User login
Progression of Merkel Cell Carcinoma With Unusual Sites of Metastasis: Two Rare Cases
Background
Merkel cell carcinoma (MCC) is a rare and aggressive primary cutaneous neuroendocrine carcinoma. The disease is associated with Merkel cell polyomavirus, immunosuppression, and ultra-violet radiation. Clinical presentation is highly varied and MCC is not suspected in majority of cases initially. It is rapidly growing and has a poor prognosis with a high mortality rate.
Case 1
A 75-year-old male initially presented with a cutaneous left facial lesion which on biopsy was confirmed to be Merkel cell carcinoma. He underwent wide local excision of the lesion with sentinel lymph node biopsy and was monitored. Six months later he was found to have palpable left parotid nodule, and underwent left parotidectomy and left neck lymph node dissection. The parotid nodule itself was of benign etiology; however, one of the cervical lymph nodes was positive for Merkel cell carcinoma with extranodal extension and lymphovascular invasion. Patient received adjuvant radiation to the site. A year after patient was noted to have bone lesions and other lesions concerning for visceral metastasis on surveillance imaging which were also confirmed to be recurrent MCC by biopsy. Patient was started on immunotherapy afterwards.
Case 2
A 60-year-male patient originally presented with a left inguinal mass. Pathology from the initial biopsy was noted to be small cell neuroendocrine carcinoma of unknown primary. Patient received six cycles of cisplatin and etoposide followed by radiation. Four months later patient was found to have local recurrence and underwent surgical resection, followed by five cycles of cyclophosphamide, doxorubicin, and vincristine. Five months later, the patient had a second recurrence in the surgical bed in addition to multiple subcutaneous nodules in the abdominal wall and lower extremity. A repeat biopsy was performed with Merkel cell polyomavirus testing by immunohistochemistry, which resulted as positive. Patient was started on immunotherapy afterwards.
Discussion
The above two cases represent how Merkel cell carcinoma can have varying presentations either on diagnosis or on progression. Thus, it is important to have a low threshold for biopsy or rebiopsy. Additionally, neuroendocrine carcinomas of unknown primary may benefit from additional Merkel cell IHC testing on diagnosis.
Background
Merkel cell carcinoma (MCC) is a rare and aggressive primary cutaneous neuroendocrine carcinoma. The disease is associated with Merkel cell polyomavirus, immunosuppression, and ultra-violet radiation. Clinical presentation is highly varied and MCC is not suspected in majority of cases initially. It is rapidly growing and has a poor prognosis with a high mortality rate.
Case 1
A 75-year-old male initially presented with a cutaneous left facial lesion which on biopsy was confirmed to be Merkel cell carcinoma. He underwent wide local excision of the lesion with sentinel lymph node biopsy and was monitored. Six months later he was found to have palpable left parotid nodule, and underwent left parotidectomy and left neck lymph node dissection. The parotid nodule itself was of benign etiology; however, one of the cervical lymph nodes was positive for Merkel cell carcinoma with extranodal extension and lymphovascular invasion. Patient received adjuvant radiation to the site. A year after patient was noted to have bone lesions and other lesions concerning for visceral metastasis on surveillance imaging which were also confirmed to be recurrent MCC by biopsy. Patient was started on immunotherapy afterwards.
Case 2
A 60-year-male patient originally presented with a left inguinal mass. Pathology from the initial biopsy was noted to be small cell neuroendocrine carcinoma of unknown primary. Patient received six cycles of cisplatin and etoposide followed by radiation. Four months later patient was found to have local recurrence and underwent surgical resection, followed by five cycles of cyclophosphamide, doxorubicin, and vincristine. Five months later, the patient had a second recurrence in the surgical bed in addition to multiple subcutaneous nodules in the abdominal wall and lower extremity. A repeat biopsy was performed with Merkel cell polyomavirus testing by immunohistochemistry, which resulted as positive. Patient was started on immunotherapy afterwards.
Discussion
The above two cases represent how Merkel cell carcinoma can have varying presentations either on diagnosis or on progression. Thus, it is important to have a low threshold for biopsy or rebiopsy. Additionally, neuroendocrine carcinomas of unknown primary may benefit from additional Merkel cell IHC testing on diagnosis.
Background
Merkel cell carcinoma (MCC) is a rare and aggressive primary cutaneous neuroendocrine carcinoma. The disease is associated with Merkel cell polyomavirus, immunosuppression, and ultra-violet radiation. Clinical presentation is highly varied and MCC is not suspected in majority of cases initially. It is rapidly growing and has a poor prognosis with a high mortality rate.
Case 1
A 75-year-old male initially presented with a cutaneous left facial lesion which on biopsy was confirmed to be Merkel cell carcinoma. He underwent wide local excision of the lesion with sentinel lymph node biopsy and was monitored. Six months later he was found to have palpable left parotid nodule, and underwent left parotidectomy and left neck lymph node dissection. The parotid nodule itself was of benign etiology; however, one of the cervical lymph nodes was positive for Merkel cell carcinoma with extranodal extension and lymphovascular invasion. Patient received adjuvant radiation to the site. A year after patient was noted to have bone lesions and other lesions concerning for visceral metastasis on surveillance imaging which were also confirmed to be recurrent MCC by biopsy. Patient was started on immunotherapy afterwards.
Case 2
A 60-year-male patient originally presented with a left inguinal mass. Pathology from the initial biopsy was noted to be small cell neuroendocrine carcinoma of unknown primary. Patient received six cycles of cisplatin and etoposide followed by radiation. Four months later patient was found to have local recurrence and underwent surgical resection, followed by five cycles of cyclophosphamide, doxorubicin, and vincristine. Five months later, the patient had a second recurrence in the surgical bed in addition to multiple subcutaneous nodules in the abdominal wall and lower extremity. A repeat biopsy was performed with Merkel cell polyomavirus testing by immunohistochemistry, which resulted as positive. Patient was started on immunotherapy afterwards.
Discussion
The above two cases represent how Merkel cell carcinoma can have varying presentations either on diagnosis or on progression. Thus, it is important to have a low threshold for biopsy or rebiopsy. Additionally, neuroendocrine carcinomas of unknown primary may benefit from additional Merkel cell IHC testing on diagnosis.