A complete surgical resection is the only proven therapeutic modality that prolongs the survival in patients with leiomyosarcoma of the inferior vena cava (IVC). Reconstruction of the IVC is not always necessary but is often required to facilitate venous drainage of the kidney for the tumors at the pararenal area of the IVC. Controversy exists in postoperative adjuvant therapy. Recently, we experienced four cases of pararenal leiomyosarcoma of the IVC, of which treatment consisted of a complete resection of the tumor, ringed polytetrafluoroethylene (PTFE) graft interposition, and bilateral renal vein reconstructions in all patients. Postoperative radiation therapy was instituted in 3 of 4 patients. One patient who did not receive the postoperative radiation therapy was treated with adjuvant chemotherapy. The kidneys were preserved in all patients and no deep vein thrombosis (DVT) or venous insufficiency of the lower extremity veins developed. Distant metastasis to the lung was noted in one patient at 18 months after surgery, who was not received the postoperative radiation therapy but chemotherapy. In conclusion, a complete resection of the tumor, IVC reconstruction, and bilateral renal vein reconstruction followed by adjuvant radiation therapy is recommended for the treatment of pararenal leiomyosarcoma of the IVC.
Adult ; Combined Modality Therapy ; Female ; Human ; Leiomyosarcoma/radiography/*radiotherapy/*surgery ; Middle Aged ; Neoplasm Recurrence, Local/radiography/radiotherapy/surgery ; Retroperitoneal Neoplasms/radiography/radiotherapy/surgery ; Retrospective Studies ; Treatment Outcome ; Vascular Neoplasms/radiography/*radiotherapy/*surgery ; *Vena Cava, Inferior

MR imaging appearances of different types of reconstructive muscle flaps following reconstructive surgery of the lower extremity with associated post-surgical changes due to altered anatomy, radiation, and potential complications, can be challenging. A multidisciplinary therapeutic approach to tumors allows for limb salvage therapy in a majority of the patients. Decision-making for specific types of soft tissue reconstruction is based on the body region affected, as well as the size and complexity of the defect. Hematomas and infections are early complications that can jeopardize flap viability. The local recurrence of a tumor within six months after a complete resection with confirmed tumor-free margins and adjuvant radiation therapy is rare. Identification of a new lesion similar to the initial tumor favors a finding of tumor recurrence.
Adult ; Female ; Hematoma/etiology ; Humans ; Limb Salvage ; Lower Extremity/anatomy & histology/radiography/*surgery ; *Magnetic Resonance Imaging ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Reconstructive Surgical Procedures ; Sarcoma/radiotherapy/*surgery ; Soft Tissue Infections/radiography/*surgery ; Soft Tissue Injuries/radiography/*surgery ; Soft Tissue Neoplasms/radiotherapy/*surgery ; Surgical Flaps/adverse effects

OBJECTIVETo study the clinical, radiologic and pathologic features, as well as differential diagnosis of teratocarcinosarcoma in nasal cavity and paranasal sinuses.

METHODSLight microscopic examination and immunohistochemical study was performed in 5 cases of sinonasal teratocarcinosarcoma. The clinical, radiologic and pathologic features were analyzed and the literature was reviewed.

RESULTSAll 5 patients were males and their age ranged from 34 to 43 years (mean age = 39 years). The clinical presentation was nasal obstruction, epistaxis and headache. Physical examination often revealed a polypoid mass with contact bleeding. Computed tomography showed a homogeneous nasal mass with obturation of sinuses. Cystic changes, calcification or ossification was not observed. Histologically, the tumor showed a heterogeneous admixture of components from the 3 germ cell layers, exhibiting various degrees of maturation. Squamous epithelium, smooth muscle cells, chondro-osseous tissue, intestinal or respiratory type epithelium, "fetal-type" clear cells and immature neuroepithelium were commonly seen. Immunohistochemical study demonstrated that the epithelial component expressed cytokeratin and epithelial membrane antigen, while the mesenchymal component variably expressed vimentin, smooth muscle actin and S-100 protein. On the other hand, the neuroepithelial component expressed neuron-specific enolase, synaptophysin and chromogranin, and the primitive component expressed CD99. The initial biopsy diagnosis included capillary hemangioma, olfactory neuroblastoma, craniopharyngioma and malignant mixed tumor. Follow-up information was available in all patients. Two of which had local recurrence and 1 had cervical lymph node metastasis.

CONCLUSIONSSinonasal teratocarcinosarcoma is a rare and highly malignant tumor occurring in sinonasal tract. It manifests mainly in adult males and is characterized by a complex admixture of teratomatous and carcinosarcomatous components. "Fetal-type" clear cells, squamous epithelium and immature neuroepithelium represent important histologic characteristics useful in diagnosis.

Adult ; Carcinosarcoma ; diagnostic imaging ; metabolism ; pathology ; radiotherapy ; surgery ; Follow-Up Studies ; Humans ; Keratins ; metabolism ; Lymphatic Metastasis ; Male ; Mucin-1 ; metabolism ; Nasal Cavity ; Neck Dissection ; Neoplasm Recurrence, Local ; Nose Neoplasms ; diagnostic imaging ; metabolism ; pathology ; radiotherapy ; surgery ; Paranasal Sinus Neoplasms ; diagnostic imaging ; metabolism ; pathology ; radiotherapy ; surgery ; Radiography ; Teratocarcinoma ; diagnostic imaging ; metabolism ; pathology ; radiotherapy ; surgery