BACKGROUND: Basal cell carcinoma (BCC) of the vulva comprises 2-4% of all vulvar cancers. In general, vulvar BCCs tend to grow at slow rates. Nonetheless, they may be locally invasive and destructive if they are neglected. Eight cases of vulvar BCC metastatic to regional lymph nodes have been documented in the literature. CASES: Two unusual cases of vulvar BCC are presented. Case 1 is an 86-year-old white woman who presented with vulvar BCC metastatic to the femoral head. This is the first report of hematogeneous metastasis of vulvar BCC. The patient was treated with palliative vulvar resection and radiation to the femoral metastasis. At 6 months, she progressed with multiple bony and intraperitoneal metastases and died with disease. Case 2 is vulvar BCC in a 90-year-old African-American woman. She was managed by wide local excision and remains disease free to date. CONCLUSION: Vulvar BCCs are rare tumors with an unclear etiology. They can be aggressive and are capable of causing significant morbidity and occasional mortality if they are neglected or improperly treated. Hematogeneous metastasis at presentation appears to result in rapidly progressive disease. The literature regarding the pathogenesis, biologic behavior, and treatment of vulvar BCC is reviewed.
BACKGROUND:Basal cell carcinoma (BCC) of the vulva comprises 2-4% of all vulvar cancers. In general, vulvar BCCs tend to grow at slow rates. Nonetheless, they may be locally invasive and destructive if they are neglected. Eight cases of vulvar BCC metastatic to regional lymph nodes have been documented in the literature. CASES: Two unusual cases of vulvar BCC are presented. Case 1 is an 86-year-old white woman who presented with vulvar BCC metastatic to the femoral head. This is the first report of hematogeneous metastasis of vulvar BCC. The patient was treated with palliative vulvar resection and radiation to the femoral metastasis. At 6 months, she progressed with multiple bony and intraperitoneal metastases and died with disease. Case 2 is vulvar BCC in a 90-year-old African-American woman. She was managed by wide local excision and remains disease free to date. CONCLUSION: Vulvar BCCs are rare tumors with an unclear etiology. They can be aggressive and are capable of causing significant morbidity and occasional mortality if they are neglected or improperly treated. Hematogeneous metastasis at presentation appears to result in rapidly progressive disease. The literature regarding the pathogenesis, biologic behavior, and treatment of vulvar BCC is reviewed.
Authors: Rama P Namuduri; Timothy Yk Lim; Philip Kl Yam; Rene Gatsinga; Soo Kim Lim-Tan; Sung Hock Chew; Mark Ja Koh; Sorsiah Mansor Journal: Singapore Med J Date: 2019-02-18 Impact factor: 1.858