Giant Solitary Sinonasal Enchondroma: A Rare Case Report

Enchondromas are benign tumors originating from the intramedullary matrix of bones, composed of nodules of hyaline cartilage [4]. They are typically solitary and have a high prevalence in hand bones, but can occur in other locations and be multiple, as seen in Ollier’s disease and Maffucci syndrome [5, 6]. Solitary cases usually manifest between the ages of 20 and 40, with no gender prevalence, while multiple cases debut in childhood or adolescence [7]. They can be asymptomatic, or, as in our case due to its location, grow and cause nasal respiratory insufficiency, eventually leading to pain and fractures due to compression.

The diagnosis is established through a combination of clinical, radiological, and histopathological findings. CT and MRI are useful tools for assessing the extent and planning treatment, which will depend on the size and location. On CT, these lesions typically appear as multiple osteolytic lesions with oval, linear, and/or pyramidal shapes and well-defined margins in the metaphysis and/or diaphysis of long tubular bones and flat bones. On MRI, they present as lobulated lesions with intermediate signal intensity on T1-weighted images and predominantly high signal intensity on T2-weighted sequences [8].

The differential diagnosis with low-grade chondrosarcoma, both through imaging and histopathology, is not always straightforward. These tumors typically exhibit low cellularity, with small, uniform chondrocytes without atypia within an abundant matrix of hyaline cartilage and some calcifications [1]. In our case, cortical disruption raised uncertainty in the diagnosis, as such findings are common in low-grade chondrosarcomas [3].

Surgery is the primary treatment and can be curative in most cases. For enchondromas in other locations, recurrence is common if the excision is incomplete, making a surgical approach aimed at achieving clear margins the standard [9, 10]. Due to the potential for transformation into chondrosarcoma, long-term follow-up with nasal endoscopies and imaging tests is advisable.

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