie Galea MD, MRCPCH, MSc3, and Kristian Aquilina MD, FRCS(SN)1
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  • 1 Departments of Neurosurgery and
  • | 2 Histopathology, Great Ormond Street Hospital for Children, London, United Kingdom; and
  • | 3 Department of Child and Adolescent Health, Mater Dei Hospital, Msida, Malta
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Tumors of the CNS represent the largest group of solid tumors found in the pediatric patient population. Langerhans cell histiocytosis (LCH) is an inflammatory lesion that may present in bone and/or soft tissue, including the CNS. Management depends on the extent of multisystem involvement, which determines resection with or without systemic chemotherapy. The authors report on the case of a child who underwent an open craniotomy for biopsy of a pituitary stalk lesion followed by neuropathological assessment, procedures used to diagnose LCH. The patient then underwent 12 months of systemic chemotherapy with subsequent resolution of the pituitary stalk lesion. Two years following pathological diagnosis, the patient presented with frontal orbital pain at the site of the prior craniotomy. Advanced imaging revealed MRI enhancement and radiotracer uptake of a soft-tissue growth at the frontal burr-hole site and MRI enhancement at a posterior burr-hole site without soft-tissue growth. The patient then underwent open biopsy and curettage that revealed LCH recurrence at the site of prior craniotomy. This case demonstrates that LCH may represent an abnormal reactive clonal proliferation of dendritic cells, rather than a de novo malignant neoplasm that can occur at sites of prior craniotomy despite systemic chemotherapy. The authors advocate close follow-up with contrast-enhanced imaging. Special attention should be given to sites of prior surgical manipulation to avoid missing distant sites of recurrence.

ABBREVIATIONS

LCH = Langerhans cell histiocytosis; PFS = progression-free survival.

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Contributor Notes

Correspondence M. Omar Iqbal: Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom. omar.iqbal@gosh.nhs.uk.

INCLUDE WHEN CITING Published online September 27, 2019; DOI: 10.3171/2019.6.PEDS19286.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

  • 1

    Berry DH, Gresik M, Maybee D, Marcus R: Histiocytosis X in bone only. Med Pediatr Oncol 18:292294, 1990

  • 2

    Curry HL, Parkes SE, Powell JE, Mann JR: Caring for survivors of childhood cancers: the size of the problem. Eur J Cancer 42:501508, 2006

    • Search Google Scholar
    • Export Citation
  • 3

    Dimentberg RA, Brown KL: Diagnostic evaluation of patients with histiocytosis X. J Pediatr Orthop 10:733741, 1990

  • 4

    Grois N, Pötschger U, Prosch H, Minkov M, Arico M, Braier J, et al. : Risk factors for diabetes insipidus in Langerhans cell histiocytosis. Pediatr Blood Cancer 46:228233, 2006

    • Search Google Scholar
    • Export Citation
  • 5

    Haupt R, Minkov M, Astigarraga I, Schäfer E, Nanduri V, Jubran R, et al. : Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer 60:175184, 2013

    • Search Google Scholar
    • Export Citation
  • 6

    Haupt R, Nanduri V, Calevo MG, Bernstrand C, Braier JL, Broadbent V, et al. : Permanent consequences in Langerhans cell histiocytosis patients: a pilot study from the Histiocyte Society-Late Effects Study Group. Pediatr Blood Cancer 42:438444, 2004

    • Search Google Scholar
    • Export Citation
  • 7

    Jubran RF, Marachelian A, Dorey F, Malogolowkin M: Predictors of outcome in children with Langerhans cell histiocytosis. Pediatr Blood Cancer 45:3742, 2005

    • Search Google Scholar
    • Export Citation
  • 8

    Kenney LB, Bradeen H, Kadan-Lottick NS, Diller L, Homans A, Schwartz CL: The current status of follow-up services for childhood cancer survivors, are we meeting goals and expectations: a report from the Consortium for New England Childhood Cancer Survivors. Pediatr Blood Cancer 57:10621066, 2011

    • Search Google Scholar
    • Export Citation
  • 9

    Lee JW, Shin HY, Kang HJ, Kim H, Park JD, Park KD, et al. : Clinical characteristics and treatment outcome of Langerhans cell histiocytosis: 22 years’ experience of 154 patients at a single center. Pediatr Hematol Oncol 31:293302, 2014

    • Search Google Scholar
    • Export Citation
  • 10

    Moteki Y, Yamada M, Shimizu A, Suzuki N, Kobayashi T: Langerhans cell histiocytosis of the skull in a burr hole site covered with hydroxyapatite material. World Neurosurg 122:632637, 2019

    • Search Google Scholar
    • Export Citation
  • 11

    Raney RB Jr, D’Angio GJ: Langerhans’ cell histiocytosis (histiocytosis X): experience at the Children’s Hospital of Philadelphia, 1970–1984. Med Pediatr Oncol 17:2028, 1989

    • Search Google Scholar
    • Export Citation
  • 12

    Sessa S, Sommelet D, Lascombes P, Prévot J: Treatment of Langerhans-cell histiocytosis in children. J Bone Joint Surg Am 76:15131525, 1994

    • Search Google Scholar
    • Export Citation
  • 13

    Yağci B, Varan A, Cağlar M, Söylemezoğlu F, Sungur A, Orhan D, et al. : Langerhans cell histiocytosis: retrospective analysis of 217 cases in a single center. Pediatr Hematol Oncol 25:399408, 2008

    • Search Google Scholar
    • Export Citation

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