Foreskin leiomyosarcoma: a case report. / [Leiomiosarcoma de prepucio: reporte de un caso]

Introduction: Primary skin leiomyosarcomas are infrequent neoplasms. They correspond to 2-3% of skin sarcomas and are most frequently located on the lower extremities, trunk and genitals. Methods: We present a case of a 73-year-old man with a 4-month evolution of foreskin leiomyosarcoma. The lesion was biopsied for histopathological study with HE and immunohistochemistry with smooth muscle actin, specific muscle actin, CD34, p63 and S-100 (-). Results: We observed a leiomyosarcoma of high histological grade and mitotic count. It was positive by immunohistochemistry for smooth muscle actin, while the other markers were negative. Surgical limits were compromised so a reoperation with wide margins of healthy tissue was necessary. Conclusion: The skin lesions should be removed all, without exception, since they can be neoplasms of variable biological behavior. The histological study must be complemented with immunohistochemistry to differentiate them from other neoplasms. For the prognosis, the histological grade, size, location and the possibility of resection with wide margins must be taken into account.

Introducción: Los leiomiosarcomas primarios de piel son neoplasias infrecuentes. Corresponden al 2-3 % de los sarcomas cutáneos y se localizan con mayor frecuencia en las extremidades inferiores, tronco y genitales. Método: Presentamos un caso de un varón de 73 años con un leiomiosarcoma en prepucio de 4 meses de evolución. Se le practicó biopsia excisional de la lesión para estudio histopatológico con HE e inmunohistoquímica con actina de músculo liso, actina muscular específica, CD34, p63 y S-100 (-). Resultados: Observamos un leiomiosarcoma de alto grado histológico y recuento mitótico. Presentó positividad por inmunohistoquímica para actina de músculo liso, en tanto que los otros marcadores fueron negativos. Los límites quirúrgicos estuvieron comprometidos por lo que fue necesaria una reintervención con amplios márgenes de tejido sano. Conclusión: Las lesiones de piel deben extirparse todas, sin excepción, ya que pueden tratarse de neoplasias de conducta biológica variable. El estudio histológico debe complementarse con inmunohistoquímica para diferenciarlas de otras neoplasias. Para el pronóstico se debe tener en cuenta el grado histológico, el tamaño, la localización y la posibilidad de resección con amplios márgenes.

[Primary intra-osseous carcinoma]. / Carcinoma primario intraóseo.

Primary intra-osseous carcinoma (PIOC) is a rare tumor, defined as squamous cell carcinoma that develops in the jaw bones, having no initial connection to adjacent skin or mucosa. It is locally aggressive, with metastases to regional lymph nodes, (28% of cases) and lung (5% of cases) at the time of diagnosis. Its origin may be di novo or from other odontogenic tumors. The maxillary bones have epithelial tissues; therefore this neoplasm is located exclusively on this site, predominantly in the jaw. PIOC diagnostic criteria are strict and include: squamous cell carcinoma histopathology, lack of commitment and sinus mucosa, ruling out the possibility of metastasis from a distant site with a thorough clinical study and complementary methods. The treatment is, whenever possible, oncologic resection, additional radio and / or chemotherapy. Reconstructive surgery with graft and / or prostheses for aesthetic and functional are also required. We report the case of a 72 years old man who consulted for sore jaw three months after molar extraction. Curettage biopsy was performed and then resected mandible with lymphadenectomy. Histopathological examination showed a poorly differentiated squamous cell carcinoma, infiltrating jawbone with morphological findings linking him to residual odontogenic cyst and metastatic lymph nodes in 15 of 48 isolates. Postoperative radiotherapy was performed, he died at 30 months of diagnosis by progressive deterioration.

Carcinoma primario intraóseo / Primary intra-osseous carcinoma

El carcinoma primario intraóseo (PIOC) es un tumor poco frecuente, definido como carcinoma escamoso que se desarrolla en huesos maxilares, no teniendo conexión inicial con mucosa ni piel adyacente. Es localmente agresivo, con una incidencia de metástasis en ganglios regionales del 28% y en pulmón del 5%, en el momento del diagnóstico. Su origen puede ser de novo o a partir de otros tumores odontogénicos. Los huesos maxilares son los únicos que tienen en su interior tejidos epiteliales, por lo cual esta neoplasia se localiza exclusivamente en este sitio, predominantemente en la mandíbula. Los criterios diagnósticos del PIOC incluyen: histopatología de carcinoma escamocelular, ausencia de compromiso de mucosa oral y senos paranasales, descartando metástasis de un sitio distante en base a estudios clínicos y métodos complementarios. El tratamiento de elección consiste, siempre que sea posible, en la exéresis con criterios oncológicos, y radio y/o quimioterapia adicional. Se requiere además, cirugía reconstructiva con injerto y/o prótesis con fines estéticos y funcionales. Presentamos el caso de un varón de 72 años, que consultó por molestias en maxilar inferior tres meses después de la extracción de un molar. Se efectuó biopsia por curetaje y luego se resecó el maxilar inferior con vaciamiento ganglionar. El estudio histopatológico mostró un carcinoma escamoso pobremente diferenciado, infiltrante en hueso maxilar, con hallazgos morfológicos que lo vinculaban a quiste odontogénico residual, y metástasis en 15 de 48 ganglios aislados. Se realizó radioterapia postquirúrgica, falleciendo a los 30 meses del diagnóstico por deterioro progresivo.

Primary intra-osseous carcinoma (PIOC) is a rare tumor, defined as squamous cell carcinoma that develops in the jaw bones, having no initial connection to adjacent skin or mucosa. It is locally aggressive, with metastases to regional lymph nodes, (28% of cases) and lung (5% of cases) at the time of diagnosis. Its origin may be di novo or from other odontogenic tumors. The maxillary bones have epithelial tissues; therefore this neoplasm is located exclusively on this site, predominantly in the jaw. PIOC diagnostic criteria are strict and include: squamous cell carcinoma histopathology, lack of commitment and sinus mucosa, ruling out the possibility of metastasis from a distant site with a thorough clinical study and complementary methods. The treatment is, whenever possible, oncologic resection, additional radio and / or chemotherapy. Reconstructive surgery with graft and / or prostheses for aesthetic and functional are also required. We report the case of a 72 years old man who consulted for sore jaw three months after molar extraction. Curettage biopsy was performed and then resected mandible with lymphadenectomy. Histopathological examination showed a poorly differentiated squamous cell carcinoma, infiltrating jawbone with morphological findings linking him to residual odontogenic cyst and metastatic lymph nodes in 15 of 48 isolates. Postoperative radiotherapy was performed, he died at 30 months of diagnosis by progressive deterioration.

Carcinoma primario intraóseo / Primary intra-osseous carcinoma

El carcinoma primario intraóseo (PIOC) es un tumor poco frecuente, definido como carcinoma escamoso que se desarrolla en huesos maxilares, no teniendo conexión inicial con mucosa ni piel adyacente. Es localmente agresivo, con una incidencia de metástasis en ganglios regionales del 28% y en pulmón del 5%, en el momento del diagnóstico. Su origen puede ser de novo o a partir de otros tumores odontogénicos. Los huesos maxilares son los únicos que tienen en su interior tejidos epiteliales, por lo cual esta neoplasia se localiza exclusivamente en este sitio, predominantemente en la mandíbula. Los criterios diagnósticos del PIOC incluyen: histopatología de carcinoma escamocelular, ausencia de compromiso de mucosa oral y senos paranasales, descartando metástasis de un sitio distante en base a estudios clínicos y métodos complementarios. El tratamiento de elección consiste, siempre que sea posible, en la exéresis con criterios oncológicos, y radio y/o quimioterapia adicional. Se requiere además, cirugía reconstructiva con injerto y/o prótesis con fines estéticos y funcionales. Presentamos el caso de un varón de 72 años, que consultó por molestias en maxilar inferior tres meses después de la extracción de un molar. Se efectuó biopsia por curetaje y luego se resecó el maxilar inferior con vaciamiento ganglionar. El estudio histopatológico mostró un carcinoma escamoso pobremente diferenciado, infiltrante en hueso maxilar, con hallazgos morfológicos que lo vinculaban a quiste odontogénico residual, y metástasis en 15 de 48 ganglios aislados. Se realizó radioterapia postquirúrgica, falleciendo a los 30 meses del diagnóstico por deterioro progresivo.(AU)

Primary intra-osseous carcinoma (PIOC) is a rare tumor, defined as squamous cell carcinoma that develops in the jaw bones, having no initial connection to adjacent skin or mucosa. It is locally aggressive, with metastases to regional lymph nodes, (28% of cases) and lung (5% of cases) at the time of diagnosis. Its origin may be di novo or from other odontogenic tumors. The maxillary bones have epithelial tissues; therefore this neoplasm is located exclusively on this site, predominantly in the jaw. PIOC diagnostic criteria are strict and include: squamous cell carcinoma histopathology, lack of commitment and sinus mucosa, ruling out the possibility of metastasis from a distant site with a thorough clinical study and complementary methods. The treatment is, whenever possible, oncologic resection, additional radio and / or chemotherapy. Reconstructive surgery with graft and / or prostheses for aesthetic and functional are also required. We report the case of a 72 years old man who consulted for sore jaw three months after molar extraction. Curettage biopsy was performed and then resected mandible with lymphadenectomy. Histopathological examination showed a poorly differentiated squamous cell carcinoma, infiltrating jawbone with morphological findings linking him to residual odontogenic cyst and metastatic lymph nodes in 15 of 48 isolates. Postoperative radiotherapy was performed, he died at 30 months of diagnosis by progressive deterioration.(AU)

Carcinoma primario intraóseo. / [Primary intra-osseous carcinoma].

Primary intra-osseous carcinoma (PIOC) is a rare tumor, defined as squamous cell carcinoma that develops in the jaw bones, having no initial connection to adjacent skin or mucosa. It is locally aggressive, with metastases to regional lymph nodes, (28

of cases) and lung (5

of cases) at the time of diagnosis. Its origin may be di novo or from other odontogenic tumors. The maxillary bones have epithelial tissues; therefore this neoplasm is located exclusively on this site, predominantly in the jaw. PIOC diagnostic criteria are strict and include: squamous cell carcinoma histopathology, lack of commitment and sinus mucosa, ruling out the possibility of metastasis from a distant site with a thorough clinical study and complementary methods. The treatment is, whenever possible, oncologic resection, additional radio and / or chemotherapy. Reconstructive surgery with graft and / or prostheses for aesthetic and functional are also required. We report the case of a 72 years old man who consulted for sore jaw three months after molar extraction. Curettage biopsy was performed and then resected mandible with lymphadenectomy. Histopathological examination showed a poorly differentiated squamous cell carcinoma, infiltrating jawbone with morphological findings linking him to residual odontogenic cyst and metastatic lymph nodes in 15 of 48 isolates. Postoperative radiotherapy was performed, he died at 30 months of diagnosis by progressive deterioration.

[Undifferentiated high grade pleomorphic sarcoma/ malignant fibrous histiocytoma associated a gouty tophus. a case report]. / Sarcoma pleomórfico indiferenciado de alto grado/ fibrohistiocitoma maligno asociado a tofo gotoso. presentación de un caso.

BACKGROUND: Gout is a metabolic disease by deposition of uric acid crystals, which undertakes joint and soft tissue in both acute and chronic stages. Is a rare event the onset of a tumor in the site of the lesion. OBJECTIVE: To present a rare case of association between sarcoma and tophi. METHODS: 83 year old man who consulted for tumor in his left elbow about 40 years of evolution, which spontaneously started to hurt. With the presumptive diagnosis of tophi treated surgically. The lesion recurred after 60 days, reintervention and radiotherapy was performed for diagnosis of malignant mesenchymal tumor associated with tophi. At 10 months developed ipsilateral nodal metastases, died within 2 years of the initial consultation. RESULTS: The diagnosis of the first material resected was tophi. In the reintervention material was diagnosed mesenchymal spindle cell high grade neoplasm associated with tophi; immunohistochemistry revealed: vimentin + / +, MYO D1 - / - ASMA - / -, FVIII - / -, A1ATT - / -, CD68-/ -, S100-/ - with final diagnosis the undifferentiated high grade pleomorphic sarcoma. CONCLUSIONS: It is uncommon for gouty tophi are associated with other diseases and even fewer do so to tumors. In the literature have reported three previous cases concurrent with neoplasms, which were angiosarcoma, giant cell tumor and malignant fibrous histiocytoma. The latter have a high tendency to recur locally and have ability to distant metastases, especially lung and regional lymph nodes.

Antecedentes: La gota es una enfermedad metabólica por depósito de cristales de ácido úrico, que compromete articulaciones y tejidos blandos tanto en sus etapas agudas como crónicas. Constituye un suceso poco común la aparición de un tumor en el sitio propio de la lesión. Objetivo: presentar un caso de asociación infrecuente entre tofo gotoso y sarcoma. Material y métodos: hombre de 83 años que consultó por tumoración en codo izquierdo de aproximadamente 40 años de evolución, que comenzó a doler espontáneamente. Con la presunción diagnóstica de tofo gotoso se trató quirúrgicamente. La lesión recidivó a los 60 días, se realizó reintervención y radioterapia por diagnóstico de tumor mesenquimal maligno asociado a tofo gotoso. A los 10 meses desarrolló metástasis ganglionar homolateral, falleció antes de los 2 años de la consulta inicial. Resultados: El diagnóstico de la primer biopsia fue tofo gotoso. En el material de reintervención se diagnosticó tofo gotoso asociado a neoplasia mesenquimal fusocelular de alto grado; la inmunohistoquímica reveló: vimentina +/+, MYO D1 -/-, ASMA -/-, FVIII -/-, A1ATT -/-, CD68-/-, S100-/- con resultado diagnóstico final de sarcoma pleomórfico indiferenciado de alto grado. Conclusión: Es infrecuente que los tofos gotosos se asocien a otras enfermedades y menos que lo hagan a tumores. En la bibliografía se han reportado tres casos previos concurrentes con neoplasias, las cuales fueron angiosarcoma, tumor de células gigantes y fibrohistiocitoma maligno. Estos últimos tienen una alta tendencia a recidivar y poseen capacidad de dar metástasis, especialmente a pulmones y ganglios regionales. Palabras clave: tofo gotoso, fibrohistiocitoma maligno, sarcoma pleomórfico indiferenciado.

[Fibrillary glomerulo-nephritis: a rare form of glomerular disease with organized deposits]. / Glomerulonefritis fibrilar: Una rara forma de enfermedad glomerular por depósitos organizados.

We describe the case of a 67 year-old female who presented weakness and fatigue. Laboratory data showed nephrotic level of proteinuria and dyslipidemia. A renal biopsy was performed, and studied by light microscopy, immuno-fluorescence and electron microscopy. Ultra-structural analysis revealed the existence of organized fibrillary deposits, straight and without ramifications, the thickness of which ranged from 15 to 20 nm. These fibres were identified, by light microscopy, as slightly nodular mesangial expansions PAS positive, Congo red negative and weakly positive for IgG. Given the above findings, the diagnosis was fibrillary glomerulonephritis. Glomerular lesions with organized deposits may exhibit syndromic and pathological overlap. For this reason it is important to initially discriminate between positive and negative Congo red deposits, using, in the latter case, transmission electron microscopy to distinguish between immuno-tactoid and fibrillary glomerulonephritis. This differentiation relies not only on ultrastructural features, but on different clinical characteristics. Unlike what happens with fibrillary glomerulonephritis, the immuno-tactoid shows a strong association with lymphoproliferative processes.

Glomerulonefritis fibrilar: Una rara forma de enfermedad glomerular por depósitos organizados / Fibrillary glomerulo-nephritis: A rare form of glomerular disease with organized deposits

Se describe el caso de una mujer de 67 años de edad que consultó por debilidad y astenia, constatándose proteinuria de rango nefrótico y dislipemia. Se realizó punción para biopsia renal, la que se analizó por microscopia óptica, inmunofluorescencia y microscopia electrónica de transmisión. El análisis ultra-estructural reveló la existencia de depósitos fibrilares organizados, rectos, no ramificados, cuyo espesor osciló entre 15 y 20 nm. Dichas fibrillas ópticamente se veían como una expansión mesangial discretamente nodular, ligeramente PAS positiva, rojo Congo negativa y débilmente positiva para IgG. El diagnóstico fue glomerulonefritis fibrilar. Las enfermedades glomerulares por depósitos organizados pueden exhibir superposición sindrómica e histopatológica. Por tal motivo, resulta de importancia una primera separación entre aquellas rojo Congo positivas o negativas, siendo en este último caso la microscopia electrónica de transmisión la que diferencia dos entidades: la glomerulonefritis fibrilar y la glomerulonefritis inmunotactoide. Esta diferencia se apoya no sólo en las características ultraestructurales, sino en sus características clínicas. La glomerulonefritis inmunotactoide muestra una fuerte asociación con procesos linfoproliferativos, a diferencia de lo que ocurre con la glomerulonefritis fibrilar.

We describe the case of a 67 year-old female who presented weakness and fatigue. Laboratory data showed nephrotic level of proteinuria and dyslipidemia. A renal biopsy was performed, and studied by light microscopy, immuno-fluorescence and electron microscopy. Ultra-structural analysis revealed the existence of organized fibrillary deposits, straight and without ramifications, the thickness of which ranged from 15 to 20 nm. These fibres were identified, by light microscopy, as slightly nodular mesangial expansions PAS positive, Congo red negative and weakly positive for IgG. Given the above findings, the diagnosis was fibrillary glomerulonephritis. Glomerular lesions with organized deposits may exhibit syndromic and pathological overlap. For this reason it is important to initially discriminate between positive and negative Congo red deposits, using, in the latter case, transmission electron microscopy to distinguish between immuno-tactoid and fibrillary glomerulonephritis. This differentiation relies not only on ultrastructural features, but on different clinical characteristics. Unlike what happens with fibrillary glomerulonephritis, the immuno-tactoid shows a strong association with lymphoproliferative processes.