Papillary cystadenocarcinoma of the lacrimal gland after radiation for bilateral retinoblastoma.

The authors report a case of papillary cystadenocarcinoma of the lacrimal gland after irradiation for bilateral retinoblastoma. A 32-year-old man with a history of bilateral retinoblastoma, diagnosed shortly after birth, was treated with enucleation of the OS and a single session of radiation to the OD. Over 30 years later, he presented with an orbital mass of the right lacrimal gland that on biopsy demonstrated papillary cystadenocarcinoma.

Case of uterine papillary serous carcinoma following tamoxifen treatment that could not be diagnosed during screening.

This case involved a 69-year-old woman who had been taking tamoxifen for 5 years after breast cancer surgery. She was referred to our clinic for endometrial cancer screening when tamoxifen was first prescribed. Subsequently, transvaginal ultrasonography and endometrial cytology were performed every 6 months. Despite these regular examinations, stage IVb papillary serous carcinoma was detected 8 months after the end of tamoxifen administration. Total abdominal hysterectomy was performed, but only a small polyp was seen upon macroscopic examination of the uterus. However, papillary serous carcinoma was found microscopically in almost all lymphovascular spaces in the uterus from the endometrium to the serosa. On the surface of the polyp, only endometrial intraepithelial carcinoma with positive immunostaining for p53 was detected. Chemotherapy, including a platinum compound, was administrated, but unfortunately it was ineffective and the patient died of her disease 14 months after the operation.

Serous papillary cystadenocarcinoma arising from autografted ovary of the abdominal wall.

A 58-year-old-woman developed a serous papillary cystadenocarcinoma between the fascia and peritoneum of the left abdominal wall. The patient had undergone bilateral oophorectomy for serous cystadenoma 17 years earlier and her residual normal ovarian parenchyma had also been transplanted to the abdominal wall. Grossly and microscopically, the current tumor arises from the autografted ovarian parenchyma. Literature review indicates that carcinoma arising from autografted ovarian tissue is extremely rare.

Malignant transformation of an intraaxial-supratentorial neurenteric cyst - case report and review of the literature.

Neurenteric cysts are "rare benign mass forming developmental abnormalities" that usually affect young adults. Neurenteric cysts are thought to be derived from primitive endoderm, and form as a result of faulty endodermal-notochordal separation at 3 weeks of embryogenesis. Neurenteric cysts are lined by simple-to-pseudostratified respiratory/gastrointestinal-like epithelium; as such, these lesions closely resemble colloid and Rathke's cleft cysts. Anatomically, neurenteric cysts most frequently arise in an intradural-extraaxial location anterior to the cervical-thoracic spinal cord. Intracranial neurenteric cysts are uncommon but have a tendency to reside in the infratentorial compartment. Malignant transformation of the epithelial component of neurenteric cysts is decidedly rare. Of the 3 reported cases of neurenteric cysts with malignant transformation, all were intracranial (2 infratentorial and 1 supratentorial) and extraaxial. We describe a 58-year-old female with a supratentorial-intraaxial lesion that is consistent with a neurenteric cyst exhibiting malignant transformation into an invasive mucinous papillary cystadenocarcinoma. Areas of direct transition between typical benign neurenteric cyst epithelia and malignant epithelia (i.e., carcinoma in situ), highlighted by an abrupt change in the Ki-67 proliferative index, were identified, and supported the primary nature of this brain neoplasm. Metastatic workup at the time of presentation was unremarkable, and immediately up until being lost to follow-up 38 months after gross total resection, routine follow-up MR imaging had not detected a recurrence. To our knowledge, this would be the first reported case of malignant transformation within a supratentorial-intraaxial neurenteric cyst.

Rapidly increasing incidence of papillary serous carcinoma of the peritoneum in the United States: fact or artifact?

Papillary serous carcinoma of the peritoneum (PSCP) has been recognized for almost 5 decades, but little is known about the etiology or pathogenesis of this uncommon malignancy. The objective of this analysis was to examine trends in the incidence of PSCP in the United States. Invasive PSCP cases (N = 4,389) were identified through 24 population-based registries in the United States during the period 1995-2004. Incidence rates were calculated per million population. PSCP is a disease of older women, with few cases diagnosed before the age of 40 years. The incidence of PSCP was 64% lower among black women and 47% lower among Asian-Pacific Islander women compared with white women. Rates among Hispanic women were 39% lower than among non-Hispanic women. The majority of PSCP (68%) was diagnosed at a distant stage, underscoring the difficulty of diagnosing this malignancy. The incidence of PSCP has increased dramatically during the past decade in the United States with the greatest rise (>13% per year) among non-Hispanic and white women. This trend was more pronounced among older women and women with early stage disease. The incidence of PSCP shows substantial racial and ethnic diversity. The increase in the rate of PSCP among all racial and ethnic groups during the 10-year observation period is cause for some alarm. Although the reason for this temporal trend is unknown, some of the increase may be attributable to reclassification of ovarian carcinoma to the peritoneum.

Uterine papillary serous carcinoma: a review.

The purpose of this article is to review the available literature for uterine papillary serous carcinoma (UPSC). A literature search was conducted to identify publications on UPSC. The literature on UPSC is composed mainly of retrospective, single-institution reports. Despite these limitations, several recommendations can be made. When UPSC is confirmed on preoperative biopsy, complete surgical staging should be performed. Although whole abdominal radiotherapy has a limited role in early-stage UPSC, there may be a role for postoperative chemotherapy in early-stage UPSC. In the setting of optimally debulked advanced-stage disease, a combination of radiation and chemotherapy may be indicated. In the setting of recurrent or suboptimally debulked advanced disease, a platinum-based regimen is recommended. Although comprising a minority of the women with endometrial cancer, women with UPSC do account for a disproportionate percentage of the recurrences. There is a need for clinical trials to determine the optimal therapy for this cohort of patients.

Malignant degeneration of perineal endometriosis in episiotomy scar, case report and review of the literature.

Although malignant degeneration of cutaneous endometriosis is rare at only 0.3-1% in endometriosis surgical scars, diagnosis and management need to be defined. A case of malignant degeneration of perineal endometriosis is reported, with a review of literature. Physiopathology, epidemiological data, diagnostic and therapeutic methods are discussed for malignant degeneration of cutaneous endometriosis. Any scar lesion that evolves in response to the menstrual cycle should be considered endometriosis until proven otherwise, and thus could require surgical resection, with histological analysis. A history of cutaneous endometriosis with frequent recurrences can indicate malignant degeneration. All cases require long-term clinical follow-up because, despite the rarity of this diagnosis, the delay between benign endometriosis and malignant transformation can vary from a few months to over 40 years.

Long-term results of cytoreductive surgery for advanced and recurrent epithelial ovarian cancers and papillary serous carcinoma of the peritoneum.

The aim of this study was to review the long-term results of cytoreductive surgery in the treatment of advanced primary and recurrent epithelial ovarian cancers and papillary serous carcinoma of the peritoneum. Our goal was to identify clinical factors by which to select patients likely to benefit from a comprehensive management plan. Clinical data of 28 females who underwent surgery were retrieved from a prospective database. Major cytoreductive procedures were possible in 25 patients. Heated intraoperative or early postoperative intraperitoneal chemotherapy was also used where appropriate. The median follow-up after cytoreduction was 26.9 months. The overall median survival after cytoreduction was 45.8 months. The prognostic indicators associated with a statistically significant impact on survival were the prior surgery score (P < 0.001), the completeness of cytoreduction score (CC; P = 0.037), and response to chemotherapy prior to surgery (P = 0.012). Our findings suggest that cytoreductive surgery can be effective when combined with perioperative intraperitoneal chemotherapy. Results can be improved by excluding cases where CC seems unlikely. Extensive prior surgery without the protection of adjunctive intraperitoneal chemotherapy is associated with a poor prognosis. This may be due to disruption of anatomical planes leading to deep abdominal and pelvic dissemination intractable to further treatment.

Computed tomography findings of primary serous papillary carcinoma of the peritoneum in women.

With the aim to describe preoperative computed tomography (CT) findings, the clinical, histopathological, and CT findings of the 12 consecutive patients with a confirmed diagnosis of primary peritoneal serous papillary carcinoma (PPSPC) were retrospectively evaluated. Of the 12 patients with a mean age of 57.5 +/- 10.3 years, ten (83.3%) were postmenopausal. Serum Ca-125 levels were elevated in all patients. Ten (83.3%) had Stage III and two (16.7%) patients had Stage IV disease and none of the excised ovaries had deep parenchymal involvement. The most common CT findings were the omental (n = 11), mesenterial (n = 11) and parietal peritoneal involvements (n = 10), and variable amount of ascites (n = 10). Pelvic peritoneal involvement in four (33.3%) patients was so extensive that it resembled a mass in the Douglas pouch. Thickening of the wall of gastrointestinal viscera (n = 9), lymphadenopathy (n = 5) and pleural effusion (n = 5) were the other CT findings and calcification was seen in only three (25.0%) patients. Although, none of them was characteristic, CT features of diffuse peritoneal, omental and mesenterial involvement especially in middle-aged or elderly postmenopausal women with normal-size ovaries in the absence of an identifiable primary site in conjunction with elevated level of serum CA-125 should suggest the possibility of PPSPC.

Dermatomyositis and peritoneal papillary serous carcinoma.

We describe an unusual case of peritoneal papillary serous carcinoma (PPSC) arising in a female patient with dermatomyositis (DM). Despite periodic extensive searches for an underlying malignancy, no malignancy had been detected in this patient during the first 2.5 years after the diagnosis of DM. It was only when the patient presented with pleural effusion and ascites that the underlying intra-abdominal malignancy was detected by laparoscopy. Treatment with four cycles of pre-operative chemotherapy (taxol and cisplatin) resulted in tumor regression with amelioration in the muscular manifestation of the DM, but without parallelic amelioration in the skin manifestations of the DM. Explorative laparotomy confirmed the presence of papillary serous carcinoma in the omentum, surface of the left ovary and the retroperitoneal lymph nodes, and established the diagnosis of PPSC. Following two cycles of postoperative chemotherapy, the patient is alive with no evidence of internal malignancy. However, although muscle strength and enzymes have remained normal, no effect on the skin manifestation of DM has been observed. This case illustrates that, alongside the more frequently occurring ovarian carcinoma, PPSC should also be considered in the differential diagnosis of the underlying malignancy that may occur in the female patient with DM.

Genetic imbalance on chromosome 17 in papillary serous carcinoma of the peritoneum.

We extend the evaluation of allelic loss patterns on chromosome 17 to papillary serous carcinoma of the peritoneum (PSCP) which is histologically identical to papillary serous ovarian carcinoma (PSOC). DNA was obtained from 11 archival cases of PSCP, with 1-11 tumor sites per case. Using ten loci spanning chromosome 17, loss of heterozygosity (LOH) was identified in all 11 cases (100%). Furthermore, 75-100% of informative cases exhibited LOH at the loci p53, D17S1322 (intragenic to the tumor suppressor gene BRCA1), D17S1327 and MPO. PSCP cases exhibit a higher rate of LOH at most loci when compared with PSOC. Alternating allelic loss at different tumor sites was identified in three cases supporting a multifocal origin of PSCP. Microsatellite instability (MI) is an uncommon event which was identified in four cases. These data implicate chromosome 17 as a potential location of genetic events important in the pathogenesis of PSCP as well as ovarian cancer.

Risk factors and hormone levels in patients with serous and endometrioid uterine carcinomas.

We performed a multi-institutional, incident case-control study of 328 endometrioid and 26 serous carcinomas to assess whether risk factors and circulating hormone levels in women with serous carcinoma differ from the expected profile for endometrial carcinoma We also evaluated exposures potentially related to endometrial cancer risk, anthropometric measurements, and circulating levels of sex hormones and related carrier proteins. Histopathologic specimens were reviewed without knowledge of the other data. As expected, a statistically significant association was observed for high body mass index (BMI) (relative risk, 3.5) and use of menopausal estrogens (relative risk, 2.4) in the endometrioid carcinoma cases, whereas serous carcinomas were not strongly associated with these factors. Smoking and oral contraceptive use decreased risk for both tumor types. For five of six sex hormones tested, age-adjusted mean serum levels in patients with serous carcinoma were significantly lower than those in women with endometrioid carcinoma. After adjustment for BMI, these differences were narrowed, but levels of albumin-bound estradiol and estrone remained significantly lower in the serous cases. Age and BMI-adjusted levels of sex hormone-binding globulin were significantly higher in patients with serous carcinoma than in women with endometrioid carcinomas. In conclusion, risk factors and sex hormone levels in patients with uterine serous carcinoma seem to differ from those in women with endometrioid carcinoma, suggesting that there may be at least two different pathways of endometrial carcinogenesis.

Peritoneal papillary serous carcinoma arising in an infertile woman during ovulation-induction therapy: immunohistochemical expression of LH/hCG receptors.

A case of peritoneal papillary serous carcinoma arising in an infertile patient during ovulation-induction therapy is presented. A 34-year-old woman with past history of ovulation-induction therapy for infertility again received fertility drugs. During the use of gonadotropic hormones, massive ascites developed and an exploratory laparotomy revealed serous papillary carcinoma on the surface of bilateral ovaries, pelvic peritoneum, and omentum. Immunohistochemical analysis showed the tumor cells to be positive for luteinizing hormone/human chorionic gonadotropin receptors and negative for sex steroid receptors. A possible relationship between the use of gonadotropic hormones and cancer development is discussed.

Uterine papillary serous carcinoma in a 32-year-old with Turner's syndrome.

The association of Turner's syndrome and endometrial carcinoma has been previously established but has never been described in conjunction with a uterine papillary serous carcinoma (UPSC). This histological variant is usually found in considerably older women and has no clear relationship to the prior use of estrogen replacement therapy. Despite presenting with stage IV disease, treated by surgery and medroxyprogesterone only, this patient has had an 8-year disease-free remission, suggesting that radical debulking of an endocrine-responsive tumor may be of considerable benefit to some women with this unfavorable histological subtype of endometrial carcinoma.

[Tumor dissemination after celioscopic treatment of a tumor of the ovary]. / Dissémination tumorale après traitement coelioscopique d'une tumeur de l'ovaire.

A 35-year-old woman with a histologically proven T2 N0 M0 adenocarcinoma of the breast was given 4 cycles of neodjuvant chemotherapy then underwent tumourectomy followed by irradiation. Lymph nodes were free from invasion. A 7 cm ovarian cyst developed during follow-up and after a 2 month regimen of lynestrenol, coelioscopy with peritoneal lavage was performed. The pathology diagnosis was papillary cystadenocarcinoma requiring laparotomy which revealed invasion of both ovaries, neoplasic granulations and involvements of the epiloon. Exceresis of the trocar tract also showed tumoural invasion. Pathology examination favoured metastatic extension of the breast cancer. A 6-month chemotherapy was programmed before a second look. This case illustrates the risk of neoplastic dissemination after puncture or rupture of a cyst misdiagnosed as benign. In patients with a history of breast cancer, the risk of discovering a primary malignant ovarian cancer at coelioscopy is 9%. Metastases are found in 15-30% of the cases compared with 0.4 to 1.8% in unselected subjects. Prevention of operative dissemination relies on cystectomy without opening the cyst. This may require transforming the coelioscopy into a laparotomy which should not be considered as an operative failure but as a necessary method of preventing dissemination and clinical aggravation. Careful history taking, a rigorous coelioscopy technique and extension to laparotomy in cases with suspected diffusion should reduce the number of dramatic situations where the malignant process had not been suspected in the precoelioscopic diagnosis.

[Risk of systematic celioscopic treatment of ovarian diseases. 2 cases]. / Danger du traitement coelioscopique systématique de la pathologie ovarienne. 2 observations.

We report 2 cases of malignant ovarian tumours. These tumours had been missed at a first laparoscopic examination, and a second examination detected the presence of cancer cells disseminated in the peritoneum and the abdominal wall. The risk of propagation of an overlooked cancer makes it necessary to carry out a preoperative thorough evaluation based on clinical and ultrasonographic data before any attempt at laparoscopic surgery. If a laparoscopic treatment is decided, it must be performed under strict conditions and include meticulous exploration of the abdominal cavity, systematic peritoneal cytology, needle cytology of the cyst, emptying of the cyst in a water-tight manner, extemporaneous biopsy in case of doubt, peritoneal cleansing, and extraction of the cyst or the ovary in a bag. If malignancy is suspected, laparotomy must be performed immediately.

Management of advanced ovarian epithelial cancer in the renal transplant patient.

Stage IIIC, grade 1 papillary serous adenocarcinoma of the ovary was diagnosed in a 28-year-old renal transplant recipient. She had been treated with the immunosuppressive agents azathioprine and methylprednisolone for 7 years prior to the discovery of the ovarian cancer. Surgical excision of the tumor was suboptimal due to involvement of the allograft; however, the patient achieved a complete clinical response after eight courses of cisplatin and cyclophosphamide. Since multiagent immunosuppressant therapy may have contributed to the development of the ovarian carcinoma, the intensity of immunosuppression was decreased by discontinuing the azathioprine as soon as the diagnosis of ovarian cancer was made. The methylprednisolone, however, was continued to decrease the possibility of organ rejection. After completion of chemotherapy, the patient was started on a daily regimen of low-dose oral cyclophosphamide as an immunosuppressant. Four months following the completion of cytotoxic therapy, she developed clinically evident disease in the pelvis. Subsequent salvage therapy with carboplatin failed. The patient died from progressive disease 26 months after initial diagnosis. She never developed evidence of renal rejection. Combined modality cancer therapy, preservation of allograft function, and modification of immunosuppressant therapy are important goals in the renal transplant patient with advanced ovarian carcinoma.