Merkel Cell Carcinoma
We report a case of recurrent Merkel cell carcinoma (MCC) of the upper extremity, treated aggressively with wide local excision, regional lymphadenectomy, and immediate reconstruction. Five years after surgery, there is no clinical or diagnostic evidence of locoregional recurrence or distant disease. The patient's upper extremity and hand remain fully functional, without evidence of median or ulnar nerve dysfunction. No donor site morbidity has been noted.
Originally described by Toker in 1972 as trabecular cell carcinoma, MCC or primary neuroendocrine tumor of the skin is an unusual, biologically aggressive, primary cutaneous neoplasm. Since this initial description, nearly 600 cases have been reported under various terms, including primary endocrine tumor of the skin, small-cell carcinoma of the skin, and apudoma of the skin. This aggressive neoplasm exhibits marked malignant potential with a high incidence of locoregional recurrence (30% to 45%), regional lymph node involvement (35% to 91%), and distant metastasis (30% to 50%). Additionally, disease-related mortality has been reported to be as high as 25% to 35%. It is evident that the clinical behavior of this neoplasm cannot be accurately predicted by histologic appearance. This aggressive neoplasm is often difficult to diagnose and even more difficult to treat, particularly in its late stages. Because of its rarity and undifferentiated histologic appearance by conventional light microscopy, this highly aggressive neoplasm is often misdiagnosed. In the past, MCC has been misclassified as poorly differentiated squamous cell carcinoma, amelanotic melanoma, sweat gland carcinoma, metastatic visceral carcinoma, Langerhans cell tumor, neuroblastoma, and metastatic oat cell carcinoma. The aggressive biologic nature, marked propensity for locoregional recurrence, and early metastatic spread of MCC mandate early accurate diagnosis and staging.
We report a case of recurrent Merkel cell carcinoma (MCC) of the upper extremity, treated aggressively with wide local excision, regional lymphadenectomy, and immediate reconstruction. Five years after surgery, there is no clinical or diagnostic evidence of locoregional recurrence or distant disease. The patient's upper extremity and hand remain fully functional, without evidence of median or ulnar nerve dysfunction. No donor site morbidity has been noted.
Originally described by Toker in 1972 as trabecular cell carcinoma, MCC or primary neuroendocrine tumor of the skin is an unusual, biologically aggressive, primary cutaneous neoplasm. Since this initial description, nearly 600 cases have been reported under various terms, including primary endocrine tumor of the skin, small-cell carcinoma of the skin, and apudoma of the skin. This aggressive neoplasm exhibits marked malignant potential with a high incidence of locoregional recurrence (30% to 45%), regional lymph node involvement (35% to 91%), and distant metastasis (30% to 50%). Additionally, disease-related mortality has been reported to be as high as 25% to 35%. It is evident that the clinical behavior of this neoplasm cannot be accurately predicted by histologic appearance. This aggressive neoplasm is often difficult to diagnose and even more difficult to treat, particularly in its late stages. Because of its rarity and undifferentiated histologic appearance by conventional light microscopy, this highly aggressive neoplasm is often misdiagnosed. In the past, MCC has been misclassified as poorly differentiated squamous cell carcinoma, amelanotic melanoma, sweat gland carcinoma, metastatic visceral carcinoma, Langerhans cell tumor, neuroblastoma, and metastatic oat cell carcinoma. The aggressive biologic nature, marked propensity for locoregional recurrence, and early metastatic spread of MCC mandate early accurate diagnosis and staging.
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