Key result
Vismodegib plus surgical excision manages recurrent basal cell carcinomas in Gorlin-Goltz syndrome.
Why the study?
Gorlin-Goltz syndrome is a rare genetic disorder with multiple systemic manifestations, and cardiac fibroma diagnosis and management in this context are not well described.
Case Report (n=1)
May support vismodegib plus excision for recurrent BCCs in Gorlin-Goltz syndrome; leaves open prospective validation and cardiac fibroma management.
Gorlin-Goltz syndrome is a rare autosomal dominant disease characterized by odontogenic keratocysts and basal cell carcinoma as well as ophthalmic and neurological implications. The following article presents the case of a 20-year-old female with Gorlin-Goltz syndrome incidentally found to have a cardiac mass. An ECG showed diffuse T-wave inversions in the lateral leads despite a lack of any acute coronary symptoms in the patient. Echocardiogram, cardiac MRI and CT scan confirmed a nonvascularized, smoothly marginated mass arising from the left ventricular apex without any hemodynamic compromise. A whole-body PET scan further demonstrated localized hyperactivity associated with a cardiac fibroma without any evidence of metastasis. The cardiac fibroma was surgically excised for definitive management to prevent the possibility of sudden cardiac death and congestive heart failure.
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Reaz et al. (2022) conducted a case report in Gorlin-Goltz syndrome (n=1). Surgical excision and vismodegib was evaluated. Multidisciplinary management involving surgical excision and targeted therapy with vismodegib was utilized to manage recurrent basal cell carcinomas in a patient with Gorlin-Goltz syndrome.
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