medigraphic.com
ISSN 2007-3178 (Print)
Asociación Mexicana de Cirugía Bucal y Maxilofacial, Colegio Mexicano de Cirugía Bucal y Maxilofacial, A.C.

2025, Number 3

<< Back Next >>

Rev Mex Cir Bucal Maxilofac 2025; 21 (3)

Langerhans cell histiocytosis in bilateral mandibular body. Literature review and case report

Naranjo VJF, Sánchez PV, Vega ARJ

Language: Spanish
References: 28
Page: 109-126
PDF size: 3553.79 Kb.


ABSTRACT

Introduction: Langerhans cell histiocytosis (LCH) comprises a group of rare disorders of the reticuloendothelial system and represents an inflammatory myeloid neoplastic proliferation with variable clinical behavior, caused by the accumulation of CD1a+/CD207+ histiocytes associated with variable numbers of eosinophils, lymphocytes, plasma cells, and multinucleated giant cells. It is uncommon, with an estimated incidence of ~5 cases per million per year, occurring more frequently in early life (< 15 years) and with the highest incidence within the first three decades. Objective: to correlate the clinical findings of the present case with the available evidence in the literature. Case report: a 47-year-old male presented to the Maxillofacial Surgery Service of the "Lic. Adolfo López Mateos" Regional Hospital with spontaneous, constant pain and a periodontal defect with gingival recession involving the left mandibular central and lateral incisors and canine, with grade III mobility. A non-contrast CT scan of the maxillofacial complex (0.6-mm slices; March 11, 2023) showed ill-defined osteolytic lesions bilaterally in the mandibular body on axial, sagittal, and coronal views. Histopathology of the biopsy revealed fragments of well-vascularized mature loose fibrous tissue with areas of recent and old hemorrhage, sheets of ovoid to polygonal cells with abundant eosinophilic cytoplasm and ovoid nuclei with indentations and central folds, accompanied by numerous eosinophils and mixed inflammatory cell aggregates. Immunohistochemistry (QA-251-23; June 8, 2023) was positive for S-100, CD1a, and langerin, confirming LCH. Results: following surgical management, the patient became asymptomatic. He is currently undergoing oncologic evaluation and follow-up with our service, with consideration of intralesional corticosteroid therapy for the mandibular lesions. Conclusions: Langerhans cell histiocytosis is a rare entity of uncertain etiology, frequently associated with mutations in cellular signaling and proliferation pathways. This case underscores the importance of interdisciplinary management, the judicious use of nuclear medicine studies, and the evaluation of MAPK-pathway mutations (e.g., BRAF) for therapeutic stratification.


REFERENCES

  1. Soares EC, Quidute AR, Costa FW, Gurgel MH, Alves AP, Fonteles CS. Monostotic Langerhans' cell histiocytosis in a child with central diabetes insipidus. J Clin Pediatr Dent. 2012; 36 (4): 377-381. doi: 10.17796/jcpd.36.4.aq167251703n767u.

  2. Coppes-Zantinga A, Egeler RM. The Langerhans cell histiocytosis X files revealed. Br J Haematol. 2002; 116 (1): 3-9.

  3. Faustino ISP, Fernandes PM, Pontes HAR et al. Langerhans cell histiocytosis in the oral and maxillofacial region: An update. J Oral Pathol Med. 2021; 50 (6): 565-571. doi: 10.1111/jop.13207.

  4. Madrigal-Martínez-Pereda C, Guerrero-Rodríguez V, Guisado-Moya B et al. Langerhans cell histiocytosis: literature review and descriptive analysis of oral manifestations. Med Oral Patol Oral Cir Bucal. 2009; 14 (5): E222-228.

  5. Chugh A, Kaur A, Kumar Patnana A, Kumar P, Chugh VK. Unisystem Langerhans cell histiocytosis in maxillofacial region in pediatrics: comprehensive and systematic review. Oral Maxillofac Surg. 2021; 25 (4): 429-444. doi: 10.1007/s10006-021-00949-9.

  6. Bedran NR, Carlos R, de Andrade BAB, et al. Clinicopathological and immunohistochemical study of head and neck Langerhans cell histiocytosis from Latin America. Head Neck Pathol. 2018; 12 (4): 431-439.

  7. Pileri SA, Feldman AL, Cheuk W, Slater L. Langerhans cell histiocytosis. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ, editors. WHO classification of head and neck tumours. 4th ed. Lyon: IARC; 2017. p. 130-131.

  8. Castellón Zirpel ML, Fuenzalida Kakarieka C, Barrios Tapia JI, Uribe Fenner F. Histiocitosis de células de Langerhans localizada en hueso malar: presentación de un caso. Rev Esp Cirug Oral Maxilofac. 2011; 33 (3): 114-119. Disponible en: http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-05582011000300004. doi: 10.4321/S1130-05582011000300004.

  9. Lichtenstein L. Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schuller-Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol. 1953; 56 (1): 84-102.

  10. AbdullGaffar B, Awadhi F. Oral manifestations of Langerhans cell histiocytosis with unusual histomorphologic features. Ann Diagn Pathol. 2020; 47: 151536. doi: 10.1016/j.anndiagpath.2020.151536.

  11. Haroche J, Cohen-Aubart F, Rollins BJ et al. Histiocytoses: emerging neoplasia behind inflammation. Lancet Oncol. 2017; 18 (2): e113-e125. doi: 10.1016/S1470-2045(17)30031-1.

  12. Henter JI, Tondini C, Pritchard J. Histiocyte disorders. Crit Rev Oncol Hematol. 2004; 50 (2): 157-174.

  13. Snyder SR, Merkow LP, White NS. 1973; Eosinophilic granuloma of bone: report of case. J Oral Surg. 1973; 31: 712-715.

  14. Badalian-Very G, Vergilio JA, Degar BA, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010; 116 (11): 1919-1923.

  15. Berres ML, Lim KP, Peters T et al. BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups. J Exp Med. 2014; 211 (4): 669-683.

  16. Cho YA, Yoon HJ, Hong SD, Lee J, Hong SP. Hypothetical pathogenesis of eosinophilic infiltration in Langerhans cell histiocytosis of the jaw. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013; 116: 734-742.

  17. Weiss LM, Chan JKC, Fletcher CDM. Indeterminate dendritic cell tumor. In: Swerdlow SH, Campo E, Harris NL, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Revised 4th ed. Lyon: IARC; 2017. p. 474.

  18. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Revised 4th ed. Lyon: IARC; 2017. p. 359.

  19. Esen A, Dolanmaz D, Kalayci A, Günhan Ö, Avunduk MC. Treatment of localized langerhans' cell histiocytosis of the mandible with intralesional steroid injection: report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2010; 109 (2): e53-e58.

  20. Kilpatrick SE, Wenger DE, Gilchrist GS, Shives TC, Wollan PC, Unni KK. Langerhans' cell histiocytosis (histiocytosis X) of bone. A clinicopathologic analysis of 263 pediatric and adult cases. Cancer. 1995; 76 (12): 2471-2484. doi: 10.1002/1097-0142(19951215)76:12< 2471::aid-cncr2820761211> 3.0.co;2-z.

  21. Monsereenusorn C, Rodriguez-Galindo C. Clinical characteristics and treatment of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 2015; 29: 853-873. doi: 10.1016/j.hoc.2015.06.005.

  22. Putters TF, de Visscher JGAM, van Veen A, Spijkervet FKL. Intralesional infiltration of corticosteroids in the treatment of localised Langerhans' cell histiocytosis of the mandible. Report of known cases and three new cases. Int J Oral Maxillofac Surg. 2005; 34 (5): 571-575.

  23. Bedran NR, Carlos R, de Andrade BAB, et al. Clinicopathological and Immunohistochemical Study of Head and Neck Langerhans Cell Histiocytosis from Latin America. Head Neck Pathol. 2018; 12 (4): 431-439.

  24. Minkov M. Multisystem Langerhans cell histiocytosis in children: current treatment and future directions. Paediatr Drugs. 2011; 13 (2): 75-86.

  25. Moralis A, Kunkel M, Kleinsasser N, Müller-Richter U, Reichert TE, Driemel O. Intralesional corticosteroid therapy for mandibular Langerhans cell histiocytosis preserving the intralesional tooth germ. Oral Maxillofac Surg. 2008; 12 (2): 105-111.

  26. Hwang DS, Lee JS, Kim UK, Park HR, Ryu MH, Lee JH, et al. Langerhans cell histiocytosis of the mandible: Two case reports and literature review. J Korean Assoc Oral Maxillofac Surg. 2019; 45 (3): 167-172.

  27. Medina Ruiz B, Arocho L, López del Valle LM, Molina Negrón D. Histiocitosis de células de Langerhans: seguimiento de 5 años del caso y revisión sistemática de literatura. Revista de Odontopediatría Latinoamericana. 2021;11(2). doi: 10.47990/alop.v11i2.231.

  28. Cisternino A, Asa'ad F, Fusco N, Ferrero S, Rasperini G. Role of multidisciplinary approach in a case of Langerhans cell histiocytosis with initial periodontal manifestations. Int J Clin Exp Pathol. 2015; 8 (10): 13539-13545.




2020     |     www.medigraphic.com