Soft tissue sarcoma of the perineal region in childhood. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1972 through 1984

Thirty-six previously untreated patients younger than 21 years of age with sarcoma arising in the perineal region were entered on the Intergroup Rhabdomyosarcoma Studies (IRS) I and II from 1972 through 1984. The tumor histologic subtype was alveolar rhabdomyosarcoma (RMS) in 20 patients (56%), embryonal RMS in 11 patients (30%), and other types of sarcoma in 5 patients (14%). Fifteen children had grossly complete surgical excision (Clinical Groups I and II), and 15 had localized gross residual tumor (Group III) after initial operative management. Six patients had distant metastases (Group IV) at diagnosis. Twelve patients without distant metastases underwent regional lymph node biopsy; tumor involvement was found in six. Subsequent treatment consisted of chemotherapy with vincristine (VCR) and dactinomycin (AMD) for all patients; patients in Groups III and IV also received cyclophosphamide (CYC) with or without Adriamycin (ADR) (doxorubicin; Adria Laboratories, Columbus, OH). Radiation therapy (RT) was administered to patients in Clinical Groups II, III, and IV. Overall, 28 (78%) patients achieved a complete clinical response. The 3-year disease-free survival rate for these patients was 42%, compared with 52% for all other patients in the combined IRS I and IRS II series (P = 0.44). The overall 3-year survival rate was 59%, compared with 64% for all other patients in IRS I and IRS II (P = 0.48). Aggressive treatment is needed in children with perineal sarcoma to improve their prognosis.     

Prognostic factors for disease-specific survival after first relapse of soft-tissue sarcoma: analysis of 402 patients with disease relapse after initial conservative surgery and radiotherapy

PURPOSE: To document the prognostic factors for survival of patients with soft-tissue sarcoma sustaining a first relapse after definitive treatment. METHODS AND MATERIALS: The clinicopathologic features, relapse patterns, and disease-specific survival rates for 402 consecutive patients sustaining a first relapse of sarcoma after combined surgery and radiotherapy were retrospectively reviewed. Factors affecting disease-specific survival after relapse were evaluated with univariate and multivariate techniques. RESULTS: The median follow-up after relapse was 6.8 years. The overall disease-specific survival rate was 25%, 19%, and 16% at 5, 10, and 15 years, respectively, after the first relapse. The median survival duration was 21 months. Patients with an isolated local recurrence had a 5- and 10-year disease-specific survival rate of 48% and 46%, respectively, and those with an initial metastatic relapse had a disease-specific survival rate of 16% and 10%, respectively (p < 0.001). For isolated local recurrences, the independent determinants of survival were (favorable feature first) the primary tumor site (extremity and superficial trunk vs. head and neck and deep trunk); tumor grade (low and intermediate vs. high); time to recurrence (>12 vs. 5 cm). Although the development of subsequent metastasis was the major cause of death, a significant fraction of patients died of uncontrolled primary tumor. For patients presenting with metastasis as the first relapse, the time to metastasis was the major determinant of survival (>12 vs. < or =12 months). Long-term salvage was largely confined to patients who could and did undergo resection of relapsed disease, either local or metastatic. CONCLUSION: On the whole, patients whose sarcoma relapses fare poorly. However, select subgroups are potentially salvageable. Patients with an isolated local recurrence at sites other than the head and neck and deep trunk have a reasonable prospect for satisfactory outcome. Surgical resection of recurrences and metastases appears to play a major role in potential salvage.     

The effect of nodal status on determinants of initial treatment response and patterns of relapse-free survival in nasopharyngeal carcinoma

PURPOSE: To study the effect of regional nodal status on predictors of treatment response, failure patterns, and the time-dependent nature of the various pattern of relapse via a hazard function analysis. METHODS AND MATERIALS: We reviews tumor control data of 496 patients with nasopharyngeal carcinoma (NPC) to whom a radical course of radiotherapy (RT) with or without induction chemotherapy (CT) was given. All alive patients had a median follow-up period of 131 months. Primary tumor (T) and nodal (N) status were staged according to the TNM system of the American Joint Committee. Remote after-loading brachytherapy may be added to teletherapy in T1-2 lesions while induction CT could be given for N3 and/or T4 lesions. Hazard function analysis over 1-year interval was carried out for locoregional or distant relapse. RESULTS: T stage and brachytherapy were two independent predictors for complete response (CR) at the primary site irrespective of nodal status, whereas N stage and brachytherapy are major determinants for regional CR in node (+) patients. Multivariate analysis revealed that contributors to a relatively long disease-free interval in (1) node (-) patients were for locoregional relapse, induction CT(-) (p = 0.0062) or brachytherapy (+) (p = 0.0268) and for distant relapse, none; (2) node (+) patients were for locoregional relapse, early T stage (p = 0.0377) or regional CR (p = 0.0075) and for distant relapse, induction CT(-) (p = 0.0001) or regional CR (p = 0.0001). In node (-) or (+) patients, primary CR rate yield no independent prognostic value on various types of disease-free survival. Hazard function analysis for relapse revealed that hazard rates are in general negatively correlated with time, being highest at the first year post-treatment, decreasing from time to time, and approaching zero after a longer follow-up period in patients with locoregional CR than in patients without. CONCLUSION: Nodal status had no significant impact on predictors of primary CR, whereas in node (+) patients regional CR rate had an independent value in predicting disease-free survival to locoregional and distant relapse. Hazard function analysis revealed a decreasing hazard rate over a protracted post-treatment time in primary and regional CR patients. This indicates the continued risk of late recurrence in this subset of patients for whom long-term observation is recommended.     

Soft tissue sarcoma in the head and neck--analysis of 87 patients

From 1964 to 1984, 87 patients with soft tissue sarcoma (excluding lymphreticulum system sarcoma) in the head and neck were treated in our hospital. All were proved by pathology. The incidence rate of fibrosarcoma was the highest (36.8%), hemangiosarcoma the lowest (5.8%). There was no liposarcoma or synovial sarcoma in this group. The most frequently involved site was the area near the nasal cavity and maxillary sinus for fibrosarcoma (66%), the base of tongue for hemangiosarcoma (60%), scalp for dermatofibrosarcoma protuberans (54%), parapharyngeal space and soft tissue of the neck for neurogenic sarcoma (45%). Metastasis rate to the lymph nodes was 16-20% for rhabdomyosarcoma, hemangiosarcoma and malignant fibrohistiocyte tumor. No distant metastasis was found in dermatofibrosarcoma protuberans and malignant fibrohistiocyte tumor. Distant metastasis rate was 10-20% for the other types. Local recurrence rate was 9.1% for dermatofibrosarcoma protuberans, 88.9% for malignant fibrohistiocyte tumor, 30-66.7% for the other sarcomas. The 5- and 10-year survival rates were 20% and 0%, 37.5% and 0%, 91.6% and 91.6% for rhabdomyosarcoma, malignant fibrohistiocyte tumor and dermatofibrosarcoma protuberans. There were differences in clinical behavior for soft tissue sarcomas in the head, neck and in the trunk. Highly malignant sarcomas, such as liposarcoma, synovial sarcoma and rhabdomyosarcoma were rare in the head and neck but the low malignant sarcomas in the head and neck were 3-10 times as common as those in the trunk. Distant metastasis rate of the fibrosarcoma was 20% in head and neck and 6.7%in trunk, the 5- and 10-year survival rates were 36% and 77.9%, 25% and 73.6%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)     

Rhabdomyosarcoma in infants younger than 1 year: a report from the Children's Oncology Group

BACKGROUND:

Rhabdomyosarcoma (RMS), the most common soft‐tissue sarcoma in children, occurs less commonly in infants. Historically, poorer outcomes have been reported for infants diagnosed with RMS than for older children.

METHODS:

The authors analyzed the characteristics, treatment administered, outcomes, and patterns of failure for infants aged < 1 year with nonmetastatic RMS who received multimodal therapy on Intergroup Rhabdomyosarcoma Study (IRS) protocols IRS‐IV, D9602, and D9803.

RESULTS:

Seventy‐six infants with nonmetastatic RMS were treated on the 3 protocols from 1991 to 2005. Their median age was 7.4 months (range, 0.1‐12 months). Tumor histology included embryonal (57%), alveolar (21%), and undifferentiated sarcoma/other (22%). A parameningeal primary tumor site was less common in this infant cohort (3%) than in all patients who were treated on IRS‐IV (25%). The estimated 5‐year failure‐free survival and overall survival rates (95% confidence interval [CI]) were 57% (95% CI, 44%‐67%) and 76% (95% CI, 65%‐85%), respectively, for infants compared with 81% (95% CI, 79%‐83%) and 87% (95% CI, 85%‐89%), respectively, for children ages 1 to 9 years. Twenty‐three of 32 infants with treatment failure had local recurrence/progression with distant failure (n = 3) or without distant failure (n = 20). The overall local failure rate was 30%. The median time to treatment failure was 13 months. The failure‐free survival rate was worse for infants who had IRS Group III tumors and for those who received less than protocol‐recommended radiation therapy.

CONCLUSIONS:

Infants with RMS appeared to have worse outcomes than older patients, in part because of high rates of local failure. The authors concluded that concerns regarding morbidity in infants and reluctance to use aggressive local control measures may lead to higher rates of local failure. Cancer 2011. © 2011 American Cancer Society.     

Soft tissue sarcomas arising in the retroperitoneal space in children. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee

One hundred one eligible children with soft-tissue sarcomas arising within the retroperitoneal space have been registered on Intergroup Rhabdomyosarcoma Study Committee (IRS) studies I and II and followed for at least 2 years or until death. The most common presenting symptoms and signs were pain in the abdomen or lower extremities, and/or an abdominal mass, usually noted by a parent or a physician seeing the child for other complaints. The median age at diagnosis was 6.5 years and the sex ratio (M/F) 1.7:1. Histologic types were embryonal or botryoid rhabdomyosarcoma (RMS) in 58 patients, alveolar RMS in 8, pleomorphic RMS in 2, undifferentiated sarcoma in 20, extraosseous Ewing's sarcoma in 4 and unspecified sarcoma in 9. Median tumor size was 10 cm, significantly larger than the 7.5 cm noted in the IRS studies as a whole (P less than 0.05). One patient had complete tumor removal (Group I); 12 had grossly complete removal with microscopic residual tumor (Group II). Fifty-one patients had residual local tumor after biopsy or partial resection (Group III) and 37 patients had distant metastases at diagnosis (Group IV). Treatment included surgery, radiation therapy (RT) and combination chemotherapy with vincristine and actinomycin D with or without cyclophosphamide and Adriamycin (doxorubicin) according to protocol. Thirty-nine of 99 patients (39%) had major difficulties in the delivery of specified RT. Seventy patients received sufficient therapy to be evaluable for treatment response. Forty-one (58%) achieved a complete remission and 16 (23%) achieved partial remission. Twenty-four of 41 children (59%) achieving a CR have relapsed. The proportion of children who remained relapse-free at 2 and 3 years of follow-up was 44% or 42%, respectively. Overall, 40 children have developed recurrent sarcoma and the median disease-free interval and overall survival times were 54 and 88 weeks, respectively. Most children experienced severe myelosuppression; there were three early deaths from infection which occurred during granulocytopenic periods. One third of the patients experienced cystitis. Children with soft-tissue sarcomas arising in the retroperitoneal space have a poor prognosis associated with large tumors which, because of location and/or extent (Groups III or IV), are unresectable and difficult to treat.     

Which patients with microscopic disease and rhabdomyosarcoma experience relapse after therapy? A report from the soft tissue sarcoma committee of the children's oncology group.

PURPOSE: To identify which patients with rhabdomyosarcoma and microscopic residual disease (group II) are likely to not respond to therapy. PATIENTS AND METHODS: Six hundred ninety-five patients with group II tumors received chemotherapy and 90% received radiation therapy on Intergroup Rhabdomyosarcoma Study (IRS)-I to IRS-IV (1972 to 1997). Tumors were subgrouped depending on the presence of microscopic residual disease only (subgroup IIa), resected positive regional lymph nodes, (subgroup IIb), or microscopic residual disease and resected positive regional lymph nodes (subgroup IIc). RESULTS: Overall, the 5-year failure-free survival rate (FFSR) was 73%, and patients with embryonal rhabdomyosarcoma treated on IRS-IV fared especially well (5-year FFSR, 93%; n = 90). Five-year FFSRs differed significantly by subgroup (IIa, 75% and n = 506; IIb, 74% and n = 101; IIc, 58% and n = 88; P = .0037) and treatment (IRS-I, 68%; IRS-II, 67%; IRS-III, 75%; IRS-IV, 87%; P < .001). Multivariate analysis revealed positive associations between primary site (favorable), histology (embryonal), subgroup IIa or IIb, treatment (IRS-III/IV), and better FFSRs. Patterns of treatment failure revealed local failure to be 8%, regional failure, 4%, and distant failure, 14%. The relapse pattern noted over the course of IRS-I to IRS-IV shows a decrease in the systemic relapse rates, particularly for patients with embryonal histology, suggesting that improvement in FFSRs is primarily a result of improved chemotherapy. CONCLUSION: Group II rhabdomyosarcoma has an excellent prognosis with contemporary therapy as used in IRS-III/IV, and those less likely to respond can be identified using prognostic factors: histology, subgroup, and primary site. Patients with embryonal rhabdomyosarcoma are generally cured, although patients with alveolar rhabdomyosarcoma or undifferentiated sarcoma, particularly subgroup IIc at unfavorable sites, continue to need better therapy.     

Indications for radiotherapy and chemotherapy after complete resection in rhabdomyosarcoma: A report from the Intergroup Rhabdomyosarcoma Studies I to III.

PURPOSE: To evaluate the outcome of patients with rhabdomyosarcoma (RMS) treated with complete surgical resection and multiagent chemotherapy, with or without local radiotherapy (RT). PATIENTS AND METHODS: Four hundred thirty-nine patients with completely resected (ie, group I) RMS were further treated with chemotherapy (vincristine and actinomycin D +/- cyclophosphamide, doxorubicin, and cisplatin) on Intergroup Rhabdomyosarcoma Studies (IRS) I to III between 1972 and 1991. Eighty-three patients (19%) also received local RT as a component of initial treatment. RESULTS: Eighty-six patients relapsed (10-year failure-free survival [FFS] 79%, overall survival 89%). Six percent of failure sites were local, 6% were regional, and 7% were distant. Poor prognostic factors were tumor size greater than 5 cm, alveolar or undifferentiated histology, primary tumor sites other than genitourinary, and treatment on IRS-I or II. For patients with embryonal RMS who were treated with RT, there was a trend for improved FFS but no difference in overall survival. On IRS-I and II, patients with alveolar or undifferentiated sarcoma who received RT compared with those who did not receive RT had greater 10-year FFS rates (73% v 44%, respectively; P =.03) and overall survival rates (82% v 52%, respectively; (P =.02). Such patients who received RT on IRS III also benefited more than those who did not receive RT (10-year FFS, 95% v 69%; P =.01; overall survival, 95% v 86%; P =.23). CONCLUSION: Patients with group I embryonal RMS have an excellent prognosis when treated with adjuvant multiagent chemotherapy without RT. Patients with alveolar RMS or undifferentiated sarcoma fare worse; however, FFS and overall survival are substantially improved when RT is added to multiagent chemotherapy (IRS-I and II). The best outcome occurred in IRS-III, when RT was used in conjunction with intensified chemotherapy.     

Paratesticular sarcoma in childhood and adolescence. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1973-1983

This article reports on the diagnostic features, radiographic findings, staging, operative management, treatments, and treatment results for 95 children (mean age, 10 years) with paratesticular sarcoma; they were treated on the Intergroup Rhabdomyosarcoma Study (IRS) I and II protocols. Embryonal rhabdomyosarcoma (RMS) was the most common histologic subtype (97% of cases). Patients were randomly assigned to receive various therapeutic regimens according to IRS protocols. Among 81 patients (85%) with localized disease, most (57) were in Clinical Group I (localized, completely excised tumors), and 20 were in Group II (gross excision with tumor-involved, regional retroperitoneal lymph nodes or microscopic residual). Only four were in Group III (gross residual disease in the primary site or retroperitoneal lymph nodes). Three of them achieved a complete response (CR) after induction therapy and two then relapsed. The relapse-free survival (RFS) estimates at 3 years from diagnosis were 93% in Group I and 90% in Group II. Distant metastases were present at diagnosis in only 14 patients. Twelve of them achieved CR, but four subsequently relapsed; their 3-year RFS estimate was 67%. Overall, survival rates among the 95 children were excellent (89% at 3 years) compared to the entire IRS series (63%). Treatment with radical orchidectomy and chemotherapy was sufficient for Group I patients. Orchidectomy, chemotherapy, and radiation therapy were highly effective in Group II patients and in a large proportion of those with more advanced tumors. Recommendations for the diagnostic examinations and management of future patients with paratesticular sarcoma are given.     

Long-term results of conventional-dose salvage chemotherapy in patients with refractory and relapsed Hodgkin's disease (Croatian experience).

BACKGROUND: The aim of this study was to analyze outcome of patients with Hodgkin's disease (HD) in whom first-line chemotherapy with mustine/vincristine/procarbazine/prednisone (MOPP) had failed. PATIENTS AND METHODS: From January 1982 to December 1989 among 210 patients treated with MOPP and radiotherapy to initial bulky sites, 65 patients were primary refractory to or relapsed after initial treatment. RESULTS: Twenty-nine of 65 patients (44%) were primary refractory to initial chemotherapy, 20 relapsed within 12 months after complete remission (CR) and 16 relapsed after CR that lasted more than 12 months. Patients with primary refractory HD and early relapse (<12 months after CR) were treated with doxorubicin/bleomycin/vinblastine/darcarbazine. In patients with late relapse (>12 months after CR) MOPP was repeated. The median follow-up for all patients was 115 months. The overall response rate was 63%. Thirty-three patients (51%) achieved a second CR and eight patients (12%) partial response. Remission rate was greatest in patients with late relapse (CR >12 months) (75 versus 55% for early relapse versus 35% for primary refractory HD) (P <0.01). At 10 years, overall and failure-free survival rates were 21 and 16%, respectively. Patients who were in first remission longer than 12 months had a superior overall survival (37 versus 18% for early relapse) and failure-free survival (24 versus 10% for early relapse). No patient with primary refractory HD was alive beyond 52 months after initial treatment failure (P <0.01). Main prognostic factors were duration of the first remission and tumor bulk at relapse. CONCLUSIONS: Our results confirm previous observations that a significant proportion of patients with HD who experience induction treatment failure cannot be cured with conventional treatment and probably need more aggressive therapy.     

Evidence-based chemotherapy for patients with bone and soft part sarcoma

In the present paper, we review the evidence for chemotherapy in patients with bone and soft part sarcoma and discuss the contributions and improvements made by chemotherapy to the treatment of patients with bone and soft part sarcoma. In the osteosarcoma and Ewing's sarcoma family, neoadjuvant and adjuvant chemotherapy have improved the 5-year disease-free survival to 60%, and limb-salvage operations have improved this to 70-80% in cases of non-metastatic malignant bone tumor. Several trials were conducted in order to overcome rate relapses and metastatic bone sarcoma. With osteosarcoma, thoracotomy improved the survival of lung metastatic patients, but CDDP-ADM branch switched according to the neoadjuvant chemotherapy and failed to elevate the continuous disease-free survival of patients. Dose intensive use of cytotoxic drugs with G-CSF or autologous bone marrow transplantation and multidrug programs were conducted in preliminary studies and achieved favorable results in a high risk factors group for tumors of the Ewing's sarcoma family. Surgical techniques have brought improvements in the treatment of soft tissue sarcoma, but there has been no impact by chemotherapy. Ifosfamide and adriamycin combination is being evaluated in the treatment of local advanced and metastatic soft part sarcoma by local control rate or survival from relapse.     

Limited small cell lung cancer: the effect of radiotherapy on local control following response to chemotherapy.

The survival and rate of chest infield relapse was examined in 48 patients with limited disease small cell lung cancer (LSCLC) who had achieved complete (CR) or partial response (PR) following three courses of chemotherapy. During 1985-1986 chemotherapy consisted of carboplatin and etoposide and during 1986-1987, of etoposide, carboplatin, cyclophosphamide, and vincristine (ECCO). After three courses of chemotherapy, chest irradiation (50 Gy in 25 fractions over 5 weeks) was given to encompass the original tumor volume. Complete responders were also given prophylactic cranial irradiation, 30 Gy in 10 fractions over 2 weeks. Overall median survival of all patients receiving chest irradiation was 17 months from commencement of radiotherapy. The infield relapse-free survival at 24 months was 49% (95% confidence interval: 32-66%). Patients who had only achieved a PR at the time of irradiation were more likely to relapse in the chest than complete responders (p = 0.09). We conclude that local relapse remains a major cause of failure in patients with LSCLC in spite of sequential high dose radiotherapy given to patients who have responded to chemotherapy.     

Treatment of stage I nasopharyngeal carcinoma: analysis of the patterns of relapse and the results of withholding elective neck irradiation

This is a retrospective analysis of 196 patients with nasopharyngeal carcinoma Stage I (Ho's classification) treated by megavoltage radiation during 1980-1984. The primary target volume included all potential sites of local invasion and the first station lymph nodes at retropharyngeal spaces. Two different dose schedules were used, both gave a total tumor dose biologically equivalent to 65 Gy by conventional fractionation, and both achieved a 5-year actuarial local-recurrence-free survival of 88%. Elective neck irradiation was withheld in all except seven patients. The overall 7-year actuarial survival was 85%, but the relapse-free survival was only 62%. The patterns of relapse, prognostic factors, and treatment complications were analyzed. Eighteen patients (9%) recurred locally. Radical retreatment with radiation achieved complete remission in seven out of fifteen cases. Distant failure occurred in 17 patients (9%). Although 57 (30%) of the 189 patients without elective neck irradiation subsequently showed lymph node involvement, none of the seven regionally-treated patients relapsed. The successful regional salvage rate was 81% overall (46 out of 57 patients), but 90% (44 of 49) for those properly treated with whole neck irradiation. However, the 7-year actuarial survival was lower in patients with nodal relapse than those without (70% versus 87%) because of the associated higher incidence of hematogenous dissemination. The various aspects of treatment, the value of elective neck irradiation in particular, are discussed.     

Patterns of relapse in locally advanced head and neck cancer patients who achieved complete remission after combined modality therapy

Relapse patterns in patients with locally advanced head and neck cancer who achieved complete remission were evaluated. After combined modality therapy with induction chemotherapy followed by surgery and/or radiotherapy, 71 of 103 patients were clinically free of disease. The 5-year recurrence rate was estimated at 51%, with a 39% local and 26% distant failure rate by 5 years. The factors significantly affecting the relapse patterns were: the site of the primary tumor (those with oral cavity lesions were more likely to fail locally, whereas hypopharynx patients had a higher risk of distant metastases); the type of definitive local treatment (those patients who received surgery and radiotherapy were at lower risk of pure local failure); TN Stage (patients with T4N3 or T3N3 tumor were at higher risk of both local and distant failure); and time to response and presence of oropharyngeal lesions (patients who had a longer period from diagnosis to final complete response [CR] and patients with oropharyngeal primaries were at higher risk for simultaneous local and distant failure). Type of chemotherapy, patient age, tumor differentiation, and response to induction chemotherapy did not significantly influence the patterns of relapse. A combined modality approach with induction chemotherapy, surgery, and/or radiotherapy does not seem to reduce the incidence of distant metastases significantly.     

Combined-modality treatment and selective organ preservation in invasive bladder cancer: long-term results.

PURPOSE: To evaluate our long-term experience with combined modality treatment and selective bladder preservation and to identify factors that may predict treatment response, risk of relapse, and survival. PATIENTS AND METHODS: Between 1982 and 2000, 415 patients with bladder cancer (high-risk T1, n = 89; T2 to T4, n = 326) were treated with radiotherapy (RT; n = 126) or radiochemotherapy (RCT; n = 289) after transurethral resection (TUR) of the tumor. Six weeks after RT/RCT, response was evaluated by restaging-TUR. In case of complete response (CR), patients were observed at regular intervals. In case of persistent or recurrent invasive tumor, salvage-cystectomy was recommended. Median follow-up was 60 months (range, 6 to 199 months). RESULTS: CR was achieved in 72% of patients. Local control after CR without muscle-invasive relapse was maintained in 64% of patients at 10 years. Distant metastases were diagnosed in 98 patients with an actuarial rate of 35% at 10 years. Ten-year disease-specific survival was 42%, and more than 80% of survivors preserved their bladder. Early tumor stage and a complete TUR were the most important factors predicting CR and survival. RCT was more effective than RT alone in terms of CR and survival. Salvage cystectomy for local failure was associated with a 45% disease-specific survival rate at 10 years. Cystectomy because of a contracted bladder was restricted to 2% of patients. CONCLUSION: TUR with RCT is a reasonable option for patients seeking an alternative to radical cystectomy. Ideal candidates are those with early-stage and unifocal tumors, in whom a complete TUR is accomplished.     

The role of neck dissection in the combined modality therapy setting

No method reliably detects residual low-volume nodal disease in a patient with a complete response (CR) to treatment. Control of the N0-1 neck is to be expected after CR; no treatment is needed. Positron emission tomography (PET) may improve patient selection, but neck dissection should still be performed for patients with good performance status and residual masses. Neck dissection reduces the incidence of regional recurrence, although the impact on survival is small. Whether the risk of tumor recurrence or the morbidity of neck dissection should be of greater concern for patients with N2-3 disease in CR is a matter of individual judgment. Combined modality therapy will control most nodal metastases (even for patients with advanced nodal disease). Neck dissection in patients presenting with bulky nodal disease who achieve a CR after combined modality therapy will diminish the regional recurrence rate by about 15%. Nowadays, patients with N3 disease and CR often still have residual neck tumor. Node dissection is to be advised. Neck dissection should still be considered an important treatment modality for patients who undergo combined modality therapy with advanced nodal disease, even if they have achieved a CR.     

Prognosis of children with soft tissue sarcoma who relapse after achieving a complete response. A report from the Intergroup Rhabdomyosarcoma Study I

Four hundred and twenty-three children with newly-diagnosed soft-tissue sarcoma were treated on the Intergroup Rhabdomyosarcoma Study I from November 1972 through December 1976. After institution of therapy, 341/423 (80.6%) achieved a complete response (no detectable tumor), but 115/341 (33.7%) subsequently relapsed. The types and efficacy of management after relapse were ascertained by questionnaire. Information sufficient for critical analysis was obtained for 98/115 patients (85%). Thirteen of the 98 patients had an isolated local recurrence; the other 85 developed metastases in regional, distant, or multiple sites simultaneously. Patients with distant metastases at diagnosis were most likely to develop recurrence, as were those with sarcoma arising in the perineum-anus, retroperitoneum-pelvis, gastrointestinal tract, or extremity. Those with tumor primary in the orbit or genitourinary tract had the lowest recurrence rate, as did those whose tumor histology was nonalveolar. Individualized treatment was given to 90 of the 98 patients after relapse, and a second complete response was achieved in 33 of them (37%). However, only five of the 90 patients (5.5%) are currently tumor-free. The outlook is poor when recurrence develops in patients still receiving chemotherapy; only two of 74 such patients survive, compared to three of 16 who have relapsed after cessation of treatment. Relapse after initial complete response to therapy has a poor prognosis, regardless of site of recurrence. Although effective retrieval therapy can prolong survival, only 2 of 12 patients (17%) with local recurrence and three of 78 patients (4%) with metastatic recurrence remain free of disease.     

Treatment and Outcomes of Ewing Sarcoma in Turkish Adults: A Single Centre Experience

Background: Ewing sarcoma is a small round cell tumor arising from soft tissue and bone that predominantlyaffects children and adolescents. The most unfavorable prognostic factor is the presence of distant metastasis atthe time of diagnosis. Materials and Methods: The records of 26 Ewing sarcoma patients (14 male, 12 female)were re-evaluated retrospectively. Results: The median age was 26.5 (19-42) years. Eight patients (31%) showed aprimary tumor in their extremities, 8 (31%) in the thorax, 4 (15%) at the vertebra, 4 (15%) in the head and neck,and 2 (8%) in the abdomen. Five patients (19%) had distant metastasis at diagnosis. The median progression-freesurvival was 72 months and 10 months in localized and metastatic disease, respectively (p=0.005). The overallsurvival rate was 19 months in metastatic disease, and the 5-year overall survival rate was 64% in localizeddisease (p=0.006). Patients who had localized disease in the extremities and were under age 30 had a favorableprognosis. Conclusions: Although Ewing sarcoma is a tumor affecting children and adolescents, it may be seenin adults, where the prognosis is generally worse. Although it is a highly malignant tumor, it is possible to achieveimproved survival with combined modality treatments.     

Conservative surgery and radiation therapy for soft tissue sarcoma of the wrist, hand, ankle, and foot

Seventy-eight patients with soft tissue sarcoma (STS) arising in the distal extremities--wrist, hand, finger, ankle, foot, and toe--who were treated with conservation surgery and radiation therapy were studied retrospectively with respect to survival, local recurrence, functional limb preservation, complications, and distant metastasis. After a median follow-up of 7.9 years, actuarial 5-year and 10-year survival rates were 80% and 69%, respectively, and disease-free rates were 61% and 51% at the same times. Actuarial local control rates were 80% and 74% at 5 and 10 years, respectively. Fifteen patients (19%) had local recurrence, but 12 of these were salvaged. Ultimately, 53 patients (68%) retained a normal or fairly normal extremity, six (8%) needed amputation for complications, and 13 (17%) needed amputation to control recurrent disease. The functional outcome was significantly better for patients with upper extremity lesions than for those with lower extremity tumors; even for the latter, this treatment strategy was preferable to amputation. The incidence of hematogenous metastases from distal extremity sarcomas depends on the size of the primary tumor. It was concluded that conservation surgery and radiation therapy (XRT) is an acceptable treatment strategy for STS arising in distal extremities; it yielded a high rate of disease control and functional limb preservation.     

安罗替尼联合阿霉素和异环磷酰胺治疗软组织肉瘤肺转移的疗效及安全性分析

目的：回顾性研究阿霉素和异环磷酰胺（adriamycin ifosfamide,AI）方案化疗联合安罗替尼维持治疗对软组织肉瘤肺转移患者的疗效与安全性。方法：收集天津医科大学肿瘤医院2018年6月至2022年4月软组织肉瘤肺转移接受AI方案化疗联合安罗替尼维持性治疗的32例患者。按照实体瘤疗效评价标准RECIST 1.1进行治疗效果评价,计算客观缓解率（objective response rate,ORR）及疾病控制率（disease control rate,DCR）,统计分析中位无进展生存期（median progression-free survival,mPFS）、中位总生存期（median overall survival,mOS）及相关不良事件。结果：32例软组织肉瘤肺转移患者中完全缓解（complete response,CR）1例（3.1%）,部分缓解（partial response,PR）10例（31.2%）,病情稳定（stable disease,SD）9例（28.1%）,疾病进展（progress disease,PD）12例（37.5%）,ORR为34.3%...