The prognosis of female genital sarcomas of the adult woman with special reference to sarcomas of the uterine corpus (author's transl)]

Report on 88 cases of sarcomas of the female genital organs. The sarcomas were confined to the uterine body in 72 cases. The prognosis of this disease depends primarily on the adequacy of the treatment. The treatment consists of as radical an operation as possible followed by external radiation and intra-vaginal application of radium. The microscopic type of the sarcoma of the uterus is of secondary importance. Patients who survived for 3 years following this treatment have a good prognosis for a 5 year or a 10 year cure. Other sarcomas of the female genital tract are mentioned.     

Microsatellite instability in gynecological sarcomas and in hMSH2 mutant uterine sarcoma cell lines defective in mismatch repair activity

We have examined a panel of gynecological sarcomas for microsatellite instability. The genomic DNA from 11 of 44 sarcomas contained somatic alterations in the lengths of one or more di-, tri-, tetra-, or pentanucleotide microsatellite sequence markers, and 6 of these cases had alterations in two or more markers. In addition, di-, tri-, and tetranucleotide microsatellites were found to be highly unstable in single cell clones of two cell lines derived from a uterine mixed mesodermal tumor. Since such instability is characteristic of cells defective in postreplication mismatch repair, we examined mismatch repair activity in extracts made from these lines. Both extracts were repair deficient, while an extract of another gynecological sarcoma cell line not exhibiting microsatellite instability was repair proficient. The repair deficiency was complemented by a colon tumor cell extract that was defective in the hMLH1 protein but not by an extract defective in hMSH2 protein. This suggested that the defect in the uterine sarcoma line could be in hMSH2. Subsequent analysis of the gene revealed a 2-bp deletion in exon 14, leading to premature truncation of the hMSH2 protein at codon 796 and no detectable wild-type gene present. These data suggest that the microsatellite instability observed in these cell lines, and possibly in a significant number of gynecological sarcomas, is due to defective postreplication mismatch repair. There was no apparent correlation with microsatellite instability and clinical outcome.     

Prognostic factors in uterine sarcomas: a 21-year retrospective study at the Clinic and Provincial Hospital of Barcelona

BACKGROUND: Uterine sarcomas show low incidence and poor outcome despite the treatment. The prognostic factors for the survival were determined in this study. PATIENTS AND METHODS: Thirty-nine females with sarcoma of the uterus have been studied retrospectively from January 1975 to December 1996. They were treated in the Gynecology and Radiation Oncology Departments at Hospital Clínic i Provincial of Barcelona. Thirty-seven patients had surgery, 22 radiotherapy and 4 chemotherapy. The influence on the disease-specific survival, disease-free survival, local relapse disease-free survival and metastasis disease-free survival from the following pronostic factors was studied: age, pathologic subtype, miometrial invasion, mitosis, vascular and lymphatic invasion, tumor size, stage, radiotherapy and local relapse. RESULTS: 1) The disease-specific survival at 2 and 5 years was 51.5% and 42.5% respectively, and the disease-free survival at 2 and 5 years was 39%; the incidence of local and distant relapses--was 28 and 33%. 2) The multivariate analysis showed that the overall survival and the disease free survival were affected by the vascular invasion (odds ratio [OR] 12 and 32.6, respectively) and the local failure (OR = 3 and 25.5, respectively); the only factor that affected the local relapse-free survival and metastasis free survival was the III and IV stages (OR = 5.6 in both cases). CONCLUSIONS: In uterine sarcomas, the vascular invasion and the local relapse were prognostic factors for overall survival and for disease-free survival. In stages III and IV there was a decrease in the local relapse-free survival and metastasis-free survival. A correlation between vascular invasion and advanced stages was found. The outcome of the uterine sarcomas is poor, local and distant failure being responsible for this bad prognosis.     

Musical auditory hallucinosis from Listeria rhombencephalitis

We present the unusual case of a 27-year-old Japanese woman who exhibited recurrences of metastatic tumors from uterine leiomyosarcoma after five operations. Chemotherapy with cisplatin, pirarubicin, and cyclophosphamide was ineffective. Substituting ifosfamide for cyclophosphamide, we obtained a complete response. The Gynecologic Oncology Group (GOG) in the United States has recognized the efficacy of ifosfamide and mesna in the treatment of patients with advanced or recurrent uterine sarcomas. Here we report on the efficacy of ifosfamide in addition to cisplatin and pirarubicin.     

Florid cystic endosalpingiosis with tumor-like manifestations: a report of four cases including the first reported cases of transmural endosalpingiosis of the uterus

Four cases of endosalpingiosis presenting as masses that resembled neoplasms are described in women 20, 41, 43, and 74 years of age. Each case was referred in consultation because of difficulties in pathologic diagnosis. In two patients, multiple cysts that involved the serosal surfaces of the uterus and adnexa in one case, and the colon, rectosigmoid, pelvic sidewalls, and the cul-de-sac in the other, were excised. In the other two cases, hysterectomy was performed for an enlarged cystic cervix in one case and presumed uterine leiomyomas in the other. In both of these cases, the uterine cervix and lower part of the uterine corpus were extensively involved by multiple cysts on gross examination, and in one of them, a frozen section of the cervical lesion was initially interpreted as "suspicious for invasive minimal deviation adenocarcinoma." On microscopic examination, benign endosalpingiotic glands and cysts were found in all four cases, with striking transmural involvement of the uterine cervix and lower uterine segment and contiguous corpus in the two cases with uterine involvement. The latter two cases are the first examples, to our knowledge, of endosalpingiosis involving the wall of the uterus; the differential diagnosis in these cases includes minimal deviation adenocarcinoma and florid tubal metaplasia with cystification. The four cases in this report, and rare previously reported cases, indicate that although usually a microscopic finding, endosalpingiosis can rarely present as a clinically or grossly evident mass that can be confused with a neoplasm.     

Manual removal of the placenta--its role in intrauterine adhesion formation

OBJECTIVE: To determine the role of postpartum intrauterine manipulation of the uterine cavity in the formation of intrauterine adhesions. PATIENTS AND METHODS: Diagnostic hysteroscopy was performed following manual removal of the placenta in 48 patients. RESULTS: Intrauterine adhesions were observed in only one case (2%), and an incomplete uterine septum was found in seven cases (15%). CONCLUSIONS: Manual removal of the placenta does not seem to predispose to the formation of uterine adhesions. Incomplete uterine septum may be involved in some of the cases of retained placenta.     

Uterine leiomyosarcoma with subsequent pregnancy]

Leiomyosarcoma are malignant tumors showing smooth muscle differentiation, they are rare, representing only about 25 percent of all uterine sarcomas and mixed malignant tumors and slightly more than 1 percent of all corporeal malignant tumors. Usually they arise in postmenopausal women and are not known to be related to the known risk factors for endometrial carcinoma (nulliparity, obesity, diabetes mellitus, hypertension, etc.) or carcinosarcoma (prior radiation therapy). They may occur in uteri that also bear typical benign leiomyomas, but leiomyosarcoma rarely can be proven to have arisen in or from benign leiomyomas. We report on two premenopausal women with leiomyosarcoma--out of totally 31 cases in our clinic from 1975-1995 who got pregnant after surgical treatment preserving the uterus. The patients are alive without evidence of disease 3 and 6 years after surgical resection.     

Gait patterns in children with hemiplegic spastic cerebral palsy

Our objective was to study uterine and umbilical artery flow resistance during the oxytocin challenge test (OCT). The study population was 21 women with suspected placental insufficiency; one woman was excluded because of a positive OCT with reactive fetal heart rate pattern. We carried out simultaneous electronic fetal heart rate monitoring and Doppler velocimetry of uterine and umbilical artery flow during the OCT. The uterine artery flow resistance increased significantly during contractions in both OCT-positive (n = 5) and OCT-negative (n = 15) cases compared with basal values, but the increase was significantly higher in positive cases. The umbilical artery flow resistance increased significantly during contractions in OCT-positive cases, but was almost unchanged in negative cases. During uterine inactivity, there were no differences between the groups for any vessel. This study showed that fetal heart rate decelerations during the OCT are associated with rapid and exaggerated increases of vascular resistance in both uterine and umbilical arteries. The causal relationship is unknown, but the findings indicate pathophysiological mechanisms revealed only during uterine contractions.     

Strain differences in susceptibility of female mice to 1,2-dimethylhydrazine

C3H, CBA, C57BL/6j, (CBA x C57BL/6j)F1, BALB/c, DBA/2, C3HA and AKR female mice were treated with 25 weekly s.c. injections of a solution of 1,2-dimethylhydrazine (DMH) in water at a dose level of 8 mg/kg body weight. BALB/c mice appeared to be most sensitive to the induction of epithelial colorectal (93.3%) and anal tumours by DMH. There was, however, a dissociation between the severity of the macroscopical tumour lesions in the colon of BALB/c mice and their relatively weak tendency to infiltrative growth. C3HA mice were more resistant to the induction of intestinal tumours (30.9%) but the tumours showed a deep invasion into the intestinal wall. There was no correlation between the strains and within a given strain between the development of colorectal and anal neoplasms. C3H and CBA mice strains developed a high incidence of uterine sarcomas (37.5 and 40.7%, respectively) which were not found at all in BALB/c, DBA/2 and C3HA mice and which appeared in C57BL/6j and AKR mice at low frequency (2.7 and 7.7%, respectively). C57BL/6j, BALB/c, DBA/2 and C3HA mice developed haemorrhagic lesions of the ovaries (35.1, 46.7, 62.9 and 85.7%, respectively). These lesions, which led to peritoneal haemorrhage, were one of the main causes of death in C3HA and DBA/2 strains. It seems that, with the exception of AKR mice, an inverse relationship exists between the occurrence of haemorrhagic ovarian lesions and development of uterine sarcomas in female mice treated with DMH.     

Suppression of E-cadherin and alpha- and beta-catenin mRNA expression in the metastatic lesions of gynecological cancers

To know the biological role of adherens junction, mainly consisting of E-cadherin and alpha- and beta-catenins, associated with invasion and metastasis of ovarian, uterine endometrial and cervical cancers, we studied the expression of E-cadherin and alpha- and beta-catenin mRNAs in the metastatic lesions in comparison with those in the primary tumors. The integral expression of E-cadherin and alpha- and beta-catenin mRNAs in the metastatic lesions in comparison with that of the primary tumors was suppressed in 4 of 5 cases of ovarian cancers, all cases of uterine endometrial cancers, and 4 of 5 cases of uterine cervical cancers. Therefore, the suppressed expression of the main adhesion molecules in the adherence junction might contribute to the cell-to-cell junctional dysfunction, which might lead to the acquisition of invasiveness and metastatic potential of gynecological cancers as one of the rate-limiting steps.     

Synovial sarcoma of the parotid gland. A case report with clinicopathological analysis and review of the literature

Primary sarcomas of the major salivary glands are exceptionally uncommon. Synovial sarcoma is a distinctive neoplasm which usually arises in the extremities in the region of joints. Although synovial sarcomas arising in the head and neck region are well described, tumours actually originating in and around the major salivary glands are exceedingly rare, with few cases reported in the literature. We report a synovial sarcoma in the right parotid gland of a 67-year-old man. The literature pertaining to salivary gland sarcomas and synovial sarcoma of the head and neck region is reviewed.     

Cytology of malignant endometrial tumors

Endometrial cytology is made by direct endometrial sampling. It allows the appreciation of the hormonal status and the diagnosis of inflammatory and/or tumoral pathology. Evidence has been presented that the rate of endometrial cancer is on the increase. Thus, detection and diagnosis have acquired a new significance, where endometrial cytology takes a particular place. Cytologic features of adenocarcinomas in their different kinds are presented. Furthermore, cytologic aspects of uterine sarcomas are outlined, including leiomyosarcomas whose diagnosis can be made by the mean of endometrial cytology.     

Nucleosome-releasing treatment makes surviving tumor cells better targets for nucleosome-specific anticancer antibodies

INTRODUCTION: Leiomyosarcomas are malignant tumours showing smooth muscle differentiation. They represent approximately 25% of all uterine sarcomas and slightly over 1% of all uterine malignancies. The purpose of the present retrospective review is to relate clinical and pathological findings of leiomyosarcomas of the female genital tract to prognosis. MATERIAL AND METHODS: During 1972-1992 eleven patients had diagnosed uterine leiomyosarcomas treated at the Department of Gynecology of the University of Saarland. The hospital records of all patients were reviewed and complete primary treatment had been performed at this center. RESULTS: The mean age was 46.92 years (SD: +/-13.85). Atypical uterine bleeding and pelvic discomfort were the most common presenting complaints (72.7%). The mean follow-up time was 59.60 months (20-96 months). Overall 2-year survival was 70% and overall 5-year survival 30%. The overall survival of patients in FIGO-stage I was 57.14%, in FIGO-stage II 100%, in FIGO-stage III 0% and in FIGO-stage IV 0%. CONCLUSION: The primary therapy should consist of an operation as radical as possible. Treatment with organ preserving seems to be reasonable if the patient desires children. Also, chemotherapy might provide a hopeful sign in the improvement of survival rates.     

Straddle injuries--is current practice safe?

OBJECTIVE: To study the management of labor following two cesarean sections, we evaluated maternal and neonatal morbidity subsequent to this attitude. PATIENTS AND METHODS: A retrospective study was conducted over a 6-year period (1-1-1990 to 31-12-1995) in 184 women presenting two uterine scars. Trial of labor was allowed in 96 cases with cephalic presentation and a normal pelvis. RESULTS: The rate of vaginal birth was 65%. Three patients presented a uterine scar dehiscence and in one of them hemostasis hysterectomy for uterine atony was performed. Neonatal outcome was good in all cases. CONCLUSION: Trial of labor after two cesarean sections is possible in the majority of cases. Rate of vaginal birth is high and maternal and fetal morbidity is low.     

Accuracy of roentgenograms of the chest in metastases to the lungs

The findings on roentgenographic and tomographic examination of the chest were compared in 63 instances of suspected pulmonary metastases. These were further compared with the findings at thoracotomy in 41 instances. Of the 60 patients, 30 had soft tissue or bone sarcomas and 30 had carcinomas arising from colon, ovary or breast, and it also included five with malignant melanoma. Three patients with sarcomas had more than one thoracotomy. The diagnosis of pulmonary metastases by roentgenography of the chest was correct in 60 of 63 instances. Tomograms showed more lesions in 14 of 33 instances of sarcomas and 14 of 30 instances of carcinomas. Thoracotomy revealed even more lesions than were detected by tomography in 21 out of 26 instances with sarcomas and eight of 15 instances of carcinomas. Of the 37 patients with a solitary metastasis detected on roentgenograms of the chest, 22 were found to have additional lesions on the tomograms, 11 of 16 sarcomas and 11 of 21 carcinomas. At thoracotomy, however, nine of ten patients with a single metastasis from sarcomas were found to have even more lesions, while, in patients with carcinomas, tomograms were found to be accurate. Routinely, prior to major ablative operations for sarcomas and before excision of pulmonary metastases, it is suggested that tomography be carried out.     

Non-pigmented extrabulbar sarcomas of the eye-region (author's transl)

The authors report on 30 cases with non-pigmented extrabulbar sarcomas of the eye-region which occurred in our clinic within the period of 1955 till 1975. The primary sarcomas of the orbit predominate with 11 cases over the secondary involvement with 9 cases. More seldom (5 cases) is the involvement of the lids and the epibulbar space. The various groups of lymphosarcoma predominate with 16 cases over the spindle-cell sarcoma with 6 cases. The age distribution shows that these tumors prefer the first three decades and the fifth and the following decennia. The prognosis is bad for the conservation of the eye as well as for the preservation of life. With reference to the localisation the distribution at the time of the beginning of the therapy as well as the histological findings an intensive therapy (operation, radiation, chemo-therapy) has to be accomplished.     

Cytomorphologic features of fine-needle aspiration of liposarcoma

The cytomorphologic features of fine-needle aspirates (FNA) of liposarcomas have not been fully characterized. In the current study, we attempted to identify cytological attributes of liposarcoma in FNA. Twelve FNAs of histologically proven liposarcomas were used for this study. They included 10 myxoid and two well-differentiated liposarcomas. Four cytological criteria were chosen for evaluation. These included a complex capillary network, increased cellularity, the presence of a metachromatic stroma for myxoid liposarcoma, and finally the identification of lipoblasts. Eighteen FNAs of lipomas and 17 FNAs of nonlipomatous soft-tissue sarcomas were used as controls for evaluation of the same features. The diagnosis of liposarcoma had been rendered on FNA in 7 cases (58%). The diagnosis was suggested in 3 cases (25%), and a suggestion of sarcoma was rendered in 2 cases. Complex capillary networks were identified in 9 of 12 cases (75%); this was seen in 8 of the 10 myxoid liposarcomas and 1 of the 2 well-differentiated neoplasms. Increased cellularity was seen in 7 of 12 cases (58%), metachromatic stroma was seen in 3 of 12 cases (25%), and lipoblasts were identified in 4 of 12 cases (33%). None of the lipomas or sarcomas exhibited a complex capillary network. When capillaries were identified in lipomas, they were straight, with minimal branching. The lipomas did not show evidence of increased cellularity in any cases. Metachromatic stroma was more frequently seen in the nonlipomatous sarcomas than in the liposarcomas. No lipoblasts were identified in any of the control FNAs.     

Primary tumors of the thoracic skeleton

From 1975 to 1990, eighty-nine primary tumors of the thoracic skeleton; ribs, sternum, scapula, clavicle, and thoracic spine, were treated. Forty-four tumors (49%) were benign lesions. Forty-five tumors were malignant and were proportionately distributed amongst the five sites. The most common malignancies were Ewing's sarcomas, chondrosarcomas, plasmacytomas, osteogenic sarcomas, and lymphomas. All patients with Ewing's sarcomas were treated with combination chemotherapy, surgical resection, and radiation therapy for those with residual disease after surgery. Only one patient has died of disease. Patients with primary chondrosarcomas were treated by surgery alone and all are free of disease or have died without disease. Patients with solitary plasmacytomas or primary lymphomas of bone were treated with radiation therapy initially. Half the patients developed systemic disease. The patients with osteogenic sarcomas included several with radiation induced lesions and Paget's osteosarcoma and all but one died of disease.     

Geographic variations in breast cancer mortality: do higher rates imply elevated incidence or poorer survival?

A 46-year-old women presented with an inoperable low-grade endometrial stromal sarcoma. Two doses of Depo-Lupron, 7.5 mg, and Megace, 160 mg/day, were given to control uterine bleeding and shrink the tumor mass. In 9 weeks, significant reduction in the tumor occurred allowing for surgical resection. Total abdominal hysterectomy with bilateral salpingo-oophorectomy is the mainstay for primary treatment. The role of chemotherapy, radiation therapy, and hormonal therapy is poorly defined. This is a case report of neoadjuvant hormonal therapy which may improve outcomes in patients with endometrial stromal sarcomas. Additional research is needed to define the exact role of these agents.