Impact of oophorectomy and hormone suppression in low grade endometrial stromal sarcoma: A multicenter review.

OBJECTIVES: Low grade endometrial stromal sarcoma (LG-ESS) is a rare cancer with an indolent course. We aimed to assess the effectiveness of adjuvant hormonal suppression (HT) with or without oophorectomy (BSO) in prolonging progression free survival (PFS) and overall survival (OS) in patients with LG-ESS. METHODS: We performed a multi-institutional retrospective review of patients treated for low grade LG-ESS from 1985 to 2014. Demographics, treatment and recurrence data were abstracted from medical records. Pathologic diagnosis was confirmed by a gynecologic pathologist. Long-term patient-reported outcomes were obtained via mailed survey. RESULTS: One-hundred-twelve patients underwent surgery for LG-ESS; 59 had postoperative data with a median follow-up of 55months (1-325months). The mean age at diagnosis was 48.5years (22-82years). Forty-nine (61%) had stage I disease. The most common presenting symptoms were abnormal uterine bleeding (38%) and pelvic mass (17%). Seventy-one (63%) patients had BSO at the time of diagnosis. Of the 59 patients with postoperative follow-up information, 49 (73%) underwent BSO, 26 (44%) received HT, 20 (33%) were expectantly managed, and 6 (10%) received chemotherapy, radiation or both. Median PFS for the entire group was 53months and OS was 63months. PFS for those who underwent BSO compared with those who retained their ovaries was 38 vs 11months, p=0.071. PFS for HT vs no HT was 28 vs 23months, p=0.77. CONCLUSIONS: Consistent with prior series, our results support BSO to prolong PFS in LG-ESS but are limited by sample size. Larger studies with more complete follow-up are needed to determine the effect of adjuvant hormonal suppression.

[Validation of the French version of the self-evaluation of negative symptoms (SNS)]. / Validation de la version française de l'échelle autoévaluation des symptômes négatifs (SNS).

INTRODUCTION: Schizophrenia is a disorder affecting 1% of the population and is associated with severe functional impairment. Negative symptoms are responsible for the majority of this impairment, and many patients with schizophrenia have negative symptoms. However, their evaluation is still a challenge. Thus, standardized assessments are needed to facilitate identification of these symptoms. Many tools have been developed, but most are based on observer ratings. Self-evaluation can provide an additional outcome measure and allow patients to be more engaged in their treatment. The Self-evaluation of Negative Symptoms (SNS) has been developed recently. This is a remarkably understandable instrument for patients with schizophrenia as it allows them to readily complete it without assistance, providing information with respect to their own perception of negative symptoms. The SNS is a self-assessment that permits patients to evaluate themselves in 5 dimensions of negative symptoms. This validation study for the SNS revealed good psychometric properties alongside satisfactory acceptance by patients. AIM: This study was to confirm the validation of the French version of the self-evaluation of negative symptoms (SNS). METHODS: Patients with schizophrenia or schizoaffective disorder according to the DSM-IV-R, with a stable regimen of anti-psychotic drugs for the last two months, aged more than 18 years old were eligible for the study. Symptoms were rated using the SNS, the Scale for the Assessment of Negative Symptoms (SANS), the Calgary Depression Scale for Schizophrenics (CDSS), the Brief Psychiatric Rating Scale (BPRS) and the Clinical Global Impression and Parkinsonism. Patients were asked to fulfill the SNS twice, 6 weeks apart. RESULTS: Sixty patients were evaluated. Cronbach's coefficient (α=0.8) showed good internal consistency. The SNS significantly correlated with the SANS (r=0.6), the negative sub-score of the BPRS (r=0.6) and the Clinician Global Impression on the severity of negative symptoms (r=0.7). SNS scores did not correlate with level of insight (r=0.08) or Brief Psychiatric Rating Scale positive sub-scores (r=0.2). SNS scores correlated with CDSS scores. However, we did not find correlation between the first item of the CDSS which evaluates depression and the "diminished emotional range" sub-score of SNS. The test-retest of SNS revealed no changes of scores at two evaluations 6 weeks apart. CONCLUSION: The acceptance by patients of the SNS was excellent. The French version of the SNS demonstrated a good internal consistency, good convergent validity and good discriminant validity. The study demonstrates the ability of patients with schizophrenia to accurately report their own experiences. Self-assessments of negative symptoms should be more widely employed in clinical practice because they may allow patients with schizophrenia to develop appropriate coping strategies.

Distinct expression of interleukin (IL)-36α, ß and γ, their antagonist IL-36Ra and IL-38 in psoriasis, rheumatoid arthritis and Crohn's disease.

Interleukin (IL)-36α, IL-36ß and IL-36γ are expressed highly in skin and are involved in the pathogenesis of psoriasis, while the antagonists IL-36Ra or IL-38, another potential IL-36 inhibitor, limit uncontrolled inflammation. The expression and role of IL-36 cytokines in rheumatoid arthritis (RA) and Crohn's disease (CD) is currently debated. Here, we observed that during imiquimod-induced mouse skin inflammation and in human psoriasis, expression of IL-36α, γ and IL-36Ra, but not IL-36ß and IL-38 mRNA, was induced and correlated with IL-1ß and T helper type 17 (Th17) cytokines (IL-17A, IL-22, IL-23, CCL20). In mice with collagen-induced arthritis and in the synovium of patients with RA, IL-36α, ß, γ, IL-36Ra and IL-38 were all elevated and correlated with IL-1ß, CCL3, CCL4 and macrophage colony-stimulating factor (M-CSF), but not with Th17 cytokines. In the colon of mice with dextran sulphate sodium-induced colitis and in patients with CD, only IL-36α, γ and IL-38 were induced at relatively low levels and correlated with IL-1ß and IL-17A. We suggest that only a minor subgroup of patients with RA (17-29%) or CD (25%) had an elevated IL-36 agonists/antagonists ratio, versus 93% of patients with psoriasis. By immunohistochemistry, IL-36 cytokines were produced by various cell types in skin, synovium and colonic mucosa such as keratinocytes, CD68⁺ macrophages, dendritic/Langerhans cells and CD79α⁺ plasma cells. In primary cultures of monocytes or inflammatory macrophages (M1), IL-36ß and IL-36Ra were produced constitutively, but IL-36α, γ and IL-38 were produced after lipopolysaccharide stimulation. These distinct expression profiles may help to explain why only subgroups of RA and CD patients have a potentially elevated IL-36 agonists/antagonists ratio.

Interleukin 34 expression is associated with synovitis severity in rheumatoid arthritis patients.

OBJECTIVES: Interleukin (IL) 34 is a new cytokine implicated in macrophage differentiation and osteoclastogenesis. This study assessed IL-34 expression in the tissue of patients with rheumatoid arthritis (RA). METHODS: Immunohistochemistry was performed in synovial biopsies from patients with RA (n=20), osteoarthritis (n=3) or other inflammatory arthritis (n=4). IL-34 was detected in the synovial fluid by ELISA and its messenger RNA expression was studied by quantitative PCR in rheumatoid synovial fibroblasts after stimulation by tumour necrosis factor α (TNFα) and IL-1ß. Wild-type, jnk1(-/-)-jnk2(-/-) and nemo(-/-) murine fibroblasts and pharmacological inhibition were used to determine the involvement of nuclear factor kappa B (NF-κB) and JNK in that effect. RESULTS: IL-34 was expressed in 24/27 biopsies, with three samples from RA patients being negative. A significant association was found between IL-34 expression and synovitis severity. Levels of IL-34 and the total leucocyte count in synovial fluid were correlated. TNFα and IL-1ß stimulated IL-34 expression by synovial fibroblasts in a dose/time-dependent manner through the NF-κB and JNK pathway. CONCLUSION: This work for the first time identifies IL-34 expression in the synovial tissue of patients with arthritis. This cytokine, as a downstream effector of TNFα and IL-1ß, may contribute to inflammation and bone erosions in RA.

Tolerance and effect of sodium thiosulfate in calcific tendinitis of the rotator cuff.

BACKGROUND: Calcific tendinitis of the rotator cuff is one of the most common causes of shoulder pain. Ultrasound-guided percutaneous lavage of calcific tendinopathy is performed when conservative treatments have failed. Sodium thiosulfate (STS) has recently been used with success in the treatment of tumoral calcinosis. The goal of this phase II study was to assess the tolerance and the feasibility of STS lavage of calcific tendinopathy. METHODS: We included patient with type hard calcifications. Patients were treated with puncture and lavage followed by injection of STS in the calcification. VAS pain at rest and during activities, ultrasound, and X-ray were evaluated at 1 week and 1 and 3 months. RESULTS: Seventeen patients were included. Baseline VAS at rest and during daily activities was a mean 40.2 ± 25.9 and 65.5 ± 21.6 respectively. All patients underwent the entire procedure with no adverse event. Calcium backflow could be obtained in 15 patients (88.2%). Five patients (30%) had more than 50% decrease of their calcific deposit size at 1 month and 8 (47%) patients at 3 months. VAS pain during activities and at rest decreased significantly at 3 months (p = 0.0004; p = 0.001). Efficacy would be demonstrated if 60% of the patients had more than 50% decrease size of their calcification CONCLUSION: Overall, STS was well tolerated with no side effect occurring during the procedure and the follow-up. However, no significant effect on calcium disappearance could be demonstrated compared with what is expected without STS. New studies using larger volume and repeated injections of STS are now needed. CLINICAL TRIAL REGISTRATION NUMBER: NCT02538939Key Points• Lavage of calcific tendinopathy of the rotator cuff with sodium thiosulfate is feasible• No adverse events have been observed after or in the 3 months after the procedure• We could not demonstrate that sodium thiosulfate increases the chance of calcium disappearance• New studies using larger volume and repeated injections of STS are needed to further explore the interest of sodium thiosulfate in the treatment of calcific tendinopathy.

US-guided temporomandibular joint injection: Validation of an in-plane longitudinal approach.

Ultrasonography of the temporomandibular joint is a non-invasive imaging technic, easy to perform in daily practice. It can be used for diagnosis and to guide intra-articular injections. The objective was to validate a longitudinal in-plane US injection approach of the joint and assess its accuracy. We performed a study in 13 non-embalmed cadavers. The injection was done under real-time US guidance using a needle inserted in-plane with an angulation of 30°and positioned under the capsule until the injection was feasible without resistance. The intra-articular injection was successful in all cases and confirmed by a liquid backflow in 96% of cases. The median duration between skin puncture and the intra-articular injection was 23 seconds. Our technique allows a direct visualization of the needle throughout its course to the joint with a high accuracy. Other studies will be needed to confirm its feasibility and usefulness in patients with TMJ disorders.

[Sciatica with motor loss revealing meningoradiculitis due to varicella-zoster virus]. / Sciatique déficitaire révélant une méningoradiculite attachée au virus zona-varicelle.

Herpes zoster is a disease which occurs secondary to the reactivation of varicella-zoster virus. Motor involvement in acute herpes zoster is rare. We report a case of sciatica L5 due to herpes zoster infection with motor loss. Typical skin lesions occurred one week before the sciatica. Radiological finding did not explain the paresis. The diagnosis of zoster sciatica with motor involvement was suspected. Serological tests and cerebrospinal fluid examination established the diagnosis. The antiviral and physical treatment was conducted in order to improve functional outcome.

DU145 human prostate cancer cells express functional receptor activator of NFkappaB: new insights in the prostate cancer bone metastasis process.

Prostate cancer metastases to bone are observed in around 80% of prostate cancer patients and represent the most critical complication of advanced prostate cancer, frequently resulting in significant morbidity and mortality. As the underlying mechanisms are not fully characterized, understanding the biological mechanisms that govern prostate cancer metastases to bone at the molecular level should lead to the determination of new potential therapeutic targets. Receptor activator of NFkappaB ligand (RANKL)/RANK/Osteoprotegerin (OPG) are the key regulators of bone metabolism both in normal and pathological condition, including prostate cancer bone metastases. In the present study, we demonstrated that human prostate cancer cell lines, DU145 and PC3 express biologically functional RANK. Indeed, soluble human RANKL (shRANKL, 100 ng/ml) treatment induced ERK 1/2, p38 and IkappaB phosphorylations in these cells. shRANKL administration also promoted DU145 and PC3 prostate cancer cell invasion in vitro. Whereas human OPG (hOPG) administration alone (100 ng/ml) had no marked effect, combined association of both agents abolished the RANKL-induced DU145 cell invasion. As RANKL had no direct effect on DU145 cell proliferation, the observed effects were indeed related to RANKL-induced cell migration. DU145 human prostate cancer cells promoted osteoclastogenesis of osteoclast precursors generated from mouse bone marrow. Moreover, DU145 cells produced soluble factor(s) that up-regulate the proliferation of MC3T3-E1 pre-osteoblasts through the activation of the ERK 1/2 and STAT3 signal transduction pathways. This stimulation of pre-osteoblast proliferation resulted in an increased local RANKL expression that can activate both osteoclasts/osteoclast precursors and prostate cancer cells, thus facilitating prostate cancer metastasis development in bone. We confirm that RANKL is a factor that facilitates metastasis to bone by acting as an activator of both osteoclasts and RANK-positive prostate cancer cells in our model. Furthermore, the present study provides the evidence that blocking RANKL-RANK interaction offer new therapeutic approach not only at the level of bone resorbing cells, but also by interfering with RANK-positive prostate cancer cells in the prostate cancer bone metastasis development.

Photoimmunotherapy of human ovarian carcinoma cells ex vivo.

Photodynamic therapy is a relatively new and potentially selective experimental approach to the treatment of malignant neoplasms. Its inherent dual selectivity is reinforced by the use of photosensitizer-monoclonal antibody conjugates. The goal of this study was to evaluate the phototoxicity and selectivity of an immunoconjugate (IC) synthesized from a chlorin derivative chlorin e6-monoethylenediamine monoamide (CMA) as the photosensitizer and an anti-ovarian carcinoma monoclonal antibody OC125. Binding efficiency and specificity of the IC were determined by enzyme-linked immunosorbent assay, and specific covalent linkage of the monoclonal antibody to the photosensitizer was demonstrated by fluorescence and electrophoresis. Phototoxicity was tested against ascites or pleural fluid cells from 15 patients with ovarian and nonovarian cancers. Tumor cells from the fluid were treated with the IC at 3 microM equivalent CMA concentration and irradiated at 654 nm (lambda max CMA in IC) at 25 J/cm2 from an argon ion-pumped dye laser. Phototoxic efficacy was assayed by [3H]thymidine incorporation. Ovarian cancer cells exhibited high cytotoxicity with [3H]thymidine incorporation of 2.4 +/- 2.2%, while nonovarian cancer cells under identical conditions exhibited none to reduced cytotoxicity with [3H]thymidine incorporation of 70 +/- 54%. Using a Wilcoxon test, there was a statistically significant difference between these two groups (P less than 0.001). Dose-response curves revealed reciprocity in photosensitizer concentration and fluence. These results demonstrate that photoimmunoconjugates retain significant antigen binding specificity and affinity, are effective in the selective photochemical eradication of target cells, and merit further evaluation as photochemotherapeutic agents.

Interaction between a tannin-containing legume and endophyte-infected tall fescue seed on lambs' feeding behavior and physiology.

It was hypothesized that a tannin-rich legume such as sainfoin attenuates the negative postingestive effects of ergot alkaloids in tall fescue. Thirty-two 4-mo-old lambs were individually penned and randomly assigned to a 2 × 2 factorial arrangement with 2 legume species, sainfoin (SAN; 2.9% condensed tannins) or cicer milkvetch (CIC; without tannins) and a mixed ration containing tall fescue seed (50:30:20 seed:beet pulp:alfalfa) with 2 levels of endophyte infection (endophyte-infected tall fescue seed [E+; 3,150 ug/L ergovaline] or endophyte-free tall fescue seed [E-]). For a 10-d baseline period, half of the lambs were fed SAN and half were fed CIC and all lambs had ad libitum amounts of E-. In an ensuing 10-d experimental period, the protocol was the same except half of the lambs fed SAN or CIC received E+ instead of E-. Subsequently, all lambs could choose between their respective legume and seed-containing ration and between E+ and E-. Finally, an in vitro radial diffusion assay was conducted to determine whether tannins isolated from SAN would bind to alkaloids isolated from E+. All groups consumed similar amounts of E- during baseline period ( > 0.10), but lambs ate more E- than E+ during the experimental period ( < 0.05) and lambs offered SAN ate more E+ than lambs offered CIC ( < 0.05). Groups fed E- during the baseline and experimental periods had similar rectal temperatures ( > 0.10), but lambs fed E+ had lower rectal temperatures per gram of feed ingested when supplemented with SAN than with CIC ( < 0.05). Lambs fed E+ had greater concentrations of hemoglobin and more red blood cells than lambs fed E- ( < 0.05), but plasmatic concentrations of cortisol and prolactin did not differ among treatments ( > 0.10). All lambs preferred their treatment ration over their treatment legume, but lambs in the SAN and E+ treatment ate more legume + ration than lambs in the CIC and E+ (CIC-E+; < 0.05) treatment. All lambs preferred E- over E+, but lambs in the CIC-E+ treatment ate the least amount of E+ ( < 0.05). Binding of isolated SAN tannins to protein was reduced by the E+ isolate ( < 0.05), suggesting a tannin-alkaloid complexation but only from tannins extracted from SAN fed early in the experimental period. In summary, SAN supplementation increased intake of and preference for E+ and reduced rectal temperatures relative to CIC supplementation. Our results suggest that these effects were mediated by the condensed tannins in SAN through alkaloid inactivation, an interaction that declined with plant maturity.

[Three cases of Jaccoud's arthropathy during systemic sclerosis]. / Trois observations de rhumatisme de Jaccoud au cours de la sclérodermie systémique.

INTRODUCTION: Jaccoud's arthropathy (JA) is a chronic and non-erosive deforming arthropathy, usually affecting the hands. JA pathophysiology is poorly known but involves periarticular structures such as tendons and the joint capsule. JA is associated with various conditions including the connective tissue disease, especially systemic lupus erythematosis. JA has been rarely described and studied in systemic sclerosis. CASE REPORTS: We report the clinical histories of 3 patients with systemic sclerosis (ScS) who developed JA. One patient had a systemic limited disease and the 2 others a cutaneous limited disease ; mean age of the patients was 79.3 years. Systemic sclerosis was diagnosed respectively 19, 1 and 21 years prior to the development of JA. One of the 3 patients had a past clinical history of discoid lupus. For 1 out of the 3 patients, JA appeared whereas the ScS was completely stable. The disease was still active in the 2 remaining patients, with concurrent pulmonary hypertension diagnosis. Deformities increased during years (Z thumbs, ulnar deviation), leading to mild to severe disability. No benefit from either prednisone (n=2) or a combination of prednisone and methotrexate (n=1) was obtained. CONCLUSION: We described 3 cases of Jaccoud's arthropathy among our scleroderma cohort of 296 patients (1%). This arthropathy worsens hand functional disability. Its pathophysiology is unknown and optimal therapeutic approach remains to establish.

Presence of an intracompartmental septum detected by ultrasound is associated with the failure of ultrasound-guided steroid injection in de Quervain's syndrome.

UNLABELLED: The purpose of this study was to find clinical or ultrasound characteristics that might predict the failure of conservative treatment in de Quervain's syndrome. A total of 42 ultrasound-guided injections have been performed in 41 patients after clinical and ultrasound examination. Patients were immobilized for 3 weeks with a spica splint cast, and clinically evaluated at 3 and 6 weeks and by phone call at the end of the study. Ultrasound showed a septum between the tendons of the first comportment in 34% of the wrists. At last follow-up (mean 15.6 months after the injection) ten patients (24%) had undergone surgery. When comparing ultrasound and clinical characteristics of the operated and non-operated wrists, we found that patients with a high baseline visual analogue scale, with all positive clinical tests and with a persistent intracompartmental septum, had a significantly higher risk of failure following conservative treatment. LEVEL OF EVIDENCE: III.

Gangliosides of human acute leukemia cells.

We characterized the gangliosides from cells of eight patients with different forms of acute leukemia (four lymphoblastic, four nonlymphoblastic) by thin-layer chromatography and high-performance liquid chromatography combined with glycosidase treatment. Our analysis indicated both quantitative and qualitative differences between the gangliosides of acute leukemia and those of normal leukocytes: 1, the absolute amount of ganglioside was decreased in the acute leukemia cells; 2, in general, acute leukemias had a more simplified ganglioside pattern in that they contained a greater proportion of the short-chain ganglioside, II3NeuAc-LacCer (GM#); 3, all of the acute leukemia cells contained reduced quantities of the ganglioside N-acetylneuraminosyl-lactotriaosylceramide, a compound previously found only in normal leukocytes; and 4, a disialylated ganglioside, II3(NeuAc)2-LacCer (GD3), which is not found in normal leukocytes, was isolated from the cells of one patient with acute nonlymphoblastic leukemia. These findings demonstrate important differences between the gangliosides of acute leukemia cells and normal leukocytes.

Extrauterine pregnancy resulting from early uterine rupture.

BACKGROUND: Cesarean scar rupture of a gravid uterus in early gestation is rare. CASE: A 38-year-old woman, gravida 4, para 2-0-1-1, presented at 13 weeks' gestation with cramping and spotting. She had a history of two cesareans. Ultrasound and magnetic resonance imaging indicated probable uterine dehiscence and a viable extrauterine pregnancy. After embolization of the uterine arteries with subsequent fetal death, the subject had a hysterectomy. Intraoperatively, she had complete rupture of the lower uterine segment, but the pregnancy was enclosed within scar tissue between the uterus and bladder. Placenta percreta was found by histologic examination. CONCLUSION: Women with histories of cesareans might be at risk of early uterine rupture.

Teaching and evaluating surgical skills.

OBJECTIVE: To examine how surgical skills are taught and evaluated in obstetrics-gynecology residency programs in the United States. METHODS: A questionnaire was mailed to the directors of all 266 residency programs in the United States and to second contact names at 51 sites. Directors were asked to evaluate how surgical skills are taught and evaluated and to rate the importance of specific techniques and procedures for residents at given points in resident training. RESULTS: Two hundred twenty-three surveys were returned (70%), representing 203 of 266 programs (76%). Among responding programs, 99% reported teaching surgical skills in operating rooms, 88% in lectures, 68% with bench procedures, and 54% with animal surgery. Twenty-nine percent indicated they had formal surgical skills curricula. A significantly higher percentage of those programs with formal curricula used animal surgery laboratories (81% versus 42%, P <.001) and were more likely to conduct formal skills assessments (88% versus 69%, P =.005) than programs without formal curricula. Overall, 74% of programs evaluated surgical skills. Of those, 56% reported using subjective faculty evaluations, 12% written evaluations (eg, checklists), 4% written and oral assessments, and 1% a test. Regardless of formal curricula, there was much agreement in respondents' ratings of 60 different skills and procedures as "essential," "important," "nice to know," or "unimportant. CONCLUSION: Most programs teach surgical skills in the operating room and through lectures. Only 29% of reporting programs provide formal surgical curricula. Evaluation of surgical skills is usually done by subjective evaluation, a technique with unknown validity and poor reliability.

Preoperative angiographic uterine artery embolization in the management of cervical pregnancy.

The diagnosis of a cervical ectopic pregnancy is often made intraoperatively in the presence of extensive hemorrhage during attempted evacuation. In some cases, hysterectomy has been the necessary treatment to control bleeding. We report two cases of cervical pregnancy detected by ultrasound. Before pregnancy termination, the uterine arteries were successfully embolized using angiographic techniques. As a result, surgical evacuation was performed with minimal hemorrhage; hysterectomy was not required and the patients' potential fertility was retained.

Small chest-tube drainage followed by bleomycin sclerosis for malignant pleural effusions.

OBJECTIVE: To review our experience with bleomycin sclerotherapy as treatment of pleural effusions due to gynecologic malignancies. METHODS: Twenty-one women with histologically documented malignant effusions (16 ovarian carcinoma, three uterine sarcoma, and two cervical cancer) were treated with small flexible chest-tube drainage followed by intrapleural bleomycin sclerotherapy (60 units). RESULTS: After placement of the chest tube, suction was required for a median of 5 days (range 3-12) before output was low enough (less than 100 mL/24 hours) to instill bleomycin. Among 24 treated effusions, there was a 71% overall response rate, including ten complete responses (42%) and seven partial responses (29%); seven effusions (29%) did not respond to therapy. Six of the seven patients whose effusions did not respond to bleomycin died of disease within 2 months of attempted sclerotherapy. Fever was the most common side effect, occurring in 13 of 21 patients (62%) following instillation of bleomycin. Pain during sclerosis was reported by only two patients. CONCLUSIONS: Bleomycin sclerotherapy after small flexible chest-tube drainage of malignant pleural effusions is an effective technique, with minimal adverse reactions, for controlling effusions that develop in women with gynecologic malignancies.

Pseudomyxoma peritonei: a review of 23 cases.

OBJECTIVE: To review the experience at the Massachusetts General and Brigham and Women's Hospitals with 23 women treated for pseudomyxoma peritonei between 1961 and 1991. METHODS: Patients were identified retrospectively from the tumor registry at the Massachusetts General and Brigham and Women's Hospitals, and all charts were reviewed. The median follow-up interval was 2.5 years (range 3 months to 31 years). RESULTS: The mean age at diagnosis was 58 years (range 26-76). Pseudomyxoma peritonei was found in association with ten (44%) ovarian tumors of borderline malignancy, nine (39%) ovarian cystadenocarcinomas, and four (17%) appendiceal cystadenocarcinomas. Three patients had synchronous tumors in the ovary and appendix. All patients underwent surgical staging and cytoreduction. Eleven patients received postoperative therapy and, of these, nine developed a recurrence; 12 patients received no further therapy and, of these, three developed a recurrence. However, these groups were not pathologically comparable. With respect to survival, of the ten patients with borderline malignancies, seven had no evidence of disease, one was alive with disease, and two died of disease. For the nine patients with ovarian cystadenocarcinomas, three had no evidence of disease, one was alive with disease, and five died of disease (median time to death 18 months). For the four patients with appendiceal carcinomas, two had no disease, one was alive with disease, and one died with disease. Among all 23 patients, 12 (52%) developed a recurrence, with a range of time to first recurrence of 3 months to 19 years. Eight women required at least one additional laparotomy because of accumulation of gelatinous material. CONCLUSIONS: Although pseudomyxoma peritonei is associated with borderline and well-differentiated tumors, recurrence is common and the prognosis after recurrence is guarded. Involvement of the appendix is common; therefore, appendectomy is indicated when pseudomyxoma is encountered. To date, surgery has been the only effective therapy for this disease, and adjuvant therapy has not been shown conclusively to be of benefit.

Endocervical glandular atypia in Papanicolaou smears.

The importance of endocervical glandular atypia in a cervicovaginal Papanicolaou smear has not been fully investigated. Between July 1988 and June 1989, 21,930 cervicovaginal smears were reviewed by the Massachusetts General Hospital Cytopathology Laboratory. One hundred smears with endocervical atypia were identified, an incidence of 0.46%. Follow-up was available on 63 cases: Seven had negative follow-up smears for at least 2 years, 15 had negative biopsies, seven had endocervical polyps, two had endometrial hyperplasia, eight had mild dysplasia, five had moderate dysplasia, six had severe dysplasia, six had squamous carcinoma in situ, five had adenocarcinoma in situ, and two had invasive adenocarcinoma. Twelve women's smears showed endocervical atypia with features suggestive of reactive atypia; three of these had dysplasia. Twenty-six (41%) of the Papanicolaou smears with endocervical atypia had coexisting squamous atypia or dysplasia. We conclude that endocervical atypia may be associated with substantial cervical disease in as many as half of cases.