[Mediastinal germ-cell tumours]. / Tumores germinales mediastínicos.

Germ-cell tumours of male ussually arise from the testis. However, in 2-5% of the cases, they also occur outside of the testis as a primary site without evidence of testicular primary tumour. This infrequent entity often appears in the body midline, predominantly in mediastinum and retroperitoneum. Mediastinal germ-cell tumours (MGCT) shall be included in the differential diagnosis of any mediastinic tumour of unknown origin. An accurate diagnosis is essential, due to the fact that these tumours are curable with chemotherapy. The histopathologic and clinical features, and its differences with germ-cell tumours from testicular origin are revised in this article.

[Adrenal failure caused by primary adrenal non-Hodgkin lymphoma: a case report and review of the literature]. / Insuficiencia suprarrenal causada por un linfoma no-Hodgkin B primario suprarrenal: presentación de un caso y revisión de la literatura.

We report a case of 78-year old man who presented with symptoms of adrenal insufficiency. The computed tomography (CT) scan showed the presence of bilateral adrenal masses. A CT-scan guided needle biopsy revealed diffuse large- B cell lymphoma. The absence of pathological findings in clinical, bone marrow and CT scan examinations supported the diagnosis of primary non-Hodgkin Lymphoma of the adrenal glands. The patient was treated with four cycles of R-CHOP chemotherapy with Rituximab, liposomal Doxorubicin, Cyclophosphamide, Vincristine and Prednisolone. At the end of fourth cycle there was radiological improvement but the chemotherapy was stopped because of IV grade toxicity. He completed treatment with radiotherapy of right adrenal mass. Few days after finishing radiation therapy the patient died due to a disseminated infection. No progressive disease was founded.

Tumores germinales mediastínicos / Mediastinal germ-cell tumours

Los tumores de células germinales del varón habitualmente se originan en los testículos. Sin embargo, en el 2-5% de los casos pueden aparecer de forma primaria en localizaciones extragonadales, sin evidencia de un tumor testicular. Esta infrecuente entidad suele aparecer en la línea media corporal, predominántemente en el mediastino y en el retroperitoneo. Los tumores germinales extragonadales mediastínicos (TGEM) deben incluirse en el diagnóstico diferencial de cualquier tumor mediastínico de origen desconocido. Un diagnóstico exacto es fundamental, debido a que son tumores potencialmente curables con quimioterapia. En este artículo se revisan las características histopatológicas y clínicas de losTGEM, y sus diferencias los tumores germinales de origen testicular

Germ-cell tumours of male ussually arise from the testis. However, in 2-5% of the cases, they also occur outside of the testis as a primary site without evidence of testicular primary tumour. This infrequent entity often appears in the body midline, predominantly in mediastinum and retroperitoneum. Mediastinal germ-cell tumours (MGCT) shall be included in the differential diagnosis of any mediastinic tumour of unknown origin. An accurate diagnosis is essential, due to the fact that these tumours are curable with chemotherapy. The histopathologic and clinical features, and its differences with germ-cell tumours from testicular origin are revised in this article

[Adjuvant treatment of operable breast cancer]. / Tratamiento adyuvante en el cáncer de mama operable.

Breast cancer(BC) is the most frequent neoplasm in women of the west countries. The treatment of BC is very complex, and include the combination of surgery, chemotherapy, radiotherapy, hormone therapy and immunotherapy. Surgery is the gold standard in the radical treatment of BC. Anthracyclines and taxanes are very important in the adjuvant treatment of BC. These drugs have shown an increased disease-free-survival and overall survival in several studies. Tamoxifen has been the gold standard adjuvant hormone therapy for the treatment of postmenopausal women with hormone-receptor-positive early BC for many years, but the third-generation aromatase inhibitors (letrozole, anastrozole, and exemestane) are now recommended as the preferred therapy. Trastuzumab in combination with adjuvant chemotherapy has changed the natural history of early Her-2 positive BC. New drugs are under investigation in the treatment of BC.

Insuficiencia suprarrenal causada por un linfoma no-Hodgking B primario suprarrenal: presentación de un caso y revisión de la literatura / Adrenal failure caused by primary adrenal non-hodking lymphoma: a case report and review of the literature

Presentamos el caso de un varón de 78 años que ingresa por un cuadro de debut de insuficiencia suprarrenal. Se realizó un estudio TC que mostró masas suprarrenales bilaterales de hasta 10 cm. Se completó estudio con biopsia percutanea de masa suprarrenal y biopsia de médula ósea, siendo diagnosticado de Linfoma no Hodgkin B difuso de células grandes primario suprarrenal con afectación suprarrenal bilateral. El paciente fue tratado con quimioterapia según esquema R-CHOP (Rituximab, Ciclofosfamida, Doxorrubicina liposomal, Vincristina y Prednisona).Tras 4 ciclos de quimioterapia se objetivo una respuesta parcial radiológica. Se suspendió la quimioterapia por toxicidad grado IV, completándose el tratamiento con RT sobre masa suprarrenal derecha. El paciente falleció por cuadro séptico pocos días después de finalizar la radioterapia, sin objetivarse progresión de la enfermedad

We report a case of 78-year old man who presented with symptoms of adrenal insufficiency. The computed tomography (CT) scan showed the presence of bilateral adrenal masses. A CT-scan guided needle biopsy revealed diffuse large- B cell lymphoma. The absence of pathological findings in clinical bone marrow and CT scan examinations supported the diagnosis of Lymphoma of the adrenal glands.The patient was treated with four cycles of R-CHOP chemotherapy with Rituximab, liposomal Doxorubicin, Cyclophosphamide, Vincristine and Prednisolone. At the end of fourth cycle there was radiological improvement but the chemotherapy was stopped because of IV grade toxicity. He completed treatment with radiotherapy of right adrenal mass. Few days after finishing radiation therapy the pacient died due to a disseminated infection. No progressive disease was founded

Tratamiento adyuvante en el cáncer de mama operable / Adjuvant treatment of operable breast cancer

El cáncer de mama (CM) es la neoplasia más frecuente en las mujeres de los países occidentales. El tratamiento del CM es muy complejo, e incluye la combinación de la cirugía, quimioterapia, radioterapia, hormonoterapia e inmunoterapia. La cirugía continúa siendo el “gold estándar”en el tratamiento radical de CM. Las antraciclinas y los taxanos son esenciales en el tratamiento adyuvante de CM. Estos fármacos han demostrado un incremento significativo de la supervivencia libre de enfermedad y global en múltiples estudios. El tamoxifeno ha sido el “gold estándar” en la hormonoterapia adyuvante de las mujeres posmenopáusicas con receptores hormonales positivos durante muchos años, pero los inhibidores de aromatasas de tercera generación (letrozol, anastrozol y exemestano) se han convertido en el tratamiento recomendado actualmente. Trastuzumab en combinación con la quimioterapia adyuvante ha modificado la historia natural del CM localizado Her-2 positivo. Nuevos fármacos están en investigación en el tratamiento del CM

Breast cancer (BC) is the most frequent neoplasm in women of the west countries. The treatment of BC is very complex, and include the combination of surgery, chemotherapy, radiotherapy, hormonetherapy and immunotherapy. Surgery is the gold standard in the radical treatment of BC. Anthracyclines and taxanes are very important in the adjuvant treatment of BC. These drugs have shown an increased disease free-survival and overall survival in several studies. Tamoxifen has been the gold standard adjuvant hormonetherapy for the treatment of postmenopausal women with hormone-receptor-positive early BC for many years, but the third-generation aromatase inhibitors (letrozole, anastrozole, and exemestane) are now recommended as the preferred therapy.Trastuzumab in combination with adjuvant chemotherapy has changed the natural history of early Her-2 positive BC. New drugs are underinvestigation in the treatment of BC

Management of gastric adenocarcinoma.

Gastric adenocarcinoma is the second most common cause of cancer death worldwide. The prognosis for patients with gastric adenocarcinoma depends on the stage of the disease at the time of diagnosis and treatment. Early gastric cancer, limited to the mucosa and submucosa, is best treated surgically and has a five-year survival rate of 70-95%. Surgical resection remains the primary curative treatment for localised disease. Despite this, the overall survival remains poor. The management of localised gastric adenocarcinoma is complex, and at present there is proven benefit of both preoperative chemotherapy and postoperative chemoradiotherapy. There is no standard regimen of chemotherapy for metastatic disease, although the regimen of ECF (epirubicin, cisplatin and fluorouracil) is the most used regimen, with a median survival of 7-9 months. With new regimens of chemotherapy, such as DCF (docetaxel, cisplatin and fluorouracil) or the combination of irinotecan, cisplatin and bevacizumab, the median survival has increased. Other new agents are under investigation.

Management of gastric adenocarcinoma

Gastric adenocarcinoma is the second most common cause of cancer death worldwide. The prognosis for patients with gastric adenocarcinoma depends on the stage of the disease at the time of diagnosis and treatment. Early gastric cancer, limited to the mucosa and submucosa, is best treated surgically and has a five-year survival rate of 70-95%. Surgical resection remains the primary curative treatment for localised disease. Despite this, the overall survival remains poor. The management of localised gastric adenocarcinoma is complex, and at present there is proven benefit of both preoperative chemotherapy and postoperative chemoradiotherapy. There is no standard regimen of chemotherapy for metastatic disease, although the regimen of ECF (epirubicin, cisplatin and fluorouracil) is the most used regimen, with a median survival of 7-9 months. With new regimens of chemotherapy, such as DCF (docetaxel, cisplatin and fluorouracil) or the combination of irinotecan, cisplatin and bevacizumab, the median survival has increased. Other new agents are under investigation (AU)

False-positive beta-human chorionic gonadotropin values in the follow-up of gestational trophoblastic disease.

Gestational trophoblastic disease consists of a pathological spectrum of entities from molar pregnancies, which are premalignant conditions, to malignant invasive choriocarcinoma. Serum Beta-human chorionic gonadotropin (hCG) levels are essential both in the diagnosis and in the follow-up. There are high rates of complete responses and long-term survivors, because of the excellent chemosensitivity of these tumours. After initial management, an increased level of Beta-hCG indicates persistent disease. However, in the absence of evidence of persistent disease, false-positive Beta-hCG values may be considered. We present here the case of a woman with a metastatic choriocarcinoma in complete response after chemotherapy, who developed later persistent false-positive values of Beta-hCG in the follow-up. Causes of false-positive Beta-hCG determinations are revised.

False-positive beta-human chorionic gonadotropin values in the follow-up of gestational trophoblastic disease

Gestational trophoblastic disease consists of a pathological spectrum of entities from molar pregnancies, which are premalignant conditions, to malignant invasive choriocarcinoma. Serum Beta-human chorionic gonadotropin (hCG) levels are essential both in the diagnosis and in the follow-up. There are high rates of complete responses and long-term survivors, because of the excellent chemosensitivity of these tumours. After initial management, an increased level of Beta-hCG indicates persistent disease. However, in the absence of evidence of persistent disease, false-positive Beta-hCG values may be considered. We present here the case of a woman with a metastatic choriocarcinoma in complete response after chemotherapy, who developed later persistent false-positive values of Beta-hCG in the follow-up. Causes of false-positive Beta-hCG determinations are revised (AU)

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[Waldemström s macroglobulinemia]. / Macroglobulinemia de Waldenström.

Waldenström s macroglobulinemia is an uncommon B-cell lymphoproliferative disorder characterized by bone marrow infiltration of luymphoplasmatic lymphoma, although sometimes there is a extramedullary lymphoma (nodal), with demonstration of an IgM monoclonal gammopathy. It must be distinguished from monoclonal gammopathy of undetermined significance and IgM- related disorders. The median survival of patients with Waldenström s macroglobulinemia averages 5 years. Asymptomatic cases should not be treated. Symtomatic cases could be treated with alkylating agents, nucleoside analogues (fludarabine or cladribine), and the monoclonal antibody rituximab. For the management of hyperviscosity and neuropathies plasmapheresis could be used successfully.

Macroglobulinemia de Waldenström / Waldenström´s macroglobulinemia

La macroglobulinemia de Waldenström es un trastorno linfoproliferativo de fenotipo B infrecuente, que se define por la presencia de infiltración de médula ósea por un linfoma linfoplasmocitoide, aunque en ocasiones se trata de un linfoma extramedular (ganglionar), asociado a una paraproteinemia IgM (monoclonal), que debe de diferenciarse de la gammapatía monoclonal de significado incierto y de los trastornos asociados a IgM. La supervivencia mediana de esta enfermedad es de unos 5 años. Los casos asintomáticos no requieren tratamiento. Para el tratamiento de la enfermedad sintomática se puede recurrir a los agentes alquilantes, a los análogos de purinas (fludarabina, cladribina) y al anticuerpo monoclonal anti-CD20 (rituximab). La plasmáferesis tiene su papel en el tratamiento del síndrome de hiperviscosidad, y de la neuropatía periférica asociados a la macroglobulinemia de Waldenström

Waldenström´s macroglobulinemia is an uncommon B-cell lymphoproliferative disorder characterized by bone marrow infiltration of luymphoplasmatic lymphoma, although sometimes there is a extramedullary lymphoma (nodal), with demonstration of an IgM monoclonal gammopathy. It must be distinguished from monoclonal gammopathy of undetermined significance and IgM- related disorders. The median survival of patients with Waldenström´s macroglobulinemia averages 5 years. Asymptomatic cases should not be treated. Symtomatic cases could be treated with alkylating agents, nucleoside analogues (fludarabine or cladribine), and the monoclonal antibody rituximab. For the managment of hyperviscosity and neuropathies plasmapheresis could be used successfully

Linfoma T angioinmunoblástico: caso clínico y revisión de la literatura / Angioimmunoblastic T-cell lymphoma: a case report and review of the literature

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[Pancreatic adenocarcinoma: therapeutical update]. / Adenocarcinoma de páncreas: actualizaciones terapéuticas.

Cancer of the exocrine pancreas continues to be a major unsolved health problem. Because of difficulties in diagnosis, the aggressiveness of pancreatic cancers, and the lack of effective systemic therapies, generally fewer than 5% of patients with adenocarcinoma of the pancreas survive 5 years after diagnosis. Thus, incidence rates and mortality rates are virtually identical. The median survival in metastatic pancreatic cancer is nearly six months.Today, surgery remains the only curative therapeutic option, and the standard treatment in patients with advanced disease is gemcitabine. New strategies for resectable and unresectable pancreatic cancer are under active investigation,such as neoadjuvant or adjuvant chemoradiotherapy or combinations of gemcitabine with new cytotoxic agents (oxaliplatin, cetuximab, gefitinib, bevacizumab) with promising results. In patients with locally advanced pancreatic cancer and good performance status, chemoradiotherapy should be considered.

Linfoma T/NK extraganglionar tipo nasal: caso clínico y revisión de la literatura / Extranodal T/NK-cell lymphoma, nasal type: a case report and review of the literature

El linfoma T/NK extraganglionar tipo nasal es un linfoma extraganglionar, que habitualmente expresa el fenotipo NK y VEB positivo, con un amplio pleomorfimo, frecuente necrosis y angioinvasión, que afecta de manera preferente a las estructuras mediofaciales, pero que también afecta a otras regiones extraganglionares. Presentamos el caso clínico de un varón de 42 años, con una masa intranasal, adenopatías submandibulares izquierdas y trombocitosis asintomática. La biopsia de la lesión intranasal consistía en un infiltrado difuso de células neoplásicas de estirpe linfoide de talla intermedia, con núcleos discretamente irregulares y pleomorfismo moderado. El inmunofenotipo de las células neoplásicas determinadas por inmunohistoquímica era: CD45+/cCD3+/Granzime B +/CD56+ , CD20-/CD4-/CD8-/LMP1(VEB)-. Todo ello era compatible con el diagnóstico de Linfoma NK extraganglionar tipo nasal. El estudio de extensión no mostró enfermedad a distancia. Con el diagnóstico de linfoma NK extraganglionar tipo nasal en estadio localizado con trombocitosis reactiva, el paciente inició de inmediato el tratamiento con poliquimioterapia seguido de radioterapia secuencial, decidiéndose realizar un trasplante autólogo de médula ósea en primera línea de tratamiento

Extranodal NK/T-cell lymphoma, nasal type, is an extranodal lymphoma, usually with an NK-cell phenotype and EBV possitive, with a broad morphologic spectrum, frequent necrosis and angioinvasion, and most commonly presenting in the midfacial region, but also in other extranodal sites. We present the case of a 42-years old-man with a intranasal mass, left cervical adenopathies and indolent thrombocytosis. The biopsy of the nasal mass showed a diffuse infiltrate of medium-sized lymphoid cells, with slightly irregular nuclei and moderate polymorphous. The immunophenotype of tumor cells was CD45+, cCD3+, Granzyme B+, CD56+, CD20-, CD4-, CD8-, LMP1 (EBV)-. The diagnosis of the case was Extranodal T/NK-cell lymphoma, nasal type. There was not disseminated disease in the extent studies. With the diagnosis of localized extranodal T/NK-cell lymphoma and reactive thrombocytosis, the patient was treated with chemotherapy and sequential radiotherapy, followed by bone marrow transplantation

[Extranodal T/NK-cell lymphoma, nasal type: a case report and review of the literature]. / Linfoma T/NK extraganglionar tipo nasal: caso clínico y revisión de la literatura.

Extranodal T/NK-cell lymphoma, nasal type: a case report and review of the literature. Extranodal NK/T-cell lymphoma, nasal type, is an extranodal lymphoma, usually with an NK-cell phenotype and EBV positive, with a broad morphologic spectrum, frequent necrosis and angioinvasion, and most commonly presenting in the midfacial region, but also in other extranodal sites. We present the case of a 42-years old-man with a intranasal mass, left cervical adenopathies and indolent thrombocytosis. The biopsy of the nasal mass showed a diffuse infiltrate of medium-sized lymphoid cells, with slightly irregular nuclei and moderate polymorphous. The immunophenotype of tumor cells was CD45+, cCD3+, Granzyme B+, CD56+, CD20-,CD4-,CD8-, LMP1(EBV)-. The diagnosis of the case was Extranodal T/NK-cell lymphoma, nasal type. There was not disseminated disease in the extent studies. With the diagnosis of localized extranodal T/NK-cell lymphoma and reactive thrombocytosis, the patient was treated with chemotherapy and sequential radiotherapy, followed by bone marrow transplantation.