Rapidly progressive primary undifferentiated ovarian carcinoma: presentation of a rare case.

Ovarian cancer is the second most common gynecologic malignancy and is one of the leading causes of death among women. The disease course and the accurate diagnosis are correlated with the early detection of the lesion. About 5% of ovarian cancers are poorly differentiated and difficult to be classified, and are referred to as undifferentiated carcinomas. They are usually large, solid with haemorrhage and necrosis, bilateral, and very difficult to be histologically classified. Generally, cases with undifferentiated components are very rare. The authors present a case of a young female patient with a rapidly progressive undifferentiated ovarian carcinoma and a final unfortunate clinical result.

Maternal and umbilical cord oxygen content and acid-base balance in relation to general, epidural or subarachnoid anesthesia for term elective cesarean section.

PURPOSE: To compare maternal and neonatal oxygenation and acid-base status after elective cesarean section (CS) under different anesthetic techniques. MATERIALS AND METHODS: Three hundred and eighty parturients undergoing elective cesarean section were randomly assigned to receive general (GA, n =140), epidural (EA, n = 117) or subarachnoid anesthesia (SA, n =123). Blood gases, oxygen content, and acid-base status parameters were measured in maternal artery and umbilical cord vessels. Neonatal Apgar scores were also recorded. RESULTS: Umbilical artery pH, HCO3-, and actual base excess (ABE) were significantly higher in the GA compared to SA group (p < 0.001, p < 0.05, andp < 0.05, respectively). Umbilical vein ABE was lower in the SA compared to GA and EA groups (p < 0.05). Oxygen content in maternal artery was higher in the GA and EA groups compared to the SA group (p < 0.05). Neonatal oxygen content in both cord vessels was higher in the GA group compared to EA and SA groups (p < 0.05). Umbilical venous-arterial difference of PO2, oxygen content, and Apgar scores did not differ significantly among groups. CONCLUSION: Neonatal oxygenation and acid-base status values were better preserved when GA was administered for elective CS compared to regional modalities. Apgar scores and neonatal outcomes were not affected by the anesthetic technique.

Autopsy findings in fetuses with cystic hygroma: a literature review and our center's experience.

PURPOSE OF INVESTIGATION: To report our experience of autopsied cases of fetal cystic hygroma (CH) and discuss the role of fetal autopsy in genetic counseling. METHODS: A review of autopsy reports at our institution revealed 18 cases of fetal CH over a 10-year period (from 2000 to 2010). The clinical data, results of cytogenetic analysis and prenatal ultrasound findings were also retrieved and compared to the autopsy findings. RESULTS: Fetal death was due to intrauterine death in eight cases, therapeutic abortion in eight cases and spontaneous abortion in two cases. Cytogenetic analysis was available in 12 cases, and the results showed an abnormal karyotype in seven cases (5 cases of Turner syndrome and 2 cases of trisomy 21). The mean size of CH was 5.4 cm. Other malformations or findings suggestive of the cause of fetal death were diagnosed in 10/18 cases (55.6%). The most common autopsy findings were hydrops and central nervous system anomalies. The autopsy findings were in agreement with the prenatal ultrasound findings in 13/18 cases (72.2%), while in five cases (27.8%) additional findings were detected during autopsy. The most common placental abnormalities were infarcts and calcifications. CONCLUSION: In addition to prenatal diagnostic studies, fetal autopsy and pathologic examination of fetal and placental tissues may help to establish the exact cause of death and disclose important information as to the presence of various fetal malformations or placental abnormalities.

Extraovarian mature cystic teratoma of the mesentery. A case report and literature review.

A case is reported of a 60-year old woman who had intermittent abdominal pain due to a mesenteric teratoma which was misdiagnosed by the standard methods of abdominal tumor diagnosis as an ovarian tumor. The neoplasm measured 9 x 8 x 8 cm, was filled by hair and sebum, and histologically presented the typical features of a mature cystic teratoma. The mesentery and overlying small intestine showed an extensive inflammatory granulomatous reaction. The uterus and adnexa were free of neoplasmatic disease. This case of extra ovarian mature cystic teratoma which developed in the mesentery is unique among > 2000 ovarian tumors examined during a 30-year period at Aretaieion Hospital Pathology Laboratory.

Endometrial carcinoma and ovarian sex cord tumor with annular tubules in a patient with history of Peutz-Jeghers syndrome and multiple malignancies.

BACKGROUND: Peutz-Jeghers syndrome is a rare syndrome which is inherited in a dominant manner. It is characterized by hamartomatous polyps of the gastrointestinal tract, hyperpigmented macules of the oral mucosa and an increased risk of developing neoplasms in the gastrointestinal tract, pancreas, breast and genital system. Women with Peutz-Jeughers syndrome often develop an ovarian sex cord tumor and cervical adenocarcinoma of minimal deviation adenoma malignum type. A case of a 58-year-old patient with Peutz-Jeghers syndrome and history of multiple malignancies (thyroid, breast and colon cancer) who presented with metrorrhagia is reported. The dilatation and curettage revealed endometrial adenocarcinoma. The patient underwent total abdominal hysterectomy with bilateral oophorectomy. The histologic examination showed an endometrioid endometrial adenocarcinoma that developed in atypical endometrial hyperplasia. The histologic examination of the right ovary revealed a sex cord tumor with annular tubules, measuring 3 cm. Sex cord tumors with annular tubules in patients with Peutz-Jeghers syndrome are usually small, bilateral tumors of the ovaries which have common characteristics with granulosa cell tumor and Sertoli cell tumor. Hyperestrogenism is a rather common finding with development of estrogen-dependent lesions.

Angiomyofibroblastoma of the vulva: a clinicopathological and immunohistochemical analysis of a rare benign mesenchymal tumor.

Angiomyofibroblastoma is a rare benign distinctive mesenchymal tumor that occurs in the genital pelviperineal region, commonly the vulva and vagina. We report a case of angiomyofibroblastoma in a 42-year-old woman, presenting as a "cystic mass" located subcutaneously in the right labial area of the vulva. Recognition of this entity is based on specific histological and immunopathological features, and the correct treatment is important because of the aggressive behavior of other related mesenchymal tumors of the vulva and vagina, such as angiomyxoma and cellular angiofibroma.

Rare case of an ovarian monodermal teratoma with functional stroma and extensive ovarian decidualization in a 74-year-old woman.

We present the clinicopathological findings of a rare case of a monodermal teratoma of the right ovary with functional ovarian stroma and extensive decidualization in a 74-year-old woman. The patient presented with vaginal bleeding. Ultrasound scan revealed a pelvic mass measuring 9.5 cm in the lower right abdomen. A right oophorectomy was performed. The tumor was cystic and multilocular filled with colloid material. Histological examination revealed follicles of thyroid type, and stromal clusters of fusiform or polygonal cells were found in the stroma. An extensive decidual reaction was observed. Morphological and immunohistochemical examination of the tumor revealed cystic struma ovarii with functional ovarian stroma and ectopic decidua. Total abdominal hysterectomy with oophorectomy was performed. A benign endometrial polyp, proliferative endometrium, two fibroids, and an ovarian cyst were observed.

Cervical adenocarcinoma with clear cell morphology. report of six cases and literature review.

Clear cell cervical adenocarcinoma (CCA) is a rather rare malignancy of the genital tract. We report six cases of CCA, diagnosed in our laboratory during a 15-year period: five patients with sporadic primary CCA and one young patient with CCA and a history of in utero exposure to DES. The possible DES exposure, clinicopathological findings as well as the differential diagnosis and the the prognosis of such patients are presented in a mini-review of the literature.

A novel technique for surgical reconstruction of the perineal floor following anteroposterior exenteration of the pelvis--case report and review of the literature.

Pelvic exenteration is the only potentially curative surgical procedure for patients with recurrent cervical, vaginal, vulvar or rectal cancers, especially following adjuvant chemotherapy or radiotherapy. Morbidity rates, however, remain high, which is significantly attributed to complications of the pelvic floor reconstruction techniques. We describe a novel reconstruction technique of the pelvic floor, involving a combination of an oblique rectus abdominis myocutaneous flap and a synthetic absorbable mesh as a pelvic sling for additional support, in a 63-year-old female patient with recurrent vulvar carcinoma. Combining the use of myocutaneous flaps and prosthetic mesh material can provide an effective alternative solution to the complications arising from pelvic floor reconstruction of large defects after exenteration procedures, especially in previously irradiated settings. Further studies are necessary to define the long-term outcomes and indications of these techniques, as well as the optimal combination between the available myocutaneous flaps and prosthetic materials.

Granular cell tumor of the female genital system. Clinical and pathologic characteristics of five cases and literature review.

The clinical and pathological characteristics of a rare granular cell tumor that developed in the vulva (4 cases) and the breast (1 case) offive women aged 35-52 years are reported. The differential diagnosis from skin carcinomas, melanoma and various soft tissue tumors is emphasized.

Granulosa cell tumors of the ovary: a clinicopathologic and immunohistochemical study of 21 cases.

PURPOSE: To further study the clinicopathologic and immunohistochemical features of ovarian granulosa cell tumors (GCTs). METHODS: We retrospectively studied all cases of GCTs diagnosed in our laboratory over the last 10-year period. Immunohistochemistry for inhibin, vimentin, cytokeratin, Ki-67 and p53 was performed on archival paraffin blocks. Pathologic and immunohistochemical findings were correlated with the clinical records of the patients. RESULTS: Twenty-one cases (15 of the adult and 6 of the juvenile type) were retrieved. All patients were FIGO Stage I at the time of diagnosis. Recurrent disease was detected in four patients (19%) during a median follow-up of 36 months (range 2-26 years). Pathology revealed a concomitant theca-cell component in three cases, a Sertoli-Leydig component in one case, and a thecoma in one case. Archival tissue material was available in 12 cases. Immunohistochemistry was positive for: beta-inhibin in 12/12 cases (100%), vimentin in 11/12 cases (91.7%), cytokeratin in 3/12 cases (25%), CD34 in 0 cases (0%), and p53 in 2/12 cases (16.7%). The Ki-67 index was < 5% in 12/12 cases (100%). No significant correlations were observed between the pathologic and immunohistochemical parameters examined and the clinical outcome. CONCLUSIONS: Despite the relatively indolent nature and favorable prognosis of most GCTs, late recurrences are not a rare event even in Stage I patients, necessitating a close and long-term follow-up. The identification of novel prognostic markers, in addition to our traditional staging parameters such as clinical staging, is needed in order to more accurately predict probabilities of recurrence in these patients.

Carcinosarcomas of the uterus and ovary: a clinicopathologic and immunohistochemical study of 11 cases.

PURPOSE: To further study the clinicopathologic features of carcinosarcomas of the uterus and ovary. METHODS: We retrospectively studied all cases of uterine and ovarian carcinosarcomas diagnosed in our laboratory over the last 5-year period. The pathologic and immunohistochemical findings were correlated with the clinical records of the patients. RESULTS: Eleven cases were retrieved . The commonest presenting symptom was vaginal bleeding (9 cases, 81.8%). Most patients (8 cases, 72.7%) were submitted to total abdominal hysterectomy with bilateral salpingo-oophorectomy and adjuvant chemotherapy was administered to all of them. In the majority of cases the tumor was located in the uterine corpus (7 cases, 63.6%), followed by the ovary (4 cases, 36.4%). The tumor was homologous in ten cases (90.9%) and heterologous in one case (9.1%). Most of our patients (6 cases, 54.6%) were diagnosed at an advanced stage (FIGO Stage III or IV). The sarcomatous element was strongly positive for vimentin in all cases and focally positive for cytokeratin 7 in four cases, while the epithelial component showed a strong positivity for cytokeratin 7 and focal staining for vimentin, cytokeratin 20, CA-125 and CEA. CONCLUSION: Carcinosarcomas of the uterus and ovary are highly aggressive biphasic neoplasms with a prominent epithelial component. Their most common location is the uterine corpus. Although distant metastases are rarely found at the time of diagnosis, the prognosis of these tumors is unfavorable. The optimal chemotherapy remains to be determined.

Synchronous ovarian and endometrial carcinoma: a strong link to endometriosis?

PURPOSE: To further study the clinicopathological features of synchronous ovarian and endometrial carcinomas. METHODS: We retrospectively studied all cases of synchronous ovarian and endometrial carcinomas diagnosed in our laboratory over the last 15-year period. The pathological findings were correlated with the clinical records of the patients. RESULTS: Seven cases of synchronous primary ovarian and endometrial carcinomas were retrieved. The most common presenting symptom was abnormal vaginal bleeding (5 cases, 71.4%). Five patients (71.4%) were postmenopausal and two (28.6%) were nulliparous. All seven patients had Stage I ovarian and endometrial carcinomas of endometrioid histology. Moreover, in all seven ovarian carcinomas endometriosis foci were observed, while atypical endometriosis was found in four of them. With the exception of one patient, who received adjuvant postoperative radiation, all remaining patients were treated with surgery alone. All patients were alive and free of disease at completion of the study. CONCLUSION: The correct classification of synchronous primary ovarian and endometrial carcinomas is often problematic because of the frequent confusion with their metastatic counterparts. Although the exact etiology remains unclear, endometriosis seems to be a major risk factor for their development.

Recurrence of granulosa cell tumor 25 years after initial diagnosis. Report of a case and review of the literature.

Granulosa cell tumors (GCTs) are rare functional sex cord-stromal ovarian tumors constituting approximately 2-3% of all ovarian malignancies. They are characterized by low malignant potential, local spread, late recurrence and high survival rates. We report a case of recurrent ovarian GCT in a 60-year-old woman 25 years after the initial diagnosis. The patient underwent surgical resection of the pelvic masses and refused to receive any adjuvant treatment, considering the late recurrence and high survival rates of this tumor. This case illustrates an example of a very late recurrence and emphasizes the importance of the extended follow-up required for these patients.

Congenital cystic adenomatoid lung malformation: report of two cases and literature review.

Congenital cystic adenomatoid malformations of the fetal lung (CCAM) are rare embryonic developmental abnormalities. They are considered as benign hamartomatous or dysplastic lung tumors characterized by overgrowth of the terminal respiratory bronchioles at the expense of the saccular spaces. A minority of cases may not be identified by prenatal imaging techniques and the pulmonary lesions are recognized postnatally. Two cases of congenital cystic adenomatoid malformation of the fetal lung diagnosed in our institution during the last four years are reported. The ultrasonographic and pathologic findings of these cases are discussed.

A malignant eccrine poroma in a pregnant woman: case report and review of the literature.

Malignant eccrine poroma is a rare cutaneous neoplasm that originates from the intraepidermal portion of the eccrine gland. It affects mainly elderly people while its occurrence in younger adults is extremely rare. We present the first reported case of a malignant eccrine poroma in a pregnant woman, with emphasis on its pathologic and immunohistochemical features. Early diagnosis and treatment of eccrine neoplasms are of crucial importance when pregnancy coexists, because of their tendency to aggravate under the influence of gestation-related changes.

Crossover analysis using immunofluorescent detection of MLH1 foci in frozen-thawed testicular tissue.

To date, the effects of freezing on spermatogenesis have not yet been fully investigated at a molecular level. Antibody localization studies have identified the MutL homolog 1 (MLH1) protein, a mis-match repair protein, at the prophase I stage of meiosis, which allows the detection of recombination foci during pachytene. This study investigated the effect of long-term testicular tissue cryopreservation on meiotic prophase I, identified by recombination foci frequency and synaptonemal complex (SC) integrity. Frozen-thawed testicular tissues from 12 males who had each fathered a child were used. Because vasectomy or reverse vasectomy procedures are rare in the locale of the investigation, it was not possible to obtain fresh testicular tissue and use the males as their own controls. Immunocytogenetic analysis of 612 spermatocytes at the pachytene stage was performed. The results indicated a mean number of MLH1 foci of 49.2 (SD +/- 5.9), and no correlation was found between the freezing period, the MLH1 frequency and the SC integrity. The results suggest that freezing of testicular tissue taken post-puberty does not appear to be detrimental to the crossover process as identified by occurrence of MLH1 loci.

Primary gestational choriocarcinoma of the uterine cervix. Report of a case and review of the literature.

Gestational choriocarcinoma usually arises in the uterine cavity and is associated with coincident or antecedent pregnancy. Extrauterine choriocarcinomas are very rare entities, and most of these are located in the uterine cervix. In our case, a 43-year-old woman was admitted in our hospital because she had amenorrhea for 2 months and elevated serum beta-human chorionic gonadotropin levels. The patient was considered to have an ectopic pregnancy. Initially, she was treated with methotrexate, but since there was a continuous rise in human chorionic gonadotropin levels, the patient underwent a laparoscopy, along with dilatation and curettage (D&C) of the uterine cavity. Histopathologic findings, including immunohistochemical study, led to the diagnosis of choriocarcinoma of the cervix. Finally, the patient underwent a transabdominal hysterectomy and received single agent chemotherapy with methotrexate. Our case represents a primary choriocarcinoma of the cervix, which was initially misdiagnosed as an ectopic pregnancy. The difficulties in differential diagnosis are discussed. Immunohistochemical evaluation remains the mainstay of the diagnosis in most cases. Choriocarcinoma has a very good prognosis even in advanced stages, since it is a very chemosensitive tumor.