Abstract

Juvenile Xanthogranulomas (JXGs) are rare, benign lesions that belong to the large group of non-Langerhans cell histiocytosis, often affecting infants and younger children. JXG usually presents with 1 or more erythematous or yellowish nodules that are most commonly located on the head or neck. On the contrary, we present a rare case of juvenile xanthogranuloma which presented as a large swelling in the gluteal region-a location that has been rarely described in literature. The baby underwent excision of gluteal swelling. Histopathology demonstrated a lymphohistiocytic lesion characterized by proliferation of mononuclear medium-sized histiocytes along with admixed multinucleated giant cells, variably lipidized xanthomatous and Touton-type giant cells. Immunohistochemistry showed immunopositivity for CD68 (Diffuse) and CD4 (Diffuse). Cutaneous JXGs are usually self-limiting and do not require treatment, unless large and causing functional impairment as seen in the case described. Systemic cases may even warrant chemotherapy. Case Report History-A 1-month-old child was brought with insidious onset right gluteal swelling over one month. The swelling was non-tender, with reddish discoloration of the overlying skin. Clinical Examination-The child was alert, active, and hemodynamically stable. Systemic examination was normal. Local examination showed a 4x2 cm swelling in the left gluteal region, which was non-tender and soft to firm in consistency. It was fixed to the underlying structures. The overlying skin was pinchable and seen to be mildly erythematous. No ulceration or discharge was present. No sacral abnormality was noted. The anal opening was normal. Investigations-Baseline investigations reported elevated AFP levels, and serial AFP levels reported a decreasing trend. Ultrasound showed a well-defined hypo-echoic lesion measuring 3.1 cm x 1.9 cm noted in the medial aspect of the left gluteal region at the level of the ischium in close relation with the perianal region, showing internal vascularity-possibly sacrococcygeal teratoma. MRI Pelvis subsequently showed a large lobulated altered signal intensity solid lesion showing predominantly peripheral diffusion restriction and heterogenous post-contrast enhancement arising adjacent to the base of the coccyx with a large exophytic component in the left gluteal region. CT Thorax showed no obvious metastases.