Humans ; Extremities ; Sarcoma/radiotherapy* ; Soft Tissue Neoplasms/radiotherapy* ; Neoplasm Recurrence, Local

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

PURPOSE: Malignant rhabdoid tumor (MRT) is a rare and highly aggressive tumor that affects young children. Due to its extreme rarity, most of the available data are based on retrospective case series. To add to the current knowledge of this disease, we reviewed the patients treated for extra-cranial MRT in our institute. MATERIALS AND METHODS: A retrospective medical record review was conducted on children treated for pathologically confirmed extra-cranial MRT at Seoul National University Children's Hospital between January 2003 and May 2013. RESULTS: Eleven patients (7 boys, 4 girls) were diagnosed with extra-cranial MRT at a median age of 9 months old. INI1 staining was important in the pathological confirmation. Six patients (55%) had renal MRT and five (45%) had soft tissue MRT. Five patients (45%) had metastases at diagnosis. All patients underwent chemotherapy, eight patients (73%) underwent surgery, six patients (55%) received therapeutic radiotherapy, and four patients (36%) underwent high dose chemotherapy with autologous stem cell rescue (HDCT/ASCR) with melphalan, etoposide, and carboplatin. Five patients (45%) died of disease following progression (n=3) or relapse (n=2), however, there was no treatment related mortality. The overall survival of the cohort was 53.0% and the event-free survival was 54.5% with a median follow-up duration of 17.8 months (range, 2.3 to 112.3 months). CONCLUSION: Extra-cranial MRT is still a highly aggressive tumor in young children. However, the improved survival of our cohort is promising and HDCT/ASCR with melphalan, etoposide, and carboplatin may be a promising treatment option.
Carboplatin ; Child* ; Cohort Studies ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Etoposide ; Follow-Up Studies ; Humans ; Kidney Neoplasms ; Medical Records ; Melphalan ; Mortality ; Neoplasm Metastasis ; Radiotherapy ; Recurrence ; Retrospective Studies ; Rhabdoid Tumor* ; Seoul ; Soft Tissue Neoplasms ; Stem Cells

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

OBJECTIVETo evaluate the value of intraoperative radiation therapy with electrons (ELIOT) in treatment of malignant bone or soft tissue tumors around the joints.

METHODSFrom October 2008 to April 2012, nineteen patients with malignant bone or soft tissue tumors around the joints were treated with ELIOT. The tumors were located around the knee joint in 8 patients, around the hip joint in 6 patients, around the elbow joint in 4 patients and around the shoulder joint in one patient. All of the patients underwent limb salvage surgeries. R0 resections were performed in 18 patients, while R1 resection was performed in one patient. The doses of intraoperative radiation ranged from 10 Gy to 22 Gy. The median dose was 19 Gy. More than one ELIOT fields were used in 10 patients because of the large tumor size.

RESULTSSeven patients suffered wound complications. No grade ≥ 3 acute toxicities were observed. One patient developed radiation ulcer and arterial fistula 15 months after surgery and ELIOT, and resulted in amputation finally (grade 4 late toxicity). The mean Musculoskeletal Tumor Society (MSTS) 93 score was 26.26 ± 4.04 (87.5% ± 13.5%), with excellent to good extremity functions in 18 patients (94.7%). Four patients had local recurrences. The estimated locoregional control rates at 1, 2, and 3 years were 81.9%, 73.7%, and 73.7%, respectively. Seven patients died of the diseases. The estimated overall survivals of the entire group of patients at 1, 2, and 3 years were 76.3%, 61.2%, and 51.0%, respectively.

CONCLUSIONSELIOT is a safe and well-tolerable technique and could be widely used for patients with malignant bone or soft tissue tumors around the joints with acceptable rates of acute and late toxicity. There is positive significance for controlling the tumor local recurrence, preserving the joint function and improving survival quality.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Bone Neoplasms ; radiotherapy ; surgery ; Elbow Joint ; Electrons ; therapeutic use ; Female ; Follow-Up Studies ; Hip Joint ; Humans ; Intraoperative Period ; Knee Joint ; Limb Salvage ; Male ; Middle Aged ; Neoplasm Metastasis ; Neoplasm Recurrence, Local ; Particle Accelerators ; Radiotherapy, Adjuvant ; Soft Tissue Neoplasms ; radiotherapy ; surgery ; Survival Rate ; Young Adult

OBJECTIVEAlbeit rare, rhabdomyosarcoma (RMS) may occur in middle to old-aged adults. The purpose of this study was to investigate the clinicopathological characteristics and prognosis of RMS in patients ≥ 40 years of age.

METHODSThe clinical, pathological and immunohistochemical profiles of the 76 cases were reviewed, and the outcomes were analyzed.

RESULTSThere were 46 males and 30 females with a median age of 55 years. Sixty cases occurred in somatic soft tissues, including extremities (n = 25), head and neck (n = 19) and trunk (n = 16). Sixteen developed in the internal organs with predilection for the urogenital tract. Histologically, 38 cases (50.0%) were of embryonal RMS (ERMS) subtype, 29 cases (38.2%) of pleomorphic RMS (PRMS), 7 cases (9.2%) of poorly-differentiated RMS (PdRMS), and 2 cases (2.6%) of alveolar RMS (ARMS). Immunohistiochemically, the tumor cells showed diffuse staining of desmin (68/68, 100%), with a variable expression of myogenin (30/43, 69.8%), MyoD1 (33/51, 64.7%) and MSA (29/40, 72.5%). The therapy data available in 54 cases showed that three inoperable cases were treated with chemotherapy and radiotherapy, 51 cases underwent local excision, including 10 cases with chemotherapy, 9 cases with radiotherapy, 8 cases with chemotherapy and radiotherapy after surgery, and the remaining 24 cases were not administrered with postoperative adjuvant therapy. The follow-up data available in 54 cases showed that 22 patients were alive with unresectable or recurrent/disease and 32 patients were alive with no evidence of disease. The median disease-free and overall survivals were 6.0 months and 7.0 months, respectively. Twenty-seven patients exhibited progression of disease with recurrence in 8 cases and metastasis in 19 cases. The median interval to progression was 6.0 months.

CONCLUSIONSThe most common subtypes of RMS in middle to old aged patients are ERMS and PRMS. In general, Rhabdomyosarcomas of middle to old aged patients shows a highly aggressive behavior. Patients may benefit from multi-disciplinary therapy.

Adult ; Aged ; Aged, 80 and over ; Chemotherapy, Adjuvant ; Desmin ; metabolism ; Disease-Free Survival ; Extremities ; Female ; Follow-Up Studies ; Head and Neck Neoplasms ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Humans ; Male ; Middle Aged ; MyoD Protein ; metabolism ; Myogenin ; metabolism ; Neoplasm Recurrence, Local ; Radiotherapy, Adjuvant ; Rhabdomyosarcoma ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Rhabdomyosarcoma, Alveolar ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Rhabdomyosarcoma, Embryonal ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Soft Tissue Neoplasms ; drug therapy ; metabolism ; pathology ; 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

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

OBJECTIVETo analyze the clinical features, diagnosis, treatment, and prognosis of epithelioid sarcoma (ES).

METHODSThe clinical data of 13 cases with epithelioid sarcoma in the Tianjin Medical University Cancer Institute and Hospital from March 1995 to December 2009 were collected and analyzed. There were 10 males and 3 females in the group, with an average age of 41.5 years (range: 13 to 68 years). Nine patients had classic ES and 4 had proximal-type ES. Surgery was the mainstay of treatment. After the operation, four patients received radiotherapy, five received chemotherapy, and one received chemoradiotherapy.

RESULTSOf the 13 cases, only 1 had multi-locus lesion. The average tumor size was (6.07 ± 1.34) cm. The lymph node involvement was found in 46.2% of the patients. Local and distant failure occurred in 50% and 30% patients, respectively. The most common site for dissemination was the lung. Four cases died within 3 years after initial operation. The 1-, 2-, 5-, 10-year overall survival rates of the 11 cases were 72.7%, 54.5%, 27.3% and 9.1%, respectively, with a median survival time of 27 months.

CONCLUSIONSEpithelioid sarcoma is a rare disease. The prognosis for patients with epithelioid sarcoma is poor because of a high propensity for local recurrence, lymph node metastases, and/or distant metastases. The definite diagnosis depends mainly on the pathologic examination. Wide surgical excision is the mainstay treatment, and radiation and chemotherapy have been used occasionally as adjuvant therapy but have had limited success.

Adolescent ; Adult ; Aged ; Chemotherapy, Adjuvant ; Extremities ; Female ; Follow-Up Studies ; Humans ; Lung Neoplasms ; secondary ; Lymph Node Excision ; Lymphatic Metastasis ; Male ; Middle Aged ; Mucin-1 ; metabolism ; Neoplasm Recurrence, Local ; Radiotherapy, Adjuvant ; Retrospective Studies ; Sarcoma ; diagnosis ; metabolism ; pathology ; therapy ; Soft Tissue Neoplasms ; diagnosis ; metabolism ; pathology ; therapy ; Survival Rate ; Vimentin ; metabolism ; Young Adult