Topical testosterone versus clobetasol for vulvar lichen sclerosus.

OBJECTIVE: To compare the effects of topical testosterone and clobetasol treatments on symptoms remission and recurrence rates in patients with vulvar lichen sclerosus (LS). METHODS: A retrospective review of the records showed that, of 140 patients with biopsy-proven vulvar LS, 80 were treated with applications of testosterone propionate 2% in petrolatum and 60 with clobetasol 17-propionate 0.05%. RESULTS: The response rates after 6 months were 77.5% for patients treated with testosterone and 91.7% for those treated with clobetasol (P=0.02). The recurrence rates were 20% and 6.7% in the 2 groups, respectively (P=0.02). Premenopausal patients had higher remission rates and lower recurrence rates than postmenopausal patients (P>0.05). Considering whole patients, low remission rates and high recurrence rates were observed in patients who had had a hysterectomy (P>0.05). CONCLUSION: Treatment of LS with a corticosteroid provided excellent remission rates. In this study, clobetasol 17-propionate 0.05% was superior to testosterone for both remission induction and maintenance therapy.

Medical treatment of vulvar squamous cell hyperplasia.

OBJECTIVE: To evaluate symptomatic response and recurrence rates of graduated topical fluorinated corticosteroid in patients with vulvar squamous cell hyperplasia. METHODS: Nine hundred seventy-six patients with biopsy-proven vulvar squamous cell hyperplasia from 1990 to 2003 were reviewed in this retrospective study. All patients were treated with graduated topical fluorinated corticosteroid. Data were obtained from hospital records. Symptomatic remission and recurrence rates were noted following six months local therapy. RESULTS: The mean age was 42.55+/-10.93 (15-85). The remission rate was 93.8% in six months. The remission rate was non-significantly higher in postmenopausal patients than that in their premenopausal counterpart (94.9% vs 93.0%, p=0.15). The disease recurred in 6.9% of patients. Of the patients that suffered recurrence 47.5% had persistent disease initially. The patients with following factors older ages (>40 years), postmenopausal period had significantly higher recurrence rates. Four patients with recurrent disease and six patients with persistent disease in the form of vulvar intraepithelial neoplasia I-II or atypical squamous hyperplasia, were treated with skinning vulvectomy. CONCLUSION: Corticosteroid in the treatment of vulvar squamous cell hyperplasia yielded excellent response rates. In the evaluation of patients without symptomatic relief, the first step should be a vulvar biopsy to exclude the presence of atypical components.

Two siblings with nemaline myopathy presenting with rigid spine syndrome.

Two siblings, ages 20 and 19 presented with more than 10 yr history of spinal rigidity and scoliosis. The parents were first cousins. Muscle biopsies were consistent with nemaline myopathy. This has been the first association between a familial rigid spine syndrome and nemaline myopathy.

Acute idiopathic demyelinating polyneuropathy: passive transfer to mice by immunoglobulin.

Systemic administration of acute idiopathic demyelinating polyneuropathy (AIDP) immunoglobulins to mice for two weeks resulted in reduced sural nerve action potential amplitudes and reduced (rotarod) motor performance. Electron microscopic examination of the sciatic nerves of the AIDP-immunoglobulin-treated animals revealed loosening of myelin lamellae with widening of interperiod lines and multivesicular disruption of myelin. Vacuolar degeneration was detected in half of the nerves examined by light microscopy. Injection of AIDP-immunoglobulins for three days led to only minor changes, and mice receiving healthy human immunoglobulins showed no abnormalities. These data show that some features of AIDP can be transferred to mice by systemic administration of immunoglobulins and suggest that humoral factors have a pathogenic role in AIDP in addition to cellular factors.

Prognostic value of mitotic activity, eosinophilic and inflammatory reaction in stage I cancer of the uterine cervix.

This study includes 110 patients treated surgically for stage I squamous cell carcinoma of the cervix. The prognostic significances of mitotic activity, stromal inflammatory and eosinophilic reactions were studied. The 5-year survival rate varied from 75.0% to 93.3% and pelvic lymph node metastases (PLNM) varied from 23.0% to 31.2% according to degree of these variables. None of these pathological parameters was found to be a significant predictor of pelvic lymph node metastases and 5-year survival.

Correlation between clinical and histopathologic risk factors and lymph node metastases in early endometrial cancer (a multivariate analysis of 183 cases).

This study includes 183 patients with clinical stage I endometrial cancer subjected to peritoneal cytology, total abdominal hysterectomy, bilateral salpingo-oophorectomy, bilateral pelvic and para-aortic lymphadenectomy and omental biopsy during a 12-year period in a single institution. The factors analyzed were age, menopausal state, cell type, grade, mitotic activity, myometrial invasion, lymphovascular space invasion, cervical involvement, microscopic vaginal metastases, adnexal metastases, peritoneal cytology, presence of concomitant endometrial hyperplasia and lymph node status. The overall incidences of pelvic and para-aortic lymph node metastases were found to be 15.3% (28/183) and 9.3% (17/183), respectively. In five of 17 patients (29.4%) with para-aortic nodal metastases, pelvic nodes were free of tumor. The most significant prognostic factors for positive pelvic and/or para-aortic nodes were found to be the depth of myometrial invasion, grade of tumor and age.

A case of late infantile neuronal ceroid lipofuscinosis associated with precocious puberty.

Neuronal ceroid lipofuscinosis is one of the hereoffegenerative diseases for which clinical and neuropathologic findings are well documented. We present a patient with late infantile neuronal ceroid lipofuscinosis with true precocious puberty; to our knowledge, this association has not been reported before. The association could be due to an underlying disturbance of hypothalamic-pituitary gonadal function, or to coincidence.

Search for problem areas in endometrial biopsies to achieve quality assurance.

The aim of this study was to underline the frequently seen problems in diagnosing the lesions seen in the hyperplasia-carcinoma sequence by evaluating the variances between the observers. Four pathologists re-evaluated 137 endometrial biopsies and grouped them into diagnostic categories. The results were analyzed by Kappa statistics. Full agreement was reached in 89 cases (64.96%), with Kappa values ranging between 0.63-0.74. Three observers rendered the same diagnosis in 34 (24.81%) cases, and only one pathologist disagreed. Two or more observers held different views in 16 cases (10.95%). The problem areas were as follows: criteria distinguishing simple hyperplasia from other benign lesions, discrimination between atypical hyperplasia and carcinoma, and decision-making regarding the presence of atypia. There was a tendency towards overdiagnosis of hyperplasia in our department. Since the progression to carcinoma is a sequential event, borderline cases will exist if categories based on simple and clear cut off points are not defined.

Primary neuroendocrine tumor of the breast.

Adult neuroendocrine tumors may present with a wide range of clinical symptoms that share specific ultrastructural and biochemical features. A 63-year-old post-menopausal female patient was admitted to the hospital with a mass in her right breast and the diagnosis was primary neuroendocrine tumor of the breast. Although neuroendocrine tumors can originate in various parts of the body and breast carcinoma with neuroendocrine differentiation has been described, primary neuroendocrine tumor of the breast is very unusual. This case is now presented and the current literature is reviewed.

The prognosis of subacute sclerosing panencephalitis in adults. Report of 8 cases and review of the literature.

In this article, 8 cases of subacute sclerosing panencephalitis (SSPE) are presented with a review of the literature. The symptoms developed during pregnancy in 2 cases. All of the patients died within 1 year duration except one. According to the reviewed literature, the prognosis in the adult cases is worse than in children; the two sexes are equally affected in adult SSPE.

Mucin production in carcinomas of the uterine cervix.

The histological types of the cervical carcinoma effect the incidence, prognosis and the treatment methods. One hundred and eighty-seven cases selected randomly among the cervical carcinoma were stained with mucicarmen, alcian blue, PAS, and PAS with diastase to identify the presence of intracellular mucin. One hundred and seventy-two squamous carcinoma were examined and 27.3% (47 cases) of them were shown to contain some degree intracellular mucin (nine cases which were originally diagnosed squamous carcinoma after the application of mucin stains to the tumors, reclassified as adenosquamous carcinoma because of demonstrated widespread intracellular mucus). Of the 14 adenocarcinomas, 13 were mucin positive. Mucin-positive tumors (independent of histological type) had metastasized to the lymph nodes in 23 of 60 cases (38.3%), in contrast to mucin-negative tumors which had metastasized in 24 of 127 cases (18.9%). The application of mucin stains can assist in the classification of poorly differentiated lesions as well as help in the planning of management and in the prognosis.

Risk factors for recurrence in clinically early endometrial carcinoma: an analysis of 183 consecutive cases.

This study includes 183 patients with clinical stage I endometrial carcinoma. All patients had standard surgical staging procedure including peritoneal cytology, total abdominal hysterectomy, bilateral pelvic and paraaortic lymphadenectomy. The factors analysed for recurrence were age, menopausal state, cell type, grade, mitotic activity, myometrial invasion, lymphovascular space invasion, cervical involvement, microscopic vaginal metastases, adnexal metastases, peritoneal cytology, concomitant endometrial hyperplasia and pelvic and paraaortic node metastases. The overall recurrence rate was 14.2% (26/183). Of the 26 patients with recurrence, 11 had local and 13 had distant metastases. In the remaining two patients (7.7%), both local and pelvic metastases were observed. Of the factors analysed, age, grade, mitotic activity, myometrial invasion, lymphovascular space invasion, microscopic vaginal metastases, adnexal involvement and pelvic and paraaortic nodal metastases were found to be significant predictors of recurrence. After multivariate analysis, advanced age (RR = 1.05), marked mitotic activity (RR = 3.11), pelvic and/or paraaortic nodal metastases (RR = 6.37) were chosen as the most important determinants of recurrence. In terms of surgical pathological stages, recurrence risk reaches up to 45.4% for stage IIIC disease. Using surgical pathological parameters, it is possible to predict recurrence but because of high rate of distant failures it still seems hard to improve survival of this group. Detection of a substantial risk of recurrence even in stage IA/B grade 1 group warrants adjuvant therapy in all patients after primary surgery.

Tumor status of lymph nodes in early endometrial cancer in relation to lymph node size.

A retrospective analysis of 36 patients with metastatic nodes out of 209 consecutively managed patients with a clinically stage I endometrial cancer was carried out. Of the 1023 lymph nodes removed, 154 nodes were found to be metastatic. The mean number of the involved nodes was 4.27 (range: 1-29). Of the 154 positive nodes, 3 had nodal diameters < or = 3 mm (1.9%), 84 had diameters of 4-10 mm (54.6%), 60 had diameters of 11-20 mm (39.0%) and 7 had diameters more than 20 mm (4.5%). With increasing lymph node size, the frequency of tumoral involvement varies from 1.0% in nodes < or = 3 mm to 63.6% in nodes bigger than 20 mm. In terms of patients, nine of them were found to have a single metastatic node ranging from 6 mm to 10 mm in diameter. In the remaining 27 patients with multiple metastatic nodes, the biggest nodes encountered were 6-10 mm in 4 (14.8%), 11-20 mm in 17 (62.9%) and more than 20 mm in 6 (22.2%) patients. Since mere sampling of the lymphatic tissue directed particularly to the enlarged nodes may not show the true incidence of positive nodes, a complete lymphadenectomy is advocated in order to obviate an understaging problem.

Risk factors for groin node metastasis in squamous carcinoma of the vulva: a multivariate analysis of 39 cases.

This study includes 39 patients with squamous carcinoma of the vulva subjected to radical surgery. Stage, tumor description, cell type, lesion localization, lesion size, depth of invasion, grade, lymphovascular space invasion, number of mitoses, degree of stromal inflammatory reaction, and tumors in surgical margins were analyzed for groin metastasis. Of the factors analyzed, stage (P = 0.018), grade (P = 0.007) and depth of invasion (P = 0.001) were found to be the most important predictors of node metastasis. While a combination of factors permitted to define a low-risk group with 0% node metastasis, it seems hard to identify preoperatively those patients with no risk of nodal metastasis.

Synchronous primary malignancies of the female genital tract.

This study includes 29 patients with synchronous primary malignancies of the female genital tract. These patients constituted 1.7% of all genital malignancies. The most frequently observed synchronous neoplasms were those of the ovary together with the endometrium (51.7%). Most patients had early-stage and low-grade disease. Stage I disease was observed in 68.1% of patients with ovarian cancer. Patients with synchronous ovarian and endometrial cancer had a 73.3% 5-year survival rate, suggesting a favorable prognosis.

Abdominal aortic aneurysm with inferior vena cava obstruction: case report.

Behçet's syndrome, a multisystem disorder, is characterized by recurrent oral and genital aphthous ulcerations, eye lesions, and skin changes. Other manifestations, although rare, may affect the nervous, gastrointestinal, or locomotor system, as well as veins and arteries. Vascular lesions occur in approximately 30% of reported cases. Although thrombosis in the larger veins is frequent, arterial thrombosis is somewhat less likely to occur. Only a few cases of arterial aneurysm have been documented in the literature. This report describes a patient who had suffered from Behçet's syndrome for fifteen years and in whom a complete obstruction of the inferior vena cava was demonstrated. An aortoiliac bypass was performed successfully, and the patient had had a good clinical recovery at long-term follow-up.

Predictors of persistent disease in women with complete hydatidiform mole.

OBJECTIVE: To determine the most significant factors for persistent disease in women with hydatidiform mole. STUDY DESIGN: Eighty-two patients who were managed consecutively without prophylactic chemotherapy between 1973 and 1993 were analyzed retrospectively for clinical and pathologic features, including age of the patient, size of the uterus, human chorionic gonadotropin (hCG) level, presence of theca lutein cysts, evidence of preeclampsia or hyperthyroidism, history of hydatidiform moles, trophoblastic hyperplasia, nuclear atypia, necrosis and hemorrhage, trophoblastic maturation, presence of fibrinoid layer and ratio of cytotrophoblast to syncytial trophoblast. RESULTS: Thirty-one patients developed persistent trophoblastic neoplasia (38%). Of the parameters evaluated, elevated hCG level, advanced age, history of hydatidiform mole, presence of hyperplasia, marked nuclear atypia and necrosis, and hemorrhage were significant risk factors for persistent neoplasia after univariate analysis. Among the factors analyzed, trophoblastic hyperplasia (relative ration [RR] = 3.56), age (RR = 2.87) and history of mole (RR = 2.57) were identified as the most powerful indicators of persistent disease after multivariate analysis. CONCLUSION: Evaluation of the clinical and pathologic features, such as age, history of mole and presence of trophoblastic proliferation may aid in defining a subset of patients at high risk for persistent disease, who require closer follow-up and administration of prophylactic chemotherapy.

Ichthyosis uteri associated with a uterine squamous papilloma.

A 60-year-old woman was admitted for postmenopausal vaginal bleeding. Hysterectomy specimens showed an intrauterine polypoid mass that was a typical papilloma microscopically, accompanying severe ichthyosis uteri. There was no sign of dysplasia or malignancy. We observed changes simulating koilocytosis in some of the papilloma cells, but these did not stain with antiHPV antibodies immunohistochemically.

Skinning vulvectomy for the treatment of vulvar intraepithelial neoplasia 2-3: a study of 21 cases.

Twenty-one cases of patients with vulvar intraepithelial neoplasia (VIN) 2-3 were reviewed. The mean age at diagnosis was 45.4 years. All of the patients presented with vulvar pruritus. Five of the patients had hypertension, two had coronary heart disease and two had diabetes mellitus as complicating medical illnesses. None of the patients had history or evidence of vaginal intraepithelial neoplasia (VAIN) or cervical intraepithelial neoplasia (CIN), and only one patient had invasive cervical cancer at diagnosis. Provided the histology confirmed VIN, the patients were subjected to a skinning vulvectomy procedure. Of the patients, 15 (71.4%) had VIN 2, and the remaining 6 (28.6%) had VIN 3 at preoperative evaluation. Histologic analysis of skinning vulvectomy specimens revealed no evidence of neoplasia in three patients (14.2%). Multifocality was observed in only three patients (14.2%). The areas involved were the perineum in four patients, labia in 15 and clitoris in two patients. Associated vulvar pathologies were condyloma acuminata in one, squamous vulvar hyperplasia in three and lichen sclerosus with squamous hyperplasia in one patient. The complications of the procedure included febrile morbidity in three patients and minor wound break-down in one patient. None of the patients in this series experienced recurrence. Skinning vulvectomy seems to have a high success rate in treatment of VIN 2-3 with minimal postoperative complications and satisfactory cosmetic results. However, observation of only three patients with multifocal lesions as well as no patient with invasive cancer adds credence to an ablative procedure after appropriate evaluation under colposcopy.

Microinvasive carcinoma of the cervix: an analysis of 31 patients.

A retrospective analysis of 31 patients subjected to radical surgery and lyphadenectomy for microinvasive cervical cancer was carried out. The mean age of the patients at diagnosis was 51.4 years. Twenty-nine of the patients had depth of invasion of 3 mm or less whereas 5 of them had lymphovascular space invasion. Thus, 24 patients had microinvasive cervical cancer according to the SGO (Society of Gynecologic Oncology) criteria. While nine patients had minimal stromal invasion (< 1 mm) at diagnosis, the remaining 22 patients fulfilled the criteria of FIGO IA2 disease. Most of the patients had grade I disease. With worsening of differentiation lymphovascular space involvement increases from approximately 11% (2/18) to 50% (1/2). None of the patients had lymph node involvement. All patients are living with no evidence of disease with a mean survival of 39 months. Current FIGO criteria for microinvasive carcinoma permits us to define a subset of patients that can safely be subjected to a more conservative approach.