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Background: The purpose of the study was to investigate the diagnostic yield and clinical utility of open muscle biopsy and to identify pre-biopsy factors that might predict useful clinical results for suspected myopathy., Methods: Two-hundred fifty-eight muscle biopsies, performed for investigation of suspected myopathy, were evaluated., Results: A specific clinical diagnosis following muscle biopsy was made in 43% of cases. As a result of the biopsy, clinical diagnosis was changed in 47% and treatment was changed in 33% of cases. Results either led to a specific clinical diagnosis or changed the diagnosis/treatment in 74% of patients. Positive family history of myopathy and findings of myopathic irritability on electromyography had a negative predictive value for diagnosis change., Conclusions: Open muscle biopsy is useful in myopathy evaluation in the modern genetic era.

Lymphangioma circumscriptum is a lymphatic malformation that involves the skin and may extend to subcutaneous tissue and muscle. Treatment of these lesions is challenging. Surgical excision may be performed but recurrence is common. Herein we report a child with a symptomatic lymphangioma circumscriptum that was treated with pulsed dye laser with good results. For superficial lymphatic malformations containing blood, pulsed dye laser might be considered as a treatment option.

Background: The purpose of this study was to investigate pretreatment variables that could predict prognosis and to evaluate the impact of postoperative adjuvant therapy on the outcomes of patients with Stage IB or II cervical carcinoma with or without pelvic lymph node metastases., Methods: Eight hundred ninety-one patients with Stage IB or II cervical carcinoma who underwent radical hysterectomy and bilateral pelvic lymphadenectomy as primary treatment at a single institution were analyzed. Potential prognostic variables were studied., Results: Among the variables that could be assessed before treatment, depth of cervical stromal invasion (determined by magnetic resonance imaging), clinical stage, tumor size, grade of differentiation, and DNA index (determined by flow cytometry) were independent predictors of outcome in multivariate analysis using a Cox regression model. Three distinct prognostic groups (low, intermediate, and high risk) were defined using these variables. Five-year recurrence free survival (RFS) rates for the low, intermediate, and high risk groups were 94.6%, 82.7%, and 62.3%, respectively (P = 0.0001), and overall survival (OS) rates were 98.4%, 84.5%, and 68.7%, respectively (P = 0.0001). Among patients with pelvic lymph node metastases who were free of parametrial extension, those who received postoperative chemotherapy or chemoradiotherapy had significantly better RFS (P = 0.017) and OS (P = 0.043) than those who received no adjuvant therapy. Among patients without pelvic lymph node metastases but at high risk of recurrence, those who received adjuvant radiotherapy had significantly better RFS (P = 0.015) and marginally improved OS (P = 0.087) compared with those who received no adjuvant therapy., Conclusions: A model containing assessable pretreatment variables for predicting the prognoses of patients with early stage cervical carcinoma was formulated. Subsets of patients for whom postoperative chemotherapy or radiotherapy might be beneficial were identified. The data from this retrospective review may be useful when future prospective trials of the treatment of early stage cervical carcinoma are designed.

Background: Small cell carcinoma of the uterine cervix is an uncommon tumor associated with high mortality even among patients with early stage disease. The role of adjuvant chemotherapy after surgery has been suggested by regimens used for small cell lung carcinoma. During the years 1980-1997, 19 cases in which various adjuvant chemotherapies were given after hysterectomy were reported in the literature published in English., Methods: Adjuvant chemotherapy was administered consecutively to 23 patients with Stage Ib-II small cell cervical carcinoma who had been primarily treated with radical hysterectomy and had adequate bone marrow, renal, and hepatic functions. A combination of vincristine, doxorubicin, and cyclophosphamide alternating with cisplatin and etoposide (VAC/PE) was administered to 14 patients during the years 1988-1996 according to a prospective study protocol. A combination of cisplatin, vinblastine, and bleomycin (PVB) was administered to 8 patients, and another regimen was administered to 1 patient during the years 1984-1988. Prognostic factors were evaluated by analyzing both the data on these 23 patients and the pooled data on the cases retrieved from the literature and our own files., Results: Ten of the 14 patients who received VAC/PE had no evidence of disease during a median follow-up of 41 months, whereas 3 of the 9 who received PVB or another regimen survived. Of the 10 patients who died of their disease, all died of distant metastasis within 10 months after recurrence. Meta-analysis of the pooled data showed that 68% of patients who received regimens containing VAC or PE survived, whereas 33% of patients who received regimens not containing VAC/PE survived (P = 0.0078, log rank test). Seventy percent of patients with no lymph node metastasis at hysterectomy and 35% with lymph node metastasis survived (P = 0.05). All patients who died of disease had extrapelvic metastasis., Conclusions: Chemotherapies containing VAC or PE are favorable regimens for patients with early stage small cell cervical carcinoma after radical hysterectomy.

Background: The presence of Epstein-Barr virus (EBV) has not been documented in previous reports of lymphoepithelioma-like carcinoma (LELC) of the uterine cervix by either polymerase chain reaction or in situ hybridization, and the histogenesis of the tumor remains unknown. Additionally, a relationship between human papillomavirus (HPV) and cervical LELC also has not been reported., Methods: In this article, the authors describe the clinical and histopathologic findings for 15 patients with cervical carcinoma that had a histologic pattern of LELC. The polymerase chain reaction detected the presence of EBV and HPV DNA sequences in cervical LELC., Results: All 15 tumors showed a typical syncytial growth pattern of undifferentiated cells with prominent lymphocytic infiltration. The detection rate of the EBV gene sequence in tissue samples from patients with LELC was more frequent than that in control patients with squamous cell carcinoma of the cervix (11 of 15 patients, 73.3%, vs. 4 of 15 patients, 26.7%; P = 0.01). However, the detection rate of HPV-16 and HPV-18 DNA was significantly lower in patients with LELC tumors than in patients with cervical squamous cell carcinoma (3 of 15 patients, 20.0%, vs. 12 of 15 patients, 80.0%; P = 0.001). After a median follow-up of 3.9 years (range, 1.8-5.3 years), the 15 patients showed no evidence of disease or metastasis after radical hysterectomy or radiotherapy., Conclusions: The finding of EBV associations in cervical LELC supports the hypothesis that EBV may be involved in the pathogenesis of tumors that arise in the cervix. It is possible that cervical LELC may follow a different pathway in the pathogenesis of LELC in Asian women as compared with the more common forms of squamous cell carcinoma.

Background: The role of flow cytometric DNA analysis in predicting prognosis of patients with borderline malignant ovarian tumors has been controversial., Methods: Fifty cases of patients with borderline malignant ovarian tumors were analyzed by histology and by flow cytometry on paraffin embedded tissue. Multiple tissue blocks and serial sections were analyzed for each tumor. The results of DNA analysis were correlated to clinicopathologic data., Results: DNA aneuploidy was demonstrable in 4 cases (8%) when the most atypical section was analyzed. The overall rate of aneuploidy was 14% if additional blocks and serial sections were studied. Two patients died from tumor. One of the two patients had an initial diagnosis of Stage IIc mucinous borderline tumor with DNA indices (DI) of 1.12, 1.42, and 2.04. She had a recurrence in the contralateral ovary 1 year later (DI = 1.83), and a second frankly malignant recurrence diffusely in peritoneum (DI = 1.89). The other patient had an initial diagnosis of Stage IIIc mucinous borderline ovarian tumor with pseudomyxoma peritonei. DNA diploidy was obtained in all of the samples from the primary tumor. An aneuploid peak (DI = 1.28) was demonstrated in only one serial section of the peritoneal implants. Of the other 5 patients who had aneuploid histograms but were disease-free, the DNA indices were 1.35, 1.14/1.18, 1.15, 1.20, and 1.31 and were demonstrable only in either 1 or 2 of the blocks or unproven on serial sections. All patients with diploid-peridiploid tumors were alive and disease free., Conclusions: Reproducible DNA aneuploidy of high DI may be predicting a poor outcome, whereas the significance of inconsistently reproducible aneuploidy of low DI remains to be determined. Further studies of prospective DNA analysis with adequate sampling are necessary to define the role of flow cytometry in patients with borderline malignant ovarian tumors.

Background: The role of flow cytometry in predicting prognosis for cervical carcinoma remains unclear., Methods: Flow cytometric analysis was performed on tissues, fixed in formaldehyde solution and embedded in paraffin, from 411 patients with Stage IB or II cervical carcinoma who had been treated with radical abdominal hysterectomy and bilateral pelvic lymphadenectomy., Results: DNA aneuploid-multiploid tumors were found in 37.5%, tetraploid in 4.6%, and diploid-peridiploid in 57.9%. Five-year recurrence-free survival rates of the three groups were 74.3%, 77.8%, and 76.4%, respectively (P > 0.05). DNA aneuploidy and DNA index (DI) of greater than 1.3 were highly correlated to parametria extension. In univariate analysis, pelvic lymph node metastases, stage, parametrial extension, depth of cervical stromal invasion, tumor size, and DI (1.3, 1.4, 1.5 as breakpoint) were significant prognostic factors. DNA ploidy, S-phase fraction, and S-G2M fraction were not significant. In multivariate analysis, DI of greater than 1.3, pelvic node metastases, clinical Stage II, and depth of stromal invasion greater than two-thirds of full cervical thickness were independent and significant variables. The prognostic index (PI), defined by the model, was able to categorize the patients into three distinct risk groups. The 5-year recurrence free survival rates of the low-, intermediate-, and high-risk groups were 89.5%, 73.0%, and 58.9%, respectively (P < 0.0001)., Conclusions: The prognostic value of the DI as a single variable is promising and warrants additional investigation to establish its appropriate use.