Sarcomatous transformation in chordoma (dedifferentiated chordoma) is a very rare condition and has been emphasized as a distinct entity because of its more aggressive clinical course. Here we describe a case of dedifferentiated chordoma (chordoma associated with malignant fibrous histiocytoma) arising from the sacrococcygeal region of a 55-year-old woman. The results of immunohistochemical stain in the chordoma area were strong positive for cytokeratin, epithelial membrane antigen and S-100 protein. The spindle and giant cells in the transitional areas of chordoma and malignant fibrous histiocytoma were positive for cytokeratin and epithelial membrane antigen in addition to vimentin and alpha-1-antichymotrypsin. The spindle and giant cells in the central area of malignant fibrous histiocytoma were negative for cytokeratin and epithelial membrane antigen, but positive for vimentin and alpha-1-antichymotrypsin. This supports the pathogenesis of sarcomatous transformation from chordoma.
Case Report ; Chordoma/diagnosis/*pathology ; Female ; Histiocytoma, Fibrous/diagnosis/*pathology ; Human ; Immunohistochemistry ; Middle Age ; Neoplasms, Multiple Primary/*pathology ; Pelvic Neoplasms/diagnosis/*pathology

Malignant mesothelioma (MM) is a relatively rare carcinoma of the mesothelial cells, and it is usually located in the pleural or peritoneal cavity. Here we report on a unique case of MM that developed in the chest, abdominal and pelvic walls in a 77-year-old female patient. CT and MRI revealed mesothelioma that manifested as a giant mass in the right flank and bilateral pelvic walls. The diagnosis was confirmed by the pathology and immunohistochemistry. Though rare, accurate investigation of the radiological features of a body wall MM may help make an exact diagnosis.
Abdominal Neoplasms/diagnosis/*pathology ; Abdominal Wall ; Aged ; Female ; Humans ; Magnetic Resonance Imaging ; Mesothelioma/diagnosis/*pathology ; Muscle Neoplasms/diagnosis/pathology ; Pelvic Neoplasms/diagnosis/*pathology ; Thoracic Neoplasms/diagnosis/*pathology ; Tomography, X-Ray Computed

A 38-year-old woman who had undergone pelvic lymphangioma resection two months previously presented with cough and dyspnea. Transthoracic echocardiography and CT demonstrated the presence of a mixed cystic/solid component tumor involving the inferior vena cava, heart and pulmonary artery. Complete resection of the cardiac tumor was performed and lymphangioma was confirmed based on histopathologic examination. To the best of our knowledge, this is the first report of lymphangiomatosis with cardiac and pelvic involvement in the published clinical literature.
Adult ; Female ; Heart Neoplasms/diagnosis/*pathology ; Humans ; Lymphangioma/diagnosis/*pathology/surgery ; Neoplasm Invasiveness ; Neoplasms, Second Primary/diagnosis/*pathology ; Pelvic Neoplasms/*pathology/surgery ; Pulmonary Artery/*pathology ; Vena Cava, Inferior/*pathology

Child ; Chondrosarcoma, Mesenchymal ; diagnosis ; drug therapy ; pathology ; Diagnosis, Differential ; Humans ; Male ; Pelvic Neoplasms ; diagnosis ; drug therapy ; pathology ; Tomography, X-Ray Computed ; Treatment Outcome

OBJECTIVETo investigate the CT findings of thorax and abdominopelvic with pulmonary lymphangiomyomatosis.

METHODThe clinical data, CT findings, and histopathological data of 14 patients with pulmonary lymphangiomyomatosis were analyzed.

RESULTSTypical high resolution CT (HRCT) findings of 14 patients with pulmonary lymphangiomyomatosis were diffusely scattered with small imperceptible-walled or thin-walled cysts (diameter < 1 cm) throughout both lungs. Other findings in thorax included: small noncalcified pulmonary nodules in 1 case, bilateral patchy shadows in 1 case, subpleural bulla in 3 cases, honeycombing sign in 1 case, mediastinal lymphadenopathy in 2 cases, pericardial effusion in 1 case, and pleural effusion in 3 cases. Nine patients had abnormal abdominopelvic findings: renal and hepatic angiomyolipoma in 1 case, retroperitoneal lymphangioleiomyoma in 6 cases, retroperitoneal lymphadenopathy in 1 case, and uterine leiomyoma in 1 case.

CONCLUSIONPulmonary lymphangiomyomatosis has typical pectoral CT findings. It is also often accompanied with abdominopelvic abnormity with typical CT findings.

Abdominal Neoplasms ; diagnosis ; Adult ; Angiomyolipoma ; diagnosis ; Female ; Humans ; Lung Neoplasms ; diagnosis ; pathology ; Lymphangioleiomyomatosis ; diagnosis ; pathology ; Middle Aged ; Pelvic Neoplasms ; diagnosis ; Thorax ; pathology ; Tomography, X-Ray Computed ; methods ; Young Adult

No abstract available.
Colectomy/methods ; Colonic Neoplasms/*diagnosis ; Colonoscopy ; Endometriosis/complications/*diagnosis/pathology/surgery/ultrasonography ; *Endosonography ; Female ; Humans ; Laparoscopy ; Middle Aged ; Muscle Neoplasms/*diagnosis ; Pelvic Pain/etiology ; Predictive Value of Tests ; Sigmoid Diseases/complications/*diagnosis/pathology/surgery/ultrasonography

Adolescent ; Adult ; CD57 Antigens ; metabolism ; Diagnosis, Differential ; Female ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Male ; Middle Aged ; Neurilemmoma ; metabolism ; pathology ; Pelvic Neoplasms ; metabolism ; pathology ; Peripheral Nervous System Neoplasms ; metabolism ; pathology ; Retroperitoneal Neoplasms ; metabolism ; pathology ; S100 Proteins ; metabolism

OBJECTIVETo evaluate the surgical treatment effect of primary malignant pelvic bone tumors.

METHODSRetrospective study was carried out in 79 patients with primary malignant pelvic bone tumors treated surgically and followed up regularly between October 1992 and July 2007. In this cohort, 23 tumors were low-grade malignant of I B, and 56 tumors were high-grade malignant of II B. According to the preoperative diagnosis and the effect of chemotherapy, different methods of tumor resection and reconstruction were applied respectively. Surgical specimens were evaluated postoperatively for the surgical margins in accordance with Enneking's criteria. Functional results were assessed by MSTS system during follow-up. The mean time of follow-up was 28.6 months (range, 0-183 months). The minimum follow-up time in survival patients was 4 months.

RESULTSSeventy limb-salvage surgeries including 28 reconstructions, and 9 hemipelvectomy were carried out. In limb-salvage group, the mean MSTS functional scores both were 15 points for patients with (range, 5-29 points) or without (range, 2-30 points) reconstructions. The overall local recurrence rate was 31.6% (25/79). Statistical difference (P = 0.023) of local recurrence rate was seen between low-grade (13.0%) and high-grade (39.3%) groups. While, there was no difference (P = 0.620) between limb-salvage group (30.0%) and hemipelvectomy group (44.4%). Significant difference (P = 0.014) in local recurrence rate was found between inadequate surgical margin (intralesional and marginal margin) group (38.1%) and adequate surgical margin (wide margin) group (6.3%). In 14 patients developed postoperative distant metastasis, 12 in local recurrence group which incidence was 48.0%, compared to 2 in no-recurrence group which incidence was 3.7%, and the difference was significant (P = 0.000). The overall survival rate was 70.9%.

CONCLUSIONSWide surgical margin is essential for successful resection of primary malignant pelvic bone tumors. How to choose and improve functional reconstruction is the focus of work in the future.

Adolescent ; Adult ; Aged ; Bone Neoplasms ; diagnosis ; pathology ; surgery ; Child ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Pelvic Bones ; Retrospective Studies ; Treatment Outcome

Objective: To investigate the clinicopathological characteristics, diagnosis, and treatment of primary seminal vesicle adenocarcinoma (SVAC). METHODS: We analyzed the clinical data and clinicopathological characteristics of 4 cases of primary SVAC treated in the Department of Urology of the Second Hospital of Tianjin Medical University and reviewed relevant literature. RESULTS: All the 4 patients were treated by open radical resection of the seminal vesicle and prostate and pathologically diagnosed with SVAC. Preoperative prostatic biopsy had shown 1 of the cases to be negative, while preoperative CT and transrectal ultrasound had revealed a huge pelvic cystic neoplasm in another patient. Immunohistochemistry manifested that the 4 cases were all negative for prostate-specific antigen (PSA), prostatic acid phosphatase (PAP), and cytokeratin 20 (CK20), but positive for cancer antigen 125 (CA125) and CK7. All the patients recovered smoothly after surgery and experienced no recurrence or metastasis during 154, 41, 20, and 12 months of follow-up. CONCLUSIONS Primary seminal vesicle carcinoma is extremely rare and presents in an advanced stage. Immunohistochemistry plays a valuable role in its differential diagnosis. Various combinations of radical surgery, radiotherapy, androgen-deprivation therapy, and chemotherapy are recommended for the treatment of the disease.
Adenocarcinoma ; chemistry ; pathology ; surgery ; Biopsy ; CA-125 Antigen ; analysis ; Diagnosis, Differential ; Genital Neoplasms, Male ; chemistry ; pathology ; surgery ; Humans ; Immunohistochemistry ; Male ; Neoplasm Recurrence, Local ; Pelvic Neoplasms ; diagnostic imaging ; Prostate-Specific Antigen ; analysis ; Prostatectomy ; Seminal Vesicles ; pathology ; surgery

OBJECTIVETo investigate the CT and MRI features of peripheral primitive neuroectodermal tumors (pPNETs) and evaluate its diagnostic value.

METHODSThe clinicopathological data of 9 surgically treated patients with peripheral primitive neuroectodermal tumors confirmed by pathology were collected, spiral CT (4/9) and MRI (6/9) plain scanning and dynamic enhancement scanning were performed preoperatively. Both CT and MRI scannings were performed in 1 case. Those CT and MR images were retrospectively reviewed and analyzed together with clinicopathological findings.

RESULTSThe 9 lesions were located in skeletal muscles (n = 6), pelvic cavity (n = 2) and thoracic cavity (n = 1). The tumor size was 7.4-18.3 cm in diameter with a mean diameter of 11.6 cm. The shape of those lesions was round or ellipse (4 lesions) and irregular (5 lesions). The tumor usually presented as ill-defined masses, with homogeneous (n = 2) or inhomogeneous density (n = 7). Seven cases, including the 3 lesions located in the chest and pelvis, showed obvious necrosis and multilocular cyst formation. The tumors showed iso-density as that of the adjacent muscles on CT plain scans and moderate heterogeneous enhancement after intravenous injection of contrast agents. The features of the tumors on the MRI including slightly low signal intensity on SE T1-weighted imaging, iso-signal intensity or slightly high signal intensity on FSE T2-weighted imaging and heterogeneous dynamic delayed contrast-enhancement with obvious necrosis in most of them. Six cases had a lesion in the skeletal muscles, presented as a giant ill-defined masse surrounding bone and extended along neural route with bone destruction to varying degrees.

CONCLUSIONPrimitive neuroectodermal tumor is a kind of malignant tumor with proliferation of small, undifferentiated neuroectodermal cells, usually occurring in children or adolescent and frequently located in the extremities, chest cavity, pelvic cavity and chest wall. It typically presents as a large, ill-defined masse extending along neural route with heterogeneous and obvious enhancement after intravenous injection of contrast agents. The tumors located in the chest and pelvic cavities and some in the extremities show obvious necrosis and multilocular cyst formation, while some of the tumors in the extremities appear as homogeneous solid masses.

Adolescent ; Adult ; Child ; Diagnostic Errors ; Female ; Histiocytoma, Malignant Fibrous ; diagnosis ; Humans ; Male ; Muscle Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Muscle, Skeletal ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; diagnostic imaging ; pathology ; Pelvic Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Radiography ; Retrospective Studies ; Thoracic Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Young Adult