02 April 2024 : Case report
[In Press] Langerhans Cell Histiocytosis in Sphenoid Sinus: Uncommon Bone Involvement
Challenging differential diagnosis, Rare disease
Taynara Luisa de Mello Heliodoro 12ABEF, Paulo Ronaldo Jubé Ribeiro 3AB, Luciana Ximenes Salustiano 3BC, Leandro Azevedo de Camargo 3AEF, Fayez Bahmad Jr. 12EFDOI: 10.12659/AJCR.942681
Am J Case Rep In Press; DOI: 10.12659/AJCR.942681
Available online: 2024-04-02, In Press, Corrected Proof
Publication in the "In-Press" formula aims at speeding up the public availability of the pending manuscript while waiting for the final publication. The assigned DOI number is active and citable. The availability of the article in the Medline, PubMed and PMC databases as well as Web of Science will be obtained after the final publication according to the journal schedule
Abstract
BACKGROUND
Langerhans cell histiocytosis (LCH) is a rare and uncontrolled proliferation of dendritic cells of myeloid origin. The incidence of LHC was estimated at 5 cases per million children ages 0-15 years old. The most common places for this tumor are the jaw, vertebra, pelvis, and the extremities. The disease with multisystem involvement can present a mortality rate of 20% and one-third of children have multisystem involvement. We present a case with unusual bone involvement of the anterior cranial base with a challenging diagnosis and a complex surgical approach.
CASE REPORT
We report the case of a 6-year-old boy who manifested the disease with daily holocranial headache, worse in the frontal region and refractory to analgesia for 10 days, strabismus homonymous, diplopia, and right palpebral ptosis. The tumor affected the sphenoid sinus, internal carotid artery, and sella turcica, and made contact with the pituitary gland. A joint surgery with Otorhinolaryngology and Neurosurgery was performed by nasal endoscopic access to the skull base by means of the right medial turbinectomy (for the access) and right sphenoid opening, septectomy and opening of the left sphenoid to work with 4 hands and, after resection of lesion, inside the sphenoid.
CONCLUSIONS
This patient had rare bone involvement from LCH and atypical clinical presentation next to the important and delicate structures of the anterior skull base, but had a satisfactory outcome.
Keywords: Dendritic Cells; Headache; Myeloid Cells; Skull Base Neoplasms; Strabismus
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