Chordoma of the Sella Turcica An Overview
Chordoma of the Sella Turcica An Overview Chordoma of the Sella Turcica: An Overview
Chordomas are rare, slow-growing malignant tumors that originate from remnants of the notochord, an embryonic structure that plays a vital role in the development of the axial skeleton. When these tumors develop in the region of the sella turcica, a saddle-shaped depression in the sphenoid bone at the base of the skull that houses the pituitary gland, they are referred to as chordomas of the sella turcica. Given their location, these tumors can present unique diagnostic and therapeutic challenges. Chordoma of the Sella Turcica An Overview
Chordoma of the Sella Turcica An Overview Typically, chordomas of the sella turcica account for a small percentage of all skull base tumors. They tend to affect adults, with a slight predilection for middle-aged individuals, although cases have been reported across a broader age range. Due to their slow growth rate, patients might experience symptoms over months or even years before diagnosis. Common presenting complaints include visual disturbances such as bitemporal hemianopsia, headaches, and sometimes hormonal imbalances resulting from compression or invasion of the pituitary gland. These clinical features often prompt imaging studies that reveal the tumor‘s location and extent.
Imaging plays a crucial role in diagnosing sella turcica chordomas. Magnetic resonance imaging (MRI) is the modality of choice due to its superior soft tissue contrast, allowing detailed visualization of the tumor’s size, invasion into adjacent structures like the cavernous sinus, and relationship to the optic chiasm. On MRI, chordomas typically appear as lobulated, midline masses that are iso- to hypointense on T1-weighted images and hyperintense on T2-weighted sequences. They may also demonstrate calcifications and bony destruction, which are characteristic features suggestive of chordoma.
Histologically, chordomas exhibit distinctive features, including physaliphorous cells—large cells with bubbly, vacuolated cytoplasm—embedded within a myxoid matrix. Immunohistochemistry further aids diagnosis, with tumor cells often testing positive for brachyury, a transcri
ption factor that is highly specific for chordomas, as well as epithelial markers like cytokeratin and EMA. Chordoma of the Sella Turcica An Overview
Treatment strategies for sella turcica chordomas primarily involve surgical resection aimed at maximal tumor removal. Due to the complex anatomy of the skull base, complete excision can be challenging, and surgeons often use approaches such as transsphenoidal or combined transcranial-transsphenoidal techniques. Achieving a gross total resection improves prognosis but is sometimes limited by tumor invasion into critical structures like the optic apparatus or carotid arteries. Because of the high risk of local recurrence, adjuvant radiotherapy—often proton beam therapy—is frequently employed to target residual tumor cells.
Prognosis depends on various factors including tumor size, extent of invasion, the success of surgical removal, and the response to radiation therapy. While chordomas are considered low-grade malignancies, their infiltrative nature makes long-term control difficult. Recurrences are common, necessitating vigilant follow-up with regular imaging. Chordoma of the Sella Turcica An Overview
In summary, chordoma of the sella turcica is a rare but significant tumor that demands a multidisciplinary approach for diagnosis and management. Advances in surgical techniques and radiotherapy have improved outcomes, but challenges remain due to the tumor’s location and propensity for recurrence. Ongoing research continues to seek more effective therapies, aiming to enhance quality of life and survival for affected patients. Chordoma of the Sella Turcica An Overview
