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  • In February a left neck infiltrating purpuric lesion around

    2019-05-10

    In February 2013, a left neck infiltrating purpuric lesion around a previous surgical scar (lymph node biopsy) with mild pain was noted. A subsequent skin biopsy revealed metastatic adenocarcinoma, compatible with ampulla of Vater primary, thereby confirming disease progression with skin metastasis. Consequently, chemotherapy with gemcitabine, oxaliplatin plus UFUR and local radiation therapy were administered. However, the lesion progressed with local itching, and chemotherapy with gemcitabine, oxaliplatin plus cetuximab was prescribed from Nov. 2013 to Feb. 2014. Unfortunately, the disease continued to progress and extended to his left chest wall, flank and back (Fig. 2A–B), so he received palliative chemotherapy with docetaxel, followed by cisplatin plus high-dose fluorouracil and FOLFIRI (last gap junction on June 17 2015). Palliative radiation therapy for skin and lymph node metastasis was also performed. Gram negative bacteremia and neutropenic fever were noted after recent chemotherapy. After further discussion with the patient about reasonable options, the patient opted to receive palliative care due to poor response to chemotherapy as well as multiple comorbidities. Presently, he remains under outpatient follow-up for palliative care.
    Discussion Cutaneous metastases can occur due to direct invasion of the tumor to the surrounding tissue, by hematogenous spread, or through lymphatic drainage. Particular sites of metastasis are seen in specific primary malignancies, but the pathogenesis is still unclear. It has been observed that skin metastasis is a poor prognostic feature. Some reports showed that skin metastasis from breast cancer had a median survival of 31–42 months, yet patient survival was less than 6 months among those from other primary cancers. According to our search of the literature, there have been only two cases of cutaneous metastasis from ampullary cancer previously reported. The ampulla of Vater is a vital structure traversed by important ducts and surrounded by the pancreas and duodenum. Preoperative staging may include such imaging and diagnostic tools as necessary, including extracorporeal ultrasonography (US), CT, MRCP, esophagogastroduodenoscopic biopsy, endoscopic US (EUS), and endoscopic retrograde cholangiopancreatography (ERCP). For non-metastatic disease, pancreaticoduodenectomy (Whipple procedure) is the gold standard treatment. Local resection or endoscopic papillectomy are also recognized as treatments of choice for selected early stage cases. Recent studies revealed that adequate regional lymph node dissection provided a survival benefit for T1 stage ampullary cancer (hazard ratio 0.19), compared with local resection/ampullectomy without regional lymphadenectomy. Our patient was diagnosed with ampullary cancer with distant lymph node metastasis by PET/CT, so he did not undergo surgical intervention but instead received biliary stenting by ERCP followed by chemotherapy. Ampullary cancer may arise either from the intestinal epithelium or the epithelium covering the pancreatobiliary ducts. Ninety percent of all ampullary malignancies are adenocarcinoma, which consist of two main histological subtypes: intestinal and pancreatobiliary. The two histological subtypes tend to have different clinical characteristics. The incidence of histological lymph node metastasis is much higher in cases of the pancreatobiliary type than in those of the intestinal type (50.0% versus 23.8%). Pancreatobiliary type also had an elevated likelihood of advanced local invasion and recurrence (5-year disease free survival rate 47.8% versus 73.1%). Our patient\'s pathology was pancreatobiliary type, and distant lymph node metastasis was noted at diagnosis. The immunohistochemical stain of skin biopsy from cutaneous metastasis was positive for cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20) (Fig. 3A–C), which was also compatible with pancreatobiliary origin instead of intestinal type (usually CK20(+) and CK7(−)).