Metastatic breast ductal carcinoma detected in a toremifene-associated endometrial polyp: case report and literature review

Uterine metastases are rare events, but when they occur, the first extragenital neoplasm responsible is breast carcinoma. Toremifene, used in breast cancer hormonotherapy, has a partial estrogenic agonist effect in the endometrium, responsible for potential abnormalities, like polyps. A 53-year-old woman, receiving toremifene due to previously excised breast ductal carcinoma, presented with endometrial thickness during an abdominopelvic ultrasound follow-up. Hysteroscopy revealed an endometrial polyp, which was removed. Microscopic examination showed infiltration by a malignant ductal pattern neoplasm, with signet ring cells. The patient underwent hysterectomy and bilateral salpingo-oophorectomy. The final pathological diagnosis was metastatic breast carcinoma to the endometrium and cervix. This is the first reported case of breast metastases detected in a toremifene-associated endometrial polyp.     

Metastatic uterine papillary serous carcinoma to the pericardium

BACKGROUND: Uterine papillary serous carcinoma is an aggressive tumor with a high propensity for distant spread. Metastases to the heart or pericardium are rare in gynecologic malignancies and usually fatal. CASE: A 64-year-old African American woman was diagnosed with recurrent uterine papillary serous carcinoma metastatic to the pericardium. Her case at presentation was significant for an elevated serum CA-125, evidence of metastatic disease to the liver, and massive cardiomegaly. Cytologic analysis of fluid obtained by pericardiocentesis confirmed recurrence. Despite treatment with paclitaxel and a pleuropericardial window, the patient succumbed to her disease. CONCLUSION: The prognosis for patients whose recurrent uterine papillary serous carcinoma has metastasized to the heart or pericardium is extremely poor. Effective adjuvant and salvage therapies are essential.     

Late recurrence of a uterine clear cell adenocarcinoma confined to an endometrial polyp: case report

A patient with an endometrial clear cell adenocarcinoma confined to a polyp developed recurrent disease in the abdomen and pelvis four years following hysterectomy. Treatment issues related to this uncommon clinical situation are discussed.     

Therapeutic approaches to uterine papillary serous carcinoma: a preliminary report

Uterine papillary serous carcinoma (UPSC) is a recently identified and characterized unique histopathologic subtype of endometrial cancer. Unlike the more common types of endometrial cancer, UPSC has a high likelihood of transperitoneal seeding and upper abdominal recurrence. Since our initial report of 26 patients with UPSC, an additional 10 patients with FIGO stage I disease have been diagnosed, operatively staged, and managed by an individualized approach. Operative staging revealed 5 of the 10 patients to have more advanced disease than had been determined clinically. Adjuvant postoperative abdominopelvic radiation was administered to 6 patients, 4 of whom remain free of disease within the treated area. Two patients received adjunctive hormonal and chemotherapy; neither has recurred. Two patients received no adjunctive therapy. One of these failed initially in the vagina with subsequent recurrence in the lungs and supraclavicular nodes. The value of operative staging and selection of appropriate adjunctive therapy awaits additional patient accrual and follow-up.     

Adenosarcoma of an endometrial polyp in a 27-year-old nulligravida: a case report

BACKGROUND: Endometrial adenosarcoma is a rare tumor first described by Clement and Scully in 1974. It consists of benign or atypical neoplastic glands within a sarcomatous stroma and represents approximately 8% of all uterine sarcomas. Patients of a variety of ages are affected: the largest series reported 100 cases in patients aged 14-89 years, with the median age at presentation 58 years. Other case reports identified on a literature search also describe patients within this age range. The typical presentation is symptoms of abnormal vaginal bleeding. On examination, tissue protruding from the external cervical os is a common finding, with a smaller number of patients having an enlarged uterus or palpable pelvic mass. The standard management is total abdominal hysterectomy and bilateral salpingo-oophorectomy. However, difficulty arises when one is faced with this condition in a young nullipara wishing to preserve her fertility. CASE: Endometrial polyp adenosarcoma occurred in a 27-year-old, nulligravid woman who presented with intermenstrual and postcoital bleeding. She was managed conservatively but will require close followup subsequently. CONCLUSION: A clinical dilemma was encountered in the management of a young woman with endometrial polyp adenosarcoma because of the necessity to preserve her fertility.     

Occurrence of endometrial glandular dysplasia precedes uterine papillary serous carcinoma.

Endometrial glandular dysplasia (EmGD) is a newly defined entity that is commonly and specifically associated with serous endometrial intraepithelial carcinoma and uterine papillary serous carcinoma (UPSC). Endometrial glandular dysplasia has been proposed as a true precancerous lesion of UPSC based on our recent studies showing morphological and molecular linkages between these 2 lesions. The present report is to examine if EmGD occurs before UPSC development and to define the period from the occurrence of EmGD to a full-blown UPSC by studying their clinicopathologic features in a retrospective setting. A total of 250 UPSC and 258 benign cases were used as initial study source. To identify if EmGD existed before the development of UPSC, we blindly reviewed all available endometrial biopsies from a period of 3 months or earlier before hysterectomies. These included an available pool of 27 biopsy specimens from UPSC group and 29 samples from benign control group. Any endometrial abnormalities, which morphologically qualified as EmGD as defined previously in preceding biopsies were recorded. Among all endometrial biopsies before hysterectomies, we morphologically identified a total of 10 EmGD cases; 9 (33%) of 27 were from UPSC group and 1 (3.5%) of 29 were from benign control group. All 10 morphologically diagnosed EmGD cases showed a high p53 staining score (>/=5) except 1 noncontributory from UPSC group and 1 from the benign control group with a score of 0. A high MIB-1 index score was seen in all EmGD cases, whereas low index was found in morphologically benign biopsies. The main purpose of this study is to report these retrospectively identified EmGD cases. The period from identifying EmGD to the presence of either a serous endometrial intraepithelial carcinoma or a full-blown UPSC ranged from 16 to 98 months with an average of 33 months. We conclude that occurrence of EmGD precedes the development of UPSC. The findings support our recently proposed UPSC development model, in which EmGD is likely to be a precursor lesion of UPSC. Further studies are needed to address issues in regard to molecular and cellular mechanisms, reversibility, risk of UPSC development, and clinical management of EmGD.     

Stage IV micropapillary serous ovarian carcinoma: a report of three cases.

Micropapillary serous carcinomas (MPSCs) have been distinguished from typical ovarian serous borderline tumors. Although the clinical features of MPSCs have been described in several studies, there is almost no clinicopathologic information regarding stage IV MPSC patients. We describe three cases of stage IV invasive MPSC with clinical and pathologic findings. One case had an umbilical metastasis (Sister Mary Joseph's nodule), and the other two cases had cytologically positive pleural effusions. These cases demonstrate the potential of MPSCs for aggressive clinical behavior and distant metastases.     

Low grade uterine stromal sarcoma and squamous cell carcinoma associated with an IUD: a report of 2 cases

BACKGROUND: Although intrauterine device (IUD) usage has not been linked to uterine cancer and has even been considered protective against neoplasia, sporadic cases of IUD-associated uterine malignancies have been reported. CASES: One cystic low grade stromal sarcoma of the uterus occurred in a 7-year IUD user, and 1 invasive endometrial squamous cell carcinoma following 25 years of IUD retention occurred in another. The latter case was notable for extensive in situ squamous cell carcinoma involving the entire uterine cavity and extending to the ectocervix and into both fallopian tubes. In situ hybridization for high-risk HPV DNA was negative. These findings raise the possibility of a non-HPV-related pathway for the squamous cell carcinoma case due possibly to prolonged chronic irritation and pyometra. CONCLUSION: The association of low grade stromal sarcoma with the IUD in the first case is extremely rare and most likely coincidental. The squamous carcinoma case, however, may have arisen via a non-HPV-related pathway, possibly related to IUD-induced chronic irritation and pyometra.     

34例子宫内膜浆液性乳头状癌临床病理分析

目的 探讨子宫内膜浆液性乳头状癌（uterine papillary serous carcinoma，UPSC）的临床病理特征，以及合理的治疗方法。方法 对2000年1月～2004年12月我院收治的34例UPSC进行回顾性研究，对其发病趋势、危险因素、临床表现、病理特征、目前诊断方法和治疗方案进行统计学分析。结果 Ⅲ、Ⅳ期患者占44．1％，深肌层及以上浸润者占70．6％。64．7％患者术后病理分期高于术前临床分期。91．2％患者雌孕激素受体阴性。全部患者手术治疗，绝大部分术后辅以化疗、放疗，生存率较以往文献提高，但仍较其他类型子宫内膜癌差。结论 UPSC不同于其他类型子宫内膜癌，其特殊的生物学行为和病理特征决定UPSC具有高度恶性表现。随着对其逐步认识，目前诊断和治疗均较以往有较大提高。     

Early stage papillary serous or clear cell carcinoma confined to or involving an endometrial polyp: outcomes with and without adjuvant therapy

Objective

To investigate clinical outcomes of stage IA uterine papillary serous (UPSC) and clear cell carcinoma (CC) arising from or associated with a polyp.

Methods

From 1995 to 2011, we identified 51 cases of stage IA UPSC (67%), CC (8%) or mixed histology (26%) endometrial cancer. Of these, 32 had disease confined to polyp (seven with no residual disease after hysterectomy), 14 had surface spread, 1 had myometrial invasion (MMI) and 4 had both. The majority of patients did not receive adjuvant therapy (80%). Patients given adjuvant treatment (either platinum-based chemotherapy alone, radiation alone, or a combination of the two) had incomplete staging or abnormal cytology.

Results

At mean follow-up of 58.3 months, only 4 patients had progressed, via pelvic adenopathy, carcinomatosis or both. There were no vaginal cuff recurrences. Kaplan–Meier 5 year estimates were pelvic control of 92.1%, disease-free survival 93% and OS 80.6%. Only 9% (3/32) of cases confined to polyp progressed. One responded to salvage chemoradiation, but two died despite salvage. Only 5% (1/19) of cases with surface and MMI progressed. On univariate analysis, only MMI and abnormal/positive cytology were significantly associated with increased pelvic recurrence (MMI p = 0.0059, cytology p = 0.0036) and worse DFS (MMI p = 0.0018, cytology p = 0.0054). Two patients given adjuvant treatment developed new gynecologic malignancies.

Conclusion

In our study, patients with limited UPSC/CC disease involving a polyp who have complete workup did well without adjuvant therapy, with recurrence rates similar to UPSC/CC stage IA disease. Late and extensive pelvic relapses may occur in the few who do relapse.     

Infectious pneumoperitoneum as an uncommon presentation of endometrial carcinoma: report of two cases

Two patients with pneumoperitoneum are reported; in both cases, the cause was severe infection of the upper genital tract. Investigation led to the finding of an underlying endometrial carcinoma. Literature review of the etiology of pneumoperitoneum, nature of the usual infecting organisms, and therapeutic principles are presented. Endometrial carcinoma should be considered in the differential diagnosis of infectious pneumoperitoneum, especially where patient risk factors are present.     

No BRCA1 germline mutation in a family with uterine papillary serous carcinoma: a case report.

The purpose of the study was to examine BRCA1 germline mutation and its relationship to BRCA1 expression in two patients, a mother and a daughter, both diagnosed with uterine papillary serous carcinoma (UPSC). DNA was screened for BRCA1 and BRCA2 germline mutations common in the Jewish population (185delAG, 5382insC, and 6174delT) by PCR-based assay and with a protein truncation test (PTT) to detect mutation in exon 11 of BRCA1 and exons 10 and 11 of BRCA2. BRCA1 expression in fixed tumor tissues was assessed by immunocytochemistry (IHC). No germline mutation in either BRCAI or BRCA2 gene was found in the two patients. Both samples showed reduced levels of BRCAI expression. Taken together, these results suggest that undetected or unscreened for germline mutation may be associated with occurrence of this rare tumor type in two members of the same family. Alternatively, an epigenetic mechanism such as BRCA1 promoter hypermethylation may be responsible for reduced expression of BRCA1 in the absence of DNA mutations.     

Metastasis from breast carcinoma to a tamoxifen-related endometrial polyp

BACKGROUND: Metastasis of extragenital neoplasms to an endometrial polyp is rare and until now, only 6 cases of such involvement has been described. CASE: A 58-year-old woman, who had been diagnosed 4 years ago with infiltrating ductal breast carcinoma and treated with surgery and tamoxifen therapy, was admitted to the gynecology clinic because of endometrial thickening observed during a routine abdominal ultrasonographic examination. A total hysterectomy with bilateral salpingo-oophorectomy was performed. Pathological examination of the specimen showed a large polyp which microscopically showed clusters of cells with signet ring morphology within the polyp stroma. The positivity of tumor cells for GCDFP-15 supported the diagnosis of metastatic breast carcinoma to endometrial polyp. CONCLUSION: Metastatic breast carcinoma should be considered in the differential diagnosis of carcinomas with signet ring cell morphology involving uterus.     

子宫内膜浆液性乳头状癌33例临床分析

目的 分析子宫内膜浆液性乳头状癌的临床病理特点,探讨其治疗及预后.方法 回顾性分析1996年1月-2005年12月在复旦大学附属肿瘤医院手术治疗的33例子宫内膜浆液性乳头状癌患者的临床病理资料.结果 33例患者中,手术病理分期Ⅰ期11例,Ⅱ期2例,Ⅲ期7例,Ⅳ期13例.早期患者（Ⅰ期和Ⅱ期）13例,占39%;晚期患者（Ⅲ期和Ⅳ期）20例,占61%.Ⅰ、Ⅲ、Ⅳ期患者的3年总生存率分别为77.4%、53.3%、12.1%,3者间比较,差异有统计学意义（P=0.008）.对手术病理分期、淋巴结受累、肌层浸润深度、病理分级、脉管浸润和p53基因表达情况与预后的关系进行统计学分析,单因素分析发现,仅手术病理分期（P=0.008）和肌层浸润深度（P=0.025）与预后有关;多因素分析发现,仅手术病理分期和病理分级与预后有关（P均＜0.05）.晚期患者中,行术后辅助化疗者的生存时间平均为30个月,明显长于未化疗者的6个月（P=0.014）.结论 子宫内膜浆液性乳头状癌确诊时多为晚期,预后差.治疗强调全面的手术分期,术后辅助化疗可改善患者的预后.     

BRCA2 germline mutation in a woman with uterine serous papillary carcinoma--case report

BACKGROUND: Recently, a high incidence of BRCA1 cancer predisposing mutation was described among patients with Uterine Serous Papillary Carcinoma (USPC). A BRCA2 germline mutation in a USPC patient has never been reported. CASE: A 65-year-old Ashkenazi Jewish woman was diagnosed with USPC Stage III A. The patient family history included a mother with ovarian carcinoma, a maternal aunt who had breast carcinoma that was diagnosed at an early age, an additional maternal aunt who suffered from gastric carcinoma, and the patient's sister who had duodenal carcinoma. The patient was found to be a carrier of the germline BRCA2 cancer predisposing mutation (6174delT). CONCLUSIONS: We report the first case of a BRCA2 mutation in a USPC patient. A strong family history of breast and ovarian cancer with the presence of the BRCA2 germline mutation is an additional hint for the possible association between BRCA cancer predisposing mutations and USPC.     

Papillary serous adenocarcinoma of the uterine cervix: a case report

Papillary serous adenocarcinoma of the endocervix (PSAE) is a rarely encountered neoplasm. The literature includes only a limited number of well documented case studies. The present study reports a case of papillary serous adenocarcinoma originating from the endocervix.     

The use of P53, PTEN, and C-erbB-2 to differentiate uterine serous papillary carcinoma from endometrioid endometrial carcinoma

Endometrial adenocarcinoma is the most common pelvic genital malignancy in the western world. The most common subtype of endometrial cancer is endometrioid endometrial carcinoma (EEC), which has a relatively good prognosis. Uterine serous papillary carcinoma (USPC) is also a subtype of endometrial carcinoma. This is an aggressive carcinoma with the majority of patients presenting at stage 3-4 and has a worse prognosis stage for stage when compared with EEC. In addition, the treatment of USPC is more extensive than that of EEC, and therefore definitive diagnosis before surgery ensures the optimum management for the patient. This study aims to determine whether P53, C-erbB-2, and PTEN antibodies have a use in the diagnosis and distinction of these cancers. We created tissue microarrays for 35 cases of EEC and 25 cases of USPC, and then we assessed the immunohistochemical expression of P53, C-erbB-2, and PTEN. There was significantly greater expression of P53 in USPC than that in EEC. However, neither C-erbB-2 nor PTEN showed any significant difference in expression between the two carcinomas. P53 may have a role in the diagnosis of USPC, but neither C-erbB-2 nor PTEN would be useful as part of a diagnostic panel.