Extensively calcified synovial sarcoma

OBJECTIVE: To determine the changes of T lymphocyte subsets, natural killer (NK) cell and serum interleukin-2 receptor (SIL-2R) in peripheral blood of the pregnant women with systemic lupus erythematosus (SLE). METHODS: T lymphocyte subsets NK cell, and SIL-2R in peripheral blood were detected by flow cytometry (FCM) and enzyme-labeled immunosorbent assay (ELISA) in 14 cases of pregnant women complicated by SLE (SLE+NP), 18 cases of stationary phase SLE (SLE), 20 cases of normal non-pregnant women (NNP) and 20 cases of normal pregnant women (NP). RESULTS: The percentages of CD4+ cell were decreased significantly in SLE+NP group as compared with the other three groups (P < 0.01); and the ratio of CD4+/CD8+ was decreased significantly in SLE+NP group, as compared that in SLE group and in NNP group (P < 0.01). There were no difference in the number of NK cells among the four groups. The SIL-2R values were found to be increased significantly in the SLE+NP group, as compared with that of other three groups (P < 0.01). CONCLUSION: The observation on the changes of peripheral T lymphocyte subsets and SIL-2R may be helpful for monitoring progress of SLE during pregnancy.     

Primary biphasic synovial sarcoma of the pleura

Synovial sarcoma most commonly occurs in the peri-articular regions of the extremities. The present report describes a rare case of primary biphasic synovial sarcoma of the pleura in an 18-year-old female. The diagnosis was made on the basis of light microscopy, immunohistochemistry, electron microscopy and the characteristic translocation found on cytogenetic analysis. Synovial sarcoma should be included in the differential diagnoses of pleural tumors.     

心包原发性滑膜肉瘤1例报道

Primary pericardial sarcomas are extremely rare. We report a case of 19 year old male who presented with cough,dyspnoea, and orthopnea. Investigations and exploratory thoracotomy revealed a large pericardial mass. Surgical debulking of the tumor was performed and the histopathological examination was compatible with synovial sarcoma. The tumor was unresectable due to its invasion and adhesion to mediastinal structures. Hence patient was started on palliative chemotherapy (adriamycin and ifosfamide based). Patient showed an initial symptomatic response but later on there was a clinical progression and died within six months of his diagnosis.     

Oropharyngeal synovial sarcoma. Report of one case]

OBJECTIVE: To assess the prognostic factors of myocardial recovery expected after coronary bypass surgery and the impact of surgical technique used, a prospective non-randomized study including a 1-year postoperative evaluation of left ventricular function was performed in patients with left ventricular dysfunction (left ventricular ejection fraction (LVEF) < 0.40). METHODS: From 1993 to 1996, 110 patients (mean age 61+/-11 years) were included in the study. The mean LVEF was 31+/-6%. All patients had preoperative radionuclide investigations based on the combination of stress/reinjection thallium single photon emission computed tomography (SPECT) and planar evaluation of LVEF; 88% of patients had reversible ischemic thallium defects. Two surgical technique were used: 53 patients received the left internal mammary artery with associated sequential vein graft, and 57 patients received only arterial grafts, internal mammary and gastroepiploic arteries. The mean number of distal anastomoses was 3.2+/-0.8 and 54% of patients had complete revascularization. At 1 year, all survivors had clinical evaluation and the same radionuclide investigations. RESULTS: The early mortality was 2.7%. At 1 year, 100 patients were surviving; on average, NYHA class decreased 1.9+/-0.8 to 1.4+/-0.6 (P < 0.01) and CCS class from 2.8+/-0.6 to 1+/-0.3 (P < 0.01). The mean LVEF increase from 31+/-9 to 34+/-10% (P < 0.01) and the mean LV end-diastolic volume decreased from 317+/-112 to 285+/-108 ml (n.s.). The postoperative improvement in LV function was higher in patients in NYHA class 3 or 4 before surgery (P < 0.05), when associated sequential vein graft had been used (P < 0.01), and in patients with low preoperative LVEF (P < 0.01). The postoperative LVEF improvement observed was significantly correlated with the improvement in left ventricular end-diastolic (LVED) volume and the improvement in redistribution/reinjection thallium uptake. Multivariate analysis showed that the surgical technique used and the preoperative LVEF were independent prognostic factors of the postoperative myocardial function recovery, with a significant positive impact of the vein use. CONCLUSION: This study confirms the excellent clinical results of coronary artery bypass grafting (CABG) in patients with coronary artery disease and LV dysfunction; improvement in LV function can be documented objectively and is correlated with reperfusion of hibernating myocardium. However, the extended use of arterial grafts does not allow to achieve the significant myocardial recovery observed with the use of one internal mammary artery (IMA) and associated sequential vein graft; it seems to be related to the preoperative selection of patients, but a direct negative impact of arterial grafts was documented and leads to be cautious in patients with severe LV dysfunction.     

Subcellular localization of Bcl-2 protein in synovial sarcoma

The subcellular localization of Bcl-2 protein in surgically resected, fixed-frozen tissue specimens of seven tumors from six cases of synovial sarcoma and a synovial sarcoma cell line was examined using laser-scanning confocal microscopy and immunoelectron microscopy. Bcl-2 protein in synovial sarcoma cells was detected in the nuclear envelope, endoplasmic reticulum membrane, and mitochondrial circumference. Based on the finding of pre-embedding immunoelectron-microscopy observation, the distribution of Bcl-2 protein in such membranous organella was patchy. A computer-based image analysis failed to reveal any quantitative differences between the inner and the outer mitochondrial membranes. The tumorigenesis of synovial sarcoma is also discussed from the viewpoint of Bcl-2 overexpression.     

Coexpression of hepatocyte growth factor and c-Met proto-oncogene product in synovial sarcoma

Hepatocyte growth factor (HGF) is a heterodimeric polypeptide growth factor that has pleiotropic roles, including those of mitogen, motogen and morphogen. The HGF receptor is characterized as a c-Met proto-oncogene product (c-Met), which is a heterodimeric tyrosine kinase receptor. Hepatocyte growth factor acts as a mediator between the mesenchymal and epithelial tissues because HGF is produced by mesenchymal cells and c-Met is mainly expressed on various epithelial cells. Furthermore, the HGF/c-Met system plays an important role in embryogenesis and the regeneration of various organs. Synovial sarcoma (SS) are unique sarcoma that show epithelial differentiation, but little is known about their histogenesis. The expression of HGF and c-Met was examined by immunohistochemistry in SS specimens from 12 patients (six each of biphasic and monophasic fibrous types). Immunohistochemical coexpression of HGF and c-Met was demonstrated in the epithelial component of five biphasic SS, while only c-Met was expressed in the epithelioid nests of three monophasic fibrous SS. The spindle cell component was negative for HGF and c-Met. In SS, positivity for epithelial markers, such as cytokeratins and epithelial membrane antigen, was diffusely observed in the epithelial component and was focally observed in spindle cells, while vimentin was positive predominantly in the spindle cell component. The areas expressing HGF and c-Met corresponded to distinct epithelial structures; however, HGF and c-Met expression were not found in any other tumor cells expressing epithelial markers in the spindle cell component of SS. Considering the morphogenic effect of HGF, which has been known to be one of its most important roles, the unique immunohistochemical localization of HGF and c-Met in SS suggests that the HGF/c-Met system may be closely related to the formation of epithelial (glandular) structures in biphasic SS.     

Intraneural cyst of the peroneal nerve

A 15-year-old girl was referred to us because of foot drop. The motor deficit was characterized by a peculiar fluctuating course related to sporting activity: improvement of symptoms during no-sport periods and worsening during sport training. Neurophysiological examination revealed marked global impairment of muscles innervated by the peroneal nerve. Ultrasound and MRI showed a mass in close proximity to the neck of the fibula extending in a tubular fashion. At operation an intraneural lesion was found and was totally removed. Histology revealed that it was an intraneural cyst. A hypothesis on the pathogenesis was drawn up. One year after the operation a clinical and neurophysiological follow-up was performed.     

Intraneural mucous cysts of peripheral nerves

We present three cases of a rare mucous cyst inside the peripheral nerves of the upper extremity; a lateral cutaneous nerve of the forearm, a dorsal sensory branch of the ulnar nerve, and a median nerve at the wrist. All our cases were intraneural, and we excised longitudinal strips of the cyst wall between nerve fascicles in all, preserving nerve function in each case.     

Fine-needle aspiration biopsy of synovial sarcoma. A cytomorphologic analysis of primary, recurrent, and metastatic tumors

Thirteen fine-needle aspiration specimens from 10 patients with histologically proven synovial sarcoma are described. The aspiration biopsy specimens were obtained from the primary tumor in five cases, locally recurrent tumors in four cases, pulmonary metastases in three cases, and mediastinal metastasis in one case. Patient's ages ranged from 22 years to 65 years; there were four women and six men. All cases had a confirmation biopsy and/or resection specimen that were reviewed. Histologic subtypes included monophasic fibrous (5 cases), monophasic epithelial (1 case), biphasic (3 cases), and poorly differentiated (1 case). The majority of the aspiration biopsy specimens were similar with moderate to marked smear cellularity dominated by cohesive clusters of spindle-shaped cells with ovoid, hyperchromatic nuclei and scanty tapering cytoplasm. Nucleoli were not prominent. Epithelial tumor cells with ovoid to round, mostly regular, centrally to eccentrically located nuclei, surrounded by scant to abundant cytoplasm predominated in one case (monophasic epithelial) and were admixed with spindle cells in a second (classical biphasic). Multi-nucleated tumor giant cells were not observed in any of the tumors. In biphasic synovial sarcoma, the neoplastic spindle cells are generally more numerous and frequent than the epithelial cells, making distinction from monophasic synovial sarcoma or other spindle cell soft tissue tumors difficult. Although synovial sarcoma may be diagnosed by fine-needle aspiration cytology, clinical correlation, especially in monophasic types, is necessary to minimize errors in sarcoma classification.     

Poorly differentiated synovial sarcoma: immunohistochemical distinction from primitive neuroectodermal tumors and high-grade malignant peripheral nerve sheath tumors

Synovial sarcoma is a relatively common sarcoma in adults, which in its classic bimorphic form infrequently poses a diagnostic problem. Monomorphic spindled variants, as well as the less common poorly differentiated variants, may be confused with other soft-tissue sarcomas; the poorly differentiated variant (PDSS), in particular, may be histologically indistinguishable from other small, blue, round cell tumors, including primitive neuroectodermal tumors (PNETs). Detection of the synovial sarcoma-associated t(X;18) by either cytogenetic or molecular genetic approaches may be necessary to confirm the diagnosis of synovial sarcoma in difficult cases. We evaluated 10 cases of PDSS from eight patients using a panel of antibodies (including those to intermediate filament proteins, nerve-sheath associated markers, and neuronal and neuroectodermal associated markers) in order to better establish the immunophenotype of this tumor and to help distinguish it from the tumors with which it may be confused, particularly PNETs and high-grade malignant peripheral nerve sheath tumors (MPNSTs). Our results showed PDSS to have significant immunophenotypic overlap with both PNETs and MPNSTs. In most instances these three entities may be differentiated by a panel of antibodies that should include those to both low and high molecular weight cytokeratins, epithelial membrane antigen, type IV collagen, CD99, CD56, and S-100 protein. Our results also suggest that synovial sarcoma may be a tumor showing combined neuroectodermal and nerve sheath differentiation--perhaps because of translocation-associated expression of specific proteins--rather than a carcinosarcoma of soft tissues or a tumor of specialized arthrogenous mesenchyme.     

Intraneural perineurioma. A clonal neoplasm associated with abnormalities of chromosome 22

The nature of perineurioma, variably termed "localized hypertrophic neuropathy," "intraneural neurofibroma," and "hypertrophic interstitial neuritis" has long been an issue of contention. Most authors consider it a neoplasm, but some a reactive process. Eight clinically and morphologically typical perineuriomas were studied by histologic, immunohistochemical and ultrastructural methods. One perineurioma was subject to tissue culture and cytogenetic study and another to fluorescence in situ hybridization (FISH) analysis. The patients, 3 males and 5 females, ranged in age from 11 to 38 years. All tumors were intraneural, and involved extremities (2 sciatic, 1 median, 1 femoral, 1 peroneal, 1 brachial plexus, 1 ulnar, and 1 radial). Neurologic symptoms, motor in all cases and sensory in 4, were present from 1 month to 7 years (mean 1.2 years). Fusiform, segmental nerve enlargement was clinically apparent in only two patients, but was evident on MRI in five of eight patients. Lesion length ranged from 3.5 to 30 cm, the largest involving the sciatic nerve from the obturator foramen to the knee. One lesion involved two nerve roots, but no association with a phakomatosis was noted. Treatment consisted of biopsy in six cases and resection in two cases. Histologically, pseudo-onion bulbs composed of epithelial membrane antigen-reactive, S-100 protein-negative perineurial cells surrounded myelinated or nonmyelinated nerve fibers. Many were accompanied by their S-100 protein-positive Schwann sheaths. Some whorls lacked a central axon. A single mitosis was noted in one case. The MIB-1 antigen labelling index ranged from 4% to 17%. Staining for p53 antigen in six cases showed no (2 of 6), rare (2 of 6), or scattered (2 of 6) immunoreactive nuclei. Cytogenetic analysis in one case demonstrated a chromosomally abnormal clone. Each of 16 metaphases was abnormal; the tumor cells appeared to be homozygously deficient for the region 22q11.2qter. In another case, 53% of interphase nuclei showed three FISH signals with a chromosome 14/22 probe, thus suggesting either monosomy for the centromere of chromosome 14 or that of chromosome 22.(ABSTRACT TRUNCATED AT 400 WORDS)     

Intraneural lidocaine uptake compared with analgesic differences between pregnant and nonpregnant rats

BACKGROUND AND OBJECTIVES: Pregnant patients need less local anesthetic in order to obtain the same quality of functional block as nonpregnant patients. Our goal was to demonstrate a similarly increased functional susceptibility to local anesthetics in the awake pregnant rat during peripheral nerve block and to investigate the pharmacokinetic and/or pharmacodynamic mechanisms responsible for this phenomenon. METHODS: Radiolabeled lidocaine uptake was determined in vivo during sciatic nerve block with 0.1 ml of 1% lidocaine in the nerves of nine pregnant and five nonpregnant female rats and six male rats at the return of deep pain sensation, assessed by withdrawal of the hindlimb from a brief squeeze of a digit with serrated forceps. During recovery from complete functional block, the time at which deep pain returned and the amount of lidocaine in the nerve at that time were compared among the three groups of rats. Lidocaine content was also determined in vitro after exposure of ensheathed sciatic nerves from pregnant and nonpregnant rats to a 0.2% lidocaine bath for specified times. RESULTS: Full block of function developed in all groups within 6 minutes of the lidocaine injection and lasted significantly longer in pregnant rats than in nonpregnant and male rats (49.0 +/- 3.3 vs 34.0 +/- 3.1 and 32.0 +/- 1.3 minutes mean +/- SEMI, respectively. At the time of deep pain return, the intraneural lidocaine content of pregnant rats was significantly lower than that of nonpregnant and male rats (2.2 +/- 0.25 vs 3.9 +/- 0.7 and 3.7 +/- 0.6 nmoles/mg of wet nerve, respectively). No difference in lidocaine uptake kinetics between P and NP nerves was observed in vitro. CONCLUSIONS: Block of peripheral neural function is prolonged in pregnant rats, and lidocaine content in the nerve is lower at a specific stage of neural block. These results are consistent with a pharmacodynamic mechanism for increased susceptibility to lidocaine neural block during pregnancy.     

Synovial sarcoma

Synovial sarcoma is a rare soft tissue tumor of children and adults that is unrelated to synovium and can occur in almost any part of the body. The familiar biphasic synovial sarcoma has discernible glandular or solid epithelial structures, and monophasic forms have characteristic ovoid or spindle cells with only immunohistochemical or ultrastructural evidence of epithelial differentiation. There are several morphologic patterns, including myxoid and hemangiopericytic, and behaviorally distinct calcifying, ossifying, and poorly differentiated subtypes can be recognized. Most synovial sarcomas are immunoreactive for cytokeratin, epithelial membrane antigen, and bc12 protein, and negative for CD34, and many express S100 protein and CD99 (MIC2). Nearly all synovial sarcomas have a specific t(x;18) (p11.2;q11.2) chromosomal abnormality, resulting in fusion of either of two variants of the SSX gene with the SYT gene; the genetic features may relate to morphology and outcome. The differential diagnosis can include a wide range of spindled, polygonal, or round cell sarcomas. Clinically, there have been marked recent improvements in local control of disease and lesser ones in management of metastases. The pathology, differential diagnosis, and behavior of this unique tumor are reviewed.     

AIDS-associated Kaposi's sarcoma

In less than two decades, the disease mechanisms have been elucidated and hypotheses for innovative treatment have been developed. Our understanding of the sarcoma's pathogenesis has led directly to general knowledge of the physiology and pathology of angiogenesis. Hence, it can be expected that new treatments for all cancer patients may someday emerge from clinical intervention trials for the AIDS-related disease.     

Analysis of cartilage oligomeric matrix protein (COMP) in synovial fibroblasts and synovial fluids

We investigated the expression of cartilage oligomeric matrix protein (COMP) in normal and rheumatoid arthritis (RA) synovial fibroblasts. In situ hybridization (ISH) was conducted on synovial specimens from five RA patients applying specific probes for COMP or fibroblast collagen type I. ISH was combined with immunohistochemistry, applying antibodies to the macrophage marker CD68. Ribonuclease protection assay (RPA) and rapid amplification of 3'-cDNA ends (3'-RACE) were performed on total RNA from normal and RA synovial fibroblast cultures. Protein extracts from fibroblasts and culture supernatants were compared with synovial fluids and protein extracts from isolated chondrocytes by Western blot utilizing polyclonal and monoclonal antibodies (18-G3 mAb) to COMP. COMP mRNA was detected in fibroblasts of RA synovium by ISH, and in normal and RA synovial fibroblast cultures by RPA. 3'-RACE demonstrated sequence homology of chondrocyte and synovial fibroblast COMP along the coding sequence. COMP protein was detected in synovial fibroblasts and culture supernatants by immunoblot. Using polyclonal antibodies, the major portion of COMP from fibroblasts and culture supernatants was present as low-molecular-weight (LMW) bands, corresponding to those found in synovial fluids. These LMW COMP bands, however, were not detected in any of the cells or tissues tested using 18-G3 mAb. In protein extracts from chondrocytes and in COMP purified from cartilage, these LMW bands could not be detected. In conclusion, the data suggest that certain forms of COMP detected in synovial fluid are secreted from synovial fibroblasts and could be distinguished by specific mAbs from COMP secreted by chondrocytes.