Tohoku J. Exp. Med., 2013 June, 230(2)

Isolated Juvenile Xanthogranuloma in the Larynx of a Three-Year-Old Child

AI KAWAMOTO,¹ YUKIO KATORI,¹ YOHEI HONKURA,¹ KEIKO ISHII,¹ MASAKI OGURA,¹ HIROSHI NAGANUMA,² RIE SHIBUYA,² TAKAHIRO SUZUKI³ and TOSHIMITSU KOBAYASHI³

¹Division of Otorhinolaryngology, Sendai City Hospital, Sendai, Miyagi, Japan
²Division of Surgical Pathology, Sendai City Hospital, Sendai, Miyagi, Japan
³Department of Otorhinolaryngology and Head and Neck Surgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan

Juvenile xanthogranuloma (JXG) is a benign manifestation of non-Langerhans cell histiocytosis characterized by yellowish cutaneous nodules. Its occurrence in the larynx is very rare, but laryngeal JXG may cause severe respiratory distress. We report a patient with isolated laryngeal JXG treated by laryngomicrosurgery, and this is the first report of JXG extending to vocal fold. A 3-year-old girl presented with hoarseness and inspiration stridor. A bulky tumor was found in right glottic to subglottic region. Subtotal resection of the tumor was carried out by laryngomicrosurgery, and airway distress was diminished after the operation. In pathological examination, the resected specimen showed proliferation of histiocytic cells and spindle cells with Touton giant cells that are characterized by polynuclei or wreath nuclei and are known to appear in JXG but not in LCH. Immunohistochemistry of histiocytic cell markers demonstrated positivity for CD68, lysozyme, alpha1-anti-chymotrypsin, factor XIIIa and vimentin, and negativity for CD1a and S-100, leading to diagnosis of JXG, but not LCH. The patient was thus expected with benign prognosis, and additional resection of the tumor including vocal fold was not indicated in the initial treatment. Six weeks later, the JXG recurred and a second procedure using CO₂ laser was needed. The tumor did not re-grow thereafter, and there was no residual voice handicap. Because of its favorable prognosis and tendency for spontaneous regression, JXG in the larynx needs to be considered carefully with regard to whether reduction surgery and/or tracheotomy are necessary, and thus precise diagnosis is required.

keywords —— juvenile xanthogranuloma; Langerhans cell histiocytosis; laryngomicrosurgery; larynx; respiratory distress

© 2013 Tohoku University Medical Press

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Tohoku J. Exp. Med., 2013, 230, 123-127

Correspondence: Ai Kawamoto, Division of Otolaryngology, Sendai City Hospital, 3-1, Shimizukoji, Wakabayashi, Sendai 984-8501, Japan.

e-mail: putchsan@mail.goo.ne.jp