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

OBJECTIVEWith the extremity soft tissue sarcoma close to neurovascular bundle, combined en bloc resection and brachytherapy or simple en bloc resection were performed to evaluate the treatment outcome of the combined en bloc resection and brachytherapy.

METHODSRetrospectively investigation was performed for the extremity soft tissue sarcoma close to neurovascular bundle between 2000 and 2009. Inclusion criteria were primary extremity soft tissue sarcoma, MRI showed that the reaction zone involved the main neurovascular bundle, and the reaction zone closed less than 1 cm to the main neurovascular bundle. 86 cases were included in the study. There were 41 men and 45 women. The average age was 38.5 years old (Range from 15 to 73). There were malignant fibrous histiocytoma, synovial sarcoma, fibrosarcoma, liposarcoma, clear cell sarcoma, epithelioid sarcoma, leiomyosarcoma, rhabdomyosarcoma and vascular sarcoma etc. The stage were IA (8), IIA (12), IIB (10), IIC (7), III (43) and IV (6).

RESULTSDuring an average follow-up of 53 months (range 24 - 102 months), the distant metastasis rate 32.56% (28/86) and the lymph node metastasis rate was 6.98% (6/86). The local recurrence rates was 13.95% (12/86). In the group of combined en bloc resection and brachytherapy with 38 cases, the local recurrence rates was 5.26% (2/38). Four cases had wound infection and six cases had wound delay healing. The MSTS functional score was 21.11 ± 1.79. In the group of simple en bloc resection with 48 cases, the local recurrence rates was 20.83% (10/48). One case had wound infection and four cases had wound delay healing. The MSTS functional score was 84.23% (26.11 ± 1.79). The local recurrence rates was significant different between.

CONCLUSIONWith the extremity soft tissue sarcoma close to neurovascular bundle, combined en bloc resection and brachytherapy could decrease the local recurrence rate.

Adolescent ; Adult ; Aged ; Brachytherapy ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Retrospective Studies ; Sarcoma ; radiotherapy ; surgery ; Soft Tissue Neoplasms ; radiotherapy ; surgery ; 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

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

Synovial sarcoma is a rare soft tissue sarcoma of the head and neck region involving the parapharyngeal space. The diagnosis of synovial sarcoma can be very challenging to the pathologists. We present a rare case of parapharyngeal synovial sarcoma in a young female patient who had a two-month history of left cervical intumescent mass at level II. The fine needle aspiration cytology of the mass was proved inconclusive. Transcervical excision of the mass was performed and the first case of parapharyngeal sarcoma was identified in our center by fluorescence in situ hybridization (FISH) technique. Repeat imaging revealed residual tumor. The patient successfully underwent a second excision of the residual tumor and received adjuvant radiotherapy.
Adult ; Biopsy, Fine-Needle ; Combined Modality Therapy ; Female ; Humans ; In Situ Hybridization, Fluorescence ; Neoplasm, Residual ; Pharyngeal Neoplasms ; diagnosis ; pathology ; radiotherapy ; surgery ; Radiotherapy, Adjuvant ; Sarcoma, Synovial ; diagnosis ; pathology ; radiotherapy ; surgery ; Tomography, X-Ray Computed

OBJECTIVEVulvar epithelioid sarcoma is a rare, undifferentiated soft-tissue sarcoma, with a high rate of local relapse, regional nodal spread and distant metastases. The aim of this study was to investigate the clinical features, diagnosis, treatment and prognosis of this malignancy.

METHODSWe studied the clinicopathologic features of 20 cases of vulvar epithelioid sarcoma, of which 4 cases were admitted to our hospital from 1999 to 2009. All of the patients received radical local excision with inguinofemoral lymphadenectomy. Seven patients were treated without adjuvant therapy. Seven patients received postoperative radiotherapy only and three underwent chemotherapy. Chemotherapy plus radiotherapy were given postoperatively in three.

RESULTSThe patients ranged in age from 23 to 80 years (median: 36 y). The tumors ranged from 1 to 10 cm in their greatest diameter (median: 5.1 cm). All cases showed immunoreactivity for both vimentin and cytokeratin. Follow-up information on all 20 patients was available, and covered periods ranging from 3 to 104 months.11 patients were alive with no evidence of disease. 2 patients developed lymph node metastases but alive. 7 patients had died of the disease. Survival of the early stage (I-II) patients was significantly longer than those in the advanced stage (III-IV) (median, 21 vs. 6 months, P < 0.01). There was no significant difference between survival of patients with or without inguinofemoral lymphadenectomy (median, 11.5 vs. 6 months, P = 0.086).

CONCLUSIONSBecause of the relatively frequent misdiagnosis, a differential diagnosis combined with immunohistochemistry is needed to determine an early and accurate diagnosis. The tumor markers exhibiting immunoreactivity includ vimentin, epithelial membrane antigen (EMA) and cytokeratin (CK). Radical local excision with adequate margin (at least 2 cm) and bilateral inguinofemoral lymphadenectomy is effective for the treatment of vulvar epithelioid sarcoma. The role of adjuvant therapy, chemotherapy and radiation remains unclear but merits consideration.

Adult ; Aged ; Aged, 80 and over ; Chemotherapy, Adjuvant ; Female ; Follow-Up Studies ; Humans ; Keratins ; metabolism ; Lymph Node Excision ; Lymphatic Metastasis ; Middle Aged ; Mucin-1 ; metabolism ; Neoplasm Staging ; Radiotherapy, Adjuvant ; Retrospective Studies ; Sarcoma ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Soft Tissue Neoplasms ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Survival Rate ; Vimentin ; metabolism ; Vulva ; surgery ; Vulvar Neoplasms ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Young Adult

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

BACKGROUND: We present here the oncological and functional outcomes of limb salvage with or without reconstruction for primary sarcomas in the pelvic bone. METHODS: Forty-four patients who underwent pelvic resection for primary sarcomas involving the pelvis were reviewed. The average follow-up period was 39 months (range, 0 to 146 months). Chondrosarcoma (n = 17) and osteosarcoma (n = 10) were the most common diagnoses. Various clinicopathologic factors were analyzed in relation to the oncological outcomes of overall survival and local recurrence. The Musculoskeletal Tumor Society functional scores and complications were compared according to the tumor location, pelvic continuity and the type of resection. RESULTS: The overall 5-year survival rate was 40%. Metastasis at the time of diagnosis, the surgical margin and the histologic grade were the independent prognostic factors for survival. The surgical margin was an independent prognostic factor for local recurrence. An ischiopubic location of the tumor, restoration of pelvic continuity and hip joint reconstruction with total hip replacement arthroplasty were related with higher functional scores. Complications occurred in 50% of the patients and the complications varied according to the tumor location with infection being the most common complication. CONCLUSIONS: Achieving an adequate surgical margin is necessary for improving the oncological outcome of pelvic sarcomas. Restoration of the pelvic continuity and the hip joint improves the functional outcome. However, complications are common and so careful selection of the reconstruction method is needed.
Adolescent ; Adult ; Aged ; Bone Neoplasms/mortality/pathology/*surgery ; Chemotherapy, Adjuvant ; Child ; Female ; Humans ; Limb Salvage ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; *Pelvic Bones/surgery ; Prognosis ; Radiotherapy, Adjuvant ; Sarcoma/mortality/pathology/secondary/*surgery ; Survival Rate ; Young Adult

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 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