Malignant salivary tumors--analysis of prognostic factors and survival

A group of 113 patients with malignant salivary gland tumors was retrospectively reviewed to analyze the association of clinical and histologic factors with survival. These factors were patient sex and age, tumor site, clinical stage, histologic diagnosis, tumor grade, and whether or not final surgical margins were clear. There were 57 parotid, 40 minor salivary, and 16 submandibular gland cancers. The histologic groups were mucoepidermoid carcinoma (49 patients), adenoid cystic carcinoma (31), adenocarcinoma not otherwise specified (18), acinic cell carcinoma (7), malignant mixed tumor (5), squamous cell carcinoma (2), and undifferentiated carcinoma (1). Univariate analysis of clinical factors showed that age and clinical stage significantly influenced survival. At 10 yr the predicted cumulative survival rates for Stage I, II, III, and IV tumors were 74%, 56%, 32%, and 10%, respectively. Tumor grade was the only significant histologic factor. This was most obviously reflected among patients with mucoepidermoid carcinomas. Cumulative survival at 5 yr was 94% for those with low-grade tumors and 26% for high-grade tumors. By multivariate analysis, clinical stage, age, and tumor grade remained highly significant. Analysis of patients with only Stage I and II disease demonstrated that the significant factors were patient age, tumor site, tumor grade, and whether or not surgical clearance was achieved. These results suggest that clinical stage should not be the exclusive determinant of the extent of surgery and that the selection of patients, for adjuvant therapy may be improved by an awareness of these prognostic factors.     

The importance of clinical staging of minor salivary gland carcinoma.

We reviewed a 45-year experience with 459 patients who had previously untreated minor salivary gland neoplasms, 378 (82%) of which were malignant. Data were adequate for retrospective clinical staging in 353 of the 378 patients with malignant tumors using criteria identical to those for squamous carcinoma in the same sites. Five-, 10-, and 15-year survival rates for the patients with malignant tumors treated after 1966 were 75%, 62%, and 56%, respectively, a significant improvement compared with results reported previously. Multivariate analysis confirms that survival was significantly influenced by the clinical stage and the histologic grade, but the applicability of grading was limited to patients with mucoepidermoid carcinoma or adenocarcinoma. Ten-year overall survival was 83%, 53%, 35%, and 24% for patients with stage I through stage IV, respectively. Results in these patients are similar to those we have recently reported in patients with major salivary gland carcinomas, but we are unable to demonstrate that postoperative radiotherapy improved survival.     

Carcinoma ex pleomorphic adenoma: a clinicopathologic review

BACKGROUND: Carcinoma ex pleomorphic adenoma (CXPA) is an aggressive, poorly understood salivary gland malignancy. Misdiagnosis is common, because the residual mixed tumor component may be small, and various carcinoma subtypes are possible. METHODS: We retrospectively reviewed the medical records of 73 patients with major salivary gland CXPA treated at our institution from 1960 to 1994. Of the 73 patients, 66 had primary tumors and 7 had recurrent tumors; 47 were men, and 26 were women; the mean age was 61 years. RESULTS: Adenocarcinoma (31 cases) and salivary duct carcinoma (24 cases) were the most common malignant subtypes. All patients were treated surgically, and 32 also had radiation therapy. Of 66 patients with primary tumors, 23% had local recurrence. Metastasis (either initial or delayed) occurred regionally in 56% and distantly in 44%. Thirty-six patients (55%) died of the disease. At 3 years, overall survival was 39% and at 5 years, 30%. CONCLUSIONS: Important prognostic factors include tumor size, grade, and clinical and pathologic stage. Patients with minimally invasive tumors (<5 mm) should do well with appropriate surgical treatment.     

Stage means more than grade in adenoid cystic carcinoma.

Our experience with 184 previously untreated patients who had adenoid cystic carcinoma of salivary gland origin is reviewed. Retrospective staging was possible in all but nine patients who had minor salivary gland primary tumors. Sixty-three percent of patients were diagnosed as having stage I or stage II disease (stage I, 64 patients; stage II, 47 patients), whereas 43 and 21 patients had stage III and IV tumors, respectively. Grading was as follows: cribriform pattern only (grade 1, 126 patients; 68%), mixed cribriform and solid features (grade 2, 48 patients; 26%), and solid only (grade 3, 10 patients; 5%). Treatment was predominantly surgical (174 patients), and relatively few patients received adjunctive, postoperative irradiation (27 patients). Cumulative 10-year survival was 75%, 43%, and 15% for stage I, stage II, and stage III and IV patients, respectively, and cause-specific survival at 10 years was as high as 94% in patients with stage I disease. Only the clinical stage had a significant impact on survival. Neither survival, regional metastases (16 patients; 11%), nor distant dissemination (64 patients; 43%) was predictable on the basis of tumor grade alone. The prognosis in patients with early stage lesions may be better than has been appreciated.     

Correlation of histopathological variants, cellular DNA content, and clinical outcome in adenoid cystic carcinoma of the salivary glands.

OBJECTIVE: To study the correlation between flow cytometrically measured DNA ploidy with prognostically important histopathologic groups and clinical outcome in patients with adenoid cystic carcinoma of the salivary glands. STUDY DESIGN: 46 tumor specimens were analyzed flow cytometrically for DNA content and assessed for histological grade. Correlations were made between tumor DNA ploidy and histopathological grade, and disease-free and overall survival of these patients. RESULTS: Of the 46 patients, 31 had a cribiform/tubular histologic pattern, and 15 had a solid pattern. 84% of the tumors with cribriform/tubular pattern were DNA diploid, compared with 33% of tumors that were graded solid. This difference proved to be statistically significant (chi(2)11.75, P = 0.0006). Overall and disease-free survival periods were longer for patients with DNA diploid tumors in both groups, 63% vs. 36% and 62% vs 38%, respectively. CONCLUSIONS: Tumor DNA ploidy correlates with prognostically important tumor histopathology as well as overall and disease-free survival in patients with adenoid cystic carcinoma of the salivary gland. EBM rating: B-3.     

Mucoepidermoid carcinoma of salivary glands in the pediatric age group: 18 clinical cases, including 11 second malignant neoplasms

BACKGROUND: Salivary gland tumors represent 1% of head and neck tumors, with only 5% of these occurring in patients younger than 20 years. Mucoepidermoid carcinoma (MEC) is one of the most frequent salivary gland cancers among adults and children. METHODS: This survey was conducted among 34 French pediatric oncology departments. From 1980 to 2000, 18 cases were reported. RESULTS: Treatment included surgery or radiotherapy, or both. The 5-year survival rate was 93.7%. Eleven patients had been previously treated by radiotherapy and/or chemotherapy for a first malignant tumor, specifically, lymphoid leukemia (n = 4), lymphoma (n = 3), brain tumor (n = 2), sarcoma (n = 1), and retinoblastoma (n = 1). CONCLUSIONS: MEC is very rare in the pediatric age group. Treatment involves surgical removal of the tumor plus radiotherapy, according to histologic staging. MEC has a good prognosis in young patients. The survival rate does not differ in the subgroup of patients with MEC as a secondary tumor.     

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.     

Malignant lymphoma of the breast. A study of 53 patients

Fifty-three patients with non-Hodgkin's lymphoma of the breast were reviewed and classified using four current classifications of lymphoma. All patients were female with a mean age of 57 years. The majority of patients had histiocytic or large-cell lesions and presented as clinical Stage I. The tumors were described clinically as primary in the breast, and mammary parenchyma was found in 79% of the diagnostic biopsy specimens. The other specimens showed lymphoma in mammary adipose tissue. Survival was not influenced by the presence or absence of breast parenchyma in the biopsy. Statistically significant survival differences were found to be related to stage at presentation as well as to tumor grade, using Kiel and Working Formulation categories. Patients with Stage I disease and those with low-grade lesions had a more favorable prognosis. No discernible factors, including stage or histologic findings, appeared to affect the recurrence rate.     

Mucoepidermoid carcinoma of salivary glands: a clinicopathologic study of 46 cases

Forty-six cases of mucoepidermoid carcinoma (MEC) of the salivary glands were reviewed with an emphasis on the clinicopathologic aspects and prognosis of the tumors. MEC occurred in a wide age range, with few cases being diagnosed in childhood. Its peak of incidence was in the fifth decade and it involved major and minor salivary glands with equal frequency; the parotid gland was the gland most commonly affected. Presence of a mass was the most common complaint of all 46 patients. About 64% of the patients presented with stages 3 and 4 disease. Three distinct histologic grades were found after analyzing cellular pleomorphism, mitotic activity, and presence of necrosis. This study showed that clinical staging and histologic grading are the most important factors influencing survival and they seem to be independent of each other. Other variables found to be important in prognosis were age, sex, and the development of recurrent disease after surgery. Surgery with total resection of the tumor was the most effective treatment in our cases and adjuvant radiotherapy proved to be of little value in improving survival of MEC in our series of cases, considering that the patients in whom this type of treatment was used had unfavorable clinicopathologic factors.     

Clinical prognostic factors in malignant parotid gland tumors.

OBJECTIVE: To analyze the factors in parotid malignant epithelial tumors influencing recurrences and disease-specific survival. METHODS: We retrospectively reviewed the files of 150 patients treated at our institution, from 1974 to 1998. Twenty-four patients were not treated by surgery and were excluded from this study. The remaining 126 patients were treated with surgery and 74 patients had postoperative radiotherapy. Thirty-three patients were treated with parotidectomy plus neck dissection. Neck lymph node metastasis was found in 22 patients, 5 patients had occult neck metastasis, and 4 periparotid lymph nodes metastasis. The mean age was 49 years old. According to the UICC/1997 TNM Classification, 49 patients were stage I, 27 stage II, 22 stage III, and 28 stage IV. The influence of selected factors on the 10 year disease-specific survival was analyzed using the Kaplan-Meier actuarial method and the log-rank test. RESULTS: Forty patients had mucoepidermoid carcinoma, 18 patients adenocarcinoma NOS, 18 patients acinic cell carcinoma, 15 patients adenoid cystic carcinoma, 11 patients malignant mixed tumor, 11 patients salivary duct carcinoma, and 13 patients other pathology. Twenty-five patients had recurrences: 17 had local recurrences, 4 patients had neck recurrences, and 4 were loco-regional recurrences. Five factors influenced negatively the prognosis: 1) T stage (p.00001), 2) grade (p.00001), 3) + lymph nodes (p.0007), 4) facial nerve dysfunction (p.0001), and 5) age (p.004). Patients with high-grade tumors and high-stage tumors had the worst prognosis according to the multivariate analysis. The 10-year disease-specific survival was 97% for stage I, 81% for stage II, 56% for stage III, and 20% for stage IV. CONCLUSION: The grade of the tumor and stage were the most important prognostic factor. EBM RATING: C.     

Sarcomatoid differentiation in renal cell carcinoma: a study of 101 cases

Sarcomatoid renal cell carcinoma is not a distinct histologic entity and represents high-grade transformation in different subtypes of renal cell carcinoma. It is not known whether any particular histologic type has a predilection for sarcomatoid change or whether the primary histologic type of renal carcinoma undergoing sarcomatoid change affects prognosis. Of 952 consecutively histologically subtyped renal cell carcinomas, the incidence of sarcomatoid differentiation was 8% in conventional (clear cell) renal carcinoma, 3% in papillary renal carcinoma, 9% in chromophobe renal carcinoma, 29% in collecting duct carcinoma, and 11% in unclassified renal cell carcinoma. One hundred one renal cell carcinomas with sarcomatoid change were studied, and clinicopathologic parameters were correlated with outcome. The mean age of patients was 60 years (range, 33-80 years), and the male-to-female ratio was 1.6:1. The median tumor size was 9.2 cm (range, 3-25 cm). The primary histologic subtype of the carcinoma component was conventional (clear cell) renal carcinoma in 80 cases, papillary renal carcinoma in eight, chromophobe renal carcinoma in seven, collecting duct carcinoma in two, and unclassified renal cell carcinoma in four. The sarcomatoid component resembled fibrosarcoma in 54 cases, malignant fibrous histiocytoma in 44, undifferentiated sarcoma (not otherwise specified) in three with focal rhabdomyosarcomatous component in two of them. The spindled elements accounted for 1% to 99% of the sampled tumor (median, 40%; mean 45%). The histologic grade of the spindled elements was intermediate to high in 92 cases and low in nine cases. Most cases were TNM stages III and IV (seven stage I, six stage II, 63 stage III, and 25 stage IV). Follow-up was available in 88 patients; 61 (69%) patients died of disease and had a median survival time of 19 months. Distant metastases, most frequently to the lungs, were documented in 51 (66%) of 77 patients who had available clinical information regarding distant metastasis. The disease-specific survival rate was 22% and 13% after 5 and 10 years, respectively, compared with a cohort of renal cell carcinomas without sarcomatoid change with a 5-and 10-year disease-specific survival of 79% and 76%, respectively. Kaplan-Meier survival analysis showed that tumors with high TNM stage (p = 0.0027), at least 50% sarcomatoid component (p = 0.0453), and angiolymphatic invasion (p = 0.0282) were associated with decreased survival rates. The primary histologic subtype of the carcinoma component and the type and grade of the sarcomatoid component did not affect survival. In a Cox proportional hazard regression model, TNM stage appeared to be the only significant variable in predicting outcome among renal cell carcinomas with sarcomatoid change (p = 0.018; risk ratio, 6.984 and 8.439). Compared with a cohort of renal cell carcinomas without sarcomatoid change, sarcomatoid tumors tended to present at a more advanced stage (p = 0.0001). Also, when adjusted for stage, necrosis, and tumor size, patients with tumors with sarcomatoid differentiation had a worse prognosis than did patients with tumors without sarcomatoid change (p = 0.0001). In conclusion, sarcomatoid change in renal cell carcinoma portends a worse prognosis. Because tumors with even a small component of sarcomatoid change may have an adverse outcome, this finding, when present, should be noted in the surgical pathology report.     

Salivary tumors in north Jordanians: a descriptive study.

OBJECTIVE: To evaluate the types and distribution of tumors of salivary glands in north Jordanians. STUDY DESIGN: The records of the Department of Pathology at Jordan University of Science and Technology were reviewed for patients who were treated for salivary gland tumors from 1991 to 2002. The tumors were analyzed for age of patient, sex of patient, tumor site, and tumor type. RESULTS: One hundred two true neoplasms (70% benign and 30% malignant) were found. The most frequent benign and malignant neoplasms found were pleomorphic adenoma (54%) and adenoid cystic carcinoma (13%), respectively. The most common major and minor salivary gland sites were the parotid (51%) and palatal glands (20%), respectively. Although most of major gland tumors were adenomas, carcinomas of the minor glands were only slightly less frequent than adenomas. The most frequent malignant parotid tumors were adenoid cystic carcinoma and mucoepidermoid carcinoma. The most frequent minor salivary gland malignant tumors were palatal adenoid cystic carcinoma. Age ranged from 1 to 94 (mean 40) years, with a male to female ratio of 1:1.2. CONCLUSION: North Jordanians with salivary gland tumors were found to have similar characteristics with patients of other countries with regard to tumor type, tumor site distribution, and age and sex of patients.     

Flow cytometric evaluation of adenoid cystic carcinoma: correlation with histologic subtype and survival.

Adenoid cystic carcinoma is an unusual but capricious tumor of salivary gland origin. Characteristically, these tumors follow a relentless clinical course, although some patients experience prolonged disease-free survival. Tumor size, site, and grade have been shown to correlate with tumor behavior. Recent investigation by others has suggested that DNA ploidy as determined by flow cytometry may provide an additional biologic marker of tumor behavior. This study was undertaken to investigate the relationship of DNA ploidy to tumor grade, biologic behavior, and patient outcome. A retrospective comparison of flow cytometric evaluations of paraffin-embedded formalin-fixed tumor specimens with patient outcome and histopathologic grade was undertaken. Follow-up of 4 to 7 years in 26 patients confirmed that the presence of a solid histologic component in the tumor correlated with the presence of recurrent or persistent disease (p = 0.04). Twenty-two of 28 tumors (78%) were found to be aneuploid on at least one section. Comparison of DNA ploidy with either patient outcome or the presence of a solid component did not achieve statistical significance, although a trend was suggested. This study confirms previous studies demonstrating the validity of histopathologic evaluation of tumor grade in the prediction of the biologic behavior of adenoid cystic carcinoma. However, our findings suggest that DNA ploidy has only limited value as an additional marker of tumor behavior in this patient population.     

Proliferating cell nuclear antigen and c-erbB-2 oncoprotein expression in adenoid cystic carcinomas of the salivary glands.

BACKGROUND: Adenoid cystic carcinoma (ACC) of the salivary gland shows a variable clinical course. It would be helpful to discover reliable biologic markers in the management of patients with ACC. METHODS: We examined proliferating cell nuclear antigen (PCNA) and c-erbB-2 oncoprotein expression on 30 cases of ACC of the salivary glands. The immunohistochemical results, and size, location, and histologic grade of the tumors were compared with the clinical outcome of the patients. RESULTS: Mean PCNA positivity of ACCs was 15%, and was higher in solid than in cribriform/tubular areas. High PCNA value was significantly correlated with shorter disease-free and overall survival of the patients with ACC. c-erbB-2 overexpression was observed in only five cases, focally in cribriform/tubular areas. High histologic grade, which was determined by the presence of solid components, showed a trend toward shorter survival. Size and location of ACC were not associated with patient outcome. CONCLUSIONS: The present study indicates that PCNA score may be one of the most useful prognostic factor of ACC.     

Phase II trial of taxol in salivary gland malignancies (E1394): a trial of the Eastern Cooperative Oncology Group

BACKGROUND: Malignant tumors of the salivary glands make up approximately 5% of head and neck cancers. The Eastern Cooperative Oncology Group (ECOG) initiated a phase II evaluation of paclitaxel in patients with locally recurrent or metastatic salivary gland malignancies. METHODS: Chemo-naive patients with histologically confirmed recurrent or metastatic carcinoma of salivary gland origin (mucoepidermoid, adenocarcinoma, or adenoid cystic) were eligible. Patients were treated with paclitaxel, 200 mg/m(2) IV, every 21 days for a minimum of four cycles. RESULTS: Forty-five patients were treated. Eight partial responses were seen among the 31 patients with mucoepidermoid or adenocarcinoma histologic findings for a response rate of 26%. No responses were seen in the adenoid cystic carcinoma group. No significant difference in overall survival was found among these three histologic subgroups. CONCLUSION: Paclitaxel demonstrates moderate activity in salivary gland tumors of mucoepidermoid and adenocarcinoma histology. The poor response rate in adenoid cystic carcinoma is consistent with prior reports in this chemoresistant histologic subtype.     

Prognostic factors in head and neck cancer: histologic grading, DNA ploidy, and nodal status.

Histopathologic malignancy score and DNA ploidy were investigated as prognostic factors for 72 cases of squamous cell carcinoma of the head and neck (HNSCC). The malignancy grading was based upon four different morphologic characteristics for the tumor cell population and four characteristics for the tumor-host relationship. DNA ploidy was determined through flow cytometry on fresh-frozen tumor samples. The median malignancy score was 20, with 71% of the tumors scoring less than 20 being diploid and 68% of the tumors scoring greater than or equal to 20 being nondiploid (p = 0.003). Univariate analysis revealed that tumors scoring less than 20 and diploid tumors had a significantly higher proportion of complete response and better survival as compared to tumors scoring greater than or equal to 20 and nondiploid tumors, respectively. There was a tendency toward better survival among patients without regional metastasis (N0) as compared with patients with regional spread (N+), whereas the other single factors, patient age, clinical stage, histologic grade, and tumor size did not correlate with prognosis. In N+ patients both malignancy score and DNA ploidy were predictive for survival, whereas in N0 patients only malignancy score was related to prognosis. A multivariate analysis showed that the combination of malignancy score and nodal status were the strongest predictors for survival. DNA ploidy did not contribute further information in this test, due to its close relation with the histopathologic malignancy score.     

Role of nuclear grading in stage I renal cell carcinoma

Through a retrospective histologic analysis of 55 cases of Stage I renal cell carcinoma, we evaluated the usefulness of the nuclear grading system (Fuhrman, Lasky, Limas) in identifying those tumors that will eventually metastasize and kill the patient. The difference in five-year survival rates between patients with combined nuclear grade 1-3 tumors (n = 50, 91%) and grade 4 tumors (n = 5, 9%) was significant (P less than 0.0046). Other predictors of death due to renal cell carcinoma included: tumor size greater than 8 cm (P less than 0.001) and mitoses greater than one per 10 high-power field (P less than 0.01). Within Stage I tumors, therefore, nuclear grade is an important morphologic variable for predicting long-term survival. Identification of nuclear grade 4 neoplasms may become prognostically indispensable to determine the metastatic potential of early-stage tumors and thereby to institute appropriate systemic therapy.     

Prognostic factors for disease progression in patients with renal cell carcinoma

IntroductionMore than 40% of patients with renal cell carcinoma present with disease progression after surgery. The objective of the current study was to identify a clinically useful set of prognostic factors that would correlate significantly with the capacity of progression.Material and methodsThe authors studied 252 patients with renal cell carcinoma who underwent radical nephrectomy. Followup ranged from 12-246 months (median 36 months). Several morphologic parameters of the tumors were considered. DNA content was analyzed by flow cytometry and tumor size was determined from the surgical specimen. A Cox proportional hazards regression model was used to identify significant independent prognostic factors for disease progression.ResultsA total of 224 out of 252 were available for suitable histograms. Of the 224 patients, 95 (42,4%) were aneuploid tumors, 106 (47,2%) were organ-confined renal cell carcinoma and 87 (39,74%) presented disease progression. At 5 and 10 years of followup, disease free survival was found to be 66,31% and 62,23%, respectively. Univariate analysis revealed that DNA ploidy, Furhman grade and stage (TNM) had a statistically significant predictive value for disease progression. Survival univariate analysis found a worse probability of survival for aneuploid tumors, grade III-IV tumors, non organ-confined tumors and conventional and undiferentiated tumors. Using multivariate survival analyses, Furhman grade, stage (TNM) and DNA ploidy were the only independent prognostic factors. So, the probability of death for aneuploid tumor was 1,7 times higher than for diploid tumors.ConclusionsStage, DNA content and Furhman grade were the only significant independent predictors of disease progression. Tumoral size and histological type did not provide more additional information.     

Myoepithelial carcinoma of the salivary glands: a clinicopathologic study of 25 patients

Salivary gland carcinomas displaying exclusively myoepithelial differentiation (myoepithelial carcinoma) are considered rare. Their histopathologic features, immunohistochemical profile, and clinical behavior are not well characterized. The authors reviewed the clinicopathologic features of 25 salivary gland tumors fulfilling two fundamental histologic criteria: unequivocally malignant and exclusively myoepithelial. For most of these, the original diagnosis was malignant mixed tumor. Thirteen men and 12 women aged 24 to 77 years (mean age, 55 yrs) participated in the study, and most presented with a painless mass. The parotid gland was the most common site (n = 15). Tumors ranged from 2.1 to 5.5 cm, arising either in association with a benign mixed tumor (n = 15) or de novo (n = 10). Histologically, all the tumors displayed infiltrative growth and most had a characteristic multinodular architecture with a cellular periphery and central necrotic/myxoid zones. Epithelioid, hyaline, spindle, clear, or mixed cell types were noted with accompanying myxoid and/or hyalinized extracellular matrix. Ten tumors were high grade cytologically and 15 were low grade. The mitotic rate ranged from three to 51 mitoses per 10 high-power fields. Necrosis was present in 15 tumors and perineural and vascular invasion were identified in 11 and four neoplasms respectively. Immunoreactivities included CAM5.2 (89%), AE1:AE3 (100%), 34betaE12 (92%), cytokeratin 7 (21%), cytokeratin 14 (53%), vimentin (100%), S-100 protein (100%), smooth muscle actin (50%), calponin (75%), muscle-specific actin (31%), glial fibrillary acidic protein (31%), carcinoembryonic antigen (0%), and epithelial membrane antigen (21%). Ultrastructural examination of three tumors showed myoepithelial features. Ten patients developed recurrences, mostly multiple. Follow up of 17 patients showed that eight patients (47%) developed metastases (six high grade, two low grade) and five patients (29%) died of disease (four high grade, one low grade) after a mean of 32 months. Two patients were alive with disease (19 and 49 mos). Ten patients (59%) were without any evidence of disease after a mean of 42.2 months. Myoepithelial carcinomas exhibit a wide spectrum of cytomorphologic features and diverse clinical outcomes. As a result of their morphologic heterogeneity, they can be confused easily with many tumors. Myoepithelial carcinomas have been underrecognized in the past, primarily by being lumped under a broader category of "malignant mixed tumor." Awareness of their unique cytoarchitectural patterns and immunohistochemical profile is crucial for accurate identification.     

上海仁济医院肾癌数据库资料分析

目的 探讨肾癌临床、病理、分期、分级与预后特征. 方法 分析2003年至2005年上海仁济医院泌尿科肾癌数据库435例患者临床和病理资料.采用WHO 1997年肾实质上皮性肿瘤组织学分类标准、2002年ATCC的TNM分期和临床分期、1982年Fuhrman病理分级.采用Kaplan-Meier法和Logrank检验对57例获随访的晚期患者行生存分析和预后因素判断. 结果 435例患者中,遗传性VHL病肾癌10例(2.4%)、散发性肾透明细胞癌372例(85.5%)、乳头状癌13例(3.0%)、嫌色细胞癌18例(4.1%)、集合管癌4例(0.9%)、嗜酸性细胞腺瘤4例(0.9 %)、未分类肾癌.14例(3.2%).行根治性肾切除术335例(77.0%),保留肾单位手术74例(17.0%),姑息性肾切除等手术26例(6.0%).遗传性VHL病肾癌均为双肾癌伴多发囊肿,临床分期Ⅰ期7例、Ⅱ期3例,病理分级Ⅰ级6例、Ⅱ级4例,基因测序均存在VHL基因突变,平均随访28.6个月,患者无肿瘤局部进展或转移,但4例患者出现同侧或双侧肿瘤再发.嫌色细胞癌临床分期均为Ⅰ期,病理分级Ⅰ级5例,Ⅱ级13例,平均随访19.8个月均存活,无肿瘤转移或复发.集合管癌临床分期均为Ⅰ期,病理分级均为Ⅲ级,平均生存时间11.3个月.肾透明细胞癌和乳头状癌临床分期Ⅰ期260例(67.6%)、Ⅱ期64例(16.6%)、Ⅲ期32例(8.3%)、Ⅳ期29例(7.5%),其中T1a 147例(38.2%)、T1b 113例(29.4 %);病理分级Ⅰ级124例(32.2%)、Ⅱ级219例(56.9%)、Ⅲ级40例(10.4%)、Ⅳ级2例(0.5%).57例晚期肾癌患者中位生存时间(16.0±1.3)个月,1年生存率55.0%,2年生存率31.0%.预后因素分析显示,临床分期、肿瘤大小、淋巴结转移、远处转移和病理分级是晚期肾癌解剖水平和组织学水平的预后影响因素. 结论 不同组织学亚型的肾癌生物学特征存在较大差异,遗传性VHL病肾癌存在基因突变,常为双侧、多中心、低Fuhrman分级透明细胞癌,易再发不易转移.肾嫌色细胞癌预后较好,而集合管癌预后差.在解剖水平和组织学水平,TNM分期、肿瘤大小、淋巴结转移、远处转移和肾癌病理分级是晚期肾癌的预后影响因素.