Juvenile xanthogranuloma of skull base. Case report and review of the literature

Abstract

Dear Editor, Juvenile xanthogranuloma (JXG) is a histiocytic nonproliferative disease of the non-Langerhans cell group. It is usually characterized by solitary or multiple skin lesions. Extracutaneous manifestations of JXG without systemic involvement are uncommon, and isolated JXG involving the skull base is extremely rare [7, 8]. We report a 3-month old patient who presented with a large mass eroding the left temporoccipital bone. A three-month old boy was referred to our hospital for a left retroauricular tumor. MRI showed an intracranial extradural retromastoid lytic lesion that was isointense on T1 and T2, and showed a slight enhancement with contrast injection (Fig. 1a). The lesion destroyed the temporal bone and displaced the left cerebellar hemisphere, producing supratentorial ventricular dilatation. Neurological examination showed no other abnormalities. Given the solitary character of the tumor and its size and location, and destructive characteristics it was decided to perform resective surgery. A retrosigmoid approach was carried out and a subperiosteal tumour was found. The lesion was completely extradural. A complete macroscopic resection was made. Histologic examination of the surgical specimen showed evidence of bone and soft tissue. The tumour was composed of histiocytic mononuclear cells accompanied by multinucleated giant cells of osteoclast and Touton types. Multinuclear and mononuclear cells were positive for CD68 and negative for CD1a and S-100 protein. Histological and immunohistochemical findings confirmed the diagnosis of JXG. A control MRI performed within 24-h post-surgery showed no residual tumour or any contrast uptake (Fig. 1b). Based on the benign nature of the tumor and after complete macroscopic resection no further treatment was given and the patient was discharged. At most recent follow-up at 3 years after surgery, the patient remains asymptomatic and free of pathology to MRI. The first report documenting single, and occasionally multiple, cutaneous lesions occurring in infants and showing spontaneous involution was written by Adamson in 1905 [1]. In 1954 Helwig and Hackney proposed the name “juvenile xanthogranuloma” to define cutaneous lesions that arise from fibrohistiocytes [8]. Juvenile xanthogranulomas have been described as solitary or multiple swellings that are nodular or plaquiform and yellow to yellowish-brown in colour. Their size varies frommillimeters to a few centimeters in diameter. As the name implies, these lesions generally occurs in the first 4 years of life [7]. The most frequently involved organ is the skin, and these tumors aremore likely to present in the head and neck. However, there are reports that document juvenile xanthogranuloma cases involving extracutaneous sites like the eyes, heart, pericardium, liver, lungs, spleen, testes and oral cavity [7]. Also documented in the literature are skeletal JXG manifestations in the context of systemic cases, but solitary osseous lesions are rare [4, 5, 13]. Involvement of the brain and its coverings are also reported [9, 12]. More often they arise in the sellar region [2, 10, 11]. Xanthogranuloma of the sella should be included in the differential diagnosis of a pediatric tumor of the sellar region. Often, xanthogranuloma is difficult to differentiate from craniopharyngioma and Rathke s cleft cyst based only on the findings of MR imaging [11]. Histopathological examination of JXG shows wellcircumscribed nodules with dense infiltrates of histiocytes. Variable numbers of multinucleated giant cells and, in 85 % of cases, Touton giant cells are present [3]. Although the appearance of histiocytic disorders may be quite similar, their P. Puerta (*) : S. Candela :G. Garcia Fructuoso Department of Neurosurgery, Sant Joan de Deu Hospital, Paseo Sant Joan de Deu, 2, 08950, Esplugues de Llobregat, Barcelona, Spain e-mail: puertaroldan82@gmail.com