A multivariate analysis of the prognosis after surgical treatment of malignant soft-tissue tumors

Ninety-seven patients who had received their primary and definitive surgical treatment for a soft-tissue sarcoma during the years 1956--1976 were studied with respect to local recurrence, metastasis, and survival. The aim of the treatment was to eradicate the tumor while preserving good function. The influence of host and tumor properties and different diagnostic and surgical procedures on the prognosis was studied by means of nonparametric multivariate analysis. Ninety-four percent of the tumors were located in the extremities. Malignant fibrous histiocytoma was the most common histologic type. A four-grade scale of histologic malignancy was used. Eighty-eight percent of the tumors were Grade III or IV. In 85% of the patients with an extremity lesion, local extirpation was carried out. The overall local recurrence rate was 21.7% and in 76 patients who underwent an adequate surgical procedure it was 6.6%. The overall five-year survival rate was 59%. The survival depended on the local control of the primary tumor, which was related to the adequacy of the surgical procedure and to the histologic grade of malignancy. No patient with a Grade I or II tumor died. The five-year survival rate for patients with Grade III tumors was 68% and for patients with Grade IV tumors 47%.     

Supratentorial gangliogliomas: histopathologic grading and tumor recurrence in 184 patients with a median follow-up of 8 years

BACKGROUND: Supratentorial gangliogliomas (GGs) are rare tumors of the central nervous system and are commonly associated with chronic seizures. To date, only case reports and small series of patients with short-term follow-up have been available for the assessment of the potential of GGs to recur and progress. METHODS: Data from 184 patients who underwent resection of GGs between 1988 and 2001 were available from the University of Bonn Epilepsy Surgery Center (Bonn, Germany). Analysis of factors that influenced tumor recurrence and patient survival, such as preoperative history, age at operation, tumor location, histopathologic findings (including immunohistochemical findings), extent of tumor resection, and recurrence evaluated on postoperative magnetic resonance imaging (MRI), was performed. RESULTS: The median follow-up period was 8 years (range, 1-14 years). One hundred seventy-eight patients (97%) presented with long-term seizures (> or = 2 years). The median age at surgery was 26 years (range, 2-65 years). Tumor location was temporal in 79% of patients and frontal in 12% of patients. Eleven tumors (6%) were classified as World Health Organization (WHO) Grade 2 lesions, and 2 tumors were classified as anaplastic WHO Grade 3 lesions. For 38 patients (21%), postoperative MRIs revealed residual tumors. Two years after surgery, 5 patients (3%) experienced tumor recurrence, which resulted in malignant progression in 3 patients (2%) and death in 2 patients (1%). Eighty-four percent of patients with epilepsy had complete and sustained seizure relief. The calculated 7.5-year recurrence-free survival rate was 97%. Lower rates of recurrence were found in patients with tumors classified as WHO Grade 1 lesions (P < 0.0001), patients with temporal lesions (P < 0.0001), patients who underwent complete tumor resection (P = 0.0278), and patients with long-standing epilepsy (P < 0.0001). CONCLUSIONS: Supratentorial GGs are benign tumors, and the surgical goal for patients with GG should be complete resection. Residual tumor masses, frontal tumor location, and WHO Grade 2 or 3 lesions are associated with a greater risk of recurrence or malignant progression. Patients with such characteristics should be considered for long-term clinical follow-up using MRI. .     

Does wide excision as the initial diagnostic procedure improve prognosis in patients with cutaneous melanoma?

502 patients with clinical stage I cutaneous melanoma were reviewed to determine if performing a wide excision (4-5 cm) at the time when the diagnosis of melanoma is suspected, improves the survival. Patients were divided into two groups based on initial biopsy type and thickness category. Group 1, wide excision; group 2, total excision with narrow margins, incisional, or punch biopsies. There was no evidence that patients who had had a diagnostic and therapeutic procedure (wide excision) as the initial approach had a better survival than those who had had another form of biopsy before definitive surgery. We cannot recommend excision with wide margins as the initial biopsy procedure for a lesion suspected to be melanoma before histologic verification, since it does not increase survival for melanoma and may result in unnecessary aggressive surgery in the case of a misdiagnosed benign lesion.     

Radiotherapy in Ewing tumors of the vertebrae: treatment results and local relapse analysis of the CESS 81/86 and EICESS 92 trials

PURPOSE: Treatment results in patients with Ewing tumors of the vertebrae enrolled in the Cooperative Ewing's Sarcoma Study (CESS) 81, 86, and the European Intergroup Cooperative Ewing's Sarcoma Study (EICESS) 92 trials were analyzed with special emphasis on radiation-associated factors. PATIENTS AND METHODS: A retrospective analysis was performed on 116 patients with primary tumors of the cervical, thoracic, or lumbar vertebrae treated between 1981 and 1999. Furthermore, a relapse analysis was done on those patients who underwent radiotherapy and subsequently had a local recurrence. RESULTS: A total of 64.6% of the patients received definitive radiotherapy; 27.5% of patients had surgery and radiotherapy. Only 4 patients (3.4%) underwent definitive surgery. Twenty-seven patients presented with metastases at diagnosis. 22.4% of the total group developed a local relapse. Among the subgroup with definitive radiotherapy, local recurrence was seen in 17 of 75 patients (22.6%). Event-free survival and survival at 5 years were 47% and 58%, respectively. Of the 14 evaluable patients with a local relapse after radiotherapy, 13 were in-field. No correlation between radiation dose and local control could be found. CONCLUSION: Surgery with wide resection margins is rarely possible. The results after definitive radiotherapy in vertebral tumors are comparable to those of other tumor sites when definitive radiotherapy is given. Nearly all local relapses after radiotherapy are in-field.     

Definitive radiotherapy in the management of isolated vaginal recurrences of endometrial cancer

PURPOSE: The aim of our study was to assess prognostic factors and overall survival after salvage radiotherapy for patients who had endometrial carcinoma and who experienced an isolated vaginal recurrence. METHODS AND MATERIALS: We reviewed the records of 50 patients treated at our institution between 1967 and 2003 for an isolated vaginal recurrence of endometrial carcinoma. Initial treatment for endometrial carcinoma was definitive surgery in 49 patients and definitive radiotherapy in 1 patient. The median time from initial diagnosis of endometrial carcinoma to recurrence was 25 months (range, 4-179 months). Three patients (6%) received external-beam radiotherapy alone, 8 patients (16%) received brachytherapy only, and 39 patients (78%) received combined external-beam radiation therapy and brachytherapy. Median dose of radiation to the recurrence was 60 Gy (range, 16-85 Gy). Overall survival was calculated by the Kaplan-Meier method. Endpoints were measured from the date of diagnosis of the vaginal recurrence. Median follow-up of survivors after recurrence was 53 months (range, 8-159 months). RESULTS: The 5-year and 10-year disease-free and overall survivals were 68% and 55%, and 53% and 40%, respectively. On multivariate analysis, age (p = 0.0242), Grade 1 or 2 vs. Grade 3 tumor (p = 0.002), and size of recurrence (p < 0.001) were significant predictors of overall survival. All patients who had Grade 3 disease were dead by 3.6 years from the time of recurrence. Five patients experienced a Grade 3 or 4 complication. CONCLUSIONS: Patients treated with radiotherapy for an isolated vaginal recurrence can be cured in over 50% the cases. Radiotherapy is well tolerated, with a low risk of complications. Factors predictive of overall survival include tumor grade, patient age at recurrence, and tumor size.     

Multivariate analysis for clinical prognostic factors in 163 patients with soft tissue sarcoma

Clinical and histologic findings in 163 patients with localized soft tissue sarcoma (STS) in the extremities and trunk were reviewed. There were 91 male patients and 72 female patients ranging in age from 2 to 84 years (median, 46 years). The histologic status of the tumors was as follows: low grade, 29 cases; intermediate grade, 52 cases; and high grade, 82 cases. The primary tumors were treated by intralesional excision (two cases), marginal (88), wide local (52), or amputation (21). Subsequent adjuvant therapy was given to 61 patients; 17 had radiotherapy (RT), 27 had chemotherapy (CH), and 17 had combined RT and CH. The overall survival (P less than 0.1) and disease-free survival (P less than 0.001) were better in the group that received multimodal treatment (radical surgery and adjuvant chemotherapy with or without radiotherapy) than in the group treated only by surgery. This suggests the favorable role of adjuvant therapy. The univariate and the Cox multivariate analysis for prognosis revealed that sex, tumor-related symptoms, tumor size, tumor depth, and histologic grade were the significant factors. Among the treatment schemes, adjuvant chemotherapy was the only one that affected survival, especially for the intermediate-grade tumors. Initial surgical treatment (marginal versus wide local excision) significantly contributed to the local control of the primary tumors.     

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.     

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.     

A staging system for soft-tissue sarcoma and its evaluation in relation to treatment

In order to define the significant factors for a staging system of soft-tissue sarcomas(STS), histologic and clinical findings in 190 adult patients with localized STS in the extremities and trunk were reviewed. The male-to-female ratio was 1.21. The histologic grading of tumors was defined according to the criteria recently proposed by us: tumors were low-grade in 65 cases, intermediate-grade in 57 cases and high-grade in 68 cases. The initial surgical procedure was as follows: intracapsular excision in 9 cases, marginal excision in 104 and wide local excision in 77, including 15 amputations. The mode of treatment was surgery alone (101 patients), surgery and chemotherapy (58), surgery and radiotherapy (22) and surgery and combined chemo- and radiotherapy (9). Univariate analysis revealed histologic grade, sex, tumor size and tumor depth to be significant prognostic factors. Multivariate analysis revealed histologic grade to be the only independent factor for prognosis. Significant clinical factors in each histologic grade were then evaluated. In the low-grade group, local recurrence significantly affected prognosis. Most of the patients with local recurrence had had marginal resection as the initial surgical procedure. No clinical factors affecting prognosis in the intermediate-grade group could be determined. In the high-grade group, patients with wide local excision and adjuvant chemotherapy had a better prognosis than those with marginal excision with or without adjuvant chemotherapy and wide local excision without chemotherapy (p=0.09). In conclusion, histologic grade was the only significant factor for the staging of STS. On the basis of our staging system, different modalities of treatment for each grade of STS might be indicated; adequate surgery is essential for the prevention of local recurrence, which resulted in reduced mortality in patients with low-grade STS. For high-grade STS, the prevention of distant metastasis by combined extensive surgery and adjuvant chemotherapy may make long-term survival possible.     

Osteosarcoma of the spine: experience of the Cooperative Osteosarcoma Study Group

BACKGROUND: Due to the low incidence rate, the optimal strategy for the treatment of patients with spinal osteosarcoma is unknown. METHODS: Twenty-two patients with osteosarcoma of the spine (15 with tumors of the sacrum and 7 with tumors at other sites) who received chemotherapy according to the Cooperative Osteosarcoma Study Group protocol were analyzed. Six patients presented with metastasis, and 16 patients had no evidence of metastasis at the time of entry into the protocol. Of 12 patients who underwent excision of their tumors, 2 patients underwent wide excision, 3 patients underwent marginal excision, and 7 patients underwent intralesional excision. Eight patients received irradiation: Six patients received conventional radiotherapy only, one patient received neutron beam therapy, and one patient received samarium-153-ethylene diamine tetramethylene phosphonate therapy. Follow-up ranged between 24 months and 105 months (median, 47 months). RESULTS: The median survival was 23 months, and three patients have survived without disease for > 6 years. Patients with primary metastases (P = 0.004), large tumors (P = 0.010), and sacral tumors (P = 0.048) had lower overall survival compared with patients who had no metastasis, small tumors, and nonsacral tumors, respectively. There was a significant difference in overall survival between 5 patients who underwent either wide or marginal surgery and 17 patients who underwent either intralesional surgery or no surgery (P = 0.033). Among 17 patients who underwent no surgery or intralesional surgery, overall survival tended to be better in 7 patients who received irradiation compared with the overall survival in 10 patients who did not receive irradiation (P = 0.059). CONCLUSIONS: Patients with metastases, a large tumors, and sacral tumors had a poor prognosis in the current study with small numbers of patients. Wide or marginal excision of the tumor improved survival. Patients with osteosarcoma of the spine should be treated with a combination of chemotherapy and at least marginal excision for those with surgically accessible tumors. Postoperative radiotherapy may be beneficial.     

Re-excision after unplanned excision of soft tissue sarcomas: Long-term results

BackgroundUnplanned excisions of soft tissue sarcomas are still not infrequent events with patients presenting to referral Centers after having received an inadequate surgical treatment. In literature, both the wait-and-see policy and the “aggressive” management with a re-excision have been advocated. The purposes of this study were to analyze the incidence of detectable residual tumor in surgical specimens following a re-excision and to verify the long-term results of patients treated with a re-excision after previous unplanned excision.MethodsWe retrospectively evaluated 131 patients affected by localized soft tissue sarcoma (95 high grade; 36 low grade) of the limbs or the superficial trunk treated at our Institution, from 2000 to 2013, with a re-excision after a previous unplanned inadequate excision. Site, size, depth, histotype, grade, adjuvant therapies, time from unplanned surgery to re-excision were recorded and evaluated in association with clinical results. We specifically evaluated the disease-specific survival, local recurrence free survival, distant metastases free survival and the event free survival.ResultsMean follow-up for living patients was 10.9 years (median 11.2 years), with a follow-up ranging from 14 to 227 months. 34% of patients underwent a re-excision within the first 2 months after unplanned surgery, while 66% of patients at more than 2 months. Residual detectable tumor cells were found on histological examination in 54% of re-excisions. A wide margin was obtained in 123 cases, a persisting positive margin in 8 patients.Disease-specific survival was 93.5%, 91.6% and 89.6% at 5, 10 and 15 years for whole series and 90.9%, 88.2% and 85.7% for high grade tumors. Event-free survival in patients affected by high grade tumors rated 75.0% at 5 years, 72.4% at 10 years and 72.4% at 15 years. Local recurrence free survival in high grade tumors was 87.6%, 86.2% and 86.2% at 5, 10 and 15 years.The grade of the tumor (high grade) and the initial dimension of the tumor (≥5 cm) were associated with worst survival. High grade tumor impacted negatively also on local recurrence free survival and event free survival. Instead, the initial size of the tumor significantly affected the event free survival but not the local recurrence free survival. No significant differences of outcome were found analyzing tumor depth, time interval to re-excision, presence of residual tumor or margins.ConclusionBased on our results and literature findings, we believe that surgeons should offer a re-excision procedure in those patients presenting with an inadequate excision of a high grade soft-tissue tumor, in particular with tumors larger than 5 cm before excision. Indeed, if an adequate second treatment is performed with surgery ± radiotherapy, the long-term results of patients receiving a re-excision after unplanned excision of a high grade soft tissue sarcoma seem to be comparable to the results generally reported for wide primary excisions. More debatable is whether to perform a re-excision or not in patients with low-grade tumors. Perhaps, in this latter group a wait and see policy can eventually be offered as well as in high grade tumors when a re-excision procedure could involve major surgery or significantly affect postoperative function.     

Phyllodes tumors of the breast: natural history, diagnosis, and treatment

Phyllodes tumors of the breast are unusual fibroepithelial tumors that exhibit a wide range of clinical behavior. These tumors are categorized as benign, borderline, or malignant based on a combination of histologic features. The prognosis of phyllodes tumors is favorable, with local recurrence occurring in approximately 15% of patients overall and distant recurrence in approximately 5% to 10% overall. Wide excision with a greater than 1 cm margin is definitive primary therapy. Adjuvant systemic therapy is of no proven value. Patients with locally recurrent disease should undergo wide excision of the recurrence with or without subsequent radiotherapy.     

Histologic grading of adenoid cystic carcinoma of the salivary glands

Seventy-nine patients with adenoid cystic carcinoma arising in salivary glands were studied to determine whether a correlation existed between the morphologic features of the tumor and the prognosis. Three histologic grades were established: Grade I, tumors with tubular and cribriform areas but without solid components; Grade II, cribriform tumors that were either pure or mixed with less than 30% of solid areas; and Grade III, tumors with a predominantly solid pattern. Cumulative survival rates at 15 years were 39%, 26%, and 5%, for Grades I, II, and III, respectively. Grade III tumors were larger, recurred frequently, and killed the patients within 4 years. Grade I lesions were smaller, were amenable to complete surgical excision, and had a protracted clinical course. Grade II tumors lay between the other two forms both clinically and pathologically. Other important prognostic features of the adenoid cystic carcinoma were its primary site, its presence or absence at surgical margins, and the anatomic structures it involved.     

Temozolomide in the treatment of recurrent malignant glioma

BACKGROUND: Options for chemotherapy at the time of recurrence in patients with malignant glioma are limited. The authors describe the efficacy and safety results of their institution's open-label, compassionate-use protocol of temozolomide for patients with recurrent malignant glioma. METHODS: Patients with recurrent malignant glioma at any time during recurrence were treated with oral temozolomide at a dose of 150 mg/m2 per day on a 5-day schedule every 28 days. If this dose was tolerated, then escalation to 200 mg/m2 was allowed. Clinical evaluations and assessments of tumor response were performed every 2 months. All patients or their surrogates signed approved Institutional Review Board consent forms. RESULTS: Among 213 patients who were treated, 33% had Grade 3 tumors, and 67% had Grade 4 tumors. The overall objective response rate was 16% in both of these patient groups; and an additional 51% and 30% of patients with Grade 3 and Grade 4 tumors, respectively, had stable disease as their best response. The 6-month progression-free survival rates were 41% and 18% for patients with Grade 3 and Grade 4 tumors, respectively. The median survival was 49 weeks for patients with Grade 3 tumors and 32 weeks for patients with Grade 4 tumors. The major toxicity was hematologic toxicity. In multivariate analysis, the Karnofsky performance score was a significant predictor of survival for patients with Grade 4 tumors. CONCLUSIONS: Temozolomide was well tolerated in patients with recurrent malignant glioma and had modest efficacy, even at the time of multiple recurrences.     

Outcome after disarticulation of the hip for sarcomas.

AIMS: To review the oncological and functional outcome in 80 patients who underwent disarticulation of the hip as part of their treatment. METHODS: Eighty patients had disarticulation, of whom 46 had a bone sarcoma and 34 a soft tissue sarcoma. In 42 patients the operation was done as the first definitive surgical procedure for that patient. In 38 patients the disarticulation followed local recurrence after unsuccessful limb salvage, three of these patients had palliative amputations already having metastatic disease. All patients had adjuvant therapy when appropriate. RESULTS: The overall survival of the patients following the amputation was 56% at 1 year, 39% at 2 years, 27% at 5 years and 21% at 10 years. The 5-year survival of patients having the amputation as a primary procedure was 32%, for those with local recurrence it was 25% whilst for those with a palliative amputation it was nil. Local recurrence developed in 10 patients following the amputation, and was related to close margins of excision; all of these patients subsequently died. Function was on the whole poor, with only one surviving patient regularly using an artificial limb. CONCLUSION: Disarticulation of the hip remains a disabling procedure usually carried out for high grade sarcomas with extensive involvement of bone and soft tissues in the thigh. Long term survival is possible if wide margins of excision can be achieved.     

Thymoma. A clinicopathologic review

The clinical and pathologic features of 283 patients with thymoma treated at the Mayo Clinic (147 female and 136 male; ages 16 years to 90 years; mean, 52 years) were examined. Forty-six percent of the patients had myasthenia gravis and 10% had other paraneoplastic phenomena. The tumors were locally invasive at operation in 32%, including 6% with metastasis to lung or pleura. Intrathoracic recurrence was noted postoperatively in 15% of those who had total excision and distant metastasis developed in 3% of patients. Thirteen percent died of their thymomas and 16% died of myasthenia. Overall 5-year survival was 67% and 10-year survival was 53%. Poor prognostic factors included presence of tumor-related symptoms, large tumor size, local invasion or metastasis in initial operation, and predominantly epithelial histologic features. Although true thymomas are composed of cytologically benign elements, they show a propensity for local invasion and intrathoracic recurrence. They rarely metastasize outside the thorax.     

Recurrence of Breast Cancer following local excision alone for ductal carcinoma in situ

OBJECTIVE: To assess recurrence of breast cancer following local excision alone for ductal carcinoma in situ. METHODS: Eighteen patients who received complete resection for noninvasive ductal carcinoma between 1982 and 1997 were investigated in this study. The mean age of the patients was 45 (29-78) years old. The initial presentation was a clinically palpable tumor in 4 patients, nipple discharge in 6, and microcalcification on mammograms in 8. Patients with palpable tumor underwent wide excision with at least a 2-cm free margin. Patients whose mammograms showed microcalcification underwent lumpectomy, and those who showed nipple discharge underwent duct-lobular segmentectomy. Five patients who underwent lymph node dissection up to level I or II had no lymph node metastasis. The mean follow-up period was 86 months. RESULTS: Local recurrence in the conserved breast was seen in five (27.8%) of 18 patients. The actuarial five-year event-free survival was 76.2%. The histological type of the recurrent tumor was ductal carcinoma in situ in three patients and invasive carcinoma in two. There was no difference in age at initial operation or histological subtype between patients with and without recurrent disease, but patients presenting with nipple discharge initially had a significantly shorter ipsilateral disease-free interval than those presenting with tumor or microcalcification on mammograms. All patients with local recurrence in the conserved breast were treated with breast-conserving surgery or subcutaneous mastectomy. CONCLUSION: Local recurrence frequently occurs in patients presenting with nipple discharge treated by duct-lobular segmentectomy for noninvasive ductal carcinoma. Either wide excision with a larger free margin or adjuvant radiation therapy following duct-lobular segmentectomy should be considered for these patients.     

Definitive surgery and multiagent systemic therapy for patients with localized Ewing sarcoma family of tumors: local outcome and prognostic factors

BACKGROUND: The local management of Ewing sarcoma family of tumors (ESFT) often centers on the surgical resectability of the primary lesion and physician biases regarding differences in the morbidity between primary surgical and radiotherapeutic management. METHODS: The authors retrospectively reviewed the records of 33 patients with localized ESFT who underwent surgery and received systemic chemotherapy at St. Jude Children's Research Hospital (Memphis, TN). Two multiagent systemic chemotherapy regimens were used: 14 patients received vincristine, doxorubicin, cyclophosphamide, and actinomycin D (VACA), and 19 received VACA in combination with ifosphamide and etoposide. The primary tumor was surgically resected via a wide, local excision (n = 32) or a marginal excision (n = 1)performed either at diagnosis or after 3-5 months of systemic chemotherapy. Clinical outcome and prognostic factors for disease control were reported in the current study. RESULTS: The median follow-up for patients was 9.9 years. The 5-year and 10-year survival rates were 84.5% and 75.8%, respectively. At 5 years, the cumulative incidence of local disease recurrence was 12.5%, and the event-free survival (EFS) rate was 71.7%. The same values were found at 10 years. The site of tumor origin was a significant predictor of EFS. The survival rate of patients whose tumors arose in bone was 78.6%, and the survival rate of patients whose tumors originated in soft tissue was 25.0% (P = 0.028). No other factors investigated were predictive of outcome. CONCLUSIONS: Local disease control and overall outcome for patients with ESFT managed by multiagent systemic therapy and surgery was excellent. Local disease control rates remained near 90% at 10-year follow-up. Patients with extraosseous primary sites of disease may fare less well with this approach to therapy.     

Relationship among outcome, stage of disease, and histologic grade for 22,616 cases of breast cancer. The basis for a prognostic index

Survival rates for 22,616 cases of breast cancer listed in the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute were stratified on outcome according to the histologic grade and stage of disease. Two different staging systems, "local, regional, and distant" and a modified American Joint Committee on Cancer (AJCC) system adopted for SEER were used. Relative survival rates were calculated at 5 and 10 years. Patients who were assigned Stage II, Grade 1 had the same survival as those assigned Stage I, Grade 3. Their survival was better than patients assigned Stage I, Grade 4. The 5-year relative survival rate for patients listed as Stage I, Grade 1 was 99% and for patients listed as Stage I, Grade 2, it was 98%. At 10 years, the survival rate of patients assigned Stage I, Grade 1 was 95%. Patients with histologic Grade 1 tumors less than 2 cm in size and with positive axillary lymph nodes had a 5-year survival rate of 99%. As breast tumors increased in size, the histologic grade also increased. The results suggest that in linking histologic grade with stage of disease, the staging system should also be considered. Histologic grade when used in conjunction with stage of disease can improve the prediction of outcome. Our results also indicate that a prognostic index can be created for breast cancer using a combination of stage of disease and histologic grade. The data suggest that only three grades are needed for breast cancer.     

Salivary gland tumors of the oral cavity

Between 1961 and 1985, 62 patients with malignant salivary gland tumors of the oral cavity underwent surgery with curative intent at the University of California, Los Angeles (UCLA) Medical center. All patients had a minimum follow-up of 2 years. Fifty of 62 (81%) patients presented with T1-2 primary tumors. The tumors arose from the palate in 41/62 (66%) patients. The most common histologic type was adenoid cystic carcinoma comprising 34 of 62 (55%) cases. Radical resection was performed in 46 cases and wide local excision in 16 patients. Postoperative radiation therapy was used in 24 cases due to advanced stage and/or positive surgical margins. Results of treatment were analyzed by stage of disease, modes of treatment, histology, and surgical extent. Local control of small lesions reached 100% at 10 years with either radical resection alone or local excision. With residual tumor at the surgical margins, the incidence of local recurrence was 4/14 (29%) for those who received adjuvant radiation therapy and 5/10 (50%) for those who did not. The vast majority of mucoepidermoid carcinomas were early stage and low grade. These lesions had an excellent prognosis with a control rate of 100%. In contrast, there was a 29% (10/34) failure rate for adenoid cystic carcinoma. The 5-, 10-, and 15-year actuarial survivals for the whole group were 94%, 84%, and 73%, respectively. Our results indicate that for early stage disease, wide local excision may offer patients the chance to avoid the cosmetically and functionally debilitating effects of radical surgery without compromising treatment outcome. Adjuvant radiation therapy appears to reduce the local recurrence for those with residual tumor at the surgical margins.