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Fatal juvenile xanthogranuloma presenting as a sellar lesion: case report and literature review

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Abstract

Introduction

Juvenile xanthogranuloma (JXG) is a histiocytic condition in the spectrum of non-Langerhans histiocytosis that preferentially affects children. Rarely this condition can involve the central nervous system (CNS) with devastating consequences.

Methods

The authors report the unique case of an 11-year-old child who initially presented with a sellar lesion without evidence of the cutaneous stigmata typical of JXG. She was later discovered to have JXG following initial diagnosis of granulomatous hypophysitis, with development of widespread intracranial disease and subsequent neurological deterioration. She underwent subtotal resection of her sellar lesion followed by whole brain radiation and systemic chemotherapy; however, she succumbed to her disseminated disease within 1 month of the JXG diagnosis.

Conclusions

This is a rare case of fatal disseminated intracranial JXG without cutaneous manifestations. Additionally, the initial presentation as a sellar lesion is particularly unusual and seldom described in the literature.

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References

  1. Freyer DR, Kennedy R, Bostrom BC, Kohut G, Dehner LP (1996) Juvenile xanthogranuloma: forms of systemic disease and their clinical implications. J Pediatr 129(2):227–237

    Article  CAS  PubMed  Google Scholar 

  2. Rajaram S, Wharton SB, Shackley F, Raghavan A, Connolly DJ (2010) Intracranial non-Langerhans cell histiocytosis presenting as an isolated intraparenchymal lesion. Pediatr Radiol 40(Suppl 1):S145–S149

    Article  PubMed  Google Scholar 

  3. Janssen D, Harms D (2005) Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the Kiel pediatric tumor registry. Am J Surg Pathol 29(1):21–28

    Article  PubMed  Google Scholar 

  4. Wolff HH, Vigl E, Braun-Falco O (1975) Juvenile xanthogranuloma and organ manifestations. Hautarzt 26(5):268–272

    CAS  PubMed  Google Scholar 

  5. Kimura H, Oka K, Nakayama Y, Tomonaga M (1991) Xanthoma in Meckel’s cave. A case report. Surg Neurol 35(4):317–320

    Article  CAS  PubMed  Google Scholar 

  6. Paulus W, Kirchner T, Michaela M, Kühl J, Warmuth-Metz M, Sörensen N, Müller-Hermelink HK, Roggendorf W (1992) Histiocytic tumor of Meckel’s cave. An intracranial equivalent of juvenile xanthogranuloma of the skin. Am J Surg Pathol 16(1):76–83

    Article  CAS  PubMed  Google Scholar 

  7. Schultz KD Jr, Petronio J, Narad C, Hunter SB (1997) Solitary intracerebral juvenile xanthogranuloma. Case report and review of the literature. Pediatr Neurosurg 26(6):315–321

    Article  PubMed  Google Scholar 

  8. Boström J, Janssen G, Messing-Jünger M, Felsberg JU, Neuen-Jacob E, Engelbrecht V, Lenard HG, Bock WJ, Reifenberger G (2000) Multiple intracranial juvenile xanthogranulomas. Case Report J Neurosurg 93(2):335–341

    Google Scholar 

  9. Gutnik H, Dolenc-Strazar Z, Popovic M (2001) September 2000 15 years old girl with intracranial lesion. Brain Pathol 11(1):123–125

    CAS  PubMed  Google Scholar 

  10. Ernemann U, Skalej M, Hermisson M, Platten M, Jaffe R, Voigt K (2002) Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis. Neuroradiol 44(9):759–763

    Article  CAS  Google Scholar 

  11. Ashley WW Jr, Narayan P, Park TS, Tu PH, Perry A, Leonard JR (2005) Incidental pediatric intraparenchymal xanthogranuloma: case report and review of the literature. J Neurosurg 102(3 Suppl):307–310

    PubMed  Google Scholar 

  12. Nakasu S, Tsuji A, Fuse I, Hirai H (2007) Intracranial solitary juvenile xanthogranuloma successfully treated with stereotactic radiosurgery. J Neurooncol 84(1):99–102

    Article  PubMed  Google Scholar 

  13. Fulkerson DH, Luerssen TG, Hattab EM, Kim DL, Smith JL (2008) Long-term follow-up of solitary intracerebral juvenile xanthogranuloma. Case report and review of the literature. Pediatr Neurosurg 44(6):480–485

    Article  PubMed  Google Scholar 

  14. Orsey A, Paessler M, Lange BJ, Nichols KE (2008) Central nervous system juvenile xanthogranuloma with malignant transformation. Pediatr Blood Cancer 50(4):927–930

    Article  PubMed  Google Scholar 

  15. Sun LP, Jin HM, Yang B, Wu XR (2009) Intracranial solitary juvenile xanthogranuloma in an infant. World J Pediatr 5(1):71–73

    Article  CAS  PubMed  Google Scholar 

  16. Deisch JK, Patel R, Koral K, Cope-Yokoyama SD (2013) Juvenile xanthogranulomas of the nervous system: a report of two cases and review of the literature. Neuropathol 33(1):39–46

    Article  CAS  Google Scholar 

  17. Gressot LV, Patel AJ, Bollo RJ, Mohila CA, Jea A (2013) Disseminated intracranial juvenile xanthogranulomatosis in a neonate without cutaneous lesions. J Neurosurg Pediatr 12(2):187–191

    Article  PubMed  Google Scholar 

  18. Tamir I, Davir R, Fellig Y, Weintraub M, Constantini S, Spektor S (2013) Solitary juvenile xanthogranuloma mimicking intracranial tumor in children. J Clin Neurosci 20(1):183–188

    Article  PubMed  Google Scholar 

  19. Chiba K, Aihara Y, Eguchi S, Tanaka M, Komori T, Nakazato Y, Okada Y (2013) Diagnostic and management difficulties in a case of multiple intracranial juvenile xanthogranuloma. Childs Nerv Syst 29(6):1039–1045

    Article  PubMed  Google Scholar 

  20. Flach DB, Winkelmann RK (1986) Juvenile xanthogranuloma with central nervous system lesions. J Am Acad Dermatol 14(3):405–411

    Article  CAS  PubMed  Google Scholar 

  21. Botella-Estrada R, Sanmartin O, Grau M, Alegre V, Mas C, Aliaga A (1993) Juvenile xanthogranuloma with central nervous system involvement. Pediatr Dermatol 10(1):64–68

    Article  CAS  PubMed  Google Scholar 

  22. Okubo T, Okabe H, Kato G (1995) Juvenile xanthogranuloma with cutaneous and cerebral manifestations in a young infant. Acta Neuropathol 90(1):87–92

    Article  CAS  PubMed  Google Scholar 

  23. Diez-Saenz A, Borbujo-Martinez J, Fernandez-Acenero MJ, Hinojosa-Mena J (2002) Juvenile xanthogranuloma of the central nervous system. Rev Neurol 35(3):206–209

    CAS  PubMed  Google Scholar 

  24. Labalette P, Guilbert F, Jourdel D, Nelken B, Cuvellier JC, Maurage CA (2002) Bilateral multifocal uveal juvenile xanthogranuloma in a young boy with systemic disease. Graefes Arch Clin Exp Ophthalmol 240(6):506–509

    Article  PubMed  Google Scholar 

  25. Dehner LP (2003) Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol 27(5):579–593

    Article  PubMed  Google Scholar 

  26. Nakatani T, Morimoto A, Kato R, Tokuda S, Sugimoto T, Tokiwa K, Tsuchihashi Y, Imashuku S (2004) Successful treatment of congenital systemic juvenile xanthogranuloma with Langerhans cell histiocytosis-based chemotherapy. J Pediatr Hematol Oncol 26(6):371–374

    Article  PubMed  Google Scholar 

  27. Dölken R, Weigel S, Schröder H, Hartwig M, Harms D, Beck JF (2006) Treatment of severe disseminated juvenile systemic xanthogranuloma with multiple lesions in the central nervous system. J Pediatr Hematol Oncol 28(2):95–97

    Article  PubMed  Google Scholar 

  28. Auvin S, Cuvellier JC, Vinchon M, Defoort-Dhellemes S, Soto-Ares G, Nelken B, Vallée L (2008) Subdural effusion in a CNS involvement of systemic juvenile xanthogranuloma: a case report treated with vinblastin. Brain Dev 30(2):164–168

    Article  PubMed  Google Scholar 

  29. Stover DG, Alapati S, Regueira O, Turner C, Whitlock JA (2008) Treatment of juvenile xanthogranuloma. Pediatr Blood Cancer 51(1):130–133

    Article  PubMed  Google Scholar 

  30. Rajendra B, Duncan A, Parslew R, Pizer BL (2009) Successful treatment of central nervous system juvenile xanthogranulomatosis with cladribine. Pediatr Blood Cancer 52(3):413–415

    Article  PubMed  Google Scholar 

  31. Lalitha P, Reddy MC, Reddy KJ (2011) Extensive intracranial juvenile xanthogranulomas. AJNR Am J Neuroradiol 32(7):E132–E133

    Article  CAS  PubMed  Google Scholar 

  32. Meshkini A, Shahzadi S, Zali A, Tajeddini A, Mirzayan J, Hamdi A (2012) Systemic juvenile xanthogranuloma with multiple central nervous system lesions. J Cancer Res Ther 8(2):311–313

    Article  PubMed  Google Scholar 

  33. Webb DK (1996) Histiocyte disorders. Br Med Bull 52(4):818–825

    Article  CAS  PubMed  Google Scholar 

  34. Favara BE, Feller AC, Pauli M, Jaffe ES, Weiss LM, Arico M, Bucsky P, Egeler RM, Elinder G, Gadner H, Gresik M, Henter JI, Imashuku S, Janka-Schaub G, Jaffe R, Ladisch S, Nezelof C, Pritchard J (1997) Contemporary classification of histiocytic disorders. The WHO committee on histiocytic/reticulum cell proliferations. Reclassification working group of the Histiocyte society. Med Pediatr Oncol 29(3):157–166

    Article  CAS  PubMed  Google Scholar 

  35. Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD (1999) World health organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the clinical advisory committee meeting-Airlie house, Virginia, november 1997. J Clin Oncol 17(12):3835–3849

    CAS  PubMed  Google Scholar 

  36. Kairouz S, Hashash J, Kabbara W, McHayleh W, Tabbara IA (2007) Dendritic cell neoplasms: an overview. Am J Hematol 82(10):924–928

    Article  PubMed  Google Scholar 

  37. Vasconcelos FO, Oliveira LA, Naves MD, Castro WH, Gomez RS (2001) Juvenile xanthogranuloma: case report with immunohistochemical identification of early and late cytomegalovirus antigens. J Oral Sci 43(1):21–25

    Article  CAS  PubMed  Google Scholar 

  38. Cambiaghi S, Restano L, Caputo R (2004) Juvenile xanthogranuloma associated with neurofibromatosis 1: 14 patients without evidence of hematologic malignancies. Pediatr Dermatol 21(2):97–101

    Article  PubMed  Google Scholar 

  39. Zvulunov A, Barak Y, Metzker A (1995) Juvenile xanthogranuloma, neurofibromatosis, and juvenile chronic myelogenous leukemia. World statistical analysis. Arch Dermatol 131(8):904–908

    Article  CAS  PubMed  Google Scholar 

  40. Raygada M, Arthur DC, Wayne AS, Rennert OM, Toretsky JA, Stratakis CA (2010) Juvenile xanthogranuloma in a child with previously unsuspected neurofibromatosis type 1 and juvenile myelomonocytic leukemia. Pediatr Blood Cancer 54(1):173–175

    Article  PubMed Central  PubMed  Google Scholar 

  41. Perez-Becker R, Szczepanowski M, Leuschner I, Janka G, Gokel M, Imschweiler T, Völpel S, Niehues T, Klapper W (2011) An aggressive systemic juvenile xanthogranuloma clonally related to a preceding T-cell acute lymphoblastic leukemia. Pediatr Blood Cancer 56(5):859–862

    Article  PubMed  Google Scholar 

  42. Zelger BW, Sidoroff A, Orchard G, Cerio R (1996) Non-Langerhans cell histiocytoses. A new unifying concept. Am J Dermatopathol 18(5):490–504

    Article  CAS  PubMed  Google Scholar 

  43. Illueca C, Cerdá-Nicolás M, Roldan P, Talamantes F, Ascaso J, Llombart-Bosch A (2002) Idiopathic granulomatous hypophysitis. Morphological and immunohistochemical study of a case. Neurocirugia (Astur) 13(2):137–141

    Article  CAS  Google Scholar 

  44. Rao S, Rajkumar A, Kuruvilla S (2008) Sellar lesion: not always a pituitary adenoma. Indian J Pathol Microbiol 51(2):269–270

    Article  PubMed  Google Scholar 

  45. Su SB, Zhang DJ, Yue SY, Zhang JN (2011) Primary granulomatous hypophysitis: a case report and literature review. Endocr J 58(6):467–473

    Article  PubMed  Google Scholar 

  46. Jaffe R (1999) The histiocytoses. Clin Lab Med 19:135–155

    CAS  PubMed  Google Scholar 

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Acknowledgments

We would like to acknowledge Dr. John Hick at Texas Children’s Hospital for his assistance in diagnosis.

Conflict of interest

The authors declare that they have no conflict of interest.

Compliance with ethical standards

This manuscript was reviewed and approved by MD Anderson Institutional Review Board (IRB).

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Authors and Affiliations

  1. Department of Neurosurgery, The University of Texas M. D. Anderson Cancer Center, 1400, Holcombe Blvd., Unit 442, Houston, TX, 77030, USA

    Sherise D. Ferguson & Ian E. McCutcheon

  2. Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas M. D. Anderson Cancer Center, 1400, Holcombe Blvd., Houston, TX, 77030, USA

    Steven G. Waguespack

  3. Department of Pathology, The University of Texas M. D. Anderson Cancer Center, 1400, Holcombe Blvd., Houston, TX, 77030, USA

    Lauren A. Langford

  4. Department of Pediatrics, The University of Texas M. D. Anderson Cancer Center, 1400, Holcombe Blvd., Houston, TX, 77030, USA

    Joann L. Ater

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  1. Sherise D. Ferguson
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  2. Steven G. Waguespack
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  3. Lauren A. Langford
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  4. Joann L. Ater
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Correspondence to Sherise D. Ferguson.

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Ferguson, S.D., Waguespack, S.G., Langford, L.A. et al. Fatal juvenile xanthogranuloma presenting as a sellar lesion: case report and literature review. Childs Nerv Syst 31, 777–784 (2015). https://doi.org/10.1007/s00381-014-2604-3

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  • DOI: https://doi.org/10.1007/s00381-014-2604-3

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