[Gastrointestinal stromal tumor and renal transplant]. / Tumor del estroma gastrointestinal y trasplante renal.

Gastrointestinal stromal tumor (GIST) accounts for nearly 1% of all gastrointestinal tumors. Its association with renal transplantation is not frequent. Approximately 95% of GIST show staining for CD177. DOG1 is a recently described monoclonal antibody that shows positivity even in the absence of CD177 staining. The diagnosis of GIST should be pursued because of the availability of very effective treatments with tyrosine-kinase inhibitors. Herein, we describe the case of a woman with renal transplant who presented a small bowel GIST and weak positivity for CD177, treated initially with surgery. Tumor recurrence was documented 3 years later and histopatology showed loss of CD177 staining and positivity for DOG1. She was treated with imatimib without further recurrence after five years of follow up.

Tumor del estroma gastrointestinal y trasplante renal / Gastrointestinal stromal tumor and renal transplant

El tumor estromal gastrointestinal (GIST) representa alrededor del 1% de todos los tumores digestivos y su aparición en pacientes trasplantados renales es infrecuente. Aproximadamente el 95% muestra tinción positiva para c-kit/CD117. DOG1 es un anticuerpo recientemente descrito que se sobre-expresa en los GIST, incluso en c-kit/ CD117 negativos. El diagnóstico preciso de GIST resulta imperativo, debido a la disponibilidad y la creciente eficacia de los inhibidores de la tirosina quinasa en estos tumores, incluso en el subgrupo c-kit/ CD117 negativo. Se presenta el caso de una mujer trasplantada renal inicialmente con GIST en intestino delgado y débil positividad para CD117 tratada con cirugía y recidiva tumoral a los tres años, pérdida de la expresión CD117 y tinción positiva para DOG1. Recibió tratamiento exitoso con imatimib sin presentar recaída tumoral durante un seguimiento de cinco años.

Gastrointestinal stromal tumor (GIST) accounts for nearly 1% of all gastrointestinal tumors. Its association with renal transplantation is not frequent. Approximately 95% of GIST show staining for CD177. DOG1 is a recently described monoclonal antibody that shows positivity even in the absence of CD177 staining. The diagnosis of GIST should be pursued because of the availability of very effective treatments with tyrosine-kinase inhibitors. Herein, we describe the case of a woman with renal transplant who presented a small bowel GIST and weak positivity for CD177, treated initially with surgery. Tumor recurrence was documented 3 years later and histopatology showed loss of CD177 staining and positivity for DOG1. She was treated with imatimib without further recurrence after five years of follow up.

Lack of TNFRI signaling enhances annexin A1 biological activity in intestinal inflammation.

We evaluated whether the lack of TNF-α signaling increases mucosal levels of annexin A1 (AnxA1); the hypothesis stems from previous findings showing that TNF-α neutralization in Crohn's disease patients up-regulates systemic AnxA1 expression. Biopsies from healthy volunteers and patients under anti-TNF-α therapy with remittent ulcerative colitis (UC) showed higher AnxA1 expression than those with active disease. We also evaluated dextran sulfate sodium (DSS)-acute colitis in TNF-α receptor 1 KO (TNFR1-/-) strain with impaired TNF-α signaling and C57BL/6 (WT) mice. Although both strains developed colitis, TNFR1-/- mice showed early clinical recovery, lower myeloperoxidase (MPO) activity and milder histopathological alterations. Colonic epithelium from control and DSS-treated TNFR1-/- mice showed intense AnxA1 expression and AnxA1+ CD4+ and CD8+ T cells were more frequent in TNFR1-/- animals, suggesting an extra supply of AnxA1. The pan antagonist of AnxA1 receptors exacerbated the colitis outcome in TNFR1-/- mice, supporting the pivotal role of AnxA1 in the early recovery. Our findings demonstrate that the TNF-α signaling reduction favors the expression and biological activity of AnxA1 in inflamed intestinal mucosa.

Expression of VEGF-A, HIF-1 A, CD34 and Ki67 in clear cell renal cell carcinomas and their relationship with conventional prognostic markers.

Clear cell renal carcinoma is the most frequent type of renal carcinoma. Recently, attention has been focused in the expression of angiogenic factors by these tumors, which would justify in part their capacity to grow, invade and disseminate, stating a worse evolution of those patients with an unfavorable angiogenic profile. 83 samples of nephrectomy with a diagnosis of clear cell renal cell carcinoma were studied. Clinical and pathological data were collected. Tumors were studied to assess immunohistochemical expression of the following markers: VEGF-A, HIF-1α, CD34 and Ki67. Results indicated a direct linear relationship between expressions of these four markers. Besides, the expression of HIF-1α was directly related to Furhman grade, invasion of the renal vein and tumor stage. Likewise, tumor proliferation index, assessed with Ki67, was directly related to the presence of necrosis, capsular invasion and advanced tumor stage. Regarding the expression of CD34, vascular density was inversely related to tumor necrosis and overall survival. These findings are controversial compared with the available literature. Then, a research scenery would be open, where the importance of generating prospective and more standardized studies are highlighted to determine the role of these angiogenic factors in tumor evolution and prognostic evaluation of these tumors.

El carcinoma renal de células claras es la variante más frecuente de carcinoma renal. En los últimos años, la atención se ha enfocado en la expresión de factores angiogénicos por estos tumores, lo que justificaría en parte su capacidad de crecer, invadir y diseminarse, determinando una peor evolución de aquellos pacientes con un perfil angiogénico desfavorable. Se estudiaron 83 piezas de nefrectomía con diagnóstico de carcinoma renal de células claras. Se recolectaron datos clínicos y patológicos. Los tumores fueron estudiados para evaluar la expresión inmunohistoquímica de los siguientes marcadores: VEGF-A, HIF-1α, CD34 y Ki67. Los resultados indicaron una relación lineal directa entre la expresión de estos cuatro marcadores. Además, la expresión de HIF-1α se encontraba directamente relacionada con el grado de Furhman, la invasión de la vena renal y el estadio tumoral. Asimismo, el índice de proliferación tumoral, evaluado con Ki67, se hallaba directamente relacionado con la presencia de necrosis, la invasión capsular y el estadio tumoral avanzado. Con respecto a la expresión de CD34, mientras mayor es la densidad vascular, menor es la necrosis tumoral y menor la sobrevida global. Los hallazgos resultan controvertidos en comparación con la literatura disponible. Se abriría, entonces, un escenario de investigación donde se destaca la importancia de generar estudios prospectivos y más estandarizados para determinar el rol que cumplen estos factores angiogénicos en la evolución tumoral y la posibilidad de estandarizar resultados que permitan un mejor estudio diagnóstico y pronóstico de estos tumores.

Expression of VEGF-A, HIF-1 A, CD34 and Ki67 in clear cell renal cell carcinomas and their relationship with conventional prognostic markers. / Expression of VEGF-A, HIF-1 A, CD34 and Ki67 in clear cell renal cell carcinomas and their relationship with conventional prognostic markers.

Clear cell renal carcinoma is the most frequent type of renal carcinoma. Recently, attention has been focused in the expression of angiogenic factors by these tumors, which would justify in part their capacity to grow, invade and disseminate, stating a worse evolution of those patients with an unfavorable angiogenic profile. 83 samples of nephrectomy with a diagnosis of clear cell renal cell carcinoma were studied. Clinical and pathological data were collected. Tumors were studied to assess immunohistochemical expression of the following markers: VEGF-A, HIF-1α, CD34 and Ki67. Results indicated a direct linear relationship between expressions of these four markers. Besides, the expression of HIF-1α was directly related to Furhman grade, invasion of the renal vein and tumor stage. Likewise, tumor proliferation index, assessed with Ki67, was directly related to the presence of necrosis, capsular invasion and advanced tumor stage. Regarding the expression of CD34, vascular density was inversely related to tumor necrosis and overall survival. These findings are controversial compared with the available literature. Then, a research scenery would be open, where the importance of generating prospective and more standardized studies are highlighted to determine the role of these angiogenic factors in tumor evolution and prognostic evaluation of these tumors.

Expression of VEGF-A, HIF-1 A, CD34 and Ki67 in clear cell renal cell carcinomas and their relationship with conventional prognostic markers / Expression of VEGF-A, HIF-1 A, CD34 and Ki67 in clear cell renal cell carcinomas and their relationship with conventional prognostic markers.

Clear cell renal carcinoma is the most frequent type of renal carcinoma. Recently, attention has been focused in the expression of angiogenic factors by these tumors, which would justify in part their capacity to grow, invade and disseminate, stating a worse evolution of those patients with an unfavorable angiogenic profile. 83 samples of nephrectomy with a diagnosis of clear cell renal cell carcinoma were studied. Clinical and pathological data were collected. Tumors were studied to assess immunohistochemical expression of the following markers: VEGF-A, HIF-1α, CD34 and Ki67. Results indicated a direct linear relationship between expressions of these four markers. Besides, the expression of HIF-1α was directly related to Furhman grade, invasion of the renal vein and tumor stage. Likewise, tumor proliferation index, assessed with Ki67, was directly related to the presence of necrosis, capsular invasion and advanced tumor stage. Regarding the expression of CD34, vascular density was inversely related to tumor necrosis and overall survival. These findings are controversial compared with the available literature. Then, a research scenery would be open, where the importance of generating prospective and more standardized studies are highlighted to determine the role of these angiogenic factors in tumor evolution and prognostic evaluation of these tumors.

Clinicopathological features of aggressive B-cell lymphomas including B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell and Burkitt lymphomas: a study of 44 patients from Argentina.

Aggressive B-cell lymphomas incorporate a wide spectrum of lymphomas that pose challenges in diagnosis as well as treatment. We evaluated the clinicopathological features of 44 patients with aggressive B-cell lymphomas which were classified into 3 groups based on the World Health Organization 2008 classification as follows: including 30 cases of diffuse large B-cell lymphoma (DLBCL), 8 cases of Burkitt lymphoma (BL) and 6 cases of B-cell lymphoma, unclassifiable, with features intermediate between Burkitt lymphoma and diffuse large B-cell lymphoma (BCLU). Male predominance was observed in BL and BCLU groups and the mean age varied from 29 years in BL, 61 years in DLBCL and 70 years in BCLU. Patients with BCLU presented at more advanced stages and had a higher international prognostic index. By immunohistochemistry, they shared characteristics of both BL (including more frequent expression of SOX11) and DLBCL. FISH analyses showed three cases with more than one rearrangement: one MYC/BCL2 and two BCL2/BCL6, in addition to which one case with BCL2/IGH translocation and another with MYC rearrangement were also detected. The mean follow-up survival time of BCLU was 6.6 months, which was significantly shorter in comparison to DLBCL (31 months) and BL (30 months), respectively. The importance of recognizing this BCLU group relies on its different clinical course, poor prognosis and shorter survival than DLBCL and BL. An accurate diagnosis is critical for risk stratification and to improve therapeutic approaches and outcomes.

[Primary hydatid disease of the tibia. case report]. / Hidiatidosis osea primaria de tibia: presentación de un caso.

Hydatidosis is an endemic disease in different parts of the world. Its causal agent is the cestode from the genus Echinococcus. The most commonly affected organs in humans are liver and lung. Bone hydatid disease is a very rare entity, accounting for 0.5 to 4% of total cases. We report a case of a 58 year-old woman from La Rioja, Argentina, who consulted for left infapatellar pain and walking disability of eight months duration. Imaging studies showed a cystic lesion which involved metaphysis and diaphysis of left proximal tibia. Surgical resection was performed and histopathological study confirmed that it was a hydatid cyst. The patient did well and completed three cycles of treatment with albendazole. Currently, she has no evidence of disease and she recovered motility of her left leg. Primary hydatid bone disease, where there is no evidence of systemic disease, is even more unusual. Tibia involvement occurs in up to 15% of the cases. These lesions clinically manifest when they suffer any type of complications. Preoperative diagnosis is mainly made by imaging studies. Lesions are usually osteolytic and can involve cortical bone and extend to soft tissues. Differential diagnosis with inflammatory processes and bone tumors should is mandatory. Treatment is surgical and prognosis is poor due to its high morbi-mortality rate and recurrence risk from 70 to 80%.

[Unusual presentation of plasma cell myeloma]. / Morfología dual de células de mieloma: inusual presentación de un caso.

A 41 year-old woman consulted because of facial pain. A magnetic resonance imaging showed a mass in the right petrous apex. A biopsy revealed a diffuse proliferation of large atypical cells with plasmablastic appearance, positive for CD138, BCL6, CD56 and p53. The proliferation factor was 80%. Monoclonal kappa light chain expression was observed. Because the unusual clinicopathological features the patient was studied to rule out systemic plasma cell myeloma. Bone scan disclosed multiple cranium osteolytic lesions; proteinogram showed hypogammaglobulinemia and immunofixation in serum and urine were negative. Afterwards, bone marrow biopsy was performed and it presented a 30% infiltration of the bone cylinder by mature plasma cells. These were monoclonal for kappa light chain with focal expression of p53 and without expression of CD56. These findings suggested the diagnosis of multiple myeloma. This case proposes a morphological spectrum of plasma cell neoplasms, showing a continuous clonal evolution of tumor cells, with an acquired plasticity of dedifferentiate, become immature and infiltrate extramedullary tissues, a fact possibly determined by accumulation of multiple genetic alterations. These findings confirm the difficulty of the differential diagnosis from histopathology study between plasmablastic lymphoma and plasmablastic transformation of plasma cell myeloma because of the nearly identical immunohistochemical profiles.

Morfología dual de células de mieloma: inusual presentación de un caso / Unusual presentation of plasma cell myeloma

Una mujer de 41 años consultó por dolor facial. En una resonancia magnética nuclear se observó una masa en el ápex del peñasco derecho. La biopsia mostró una infiltración difusa por células grandes atípicas con morfología plasmablástica, positivas para CD138, BCL6, CD56 y p53, con expresión monoclonal de cadena liviana kappa y factor de proliferación del 80%, planteando el diagnóstico diferencial entre linfoma plasmablástico versus plasmocitoma plasmablástico. Un mapeo óseo evidenció múltiples lesiones osteolíticas en cráneo; el proteinograma reveló hipogamaglobulinemia y la inmunofijación en suero y orina fueron negativas. Se realizó biopsia de médula ósea donde se observó infiltración en un 30% del cilindro óseo por células plasmáticas maduras monoclonales para kappa, con expresión focal de p53 y negativas para CD56. Estos hallazgos confirmaron el diagnóstico de mieloma múltiple. Este caso pone de manifiesto la existencia de un espectro morfológico de las neoplasias de células plasmáticas, mostrando una evolución clonal continua con una plasticidad adquirida para desdiferenciarse, volverse inmaduras e infiltrar tejidos extramedulares, posiblemente debido a acumulación de alteraciones moleculares. Por lo tanto, se evidencia la dificultad del diagnóstico diferencial histopatológico entre linfoma plasmablástico y transformación plasmablástica de mieloma múltiple, debido a sus perfiles inmunohistoquímicos casi idénticos.(AU)

A 41 year-old woman consulted because of facial pain. A magnetic resonance imaging showed a mass in the right petrous apex. A biopsy revealed a diffuse proliferation of large atypical cells with plasmablastic appearance, positive for CD138, BCL6, CD56 and p53. The proliferation factor was 80%. Monoclonal kappa light chain expression was observed. Because the unusual clinicopathological features the patient was studied to rule out systemic plasma cell myeloma. Bone scan disclosed multiple cranium osteolytic lesions; proteinogram showed hypogammaglobulinemia and immunofixation in serum and urine were negative. Afterwards, bone marrow biopsy was performed and it presented a 30% infiltration of the bone cylinder by mature plasma cells. These were monoclonal for kappa light chain with focal expression of p53 and without expression of CD56. These findings suggested the diagnosis of multiple myeloma. This case proposes a morphological spectrum of plasma cell neoplasms, showing a continuous clonal evolution of tumor cells, with an acquired plasticity of dedifferentiate, become immature and infiltrate extramedullary tissues, a fact possibly determined by accumulation of multiple genetic alterations. These findings confirm the difficulty of the differential diagnosis from histopathology study between plasmablastic lymphoma and plasmablastic transformation of plasma cell myeloma because of the nearly identical immunohistochemical profiles.(AU)

Morfología dual de células de mieloma: inusual presentación de un caso / Unusual presentation of plasma cell myeloma

Una mujer de 41 años consultó por dolor facial. En una resonancia magnética nuclear se observó una masa en el ápex del peñasco derecho. La biopsia mostró una infiltración difusa por células grandes atípicas con morfología plasmablástica, positivas para CD138, BCL6, CD56 y p53, con expresión monoclonal de cadena liviana kappa y factor de proliferación del 80%, planteando el diagnóstico diferencial entre linfoma plasmablástico versus plasmocitoma plasmablástico. Un mapeo óseo evidenció múltiples lesiones osteolíticas en cráneo; el proteinograma reveló hipogamaglobulinemia y la inmunofijación en suero y orina fueron negativas. Se realizó biopsia de médula ósea donde se observó infiltración en un 30% del cilindro óseo por células plasmáticas maduras monoclonales para kappa, con expresión focal de p53 y negativas para CD56. Estos hallazgos confirmaron el diagnóstico de mieloma múltiple. Este caso pone de manifiesto la existencia de un espectro morfológico de las neoplasias de células plasmáticas, mostrando una evolución clonal continua con una plasticidad adquirida para desdiferenciarse, volverse inmaduras e infiltrar tejidos extramedulares, posiblemente debido a acumulación de alteraciones moleculares. Por lo tanto, se evidencia la dificultad del diagnóstico diferencial histopatológico entre linfoma plasmablástico y transformación plasmablástica de mieloma múltiple, debido a sus perfiles inmunohistoquímicos casi idénticos.

A 41 year-old woman consulted because of facial pain. A magnetic resonance imaging showed a mass in the right petrous apex. A biopsy revealed a diffuse proliferation of large atypical cells with plasmablastic appearance, positive for CD138, BCL6, CD56 and p53. The proliferation factor was 80%. Monoclonal kappa light chain expression was observed. Because the unusual clinicopathological features the patient was studied to rule out systemic plasma cell myeloma. Bone scan disclosed multiple cranium osteolytic lesions; proteinogram showed hypogammaglobulinemia and immunofixation in serum and urine were negative. Afterwards, bone marrow biopsy was performed and it presented a 30% infiltration of the bone cylinder by mature plasma cells. These were monoclonal for kappa light chain with focal expression of p53 and without expression of CD56. These findings suggested the diagnosis of multiple myeloma. This case proposes a morphological spectrum of plasma cell neoplasms, showing a continuous clonal evolution of tumor cells, with an acquired plasticity of dedifferentiate, become immature and infiltrate extramedullary tissues, a fact possibly determined by accumulation of multiple genetic alterations. These findings confirm the difficulty of the differential diagnosis from histopathology study between plasmablastic lymphoma and plasmablastic transformation of plasma cell myeloma because of the nearly identical immunohistochemical profiles.

A renal variant of Fabry disease: A case with a novel Gal A hemizygote mutation.

BACKGROUND: Fabry disease is caused by an X-linked recessive inborn error of glycosphingolipid metabolism with deficient activity of a lysosomal enzyme, alpha-galactosidase A (α-GalA). CASE PRESENTATION: A 46 year-old man with progressive kidney disease showed on kidney biopsy electron microscopic evidence of Fabry disease. The patient had no systemic manifestations of Fabry disease, despite residual α-GalA activity, therefore genetic testing was done by direct DNA sequencing, demonstrating a new GAL A gene mutation (C174G-exon 3). After three years of enzyme replacement therapy (agalsidase beta) treatment, a second biopsy was done. Although there was demonstrable clearance of intracellular inclusions, remarkable podocyte activation was evident. CONCLUSIONS: This report represents an unusual renal variant of Fabry disease and provides histologic data on long-term follow up after enzyme replacement therapy.

[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.

[Metastatic cardiac tamponade as initial manifestation of papillary thyroid carcinoma]. / Taponamiento cardíaco secundario a carcinoma papilar esclerosante difuso de tiroides.

Papillary carcinoma, diffuse sclerosing variant corresponds to 2% of all papillary thyroid carcinomas. It is usually diffuse and bilateral, affecting the entire gland. At the time of diagnosis, patients present lymph node and lung metastasis. It affects mainly young women. This case report describes a cardiac tamponade as the initial manifestation of an unusual variant of papillary thyroid carcinoma. A 32 year-old woman was attended at the emergency room with epigastric pain and dry cough. Physical examination revealed hypotension, tachycardia and decreased heart sounds. An echocardiogram confirmed severe pericardial effusion. Pericardial fluid cytology was positive for malignancy. The patient evolved with recurrent pericardial effusion and a pleuropericardial window was performed. At this procedure, a subpleural nodular lesion was found, which histology corresponded to metastases of papillary carcinoma, probably from thyroid origin. Total thyroidectomy was performed. The final diagnosis was papillary carcinoma, diffuse sclerosing variant. This variant infiltrates the connective tissue of the interfollicular spaces, mimicking thyroiditis and it is associated with early vascular permeation. This tumor, compared to the classic variants of thyroid carcinoma, is more aggressive and it has higher risk of recurrence. Papillary thyroid carcinoma should be considered as differential diagnosis in our population, in all metastatic papillary lesions, and even more in young female patients.

Taponamiento cardíaco secundario a carcinoma papilar esclerosante difuso de tiroides / Metastatic cardiac tamponade as initial manifestation of papillary thyroid carcinoma

El carcinoma papilar, variante esclerosante difusa, corresponde al 2% de todos los carcinomas papilares de la tiroides. Se caracteriza por comprometer de manera difusa y bilateral a la glándula tiroides. Clínicamente se manifiesta con metástasis ganglionares y pulmonares, afectando predominantemente a mujeres jóvenes. Se describe un caso de taponamiento cardíaco como presentación inicial de un carcinoma papilar de tiroides variante esclerosante difusa. Una mujer de 32 años concurrió al servicio de emergencias médicas refiriendo epigastralgia y tos seca. Durante el examen físico se constató hipotensión arterial, taquicardia y ruidos cardíacos disminuidos. Se realizó un ecocardiograma, observándose derrame pericárdico. Por medio de una pericardiocentesis se obtuvo líquido pericárdico, cuyo análisis mostró células neoplásicas. Durante la evolución la paciente presentó recurrencia del derrame pericárdico por lo que se realizó una ventana pleuropericárdica, detectándose durante la cirugía una lesión nodular subpleural, la cual fue biopsiada e informada posteriormente como una metástasis de carcinoma papilar vinculable a origen tiroideo. Se realizó una tiroidectomía total con linfadenectomía cervical bilateral. El diagnóstico final fue carcinoma papilar, variante esclerosante difusa. Esta variante infiltra el tejido conectivo de los espacios interfoliculares, simulando una tiroiditis y se caracteriza por una permeación vascular temprana. En oposición a la variante clásica, la esclerosante difusa presenta mayor agresividad y mayor tasa de recurrencia. El carcinoma papilar de tiroides debe tenerse presente como diagnóstico diferencial en nuestro medio, en todas aquellas lesiones neoplásicas papilares metastásicas, más aún si se trata de mujeres jóvenes.

Papillary carcinoma, diffuse sclerosing variant corresponds to 2% of all papillary thyroid carcinomas. It is usually diffuse and bilateral, affecting the entire gland. At the time of diagnosis, patients present lymph node and lung metastasis. It affects mainly young women. This case report describes a cardiac tamponade as the initial manifestation of an unusual variant of papillary thyroid carcinoma. A 32 year-old woman was attended at the emergency room with epigastric pain and dry cough. Physical examination revealed hypotension, tachycardia and decreased heart sounds. An echocardiogram confirmed severe pericardial effusion. Pericardial fluid cytology was positive for malignancy. The patient evolved with recurrent pericardial effusion and a pleuropericardial window was performed. At this procedure, a subpleural nodular lesion was found, which histology corresponded to metastases of papillary carcinoma, probably from thyroid origin. Total thyroidectomy was performed. The final diagnosis was papillary carcinoma, diffuse sclerosing variant. This variant infiltrates the connective tissue of the interfollicular spaces, mimicking thyroiditis and it is associated with early vascular permeation. This tumor, compared to the classic variants of thyroid carcinoma, is more aggressive and it has higher risk of recurrence. Papillary thyroid carcinoma should be considered as differential diagnosis in our population, in all metastatic papillary lesions, and even more in young female patients.

[26 year-old male patient with bone marrow transplantation. Report of a partial autopsy]. / Paciente de 26 años trasplantado de médula ósea. Reporte de autopsia parcial.

26 year-old male patient with diagnosis of acute lymphoblastic leukemia in 2006, who underwent chemotherapy and suffered a relapse and pulmonary aspergillosis as a complication. In 2009, he received bone marrow transplant. After it, he developed cutaneous and intestinal graft versus host disease (GVH). He was admitted for diarrhea. Then he presented grade IV dyspnea, patchy alveolar infiltrates on chest computed tomography and pancytopenia with impaired renal function as laboratory findings. He entered Intensive Care Unit, dying 7 days later. The oncologist who discussed the case defined this patient as a high risk case because of type of transplant received, relapse and complications. His diagnostic hypotheses were: CMV infection, pulmonary aspergillosis reactivation, chronic GVH, Pneumocystis jiroveci infection, mycobacteriosis and pseudomembranous colitis. Partial autopsy revealed diffuse intra-alveolar hemorrhage, diffuse alveolar damage, right pulmonary infarction with microthrombosis and bronchiolitis obliterans organizing pneumonia.

Relación de la biopsia temporal en el diagnóstico de arteritis de células gigantes y sus manifestaciones clínicas / Relation of the temporal artery biopsy in the diagnosis of gigant cell arteritis and its clinic manifestations

Objetivo: determinar el papel de la biopsia de arteria temporal en el diagnóstico de arteritis temporal así como evaluar la efectividad de los criterios del ACR en el diagnóstico de esta entidad. Materiales y métodos: se realizó un estudio retrospectivo observacional descriptivo. Se revisaron informes de 40 biopsias de arteria temporal recibidas en el servicio de patología del Hospital Privado de Córdoba, Argentina entre 2000 y 2008. El total de biopsias se correlacionó con los hallazgos clínicos y de laboratorio. Resultados: 43% de las biopsias resultaron positivas para arteritis de células gigantes, mientras que 57% restante no cumplió los criterios histológicos. Al aplicar los criterios del Colegio Americano de Reumatología, 65% de los pacientes cumplió los criterios necesarios. De estos 26 individuos, 61% presentó biopsias positivas. De los pacientes que no reunían los criterios americanos, solo uno presentó positividad en la biopsia. Al tomar dichos criterios como parámetro de diagnóstico de la enfermedad y compararlos con la biopsia, cuentan con una sensibilidad de 94% y una especificidad de 56%, un valor predictivo positivo de 61% y un valor predictivo negativo de 93%. Los principales predictores de positividad en la biopsia fueron la claudicación mandibular (OR:6,76), las alteraciones visuales (OR:1,98) y las anomalías en el examen físico de las arterias temporales (OR:2,77). Discusión: el diagnóstico de arteritis de células gigantes surge a partir de la sospecha clínica y no siempre es confirmado por la histopatología. Es importante llegar al mismo debido al riesgo, sobretodo visual, que reviste no iniciar tratamiento con esteroides lo antes posible...

Objectives: to determine the role of temporal artery biopsies in the diagnosis of temporal arteritis and to assess the efficacy of ACR criteria in the recognition of this disease. Material and methods: a retrospective, descriptive, observational study was performed. A total of 40 reports of temporal artery biopsies were reviewed at the Pathology service of our institution between 2000 and 2008. These results were correlated with clinical and laboratory findings. Results: 43% of biopsies were positive for giant cell arteritis, while 57% did not meet histological criteria for giant cell arteritis. By applying the diagnostic criteria of the American College of Rheumatology, 65% of patients met the criteria for giant cell arteritis. Of these 26 individuals, 61% had positive biopsies, while the rest had negative results. From the patients who did not meet American criteria, only one had a positive biopsy. If we take the parameters of the American College of Rheumatology criteria for diagnosing the disease and compared them with the biopsy, we see that they have a sensitivity of 94% and a specificity of 56%; a positive predictive value of 61% and a negative predictive value of 93%. The main predictors of positive biopsy were jaw claudication (OR:6.76), visual disorders (OR:1.98) and abnormalities on physical examination of temporal arteries (OR:2.77). Discusion: giant cell arteritisdiagnosis mainly arises from the clinical suspicion and is not always confirmed by histopathology. It is important to reach its diagnosis because of the risk, primarily visual, that lies in not starting the steroid treatment as early as possible...