Clinical outcome of epithelial tumors of the thymus

OBJECTIVE AND METHODS: We retrospectively reviewed treatment and clinical outcome of thymic epithelial tumors of 64 patients over a 20-year period. Clinical staging of the tumor was done by according to Masaoka classification. Histological diagnosis of the tumors was done by according to the second edition of the WHO histologic classification system for thymic epithelial tumors. Survival rate was calculated after Kaplan-Meire method. RESULTS: Median age of patients was 53.7 years (ranged from 16 to 81). There were 30 men and 34 women. Eighteen patients had auto-immuno diseases. Sixty-two patients underwent surgery. In 57 patients resection was complete (extended thymo-thymectomy), but in the other five incomplete. The operative approach was median sternotomy in 51 patients and video-assisted thoracoscopic surgery in 6. Stage II to IV patients had postoperative mediastinal irradiation. Stage III to IV patients had postoperative cisplatin (CDDP) based chemotherapy. Inoperable patients were treated by chemo-radiotherapy. There were 42 stage I, 7 stage II, 11 stage III, 3 stage IV a, 1 stage IV b. The 5-year/10-year survival rates were 93%/89%, 71%/71%, 68.5%/--in patients with stage I, II and III. There were 5 type A tumors, 8 type AB tumors, 11 type B1 tumors, 11 type B2 tumors, 9 type B3 tumors, 11 type C tumors, the respect 5-year survival rates were 100%, 100%, 87.5%, 60%, 85.7% and 90%. Masaoka stage II to IV patients classified in B2, B3 and C type except one case. CONCLUSION: Histologic type B2, B3 and C tumors may reflect the invasive nature. Masaoka staging system and the WHO histologic classification may help the assessment and treatment of patients with thymic epithelial tumor.     

Adrenocortical carcinoma: surgical progress or status quo?

HYPOTHESIS: Outcome of patients with adrenocortical carcinoma (ACC) has improved with the advent of more widely available and higher quality imaging. Operative management strategies and use of adjuvant therapy have not changed. DESIGN: Retrospective review of patient histories, imaging studies, operative data, adjuvant therapy, and outcomes at a single institution. Follow-up was complete for a mean of 53 months. Data was compared with prior institutional experience. SETTING: Tertiary care referral center. PATIENTS: All patients undergoing operative management for ACC during the period from 1980 to 1996. MAIN OUTCOME MEASURES: Determinants of recurrence, survival, and the effect of adjuvant therapy on overall outcome. RESULTS: Fifty-eight patients (30 men, 28 women) with a mean age of 53 years underwent primary operative management for ACC. Functional tumors were identified in 27 patients (47%). Mean tumor size was 12.5 cm. Stage according to the TNM staging system (AJCC Cancer Staging Manual) at presentation was I (n = 0), II (n = 30), III (n = 7), and IV (n = 21). Surgical management included curative resection in 41 (71%), noncurative resection in 14 (24%), and open biopsy in 3 (5%). Perioperative mortality was 5%. Recurrence occurred in 30 patients (73%) with a median time to recurrence of 17 months. Five-year survival by the Kaplan-Meier method was 37%. Prognostic factors (P<.05) included functional status, stage, and chemotherapy in stage III/IV patients. When compared with our prior institutional experience (1960-1980), current patients were more likely to present with stages I to II (52% vs. 34%), have curative resections (71% vs. 50%), and have improved 5-year survival (37% vs. 16%). CONCLUSIONS: (1) Surgical resection remains the principal treatment for stage I to III disease. (2) Adjuvant therapy may improve survival in patients with stage III or IV disease. (3) Current patients were more likely to present at an earlier stage, undergo curative resections, and have improved 5-year survival than institutional historical comparisons.     

Rhabdomyosarcoma in childhood: analysis of survival in 98 cases

Ninety-eight infants and children with rhabdomyosarcoma were analyzed for age, stage, site, and therapy as they relate to survival. Age and sex were not factors. Survival was 91% (10/11) for Stage I, 86% (13/15) for Stage II, 35% (12/34) for Stage III, and 5.2% (2/38) for Stage IV. Overall survival was 37% (37/98); however, 75% had advanced disease at diagnosis. Primary tumor site was genitourinary (GU) (31), extremity (17), head-neck (14), trunk (14), orbit (8), paratesticular (4), retroperitoneal (3), paraspinal (3), buttocks (3), and perianal (1). Survival was favorable in orbital, paratesticular, and (GU) sites. Survival was 20% (9/45) before and 52% (28/53) after chemotherapy and irradiation. The only survivors had embryonal cell histology. Tumor stage and site are important prognostic indicators. Chemotherapy improves survival in Stage I (91%) and Stage II (86%) and shrinks bulky Stage III tumors allowing less radical procedures in selected sites (e.g., GU). Survival is poor in Stage III (35%) and dismal in Stage IV (5.2%) despite combined therapy. Relapse was fatal despite attempts at second-look resection, and altered chemotherapy and irradiation.     

A comparison of the feasibility of laparoscopic resection of the primary tumor in patients with stage IV colon cancer with early and advanced disease: the short- and long-term outcomes at a single institution

Purpose

The role of resection of the primary tumor in patients with stage IV colorectal cancer (CRC) remains controversial. Laparoscopic resection has become an accepted therapeutic option for treating early stage I–III CRC; however, it has not been evaluated for use in patients with advanced stage disease.

Methods

We conducted a retrospective observational study to evaluate the feasibility of laparoscopic resection of the primary tumor exclusively in patients with stage IV colon cancer compared to open resection in patients with stage IV colon cancer and laparoscopic resection in patients with stage I–III colon cancer in terms of operative results and short- and long-term outcomes.

Results

Laparoscopic resection was performed in 35 stage IV patients and open resection was performed in 40 stage IV patients. One hundred and eighteen stage I–III patients who underwent laparoscopic resection were evaluated. In the comparison between the laparoscopic group and the open group among patients with stage IV colon cancer, postoperative recovery appeared to be better in the laparoscopic group than in the open group, as reflected by shorter times to resumption of a regular diet (p = 0.049), shorter lengths of hospitalization (p = 0.083), increased feasibility of postoperative chemotherapy (p < 0.001), shorter time intervals from surgery to chemotherapy (p = 0.031) and longer median survival (p = 0.078) at the expense of longer operative times (p = 0.025). In the comparison between the laparoscopic resection in stage IV and stage I–III disease groups, no significant differences were observed in operative results and short- and long-term outcomes, except for the rate of ostomy creation (48.5 vs. 8.5 %, p = 0.02).

Conclusion

Laparoscopic resection of the primary tumor in patients with stage IV colon cancer achieves equivalent results to that performed in patients with stage I–III disease and that performed in patients with stage IV disease using open resection. The use of a minimally invasive approach in the laparoscopic procedure is beneficial because it results in shorter times to resumption of a normal diet, shorter lengths of hospitalization, increased feasibility of postoperative chemotherapy and shorter time intervals from surgery to chemotherapy at the expense of longer operative times. We believe that patients undergoing laparoscopic resection can receive targeted chemotherapy earlier and more aggressively, which might provide a survival benefit.     

Surgical treatment results in colonic obstruction

Between 1982 and 1988 94 patients with obstruction of the colon were operated (52 male, 42 female, median age 66 years). Carcinoma accounted for the majority of cases: in 51% of patients enteral, in 9% local tumor growth extraenteral, in 17% peritoneal carcinosis. Therapeutic procedures were chosen according to the necessities in benign courses and in extraenteral tumor growth. In obstructing colorectal carcinoma (48 patients) a policy of primary tumor resection was pursued. 48 patients (40%) developed postoperative complications, 3 patients (3.1%) had to be reoperated. The overall mortality rate in patients with acute obstructions of all courses was 9.6%, in patients with obstruction secondary to carcinoma 14.6%. 74% of patients with colorectal tumors had TNM tumor stage III and IV. The 5-years-survival rate amounted to 21% overall, but after primary tumor resection it was 47%.     

A better prognostic value from a modification of lung cancer staging

This prospective study analyzes the survival rate, according to TNM and staging, of 538 patients who underwent curative pulmonary resection for non-oat cell tumors and who survived the operative period. A total of 279 patients had Stage I disease, 113 Stage II, and 146 Stage III. The overall survival rates were 72% at 1 year, 54% at 2 years, 47% at 3 years, 43% at 4 years, and 39% at 5 years. The survival curves of Stages I, II, and III are significantly different. Nevertheless, in Stage I, T1 N0 tumors presented the best survival rate (71% at 5 years), and this was significantly different from those of all other groups. For Stage II, the survival curves were significantly different according to hilar or lobar location of N1. The survival rate of T2 N1 hilar tumors was similar to that of T2 N2 tumors. In Stage III, the survival of T3 N2 tumors was the worst of all classifications. These results may contribute to a reappraisal of the surgical classification. T1 N0 tumors are worthy, on their own, of forming Stage I. T2 N0, T1 N1 lobar, and T2 N1 lobar can constitute Stage II. Stage III or IIIa would comprise carcinomas classified T2 N1 hilar, T1 N2, T2 N2, and perhaps T3 N0 and T3 N1. T3 N2 should probably be isolated in a IIIb or IV stage.     

Review of Our Experience With Neuroblastoma and Ganglioneuroblastoma in Adults

Background

Neuroblastomas and ganglioneuroblastomas (NB/GNB) are malignant tumors that rarely occur in adults. Their disease progression and appropriate treatment are unclear.

Methods

All adults (age ≥18 years) were evaluated for histologically confirmed NB/GNB within our institution. Data were collected via chart review and direct patient contact.

Results

From 1980 to 2009, a total of 15 adult patients with NB/GNB were evaluated: six men (mean age 23 years, range 19–33 years) and nine women (mean 34 years, range 20–66 years). Their overall average age at diagnosis was 30 years. Tumor-related symptoms occurred in ten patients: Pain (abdominal 3, back 2, pelvic 1, groin 1) was more common than a mass (abdominal 2) or dysmenorrhea (1). Five patients had tumors found incidentally by computed tomography (4) or chest radiography (1). Primary tumor origins were in the pelvis (4), mediastinum (3), abdomen (2), adrenal gland (2), retroperitoneum (2), and mixed locations (2). Altogether, 12 patients underwent surgical resection (biopsy in 3; resections of R0 in 5, R1 in 3, R2 in 4). Ten underwent chemotherapy. Histology showed four GNBs and 11 NBs. Five patients with stage I disease survived a mean of 21 years (range 10–40 years). Two are alive today. Three stage III patients died at 2, 6, and 9 years after diagnosis (mean 5.7 years). Six of seven patients (one was lost to follow-up) with initial stage IV neuroblastoma died within 5 years (mean 2.7 years). NB and GNB patients had similar survivals.

Conclusions

Adult-onset NB/GNB is rare. Symptoms appear to occur later when incurable stage IV disease is detected. Complete surgical resection can lead to long-term, disease-free survival in stage I patients.     

Clinical pattern and pathologic stage but not histologic features predict outcome for bronchioloalveolar carcinoma

BACKGROUND: The histologic criteria defining bronchioloalveolar carcinoma (BAC) were recently revised, but it is unclear whether these criteria predict clinical behavior. This study determined the outcome of resected BAC in relationship to clinical and radiologic disease pattern, and pathologic features. METHODS: Between 1989 and 2000, 100 consecutive surgically treated patients with adenocarcinomas exhibiting various degrees of BAC features were retrospectively studied. Histology was reviewed; tumors were classified as pure BAC, BAC with focal invasion, and adenocarcinoma with BAC features. Clinical and radiologic pattern were classified as unifocal, multifocal, or pneumonic. Demographic data, tumor stage, and outcome were recorded. Survival was analyzed by the Kaplan-Meier method, and prognostic factors were determined by the log-rank test. RESULTS: Patient median age was 65, and 74% of the patients were female. Pure BAC, BAC with focal invasion, and adenocarcinoma with BAC features occurred in 47, 21, and 32 patients, respectively. Unifocal disease occurred in 64 patients, multifocal in 29, and pneumonic in 7. Seventy-one patients had stage I/II tumors, 22 had stage III/IV, and 7 patients had Stage X tumors. Overall 5-year survival was 74%. There was no significant difference in survival among the three histologic subtypes. The pneumonic pattern had significantly worse survival compared with unifocal and multifocal patterns. Pathologic stage predicted survival, with 5-year survivals for I/II and III/IV of 83.7% and 59.6%, respectively. CONCLUSIONS: Clinical pattern and pathologic stage, but not the degree of invasion on histologic examination predict survival. Multifocal disease is associated with excellent long-term survival after resection. The favorable survival of stage III/IV BAC indicates that the current staging system does not fully describe this disease in patients undergoing resection because of its distinct tumor behavior.     

Outcome and staging evaluation in malignant germ cell tumors of the ovary in children and adolescents: an intergroup study

Purpose

The aim of this study was to perform an evaluation of outcome and the role of surgical staging components in malignant germ cell tumors (GCT) of the ovary in children and adolescents.

Methods

From 1990 to 1996, 2 intergroup trials for malignant GCT were undertaken by Pediatric Oncology Group (POG) and Children’s Cancer Study Group (CCG). Stage I-II patients were treated with surgical resection and 4 cycles of standard dose cisplatin (100 mg/m²/cycle), etoposide, and bleomycin (PEB) chemotherapy. Stage III-IV patients were treated with surgical resection and randomly assigned to chemotherapy with PEB or high-dose cisplatin (200 mg/m²/cycle) with etoposide and bleomycin (HDPEB). Patients unresectable at diagnosis had second-look operation after 4 cycles of chemotherapy if residual tumor was seen on imaging studies. IRB approval of the protocols was obtained at each participating institution. An analysis of outcome data, operative notes, and pathology reports in girls with ovarian primary site was done for this report.

Results

There were 131 patients with ovarian primary tumors of 515 entered on these studies. Mean age was 11.9 years (range, 1.4 to 20 years). Six-year survival rate was stage, I 95.1%; stage II, 93.8%; stage III, 98.3%; stage IV, 93.3%. In only 3 of 131 patients were surgical guidelines followed completely. Surgical omissions resulting in protocol noncompliance resulted from failure to biopsy bilateral nodes (97%), no omentectomy (36%), no peritoneal cytology (21%), no contralateral ovary biopsy (59%). More aggressive procedure than recommended by guidelines included total hysterectomy and bilateral salpingo-oophorectomy in 6 patients and retroperitoneal node dissection in 10 patients. Correlation of gross operative findings with pathology results was carried out for ascites, lymph nodes, implants, omentum, and contralateral ovary.

Conclusions

Pediatric ovarian malignant GCT (stages I-IV) have excellent survival with conservative surgical resection and platinum-based chemotherapy. Survival appears to have been unaffected by deviations from surgical guidelines. New surgical guidelines are proposed based on correlation of gross findings, histology, and outcome in these intergroup trials.     

Surgical aspects of N-myc oncogene amplification of neuroblastoma

The surgical aspects of N-myc oncogene amplification of neuroblastic tumors were studied in 42 patients with ganglioneuroblastoma or neuroblastoma. The cumulative survival rate of patients with fewer than 10 copies of N-myc (L group) was 73.7% 48 months after initiation of therapy, whereas the rate for those with more than 10 copies of N-myc (H group) was 0% by 20 months (P less than 0.000001). Clinical prognostic factors of neuroblastic tumors such as age, stage, histologic findings, and primary site correlated with the amplification of N-myc. N-myc amplification of more than 10 copies was evident in one of 16 (6.3%) patients less than 1 year of age and in 13 of 26 (50%) patients over 1 year of age and was detected in one of six tumors in stage II, four of 10 in stage III, seven of 16 in stage IV, and two of six in stage IV-S. The amplification occurred more frequently in patients with neuroblastoma than in those with ganglioneuroblastoma and was observed only in tumors of a suprarenal region. Preoperative chemotherapy was prescribed for 19 of the 26 patients with stage III or IV tumors and was similarly effective in both L and H groups. Ipsilateral nephrectomy or combined resection of a part of liver had to be performed in nine of 11 (82%) patients with stage III or IV tumors in the H group but in only one of 15 (6.7%) in the L group, thereby suggesting that the tumor with an amplified N-myc is more invasive. In patients in the advanced stage, total or nontotal resection of the tumor did not affect the survival in the L group, but the survival interval was prolonged significantly by the total removal of the tumor in patients in the H group. These data should aid in the surgical treatment of patients with poor-prognostic neuroblastoma and an amplified N-myc oncogene.     

Surgery in rhabdomyosarcoma of the bladder,prostate and vagina

Summary The treatment of bladder and prostate rhabdomyosarcoma (RMS) is highly controversial. Aside from chemotherapy, treatment modalities include conservative surgery, radical surgery, and pre-, intra-, or postoperative irradiation. Between 1968 and 1993, 78 children with RMS were treated at our institution. In all, 22 tumors were located in the urogenital tract (bladder/prostate, 13; paratesticular, 5; vaginal, 2; others, 2). Altogether, 6 patients had stage II disease; 7, stage III disease; and 2, stage IV disease. All 15 patients with RMS of the bladder, prostate, or vagina received chemotherapy, and 4 had additional radiotherapy. Surgery was also done in 10 patients; parents refused an operation in 3 cases. In all, 3 patients in an advanced tumor stage died of their disease. All other children currently show no evidence of disease (mean follow-up, 6 years; range, from 2 months to 18 years). After chemotherapy, radical operative intervention with multiple biopsies (encircling the tumor) — in contrast to local tumor excision or partial resection — permits complete tumor resection followed by excellent long-term results. Following radiotherapy, often a consequence of organ-sparing therapy, many complications ensued (60%); therefore, irradiation should be restricted to highly selected cases.     

Squamous cell carcinoma of temporal bone: reported on 33 patients.

BACKGROUND: This study assessed the treatment results of a series of 33 patients with squamous cell carcinoma (SCC) of the temporal bone and evaluated the efficacy of mastoidectomy combined with perioperative radiation therapy protocol. METHODS: Thirty-three patients with biopsy-proven SCC invaded to the temporal bone were reviewed retrospectively and staged into three subgroups according to the University of Pittsburgh TNM Staging System. There were 3 patients with Stage I and II disease(tumor confined to auditory canal), 17 patients with Stage III (tumor involving the middle ear or mastoid), and 13 patients with Stage IV(more extensive disease). Two patients were treated by surgery alone. Eleven patients received irradiation only, and the remaining 20 patients underwent combined surgery and perioperative radiotherapy. The surgical intervention included sleeve resection for patients with Stage I and II lesions and mastoidectomy for all patients with Stage III and IV lesions except 1 who had subtotal temporal bone resection. The radiation dose delivered was in the range of 3500 approximately 10 000 cGy, with an average dose of 6560 cGy. RESULTS: The five-year survival rate for the whole series was 51.7% by the life-table analysis. After being staged into three subgroups (ie, Stage I + II, Stage III, and Stage IV), the estimated five-year survival rates were 100%, 68. 8%, and 19.6%, respectively (p = 0.04). Radiation alone yielded a 28. 7% five-year survival, while combined surgery and irradiation gave a result of 59.6% (p = 0.80). For patients treated with planned combined therapy, the actuarial five-year survival rates were 72.7% (8/11) for Stage III disease and 12.5% (1/8) for Stage IV disease (p = 0.02). Twelve patients who died of disease did so of local recurrence (10 cases), cervical metastases (1 case), and liver metastases (1 case), with 70% of succumbing to their diseases within two years. Complications include osteonecrosis (n = 1), osteitis (n = 3), radiation dermatitis (n = 2), facial nerve palsy (n = 2), and delayed healing (n = 2). Data on clinical presentation and treatment modality were also analyzed. CONCLUSION: The results of mastoidectomy with removal of all gross tumor, combined with planned perioperative irradiation therapy, seems to be a useful approach for SCC of the temporal bone. This gives at least as good, and possibly better, five-year survival than temporal bone resection. The mastoidectomy procedure creates less operative morbidity and mortality. To facilitate the development of more effective means of treating advanced disease, an accepted staging system and cooperative group investigation is necessary.     

Primary gallbladder carcinoma: significance of subserosal lesions and results of aggressive surgical treatment and adjuvant chemotherapy

Advances in methods to diagnose biliary disease have not improved the dismal survival rates reported for primary gallbladder cancer. We analyzed the results of operation in 112 patients with gallbladder cancer. Tumors limited to the gallbladder wall and those that required aggressive surgical therapy and adjuvant chemotherapy for advanced disease were analyzed separately according to the following staging system: tumors that were localized to the mucosa (stage, I, n = 4); tumors that penetrated the muscularis (stage II, n = 4); subserosal tumors (stage III, n = 3); tumors with cystic node involvement (stage IV, n = 13); and tumors that had spread to adjacent organs (stage V, n = 88). Of the 11 patients (10%) with tumor limited to the gallbladder wall (stages I to III), one of five patients (20%) treated with cholecystectomy alone and four of six patients (60%) treated with cholecystectomy and lymphadenectomy (with hepatic wedge resection in three and pancreaticoduodenectomy in one) were alive and tumor free 3 to 6 years after operation. Three patients treated with cholecystectomy alone died of recurrent cancer at 18, 48, and 60 months after operation. Of the 13 patients with cystic node involvement (stage IV), nine were treated by cholecystectomy alone, three with lymphadenectomy, and one with pancreaticoduodenectomy; the cumulative survival rate was only 37% at 6 months, and all patients were dead within 18 months. Of 14 patients with advanced disease (stage V) treated with aggressive surgical therapy, including lymphadenectomy in six patients, hepatic wedge resection in six patients, and right hepatic lobectomy in two patients, the mean survival rate was only 3 months.     

Patient and Tumor Characteristics Associated with Primary Tumor Resection in Women with Stage IV Breast Cancer: Analysis of 1988–2003 SEER Data

Abstract: Surgery is the cornerstone of treatment for women with nonmetastatic breast cancer. In contrast, standard treatment for patients with Stage IV disease includes chemotherapy and radiation, with surgery usually reserved for local tumor‐related complications. Little is known about the predictive factors associated with primary tumor resection for Stage IV breast cancer. We conducted a retrospective, population‐based, case–control study using the 1988–2003 Surveillance Epidemiology and End Results (SEER) data. Using multiple logistic regression, we identified patient and tumor characteristics from among SEER region, age at diagnosis, year of diagnosis, marital status, race, Hispanic ethnicity, tumor grade, and size that were associated with surgical resection of the primary breast tumor (compared with no surgical resection) among women with stage IV breast cancer. Adjusted odds ratios and 95% confidence intervals are reported. Of 10,017 patients, 4,836 (48%) underwent surgical resection of the primary breast tumor. Patients in the Northeast and Midwest and patients presenting with two or more primary breast tumors were more likely to have surgical resection. Patients who were older, diagnosed after 1992, unmarried, black, and whose tumors were >5 cm, inflammatory, of unknown size, indeterminate grade, or unknown progesterone status were less likely to have had surgical resection of the primary tumor. Several patient and tumor characteristics were significantly associated with surgical resection of the primary breast tumor in Stage IV disease. Further study of the surgery decision‐making process is recommended.     

Preoperative staging, prognostic factors, and outcome for extremity rhabdomyosarcoma: a preliminary report from the Intergroup Rhabdomyosarcoma Study IV (1991-1997)

BACKGROUND: During the fourth Intergroup Rhabdomyosarcoma (RMS) Study (IRS IV, 1991-97), a preoperative staging system was evaluated prospectively for the first time. The authors evaluated this staging system and the role of surgery in extremity RMS in contemporary multimodal therapy. METHODS: A total of 139 patients (71 girls; median age, 6 years) were entered in IRS IV with extremity-site RMS. Stage was assigned by the IRSG Preoperative Staging System. Postsurgical group was determined by tumor status after initial surgical intervention. Multivariate analysis was performed using all pretreatment factors that were significant by univariate analysis, including clinical Group (i.e., I through IV), tumor invasiveness (T1,T2), nodal status (N0,N1), and tumor size (< or > or =5 cm). Failure-free survival rates (FFS) and survival rates were estimated using the Kaplan and Meier method. RESULTS: Preoperative staging and clinical group distribution were as follows: Stage 2, n = 34; Stage 3, n = 73; Stage 4, n = 32; Group I, n = 31; Group II, n = 21; Group III, n = 54; Group IV, n = 33. Three-year FFS was 55%, and the overall survival rate was 70%. Eighty-seven patients had either unresectable, gross residual disease (Group III) or metastases (Group IV). FFS was significantly worse for these patients with advanced disease, compared with that for patients with complete resection or with only microscopic residual tumor (i.e., Group I or II; Group I, 3-year FFS, 91%; Group II, 72%; Group III, 50%; Group IV, 23%; P<.001). Lymph nodes were evaluated surgically in 76 patients with positive results in 38. Clinically, 13 additional patients had nodal disease. Both stage and group were highly predictive of outcome and were highly correlated. By multivariate analysis, none of the other variables were predictors of FFS. CONCLUSIONS: This review confirms the utility of pretreatment staging for stratification of patients with extremity RMS with widely different risks of relapse, thereby paving the way for development of risk-based therapy. Group (operative staging) remains the most important predictor of FFS, emphasizing the importance of complete gross resection at initial surgical intervention, when feasible without loss of limb function. The high incidence of nodal disease in the patients who had lymph node biopsy confirms the need for surgical evaluation of lymph nodes to ensure accurate staging in children with extremity rhabdomyosarcoma.     

Carcinoma of the gallbladder--a clinical appraisal and review of 40 cases

Prognosis of 40 patients with gallbladder carcinoma who had undergone curative resection was investigated. Five-year survival rate calculated from Kaplan & Meier's method was 67% in 16 cases in Stage I, 43% in 8 cases in Stage II and 22% in 10 cases in Stage III, respectively. In 6 cases, classified as Stage IV, no case survived more than 2 years postoperatively. Most patients in Stage I had the tumors of papillary type in macroscopic appearance, papillary adenocarcinoma, and negative vascular and perineural invasions and showed better prognosis. In Stages II, III and IV, in contrast, most tumors were infiltrative or nodular type, tubular adenocarcinoma, and positive vascular and perineural invasions and demonstrated poorer prognosis. Patients in Stage I who had undergone simple cholecystectomy showed 5-year survival rate of 57%, and who underwent cholecystectomy with wedged resection of the gallbladder bed of the liver and regional lymphadenectomy (extended cholecystectomy) showed that of 100%. Extended cholecystectomy, therefore, is the procedure of choice in patients in Stage I. In patients in Stages II, III and IV, extended cholecystectomy yielded 5-year survival rate of 33%. More radical procedure or combined modality therapy must be indicated in advanced stage of the disease.     

Long-term outcome after multimodality treatment for stage III thymic tumors

BACKGROUND: Surgery remains the cornerstone of therapy for thymic tumors, but the optimal treatment for advanced, infiltrative lesions is still controversial. The introduction of multimodality protocols has substantially modified survival and recurrence rate. We reviewed our 13-year prospective experience with multimodality treatment of stage III thymoma and thymic carcinoma. METHODS: Since 1989 we have prospectively used a multimodality approach in 45 stage III thymic tumors. Sixteen patients (35%) had myasthenia gravis. Twenty-three patients (51%) had pure or predominantly cortical thymoma (group 1), 11 (24.5%) had well-differentiated thymic carcinoma (group 2), and 11 (24.5%) had thymic carcinoma (group 3). Tumors that were not considered radically resectable at preoperative workup underwent biopsy and induction chemotherapy (15 patients, 33%) followed by surgical resection; all patients were referred for adjuvant chemoradiotherapy. RESULTS: No operative mortality was recorded; 1 treatment-related death during adjuvant chemotherapy was observed in group 1. Complete resection was feasible in 91% of patients in groups 1 and 2 and 82% in group 3. The overall 10-year survival was 78%. Ten-year survival for groups 1 and 2 was 90% and 85%, respectively; 8-year survival for group 3 was 56%. During follow-up, tumor recurrence was noted in 3 patients (13%) from group 1, 3 (27%) from group 2, and 3 (27%) from group 3. CONCLUSIONS: Multimodality treatment with induction chemotherapy (when required) and adjuvant chemoradiotherapy offers encouraging results for stage III thymic tumors; the outcome is more favorable for cortical thymoma and well-differentiated thymic carcinoma.     

非小细胞肺癌术后支气管切缘癌与预后

为探讨肺癌术后支气管切缘癌的发生率及预后特点，提高肺癌５年生存率，回顾总结１９８１～１９９０年经手术治疗１０２４例非小细胞肺癌，显微镜下发现支气管残端癌阳性者８９例占８．７％。此８９例病人平均５年生存率２３．７％。中位数生存期２７．６个月。其中ＰＴＮＭＩ、ＩＩ、ＩＩａ和ＩＩｂ期病人平均生存期分别为４４．３、２７．１、１２．４和１２．６个月，鳞癌、腺癌、大细胞癌及鳞腺混合癌病人的平均生存期分别为３１．０、１９．３、１４．８和２８．３个月。结论：影响病人预后的因素主要是病变的ＴＮＭ分期及细胞类型，对有残端癌的病人应强调术后综合治疗     

Surgical treatment of malignant mediastinal neurogenic tumors in children.

INTRODUCTION: The aim of this study was to identify the role of surgical resection in the treatment of malignant mediastinal neurogenic tumors in children. MATERIALS AND METHODS: Thirty-eight consecutive children, who underwent surgical resection of a malignant mediastinal neurogenic tumor between 1986 and 2004, were included in this study. The tumor cell types were neuroblastoma in 23 patients (60.5%), ganglioneuroblastoma in 14 (36.8%), and malignant neuroepithelioma in 1 (2.6%). Surgery was performed for curative resection in localized tumors and salvage resection of residual mediastinal masses after chemotherapy in stage IV tumors. Of the 16 patients (42.1%) who underwent salvage resection, 14 had neuroblastoma and 2 ganglioneuroblastoma. RESULTS: Mean patient age was 3.4+/-3.0 years (1 month-13 years) and 26 patients (68.4%) were symptomatic at presentation. Adjacent structure invasion was found in eight patients (21.1%), invasion of chest wall in four, heart and vena cava in two, lung in one, and chest wall and lung in one. Complete gross resection was possible in 30 patients (78.9%) and there was no surgical mortality. Surgical morbidity occurred in 10 patients (26.3%) and Horner's syndrome was the most frequent complication (n=7). The 5-year survival was 95.2% for a localized tumor and 52.5% for a stage IV tumor (p=0.004). The significant risk factors of long-term survival were adjacent structure invasion (p=0.002) and a stage IV tumor (p=0.002) by multivariate Cox regression analysis. CONCLUSIONS: Surgical resection of localized malignant mediastinal neurogenic tumor in children showed good long-term survival, and salvage operations after chemotherapy showed acceptable long-term survival.     

Adenocarcinomas of the jejunum and ileum: A 25-year experience

Adenocarcinomas of the jejunum and ileum are rare gastrointestinal malignancies. Because few large published experiences exist, we reviewed patients with jejunal and ileal adenocarcinoma treated at our institution over the last 25 years. Between January 1976 and December 2001, 77 patients had an operation for a jejunal or ileal adenocarcinoma. Records were retrospectively reviewed for patient, tumor, and treatment variables. Factors affecting disease recurrence and patient survival were investigated. Fifty-two of the adenocarcinomas (67%) occurred in the jejunum and 25 occured in the ileum (33%). Mean patient age was 63 ±14 years. Segmental bowel resection was performed in 50 patients (65%) with curative intent. Palliative operative procedures including resection or bypass were performed in 27 patients (35%). One (1%) patient had stage I, 18 (23%) stage II, 19 (25%) stage III, and 39 (51%) stage IV adenocarcinoma at diagnosis. Postoperatively, 12 patients had palliative and 18 adjuvant chemotherapy (n 5 30), radiation therapy (n = 1), or combination treatment (n = 7). Median patient survival was 19 months. Sixty-six percent of patients who had a curative operation had a tumor relapse. Tumor stage had a highly significant effect (P < 0.0001) on median survival (72 months for stage I and II, 30 months for stage III, and 9 months for stage IV disease). In multivariate analysis of patients having curative treatment, tumor recurrence (P < 0.0001), stage (P < 0.0002), and weight loss (P < 0.001) were significant negative prognostic indicators. Most patients with adenocarcinoma of the jejunum or ileum present with advanced disease. Tumor stage, disease recurrence, and weight loss predicted patient outcome following a curative operation. Early recognition of these tumors requires a high index of suspicion. Presented at the Forty-Fifth Annual Meeting of The Society for Surgery of the Alimentary Tract, New Orleans, Louisana, May 15–19, 2004 (poster presentation).