RESUMEN
BACKGROUND: Giant basal cell carcinoma (GBCC) is an aggressive malignant neoplasm. Because of the rarity of the tumor and its recognized high risk of recurrence, there are no guidelines for its treatment. PATIENTS AND METHODS: Published articles in PubMed Central were carefully reviewed. Data from 48 patients obtained from 30 individual articles were added to our 3 cases, producing a total number of 51 cases of GBCC. A clinical database was established in order to define the behavior of this tumor, prognostic factors and optimal treatment. RESULTS: GBCC mostly occurs in elderly male patients, with a peak incidence in the seventh decade of life. It develops as long-standing dermal tumor with mean disease duration of 14.5 years and is most commonly located on the back, followed by the face and upper extremity. The most common histological subtype is nodular. The average size at presentation is 14.77 cm in its largest diameter. The presence of metastasis at the time of presentation represents the most significant adverse prognostic factor. Local recurrence or metastasis develops in 38.3% of patients despite optimal therapy. The overall reported cure rate is 61.7% by a mean follow-up of 2 years. Wide local excision of the tumor with or without postoperative radiochemotherapy represents the optimal treatment. CONCLUSION: Optimal management of GBCC consists of wide local excision with histologically confirmed tumor-free margins, frequently followed by adjuvant therapy. In cases of lymphatic spread, a regional lymphadenectomy is also necessary. In addition, consideration should be given to a close and long-term follow-up because of the high rate of locoregional recurrence.