Metastatic leiomyosarcoma of the intrapancreatic bile duct
A 57-year-old female patient was referred to our department for surgical treatment of an intrapancreatic CBD tumor. The patient's past medical history was significant for hysterectomy and unilateral oophorectomy due to uterine leiomyoma ten years prior., and primary pulmonary leiomyosarcoma, which was treated with right upper lobectomy and lymph node dissection six months prior to her presentation. In order to diagnose the primary origin of the tumor, the patient underwent abdominal magnetic resonance imaging (MRI), which was negative. Histopathologic examination of the specimen revealed a malignant mesenchymal neoplasm, measuring 6 cm in greatest dimension, with morphological characteristics and immunophenotype compatible with leiomyosarcoma. Cell proliferation marker Ki-67 was positive in the majority of cells (>85%) and extensive necrosis was present. Immunohistochemical examination was positive for smooth muscle actin (SMA) and negative for S100. The neoplasm had locally infiltrated small vessels, one lymph node and the pleura.\nDuring the next six months the patient suffered three episodes of acute cholangitis. Conservative treatment resulted in remission of symptoms. Abdominal computed tomography (CT) scan showed dilation of intrahepatic and extrahepatic bile ducts and a tumor located in the distal CBD. Chest CT scan revealed bilateral micronodural lung lesions, compatible with metastases. An endoscopic ultrasonography confirmed the existence of the tumor in the endopancreatic portion of the CBD. Bile duct brushing cytology and cytological examination of aspirated bile fluid were negative for malignancy, while biopsies taken from the mass revealed high grade malignant neoplasm, without defining its exact histological type. The patient was then referred to our department for surgical treatment.\nUpon admission, physical examination and laboratory findings, including tumor markers, were normal. Under the preoperative diagnosis of periampullary tumor, pylorus-preserving pancreaticoduodenectomy was performed. Postoperative course was uneventful and the patient was discharged on the 12th postoperative day.\nHistopathologic examination of the specimen revealed a polypoid tumor, 1.5 × 1.4 × 2 cm in size, infiltrating the full thickness of the common bile duct, extending into its lumen, without obvious invasion of the pancreatic tissue (-). Tumor cell necrosis, high cellularity, many mitotic counts, pleomorphism, and nuclear atypia were present in the neoplasm. Immunohistochemical study gave positive results for HHF-35, SMA and desmin, focal positivity for CD68 and negative results for keratin 7, keratin 20, keratin AE1/AE3, careinoembryonal antigen, epithelial membrane antigen, synaptophysin, S-100, CD117 (c-kit) and CD34. Cell proliferation marker Ki-67 (MIB-1) was estimated at 40%. Morphological and immunohistochemical characteristics of the neoplasm were compatible with high grade polymorphous sarcoma with muscular differentiation. Resection margins were clear and none of the five lymph nodes found in the specimen was involved.\nSoon after the second surgery, the patient was set on adjuvant chemotherapy with gemcitabine plus docetaxel for the pulmonary metastases. Effective eradication of lung lesions was never accomplished and fifty eight months after the first surgery, the patient developed bone metastases. Ten months later the patient died, five and a half years after the first diagnosis of leiomyosarcoma.