A Comparative Ultrastructural Study of Chondrosarcoma, Chordoid Sarcoma, and Chordoma

A morphologic and electron microscopic study was made of two chordoid sarcomas. These lesions were compared with two classical chondrosarcomas and two chordomas. These chondrosarcoma cells showed many features common to chondrocytes, such as abundant RER, well-developed Golgi complexes, and microvillous cytoplasmatic membranes. The chordoid sarcomas bore a close morphologic resemblance to the chordomas but the ultrastructural features revealed a close relationship to the chondrosarcomas. The chordoid sarcoma and chondrosarcoma cells had scalloped cytoplasmatic membranes, variable amounts of glycogen, round or oval nuclei and microfibrils, collagen, and electron-dense granules in the ground substance. The chordoma was characterized by the presence of stellate and physalipherous cells, as well as many transitional cells, with varying nuclear morphology; dilated and irregular RER in contact with mitochondria and morphologically varied vacuoles are the main features in the cytoplasm. This study suggests that chordoid sarcoma represents a variety of the chondrosarcoma rather than a form of chordoma. These findings also support the suggestion of Weiss that chordoid sarcoma is an extraskeletal myxoid chondrosarcom

Decrease of apoptosis rate in patients with renal transplantation treated with mycophenolate mofetil

We conclude that treatment with MMF of kidney transplant patients does not affect the proliferative rate of cells of the allograft, but decreases the number of apoptotic cells in tubular epithelium

Ewing Family Tumors: Potential Prognostic Value of Reverse-Transcriptase Polymerase Chain Reaction Detection of Minimal Residual Disease in Peripheral Blood Samples

In more than 95% of patients, the Ewing family of tumors (ET) has chimeric transcripts caused by fusion of the EWS gene to either FLI1 or ERG. The presence of specific EWS-FLI1 or EWS-ERG transcripts in peripheral blood (PB) samples of patients being treated for ET was prospectively evaluated, and these data were correlated to their clinical status. The authors studied 113 PB samples from 28 patients with ET. Treatment included chemotherapy, radiotherapy, and surgical excision of tumor after induction therapy. PB samples were taken prospectively at least 2 weeks after resection of tumor. Nested reverse-transcriptase polymerase chain reaction (RT-PCR) followed by Southern blot was performed in all samples. Resected tumors were reviewed for the degree of response to chemotherapy and volume. Seventy-seven PB samples from 28 patients had EWS-FLI1/ERG transcripts. In 11 patients, PB samples became negative with treatment, and, in 5 of them, the samples remained negative throughout the study. Samples taken during progression were always positive and, in 4 patients, became positive before progression was clinically evident. All patients with transcripts other than EWS-FLI1 type 1 (n = 3) died from tumor progression. This is a sensitive assay to monitor circulating tumor cells in Ewing tumors. The preliminary data suggest that progression is preceded by positive samples and may be related to specific transcript types

Adenoma de las glándulas de Brunner

El adenoma de glándulas de Brunner representa el 10% de los tumores benignos del duodeno. Su localización más frecuente es en la primera porción del duodeno y es extremadamente raro por debajo de la ampolla de Vater. Presentamos dos casos, uno de ellos asociado a adenocarcinoma de la ampolla de Vater. Esta asociación no está descrita en la literatura. No existe ningún caso en que se demuestre una transformación de adenoma a adenocarcinoma. Para estas lesiones nodulares solitarias es más correcto el término de adenoma que el de hiperplasia, debiéndose reservar este último para las proliferaciones más difusas

Association of EWS-FLI1 Type 1 Fusion with Lower Proliferative Rate in Ewing’s Sarcoma

The Ewing's sarcoma (ES) family of tumors, including peripheral neuroectodermal tumor (PNET), is defined genetically by specific chromosomal translocations resulting in fusion of the EWS gene with a member of the ETS family of transcription factors, either FLI1 (90-95%) or ERG (5-10%). A second level of molecular genetic heterogeneity stems from the variation in the location of the translocation breakpoints, resulting in the inclusion of different combinations of exons from EWS and FLI1 (or ERG) in the fusion products. The most common type of EWS-FLI1 fusion transcript, type 1, is associated with a favorable prognosis and appears to encode a functionally weaker transactivator, compared to other fusion types. We sought to determine whether the observed covariation of structure, function, and clinical course correlates with tumor cell kinetic parameters such as proliferative rate and apoptosis, and with expression of the receptor for insulin-like growth factor I (IGF-1R). In a group of 86 ES/PNET with defined EWS-ETS fusions (45 EWS-FLI1 type 1, 27 EWS-FLI1 non-type 1, 14 EWS-ERG), we assessed proliferation rate by immunostaining for Ki-67 using MIB1 antibody (n = 85), apoptosis by TUNEL assay (n = 66), and IGF-1R expression by immunostaining with antibody 1H7 (n = 78). Ki-67 proliferative index was lower in tumors with EWS-FLI1 type 1 than those with non-type 1 EWS-FLI1, whether analyzed as a continuous (P = 0.049) or categorical (P = 0.047) variable. Logistic regression analysis suggests that this association was secondary to the association of type 1 EWS-FLI1 and lower IGF-1R expression (P = 0.04). Comparing EWS-FLI1 to EWS-ERG cases, Ki-67 proliferative index was higher in the latter (P = 0.01, Mann-Whitney test; P = 0.02, Fisher's exact test), but there was no significant difference in IGF-1R. TUNEL results showed no significant differences between groups. Our results suggest that clinical and functional differences between alternative forms of EWS-FLI1 are paralleled by differences in proliferative rate, possibly mediated by differential regulation of the IGF-1R pathway

Sarcoma cordoide de fémur. Estudio a microscopio óptico y electrónico de un caso

Areas of chordoma and chondrosarcoma have been reported extensively in the same tumoral mass located in espheno-palatine region. The same association in long bones of the extremities have been reported recently, with the name of "chordoide sarcoma", "parachordoma" or "chondroid chordoma". We present a case of "chordoid sarcoma". The cells of this tumor have morphologic features of chordoma and chondrosarcoma in both the optical and ultrastructural study. However some morphological, radiological and clinical aspects, suggest that this tumor possesses characteristics that define it as a separate entity

Metástasis ganglionares de osteosarcomas

En este artículo presentamos dos pa cientes con osteosarcoma osteoblástico de tercio distal de fémur que cursaron con afectación ganglionar loco - regional. En el primer caso, el paciente presentó dos metástasis ganglionares en re gión inguinal y pél vica dos años después del diagnóstico del tumor pri mario. Actualmente tres meses después de la linfadenectomía se encuentra libre de enfermedad. En el segundo caso, durante el estudio de extensión del tu mor primario, se observaron imá genes nodulares de alta densidad en zona inguinal derecha, que corres pondieron a metástasis del tumor primari

Carcinomas renales con rasgos sarcomatoides y rabdoides: estudio clínico-patológico de 74 casos

Fundamento. Nuestro objetivo fue comparar las variables clínico-patológicas de los carcinomas renales (CCR) con fenotipos sarcomatoide y rabdoide. Material y métodos. Se revisaron 1.258 CCR de pacientes consecutivos nefrectomizados entre 1988 y 2015, y se seleccionaron aquellos con ≥1% de cambio sarcomatoide y/o rabdoide. Se clasificaron como sarcomatoide o rabdoide según el fenotipo predominante, considerándose componente desdiferenciado la suma del porcentaje de ambos. Se recopilaron: sexo y edad de los pacientes, síntomas y existencia de metástasis al diagnóstico, parámetros del protocolo de CCR del Colegio Americano de Patólogos, patrón de crecimiento tumoral, invasión perineural, porcentaje de necrosis tumoral y características del infiltrado inflamatorio. Se describieron mediante la media/mediana o el porcentaje y se compararon mediante t de Student/U de MannWhitney o χ2 /F de Fisher. Resultados. Se identificaron 45 CCR con predominio sarcomatoide (3,6%) y 29 con rabdoide (2,3%); los primeros mostraron mayor componente indiferenciado e invasión perineural respecto a los CCR con rasgos rabdoides (27,5 vs. 13,5%; p=0,003 y 28,9 vs. 3,4%, p=0,006, respectivamente), mientras que estos mostraron doble frecuencia de inflamación neutrofílica (44,8 vs. 22,2%, p=0,04) y surgieron más frecuentemente sobre un CCR de alto grado (55,9 vs. 90,5%, p<0,001). Conclusiones. Los CCR con fenotipos sarcomatoide y rabdoide compartieron características clínico-patológicas, excepto para componente desdiferenciado, invasión perineural, inflamación neutrofílica y origen en CCR de alto grado. Esta similitud sugiere la presencia de un mecanismo común, la transición epitelio-mesénquima, con una expresión morfológica doble que, de confirmarse, podría suponer la posibilidad de seleccionar pacientes para tratamiento o seguimiento a partir de sus características moleculares

Molecular features in a biphenotypic small cell sarcoma with neuroectodermal and muscle differentiation

We report a case of a 13-year-old girl with soft tissue sarcoma of the hand, which showed muscle and neuroectodermal immunophenotypes. Molecular studies were performed on RNA collected from fine-needle aspiration (FNA) cytology and peripheral blood samples by nested reverse transcriptase-polymerase chain reaction (RT-PCR) and Southern blot analysis. This biphenotypic tumor showed simultaneous expression of EWS-FLI1 and PAX3-FKHR transcripts, specific of Ewing family tumors and alveolar rhabdomyosarcoma, respectively. Although childhood sarcomas with simultaneous muscle and neural differentiation have been described to have EWS-FLI1 transcripts, there are no reports of tumors with both transcripts. Cytological specimens are a good source of RNA for molecular studie

Plasma levels of leukotriene B4 during hepatic allograft rejection

At the present time, rejection is the most frequent cause of graft dysfunction in liver transplantation. Differential diagnosis between this and other possible causes of dysfunction—preservation injury, vascular, biliary, viral—may well be difficult, as the clinical and analytical findings are often similar; moreover, no markers specific to rejection are available, and histological studies are necessary for a definitive diagnosis. For this reason, markers indicating activity within the immune system need to be established so as to provide a more specific means of distinguishing rejection from other causes of graft dysfunction. The immune response to an allograft is complex, and the intricate mechanisms regulating it are still not entirely understood. Nevertheless, several specialists have drawn connections among changes in the lymphocyte subpopulations, rises in the interleukin-2 levels, expression of the interleukin-2 receptor, and alteration in the expression of antigens belonging to class II in the greater complex of histocompatibility, with rejection of the allograft. Leukotriene B4 (LTB4) is a derivative of the metabolism of arachidonic acid via 5- lipoxygenase, whose in vitro behaviour is to encourage rejection by favoring leukocyte aggregation, proliferation of T lymphocytes, interleukin-1 and -2 secretion, and the development of "natural killer" cell subpopulations. This study examines the role of LTB4 in mediating the immune response to the hepatic allograft in order to assess its usefulness in early diagnosis of rejection