A Rare Presentation of Langerhans Cell Histiocytosis Tonsil Infiltration: Review of the Literature: Atypical Presentation of Langerhans Cell Histiocytosis

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease that can infiltrate various organs. LCH presents with solitary organ involvement or as a multi-system disease. We present a patient who has tonsillary infiltration with LCH. A 74 year-old Caucasian male was admitted for swelling of the neck and difficulty swallowing for 3 months. Physical examination showed submandibular lymph node enlargement of approximately 3 cm and tonsil enlargement. A tonsillectomy and excisional biopsy of the lymph node were done. Histiocyte-like cell infiltration was seen in the tonsil biopsy. CD3, CD20, CD15, CD30, CD5, CD138, Lambda, Kappa, Bcl-2, ALK, CD23, CD10, Bcl-6, keratin, EMA, HMB-45, and Cyl D1 were negative. CD68, S-100, CD1a, and fascin were positive, and the Ki-67 proliferation index was 20 % in immunocytochemical staining. The most commonly infiltrated bones are the skull, femur, lower jaw, pelvis, and vertebrae in LCH. Oral or perioral lesions are present in 30 % of cases. Oral lesions most often involve bone loss, unexpected tooth loss, and gum inflammation. We administered oral prednisolone to our patient due to the presence of lytic lesion of the bone, mild anemia and a higher sedimentation rate, which was from a separate, explained cause. Isolated tonsillar involvement in adult LCH was reported in only 2 cases in the literature. There is no standard recommendation for treatment. Our patient responded well to steroid therapy.

Bilateral Basis Mandibulariste görülen Langerhans Hücreli Histiyositozis: Nadir Bir Olgu

“…Oral semptomları genellikle ağrı, şişlik, kemik kaybı, mobil dişler, diş kayıpları ve gingivitistir. Oral bölgede izole tonsil tutulumu sadece birkaç olguda bildirilmiştir(Atalay et al 2014). LHH için intraoral bölge genellikle ilk tutulum alanıdır.…”

Langerhans Cell Histiocytosis of the Tonsil

“… 1 , 2 The most commonly affected organs include the bones, skin, and pituitary gland; the hematopoietic system, lymph nodes, and lungs are rarely affected. 3 Tonsillar involvement is extremely rare. We report the case of a 36-year-old female patient with unusual unilateral tonsillar palatine involvement of LCH.…”