Langerhans Cell Histiocytosis of the External Auditory Canal in an Adult Patient with Myelodysplastic Syndrome
Keywords:
Langerhans cell histiocytosis, external auditory canal, Adult, Myelodysplastic syndromeAbstract
A 68-year-old female with myelodysplastic syndrome presented with a 1-year history of gradually enlarging bilateral external auditory canal masses without temporal bone involvement. Material from the bilateral external auditory masses showed intraepidermal and dermal proliferation of cells exhibiting ovoid grooved or folded nuclei, fine chromatin and moderate amount of eosinophilic cytoplasm. The neoplastic cells are strongly and diffusely positive for CD1a and Langerin (CD207). A diagnosis of Langerhans cell histiocytosis was made and the patient administered with topical steroids. The patients’ response to topical corticosteroid administration was less than favorable.
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References
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2. Abla O, Egeler RM, Weitzman S. Langerhans cell histiocytosis:current concepts and treatments. Cancer Treat Rev. 2010;36(4):354-9.354-9. https://doi.org/10.1016/j.ctrv.2010.02.012.
3. Aricò M. Langerhans cell histiocytosis in adults: more questions than answers? Eur J Cancer. 2004;40(10):1467-73. https://doi.org/10.1016/j.ejca.2004.01.025.
4. Billings SD, Hans CP, Schapiro BL, Martin RW, Fivenson D, Fruland JE, et al. Langerhans cell histiocytosis associated with myelodysplastic syndrome in adults. J CutanPathol. 2006;33(2):171-4. https://doi.org/10.1111/j.0303-6987.2006.00299.x.
5. Stefanato CM, Andersen WK, Calonje E, Swain FA, Borghi S, Massone L, et al. Langerhans cell histiocytosis in the elderly: a report of three cases. J Am Acad Dermatol. 1998; 39(2, Suppl):375-8. https://doi.org/10.1016/S0190-9622(98)70395-0.
6. Song HL, Huang WY, Chen YP, Chang KC. Tumorous proliferations of plasmacytoid dendritic cells and Langerhans cells associated with acute myeloid leukaemia. Histopathology. 2012;61(5):974-83. https://doi.org/10.1111/j.1365-2559.2012.04282.x.
7. Favara BE, Feller AC, Pauli M, Jaffe ES, Weiss LM, Arico M, et al. Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/ Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society. Med Pediatr Oncol. 1997;29(3):157–66. PMID: 9212839.
8. Andersen WK, Knowles DM, Silvers DN. CD1(OKT6)-positive juvenile xanthogranuloma. OKT6 is not specific for Langerhans cell histiocytosis (histiocytosis X). J Am Acad Dermatol. 1992;26(5 Pt 2):850–4. https://doi.org/10.1016/0190-9622(92)70120-5.
9. de Graaf JH, Timens W, Tamminga RY, Molenaar WM. Deep juvenile xanthogranuloma: a lesion related to dermal indeterminate cells. Hum Pathol. 1992;23(8):905–10. https://doi.org/10.1016/0046-8177(92)90403-P.
10. Frater JL, Kling CW, Obadiah JM, Gardner LJ, Grosso LE, Resh B, et al. Histiocytic sarcoma with secondary involvement of the skin and expression of CD1a: Evidence of indeterminate cell differentiation? J Cutan Pathol. 2006;33(6):437–42. https://doi.org/10.1111/j.0303-6987.2006.00453.x.
11. Hornick JL, Jaffe ES, Fletcher CD. Extranodal histiocytic sarcoma: clinicopathologic analysis of 14 cases of a rare epithelioid malignancy. Am J Surg Pathol. 2004;28(9):1133–44. PMID: 15316312.
12. Paulli M, Bergamaschi G, Tonon L, Viglio A, Rosso R, Facchetti F, et al. Evidence for a polyclonal nature of the cell infiltrate in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Br J Haematol. 1995;91(2):415–8. https://doi.org/10.1111/j.1365-2141.1995.tb05313.x.
13. Mc Dermott R, Ziylan U, Spehner D, Bausinger H, Lipsker D, Mommaas M, et al. Birbeck granules are subdomains of endosomal recycling compartment in human epidermal Langerhans cells, which form where Langerin accumulates. Mol Biol Cell. 2002;13(1):317–35. https://doi.org/10.1091/mbc.01-06-0300.
14. Valladeau J, Ravel O, Dezutter-Dambuyant C, Moore K, Kleijmeer M, Liu Y, et al. Langerin, a novel C-type lectin specific to Langerhans cells, is an endocytic receptor that induces the formation of Birbeck granules. Immunity. 2000;12(1):71–81. https://doi.org/10.1016/S1074-7613(00)80160-0.
15. Dziegiel P, Dolilńska-Krajewska B, Dumańska M, Wecławek J, Jeleń M, Podhorska-Okołów M, et al. Coexpression of CD1a, langerin and Birbeck’s granules in Langerhans cell histiocytoses (LCH) in children: Ultrastructural and immunocytochemical studies. Folia Histochem Cytobiol. 2007;45(1):21–5. PMID: 17378241.
16. Aricò M, Girschikofsky M, Généreau T, Klersy C, McClain K, Grois N, et al. Langerhans cell histiocytosis in adults: Report from the International Registry of the Histiocyte Society. Eur J Cancer. 2003;39(16):2341-8. https://doi.org/10.1016/S0959-8049(03)00672-5.
Published
12/13/2016
How to Cite
Hung, J., & Chang, A. M. (2016). Langerhans Cell Histiocytosis of the External Auditory Canal in an Adult Patient with Myelodysplastic Syndrome. PJP, 2(1), 34. Retrieved from https://philippinejournalofpathology.org/index.php/PJP/article/view/51
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The Philippine Journal of Pathology is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License. Based on works made open access at http://philippinejournalofpathology.org
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