Chondrosarcoma of the scapula: long-term oncologic outcome

BACKGROUND: Chondrosarcoma is the second most common primary sarcoma of bone. It often develops within flat bones, such as the pelvis, ribs, and scapula. In the current study, the authors reviewed the surgical experience and long-term oncologic outcomes of patients with chondrosarcoma arising in the scapula. METHODS: The medical records of 29 consecutive patients with chondrosarcoma of the scapula were reviewed. The patients were treated between 1954 and 1994. All patients had localized disease at the time of presentation. The tumors were classified histologically as Grade 1 (10 patients), Grade 2 (10 patients), Grade 3 (7 patients), dedifferentiated (1 patient), and mesenchymal (1 patient) (using the criteria of Evans et al.). The mean maximal dimension of the tumors was 11 cm. Twenty-five patients underwent limb-sparing surgical resection and 4 patients underwent forequarter amputations. The median follow-up was 13 years (range, 1-35 years). RESULTS: At last follow-up, 22 patients (76%) were free of disease and 7 patients (24%) had died of their disease. Local recurrence occurred in 4 patients at 7 months, 16 months, 40 months, and 43 months, respectively. The local recurrence-free survival rate was 86% at 5 years, 10 years, and 20 years. Disease-specific survival was 83% at 5 years, 74% at 10 years, and 74% at 20 years. Patients who had low-grade chondrosarcomas had better survival compared with patients who had high-grade chondrosarcomas (P = 0.07). CONCLUSIONS: Patients who had localized chondrosarcoma of the scapula had a favorable long-term outcome, most likely due to the unique anatomic features that improved the likelihood of achieving wide surgical margins with limb-sparing surgery, despite the frequent presentation of locally advanced disease.     

High‐risk extracranial chondrosarcoma

BACKGROUND:

A study was undertaken to evaluate results of surgery and radiotherapy (RT) for high‐risk extracranial chondrosarcomas.

METHODS:

Between 1986 and 2006, 60 patients underwent surgery and RT for extracranial high‐risk chondrosarcoma. Preoperative RT (median, 50 gray [Gy]) and postoperative RT (median, 60 Gy) were used in 40% and 60% patients, respectively. Sites included pelvis/lower extremity (48%), chest wall (22%), spine/paraspinal (17%), and head and neck (13%). Overall, median tumor size was 7 cm (range, 1‐22 cm), and tumor grade was I, II, and III in 22%, 64%, and 14% of cases, respectively.

RESULTS:

Pathologically clear surgical margins (R0) were present in 50%, microscopic positive margins (R1) in 28%, and gross positive margins (R2) in 13%, half of whom had clinically detectable residual disease; surgical margin was unknown in 8%. Median follow‐up was 75 months (range, 5‐230 months). The crude local control rate was 90%. Patients with R0, R1, and R2 resections had local control of 100%, 94%, and 42%, respectively. Of the 8 cases that had R2 resection, 3 experienced uncontrolled progression, but 5 patients had stable disease with long‐term follow‐up. The 10‐year overall survival, progression‐free survival, and cause‐specific survival were 86%, 80.5%, and 89.4%, respectively. Younger age and grade III tumors were associated with worse progression‐free survival (P = .03 and .0003, respectively).

CONCLUSIONS:

Although surgery with complete resection is paramount in management of chondrosarcoma, RT is a useful adjuvant treatment and appears to offer excellent and durable local control where wide surgical resection is difficult to accomplish. Cancer 2011. © 2011 American Cancer Society.     

Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients

BACKGROUND: The prognosis of patients with chordoma of the sacrum and mobile spine has been reported to be dismal and attributable in the majority of cases to intralesional surgery. The purpose of this study was to evaluate the clinical outcome of these patients using modern surgical principles aimed at complete resection and to identify prognostic factors. METHODS: The clinical and morphologic features, type of surgery, and follow-up of 39 consecutive patients with chordoma were reviewed and analyzed statistically. RESULTS: Thirty sacral and 9 mobile spine chordomas (size range, 3-20 cm; mean, 8 cm) occurring in 22 women and 17 men (median age, 55 years) were analyzed. The preoperative morphologic diagnosis was based on fine-needle aspiration (FNA) biopsy, core needle biopsy, or incisional biopsy. The final surgical margins were wide in 23 patients and marginal or intralesional in 16. The mean follow-up was 8.1 years (range, 0.1-23 years). Seventeen patients (44%) developed local recurrences and 11 patients (28%) developed metastases. The estimated 5-, 10-, 15-, and 20-year survival rates were 84%, 64%, 52%, and 52%, respectively. Local recurrence was associated significantly with an increased risk of metastasis and tumor-related death (P < 0.001). CONCLUSIONS: New surgical techniques have improved local control and survival of patients with sacral or spinal chordoma significantly and have decreased progressive neurologic deterioration. Larger tumor size, performance of an invasive morphologic diagnostic procedure outside of the tumor center, inadequate surgical margins, microscopic tumor necrosis, Ki-67 > 5%, and local recurrence were found to be adverse prognostic factors. FNA is the preferred method for establishing the preoperative morphologic diagnosis of chordoma.     

Chondrosarcoma of the phalanx: a locally aggressive lesion with minimal metastatic potential: a report of 35 cases and a review of the literature.

BACKGROUND: Enchondroma is the most common primary benign bone tumor of the hand, especially the phalanges, whereas chondrosarcoma is uncommon at this site. Although phalangeal chondrosarcoma may have ominous histologic features, its biologic behavior is relatively indolent. METHODS: Thirty-five cases of phalangeal lesions previously diagnosed as chondrosarcoma were studied. Histologic and tumor-biologic parameters (Ki-67 and p53 immunohistochemistry) were investigated and correlated with clinical behavior. RESULTS: All cases were characterized by unequivocal malignant histologic features (Grade 2 or higher) or Grade 1 malignant histologic features combined with the presence of cortical destruction and soft tissue extension. The median age of the patients at the time of diagnosis was 67 years (range 21-87 years), with a slight female predominance. Occurrence in the hand was more common than in the foot, with the proximal phalanx affected most often. Treatment varied from local therapy (curettage or local excision) in 16 patients to amputation or exarticulation in 19 cases. Follow-up ranged from 8-432 months for 28 patients. Ten of 15 tumors treated by local therapy recurred whereas none of 13 tumors treated by radical surgery recurred. The median survival was 20.8 years; none of 28 patients developed metastases nor died of disease. Both the type of treatment and localization in the proximal phalanx were associated independently with local recurrence. CONCLUSIONS: Phalangeal chondrosarcoma behaves as a locally aggressive lesion and, in contrast to chondrosarcomas located elsewhere, rarely metastasizes. Treatment is indicated only because of its locally destructive growth. The authors believe that given the excellent survival data, curettage with adequate follow-up should be considered as the treatment of choice if technically feasible, especially in those cases in which amputation would lead to a significant loss of hand function.     

Dedifferentiated peripheral chondrosarcomas. A report of seven cases

Peripheral dedifferentiated chondrosarcoma (CS) is an exceedingly rare variant of the highly malignant entity of dedifferentiated chondrosarcoma. Only five such cases have previously been reported. Seven cases are analyzed and evaluated for the presentation and natural history of this highly malignant lesion when it arises in a tumor that was previously an osteochondroma. Both peripheral and central dedifferentiated chondrosarcomas are high-grade malignant lesions and require wide or radical surgical margins for adequate treatment. Despite adequate resection, survival is poor; five of the seven cases presented herein died of metastatic spread of their disease. These cases of the peripheral variant of dedifferentiated chondrosarcoma occurred in patients who were younger than patients with central dedifferentiated chondrosarcomas and may be present with longer duration of symptoms because they occur in previously long-standing benign osteochondromas. As such, they may be easily overlooked clinically and radiographically. Therefore, careful histologic analysis of all cartilage lesions arising on the surface of bone is essential to prevent overlooking foci of high-grade sarcomatous dedifferentiation.     

Overexpression of cathepsin B and urokinase plasminogen activator is associated with increased risk of recurrence and metastasis in patients with chondrosarcoma

BACKGROUND: Deregulation of the cellular protease network has been shown to be responsible for aggressive clinical behavior in several common human malignancies. In the current study, the authors evaluated the expression patterns of proteases in patients with chondrosarcoma of bone and correlated these patterns with clinical outcome. METHODS: The expression levels of urokinase plasminogen activator; matrix metalloproteinase types-1, -2, and -9; and cathepsins B and L were determined immunohistochemically in 114 cases of chondrosarcomas of bone and were correlated with their clinicopathologic parameters as well as with long term follow-up data. RESULTS: Overexpression of cathepsin B was associated with a high rate of local recurrence (P = 0.006) and a decreased recurrence free survival (P = 0.005). Overexpression of urokinase plasminogen activator was associated with an increased rate of metastasis (P = 0. 013), a decreased metastasis free survival (P = 0.016), and a decreased 5-year overall survival rate (P = 0.048). The univariate Cox model showed that tumor extension into soft tissue, high histologic grade, and overexpression of cathepsin B were predictors of adverse outcome. Multivariate analysis showed only overexpression of cathepsin B and tumor extension into soft tissue to be independent predictors of local recurrence. CONCLUSIONS: Overexpression of cathepsin B and urokinase plasminogen activator can be used to identify those patients with chondrosarcoma of bone who have an increased risk of local recurrence and distant metastases.     

Do pathological fractures influence survival and local recurrence rate in bony sarcomas?

The influence of pathological fracture on surgical management, local recurrence and survival was established in patients with high grade, localised, extremity osteosarcoma (n=484), chondrosarcoma (n=130) and Ewing's sarcoma (n=156). Limb salvage was possible in 79% of patients with a fracture compared to 84% of patients without a fracture (p=0.17). No difference in local recurrence was found between fracture and control groups. In univariate analysis, survival in the fracture group was lower than in the control group for osteosarcoma (34% versus 58%, p<0.01) and chondrosarcoma (35% versus 63%, p=0.04), but not for Ewing's sarcoma (75% versus 64%, p=0.80). In multivariate analysis, fracture remained a significant predictor of survival for osteosarcoma, but not for chondrosarcoma, where dedifferentiated subtype appeared to be decisive. Pathological fracture independently predicts worse survival in osteosarcoma, but not chondrosarcoma and Ewing's sarcoma. Limb saving surgery seems safe, if adequate resection margins are achieved.     

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.     

Clinical outcomes of 54 pelvic osteosarcomas registered by Japanese musculoskeletal oncology group

BACKGROUND: To determine the clinical outcomes in patients with pelvic osteosarcoma, we reviewed the experience with 54 pelvic osteosarcoma patients. METHODS: Thirty-five patients underwent surgical treatment with 30 undergoing limb salvage procedures and 5 requiring hemipelvectomies. RESULTS: Oncological outcome was continuously disease free in 12 cases, no evidence of disease in 3, alive with disease in 3, died of disease in 34 and died of other causes in 2. The 5-year survival rate was 29.5%. For the patients who underwent surgery, statistically significant prognostic factors were chemotherapy, surgical margin, tumor location in sacrum, efficacy of chemotherapy, local recurrence, metastasis and treatment for metastases. With univariate analysis, local recurrence, surgical margin and tumors resected from the sacrum were significant prognostic factors for metastasis. Surgical margin, tumor resected from sacrum and metastasis were significant prognostic factors for local recurrence. With regard to postoperative functional mobility, patients with tumors resected from the sacrum exhibited poorer functional mobility when compared with other sites. CONCLUSIONS: Patients with tumors located in the sacrum had worse prognosis, and significant prognostic factor for local recurrence and metastasis. Treatment of pelvic osteosarcoma especially located in the sacrum requires new approaches and further improvements in order to ensure successful outcomes.     

Local and systemic control for osteosarcoma of the extremity treated with neoadjuvant chemotherapy and limb salvage surgery: the Rizzoli experience

Five-hundred and twenty-six patients with non-metastatic osteosarcoma of the extremities treated at Istituto Ortopedico Rizzoli from 1983 to 1995 with neoadjuvant chemotherapy and limb salvage, were retrospectively studied to evaluate the rate of local and systemic control. At a mean follow-up of 9.5 years (3-17), 320 patients remained continuously free of disease and 206 relapsed. The 5-year disease-free survival and overall survival were 64% and 70% respectively. In patients who relapsed, there were 31 local recurrences (6%). The rate of local failures was significantly higher in the 79 patients with inadequate surgical margins (marginal, intralesional, and contaminated margins) than in the 486 patients with wide surgical margins (2.6% vs. 25.0%; P<0.0001). Twenty-nine of the 31 patients (94%) with local recurrence also had metastases and died of the tumor. In comparison with patients who only had a systemic relapse, patients with local recurrences had a higher rate of metastases located in bones (41% vs. 7%; P<0.001), and a worse post-relapse outcome (5-year overall survival: 6% vs. 24%; P<0.04). We concluded that in osteosarcoma of the extremity treated with neoadjuvant chemotherapy: i) limb salvages procedures do not compromise the outcome of patients, provided the achievement of adequate surgical margins; ii) local recurrences are a marker either of the inadequacy of local treatment or of the high local and systemic aggressiveness of the tumor.     

Dermatofibrosarcoma protuberans: review of 20-years experience

Introduction Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue neoplasm with low-intermediate grade of malignancy. It is a locally aggressive tumour with a high recurrence rate. Surgical excision with adequate margins is the main treatment. Materials and methods We describe the clinicopathological features of 21 cases of DFSP. The mean size of the lesions was 5.6 cm, mostly located in trunk. 61.9% of the cases underwent sugical excision without previous biopsy. 52.4% of the patients presented positive margins, that required surgical extension. Results The recurrence rate was 28.6% (6 cases), five of them local recurrences treated with new surgical excision with wide margins. Median period free of illness was 52.5 months. Medium follow-up period was 55.25 months. Conclusions DFSP has a locally high recurrence rate, that has been associated to inadequate surgical margins. The histological knowledge preoperatively would permit surgical excision with adequate margins, and probably this could reduce the recurrence rate. Radiotherapy could avoid the surgical extension of margins in these cases with positive ones. Chemotherapy could be indicated in metastasic cases. Most recurrences appear in the first 3 years, but it is important a long-term follow-up of these patients.     

Dermatofibrosarcoma protuberans: A clinicopathologic analysis of patients treated and followed at a single institution

BACKGROUND: Despite optimal surgical therapy for patients with dermatofibrosarcoma protuberans (DFSP), some patients still continue to develop local recurrence. The authors' objective was to identify and analyze clinicopathologic factors for disease free survival in a large group of patients who were followed prospectively at a single institution. METHODS: Prospectively collected data and pathology slides were available for review from 159 patients with primary or recurrent DFSP who underwent treatment between July 1950 and July 1998. The study group was comprised of patients with either the "classic" form of DFSP or the fibrosarcomatous "high grade" variant of DFSP (FS-DFSP). Patient, tumor, pathologic, and treatment factors were analyzed using the log rank test for univariate influence and Cox regression analysis for multivariate influence. Local recurrence free survival was determined by the Kaplan-Meier actuarial method. RESULTS: Of the 159 patients who comprised the current study group, 134 (84%) had the classic form of DFSP. The FS-DFSP variant was found in the remaining 25 patients (16%). The overall 5-year local recurrence free survival rate was 75%, with a median follow-up of 4. 75 years. The 5-year recurrence free survival rate for each group was 81% and 28%, respectively. On univariate analysis, age > 50 years, very close (< 1 mm) to positive microscopic margins, FS-DFSP variant, high mitotic rate, and increased cellularity were unfavorable prognostic factors. Multivariate analysis determined very close (< 1 mm) to positive microscopic margins and FS-DFSP variant to be independent adverse prognostic factors. For the 34 patients who developed a recurrence after surgical resection (21%), the median time to local recurrence was 32 months. Of the patients in this group, two died from metastatic disease. CONCLUSIONS: The prognosis after surgical resection with negative and sometimes positive microscopic margins for patients with DFSP is very good. However, increased age, high mitotic index, and increased cellularity are predictors of poor clinical outcome. The FS-DFSP variant represents a much more aggressive tumor with metastatic potential. Patients who are treated with curative intent for FS-DFSP should undergo aggressive attempts at complete surgical resection. Patients with recurrent classic DFSP without evidence of adverse prognostic features may benefit from conservative management, especially in the setting of potentially unresectable disease.     

骨盆软骨肉瘤的手术治疗

目的总结骨盆软骨肉瘤的手术切除、重建、并发症和疗效。方法回顾性分析手术治疗的21例骨盆软骨肉瘤患者的临床资料。其中男性9例,女性12例;年龄16~65岁,中位年龄46岁。根据Ennek ing骨盆肿瘤分区：Ⅰ区2例,Ⅰ和Ⅱ区5例,Ⅱ区6例,Ⅱ和Ⅲ区7例,Ⅲ区1例。高分化软骨肉瘤12例,中分化软骨肉瘤5例,低分化软骨肉瘤2例,间叶性软骨肉瘤2例。肿瘤刮除灭活＋异体骨植骨2例;髂骨翼大块切除＋钉棒系统重建1例;髂骨大块切除＋异体肱骨螺钉重建1例;耻骨切除自体髂骨移植钢板螺钉重建1例;半骨盆截肢2例;内半盆切除5例;围髋臼切除重建9例。结果21例患者随访时间7-100月,平均39月。17例患者无瘤生存,2例带瘤生存,2例死亡。16例行广泛切除术中5例局部复发,5例行边缘性和经病灶切除术中3例局部复发。软骨肉瘤的复发率为38.1%,死亡率为9.5%。19例生存患者参加功能评价,根据国际保肢学会功能评分系统,总分7~25分,平均19分。8例患者术后出现可控性并发症。结论骨盆软骨肉瘤治疗首选广泛性切除,积极预防和处理并发症是保证手术成功的关键。     

Dedifferentiated chondrosarcoma of the pelvis: clinical outcomes and current treatment

Background

Dedifferentiated chondrosarcomas (CS) are a high-grade variant of CS that confers a 5-year survival of around 10–24%. Dedifferentiated CS arising from the pelvis confers an even worse prognosis.

Questions

(1) What is the prognosis of patients with dedifferentiated CS of the pelvis? (2) Do wide margins or type of surgical intervention influence outcome? (3) Does the use of adjuvant therapy affect outcome?

Methods

Patients were retrospectively reviewed from a prospectively collated musculoskeletal oncology database from 1995 to 2016. Thirty-one cases of dedifferentiated CS arising from the pelvis were included. Wide margins were defined as greater than 4 mm. The mean age was 55.6 years (range 33 to 76 years) and there were 19 males (61.3%) and 12 females (38.7%).

Results

The disease presented at a locally or systemically advanced stage in 13 patients (41.9%). Eighteen patients (58.1%) underwent surgery with curative intent. Overall survival at 12 months was 15.4% for patients treated with palliative intent and 50% for those treated with surgery. In the surgical group, there were higher rates of disease-free survival in patients who underwent hindquarter amputation and those who received wide surgical margins (p?=?0.047 and p?=?0.019, respectively). Those who underwent hindquarter amputation were more likely to achieve wide margins (p?=?0.05). Time to recurrent disease (local or systemic) was always less than 24 months. No hindquarter amputation for recurrent disease resulted in disease-free survival. No patient who received adjuvant therapy for palliative or recurrent disease had disease control.

Conclusions

Pelvic dedifferentiated CS often presents at an advanced local or systemic stage and confers a poor prognosis. Achieving wide surgical margins (>?4 mm) provided the highest rate of long-term disease-free survival. Failing to achieve wide margins results in rapid disease recurrence, conferring deleterious consequences.

     

Radiotherapy in the management of giant cell tumor of bone

PURPOSE: To evaluate the outcomes of patients with giant cell tumor of bone (GCTB) treated with radiotherapy (RT) with or without surgical resection. METHODS AND MATERIALS: We performed a retrospective review of the records from 25 consecutive patients with pathologically confirmed GCTB who had undergone RT between 1956 and 2000. RESULTS: Patients ranged in age from 11 to 69 years (median 32); 16 were female and 9 were male. The anatomic distribution of lesions was as follows: cervical spine, 3; temporal bone, 1; thoracic or lumbar spine, 9; sacrum, 8; ilium, 1, and humerus, radius, and thumb metacarpal, 1 each. Tumors ranged in size from 2 to 20 cm (median 9.5) at their maximal dimension. Thirteen patients had been referred for RT for primary GCTB and 12 had been referred with locally recurrent disease after having undergone one or more other treatments. Fourteen patients had undergone RT for gross disease, and the remaining 11 had been treated with RT after gross total resection. In 10 of these 11 patients, the treatment margins were positive or uncertain. Radiation doses ranged from 25 to 65 Gy (median 46). At a median follow-up of 8.8 years (range 0.67-34), 7 patients had developed isolated local recurrence, 2 had developed isolated distant recurrence, and 3 had developed both. The actuarial 5-year overall and disease-free survival rate was 91% and 58%, respectively, and the actuarial 5-year local control and distant metastasis-free survival rate was 62% and 81%, respectively. Univariate analysis suggested that treatment for recurrent disease correlated with a lower disease-free survival rate (83% vs. 33%, p = 0.06), distant metastasis-free survival rate (100% vs. 64%, p = 0.08), and local control rate (83% vs. 42%, p = 0.08) at 5 years. Of the 12 cases of recurrence, 7 were ultimately successfully treated with additional salvage therapy. In 4 of these patients, salvage therapy included interferon-alpha 2b. CONCLUSION: RT should be considered an adjuvant to surgery or as alternative therapy in cases of GCTB that are unresectable or in which excision would result in substantial functional deficits. When RT is used as primary therapy, the rate of local control seems to be satisfactory. In heavily pretreated patients, however, RT delivered as it was in this series can result in poor local control, and alternative therapies should be considered.     

Surgical management of soft tissue sarcomas of the hand and foot

BACKGROUND: Soft tissue sarcomas of the hand and foot present unique management challenges. The purpose of the current study study was to determine oncologic outcome, particularly with respect to factors affecting local recurrence, distant recurrence, and disease-specific survival. METHODS: A retrospective study was performed on 115 patients with soft tissue sarcomas of the hand or foot who were evaluated, treated, and followed at the authors' institution between 1980 and 1998. The medical records and radiographs were reviewed. Kaplan-Meier analysis was used to assess patient survival. RESULTS: Most patients (95%) were referred after previous surgery. The majority of tumors (75%) were T1 lesions (less than 5 cm), and most tumors (81%) were high grade. Patients who were treated by definitive, wide re-excision (n = 43) had a 10 year local recurrence-free survival of 88%, which was significantly better than the corresponding rate of 58% for patients who did not have re-excision (n = 40, P = 0.05). Radiation improved local control in patients who did not undergo re-excision (n = 17, P = 0.02). However, radiation did not improve local control in patients who had definitive re-excision with negative margins (n = 13, P = 0.51). The disease-specific survival at 5 and 10 years was 76% and 65%, respectively, for patients who presented with localized disease. Disease-specific patient survival was significantly worse for patients who had regional or distant metastasis. Radical amputation as initial surgical treatment did not decrease the likelihood of regional metastasis and did not improve disease-specific patient survival. The presence of distant metastasis at presentation was an independent predictor of local recurrence. CONCLUSION: Limb sparing treatment is possible in many patients with soft tissue sarcomas of the hand and foot. Re-excision to achieve microscopically negative surgical margins is an effective method of achieving a high rate of local control in appropriately selected patients who present after unplanned excision of the primary tumor. There does not appear to be a survival benefit to immediate radical amputation, which should be reserved for cases where surgical excision or re-excision with adequate margins cannot be performed without sacrifice of functionally significant neurovascular or osseous structures.     

Rb-loss is associated with high malignancy in chondrosarcoma

Loss of function of the human retinoblastoma gene (Rb) is a frequent genetic abnormality in human malignancies and causes a disturbance in the cell cycle and loss of normal proliferation and differentiation. We studied the loss of heterozygosity (LOH) of the Rb gene in 31 formalin-fixed, paraffin-embedded cartilaginous tumors using polymerase chain reaction. The tumors were subdivided into 8 cases of dedifferentiated (DD) chondrosarcoma, 17 cases of conventional chondrosarcoma (nine grade 1, seven grade 2 and one grade 3), 4 enchondromas and 2 chondroblastomas. Both components of DD chondrosarcoma, the low-grade and anaplastic components, were separated by a microdissection approach. The genetic data were correlated with the expression of the Rb protein examined by Rb immunohistochemistry. We found Rb-LOH in one grade 3 chondrosarcoma, and in the anaplastic component in 7 of 8 cases of DD chondrosarcoma (89% of all high-grade chondrosarcomas). All tumors with Rb-LOH were immunohistochemically Rb-negative. The only case of DD chondrosarcoma negative for Rb-LOH in both components of the tumor also showed weak expression of the Rb protein in the anaplastic component. All benign cartilaginous tumors, low-grade chondrosarcomas and low-grade tumor components of DD chondrosarcomas were negative regarding Rb-LOH but positive in Rb immunohistostaining. We concluded that Rb-LOH predominantly occurs in high-grade chondrosarcomas. However, it is not a marker for identifying low-grade tumors with a tendency towards progression or local recurrence.     

Impact of surgical and pathologic variables in rectal cancer: a United States community and cooperative group report.

PURPOSE: Substantial and successful effort has been focused on decreasing the risk of local failure after rectal cancer surgery through the use of adjuvant therapies. Our study examined data from studies conducted by United States cooperative groups to investigate the impact of surgical and pathologic variables in rectal cancer outcomes. PATIENTS AND METHODS: Surgical and pathologic reports from 673 patients with stage II/III rectal cancer enrolled onto three adjuvant clinical trials were reviewed for tumor and surgical variables. Additional information on individual institutions and operating surgeon was collected. Variables were tested for association with 5-year local recurrence and survival after adjustment for adjuvant treatments and other important prognostic factors. RESULTS: Five-year local recurrence and survival rates were 16% and 59%, respectively. Surgeons treating more than 10 study cases had lower local recurrence rates than those treating < or = 10 (11% v 17%, P =.02). Free radial margins also correlated with local recurrence (P =.01). Type of surgery, distal margins, and tumor radial spread were not significant. Tumor adherence to adjacent structures predicted local recurrence (35% v 14%, P <.001) and survival (30% v 63%, P <.001), regardless of en bloc resection. Although T and N classification predicted survival (P <.001), only N classification correlated with local recurrence. The number and percentage of positive nodes correlated with survival, but only the percentage independently predicted local recurrence. Several pathologic and surgical variables were reported suboptimally. CONCLUSION: Moderate variability in outcomes among surgeons was detected in this high-risk population. Efforts to improve surgical results will require changes in reporting practices to allow for more accurate assessment of the quality of surgery.     

Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution.

PURPOSE: To explore prognostic factors in surgically treated aggressive fibromatosis (extra-abdominal desmoid tumor). PATIENTS AND METHODS: A total of 203 consecutive patients treated with surgery over a 35-year period at a single referral center were retrospectively reviewed. One hundred twenty-eight were first seen at our institution with primary disease, whereas 75 had a recurrent tumor. All patients underwent macroscopically complete resection. Margins were rated as negative in 146 (97 with primary tumors, 49 with recurrences) and positive in 57 (31 in primary, 26 in recurrences) patients. Median follow-up was 135 months. RESULTS: Patients with primary disease had a better disease-free survival rate than those with recurrence (76% v 59% at 10 years). Presenting with a recurrence was also the strongest predictor of local failure in the multivariate analysis. In patients first treated for primary disease, size and site had prognostic significance, whereas microscopically positive surgical margins did not. In contrast, in patients with recurrence, there was a trend toward better prognosis if margins were negative (although this was not significant at multivariate analysis). CONCLUSION: Presence of microscopic disease does not necessarily affect long-term disease-free survival in patients with primary presentation of extra-abdominal desmoid tumors. Thus, function-sparing surgery may be a reasonable choice when feasible without leaving macroscopic residual disease. In patients with recurrences, positive margins may more clearly affect prognosis, potentially necessitating adjuvant radiation in selected cases.     

Influence of radiation dose on positive surgical margins in women undergoing breast conservation therapy

PURPOSE: Positive surgical margins adversely influence local tumor control in breast conservation therapy (BCT). However, reports have conflicted regarding whether an increased radiation dose can overcome this poor prognostic factor. In this study, we evaluated the influence of an increased radiation dose on tumor control in women with positive surgical margins undergoing BCT.METHODS AND MATERIALS: Between 1978 and 1994, 733 women with pathologic Stage I-II breast cancer and known surgical margin status were treated at Thomas Jefferson University Hospital with BCT. Of these 733 patients, 641 women had a minimal tumor bed dose of 60 Gy and had documentation of their margin status; 509 had negative surgical margins, and 132 had positive surgical margins before definitive radiotherapy. Complete gross excision of the tumor and axillary lymph node sampling was obtained in all patients. The median radiation dose to the primary site was 65.0 Gy (range 60-76). Of the women with positive margins (n = 132), the influence of higher doses of radiotherapy was evaluated. The median follow-up time was 52 months.RESULTS: The local tumor control rate for patients with negative margins at 5 and 10 years was 94% and 88%, respectively, compared with 85% and 67%, respectively, for those women with positive margins (p = 0.001). The disease-free survival rate for the negative margin group at 5 and 10 years was 91% and 82%, respectively, compared with 76% and 71%, respectively, for the positive margin group (p = 0.001). The overall survival rate of women with negative margins at 5 and 10 years was 95% and 90%, respectively. By comparison, for women with positive surgical margins, the overall survival rate at 5 and 10 years was 86% and 79%, respectively (p = 0.008). A comparison of the positive and negative margin groups revealed that an increased radiation dose (whether entered as a dichotomous or a continuous variable) >65.0 Gy did not improve local tumor control (p = 0.776). On Cox multivariate analysis, margin status and menopausal status had prognostic significance for local tumor control and DFS.CONCLUSION: Patients with positive surgical margins have a higher risk of local tumor recurrence and worse survival when undergoing BCT. Higher doses of radiation are unable to provide an adequate level of local control in patients with positive margins.