A Constellation of Rare Gynecological Malignancies and Familial Adenomatous Polyposis Gastrointestinal Adenocarcinoma: A Case Report.

Ovarian microcystic stromal tumors (MST) are a rare subtype of sex-cord stromal tumors. We are presenting a case of a MST arising in a patient with familial adenomatous polyposis (FAP) and concurrent colonic adenocarcinoma. During the patient's workup of an ampullary adenoma associated with her FAP, she was found to have an enlarged uterus with a thickened endometrium and an incidental pelvic mass on the fundus of the uterus. Subsequent imaging identified heterogenous bulky ovaries. This patient underwent surgical resection including a total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, bilateral pelvic sentinel lymph node biopsy during her planned total proctocolectomy and transduodenal ampullectomy. Extensive histologic and immunohistochemical investigations were completed and the final pathology report revealed a unique compilation of International Federation of Gynecology and Obstetrics Stage II, grade 1 endometrioid endometrial adenocarcinoma, bilateral ovarian MST, a sperate pedunculated mass favoring a diagnosis of uterine tumor resembling ovarian sex cord tumor (UTROSCT), 2 distinct adenocarcinomas of the colon (T2N0 and T1N0) and a tubular adenoma of the ampulla. The pathology showed the endometroid adenocarcinoma was ß-catenin negative while the MST and UTROSCT both showed nuclear positivity with ß-catenin. To our knowledge this is the first reported case of a UTROSCT with concurrent endometrial adenocarcinoma presenting with bilateral ovarian MST's and adenomatous polyposis coli gene positive FAP colon adenocarcinoma.

Pegylated Liposomal Doxorubicin Is an Active Agent for Chemotherapy-Resistant Choriocarcinoma: A Report of Two Cases.

BACKGROUND: Despite advances in chemotherapy, radiation, surgery, and supportive treatments, a significant proportion of high-risk metastatic gestational trophoblastic disease patients develop resistant disease and die. Of those cured, protracted treatments can lead to long-term morbidity or later toxicity and death. Here we describe 2 patients with brain metastases who failed multiple lines of standard chemotherapy and radiation but had complete response to pegylated liposomal doxorubicin (PLD). CASE 1: A 35-year-old woman presented with choriocarcinoma in the brain, lungs, and subcutaneous tissues 11 months after full-term delivery. Her FIGO risk score was 14. Over 3 years she was treated with EMA-CO, EMA-CE, Taxol, gemcitabine, brain radiation, and excisional craniotomy for recurrent choriocarcinoma. She showed complete response of choriocarcinoma brain metastases following 2 cycles of PLD. She was choriocarcinoma free until her death 9 months later from acute myelogenous leukemia. CASE 2: A 52-year-old multigravid woman presented with choriocarcinoma 3 years following miscarriage. Her FIGO score was 16. Over 18 months she was treated with EMA-CO, TP/TE and IT MTX, and radiation. Her disease proved resistant and midbrain tumor unresectable. She showed complete response to PLD following 3 cycles but ultimately died from neurologic complications. CONCLUSION: PLD is an active agent in the treatment of high-risk choriocarcinoma.

The Investigations Required Before Referring a Patient to a Gynaecologic Oncologist.

OBJECTIVE: To provide guidance for referring physicians regarding what gynaecologic oncologists want and do not require in the referral package for a new patient. METHODS: An email survey was circulated to all members of the Society of Gynecologic Oncology of Canada (GOC) asking what they felt was required in a new patient referral package so that they could provide a timely consultation and management plan. RESULTS: The survey had a 79% response rate among 121 GOC members. Before referral of patients with endometrial cancer, 50% of respondents did not want additional investigations; only 4% wanted an MRI performed prior to them seeing the patient. For patients with high-grade cancers of the uterus (including serous), 40% wanted to see the patient without further investigations, while 42% wanted a CT scan report to be included in the referral package. For patients with cervical cancer, 56% of respondents wanted to see the patient without any further investigations, while 24% wished to have an MRI report included in the referral package. For patients with vulvar cancer, 50% of respondents did not want any further investigations; for patients with a pelvic mass, the majority of respondents wanted a serum CA 125 level in the referral package, while 0% to 3% only wanted an MRI. The preferred modality for imaging of the chest was a chest X-ray only. CONCLUSION: Our survey indicated that gynaecologic oncologists want little information in the referral package beyond the biopsy result. MRI is not required in the workup of most patients with a pelvic mass or uterine cancer.

RETIRED: The role of surgery in endometrial cancer.

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Initial evaluation and referral guidelines for management of pelvic/ovarian masses.

OBJECTIVES: To optimize the management of adnexal masses and to assist primary care physicians and gynaecologists determine which patients presenting with an ovarian mass with a significant risk of malignancy should be considered for gynaecologic oncology referral and management. OPTIONS: Laparoscopic evaluation, comprehensive surgical staging for early ovarian cancer, or tumour debulking for advanced stage ovarian cancer. OUTCOMES: To optimize conservative versus operative management of women with possible ovarian malignancy and to optimize the involvement of gynaecologic oncologists in planning and delivery of treatment. EVIDENCE: Published literature was retrieved through searches of PubMed or MEDLINE, CINAHL, and the Cochrane Library, using appropriate controlled vocabulary and key words. Results were restricted to systematic reviews, randomized control trials/controlled clinical trials, and observational studies. Grey (unpublished) literature was identified by searching the web sites of health technology assessment and health technology assessment-related agencies, clinical practice guideline collections, clinical trial registries, and national and international medical specialty societies. RECOMMENDATIONS: 1. Primary care physicians and gynaecologists should always consider the possibility of an underlying ovarian cancer in patients in any age group who present with an adnexal or ovarian mass. (II-2B) 2. Appropriate workup of a perimenopausal or postmenopausal woman presenting with an adnexal mass should include evaluation of symptoms and signs suggestive of malignancy, such as persistent pelvic/abdominal pain, urinary urgency/frequency, increased abdominal size/bloating, and difficulty eating. In addition, CA125 measurement should be considered. (II-2B) 3. Transvaginal or transabdominal ultrasound examination is recommended as part of the initial workup of a complex adnexal/ovarian mass. (II-2B) 4. Ultrasound reports should be standardized to include size and unilateral/bilateral location of the adnexal mass and its possible origin, thickness of septations, presence of excrescences and internal solid components, vascular flow distribution pattern, and presence or absence of ascites. This information is essential for calculating the risk of malignancy index II score to identify pelvic mass with high malignant potential. (IIIC) 5. Patients deemed to have a high risk of an underlying malignancy should be reviewed in consultation with a gynaecologic oncologist for assessment and optimal surgical management. (II-2B).

Efficacy of pegylated liposomal doxorubicin (PLD) plus carboplatin in ovarian cancer patients who recur within six to twelve months: a phase II study.

OBJECTIVES: Pegylated liposomal doxorubicin is one of the preferred alternatives for ovarian cancer patients with early relapse (<6 months) and taxane/carboplatin for late relapse (>12 months), but the optimal therapy for the partially platinum-sensitive (6-12 months) population has not been defined. This single-arm phase II trial was designed to assess the efficacy of pegylated liposomal doxorubicin (PLD)/carboplatin in ovarian cancer patients who relapse between 6 and 12 months after initial treatment with platinum-based chemotherapy. METHODS: Ovarian cancer patients who previously completed a course of therapy with paclitaxel/carboplatin were administered PLD 30 mg/m(2) followed by carboplatin AUC 5 mg/mL/minute every 4 weeks. RESULTS: Fifty-eight patients were enrolled in the study and 54 were eligible for the efficacy analysis, of whom most (75%) received at least 6 cycles of PLD/carboplatin. The objective response rate was 46% (4% CR and 42% PR), with an additional 33% experiencing disease stabilization >6 months. For those patients with measurable CA-125, the response rate was 66% (28% CR and 38% PR), with an additional 18% experiencing disease stabilization >6 months. Median time-to-progression was 10 months (1.5-25). Median overall survival was 19.1 months (2.2-38.9). The most frequent adverse effects were neutropenia, thrombocytopenia, and constipation. CONCLUSIONS: The combination of PLD/carboplatin is efficacious and well tolerated in women with partially platinum-sensitive ovarian cancer and represents a valuable alternative for patients who relapse within 6-12 months of completing paclitaxel/carboplatin chemotherapy.

Low-grade pap smears containing occasional high-grade cells as a predictor of high-grade dysplasia.

OBJECTIVES: This study compared clinical outcomes associated with Pap smear reports of atypical squamous cells cannot exclude HSIL (ASC-H) and those associated with "low-grade smears containing occasional cells suggestive of HSIL" (LSIL-H). In Alberta, women with ASC-H are referred for colposcopy, and women with LSIL are managed with follow-up Pap smears. LSIL-H is not included in the Bethesda classification and has not been characterized in terms of cytological features, reporting, management, and clinical course. METHODS: All ASC-H (n = 153) and LSIL-H (n = 189) Pap smears recorded in the regional laboratory information system between December 2000 and December 2001 were identified. All available histology for each associated patient over the subsequent two&year period was reviewed to determine if a high-grade histological lesion was ever confirmed in a biopsy, loop electrosurgical excision procedure (LEEP), or hysterectomy specimen. RESULTS: A high-grade squamous intraepithelial lesion was identified in 48% of the ASC-H group and 40% of the LSIL-H group (P = 0.136). Most of the HSIL histopathology was identified on the first colposcopic visit, suggesting that high-grade dysplasia may have been present at the time of the original Pap smear. CONCLUSIONS: Pap smears reporting ASC-H and LSIL-H predict a high-grade squamous intraepithelial lesion with similar accuracy. Since approximately 40% of patients with an LSIL-H Pap smear have either concurrent or subsequent high-grade cervical pathology, we also recommend immediate referral for colposcopy in this group of patients.