Clinical outcome of parosteal osteosarcoma

BACKGROUND: Parosteal osteosarcoma is a rare type of osteosarcoma with distinct characteristics. Clinical outcome of 21 patients was analyzed to assess the predictive relevance of surgical margin, intramedullary tumor extension and histologic grade. METHODS: There were 5 mens and 16 womens with an average age of 26 years. Average follow-up was 9.1 years (range 2.5-22.1). Most common sites were distal femur (15) and proximal humerus (2). No patient presented with metastasis. Surgical margin was wide in 13 and marginal in 8. Intramedullary extension was seen in 10 patients (48%). Focal high-grade 2 and 3 tumors were seen in 11 (52%) and 3 (14%) patients respectively. RESULTS: Twenty patients (95%) were alive without disease. Two (10%) had relapse, one with local recurrence and another with local recurrence and lung metastasis. Of eight marginal procedures performed, 2 turned out to have histologically tumor-positive margins, both of whom later developed relapses. All patients with histologically negative margins remained disease-free. Presence of intramedullary extension and focal high-grade tumor was not significantly associated with relapse. CONCLUSIONS: A marginal but histologically negative margin of excision appears adequate for parosteal osteosarcoma. However, long-term follow-up is warranted for monitoring of rare incidences of local recurrences or distant metastases.     

Surgical outcomes and prognostic factors of non-metastatic radiation-induced sarcoma of bone

BackgroundThe survival and prognostic factors in non-metastatic, radiation-induced bone sarcomas of bone have not been described. Moreover, the quantitative data about surgical outcomes and complications after limb-salvage surgery versus amputation are quite limited.MethodsTwenty-five patients with non-metastatic, radiation-induced sarcoma of bone who underwent definitive surgery were analysed. Histological diagnosis was osteosarcoma in 19 and undifferentiated pleomorphic sarcoma in six. The definitive surgery was limb-salvage surgery in 15 patients and an amputation in 10.ResultsThe 5-year overall survival rate (OS) and the 5-year event-free survival rate (EFS) were 53% (95% CI 31%–70%) and 40% (21%–59%), respectively. Patients with wide or radical surgical margins (n = 13) showed significantly better OS compared with those with marginal (n = 8) or intralesional (n = 2) margins (5-year OS, radical or wide = 74%, marginal = 17%, intralesional = 0%, p = 0.044). The risk of local recurrence was significantly higher in the limb-salvage group compared to the amputation group (49% vs 0%, p = 0.011). OS and EFS were not significantly different between limb-salvage group and an amputation group (p = 0.188 and 0.912, respectively).ConclusionsWe believe non-metastatic, radiation-induced sarcoma of bone should be resected with the aim of achieving wide or radical margins. Although limb-salvage surgery was related to higher rates of local recurrence compared with those of the amputation group, OS and EFS were not different among two groups. Surgeons need to discuss the higher risk of local recurrence in limb-salvage surgery.     

Periosteal chondrosarcoma: A case series in a referral center with survivorship analysis

BackgroundPeriosteal chondrosarcomas are among the rarest types of chondrosarcomas dealt with in few small series of cases. In this study, we aimed to present our experience with this chondrosarcoma, seek for prognostic factors for OS and DFS and survey the status of IDH1 and IDH2.Results55 periosteal chondrosarcomas were retrospectively identified. Median age was 37 years, there was a male predominance (62%). The great majority of cases involved the metaphysis of long bones of the extremities. The median size of the tumors was 7.5 cm. Thirty patients underwent to subtotal surgical resection, 22 to tangential resection and the remaining 3 to amputation. The margins, reported in 54 cases, were wide/radical in 38 patients (70.4%), marginal in 9 (16.7%) and intralesional in 7 (12.9%). Histologically, 23 (42%) were grade 1; 27 (49%), grade 2; 3 (5%), grade 3 and 2 (4%) were dedifferentiated.A third of cases in which mutational analysis was feasible harbored heterozygous mutations in codon 132 of IDH1. Fifty-four cases were included for follow-up (median, 137 months). Four patients had local recurrences and six patients developed metastasis to the lungs. All patients that developed metastasis died of disease, two died of unrelated causes and 46 were alive without disease. OS and DFS was not found to be statistically associated with clinical and pathological parameters considered.Conclusionsperiosteal chondrosarcomas exhibit a low-grade behavior that can be adequately treated with marginal excisions. Clinical and morphologic parameters do not seem to predict their outcome.     

Malignant fibrous histiocytoma of jaws. A clinicopathologic study of 11 cases

Malignant fibrous histiocytoma (MFH) of the jaws is a highly malignant tumor that recurs, metastasizes, and usually causes death despite aggressive surgical therapy. This clinicopathologic review looks at five patients with MFH of the maxilla and six with MFH of the mandible. Five male and six female patients ranged in age from 12 to 75 years (mean, 35.4 years). All patients had large lytic areas of bone destruction, often with soft tissue extension. Two cases were postirradiation sarcomas, one of the maxilla and the other of the mandible. All patients underwent surgery and eight patients received chemotherapy when disease recurred locally or metastasized. Seven patients had local recurrences 3 to 13 months following surgery, and six patients had distant metastases. Of the 11 patients, 7 died of their disease, 1 died of unknown causes, and another with extensive local disease was lost to follow-up after 1.7 years. Two patients with recurrent disease are alive at 18 and 27 months postoperatively.     

Malignant fibrous histiocytoma: a retrospective study of 109 cases

The purpose of this report is to assess the prognostic factors that could influence management and clinical outcome of malignant fibrous histiocytoma (MFH) of soft tissues. Between 1975 and 1998, 109 patients diagnosed with MFH of the soft tissues, seen at King Faisal Specialist Hospital and Research Center, have been reviewed. Of the 109 patients, 75 were men and 34 were women. The median age at presentation was 48 years (range: 3-94). Seven patients (6%) had regional nodal disease and 10 other patients (9%) with distant metastases were excluded from survival analysis. The remaining 92 patients had localized disease and had surgery as the primary treatment modality with or without radiotherapy and/or chemotherapy. Extremities were the most common location (58%). Tumors less than 5 cm represented 32%, whereas 68% had tumors 5 cm or more. Low-grade tumors constituted 46%, and the remaining 54% were high grade. Thirty-seven percent of patients had positive surgical margins histologically after complete gross resection. The 5- and 10-year relapse-free survival (RFS) rates were 39% and 36%, respectively. Isolated local recurrence occurred in 20 patients (22%), isolated metastatic disease without local recurrence in 9 patients (10%), and combined local and metastatic disease occurred in 20 patients (22%). The overall 5- and 10-year overall survival (OS) rates were 50% and 43%, respectively. On multivariate analysis, tumor size and radiation dose were significant factors for RFS (p = 0.04 and 0.0005, respectively). In terms of OS, size, histologic grade, and surgical margins were significant factors on multivariate analysis (p = 0.001. 0.006, and 0.0001, respectively). Complete surgical resection at the time of primary tumor presentation is likely to afford the best chance for RFS and OS. Radiation therapy plays an important role, in combination with surgery for better local control, particularly in high-grade lesions, and in cases with positive surgical margins after wide complete gross excision. The role of adjuvant chemotherapy remains investigational.     

Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities.

AIMS: Dermatofibrosarcoma protuberans is a rare condition which is frequently misdiagnosed at presentation, resulting in a high incidence of local recurrence due to inadequate resection. The archives of the Department of Orthopaedics at the University of Florida were analysed to investigate the natural history and results of treatment for this tumour. METHODS: Between 1975 and 1996, 35 cases of DFSP were treated at the University of Florida. Of these, one was treated primarily, five were treated for local recurrence, 17 had tumour bed excisions following inadequate primary excisions elsewhere and 12 had tumour bed excisions following inadequate resection of local recurrences elsewhere. The data were analysed to assess the impact of age, gender, duration of symptoms, tumour site and size, surgical margin, number of operations and adjuvant treatments on survival and local recurrence outcomes. RESULTS: Complete follow-up was available for 34 patients. Mean follow-up was 58 months (range 12-144 months). Thirty-three patients remain alive and disease-free. One patient died of unrelated causes. The margins obtained were wide in 28 patients, marginal in six and intralesional in one. Of the seven patients with inadequate surgical margins, four received adjuvant radiation therapy and remain disease-free. No patient with an adequate margin developed a local recurrence, but there were three local recurrences in the patients with an inadequate margin who did not receive adjuvant radiation therapy (local recurrence rate: 8%). No patient developed lymphatic or distant metastasis. Local recurrences were more likely to be classified Stage IB (17/17) than primary tumours (1/18) (P<0.001). Local recurrence was more likely where the surgical margin was less than 2.5 cm from the lesion. CONCLUSIONS: Dermatofibrosarcoma protuberans is a low-grade tumour that has a high potential for local recurrence unless it can be completely excised. The overall rate of local recurrence in referred patients in this series was 20/35 cases (57%). All occurred after inadequate margins at previous surgery in other institutions. Revision surgery in these patients showed a local recurrence rate of 8%. To avoid extensive surgery for recurrences, initial treatment should be by wide excision incorporating the underlying deep fascia and a cuff of 2.5-3 cm of normal skin tissue. Radiation therapy provides a useful adjunct where adequate margins cannot be obtained.     

Status of surgical margins and prognosis in adult soft tissue sarcomas of the extremities: a series of patients treated at a single institution.

PURPOSE: To explore the prognostic effect of microscopic marginal status after surgery for extremity soft tissue sarcomas. PATIENTS AND METHODS: We analyzed 911 consecutive patients surgically treated throughout a 20-year span at a single referral center. Six hundred forty-two were first seen with a primary tumor, and 269, with a locally recurrent tumor. All patients underwent macroscopically complete resection. Microscopic marginal status was negative (tumor size > 1 mm) in 748 patients and positive (相似文献   

Perioperative fractionated high-dose rate brachytherapy for malignant bone and soft tissue tumors

Purpose: To investigate the viability of perioperative fractionated HDR brachytherapy for malignant bone and soft tissue tumors, analyzing the influence of surgical margin.

Methods and Materials: From July 1992 through May 1996, 16 lesions of 14 patients with malignant bone and soft tissue tumors (3 liposarcomas, 3 MFHs, 2 malignant schwannomas, 2 chordomas, 1 osteosarcoma, 1 leiomyosarcoma, 1 epithelioid sarcoma, and 1 synovial sarcoma) were treated at the Osaka University Hospital. The patients’ ages ranged from 14 to 72 years (median: 39 years). Treatment sites were the pelvis in 6 lesions, the upper limbs in 5, the neck in 4, and a lower limb in 1. The resection margins were classified as intracapsular in 5 lesions, marginal in 5, and wide in 6. Postoperative fractionated HDR brachytherapy was started on the 4th–13th day after surgery (median: 6th day). The total dose was 40–50 Gy/7–10 fr/ 4–7 day (bid) at 5 or 10 mm from the source. Follow-up periods were between 19 and 46 months (median: 30 months).

Results: Local control rates were 75% at 1 year and 48% in 2 years, and ultimate local control was achieved in 8 (50%) of 16 lesions. Of the 8 uncontrolled lesions, 5 (63%) had intracapsular (macroscopically positive) resection margins, and all the 8 controlled lesions (100%) had marginal (microscopically positive) or wide (negative) margins. Of the total, 3 patients died of both tumor and metastasis, 3 of metastasis alone, 1 of tumor alone, and 7 showed no evidence of disease. Peripheral nerve palsy was seen in one case after this procedure, but no infection or delayed wound healing caused by tubing or irradiation has occurred.

Conclusion: Perioperative fractionated HDR brachytherapy is safe, well tolerated, and applicable to marginal or wide surgical margin cases.     

Treatment strategies and outcomes of primary Myxofibrosarcomas in a large patients cohort

Backgroundthis study analysed primary myxofibrosarcoma (MFS) to investigate patient outcomes focusing on histopathologic margins and perioperative treatments.Patients and methodsdata from consecutive patients affected by primary and localized MFS of the extremities or trunk wall who underwent surgery (2002–2017) were analysed. Local recurrence (LR), amputation rate, incidence of distant metastasis (DM), and overall survival (OS) were studied.ResultsOf 293 included patients, 52 (17%) patients received perioperative treatments and 54 (18%) had positive microscopic histopathologic margins (R1). Median follow-up was 80 months (IQR, 49–109). 5-yr CCI of LR was 0.12 (SE: 0.02). Status of histopathologic margins (P < 0.001), tumour malignancy grade (P = 0.018) and size (P = 0023) were independent prognostic factor for LR. Nine amputations (amputation rate: 3%) were performed (N = 1 for primary tumour; N = 8 for LR). Larger tumour size (P = 0.015) and higher grade (P = 0.025) were independent prognostic factor for DM. 5-year OS was 0.84 (95%CI 0.79–0.88). Patient age (P = 0.008), tumour size (P = 0.013) and malignancy grade (P = 0.018) were independently associated to OS. In the subgroup of patients who had a re-excision for a primary MFS (N = 116, 40%), the presence of residual disease was not associated with LR, DM, or OS.Conclusionin this study 5-year LR, DM and OS were 12%, 17%, and 84%, respectively. One in six patients had a positive surgical margin, which was a prognostic factor for LR, while DM and OS were predicted by tumour grade and size. Findings from this large patient cohort may set benchmarks for investigating new treatment options for MFS.     

骶骨脊索瘤外科治疗长期随访

目的 回顾骶骨脊索瘤外科治疗后长期随访的结果,分析评估影响外科治疗效果的临床因素.方法 1978年10月至2000年10月,我院共收治68例骶骨脊索瘤,男性60例,女性8例,年龄25～74岁,中位年龄55.5岁,部位为S1～5 7例,S2～5 15例,S3～5 33例,S4～5 5例,其他8例.外科手术共104例次,首次在我院外科治疗的原发病例48例,肿瘤外科切除边界为广泛切除4例（8.3%）;边缘切除21例（43.7%）;囊内切除23例（48.0%）;复发病例20例.结果 随访1-365个月,平均81.84个月.存活53例（77.9%）;死亡15例（22.1%）,其中,围手术期内死亡7例,占死亡总数的46.7%.总体5年生存率87.3%,10年生存率73.3%,中位生存时间（月）282.0±88.7.其中囊内切除者、边缘切除者和广泛切除者5年生存率,三者比较统计学无明显差异（P=0.18）.手术囊内切除者复发34例（81.0%）,边缘切除者复发8例（36.4%）,统计学差异明显（P=0.000）;总体1年无复发生存率77.4%;3年33.2%,5年24.3%.我院首次手术与非我院首次手术患者的无复发生存率比较,统计学差异明显（log-rank P=0.000）.边缘切除与囊内切除患者的无复发生存率比较,统计学差异明显（log-rank P=0.000）.Cox回归分析显示是否我院首次手术及不同手术边界均为预测局部复发的独立因素.结论 骶骨脊索瘤外科切除,局部复发率高,生存期较长,外科切除边界是影响局部复发的重要因素,首次手术对于预后有重要影响.     

The role of up-front radiation therapy for incompletely resected pediatric WHO grade II low-grade gliomas

The purpose of this study was to assess the impact of early radiation therapy and extent of surgical resection on progression-free survival (PFS) and overall survival (OS) in children with WHO grade II low-grade gliomas (LGGs). We conducted a historical cohort study of 90 patients, ages 21 or younger, diagnosed with WHO grade II LGGs between 1970 and 1995. Median follow-up for surviving patients was 9.4 years (range, 0.5-22.6 years). Tests for variables correlating with OS and PFS were conducted by using log-rank tests and Cox proportional hazards models. Eleven patients underwent gross total resections (GTRs), 43 had subtotal resections, and 34 underwent biopsy only at diagnosis. Two patients underwent biopsy at time of recurrence. Of the 90 patients, 52 received radiation as part of their initial therapy following diagnosis (early-RT group). The overall five-year PFS and OS rates +/- SE were 56% +/- 5% and 90% +/- 3%, respectively. Ten-year PFS and OS rates were 42% +/- 6% and 81% +/- 5%, respectively. For patients older than three years and without GTRs, administration of early radiation did not appear to influence PFS or OS (P = 0.98 and P = 0.40, respectively; log-rank test). This was confirmed by multivariate analyses (P = 0.95 and P = 0.33 for PFS and OS, respectively). Of the 11 patients with GTRs, disease progressed in only two, and all were alive with no evidence of disease at last follow-up. Patients who underwent GTRs had significantly longer PFS (P = 0.02), but did not have significantly improved OS. Excellent long-term survival rates were achieved for children with WHO grade II LGGs. We were unable to demonstrate a benefit for administering radiation as part of initial treatment. An outcome benefit was seen with greater extent of resection.     

Osteosarcoma of the pelvis: experience of the Cooperative Osteosarcoma Study Group.

PURPOSE: To define patients and tumor characteristics as well as therapy results, patients with pelvic osteosarcoma who were registered in the Cooperative Osteosarcoma Study Group (COSS) were analyzed. PATIENTS AND METHODS: Sixty-seven patients with a high-grade pelvic osteosarcoma were eligible for this analysis. Fifteen patients had primary metastases. All patients received chemotherapy according to COSS protocols. Thirty-eight patients underwent limb-sparing surgery, 12 patients underwent hemipelvectomy, and 17 patients did not undergo definitive surgery. Eleven patients received irradiation to the primary tumor site: four postoperatively and seven as the only form of local therapy. RESULTS: Local failure occurred in 47 of all 67 patients (70%) and in 31 of 50 patients (62%) who underwent definitive surgery. Five-year overall survival (OS) and progression-free survival rates were 27% and 19%, respectively. Large tumor size (P =.0137), primary metastases (P =.0001), and no or intralesional surgery (P <.0001) were poor prognostic factors. In 30 patients with no or intralesional surgery, 11 patients with radiotherapy had better OS than 19 patients without radiotherapy (P =.0033). Among the variables, primary metastasis, large tumor, no or intralesional surgery, no radiotherapy, existence of primary metastasis (relative risk [RR] = 3.456; P =.0009), surgical margin (intralesional or no surgical excision; RR = 5.619; P <.0001), and no radiotherapy (RR = 4.196; P =.0059) were independent poor prognostic factors. CONCLUSION: An operative approach with wide or marginal margins improves local control and OS. If the surgical margin is intralesional or excision is impossible, additional radiotherapy has a positive influence on prognosis.     

Metastatic Primary Leiomyosarcoma of the Pancreas to the Liver: Report of a Surgically Treated Case

Purpose

The aim of this study was to evaluate the role of stereotactic body radiotherapy (SBRT) as adjuvant therapy for resected pancreatic adenocarcinoma with close or positive margins.

Methods

Between September 2006 and January 2010, 24 patients were treated with adjuvant SBRT following surgical resection. Eight (33.3%) patients had close margins of 1?C2.5?mm to the retroperitoneal, vascular structures, and periduodenal adipose tissue. Sixteen (66.7%) patients had positive margins at retroperitoneal margin and vascular structures. Twenty-three patients received 24?Gy (20?C24?Gy) in one fraction, and one had 30?Gy in three fractions. The median target volume was 11?cc (4.5?C30?cc). Eighteen patients were treated with the Cyberknife? Robotic Radiosurgery System and six patients were treated with Trilogy? intensity-modulated radiosurgery. Kaplan?CMeier survival analyses were used to estimate freedom-from-local-progression (FFLP), and overall survival (OS) rates. PET/CT or CT was used to monitor disease recurrence following SBRT.

Results

The median follow-up for all patients was 12.5?months (1.4?C39.5?months), and among surviving patients it was 16.3?months (2?C39.5?months). The FFLP rates at 6?months, 1 and 2?years were 94.7%, 66%, and 44%, respectively. Overall, FFLP was achieved in seven (87.5%) patients with close margins, and 10 (62.5%) with positive margins. After SBRT, 19 patients resumed or started a 6-month course of gemcitabine-based chemotherapy at a median interval of 18?days (range, 9?C31?days) post-SBRT. The median OS was 26.7?months and the 1- and 2-year OS rates were 80.4% and 57.2%, respectively. Of the 24 patients, 12 (50%) developed distant metastases of whom two (25%) had close margins and 10 (62.5%) had positive margins. Ten patients (41.7%) were free of progression at last follow-up (range, 3?C39.5?months). Three patients (12.5%) had grade 1?C2 acute GI toxicities, and two patients (8.3%) had grade 1 and 2 late toxicities. No patients experienced grade 3 or 4 toxicity, including bowel perforation, secondary to SBRT.

Conclusions

Our data suggest that adjuvant SBRT for resected pancreatic cancer can be achieved with minimal toxicity. This shorter treatment course allowed initiation of systemic chemotherapy shortly after the completion of SBRT.     

Osteosarcoma of the pelvis.

AIMS: To describe the outcomes of a large number of patients with pelvic osteosarcoma, and to define the guidelines for appropriate treatment. METHODS: We reviewed 60 consecutive patients with primary pelvic high-grade osteosarcoma. The tumour involved the whole hemipelvis in 15 cases, while the most common location was the iliac wing in 29 cases (48.3%): 25 of these adjacent to or passing the sacroiliac joint. RESULTS: Thirty patients underwent surgery; there were 16 hindquarter amputations and 14 internal hemipelvectomies. All the patients who presented with metastasis died of their disease. In 18 cases wide margins were achieved, however, eight patients experienced local recurrence. Of the series, only eight patients are still alive. CONCLUSION: The use of intense chemotherapy and surgical wide margin, hardly seems to achieve local control, however, tumour necrosis was correlated with positive prognosis. When internal hemipelvectomy it is not safe enough, amputation must be considered, particularly for cases with sacrifice of the sciatic nerve roots or for older patients where a shorter surgical procedure can be less risky.     

Preoperative irradiation for soft tissue sarcomas of the trunk and extremities in adults

Between May 1978 and January 1987, 58 adult patients with previously untreated sarcomas of the trunk and extremities were treated with preoperative irradiation and surgery at the University of Florida. All patients had a minimum of 2 years of follow-up; 24 had a minimum of 5 years of follow-up. The preoperative dose was usually 5040 cGy, with 120-125 cGy per fraction delivered twice daily. Operations were performed 2 to 6 weeks after radiation therapy. Eight patients received adjuvant chemotherapy. The tumors were high grade in 52 (90%), measured greater than 10 cm in 45 (78%), and were extracompartmental in 49 (84%). The surgical margins were wide in 17, marginal in 31, and intralesional in 10 patients. A functional extremity was preserved in 47 of 54 patients who would have required an amputation had they been treated by operation alone. Five of 58 patients (9%) developed local failure; in three, the failure occurred outside of the irradiated volume. Survival rates (product-limit method) at 5 years according to grade and size of lesion were as follows: low grade, 100%; high grade, 10 cm or less in largest diameter, 68%; high grade, 11-20 cm, 39%. Data are insufficient for a 5-year analysis of high-grade lesions greater than 20 cm; to date, there are no 5-year survivors in these patients. Moderate and severe wound complications occurred in 16%. There were four pathological fractures in 52 long bones at risk.     

Comparison of patient profiles from a comprehensive cancer center and the general population

Of the 98 soft-tissue sarcomas treated between 1973-1978, 12 were retroperitoneal in origin. Fifty percent underwent one to three previous surgical procedures. All patients were operated on. In 75% (nine out of 12), the tumor was completely resected. The operative mortality for total tumor excision was 11%. There were five liposarcomas, three leiomyosarcomas, two neurofibrosarcomas, and one synovial sarcoma and one unclassified sarcoma. Six of the 12 patients survived five years and three (25%) were alive without evidence of disease at 5 years. Three of the completely resected group received no further treatment; 2/3 survived 5 years; three had postoperative chemotherapy; 3/3 survived; and three others had radiation and chemotherapy subsequent to surgery; all three have died. The two most important prognostic factors were tumor grade and surgical excision. Only one patient with high grade tumor survived. Total surgical excision of the tumor offers the only hope for cure. Aggressive surgery in treating retroperitoneal soft part sarcomas is strongly recommended.     

Chondrosarcoma of the pelvis: oncologic and functional outcome

Purpose. Chondrosarcoma (CS) most commonly involves the pelvis. The factors that influence local and systemic control of pelvic CS and the functional outcome should be evaluated.Patients. Fifty-one patients (37 males and 14 females; mean age, 39.4 years) with pelvic CS were included in this retrospective study.Methods. The tumor stage, surgical treatment, surgical margin achieved, complications, incidence of local recurrence (LR), incidence of distant metastases, and the oncologic and functional status were evaluated. Oncologic outcome was estimated by the method of Kaplan and Meier, and the functional status was scored according to Musculoskeleral Tumor Society (MSTS) criteria. Analysis of variance was used to determine the factors that influence the oncologic and functional outcome.Results. Surgical stages were IA in three cases, IB in 23, IIB in 23, and III in two. Hemipelvectomy (H) was performed in 13 cases, internal hemipelvectomy (IH) with endoprosthetic replacement in 17, and continuity resection (CR) in 23.Two patients received IH and CR, one due to LR, and one due to instability. Radical or wide margins were achieved in 27 cases, marginal margins in 16, and intralesional margins in eight. Local complication required additional surgery in 10 cases due to local infections and/or hematomas.Two patients died perioperatively. In 48 out of the 49 remaining patients, follow-up was available with a mean duration of 73.4 months (range, 4-229 months).Twenty patients died of the disease, two patients are alive with metastases, four patients are disease free after LR, and 22 patients show no evidence of the disease. LR occurred in 10 cases (20.4%), and 17 patients (34.6%) developed distant metastases. Functional evaluation of the 28 survivors revealed good and excellent results in 19 cases, fair in three and poor in six.The mean MSTS score of all survivors was 69.2%, after H it was 37.6%, after IH was 61.4%, and after CR was 79.5%.Conclusion. In pelvic chondrosarcoma, survival was determined by the tumor stage and the surgical margin achieved.The incidence of LR was influenced by the surgical margin achieved, whereas the incidence of distant metastases was influenced by the tumor stage. The best oncologic results in chondrosarcoma involving the innominate bone could be found in low-grade tumors, and the best functional results after continuity resection and restoration of the pelvic girdle.     

Treatment of osteosarcoma at first recurrence after contemporary therapy: the Memorial Sloan-Kettering Cancer Center experience

BACKGROUND: Overall survival after recurrence of osteosarcoma (OS) is < 30%. The authors reported their experience treating recurrent OS at the time of first recurrence (R1). METHODS: Patients with high-grade OS who achieved complete disease remission (CR) after primary surgery and chemotherapy, and patients who were treated at R1 at Memorial Sloan-Kettering Cancer Center (New York, NY) after 1990 were analyzed by retrospective chart review. RESULTS: For 43 eligible patients, the median time to R1 from initial diagnosis was 21.7 months (range, 4.6-135.7 mos). The lungs were the most common sites of disease recurrence (n = 33 of 43). With a median follow-up of 15.2 months (range, 0.7-158.3 mos) after R1, 15 of 43 (35%) patients were alive. Four of 43 patients were treated with surgery alone (3 patients were alive and 1 had died of progressive disease at the time of last follow-up). Due to unresectable disease, eight patients received only chemotherapy, none of whom survived. For patients with disease recurrence treated with chemotherapy and surgery (n = 31), 22 patients achieved a second CR (CR2). Nine patients were alive and in disease remission (29%) at the time of last follow-up. Twenty-three patients received ifosfamide as part of their retrieval regimen. Of the 18 who achieved a CR2, 8 experienced disease recurrence, 7 remain alive in CR2, and 3 died due to toxicity. Eight patients did not receive ifosfamide. Of these, 4 achieved a CR2 but 3 subsequently experienced disease recurrence. CONCLUSIONS: At R1, 22 of 31 patients achieved a CR2 with aggressive surgery and chemotherapy. The majority of these patients subsequently developed a disease recurrence. Patients appeared to benefit from the addition of ifosfamide to their retrieval regimens. In the end, the role of chemotherapy in recurrent OS continues to remain undefined.     

Intraoperative radiation therapy of extrahepatic biliary carcinoma: a report of RTOG-8506.

Twenty-three patients with unresected, resected but residual, or locally recurrent biliary duct cancer were entered on a Radiation Therapy Oncology Group (RTOG) Phase I-II study. Of 16 patients who were properly entered and eligible for study, eight patients (50%) completed protocol treatment including intraoperative radiation therapy (IORT). Minor acute complications from therapy were common. There was no early grade 3 or 4 toxicity. Major long-term toxicity (grade 4) was noted in one patient who suffered a perforated viscous related to salvage radiation therapy for a scar recurrence and died of infection 1 month after retreatment. With a median follow up of 10.5 months, two of the eight patients who received IORT are alive. Of the six patients who died, one had persistent disease, one had the tumor recur in the surgical wound and subsequently died of complications related to retreatment with high dose radiation, and one died of intercurrent disease. In addition, two patients died with liver metastasis, and one died with intraabdominal disease outside of the IORT volume. Bulk of disease may be an important determinant of outcome.     

Extrapulmonary small cell carcinoma: single center experience with 61 patients

Extrapulmonary small-cell carcinoma (EPSCC) is a clinicopathological entity distinct from small-cell carcinoma (SCC) of the lung. The aim of this study was to review the clinico-pathologic features, treatment modalities, and factors prognostic for survival in patients with EPSCC. We retrospectively reviewed the medical records of patients with EPSCC diagnosed between January 1995 and July 2004 at the Asan Medical Center. We identified 61 patients with EPSCC, 37 with limited disease (LD) and 24 with extensive disease (ED). The most common primary sites were the gastrointestinal (GI) tract (56%) and uterine cervix (18%). Overall survival (OS) at 1 and 3 years was 59% and 29%, respectively, with a median survival of 16 months (range, 1 approximately 56 months). Treatment information was available for 51 patients, 34 with LD and 17 with ED. Of the 34 LD patients, 25 underwent surgery. Surgery was the only treatment modality in five patients, two of whom remained alive and disease free at last follow-up, 27 and 47 months after surgery, respectively. Adjuvant chemoradiotherapy was administered to 11 patients, nine of whom (82%) had distant failure with a median overall survival of 23 months. Of the eight patients who received adjuvant chemotherapy, four (50%) had distant failure, with a median survival of 21.7 months. In univariate analysis, advanced disease status, as measured by VALSG (LD vs. ED) stage, was a significant prognostic factor for OS (p<0.001). Interestingly, there were no statistically significant differences in progression-free survival or OS between patients with pure (n=45) and mixed (n=16) EPSCC. Overall, the response to various treatment modalities and the median survival time observed were discouraging. Patients with GI primary tumors had poorer prognoses than those with primary tumors at other locations. Fifty six percent of patients with a GI primary tumor had ED at the time of diagnosis, whereas 100% of patients with SCC of the uterine cervix had LD at the time of diagnosis and showed a favorable clinical course. The majority of patients with LD SCC who underwent surgery, followed by adjuvant chemotherapy or chemoradiotherapy, showed tumor recurrence and/or systemic metastases. Clinical trials are needed to define adequate treatment strategies for EPSCC.