1b - Case I-B Hsin-Hung Chen, MD Kaohsiung Medical...

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Unformatted text preview: Case I-B Hsin-Hung Chen, MD Kaohsiung Medical University Chung-Ho Memorial Hospital Department of Pathology Clinical History ! ! 62 y/o, female, G4P2A2 Clinical symptoms ! ! ! Abdominal fullness, lower abdominal pain, dysuria and frequency for 2 weeks Pelvic examination: huge palpable mass Laboratory data: ! Elevation: LDH, CA125 Image study Gross Feature 11.5 x 10.0 x 7.5 cm ! Tumor excision, segmental resection of small intestine and right salpigooophorectomy Differential Diagnosis ! ! ! ! ! Malignant mesothelioma Synovial sarcoma Dedifferentiated liposarcoma Carcinosarcoma, secondary Carcinosarcoma, primary CK EMA Vimentin Calretinin Thrombomodulin CD99 Bcl-2 Summary of IHC study Positive Negative CK S-100 Calretinin OCT-4 EMA WT-1 Thrombomodulin AFP Vimentin   CD99 β-hCG NSE   Bcl-2 Inhibin Synaptophysin   Chromogranin-A CD117 CD56 (N-CAM)   SMA p53     Desmin   Diagnosis Malignant mixed müllerian tumor (carcinosarcoma) with neuroendocrine differentiation possibly primary in the mesentery Malignant mixed müllerian tumor (MMMT) ! Definition: ! ! A neoplasm composed of an admixture of malignant epithelial and mesenchymal components Synonyms Carcinosarcoma ! Malignant mixed mesodermal tumor ! Metaplastic carcinoma ! Clinical Manifestations ! Age: elder postmenopausal women Median age: 65 years ! Younger than 50 years: < 5% ! ! Aetiology Prior pelvic irradiation ! Long-term tamoxifen therapy ! Int J Gynecol Cancer 2000;10:280–284. J Natl Cancer Inst. 2004 Jan 7;96(1):70-4 ! Location ! Uterus (35%)> ovary > fallopian tube > vagina Primary peritoneal malignant mixed müllerian tumor ! Rare ! ! ! Age: postmenopausal women almost ! ! 40 ~ 84 years (mean age: 68 years) Location ! ! First case reported by Ober and Black in 1955 32 cases are reported Pelvic peritoneum (59.3%) Clinical symptoms ! Abdominal distension, abdominal pain, abdominal or Cancer 1994; 74:854-63. pelvic mass Acta Oncologica, 2005; 44: 756-760 Pathologic Characteristics ! Gross feature ! ! Bulky, grayish with focal necrosis, hemorrhage or cystic changes Microscopic feature Biphasic pattern " Malignant epithelial components " Malignant stromal components ! ! Homologous ; Heterologous Immunohistochemical Study Carcinomatous components Sarcomatous components Cytokeratin Positive Variable EMA Positive Variable Vimentin Variable Positive ! ! Rhabdomyosarcomatous component: Desmin (+) Cartilaginous components: S-100 protein (+) Histogenesis (I) ! Collision theory ! ! Combination theory ! ! Carcinoma and sarcoma are two distinct neoplasms Both components are derived from a single stem cell that undergoes divergent differentiation early in the evolution of tumor Conversion theory ! Sarcomatous element derives from the carcinoma during the evolution of the tumor Histogenesis (II) - Conversion theory - ! Clinical data Pathological data Immunohistochemical data Ultrastructural data Molecular data Tissue culture data ! Conclusion ! ! ! ! ! ! MMMTs are metaplastic neoplasms Int J Gynecol Cancer 2002;12:687–690. J. Clin. Pathol. 2002;55;321-325 Behavior and Treatment - Peritoneal MMMTs ! Clinical behavior ! ! Aggressive Treatment No standard protocol due to few cases ! Surgical excision: most effective ! Combined chemotherapy ! Adjuvant radiotherapy " Prognosis - Peritoneal MMMTs ! Peritoneal MMMT ! ! Poor, most died within 1 year (7 days ~ 73 months) Uterine MMMT ! 5-year survival Early stage: 40 ~ 60% ! Advanced stage: 15 ~ 30% ! ! Predictor factors ! No relationship with stage of disease (number Cancer 1994; 74:854-63. of patients is too small) Acta Oncologica, 2005; 44: 756-760 Summary ! MMMTs are metaplastic neoplasms ! MMMTs are biphasic neoplasms composed of carcinomatous and sarcomatous components ! Peritoneal MMMTs are more aggressive than uterine MMMTs Follow up ! Treatment ! ! Surgery & Chemotherapy Two months later Abdominal pain ! Abdominal CT: recurrent tumor at right lower abdomen ! Thank you for your attention. ...
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This note was uploaded on 05/24/2010 for the course MED 122 taught by Professor Tera during the Winter '08 term at Anadolu University.

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