Langerhans cell sarcoma presenting as a mediastinal mass in a young infant: a case report
Keywords:
Infant, Langerhans cell sarcoma, Mediastinal mass
Abstract
Langerhans Cell Sarcoma is an extremely rare disease. Its manifestations vary though skin, lymph-node and bone involvement are most commonly reported. It is an aggressive disease with a poor outcome. We report a six-week-old infant with Langerhans cell sarcoma who presented with a mediastinal mass causing respiratory distress. The diagnosis was made on histopathology and immunohistochemistry of biopsy of the mass. The tumour did not respond to chemotherapy and the baby died within weeks of diagnosis due to respiratory failure. To the best of our knowledge, this is among the youngest patients reported to have a Langerhans cell sarcoma.
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References
1. Lee JS, Ko GH, Kim HC, Jang IS, Jeon KN, Lee JH. Langerhans cell sarcoma arising from Langerhans cell histiocytosis: A case report. J Korean Med Sci 2006; 21(3):577-80. doi:10.3346/jkms.2006.21.3.577. [PubMed]
2. Howard JE, Dwivedi RC, Materson L, Jani P. Langerhans cell sarcoma: a systematic review. Cancer Treat Rev 2015;41(4):320-31. doi: 10.1016/j.ctrv.2015.02.011. [PubMed]
3. Zhang Y, Qu Z, Fang F. Langerhans cell sarcoma originating from left knee subcutaneous tissue: A case report and literature review. Oncol Lett 2016; 12(5):3687-94.
4. Liu DT, Friesenbichler J, Holzer LA, Liegl-Atzwanger B, Beham-Schmid C, Leithner A. Langerhans cell sarcoma: a case report and review of the literature. Pol J Pathol 2016; 67(2):172-8. [PubMed]
5. Mourah S, Lorillon G, Meignin V, Vercellino L, de Margerie-Mellon C, Pages C et al. Dramatic transient improvement of metastatic BRAF(V600E)-mutated Langerhans cell sarcoma under treatment with dabrafenib. Blood 2015;126(24):2649-52. doi: 10.1182/blood-2015-06-650036.
6. Ma X, Li W, Du J, Cai L, Zhang J. A case of an adult Langerhans cell sarcoma. Int J Dermatol 2016; 55(1):92-6. doi: 10.1111/ijd.12440. [PubMed]
7. Lakshmaiah KC, Smitha CS, Lokanatha D, Linu AJ, Premalata CS, Usha Aet al. Langerhans Cell Sarcoma – Review of Literature and a Rare Case Report . J Mol Biomark Diagn 5:181. doi:10.4172/2155-9929.1000181.
8. Ben-Ezra J, Bailey A, Azumi N, Delsol G, Stroup R, Sheibani K et al. Malignant histiocytosis X. A distinct clinicopathologic entity. Cancer. 1991; 68(5):1050-60.
9. Langerhans Cell Histiocytosis. Histiocyte Society Evaulation and Treatment Guidelines. Available from: https://histiocytesociety.org/document.doc?id=290 [Accessed 10th March 2015]
10. Zwerdling T, Won E, Shane L, Javahara R, Jaffe R. Langerhans cell sarcoma: case report and review of world literature. J Pediatr Hematol Oncol 2014; 36 (6): 419-25. doi:10.1097/MPH.0000000000000196. [PubMed]
11. Chung WD, Im SA, Chung NG, Park GS. Langerhans Cell Sarcoma in Two Young Children: Imaging Findings on Initial Presentation and Recurrence. Korean J Radiol 2013;14(3):520-4. doi: 10.3348/kjr.2013.14.3.520.
12. Luo B, Pian B, Peng Z, Jintao D, Shixi L. Pharyngeal tonsil manifestation of Langerhans cells sarcoma: a case report and review of the literature. Int J Pediatr Otorhinolaryngol Extra 2011;6:156-8. DOI: http://dx.doi.org/10.1016/j.pedex.2010.06.004.
13. Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48(2):124-31. DOI: 10.1002/pbc.21039.
14. Uchida K, Kobayashi S, Inukai T, Noriki S, Imamura Y, Nakajima H et al. Langerhans cell sarcoma emanating from the upper arm skin: successful treatment by MAID regimen. J Orthop Sci 2008;13(1): 89-93. DOI:10.1007/s00776-007-1184-4. [PubMed]
15. Yoshimi A, Kumano K, Motokura T, Takazawa Y, Oota S, Chiba S et al. ESHAP therapy effective in a patient with Langerhans cell sarcoma. Int J Hematol 2008; 87(5): 532-7. doi: 10.1007/s12185-008-0075-6. [PubMed]
16. Tatsuno M, Shioda Y, Iwafuchi H, Yamazaki S, Iijima K, Takahashi C, et al. BRAF V600 mutations in Langerhans cell histiocytosis with a simple and unique assay. Diagn Pathol 2016; 11: 39. doi: 10.1186/s13000-016-0489-z. [PubMed]
17. Chen W, Jaffe R, Zhang L, Hill C, Block AM, Sait S et al. Langerhans Cell Sarcoma Arising from Chronic Lymphocytic Lymphoma/Small Lymphocytic Leukemia: Lineage Analysis and BRAF V600E Mutation Study. N Am J Med Sci 2013; 5(6):386-91. doi: 10.4103/1947-2714.114172.
18. O'Malley DP, Agrawal R, Grimm KE, Hummel J, Glazyrin A, Dim DC, et al. Evidence of BRAF V600E in indeterminate cell tumor and interdigitating dendritic cell sarcoma. Ann Diagn Pathol 2015; 19(3):113-6. doi: 10.1016/j.anndiagpath.2015.02.008. [PubMed]
2. Howard JE, Dwivedi RC, Materson L, Jani P. Langerhans cell sarcoma: a systematic review. Cancer Treat Rev 2015;41(4):320-31. doi: 10.1016/j.ctrv.2015.02.011. [PubMed]
3. Zhang Y, Qu Z, Fang F. Langerhans cell sarcoma originating from left knee subcutaneous tissue: A case report and literature review. Oncol Lett 2016; 12(5):3687-94.
4. Liu DT, Friesenbichler J, Holzer LA, Liegl-Atzwanger B, Beham-Schmid C, Leithner A. Langerhans cell sarcoma: a case report and review of the literature. Pol J Pathol 2016; 67(2):172-8. [PubMed]
5. Mourah S, Lorillon G, Meignin V, Vercellino L, de Margerie-Mellon C, Pages C et al. Dramatic transient improvement of metastatic BRAF(V600E)-mutated Langerhans cell sarcoma under treatment with dabrafenib. Blood 2015;126(24):2649-52. doi: 10.1182/blood-2015-06-650036.
6. Ma X, Li W, Du J, Cai L, Zhang J. A case of an adult Langerhans cell sarcoma. Int J Dermatol 2016; 55(1):92-6. doi: 10.1111/ijd.12440. [PubMed]
7. Lakshmaiah KC, Smitha CS, Lokanatha D, Linu AJ, Premalata CS, Usha Aet al. Langerhans Cell Sarcoma – Review of Literature and a Rare Case Report . J Mol Biomark Diagn 5:181. doi:10.4172/2155-9929.1000181.
8. Ben-Ezra J, Bailey A, Azumi N, Delsol G, Stroup R, Sheibani K et al. Malignant histiocytosis X. A distinct clinicopathologic entity. Cancer. 1991; 68(5):1050-60.
9. Langerhans Cell Histiocytosis. Histiocyte Society Evaulation and Treatment Guidelines. Available from: https://histiocytesociety.org/document.doc?id=290 [Accessed 10th March 2015]
10. Zwerdling T, Won E, Shane L, Javahara R, Jaffe R. Langerhans cell sarcoma: case report and review of world literature. J Pediatr Hematol Oncol 2014; 36 (6): 419-25. doi:10.1097/MPH.0000000000000196. [PubMed]
11. Chung WD, Im SA, Chung NG, Park GS. Langerhans Cell Sarcoma in Two Young Children: Imaging Findings on Initial Presentation and Recurrence. Korean J Radiol 2013;14(3):520-4. doi: 10.3348/kjr.2013.14.3.520.
12. Luo B, Pian B, Peng Z, Jintao D, Shixi L. Pharyngeal tonsil manifestation of Langerhans cells sarcoma: a case report and review of the literature. Int J Pediatr Otorhinolaryngol Extra 2011;6:156-8. DOI: http://dx.doi.org/10.1016/j.pedex.2010.06.004.
13. Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48(2):124-31. DOI: 10.1002/pbc.21039.
14. Uchida K, Kobayashi S, Inukai T, Noriki S, Imamura Y, Nakajima H et al. Langerhans cell sarcoma emanating from the upper arm skin: successful treatment by MAID regimen. J Orthop Sci 2008;13(1): 89-93. DOI:10.1007/s00776-007-1184-4. [PubMed]
15. Yoshimi A, Kumano K, Motokura T, Takazawa Y, Oota S, Chiba S et al. ESHAP therapy effective in a patient with Langerhans cell sarcoma. Int J Hematol 2008; 87(5): 532-7. doi: 10.1007/s12185-008-0075-6. [PubMed]
16. Tatsuno M, Shioda Y, Iwafuchi H, Yamazaki S, Iijima K, Takahashi C, et al. BRAF V600 mutations in Langerhans cell histiocytosis with a simple and unique assay. Diagn Pathol 2016; 11: 39. doi: 10.1186/s13000-016-0489-z. [PubMed]
17. Chen W, Jaffe R, Zhang L, Hill C, Block AM, Sait S et al. Langerhans Cell Sarcoma Arising from Chronic Lymphocytic Lymphoma/Small Lymphocytic Leukemia: Lineage Analysis and BRAF V600E Mutation Study. N Am J Med Sci 2013; 5(6):386-91. doi: 10.4103/1947-2714.114172.
18. O'Malley DP, Agrawal R, Grimm KE, Hummel J, Glazyrin A, Dim DC, et al. Evidence of BRAF V600E in indeterminate cell tumor and interdigitating dendritic cell sarcoma. Ann Diagn Pathol 2015; 19(3):113-6. doi: 10.1016/j.anndiagpath.2015.02.008. [PubMed]
CITATION
DOI: 10.17511/ijpr.2017.i02.19
Published: 2017-02-28
How to Cite
Dr. Hosalli S Surendra, Dr. Sumant Prabhudesai, Dr. Arathi Srinivasan, Dr. Bala Ramachandran, & Dr. Julius Xavier Scott. (2017). Langerhans cell sarcoma presenting as a mediastinal mass in a young infant: a case report. Pediatric Review: International Journal of Pediatric Research, 4(2), 191-194. https://doi.org/10.17511/ijpr.2017.i02.19
Section
Case Report
Copyright (c) 2017 Author (s). Published by Siddharth Health Research and Social Welfare Society
This work is licensed under a Creative Commons Attribution 4.0 International License.
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