Concomitant Langerhans cell histiocytosis of cervical lymph nodes in adult patients with papillary thyroid carcinoma: A report of two cases and review of the literature

Authors

  • Bayan Maraqa King Hussein Cancer Center, Department of Pathology and Laboratory Medicine, Amman, Jordan
  • Maxim Al-Ashhab King Hussein Cancer Center, Department of Pathology and Laboratory Medicine, Amman, Jordan
  • Nazmi Kamal King Hussein Cancer Center, Department of Pathology and Laboratory Medicine, Amman, Jordan
  • Mousa El Khaldi King Hussein Cancer Center, Department of Radiology, Amman, Jordan
  • Maher Sughayer King Hussein Cancer Center, Department of Pathology and Laboratory Medicine, Amman, Jordan

DOI:

https://doi.org/10.4322/acr.2021.253

Keywords:

Langerhans cell histiocytosis, papillary thyroid carcinoma, adults, BRAF

Abstract

Objective: Langerhans cell histiocytosis (LCH) is an uncommon entity of unknown etiology. It contains a wide range of clinical presentations. The discovery of oncogenic BRAF V600E mutation in LCH has provided additional evidence that LCH is a neoplasm. Papillary thyroid carcinoma is the most common cancer of the thyroid characterized by a high incidence of BRAF V600E mutations. LCH with concomitant PTC is rare, with few cases reported in the literature. Cases summary: We identified two cases of LCH with concomitant papillary thyroid carcinoma in adult patients. The first was a 49-year-old female with a thyroid nodule diagnosed with papillary thyroid carcinoma. Later, the patient had a left neck mass; Ultrasound-guided lymph node FNA was diagnosed with Langerhans histiocytosis. Subsequently, a chest CT scan revealed signs of Langerhans cell histiocytosis in the lung. The second case refers to a 69-year-old male who presented with a left thyroid nodule diagnosed on FNA cytology as papillary thyroid carcinoma. The patient was found to have multiple bone lytic lesions. Biopsies revealed Langerhans cell histiocytosis. Later, the patient experienced LCH involvement of the bone marrow with associated secondary myelofibrosis. Conclusions: LCH is rare in adults; the association with papillary thyroid carcinoma is reported and should be considered in the presence of Langerhans cell groups along with PTC, whether in the thyroid gland or cervical lymph nodes. Once LCH has been diagnosed, pulmonary involvement should also be investigated. This will direct treatment plans for patients with pulmonary or systemic disease involvement.

Downloads

Download data is not yet available.

References

Harmon CM, Brown N. Langerhans cell histiocytosis: A clinicopathologic review and molecular pathogenetic update. Arch Pathol Lab Med. 2015;139(10):1211-4. http://dx.doi.org/10.5858/arpa.2015-0199-RA. PMid:26414464.

Swerdlow S, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. IARC: Lyon. 2017. 421 p.

Orkin SH, Nathan DG, Ginsburg D, Look AT, Fisher DE, Lux S. Nathan and Oski's hematology and oncology of infancy and childhood. Philadelphia: Elsevier Health Sciences; 2014.

Choi JE, Lee HR, Ohn JH, et al. Adult multisystem langerhans cell histiocytosis presenting with central diabetes insipidus successfully treated with chemotherapy. Endocrinol Metab (Seoul). 2014;29(3):394-9. http://dx.doi.org/10.3803/EnM.2014.29.3.394. PMid:25309800.

Peters GE, Harder SL, Fladeland D, Ward HA. Occult presentation of adult Langerhans Cell Histiocytosis with extrapulmonic manifestations. Chest. 2009;136(4):54S. http://dx.doi.org/10.1378/chest.136.4_MeetingAbstracts.54S-c.

Girschikofsky M, Tazi A. Adult langerhans cell histiocytosis. Histiocytic Disorders. Springer; 2018. p. 139-53.

Minkov M, Pötschger U, Grois N, Gadner H, Dworzak MN. Bone marrow assessment in Langerhans cell histiocytosis. Pediatr Blood Cancer. 2007;49(5):694-8. http://dx.doi.org/10.1002/pbc.21227. PMid:17455318.

Attakkil A, Thorawade V, Jagade M, Kar R, Parelkar K. Isolated Langerhans histiocytosis in thyroid: thyroidectomy or chemotherapy? J Clin Diagn Res. 2015;9(9):XD01-03. http://dx.doi.org/10.7860/JCDR/2015/12313.6466. PMid:26500997.

Willman CL, Busque L, Griffith BB, et al. Langerhans’-cell histiocytosis (histiocytosis X)--A clonal proliferative disease. N Engl J Med. 1994;331(3):154-60. http://dx.doi.org/10.1056/NEJM199407213310303. PMid:8008029.

Badalian-Very G, Vergilio JA, Degar BA, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010;116(11):1919-23. http://dx.doi.org/10.1182/blood-2010-04-279083. PMid:20519626.

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):669-83. http://dx.doi.org/10.1084/jem.20130977. PMid:24638167.

Go H, Jeon YK, Huh J, et al. Frequent detection of BRAFV 600E mutations in histiocytic and dendritic cell neoplasms. Histopathology. 2014;65(2):261-72. http://dx.doi.org/10.1111/his.12416. PMid:24720374.

Roden AC, Hu X, Kip S, et al. BRAF V600E expression in Langerhans cell histiocytosis: clinical and immunohistochemical study on 25 pulmonary and 54 extrapulmonary cases. Am J Surg Pathol. 2014;38(4):548-51. http://dx.doi.org/10.1097/PAS.0000000000000129. PMid:24625419.

Girschikofsky M, Arico M, Castillo D, et al. Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net. Orphanet J Rare Dis. 2013;8(1):72. http://dx.doi.org/10.1186/1750-1172-8-72. PMid:23672541.

Hyman DM, Puzanov I, Subbiah V, et al. Vemurafenib in multiple nonmelanoma cancers with BRAF V600 Mutations. N Engl J Med. 2015;373(8):726-36. http://dx.doi.org/10.1056/NEJMoa1502309. PMid:26287849.

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.

Czarniecka A, Oczko-Wojciechowska M, Barczyński M. BRAF V600E mutation in prognostication of papillary thyroid cancer (PTC) recurrence. Gland Surg. 2016;5(5):495-505. http://dx.doi.org/10.21037/gs.2016.09.09. PMid:27867864.

Goldstein N, Layfield LJ. Thyromegaly secondary to simultaneous papillary carcinoma and histiocytosis X. Report of a case and review of the literature. Acta Cytol. 1991;35(4):422-6. PMid:1927175.

Safali M, McCutcheon JM, Wright DH. Langerhans cell histiocytosis of lymph nodes: draining a papillary carcinoma of the thyroid. Histopathology. 1997;30(6):599-603. http://dx.doi.org/10.1046/j.1365-2559.1997.5590802.x. PMid:9205869.

Saiz E, Bakotic BW. Isolated Langerhans cell histiocytosis of the thyroid: a report of two cases with nuclear imaging-pathologic correlation. Ann Diagn Pathol. 2000;4(1):23-8. http://dx.doi.org/10.1016/S1092-9134(00)90006-6. PMid:10684377.

Foulet-Rogé A, Josselin N, Guyetant S, et al. Incidental langerhans cell histiocytosis of thyroid: case report and review of the literature. Endocr Pathol. 2002;13(3):227-33. http://dx.doi.org/10.1385/EP:13:3:227. PMid:12446922.

Jamaati HR, Shadmehr MB, Saidi B, Khosravi A, Arab M, Mohammadi F. Langerhans cell histiocytosis of the lung and thyroid, co-existing with papillary thyroid cancer. Endocr Pathol. 2009;20(2):133-6. http://dx.doi.org/10.1007/s12022-009-9068-0. PMid:19337865.

Vergez S, Rouquette I, Ancey M, Serrano E, Caron P. Langerhans cell histiocytosis of the thyroid is a rare entity, but an association with a papillary thyroid carcinoma is often described. Endocr Pathol. 2010;21(4):274-6. http://dx.doi.org/10.1007/s12022-010-9134-7. PMid:20848238.

Chung DH, Ha SY, Cho HY, et al. Langerhans cell histiocytosis in the thyroid and draining lymph nodes: a case report. Endocrinol Metab (Seoul). 2012;27(2):138-41. http://dx.doi.org/10.3803/EnM.2012.27.2.138.

Ceyran AB, Senol S, Bayraktar B, Ozkanlı S, Cinel ZL, Aydın A. Langerhans cell histiocytosis of the thyroid with multiple cervical lymph node involvement accompanying metastatic thyroid papillary carcinoma. Case Rep Pathol. 2014;2014:184237. http://dx.doi.org/10.1155/2014/184237. PMid:25349760.

Gordon MS, Gordon MB. Occult Langerhans cell Histiocytosis presenting with papillary thyroid carcinoma, a thickened pituitary stalk and diabetes insipidus. Case Rep Endocrinol. 2016;2016:5191903.

AlZahrani R, Algarni M, Alhakami H, et al. Thyroid Langerhans cell histiocytosis and papillary thyroid carcinoma. Gland Surg. 2016;5(5):537-40. http://dx.doi.org/10.21037/gs.2016.09.03. PMid:27867869.

Wu X, Chen S, Zhang LY, Luo YP, Jiang Y, Feng RE. Langerhans cell histiocytosis of the thyroid complicated by papillary thyroid carcinoma: A case report and brief literature review. Medicine (Baltimore). 2017;96(35):e7954. http://dx.doi.org/10.1097/MD.0000000000007954. PMid:28858125.

Al Hamad MA, Albisher HM, Al Saeed WR, Almumtin AT, Allabbad FM, Shawarby MA. BRAF gene mutations in synchronous papillary thyroid carcinoma and Langerhans cell histiocytosis co-existing in the thyroid gland: a case report and literature review. BMC Cancer. 2019;19(1):170. http://dx.doi.org/10.1186/s12885-019-5372-3. PMid:30795755.

Ozisik H, Yurekli BS, Demir D, et al. Langerhans cell histiocytosis of the thyroid together with papillary thyroid carcinoma. Hormones (Athens). 2020;19(2):253-9. http://dx.doi.org/10.1007/s42000-020-00181-2. PMid:32314308.

Burnett A, Carney D, Mukhopadhyay S, Scalzetti EM, Leino D, Souid AK. Thyroid involvement with Langerhans cell histiocytosis in a 3-year-old male. Pediatr Blood Cancer. 2008;50(3):726-7. http://dx.doi.org/10.1002/pbc.21030. PMid:16944476.

Thompson LD. Langerhans cell histiocytosis isolated to the thyroid gland. European archives of oto-rhino-laryngology: official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS): Affiliated with the German Society for Oto-Rhino-Laryngology. Head Neck Surg. 1996;253(1-2):62-5.

Schofield JB, Alsanjari NA, Davis J, MacLennan KA. Eosinophilic granuloma of lymph nodes associated with metastatic papillary carcinoma of the thyroid. Histopathology. 1992;20(2):181-3. http://dx.doi.org/10.1111/j.1365-2559.1992.tb00952.x. PMid:1559673.

Richmond I, Eyden BP, Banerjee SS. Intranodal Langerhans’ cell histiocytosis associated with malignant melanoma. Histopathology. 1995;26(4):380-2. http://dx.doi.org/10.1111/j.1365-2559.1995.tb00205.x. PMid:7607630.

Egeler RM, Neglia JP, Puccetti DM, Brennan CA, Nesbit ME. Association of Langerhans cell histiocytosis with malignant neoplasms. Cancer. 1993;71(3):865-73. http://dx.doi.org/10.1002/1097-0142(19930201)71:3<865::AID-CNCR2820710334>3.0.CO;2-0. PMid:8431870.

Ma J, Laird JH, Chau KW, Chelius MR, Lok BH. Langerhans cell histiocytosis in adults is associated with a high prevalence of hematologic and solid malignancies. Cancer Med. 2019;8(1):58-66.

Xing M. BRAF mutation in papillary thyroid cancer: pathogenic role, molecular bases, and clinical implications. Endocr Rev. 2007;28(7):742-62. http://dx.doi.org/10.1210/er.2007-0007. PMid:17940185.

Lloyd RVOR, Klöppel G, Rosai J. WHO classification of tumours of endocrine organs. Lyon, France: IARC; 2017.

Davies H, Bignell GR, Cox C, et al. Mutations of the BRAF gene in human cancer. Nature. 2002;417(6892):949-54. http://dx.doi.org/10.1038/nature00766. PMid:12068308.

Lo WC, Chen CC, Tsai CC, Cheng PW. Isolated adult Langerhans’ cell histiocytosis in cervical lymph nodes: should it be treated? J Laryngol Otol. 2009;123(9):1055-7. http://dx.doi.org/10.1017/S0022215108004155. PMid:19046468.

Downloads

Published

2021-03-24

Issue

Section

Clinical Case Report and Review

How to Cite

Maraqa, B., Al-Ashhab, M., Kamal, N., El Khaldi, M., & Sughayer, M. (2021). Concomitant Langerhans cell histiocytosis of cervical lymph nodes in adult patients with papillary thyroid carcinoma: A report of two cases and review of the literature. Autopsy and Case Reports, 11, e2021253. https://doi.org/10.4322/acr.2021.253