Malignant Peripheral Nerve Sheath Tumor of the Vulva: A Multimodal Treatment Approach

Background. Malignant peripheral nerve sheath tumors (MPNSTs) are rare in the gynecological population and have a high risk for local and distant failures. Multimodal management of a patient with MPNST of the vulva and review of the literature are outlined. Case. A 34-year-old woman presented with a complaint of a rapidly increasing pelvic mass, pain, and difficulty ambulating. A disfiguring 20 × 20-cm vulvar mass was identified and a recurrent MPNST diagnosed. Therapy included external-beam radiation, anterior pelvic exenteration with pelvic reconstruction, and adjuvant chemotherapy without complication. Conclusion. It is recommended that for malignant peripheral nerve sheath tumors of the vulva, complete surgical resection be performed with adjuvant radiation and chemotherapy in selected cases

Combined Keratocystic Odontogenic Tumor and Basal Cell Ameloblastoma: A Rare Case Report

Ameloblastoma is an odontogenic tumor with diverse clinical behavior and histomorphologic presentations. Basal cell ameloblastoma are extremely rare variants of ameloblastoma. Keratocystic odontogenic tumor (KCOT) is a distinct form of odontogenic cyst that is considered a benign cystic neoplasm and not a cyst. Co-occurrence of these odontogenic tumors has been rarely reported.This paper reports a 34-year old female patient with a unilocular radiolucency around the crown of a partially erupted third molar that was detected accidentally on a routine radiographic examination. She underwent excisional biopsy with a clinical diagnosis of dentigerous cyst. Histopathologic examination revealed a cystic lesion with criteria of OKC and islands and nests reminiscent of basal cell ameloblastoma and acanthomatous ameloblastoma in the stroma. Follow-up showed no recurrence of lesion to date. Due to the rarity of the lesion, the diagnosis was challenging but altogether we made a diagnosis of keratoameloblastoma with basaloid features

Papillary squamous cell carcinoma of the uterine cervix: an immunophenotypic appraisal of 12 cases

The objective was to evaluate the role of human papillomavirus (HPV) in the pathogenesis of papillary squamous cell carcinoma (PSCC) of the cervix and to determine cell proliferative activity and p53 abnormalities in these rare variants of cervical cancer. Twelve examples of PSCC of the cervix were diagnosed between 1990 and 1999. Formalin-fixed paraffin sections of each tumor were stained by immunoperoxidase method using antibodies to p53 gene product (CM-10) and Ki-67 (MIB-1). In situ hybridization for HPV DNA (ENZO) was used to detect specific sequences of DNA shared by most types of genital HPV, followed by confirmatory PCR analysis. The nuclear staining for Ki-67 was graded as minimal (50% of cells). Fifty-percent of the tumors showed presence of HPV DNA. Three tumors (25%) showed nuclear accumulation of p53. Moderate and high proliferative activity was observed in four and eight of tumors, respectively. Eight patients presented with stage IB1 tumor (67%), 3 with stage IA1 tumor (25%), and 1 with stage IIIA tumor (8%). Eleven patients (92%) were alive as of last contact with a mean follow-up of 34.2 months (range: 5 days to 84 months). In this series of patient, PSCC of the uterine cervix had a low rate of HPV DNA in their genome and a low rate of p53 gene abnormality. These genotypic differences may explain the differences between the clinical behavior of PSCC and the common types of squamous cell carcinomas of the cervix

Optimal surgical cytoreduction in patients with Stage III and Stage IV endometrial carcinoma: a study of morbidity and survival

Objective. To evaluate the survival impact of residual disease at the time of primary surgery for patients with Stage III and IV endometrial carcinoma; to assess morbidity associated with surgical cytoreduction. Method. All patients with endometrial carcinoma who underwent primary surgical therapy at the University of Miami between January 1, 1990 and June 1, 2002 were identified. Patients meeting FIGO criteria for Stage III or IV disease were selected. Papillary serous and clear cell histology was excluded. Results. Eighty-five patients were identified: 66 Stage III and 19 Stage IV. Only Stage IIIC and Stage IV were included in survival analysis: 72% (33 Stage IIIC, 9 Stage IV) had optimal cytoreduction and 28% (6 Stage IIIC, 10 Stage IV) had suboptimal cytoreduction. The median survival for Stage IIIC and IV disease was 6.7 months for patients with suboptimal cytoreduction and 17.8 months for patients with optimal cytoreduction ( P = 0.001). The proportion of patients with major postoperative complications (37.50% vs. 7.25%, P = 0.005), unplanned postoperative SICU admissions (31.25% vs. 7.25%, P = 0.018), and length of hospital stay exceeding 15 days (31.25% vs. 4.35%, P = 0.005) was greater in patients with suboptimal cytoreductive surgery. Conclusions. Overall survival is lower and morbidity is higher in patients with advanced endometrial carcinoma having suboptimal cytoreduction at the time of primary surgery. Patients with suspected advanced stage endometrial carcinoma should be counseled on the potential benefits of optimal cytoreductive surgery. Alternative treatment options should be considered in those patients with surgically unresectable disease

Guidelines of how to manage vesicovaginal fistula

Vesicovaginal fistulas are among the most distressing complications of gynecologic and obstetric procedures. The risk of developing vesicovaginal fistula is more than 1% after radical surgery and radiotherapy for gynecologic malignancies. Management of these fistulas has been better defined and standardized over the last decade. We describe in this paper the success rate reported in the literature by treatment modality and the guidelines used at our teaching hospitals, University of Rome Campus Biomedico and University of Miami School of Medicine. In general, our preferred approach is a trans-vaginal repair. To the performance of the surgical treatment, we recommend a minimum of a 4-6 week's wait from the onset of the fistula. The vaginal repair techniques can be categorized as to those that are modifications of the Latzko procedure or a layered closure with or without a Martius flap. The most frequently used abdominal approaches are the bivalve technique or the fistula excision. Radiated fistulas usually require a more individualized management and complex surgical procedures. The rate of successful fistula repair reported in the literature varies between 70 and 100% in non-radiated patients, with similar results when a vaginal or abdominal approach is performed, the mean success rates being 91 and 97%, respectively. Fistulas in radiated patients are less frequently repaired and the success rate varies between 40 and 100%. In this setting many institutions prefer to perform a urinary diversion. In conclusion, the vaginal approach of vesicovaginal fistulas repair should be the preferred one. Transvaginal repairs achieve comparable success rates, while minimizing operative complications, hospital stay, blood loss, and post surgical pain. We recommend waiting at least 4-6 weeks prior to attempting repair of a vesicovaginal fistula. It is acceptable to repeat the repair through a vaginal approach even after a first vaginal approach failure. In the more individualized management of fistulas associated with radiation, the vaginal approach should still be considered. (C) 2003 Elsevier Ireland Ltd. All rights reserved

The impact of intraoperative autologous blood transfusion during type III radical hysterectomy for early-stage cervical cancer

Objective: The aim of this study was to determine the effects on transfusion rates, perioperative complications, and survival of using intraoperative autologous blood transfusions for patients undergoing type III radical hysterectomy and lymphadenectomy. Study Design: A retrospective analysis was conducted on 156 patients treated with type III radical hysterectomy and lymphadenectomy at the University of Miami School of Medicine from 1990 to 1997. One group of patients (n = 50) had intraoperative autologous blood transfusions and the other (n = 106) did not. Results: The group that received intraoperative autologous blood transfusion had a significant reduction in homologous blood transfusions (12% vs 30%; P = .02). Patient demographic data, histologic parameters, and operative factors were similar between the 2 groups. There was a higher percentage of patients with positive pelvic lymph nodes in the group that did not receive intraoperative autologous blood transfusion (10% vs 30%; P = .02). Seven patients in the intraoperative autologous blood transfusion group (14%) died with disease present and all the recurrences in this group were local. Conclusion: The use of intraoperative autologous blood transfusions during type III radical hysterectomy and lymphadenectomy appears to be safe and effective without compromising rates and patterns of recurrence. (Am J Obstet Gynecol 1999;181:1310-6.