Malignant Peripheral Nerve Sheath Tumour of Uterine Cervix-a Case Report and Review of Literature.

Malignant peripheral nerve sheath tumour (MPNST) is a rare variety of sarcoma occurring in the uterine cervix. Only 16 cases have been reported in literature till date. There is no clear consensus on the treatment due to the rarity of the disease. This is primarily managed by surgery. Here, we present a case of MPNST of the cervix who was treated with preoperative radiotherapy followed by radical hysterectomy and bilateral salpingo-oophorectomy.

Adenoid Cystic Carcinoma of Vulva-11 Years' Single-Institution Experience.

INTRODUCTION: Adenoid cystic carcinoma of vulva (ACC-vulva) is an extremely rare entity with <100 cases reported in the literature so far. OBJECTIVE: To study the clinical profile and outcome of ACC-vulva treated at a tertiary cancer care centre in South India. METHODS: This is a retrospective, record-based study of histopathologically confirmed cases of ACC-vulva treated at our centre from January 2005 to March 2016. RESULTS: Only four patients were diagnosed with ACC-vulva during the 11-year period under study. The longest duration of follow-up was 129 months. The age at diagnosis ranged from 32 to 43 years, with a median of 40 years. All patients were married, parous and premenopausal and presented with a painless unilateral vulval swelling. All patients had involvement of the Bartholin's gland site with normal overlying skin. In all patients, wide excision was performed. Unilateral inguinal node dissection was done in one case. Perineural infiltration was documented in two cases, while positive excision margins were present in three cases. None of the patients had any lymph node involvement at diagnosis or during follow-up. Two patients had recurrence of disease. The disease-free interval was 23 months for one patient and 118 months for the other. In both, local (vulval) and distant (multiple lung) metastases were detected simultaneously. CONCLUSION: Adenoid cystic carcinoma of vulva is an extremely rare, slowly progressing neoplasm mostly involving the Bartholin's gland. The usual treatment includes wide excision and adjuvant radiotherapy (if required). There may be late local and distant recurrence.

Role of laparoscopy in predicting surgical outcomes in patients undergoing interval cytoreduction surgery for advanced ovarian carcinoma: A prospective validation study.

OBJECTIVE: The objective of this study was to evaluate the safety and efficacy of laparoscopy in detecting inoperable disease in patients undergoing interval cytoreduction (ICR) for advanced ovarian carcinoma (AOC). The primary outcome measured was the performance of laparoscopy-based predictive index value (PIV) score developed by Fagotti et al. The secondary outcomes measured were performance of individual parameters included in PIV score and optimal cytoreduction (OCR) rates in our population. PATIENTS AND METHODS: This is a single-arm, prospective validation trial. Patients undergoing ICR for AOC in our institution were evaluated prospectively with laparoscopy before planned attempt at debulking surgery. Seven laparoscopic parameters included in laparoscopic PIV score were evaluated. Laparoscopic findings were compared with the final outcomes of definitive surgery. OCR was defined as residual disease <1 cm. The efficiency of the individual laparoscopy score was analyzed using receiver operating characteristic (ROC) curves. RESULTS: A total of 73 patients planned for ICR for AEOC were included in the study. Laparoscopic PIV score could successfully predict inoperability in 12 (16.4% of total study population) out of 14 inoperable patients in the total population and thus could avoid 85% of unsuccessful surgeries at a PIV score cutoff of ≥8. Performance of individual parameters included in PIV score was also evaluated. Two parameters out of seven, that is, mesenteric retraction and stomach infiltration had poor performance on ROC curve. Modified PIV score was calculated for each patient after excluding these two parameters. Modified PIV score had similar performance as Fagotti's PIV score at cutoff ≥6 (P = 0.728, for difference in area under the curve). No staging laparoscopy-related serious adverse events were noted in any of the patients. CONCLUSIONS: Laparoscopy is a safe, effective, and accurate method for predicting inoperability in patients undergoing ICR for AEOC.

Scar recurrence of uterine sarcoma: a case report.

BACKGROUND: Uterine leiomyosarcoma is an aggressive tumor that has a propensity for recurrence. Most of the recurrences occur at either pelvic or distant sites, such as lung or liver. Recurrences in the laparotomy scar are extremely rare. CASE: A 52-year-old woman underwent hysterectomy for stage I, grade 2 uterine leiomyosarcoma. She did not receive any adjuvant treatment. She presented 2 years later with wound recurrence. She had no evidence of intraabdominal disease. She underwent radical resection with mesh repair. Pathology revealed high grade spindle cell sarcoma very similar to the previous cancer. After completion of 18 months of follow-up, she was disease free. CONCLUSION: Radical resection of isolated metastases may be of benefit for these patients.

Extra Mammary Paget's Disease of Vulva-a Case Report.

Extra mammary Paget's disease (EMPD) is a rare condition involving the vulva, anogenital region, and axilla. Vulvar disease usually presents as a slow growing well-defined itchy plaque with crustations or ulcerations over the affected area in postmenopausal women. Well-established guidelines for diagnosis and management are not available for this rare condition. Our patient is a 64-year-old postmenopausal woman with a history of similar complaints of 2 years duration, not responding to multiple topical treatments. She was diagnosed with EMPD on incisional biopsy and treated with surgery at our centre.

Role of frozen section in intraoperative assessment of ovarian masses: a tertiary oncology center experience.

Surgical management of ovarian lesions vary considerably depending on the nature of the lesion. As the preoperative imaging and serum tumor marker levels are of limited value in the proper categorization of ovarian lesions, intraoperative pathological assessment is commonly requested for a primary diagnosis. Aim of the study is to assess the accuracy of the frozen section in the diagnosis of ovarian masses in our center and to analyze the causes of diagnostic discrepancies. In this retrospective study, frozen section diagnosis of 233 cases of ovarian masses was compared with the permanent section diagnosis. The overall accuracy of frozen section was 91.85 %. The sensitivity of frozen section diagnosis for benign, borderline and malignant tumors was 99.2, 88.46 and 82.95 % respectively. The corresponding specificity was 96.5, 93.23 and 99.3 %. There were 19 discordant cases including 18 false negative cases and one false positive case. Frozen section is an important diagnostic tool to determine the nature of ovarian masses. Careful macroscopic examination, evaluation of multiple sections along with clinical and radiological findings helps to reduce false positive and false negative results. Frozen section examination has limitations especially in cases of borderline tumors. This modality is most effective when the pathologist and surgeon are aware of the advantages and limitations.