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.

Keywords: Langerhans cell histiocytosis; Prednisolone; Tonsillar neoplasms.

Fig. 1, 2

Langerhans cell histiocytosis (LCH) in the tonsil. Low-power view shows sheets of tonsil LCH cells with grooved, folded nuclei; pale eosinophilic cytoplasm; and interspersed eosinophils (Fig. 1; H + E, X50) (Fig. 2; H + E, X200)

Fig. 3

CD1a staining of the sinusoidal LCH cells are demonstrated (immunoperoxidase, CD1a X200)

Fig. 4

LCH cells are cytoplasmic positive with fascin (immunoperoxidase, Fascin X200)