Sarcomatoid Squamous Cell Carcinoma Of The Vulva:A Case Report.

BACKGROUND: A sarcomatoid squamous cell carcinoma of the vul-va is a very rare malignancy of the female genital tract . It has been characterized as an aggressive disease typically associated with early development of local recurrences as well as distant metastases. CASE: A 77-year old woman presented to our hospital with an aggravated vulvar mass, complaining about pruritus and pain. The patient underwent a radical vulvectomy with bilateral inguinal lymph node dissection and bilateral pelvic lymph node sampling. The histo-pathologic examination was consistent with a sarcomatoid squamous cell carcinoma of the vulva with an identical microscopic and im-munohistochemical appearance. The FIGO stage was IB and further management was decided to be radiotherapy. CONCLUSION: A sarcomatoid squamous cell carcinoma is a rare entity with an aggressive biological behavior. To date, there have been only a few cases of the disease reported in the literature. Therefore a more systematic collection and closer study of such cases would be helpful for achieving an early diagnosis and more effective therapeutic strategies in the future.

Concurrent gynecologic surgery and panniculectomy in morbidly obese women with gynecologic cancer, a single-center experience.

PURPOSE: As the prevalence of obesity has been rising during the past decades worldwide and especially in Greece, surgeons have faced significant challenges concerning the treatment of morbidly obese women with gynecologic cancer. Panniculectomy is a safe procedure that offers better visualization of pelvic anatomy and prevention of major complications in these women. METHODS: Aim of this study is to describe a single-center experience of surgical treatment of morbidly obese women with gynecologic cancer and concurrent panniculectomy. We, also, review the literature for articles that report concurrent gynecologic surgery and panniculectomy, to summarize the complications that were encountered. RESULTS: From 2015 to 2018, 38 obese women were treated with concurrent panniculectomy at the time of gynecologic surgery for cancer in a single institution. Median age was 55.62 years old, median BMI 43.79 kg/m2, median blood loss 243.75 ml and median operative time approximately 200 min. Twenty-nine patients had endometrial cancer, one patient had concurrent endometrial cancer and fallopian tube cancer, six patients had borderline ovarian cancer, and two had adult granulosa ovarian tumour. Intraoperative and postoperative complications were documented. Five patients suffered from wound infection and were treated with antibiotics and one patient died after wound infection, wound dehiscence, and renal failure. CONCLUSION: For selected obese patients, concurrent panniculectomy with surgical treatment of gynecological cancer is a safe and efficient procedure.

Is fertility sparing surgery a treatment option for premenopausal patients with dysgerminoma?

Dysgerminoma is the most common ovarian germ cell type ovarian tumour. Primarily, it presents in young women at reproductive age and thus, the preservation of fertility is considered to be fundamental when it is possible for these patients. In comparison to the past the restriction of the extent of the surgical procedure as well as the introduction of innovative chemotherapeutic regimens improved significantly both, the prognosis and the clinical outcomes of this rare neoplasia. As dysgerminomas are extremely radio- and chemosensitive, fertility sparing approach and less aggressive operations should be favoured. We present a narrative review of the multispecialty fertility sparing surgical and medical approach for women with dysgerminoma (Ref. 21).

Uterobrush method in the detection of endometrial pathology.

BACKGROUND: Endometrial brush cytology is a widely accepted method for the detection of endometrial lesions. The aim of this study was to evaluate the role of cytological sampling using Uterobrush in the screening of endometrial pathology. PATIENTS AND METHODS: This is a prospective double-blind study evaluating the efficacy of the Uterobrush method (Cooper Surgical, Trumbull, USA) in the detection of endometrial abnormalities. Endometrial cytology was performed during the period January 2009 to April 2010 in all symptomatic patients that underwent dilatation and curettage. The collected samples were firstly smeared directly onto a glassslide and consequently into Thin-Prep buffer. Cytologic features were evaluated according to the criteria of Tao. The main objective was to evaluate the efficacy of Uterobrush method comparing the results of cytologic and histopathologic examination. RESULTS: The sample of the study consisted of 100 women aged 55.8 years (range 38-78 years) with recorded data regarding Uterobrush test and classic histologic examination. Fifty-five patients were postmenopausal. A total of 92% of the samplings were performed by trainees. Endometrial carcinoma was cytologically diagnosed in 8/9 patients, whereas endometrial polyps were diagnosed in 5/34 patients (14.7%). All the patients with simple hyperplasia were correctly diagnosed with the Uterobrush method, whereas the diagnosis of complex hyperplasia with or without atypia was correct in 85.7% and 100% of patients, respectively. Regarding endometrial carcinoma, the sensitivity, specificity, positive and negative predictive values were 88.9%, 100%, 100% and 98.9%, respectively. On the other hand, regarding endometrial polyps, the sensitivity, specificity, positive and negative predictive values were 14.7%, 100%, 100% and 69.5%, respectively. CONCLUSION: Uterobrush is a reliable direct intrauterine sampling for detecting endometrial abnormalities especially endometrial carcinoma and hyperplasia, but not endometrial polyps. It is a well-tolerated, easy to use method, which provides generous endometrial sampling without contamination from the endocervix or the vagina.

Paclitaxel-ifosfamide-carboplatin combination chemotherapy regimen in advanced uterine and adnexal malignant mixed Mullerian tumours.

BACKGROUND: Malignant mixed Mullerian tumours (MMMTs) of the uterus and adnexa represent aggressive gynaecologic malignancies with a high rate of loco-regional and distant failure. For that reason, we evaluated the paclitaxel-ifosfamide-carboplatin (TICb) combination in patients with advanced MMMTs. METHODS: Female patients with advanced MMMTs, WHO-PS 0-2, no prior chemotherapy for systemic disease, unimpaired haemopoietic and organ function were eligible. Chemotherapy was administered at the following doses; paclitaxel: 175 mg m(-2) on day 1, ifosfamide: 2.0 g m(-2) day(-1)--days 1 and 2, and carboplatin at a target area under the curve 5 on day 2, with prophylactic G-CSF from day 3. RESULTS: Forty patients of a median age 61 (45-72) years, performance status 0-2 with advanced MMMTs of the uterus (n=34), tubes (n=2) or ovary (n=4) have entered and all were evaluable for response and toxicity. Responses were as follows: 27 out of 40 (67.5%) evaluable patients responded, with 11 complete responses and 16 partial responses, while 10 had stable disease, and 3 developed progressive disease. The median response duration was 9 months (range, 4-40 months), median progression-free survival 13 months (range, 3-42 months), while median overall survival 18 months (range, 4-48 months). Grade 3/4 neutropenia was recorded in 22 out of 40 (55%)--with 13 developing grade 4 (≤7 days) and 7 out of 40 (17.5%) of patients at least one episode of febrile neutropenia. CONCLUSION: In this study, it appears that the TICb combination, yielded important activity with manageable toxicity in females with advanced MMMTs warranting further randomised comparison with current standard regimens.

Vulvar merkel carcinoma: a case report.

This is a new case of Merkel cell carcinoma of the vulva. It is a rare neuroendocrine carcinoma with an aggressive behavior. Because of its rarity in this location, it is not clear whether it behaves differently from the usual neuroendocrine carcinomas of the skin. A case of a 63-year-old patient with vulvar Merkel carcinoma is presented. The clinical presentation, microscopic and immunohistochemical features, and treatment are discussed.

The role of lymphadenectomy in uterine carcinosarcomas (malignant mixed mullerian tumours): a critical literature review.

BACKGROUND: Uterine carcinosarcomas are rare and highly aggressive tumours. Although surgery is the cornerstone of treatment, the extent of the procedure remains controversial. We sought to evaluate the available literature data regarding the rationale of lymphadenectomy and its possible impact on survival. METHODS: A systematic Medline, PubMed and Scopus search with special focus on the publications of the last decade. RESULTS: Carcinosarcomas have similar clinical characteristics and behaviour with grade 3 endometrioid or aggressive variants of uterine adenocarcinoma. All studies have demonstrated that the FIGO stage of disease is the most important prognostic factor, followed by the depth of myometrial invasion, extra-uterine spread and positive peritoneal cytology. Moreover, lymph node involvement will be found in 14-38% of patients undergoing lymphadenectomy. This figure is similar to the one reported for endometrial carcinoma. Therefore, lymphadenectomy is mandatory for staging purposes. Regarding its impact on survival, the majority of studies confirm a significant survival benefit. The possible mechanisms for the improvement of survival from lymphadenectomy include removal of micro-metastatic foci, reduction of recurrence risk (removal of "target tissue") and mechanical circumvallate of the disease. Given that 5-38% of the patients will experience local recurrence and 30-83% distant metastases, lymphadenectomy reduces the risk of the first and identifies patients in advanced stage that may benefit from adjuvant chemotherapy, aiming to reduce the second and ultimately improve overall survival. CONCLUSIONS: Our review data fully justifies the rationale of lymphadenectomy, which beyond staging information seems to offer a measurable survival benefit.

Evaluation of the paclitaxel-ifosfamide-cisplatin (TIP) combination in relapsed and/or metastatic cervical cancer.

BACKGROUND: Recurrent or metastatic cervical cancer represents an aggressive malignancy with a high rate of locoregional and distant failure. Therefore, we evaluated the three-drug combination of paclitaxel-ifosfamide-cisplatin (TIP). METHODS: Systemic chemotherapy-naive patients with advanced metastatic/relapsed cervical cancer and a World Health Organization (WHO) performance status (PS) of 0-2 were eligible. TIP chemotherapy doses were paclitaxel 175 mg m(-2) on day 1, ifosfamide 2.5 g m(-2) on days 1+2, and cisplatin 40 mg m(-2) on days 1+2, with prophylactic granulocyte-colony stimulating factor. RESULTS: A total of 42 patients with recurrent/metastatic cervical cancer are evaluable for response and toxicity: median age: 56 (25-74) years; PS: 1 (0-2); histologies - squamous: 35, adenosquamous: 5, and adenocarcinoma: 2. Responses were overall response rate (RR): 62% (95% confidence interval (CI): 47.3-76.7%), with complete response (CR): 26% (95% CI: 12.7-39.3%), and partial response (PR): 36% (95% CI: 21.5-49.9%). Responses according to the relapse site were overall RR: 32% (95% CI: 13.7-50.3%) within previously irradiated pelvis vs 75% (95% CI: 57.7-92.3%) in extra-pelvic sites. Median time to progression (TTP) was 7 (range, 2-34+) months and median overall survival (OS) was 16.5 (range, 3-36+) months. Toxicities included grade 3-4 neutropenia: 83% (21% febrile neutropenia), grade 3-4 thrombocytopenia: 9%, no grade 3 neuropathy (35% grade 2), grade 2 asthenia/fatigue 15%, and no treatment-related deaths. CONCLUSION: TIP is an active regimen with acceptable toxicity in advanced/relapsed cervical cancer.

Evaluation of the possible benefits of post-radiotherapy surgery after concomitant chemoradiotherapy with a new radio-sensitizing regimen (irinotecan / CPT-11, interferon A2b and amifostine) for advanced-stage cervical carcinoma. Preliminary results of a pilot phase-II study.

PURPOSE: This phase II pilot study was conducted to evaluate the results of a three-modality approach (which included post-chemoradiotherapy surgery) in advanced-stage cervical carcinomas. PATIENTS AND METHODS: Thirty-six patients underwent either surgery or were put on follow-up after having received radical cervical radiotherapy (RT) combined with radiosensitizing chemoimmunotherapy with irinotecan (CPT-11), interferon (IFN) A2b, and amifostine. The last selection (surgery or follow-up) was based on clinical evaluation (downstaged or not). Feasibility, morbidity, surgical outcome and survival were evaluated. RESULTS: Twenty-six patients had stage IIb and 10 IIIb disease at diagnosis. Sixteen (44%) were clinically downstaged, thus becoming eligible for surgery. Twelve (33%) were operated and the others were put on follow-up. There was no significant increase in treatment-related morbidity of the group of patients receiving three-modality therapy, since only one intraoperative complication had occurred. In 58% of the operated patients, chemoradiotherapy-resistant tumor was found on pathology of the cervical specimens, while 29% of them had lymph nodes infiltrated by the tumor. After a median follow-up of 42.5 months, overall survival (OS) of operated vs. non-operated patients (88 vs. 56%, respectively) show only a trend toward significance (p=0.10). The overall recurrence/metastasis rate was 36.1% and the disease-free survival (DFS) 56% for operated vs. 76% for non-operated patients, respectively (p=0.63). CONCLUSION: These results indicate that post-chemoradiotherapy surgery is justified because of the high rate of residual disease found. Morbidity can be effectively limited with proper patient selection. A considerable survival benefit is expected, although this remains to be confirmed with phase III studies.

A two eterochronous primary gynaecological malignancies of different origin.

AIM: The objective of our study is to present a rare case of two eterochronous primary gynaecological malignancies. CASE: A 65-year-old para-2, white obese female, presented in our department 4 years ago, due to a single event of vaginal spotting. Curettage revealed an endometrial cancer. A total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed. Histology showed an endometrioid adenocarcinoma of endometrium stage Ib, moderately differentiated. No additional therapy was given. Twenty seven months later, a pathologic Papanicolaou smear came out on her routine follow-up. Although, recurrence on vaginal cuff was possible, the biopsies of anterior vaginal wall showed a poorly differentiated squamous cell carcinoma of the vagina. An exploratory laparotomy was performed, but tumor resection was not possible. The patient was classified as stage II vaginal carcinoma and underwent complete radiotherapy and chemotherapy. CONCLUSION: This case indicates that female genital carcinomas of different histological origins may occur with minimal time-interval, even in the absence of known predisposing factors like previous chemo-radiotherapy, HPV infection or diethylstilbestrol exposure. The role of close follow up of hysterectomised patients should also be mentioned (Fig. 1, Ref. 15). Full Text (Free, PDF) www.bmj.sk.

Primary peritoneal serous papillary carcinoma: clinical and laboratory characteristics.

BACKGROUND: Primary peritoneal papillary serous carcinoma (PPPSC) is an uncommon primary malignancy of the peritoneum. The aim of our study is to present the characteristics of such a rare entity through our case series. METHOD: This is a retrospective study of nine cases of PPPSC who were treated between January 2002 and April 2007 in METAXA Memorial Cancer Hospital, Piraeus, Greece. Medical files and histopathological diagnosis of each patient were retrospectively studied. RESULTS: The median age of the patients was 63 years ranging from 44 up to 74 years. Clinically PPPSC presented with general abdominal discomfort in all of the patients. Three out of nine patients presented with constipation, 5/9 with distention, and 7/9 with ascites. All of the patients referred loss of appetite with weight gaining due to ascites. All the patients had pathological values of CA125 (over 35 U/ml) ranging from 125 up to 1,255 U/ml with median value 565 U/ml. Optimal debulking was possible in 3/9 of patients who were consequently treated with standard taxol-platin chemotherapy. Complete response was achieved in one woman. The median disease-free survival was 7 months and the median overall survival rate was 2.5 years. CONCLUSION: PPPSC mimics ovarian papillary serous carcinoma regarding the clinical and laboratory characteristics but it has worse prognosis.

Accidential detection of salpinx carcinoma after omphalocele repair.

BACKGROUND: Carcinoma of the fallopian tube is a rare disease accounting for < 1% of all gynecologic malignancies. CASE: We present the first case of an accidental diagnosis of fallopian tube carcinoma after omphalocele repair. CONCLUSION: Between 9 to 14% of cases are incidental findings during the work-up or exploratory laparotomy. A review of the literature regarding predisposing factors, histology, clinical and ultrasound appearance, treatment as well as survival rates was also presented (Tab. 1, Ref. 33).

The histological outcome of glandular dyskaryosis--AGUS--reported in Papanicolaou smears.

PURPOSE: The objective of this study was to investigate the prognostic significance of glandular dyskaryosis/AGUS, reported in Papanicolaou (Pap) smears. MATERIALS AND METHODS: During a 4-year period 26,408 smears were assessed at the Department of Cytology of our hospital. Thirty (0.11%) smears were reported as having glandular dyskaryosis. The studied material was taken by colposcopy, fractional curettage and/or cone biopsy. RESULTS: The final diagnosis included 2 cases of invasive cervical carcinoma (1 squamous cell and 1 adenocarcinoma), 1 case with ovarian cancer, 8 cases with high grade squamous intraepithelial lesion (HGSIL) and 10 cases with other nonmalignant pathology (polyps, hyperplasia) of endometrial origin. These findings reflect a 36.7% positive predictive value for significant squamous and glandular pathology. CONCLUSION: Patients with glandular dyskaryosis require further evaluation because it may hide serious pathology from all internal genital organs. Colposcopy in combination with fractional curettage and/or cone biopsy are proposed as the appropriate diagnostic tools in women with such cytological abnormality.

Laparoscopic pelvic exenteration: a new option in the surgical treatment of locally advanced and recurrent cervical carcinoma.

Pelvic exenteration is an option in the treatment of persistent or recurrent cervical carcinomas confined to the central pelvis. The improvement of laparoscopic techniques and equipment in combination with broader experience of surgeons in laparoscopy made laparoscopic pelvic exenteration possible. This article reviews present experience with pelvic exenteration and analyses the advantages and disadvantages of the method compared to classical method (Tab. 2, Ref. 10).

Late struma ovarii diagnosis many years after total thyroidectomy. A rare entity.

We present the case of a 53-year-old lady with incidental diagnosis of struma ovarii 10 years after total thyroidectomy due to papillary thyroid carcinoma. The disease was diagnosed owing to high levels of serum thyroglobulin. The patient underwent exploratory laparotomy with total hysterectomy and bilateral salpingo-oophorectomy. Close follow-up was proposed and her prognosis is excellent.

Extragastrointestinal stromal tumor mimicking a uterine tumor. A rare clinical entity.

Gastrointestinal stromal tumors are the most common mesenchymal tumors of the gastrointestinal tract. These tumors are present in almost all case mutations of KIT-CD117. When located in different places other than the gastrointestinal tract they are called extragastrointestinal stromal tumors (EGISTs). We present the case of a 70-year old patient with abdominal pain. Computed tomography (C/T) and ultrasound (U/S) indicated the existence of a hypoechoic enlarged 9.6 x 10 cm uterus due to leiomyoma. The clinical condition of the patient deteriorated and obtained the characteristics of an acute abdomen. The patient underwent urgent exploratory laparotomy, which revealed the incidental existence of a large tumorous formation in the pouch of Douglas. A total abdominal hysterectomy, bilateral ovarectomy, omentectomy, and tumor resection from the rectouterine pouch were performed. Histology analysis confirmed the diagnosis of a malignant EGIST. EGISTs are infrequent in pelvis. In our case, pelvic EGIST led to an acute pain symptomatology obtaining characteristics of invasive uterine leiomyosarcoma. Pelvic imaging with classical methods of U/S and C/T may lead to confusion and false diagnosis. To the best of our knowledge, this is the first case report about an EGIST located in the rectouterine pouch of Douglas leading to acute abdomen symptomatology.

Thermal balloon endometrial ablation: a systematic review.

OBJECTIVE: The aim of our study is to review the role of thermal balloon endometrial ablation (TBEA) as an alternative in treating abnormal uterine bleeding. METHODS: Articles relevant to our review and relevant references from the initially identified articles on the field that were archived by May 2007, were retrieved from Pubmed. RESULTS: Success rates ranged from 83 up to 94%, with patient's satisfaction ranging from 57 up to 94%. Persisted menorrhagia could reach 17% in some studies. CONCLUSION: TBEA is an effective alternative method used in the treatment of menorrhagea which results in a significant reduction in menstrual bleeding and high satisfaction rates. However, a longer follow-up is required to determine the role of such a treatment.

Meig's or Pseudomeig's syndrome?

The triad of ascites, hydrothorax in association with a benign ovarian tumor is defined as Meig's syndrome. It is a rare clinical entity. A case of a 62 year-old woman whith dyspnoe, abdominal discomfort and ascites is presented. Clinical and ultrasonographic findings revealed extended palpable pelvic mass originating from the ovaries and ascites as well as hydrothorax of the left lung by chest radiography. The treatment method was surgical intervention. Cytomorphologic studies were positive for malignacy and adenocarcinoma cells were confirmed. The pathogenesis of the pleural and ascites fluids and the importance of CA-125 are discussed (Fig. 2, Ref. 21).

Polymyositis in a patient with recurring ovarian cancer and history of unrelated breast cancer.

BACKGROUND: Polymyositis (PM) is an idiopathic inflammatory myopathy. Occasionally, it may present as a paraneoplastic syndrome and it is strongly associated with ovarian and weakly with breast cancer. We present here a case of a 60-year-old patient with sequential breast and ovarian (second primary) carcinomas followed by PM as a paraneoplastic disorder. CASE REPORT: The patient had been diagnosed with stage I breast carcinoma 3 years ago and had been treated with conservative surgery followed by radiotherapy and six cycles of chemotherapy (CMF). One and a half year later an ovarian carcinoma was diagnosed for which the patient underwent abdominal hysterectomy oophorectomy and omentectomy. The pathological report characterized it as second primary. Adjuvant chemotherapy with carboplatin and taxol was administered. Fourteen months after the initial laparotomy, the patient was re-operated due to ovarian carcinoma recurrence which was involving all lesser pelvis organs. After a successful radical removal of the recurrence the patient developed a fully expressed PM. This case serves to remind that this disease can occur as a paraneoplastic disorder.