Childhood soft tissue sarcoma: a 20-year experience

PURPOSE: To evaluate the disease-free and overall survival of pediatric patients with nonrhabdomyosarcoma soft-tissue sarcomas. METHODS: We retrospectively analyzed the records of 67 pediatric patients with a diagnosis of nonrhabdomyosarcoma soft tissue sarcoma treated with curative intent between 1970 and 1992. Median follow-up time for the 52 survivors was 120 months (range, 7 to 277 months). Fifty-nine patients received external beam radiotherapy, in a median dose of 5400 cGy (range, 1800 to 6660 cGy.) All patients underwent an initial surgical procedure. Eighteen patients had gross residual disease, and 15 had gross total excision with microscopic residual disease or positive margins. Adjuvant chemotherapy was administered to 44 patients (65%). RESULTS: The actuarial 10-year freedom from progression or recurrence and overall survival rates were 76% and 75%, and the 20-year rates were the same. Of 18 patients with gross residual disease, 9 (50%) had local progression and 6 died of local-only disease. By contrast, only one patient with microscopic residual disease who received postoperative radiotherapy had a local recurrence. The disease-free survival rate also correlated with histologic grade. CONCLUSIONS: As with adult soft tissue sarcomas, gross residual disease predicts local failure. Our results suggest that pediatric patients with soft tissue sarcomas treated with surgery and postoperative radiotherapy generally have a favorable overall survival rate.     

Functioning muscle transplantation after wide excision of sarcomas in the extremity

Free functioning muscle transplantation was performed after resection of 23 sarcomas in the extremity. There were 21 soft tissue sarcomas and two malignant bone tumors. The tumor resection was performed with a wide margin in all except two patients who had a marginal margin in a limited area. The consequent extensive soft tissue defect received free musculocutaneous flaps, the motor nerve of which was repaired in the recipient site. The most frequent procedure was latissimus dorsi transplantation to replace thigh muscles in 17 cases. The other donors included gracilis, tensor fascia lata, and rectus femoris, which were selected according to the site of defects. Patients were followed up for a mean of 60 months (range, 13-119 months). The grafted muscles showed reinnervation at a mean of 6 months postoperatively in all patients except for a 75-year-old patient. Obtained contraction of the muscles was powerful in 18 patients and fair in four patients. Performance of the salvaged limb significantly improved after recovery of the muscles. Although there were five distant recurrences, local recurrence was seen in one patient with systemic metastases. Because muscle loss could be compensated functionally for by the innervated free muscle transfer, the method encouraged surgeons to perform more radical tumor excisions and this may have contributed to the excellent local tumor control that was achieved. Thus, functioning muscle transplantation was extremely useful in limb salvage surgery from the functional and oncologic viewpoints.     

Extremity soft tissue sarcoma with metastases to abdominopelvic surfaces

BACKGROUND: In a majority of patients with extremity soft tissue sarcoma, the lungs are the first site at which recurrent disease is detected. Other common sites of recurrence are the resection site, bone metastasis, and liver metastasis. Although abdomino pelvic sarcomatosis is common as a site of recurrence for visceral and retroperitoneal sarcoma, it has not been previously reported for extremity sarcoma. METHOD: We present three patients in whom extremity soft tissue sarcoma metastasized to the peritoneal surfaces, and in all three patients this was symptomatically the dominant site for recurrence. All of them underwent a palliative surgical cytoreduction and were then treated with intraperitoneal chemotherapy. RESULTS: In two of the three patients, isolated progression of peritoneal sarcomatosis has again occurred: one patient remains disease free at 14 months. CONCLUSIONS: Metastasis to peritoneal surfaces within the abdomen and pelvis must be considered a possible site for systemic dissemination of extremity soft tissue sarcomas. Effective treatments for sarcoma spread to peritoneal surfaces would benefit this small but symptomatic group of patients.     

Recovered memory therapy: a dubious practice technique

BACKGROUND: Adjuvant chemotherapy and endoprosthetic replacement for bone sarcomas of the lower extremity is well established. The specific long-term consequences of these endoprosthetic reconstructions for the patient's affected limb are unknown. METHOD: The oncologic results and the survival of the endoprostheses were reviewed in 32 patients with primary bone sarcoma of the femur or proximal tibia. There were 26 high-grade sarcomas, and 6 low-grade sarcomas. A proximal femoral endoprosthesis was used for reconstruction in 4 patients, a total or push-through femoral endoprosthesis in 11 patients, a distal femoral endoprosthesis in 15 patients, and a proximal tibial endoprosthesis in two patients. RESULTS: Median survival was 10 years (range, 1.1 to 18.9 years) for patients with high-grade sarcoma, and 8.1 years (range, 7.1 to 10 years) for patients with low-grade sarcomas. Distant metastases developed in seven patients (22%), all with stage IIB sarcoma, with concomitant local recurrence in 3 patients (9%). Five-year overall and disease-free survival rates for high-grade sarcomas were 81% and 73%, respectively. The overall endoprosthetic survival rate was 87% at 5 years, 80% at 10 years, and 56% at 15 years. Median follow-up of the original endoprostheses was 8.3 years (range, 0.6 to 18.7 years). Endoprosthesis-related complications occurred in 13 patients (41%); most complications were mechanical failures. The highest complication rate was found in distal femoral replacements (60%); amputation was necessary in both patients treated with a proximal tibial endoprosthesis. Five endoprostheses (16%) were revised. An amputation of the involved limb was performed in four patients (13%): in two patients because of local recurrence and in the other two patients because of infection. For patients alive at follow-up, the median functional Enneking evaluation score was 22 points (range, 12 to 28 points), with the highest functional scores in patients with a distal femoral endoprosthesis, and the lowest functional scores in patients with total or push-through femoral replacements. CONCLUSION: Endoprosthetic reconstructions gave satisfying functional results in most patients after long-term survival. However, the proximal tibial and distal femoral endoprosthesis are particularly at risk for long-term endoprosthetic complications requiring additional surgical procedures.     

Soft tissue sarcomas

Soft tissue sarcomas are relatively rare tumors with an annual incidence of 5000 to 6000 in the United States. The primary therapy is surgical resection with an adequate margin of normal tissue. For patients at high risk local control is improved with postoperative adjuvant radiation. Local recurrence rates vary depending on the anatomic site. In extremity lesions one third of patients will have locally recurrent disease with a median disease-free interval of 18 months. Treatment results for extremity local recurrence may approach those for primary disease. Isolated pulmonary metastases may be resected with 20% to 30% 3-year survival rates. Patients with sarcomas in other sites present similar but more difficult problems in terms of local control and management of disseminated disease. Patients with unresectable pulmonary metastases or extrapulmonary metastatic sarcoma have a uniformly poor prognosis and are best treated with systemic chemotherapy.     

Surgical aspects in the multidisciplinary treatment of soft tissue sarcomas

Sarcomas are rare malignant tumors with a large variety of histologic subtypes. The surgical approach depends more on the histologic grade, the size and the site of the tumor. Radiologic diagnosis relies predominantly on MR-imaging. Discernible improvements have taken place in soft tissue sarcoma patient survivorship and quality of life over the past 20 years, with overall 5-year survival currently at approximately 50-80%. The place of surgery in the treatment of soft-tissue sarcoma is defined in the light of a review of the recent literature. Radical surgical resection is the mainstay of therapy. Local recurrence is the most common type of failure. Local recurrence is resectable and limb preservations possible in the majority of patients. Survival after treatment of local recurrence is determined mainly by the grade and secondarily by the size of the tumor. The essential risk factor for local recurrence is the quality of surgical resection, defined by the definitive resection margins. A lateral safety margin of 5 cm and of 2 cm to the depth should be respected. In sarcoma of the extremity the compartment is defined based on clinical, radiographic, histopathologic and operative findings. The use of muscle flaps to fill the surgical defects can improve the functional result and reduce the complication rate. Only about 5% of the patients need amputation. Evaluation of functional results must be based on objective criteria. In retroperitoneal sarcoma the significant factors for determining prognosis are grade and completeness of exzision. Multidisciplinary treatment according to common protocols is essential. Shifts in treatment have taken place over the past decade, from single-modality treatment involving radical surgery with compartment resection to sophisticated limb-salvage strategies combined with radiation therapy. In case of inadequate surgery e.g. in a large tumor with positive margins in high-grade soft tissue sarcomas the addition of radiotherapy can improve local control, but cannot ensure that obtained by adequate surgery. Patients with large (greater than 5 cm), high grade soft tissue sarcoma are at high risk for distant recurrence and disease-related mortality. Investigations of combined modality therapy with newer chemotherapy agents and dose intensification treatment strategies are warranted.     

Spontaneous arterial thrombosis in association with pancreatic carcinoma: diagnosis and interventional management

BACKGROUND: Soft tissue sarcomas of the hands and feet present a challenge for limb-preserving resections. METHODS: A retrospective review of 19 patients with sarcomas of the hand or foot was done. Wide or local excision was performed in 14 patients (74%), and amputation in 5 patients (26%). Of the latter group, three amputations involved a digit or toe, and two (10%) were major amputations (one Syme amputation and one below-knee amputation). When the minimum surgical margin was narrow (1 to 2 mm), adjuvant radiation was given postoperatively (n = 4). RESULTS: Local recurrence was observed in four patients (21%). Two of these required an amputation for local control. Local recurrence was observed in one of four patients (25%) treated with marginal resection and radiation and three of 15 (20%) of those with resection alone. CONCLUSIONS: A sizable percentage (37%) of patients with soft tissue sarcomas of the hand and foot ultimately required an amputation, although often the amputation was a minor one involving only a toe or a digit. Limb preservation was successful in the majority of patients (63%). The local recurrence rate was 21%, which may be improved with more frequent use of adjuvant therapy. The 5-year survival rate was 82%, which is better than that usually quoted for overall extremity soft tissue sarcomas.     

Effect of indomethacin and prostaglandin on the smooth muscle contracting activity of angiotensin and other agonists

The results of palliative chemotherapy applied to 123 patients with disseminated soft tissue sarcomas are reviewed with the recent literature data. With the exception of embryonal rhabdomyosarcomas of children, which are considered separately, the response of these tumors to chemotherapy is poor but not negligible. The association of several active drugs should permit substantial progress, especially in the initial stage of local treatment, when the number of disseminated cells is still reduced. A follow-up of controlled clinical studies must specify the modalities of multidisciplinary treatment in an effort to reduce regional recurrence or metastasis.     

Adjuvant radiotherapy and chemotherapy for stage II or IIIA non-small-cell lung cancer after complete resection. Provincial Lung Cancer Disease Site Group

GUIDELINE QUESTIONS: 1) Does the use of postoperative, adjuvant radiotherapy or chemotherapy, alone or in combination, improve survival rates among patients with completely resected, pathologically confirmed stage II or IIIA non-small-cell lung cancer (NSCLC)? 2) Does the use of radiotherapy reduce the risk of local recurrence among patients with completely resected stage II or IIIA NSCLC? OBJECTIVE: To make recommendations about the use of postoperative adjuvant radiotherapy and chemotherapy in the treatment of patients with completely resected stage II or IIIA NSCLC. OUTCOMES: Overall survival and disease-free survival are the primary outcomes of interest. A secondary outcome of interest is local disease control. PERSPECTIVES (VALUES): Evidence was collected and reviewed by 4 members of the Lung Cancer Disease Site Group (Lung Cancer DSG) of the Cancer Care Ontario Practice Guidelines Initiative. The evidence-based recommendation resulting from this review was approved by the Lung Cancer DSG, which comprises medical oncologists, radiation oncologists, pathologists, surgeons and a medical sociologist. A community representative was present at 1 meeting during which the recommendation was discussed. QUALITY OF EVIDENCE: One meta-analysis and 22 randomized controlled trials (RCTs) were published between 1962 and 1996. The RCTs compared surgery plus radiotherapy with surgery alone; surgery plus adjuvant chemotherapy with surgery alone; surgery plus radiotherapy with surgery plus both chemotherapy and radiotherapy. Many studies included patients with stage IIIB NSCLC; some included patients with incompletely resected stage I NSCLC or with small cell lung cancer (maximum 10%). Older studies used chemotherapy or radiation that would now be considered inferior according to current standards of practice. BENEFITS: There was no survival benefit with adjuvant radiotherapy alone, although 3 RCTs reported a reduction in the rate of local recurrence among patients treated with adjuvant radiotherapy. The meta-analysis showed that postoperative, cisplatin-based chemotherapy alone reduced the relative risk of death by 13% (hazard ratio [HR] 0.87, 95% confidence interval [CI] 0.74 to 1.02); in combination with radiotherapy it resulted in a 6% reduction in the relative risk of death (HR 0.94, 95% CI 0.79 to 1.11). HARMS: Postoperative adjuvant chemotherapy with alkylating agents was found in the meta-analysis to increase the relative risk of death by 15%. A study involving prolonged adjuvant chemotherapy (busulfan or cytoxan daily for 2 years) reported that 4 of 726 patients had hematologic malignancies. In 1 study, only 53% of patients received all 4 cycles of chemotherapy with cyclophosphamide-doxorubicin-cisplatin (CAP); in another, 22% of patients refused therapy with CAP because of nausea and vomiting. PRACTICE GUIDELINE: There is evidence from RCTs that postoperative radiotherapy reduces rates of local recurrence by 11% to 18% (or 1.6 to 19-fold) among patients with completely resected, pathologically confirmed stage II or IIIA NSCLC. Therefore, if the outcome of interest is a reduction in the frequency of local tumour recurrence, radiotherapy is recommended. However, there is no evidence of a survival benefit from postoperative radiotherapy alone. In a meta-analysis, postoperative chemotherapy with or without radiotherapy resulted in a slightly reduced (statistically nonsignificant) risk of death among patients with surgically resected stage II or IIIA NSCLC. The survival benefit was small and achieved only with chemotherapy regimens that produced substantial toxic effects and that are no longer used. Newer chemotherapy regimens are currently being evaluated as adjuvant therapy, but there is insufficient evidence of benefit at this time to recommend them. Therefore, if the outcome of interest is survival, there is insufficient evidence to recommend current chemotherapy regimens with or without radiotherapy as postoperative, adjuvant the     

Resectability of retroperitoneal sarcomas: a matter of surgical technique?

The management of retroperitoneal sarcomas has been hampered by the difficulty in complete resection, the resectability rate in the literature being about 53%. In a review of the last 88 consecutive patients with retroperitoneal sarcomas the resectability rate was 95%. At a mean follow-up of 48 months, the local recurrence rate was 17% following wide resection and 59% following local excision (P = 0.0002). For patients with minimum follow-up of 5 years, the local recurrence rate was 39% for those with primary tumours and 57% for those referred with local recurrence. Local recurrence diminished the rate of long-term survival. The 5- and 10-year survival rates for the primary retroperitoneal sarcomas (n = 55) were 66% and 57% and for those referred with locally recurrent sarcoma (n = 33) 57% and 26%, respectively. The 5-year survival rate varied significantly with the grade of the tumour, from 88% for Grade I to 44% for Grade III tumours (P = 0.006). In conclusion, with modern surgical techniques the resectability rate of retroperitoneal sarcomas is about 95%, and the survival rate of the primary tumours approximates that of the primary soft tissue sarcomas of the extremities.     

Soft tissue tumours

Any soft tissue swelling beneath the deep fascia should be considered a sarcoma until proven otherwise. As the most important factor in the primary treatment of these cancers is the adequacy of the primary surgical resection, it is vital to diagnose these malignant tumours pre-operatively. The modern treatment of soft tissue sarcomas may involve all modalities, but the most important aspect of treatment of a primary localised sarcoma is wide excisional surgery preserving limb function. Radiotherapy is a vital adjunct in high-grade tumours, or in tumours whose resectability is limited either by size or anatomical proximity to vital structures. Apart from a few chemosensitive sarcomas, the role of chemotherapy is limited to treatment of metastatic disease where documented response rates are no greater than 30%. As 50% of patients with high-grade sarcomas will die from metastatic disease, improvements in survival rates will only come from improvements in response to systemic therapy. No controlled trials have shown any survival benefit for adjuvant chemotherapy, although a recent meta-analysis of published data has shown a trend to increased survival at two years. Multicentre randomised trials are ongoing. The prognosis of these lesions is highly variable, but is intimately related to the anatomical site (i.e., resectability), and also the grade and size of the tumour.     

Treatment of chondrosarcoma. A retrospective study at the Norwegian Radium Hospital

The authors present a retrospective analysis of 59 chondrosarcoma patients treated at the Norwegian Radium Hospital during the period 1981 to 1993. 31 patients were admitted with untouched tumour, seven after fine needle cytology and 20 after open biopsy or partial excision. One patient had recurrent local disease. Only 20% of the tumours were of high grade malignancy. 51 patients were treated by surgery. Reconstructions were performed in 16 patients, using allografts or endoprostheses. Amputations were performed in six cases and wide excision in 12 cases. In these 18 patients local recurrence appeared in one case, and two developed lung metastases. Only one of the 18 patients operated by amputation or wide excision has since died from chondrosarcoma. Marginal excisions were performed in 26 cases. Nine of these patients developed a local recurrence, five developed metastases and three have died. Six patients had partial excisions. Postoperative radiotherapy was given to one patient only. Five of the six are alive. In one case, the quality of the margins could not be evaluated. A total of 45 of the 51 patients treated for the primary tumour by surgery are alive. The median observation time is four years. Treatment of nonmetastatic chondrosarcoma should be surgical. Chondrosarcoma patients show wider variations in age, localization of tumour and tumour growth rate than patients with other bone sarcomas. Although wide excisions provide the best local control of any grade of malignancy, the mutilation or risk involved may be so great that some patients may benefit from marginal or even partial excision.     

Radiotherapy in the treatment of solitary plasmacytoma

Solitary plasmacytoma of bone (SPB) and extramedullary plasmacytoma (EMP) are rare. High local control rates are reported with radiotherapy, although the optimal dose and extent of radiotherapy portals remains controversial. Between 1983 and 1993, 30 patients with solitary plasmacytoma were seen at the Regional Cancer Centre, Trivandrum, India. 23 patients had SPB and seven EMP. The mean age was 52 years and the male to female ratio 3.2:1. Diagnosis of SPB was confirmed by biopsy in 16 patients and tumour excision in seven. 20 patients received megavoltage radiotherapy to the bone lesion with limited margins, and one received chemotherapy. Two patients who underwent complete tumour excision received no further treatment. All seven patients with EMP received megavoltage radiotherapy, four following biopsy and three after tumour excision. Local control was achieved in all patients with SPB. Nine progressed to multiple myeloma and one developed a solitary plasmacytoma in another bone. Six patients with EMP achieved local control. Three later progressed to multiple myeloma and one had local relapse. Median time to relapse was 28 months in SPB and 30 months in EMP. 5-year overall survival rates were 82% and 57% for patients with SPB and EMP, respectively. The corresponding progression free survival rates were 55% and 50%, respectively. Age, sex, site of tumour, serum M protein and haemoglobin levels did not significantly influence progression free survival. The extent of surgery, radiotherapy dose or time to relapse were not significant prognostic factors. Radiotherapy appears to be an effective modality of treatment of solitary plasmacytoma. No dose-response relationship is observed, and high local control rates are achieved with limited portals. Progression to multiple myeloma is the commonest pattern of failure, although no prognostic factors for progression are identified. The role of chemotherapy in preventing disease progression needs further evaluation.     

Placental transfer and maternally acquired neonatal IgG immunity in human immunodeficiency virus infection

PURPOSE: The effect of the sensitizer razoxane on soft tissue sarcomas (STS) was prospectively evaluated in a randomized, controlled trial. The main purpose of the study was to determine the response rates and local control under the combined treatment compared to irradiation alone. METHODS AND MATERIALS: Between 1978 and 1988, 144 patients entered the study; 130 were evaluable for response, toxicity, or survival. The patients were randomized to receive radiotherapy alone or radiotherapy with razoxane. They were divided into postoperative cases and patients with gross disease (unresectable primaries, recurrent disease, or metastatic disease). The median radiation dose was 60 Gy postoperatively, and 56-58 Gy in patients with gross disease. The dose difference has palliative reasons. Razoxane was given orally at a daily dose of 150 mg/m2 during the time of the radiotherapy, starting 5 days before the first irradiation. In general, the groups were comparable as to their prognostic factors. There was some imbalance, however, in favor of the postoperative group reveiving radiotherapy alone. RESULTS: Between the patient groups treated postoperatively in an adjuvant form, there were no substantial differences in local control and survival. Among 82 patients with gross disease, the treatment with radiotherapy and razoxane led to an increased response rate compared to photon irradiation alone (74 vs. 49%). The local control rate was likewise improved (64 vs. 30%;p < 0.05). The acute toxicity was somewhat higher in the sensitizer arm, but there was no difference in the occurrence of late complications. CONCLUSIONS: Radiotherapy combined with razoxane seems to improve the local control in inoperable, residual, or recurrent STS compared to radiotherapy alone. The combined treatment is a fairly well tolerated procedure at low costs. It can be recommended for inoperable primary STS or gross disease after incomplete resection, conditions which are still associated with limited local control and a grave prognosis.     

Tumour bed positivity predicts outcome after breast-conserving surgery

BACKGROUND: Local recurrence after breast-conserving surgery is associated with a short distant disease-free survival, particularly if it occurs early. Early recurrence is caused by residual disease left at the time of surgery. Previous studies have demonstrated that disease in the tumour bed is a common finding after breast-conserving surgery. METHODS: The follow-up (mean 4.4 years) of 300 patients who had tumour bed analysis performed by the cavity shaving technique following breast-conserving surgery is presented. Postoperative radiotherapy was administered to all patients. RESULTS: The incidence of tumour bed positivity was 39.3 per cent. With a selective re-excision policy the local recurrence rate was 2.0 per cent and distant recurrence rate 10.4 per cent. Multivariate analysis identified lymphovascular invasion, oestrogen receptor status and tumour bed status as independent predictors of time to distant recurrence. CONCLUSION: A low rate of local recurrence can be achieved using this technique of margin assessment. Tumour bed status may be a useful prognostic factor following breast-conserving surgery.     

Malignant vascular tumors in young patients

BACKGROUND: To the authors' knowledge there are few published series of malignant vascular tumors in patients age < or = 21 years. METHODS: The authors retrospectively documented the clinical presentation, pathology, treatment, and outcome of patients age < or = 21 years with malignant vascular tumors treated between 1970-1995 at Memorial Sloan-Kettering Cancer Center. The histologic sections were rereviewed to confirm the diagnosis. RESULTS: Four patients were identified with angiosarcoma and two with malignant hemangioendothelioma. Five patients were female and one was male; the median age at diagnosis was 11.8 years (range, 8 months-21 years). The tumor involved the skin in one patient, soft tissue in one patient, bone in two patients, and internal organs in two patients. One patient had associated diffuse angiomatosis and another had the Klippel-Trenaunay-Weber syndrome. This patient received prior radiation therapy to the primary site with the subsequent development of a vascular sarcoma. None of the patients had distant metastases at diagnosis. Resection was attempted in five patients and completed in four. Chemotherapy alone was given to three patients whereas radiation therapy alone and radiation plus chemotherapy were administered to one patient each. The median follow-up was 4.9 years (range, 1 month-12 years). There were three deaths from progressive disease and two deaths from locoregional recurrences. Reexcision and radiotherapy controlled one local recurrence. Another patient developed recurrence to regional lymph nodes and further dissemination. The primary tumor in all three survivors was excised completely whereas two of the three patients who died of progressive disease underwent an incomplete excision or biopsy alone. CONCLUSIONS: Malignant vascular tumors are rare in the first two decades of life and when they do occur are very aggressive. Complete resection is curative for patients with localized lesions.     

Effectiveness of mastectomy by response to induction chemotherapy for control in inflammatory breast carcinoma

BACKGROUND: Controversy exists as to the treatment regimen necessary to best provide optimal local control for inflammatory breast carcinoma (IBC). This study was conducted to determine if mastectomy combined with radiotherapy offered any advantages over radiotherapy alone in patients with IBC who had been treated with doxorubicin-based combination chemotherapy. METHODS: A retrospective review of 178 women treated for IBC on doxorubicin-based multimodality therapy protocols between January 1974 and September 1993 was performed. Clinical and histologic response to treatment, time to local recurrence, survival, and ultimate control of local disease were analyzed. Kaplan-Meier analysis was used to examine survival and relapse times, and Fisher's exact test was used to test differences in treatment outcomes. Significance was determined at p < or = 0.05. RESULTS: Median follow-up was 89 months (range 22 to 223 months). Locoregional disease persisted in seven patients and recurred in 44 patients who had been rendered disease free at a median time of 10 months. The mortality rate after a local recurrence (LR) was 98%, and all patients but one with LR developed systemic metastases. Response to induction chemotherapy influenced the incidence of LR, and the amount of residual disease found on histologic examination of mastectomy specimens was highly prognostic for local failure. Patients who underwent mastectomy in addition to radiotherapy had a lower incidence of LR than did patients who received radiotherapy alone (16.3% vs. 35.7%, p = 0.015). CONCLUSIONS: The addition of mastectomy to combination chemotherapy plus radiotherapy improved local control in patients with IBC. The addition of mastectomy to chemotherapy plus radiotherapy improved distant disease-free and overall survival in patients with a clinical complete or partial response to induction chemotherapy. Patients who had no significant response to induction chemotherapy received no survival or local disease-control benefit from the addition of mastectomy to their treatment regimen. These patients should be considered for entry into clinical trials of new treatment regimens.     

Bronchopulmonary atypical carcinoid tumour metastatic to the orbit

BACKGROUND: Bronchopulmonary carcinoid tumours metastatic to the orbit are rare. A case is presented demonstrating presentation, histopathology and treatment. METHODS: A 64-year-old man with a history of bronchopulmonary atypical carcinoid presented with an orbital mass. The nature of this metastasis was confirmed with histology and it was surgically excised and, in addition, local radiotherapy and chemotherapy were administered. RESULTS: There was a good response to the treatment for the orbital mass but other metastases developed, causing death. CONCLUSIONS: In this case of atypical carcinoid, orbital metastasis was the first sign of disease progression. Histological confirmation of the diagnosis was important in this case to allow planning of oncological therapy and for appropriate patient counselling. A good local response was obtained with a combination of surgical excision, local radiotherapy and chemotherapy. The systemic prognosis remains poor in cases of metastatic bronchopulmonary atypical carcinoid tumour, in contrast to typical carcinoid.     

Regional hyperthermic perfusion with cisplatin following surgery for malignant melanoma of the extremities

BACKGROUND: The study was undertaken to review our experience in the treatment of extremity melanoma with hyperthermic isolated limb perfusion (HILP), using cisplatin as the chemotherapeutic agent. We also evaluated the best timing for regional lymph node dissection in relation to the perfusion. PATIENTS AND METHODS: Sixty patients with advanced malignant melanoma of the limbs were treated with HILP used mainly as an adjuvant treatment. There were 56 lower- and 4 upper-limb HILPs. Cisplatin was used at a dose of 20 mg/L of limb volume. Temperature at the tumor site was 39 degrees C to 40 degrees C. Postoperative complications, disease-free period, and time to recurrence were recorded. RESULTS: There were no deaths related to the procedure. Forty-seven percent of the patients developed local complications; most complications were minor and resolved within 60 days. The local complication rate was higher when HILP was performed shortly after or simultaneously with regional lymph node dissection. None of the patients had systemic complications. Mean survival time from treatment was 87.2 months. Currently, 35 patients (58%) are alive and free of disease 52.7 +/- 22.5 months after HILP. Twelve patients (20%) are alive with recurrent disease, of which 5 recurred locally. The average time (+/- standard error of the mean) to recurrence was 24.5 +/- 13.8 months after perfusion. CONCLUSIONS: HILP with cisplatin is a relatively safe procedure, which seems to increase locoregional control of advanced malignant melanoma of the extremity. Separating the timing of lymph node dissection from HILP by 6 to 8 weeks reduces the complication rate.     

Role of surgery in the treatment of brain metastases in patients with breast cancer

BACKGROUND: Patients whose brain metastases from breast cancer are treated nonsurgically have a median length of survival ranging from 2.5 to 7.5 months, and a median time to recurrence ranging from 2 to 5 months. Patients treated with radiotherapy have a median length of survival ranging from 3 to 4 months. Those treated with chemotherapy have a median length of survival ranging from 5.5 to 7.5 months. METHODS: We conducted a retrospective analysis on 63 patients treated over a 10-year period. Only patients who underwent surgery for nonrecurrent brain metastases were studied. Sixty-one patients (97%) underwent surgery within 2 weeks of diagnosis of the brain metastases. RESULTS: The median length of survival was 16 months (95% confidence interval [CI] 11 to 22 months), and the 5-year survival rate was 17% (CI 9% to 29%). Brain metastases recurred in 27 patients at a median interval of 15 months (CI 12 to 24 months). Eleven patients had local recurrence, 10 had distal recurrence, and seven developed leptomeningeal disease. Significant prognosticators of length of survival were age (p = 0.011), menopause status (p = 0.10), postoperative radiotherapy (p = 0.054), preoperative neurologic status (p = 0.011), and preoperative systemic disease status (p = 0.0003). Systemic disease status had a significant effect on the length of survival but not on the time to recurrence.