Primary retroperitoneal mucinous cystadenocarcinoma is a rare tumor. Only about 30 such cases have been reported in the worldwide literature, and a few Korean cases have been reported. The pathogenesis is not clear, and coelomic metaplasia of the retroperitoneal mesothelium has gained wide support. There is no consensus on the appropriate treatment, but surgical exploration is needed for the diagnosis and treatment, and adjuvant chemotherapy may be recommended following complete surgical excision. The long-term prognosis has not been established. We report here on a 32-year-old woman who was diagnosed as having a retroperitoneal mucinous cystadenocarcinoma with mural nodules of sarcomatoid change. Tumor excision and adjuvant chemotherapy were done and the patient is doing well without any evidence of recurrence at 42 months postoperatively.
Adult ; Cystadenocarcinoma, Mucinous/*diagnosis/pathology/surgery ; Female ; Humans ; Retroperitoneal Neoplasms/*diagnosis/pathology/surgery

BACKGROUND/AIMS: Intraductal papillary mucinous neoplasms (IPMN) and mucinous cystic neoplasms are included in mucin-producing pancreatic tumors. The reports about IPMN are not uncommon but those about the mucinous cystic neoplasms are relatively few. The aims of this study were to define the natural history of resected mucinous cystic neoplasms of the pancreas and to identify the findings which suggest malignancy. METHODS: The authors retrospectively evaluated the clinical outcomes of 41 patients with mucinous cystic neoplasms who were surgically resected at Asan Medical Center between 1995 and 2004. RESULTS: Women (n=33) were more frequently affected than men (n=8). Thirty three patients (80.6%) had adenoma, 1 (2.4%) borderline malignancy, 1 (2.4%) carcinoma in situ, and 6 (14.6%) invasive mucinous cystadenocarcinoma. The most frequent symptom was abdominal pain (39%). About half of the enrolled patients were asymptomatic. Unilocular type (79%) was more frequent than the multilocular type (21%) on gross morphology. The tumor size of invasive mucinous cystic neopolasms was larger than that of non-invasive mucinous cystic neoplalsms (p=0.01). Abdominal pain was more frequent in invasive mucinous cystic neoplasms (p=0.026). On gross morphology, mural nodules were detected in 4 of 6 patients with invasive mucinous cystic neoplasms. However, they were not detected in any patients with non-invasive mucinous cystic neoplasms. Recurrence developed in none of the 35 patients with non-invasive mucinous cystic neoplasms, however 2 of the 6 patients with invasive mucinous cystic neoplasms died within 5 years. CONCLUSIONS: Clinical predictors of invasive mucinous cystic neoplasms are suggested to be tumor size and abdominal pain. The prognosis of the non-invasive mucinous cystic neoplasms is excellent when curative resection is performed.
Adenocarcinoma, Mucinous/*diagnosis/pathology/surgery ; Adult ; Aged ; Carcinoma, Pancreatic Ductal/diagnosis/pathology/surgery ; Cystadenocarcinoma, Mucinous/diagnosis/pathology ; Diagnosis, Differential ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Invasiveness ; Pancreatic Neoplasms/*diagnosis/pathology/surgery ; Retrospective Studies ; Survival Analysis

Adenocarcinoma, Mucinous ; metabolism ; pathology ; surgery ; Aged ; Biomarkers, Tumor ; metabolism ; Carcinoembryonic Antigen ; metabolism ; Carcinoma, Pancreatic Ductal ; metabolism ; pathology ; surgery ; Carcinoma, Papillary ; metabolism ; pathology ; surgery ; Cystadenocarcinoma, Mucinous ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Duodenal Neoplasms ; metabolism ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Mucin-2 ; Mucins ; metabolism ; Neoplasm Invasiveness ; Pancreatic Neoplasms ; metabolism ; pathology ; surgery ; Pancreaticoduodenectomy

A pulmonary mucinous cystadenocarcinoma is an extremely rare tumor that is considered to be a cystic variant of mucin-producing lung adenocarcinoma. We present a case of pulmonary mucinous cystadenocarcinoma in a 54-year-old woman. Chest CT scans showed a 4.3-cm-sized, lobulated, well-defined, and homogeneous mass in the right middle lobe with peripheral stippled calcifications that demonstrated low-attenuation with no enhancement after contrast administration; 18F-fluorodeoxyglucose (FDG) PET/CT demonstrated mild heterogeneous FDG uptake. The mass was diagnosed as adenocarcinoma with mucin production by transbronchial lung biopsy. Right middle lobectomy was performed, and the pathologic examination disclosed a pulmonary mucinous cystadenocarcinoma.
Cystadenocarcinoma, Mucinous/pathology/*radiography/surgery ; Diagnosis, Differential ; Female ; Fluorodeoxyglucose F18/diagnostic use ; Humans ; Lung Neoplasms/pathology/*radiography/surgery ; Middle Aged ; Positron-Emission Tomography and Computed Tomography ; Radiopharmaceuticals/diagnostic use ; Tomography, X-Ray Computed/*methods