Synovial sarcoma is a rare tumour found in soft tissue; it is a mesenchymal spindle cell tumour that is not related to the synovial membrane. This tumour has a low incidence, and the most frequent place of occurrence is the lower extremities in young adults. Synovial sarcoma of the head and neck accounts for 3%-5% of sarcomas in this anatomical region. The tumor in the nasal cavity is less than 1%. The treatment of choice for synovial sarcoma of the head and neck is complete surgical excision of the tumour mass followed by adjuvant radiotherapy.
Humans ; Nasal Cavity ; pathology ; Nose Neoplasms ; diagnosis ; radiotherapy ; surgery ; Paranasal Sinuses ; pathology ; Radiotherapy, Adjuvant ; Sarcoma, Synovial ; diagnosis ; radiotherapy ; surgery

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

Adenoma ; metabolism ; pathology ; Adult ; Alkaline Phosphatase ; metabolism ; Antibodies, Monoclonal, Murine-Derived ; metabolism ; Diagnosis, Differential ; Ear Canal ; Ear Neoplasms ; metabolism ; pathology ; radiotherapy ; surgery ; GPI-Linked Proteins ; metabolism ; Humans ; Isoenzymes ; metabolism ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Mast-Cell Sarcoma ; metabolism ; pathology ; Proto-Oncogene Proteins c-kit ; metabolism ; Seminoma ; metabolism ; pathology ; radiotherapy ; surgery ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo review the outcomes and complications of 21 consecutive patients with soft tissue sarcomas of the adductor compartment treated in our hospital from July 2006 to March 2012.

METHODSMedical records of 21 patients who underwent resection of soft tissue sarcomas of the adductor compartment were reviewed. Eight of the patients had primary operation, other eight had secondary radical excision, and five were admitted for tumor recurrence after operation in local hospitals. Six cases used gracilis or sartorius muscle to fill the cavity after removal of adductor muscle group. Fifteen patients underwent adjuvant treatment including radiotherapy and/or chemotherapy.

RESULTSSeven patients (33.3%) developed wound complications. Five required further surgery and two received dressing changes. All the patients were followed up for 3 to 60 months. During the follow-up period, no tumor recurrence was found in all the patients, only one case had multiple bone and pulmonary metastases, and two cases died (one for pulmonary metastasis at 11 months after surgery, and the other died of heart disease at 36 months post operation).

CONCLUSIONSGood local control rate can be achieved in patients with soft tissue sarcomas of the adductor compartment by using adductor muscle group resection, but it carries a relatively high rate of wound complications requiring proper management.

Adolescent ; Adult ; Aged ; Bone Neoplasms ; secondary ; Chemotherapy, Adjuvant ; Female ; Follow-Up Studies ; Humans ; Lung Neoplasms ; secondary ; Male ; Middle Aged ; Muscles ; surgery ; Postoperative Complications ; Radiotherapy, Adjuvant ; Sarcoma ; drug therapy ; radiotherapy ; secondary ; surgery ; Soft Tissue Neoplasms ; drug therapy ; radiotherapy ; surgery ; Survival Rate ; Thigh ; Young Adult

OBJECTIVETo analyze the clinical features, treatment and prognosis of Stage IV alveolar soft part sarcoma.

METHODSTo analyze the clinical and pathological features, therapeutic methods and follow-up results in 21 patients with stage IV alveolar soft part sarcoma. There were 11 males and 10 females, in the age of 26-57 years (average 37.0 years old). All the 21 patients had metastasis: nine cases had multiple pulmonary metastasis, three cases had multiple pulmonary and brain metastasis, two cases had multiple brain metastasis, two cases had multiple pulmonary and bone metastasis, two cases had single pulmonary metastasis, one case had single bone metastasis, one case had single brain metastasis and one case had single soft tissue metastasis. Eight patients were treated by surgical operation, including five cases of complete resection for the primary and (or) metastatic tumor and 3 cases of palliative operation for the primary tumor. All patients received chemotherapy, including seven cases of CAVD regimen and 14 cases of MAID regimen treatment. One patient with single bone metastasis and five patients with multiple brain metastasis received post-operative whole brain radiation therapy.

RESULTSAll the eight patients with surgical operation had healing by first intention, and pathological examination showed that seven patients achieved R0 surgical margin and one case with R2 status. One patient with single brain metastasis had recurrence after operation. The toxic and adverse reactions of all patients treated with chemotherapy were tolerable. Among them, 17 cases had stable disease and 4 cases had disease progression after chemotherapy. The disease control rate (DCR) was 81.0%. The DCR of patients with CAVD regimen chemotherapy was 85.7% and that of patients treated with MAID regimen was 78.6% (P = 0.862). All patients were followed up for 8 - 86 months (average 32.4 months). The median survival time of all patients was 32.6 months. The 2-year survival rate was 55.1% and the 5-year survival rate was 21.8%. The median survival time in the patients with complete resection was 60.0 months, and that in patients with palliative operation was 27.0, showing a significant difference between them (P = 0.048). The median progression-free survival in patients with complete excision was 57.2 months and that in patients with palliative operation or without operation was 19.6 months, with a significant difference (P = 0.029). The median survival time in patients who received CAVD regimen chemotherapy was 30.0 months, and that in patients with MAID regimen was 51.0 months, with a non-significant difference (P = 0.511). The median progression-free time in patients with CAVD regimen chemotherapy was 13.0 months, and that in patients with MAID regimen was 38.0 months, also with a non-significant difference (P = 0.066).

CONCLUSIONSAlveolar soft part sarcomas are rarely seen and highly malignant tumors, and the prognosis of stage IV ASPS is poor. Complete resection of all tumors is the key of successful treatment of Stage IV ASPS, and the site and number of tumor metastasis are important factors affecting prognosis. The curative effects of radiotherapy and chemotherapy for ASPS need to be further investigated.

Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bone Neoplasms ; radiotherapy ; secondary ; surgery ; Brain Neoplasms ; radiotherapy ; secondary ; surgery ; Dacarbazine ; therapeutic use ; Disease-Free Survival ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Ifosfamide ; therapeutic use ; Lower Extremity ; Lung Neoplasms ; secondary ; surgery ; Male ; Mesna ; therapeutic use ; Middle Aged ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Particle Accelerators ; Remission Induction ; Salvage Therapy ; Sarcoma, Alveolar Soft Part ; drug therapy ; radiotherapy ; secondary ; surgery ; Soft Tissue Neoplasms ; drug therapy ; radiotherapy ; surgery ; Survival Rate

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Dioxoles ; therapeutic use ; Humans ; Sarcoma ; drug therapy ; radiotherapy ; surgery ; Soft Tissue Neoplasms ; drug therapy ; radiotherapy ; surgery ; Tetrahydroisoquinolines ; therapeutic use

OBJECTIVETo analyze the complications and short-term outcomes of patients receiving intraoperative electron radiotherapy (IOERT) for locally advanced and recurrent periarticular soft tissue sarcomas of the extremities.

METHODSTwenty-one adult patients with locally advanced and/or recurrent periarticular soft tissue sarcomas of the extremities treated with IOERT were included in this study. Among them 14 patients had recurrent diseases after prior operation and 7 patients with locally advanced disease with primary treatment. The total dose of radiation ranged from 11 Gy to 21 Gy of 6 - 12 MeV beta ray given by intraoperative radiotherapy after complete tumor resection with negative margins. Five patients were given external beam radiotherapy (EBRT) with a total dose of 40 - 50 Gy, and 10 patients received chemotherapy.

RESULTSThe median follow-up time was 15 months. Five patients (23.8%) had tumor relapse, including one patients with local recurrence, one patient with regional recurrence, two patients with distant metastasis and one patient with local recurrence and distant metastasis. The actuarial local control rate at 1 year was 95.2% (20/21), and at 2 years was 90.5% (19/21). Acute skin toxicity (RTOG) within 3 months after surgery included grade I in 6 patients and grade II in 3 patients. Two year late skin toxicity (RTOG/EORTC) included grade I in 4 patients and grade II in 2 patients. Fibrosis included grade I in 3 patients and grade II in 3 patients. Six patients had joint dysfunction (3 patients with grade I and 3 with grade II) and 6 patients had healing problems. One patient got ipsilateral schiatic neuritis 9 months after IOERT. No adverse events occurred during surgery.

CONCLUSIONSIOERT brings tolerable complications of acute and late toxicities and favorable local control rate. IOERT should be followed by postoperative radiotherapy or increase the intraoperative radiation dose for locally advanced and recurrent sarcomas to get a better local control. Otherwise, distant metastasis is one of the main reasons of treatment failure, so chemotherapy should be added to the treatment regimen.

Adolescent ; Adult ; Aged ; Atrophy ; etiology ; Electrons ; adverse effects ; therapeutic use ; Extremities ; Female ; Fibrosis ; etiology ; Follow-Up Studies ; Humans ; Intraoperative Period ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; radiotherapy ; surgery ; Neoplasm, Residual ; radiotherapy ; Radiotherapy Dosage ; Radiotherapy, Adjuvant ; adverse effects ; Sarcoma ; drug therapy ; radiotherapy ; surgery ; Skin ; pathology ; Young Adult

BACKGROUNDThe primary ovarian sarcoma is a very rare malignancy. The objective of this study was to further investigate the clinicopathologic features and outcome in patients with primary sarcoma of the ovary.

METHODSBetween 1988 and 2007, 24 patients with primary ovarian sarcoma who underwent treatment at Peking Union Medical Hospital were reviewed retrospectively. Response to treatment, progression and overall survival were analyzed.

RESULTSPatients with ovarian sarcoma had a mean age of (54.3 ± 10.3) years, and 16 of them were postmenopausal. The most common symptom was abdominal pain, present in 14 patients. Of the 24 patients, 16 patients were pathologically diagnosed as carcinosarcoma (known as malignant mixed mesodermal tumor (MMMT)), 2 as ovarian leiomyosarcoma (LS) and 6 patients as ovarian endometrial stromal sarcoma (ESS). The patients in optimal debulking group had a median survival period of 28 months and 1-year survival rate of 71%. The patients in suboptimal debulking group had a significantly lower median survival of 6 months (P = 0.02) and 1-year survival rate of 29%. Among the patients, 23 patients received chemotherapy and most of regimens were based on platinum, 3 patients received chemoradiation. The mean number of courses of combined chemotherapy was 6.6 ± 5.0, and the response was unsatisfactory. The median survival for the entire group was 18.7 months. The one-year survival rate was 58%, and two-year survival rate only 29%.

CONCLUSIONSOvarian primary sarcoma has a poor overall prognosis. Optimal debulking surgery appears to be of prognostic significance. There is a clear need for further study to explore the role and the regimen of platinum-based chemotherapy in primary ovarian sarcoma.

Adult ; Aged ; Female ; Humans ; Kaplan-Meier Estimate ; Middle Aged ; Ovarian Neoplasms ; diagnosis ; drug therapy ; radiotherapy ; surgery ; Retrospective Studies ; Sarcoma ; diagnosis ; drug therapy ; radiotherapy ; surgery ; Survival Rate

Adult ; Desmin ; metabolism ; Diagnosis, Differential ; Diagnostic Errors ; Humans ; Male ; Melanoma-Specific Antigens ; metabolism ; Meningeal Neoplasms ; metabolism ; pathology ; radiotherapy ; surgery ; Meningioma ; metabolism ; pathology ; Paraganglioma ; metabolism ; pathology ; Sarcoma, Alveolar Soft Part ; metabolism ; pathology ; radiotherapy ; surgery ; Sarcoma, Clear Cell ; metabolism ; pathology ; Tomography, X-Ray Computed ; Vimentin ; metabolism

OBJECTIVETo determine the independent prognostic factors of primary synovial sarcoma.

METHODSThe clinical data of 52 patients followed up from 66 patients with synovial sarcoma treated between September 1997 and September 2008 was analyzed retrospectively. There were 28 male and 24 female patients aged from 11 to 71 years old. Three and five-year overall survival (OS), recurrence rate and 9 prognostic factors were analyzed in this study. Univariate and multivariate analysis were performed to determine the prognostic factors of OS.

RESULTSFifty-two patients were followed up with the follow-up time ranged from 6 to 88 months (median 32 months). The 3-, 5-year overall survival rate and local recurrence rate were 52.8%, 30.3% and 32.7% respectively. Univariate showed tumor size < 5 cm, tumor located at extremities, adequate surgical margin and radical resection combined with radiotherapy had better survival rate (P < 0.05). Multivariate analysis demonstrated that tumor size, primary site and adequate surgical margin were independent prognostic factors for OS. Patients received radical resection combined with radiotherapy have longer median relapse time (25 months) compared with marginal resection combined with radiotherapy (18 months) and single radical resection (12 months). Thirty-five (67%) patients were treated with chemotherapy and seventeen (33%) patients received no chemotherapy for the primary tumor. Treatment with chemotherapy was not associated with an improved OS (P = 0.52).

CONCLUSIONSThe independent prognostic factors of synovial sarcoma are tumor size, primary site and adequate surgical margin. Doxorubicin and ifosfamide based chemotherapy was not associated with an improved OS in patients with synovial sarcoma. Radical resection combined with radiotherapy can best control local condition.

Adolescent ; Adult ; Aged ; Antineoplastic Agents ; therapeutic use ; Child ; Female ; Follow-Up Studies ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Multivariate Analysis ; Neoplasm Recurrence, Local ; Prognosis ; Regression Analysis ; Retrospective Studies ; Sarcoma, Synovial ; diagnosis ; drug therapy ; radiotherapy ; surgery ; Young Adult