Lymphoid follicular hyperplasia in patients with systemic lupus erythematosus after multiple cycles of rituximab.

Rituximab is indicated in some patients with refractory systemic lupus erythematosus (SLE). Occasionally, this medication is required in chronic form to maintain control of the disease. We described two patients who developed lymphoid follicular hyperplasia (LFH) after multiple cycles of rituximab and evaluated the expression of B cell activating factor belonging to the tumor necrosis factor (TNF) family (BAFF) and its receptors [BAFF-receptor (BAFF-R) and B cell maturation antigen (BCMA)], as possible factors related to lymphoid node enlargement. Two patients with SLE completed six and nine cycles of rituximab (1 g every 2 weeks) indicated each 9 months, achieving remission for 5 and 7 years, respectively, when developed prominent lymphadenopathies. Biopsies showed LFH. Haematological neoplasms were ruled out. Immunohistochemistry showed BAFF overexpression in the follicles, and moderate expression of BAFF-R confined to the mantle zone and BCMA to the germinal centre. Belimumab B cell activating factor belonging to the TNF family (anti-BAFF therapy) was started with positive effects on the clinical condition. LFH can develop in patients with SLE who received multiple cycles of rituximab. BAFF overexpression and moderate expression of BAFF-R and BCMA in lymph nodes were seen. These findings added to the improvement with the change to belimumab could suggest that LFH after cluster of differentiation (CD20) depletion therapy may be associated with a compensatory overexpression of BAFF and its receptors.

TAFRO syndrome mimicking systemic lupus erythematosus: Case report and literature review

TAFRO syndrome is a very rare disease, with less than 100 cases reported in the literature. It is classified as a type of idiopathic multicentric Castleman disease, but it has clinical, paraclinical, and histopathological characteristics that differentiate between TAFRO and idiopathic forms of Castleman disease not otherwise specified. However, it is a challenging exclusion diagnosis. TAFRO syndrome is characterized by systemic inflammatory involvement, often severe, which can present with kidney failure, and become a severe disease with a high mortality rate. The clinical manifestations of TAFRO can be confused with hematology malignancies or various autoimmune diseases. Although there are some reports of TAFRO syndrome associated with autoimmune compromise, there is no published consensus for the diagnosis or treatment. The case presented is a patient who meets the criteria to be classified as SLE, and with manifestations with significant clinical involvement, but with no improvement with standard treatment. It was found that the patient's systemic involvement was due to TAFRO, and that therefore the TAFRO syndrome could simulate SLE, something previously not described in the literature.

El síndrome TAFRO es una enfermedad muy poco común, con menos de 100 casos reportados en la literatura. Se clasifica como un tipo de enfermedad de Castleman multicéntrica idiopática, pero tiene características clínicas, paraclínicas e histopatológicas que permiten diferenciarla de las formas de la enfermedad Castleman idiopática no clasificadas de otra manera; sin embargo, es un diagnóstico de exclusión difícil de hacer. El síndrome TAFRO se caracteriza por compromiso inflamatorio sistémico, en muchas ocasiones severo, que puede presentarse con falla renal y convertirse en una enfermedad grave, con una alta tasa de mortalidad. Las manifestaciones clínicas de TAFRO pueden confundirse con neoplasias hematológicas o varias enfermedades autoinmunes. En la literatura existen algunos reportes de síndrome TAFRO asociados con compromiso autoinmune, pero no se ha publicado un consenso para su diagnóstico ni para su tratamiento. El caso que se presenta es un paciente que cumple con los criterios para ser clasificado como LES, que tenía manifestaciones con gran compromiso clínico, pero sin mejoría con el tratamiento estándar. Se encontró que el compromiso sistémico del paciente era por TAFRO y que, por lo tanto, el síndrome TAFRO podría simular LES, algo no descrito previamente en la literatura.

Ossifying Lipoma in Costal Arches That Suggest Extensive Pulmonary Involvement: Case Report.

BACKGROUND: Lipomas are the most common benign soft tissue tumors in the general population. These lesions can appear on any part of the body and usually develop in the subcutaneous superficial tissue. Lipomas that show ossifying changes are very rare, representing less than 1% of the reported lipomas. They usually manifest as hard nodular lesions in the head and neck, the extremities, the sternoclavicular region, and the subcutaneous tissue in general; they are rare in the costal arches. CASE PRESENTATION: We report the case of a patient with a history of multiple diseases and 2 tumor-like lesions with internal lytic areas detected in the fourth right costal arch and in the eighth left costal arc; we describe his clinical manifestations, radiological and laboratory findings as well as the pathological results and outcome. CONCLUSIONS: Ossifying lipomas are rare benign tumors with asymptomatic clinical presentation. It is important to perform an adequate radiological differentiation from other more aggressive lesions such as liposarcomas.

Prevalencia y características clínicas y anatomopatológicas de los pacientes con diagnóstico de linfoma difuso de células B grandes y positivo al virus Epstein-Barr en un hospital de alta complejidad en Cali (Colombia) / Prevalence and clinico-pathological characteristics in patients with Epstein-Barr virus-positive diffuse large B-cell lymphoma in a high complexity hospital in Cali, Columbia

INTRODUCCIÓN: El linfoma difuso de células B grandes (LDCBG) constituye el 35% de los linfomas no Hodgkin y su incidencia aumenta con la edad. El virus Epstein-Barr (VEB) está ampliamente distribuido a nivel mundial. La asociación entre el LDCBG y VEB está cerca del 10% en pacientes inmunocompetentes; este tipo de linfoma tiene alta prevalencia en países de Latinoamérica. OBJETIVO: Conocer la frecuencia del LDCBG asociado al VEB y describir sus características demográficas, clínicas, inmunofenotípicas y desenlace de los pacientes en un centro de alta complejidad en Cali (Colombia). MATERIALES Y MÉTODOS: Estudio observacional descriptivo de una cohorte histórica. Se revisaron los registros clínicos y de anatomía patológica de pacientes con diagnóstico de LDCBG y se realizó la hibridación in situ para la detección del VEB (EBER). Se realizó un análisis descriptivo. RESULTADOS: Entre 2011 y 2017 se revisó la historia clínica de 55 pacientes con diagnóstico de LDCBG. El 16% fueron VEB positivos, los cuales fueron en su mayoría del subtipo no centro germinal (89%), con presentación nodal (56%); hubo mayor prevalencia en hombres (68%), menor edad de presentación (mediana 48 años) y muerte en el 56% de los casos. CONCLUSIONES: Los pacientes con LDCBG y VEB positivo presentan con mayor frecuencia el subtipo no centro germinal, el cual, según nuestros hallazgos, se presenta en pacientes más jóvenes y se asocia a peor pronóstico. El EBER no es un examen que se hace de rutina, por lo cual se recomienda realizar pruebas para la detección del VEB en pacientes con diagnóstico de LDCBG

INTRODUCTION: Diffuse large B-cell lymphoma (DLBCL) accounts for 35% of non-Hodgkin lymphoma and its incidence increases with age. Epstein Barr virus (EBV) is widely spread worldwide. There is a 10% association between EBV and DLBCL in immunocompetent patients; this type of malignancy has a high prevalence in Latin American countries. OBJECTIVE: Estimate the percentage of association between DLBCL and EBV patients, describing demographics, clinical and immunological features, as well as phenotype and clinical outcome in a high complexity healthcare institution in Colombia. MATERIALS AND METHODS: This is an analytic observational study from an historical cohort. Clinical and pathological records were revised among DLBCL patients and subsequent in-situ hybridization was performed for EBV detection. A descriptive analysis of the data was carried out. RESULTS: From 2011 to 2017, 55 DLBCL patients were identified.16% were positive on ISH for EBV, most of which belonged to the non-germinal center B-cell immunophenotype (89%), with a nodal presentation (56%). DLBCL EBV positive was more prevalent among males (67%) and in younger patients (median of 48 years) where the mortality rate was 56%. CONCLUSIONS: DLBCL patients positive for EBV are more prone to belong to the non-germinal center B-cell immunophenotype which, according to our findings, is associated with a younger age and worse prognosis. Presently, EBER in-situ hybridization is not a part of routine tests, but we recommend its inclusion in the pathology package for DLBCL patients, as it can influence clinical outcomes

[Prevalence and clinico-pathological characteristics in patients with Epstein-Barr virus-positive diffuse large B-cell lymphoma in a high complexity hospital in Cali, Columbia]. / Prevalencia y características clínicas y anatomopatológicas de los pacientes con diagnóstico de linfoma difuso de células B grandes y positivo al virus Epstein-Barr en un hospital de alta complejidad en Cali (Colombia).

INTRODUCTION: Diffuse large B-cell lymphoma (DLBCL) accounts for 35% of non-Hodgkin lymphoma and its incidence increases with age. Epstein Barr virus (EBV) is widely spread worldwide. There is a 10% association between EBV and DLBCL in immunocompetent patients; this type of malignancy has a high prevalence in Latin American countries. OBJECTIVE: Estimate the percentage of association between DLBCL and EBV patients, describing demographics, clinical and immunological features, as well as phenotype and clinical outcome in a high complexity healthcare institution in Colombia. MATERIALS AND METHODS: This is an analytic observational study from an historical cohort. Clinical and pathological records were revised among DLBCL patients and subsequent in-situ hybridization was performed for EBV detection. A descriptive analysis of the data was carried out. RESULTS: From 2011 to 2017, 55 DLBCL patients were identified. 16% were positive on ISH for EBV, most of which belonged to the non-germinal center B-cell immunophenotype (89%), with a nodal presentation (56%). DLBCL EBV positive was more prevalent among males (67%) and in younger patients (median of 48 years) where the mortality rate was 56%. CONCLUSIONS: DLBCL patients positive for EBV are more prone to belong to the non-germinal center B-cell immunophenotype which, according to our findings, is associated with a younger age and worse prognosis. Presently, EBER in-situ hybridization is not a part of routine tests, but we recommend its inclusion in the pathology package for DLBCL patients, as it can influence clinical outcomes.

Caracterización histológica y clínica de pacientes con enfermedad relacionada con inmunoglobulina G4 en un centro de alta complejidad en Cali, Colombia / Histological and clinical characteristics of cases of immunoglobulin G4-related disease in a tertiary hospital, in Cali, Colombia

Introducción: La enfermedad relacionada con inmunoglobulina G4 (IgG4) ha sido reconocida durante la última década. Es una condición fibroinflamatoria con capacidad de comprometer casi cualquier órgano. El diagnóstico requiere la confirmación histológica, clínica y paraclínica. En Colombia, este es el estudio con mayor número de casos. Objetivo: Describir las características clínicas e histopatológicas de los pacientes diagnosticados con enfermedad relacionada con IgG4 en la Fundación Valle del Lili. Métodos: Estudio observacional descriptivo retrospectivo. Se revisaron los registros clínicos y patológicos de pacientes a quienes se les diagnosticó enfermedad relacionada con IgG4 en la institución. Se utilizó estadística descriptiva. Resultados: Entre 2013 y 2016 se diagnosticaron 16 pacientes. La mediana de edad fue 44 años, rango intercuartílico 30-58 y 10 (62,5%) fueron mujeres. La presentación clínica más común fue la asociación de masa+síntomas constitucionales+síntomas relacionados con el sitio de localización 43,8% (n=7). No hubo predominancia por algún órgano. En la histopatología todos presentaron infiltrado linfoplasmocitario y fibrosis estoriforme, el 75% flebitis obliterante; en todos los casos se evidenció≥10 células/CAP de IgG4+ y el 81% tuvieron una razón de IgG4+/IgG+>50%. Conclusión: Dada la baja sospecha y el amplio espectro clínico, se cree que existe un subdiagnóstico de la enfermedad. De acuerdo a nuestros hallazgos se recomienda que ante la presencia de infiltrado linfoplasmocitario, fibrosis estoriforme o flebitis obliterante en la evaluación histológica, se solicite inmunohistoquímica para IgG e IgG4, cuya positividad deberá ser correlacionada con estudios complementarios para la confirmación diagnóstica

Introduction: Immunoglobulin G4 (IgG4)-related disease has been described in the last decade. It is a fibro-inflammatory condition capable of affecting almost every organ and diagnosis requires both clinical and paraclinical confirmation. We present the largest study to date in Colombia. Objective: To describe the clinical and histopathological characteristics of patients diagnosed with IgG4-related disease at the Fundación Valle del Lili. Methods: Observational-descriptive retrospective study. The clinical and pathological records of patients diagnosed with IgG4-related disease at the Fundación Valle del Lili were reviewed and a descriptive statistical analysis made. Results: From 2013-2016, 16 patients were diagnosed. Median age was 44 years (RIC 30-58) and 10 (62.5%) were women. The most common clinical presentation was a combination of a tumefactive mass, constitutional symptoms and site-related symptoms (43.8%) (n=7). No preference for any organ was seen. Histopathology revealed all cases had dense lymphoplasmacytic infiltrate and storiform-type fibrosis; 75% also had obliterative phlebitis. In all cases≥10 cells/HPF of IgG4+ were found and 81% had a ratio of IgG4+/IgG+>50%. Conclusion: IgG4-related disease appears to be underdiagnosed, probably due to its broad clinical spectrum as well as a low index of awareness among clinicians. We recommend that, when dense lymphoplasmacytic infiltrates, storiform-type fibrosis or obliterative phlebitis are found, immunohistochemistry for IgG and IgG4should be requested. Positive results then must be correlated with complementary studies to confirm the disease

Disseminated histoplasmosis as a first clinical manifestation in a patient with small lymphocytic lymphoma: A case report

The small lymphocytic lymphoma is a mature B cell neoplasm with a broad spectrum of clinical presentations. Opportunistic infections that are not related to the treatment, even in advanced stages, have a low incidence rate. There are few case reports in the medical literature of patients who have not received immunosuppressive therapy and present with small lymphocytic lymphoma associated with disseminated histoplasmosis at diagnosis. A female 82-year-old patient was admitted due to an intermittent dry cough, asthenia, and adynamia that had persisted for one month. Multiple studies to detect infections and immuno-rheumatic conditions were performed and an extensive cervical, thoracic and peritoneal adenopathic syndrome was diagnosed. A flow cytometry and a cervical lymph node biopsy were performed reporting CD19+, CD20dim, CD5+, CD45+, CD23+, CD43neg, and CD10neg phenotypes with restriction in the light kappa chain compatible with a small lymphocytic lymphoma. Epithelioid granulomas without necrosis were observed in the lymph node histopathology and special colorations showed no microorganisms. The culture from the lymph node was positive for Histoplasma capsulatum. We initiated treatment with amphotericin B and itraconazole with an adequate response. In the absence of compliance with oncology treatment criteria, the patient was managed on a "watch and wait" basis. Opportunistic infections could be the initial clinical manifestation in patients with low-grade lymphoproliferative syndromes. This case report shows that they can develop even in the absence of chemotherapy.

[Histological and clinical characteristics of cases of immunoglobulin G4-related disease in a tertiary hospital, in Cali, Colombia]. / Caracterización histológica y clínica de pacientes con enfermedad relacionada con inmunoglobulina G4 en un centro de alta complejidad en Cali, Colombia.

INTRODUCTION: Immunoglobulin G4 (IgG4)-related disease has been described in the last decade. It is a fibro-inflammatory condition capable of affecting almost every organ and diagnosis requires both clinical and paraclinical confirmation. We present the largest study to date in Colombia. OBJECTIVE: To describe the clinical and histopathological characteristics of patients diagnosed with IgG4-related disease at the Fundación Valle del Lili. METHODS: Observational-descriptive retrospective study. The clinical and pathological records of patients diagnosed with IgG4-related disease at the Fundación Valle del Lili were reviewed and a descriptive statistical analysis made. RESULTS: From 2013-2016, 16 patients were diagnosed. Median age was 44 years (RIC 30-58) and 10 (62.5%) were women. The most common clinical presentation was a combination of a tumefactive mass, constitutional symptoms and site-related symptoms (43.8%) (n=7). No preference for any organ was seen. Histopathology revealed all cases had dense lymphoplasmacytic infiltrate and storiform-type fibrosis; 75% also had obliterative phlebitis. In all cases≥10 cells/HPF of IgG4+ were found and 81% had a ratio of IgG4+/IgG+>50%. CONCLUSION: IgG4-related disease appears to be underdiagnosed, probably due to its broad clinical spectrum as well as a low index of awareness among clinicians. We recommend that, when dense lymphoplasmacytic infiltrates, storiform-type fibrosis or obliterative phlebitis are found, immunohistochemistry for IgG and IgG4should be requested. Positive results then must be correlated with complementary studies to confirm the disease.

Histoplasmosis diseminada como manifestación inicial del linfoma linfocítico de células pequeñas / Disseminated histoplasmosis as a first clinical manifestation in a patient with small lymphocytic lymphoma: A case report

RESUMEN El linfoma linfocítico de células pequeñas es una neoplasia de células B maduras con un amplio espectro de presentaciones clínicas. Las infecciones por gérmenes oportunistas no asociadas con el tratamiento, incluso en estadios avanzados de la enfermedad, tienen baja incidencia. Se han reportado muy pocos casos de pacientes con linfoma linfocítico de células pequeñas asociado a histoplasmosis diseminada que no habían recibido quimioterapia en el momento del diagnóstico. Se presenta el caso de una paciente de 82 años que fue hospitalizada por presentar tos seca intermitente, astenia y adinamia de un mes de evolución. Se le practicaron múltiples estudios para detectar infecciones o compromiso inmunológico o reumático, y se diagnosticó un síndrome adenopático extenso con compromiso cervical, torácico y retroperitoneal. En la citometría de flujo y en la biopsia de ganglio linfático cervical, se reportaron los fenotipos CD19+, CD20dim, CD5+, CD45+, CD23+, CD43neg y CD10neg, con restricción de la cadena ligera kappa, lo cual confirmó un linfoma linfocítico de células pequeñas. En la histopatología del ganglio, se observaron granulomas epitelioides sin necrosis, pero las coloraciones especiales no mostraron la presencia de microorganismos, en tanto que el cultivo del ganglio fue positivo para Histoplasma capsulatum. Se inició el tratamiento antifúngico con anfotericina B e itraconazol, y la paciente tuvo una adecuada evolución. Dado que no se cumplían los criterios para el tratamiento oncológico, se continuó con su observación mediante controles periódicos. Las infecciones oportunistas pueden ser la manifestación clínica inicial en pacientes con síndromes linfoproliferativos de bajo grado. Este caso demuestra que pueden desarrollarse, incluso, en ausencia de quimioterapia.

ABSTRACT The small lymphocytic lymphoma is a mature B cell neoplasm with a broad spectrum of clinical presentations. Opportunistic infections that are not related to the treatment, even in advanced stages, have a low incidence rate. There are few case reports in the medical literature of patients who have not received immunosuppressive therapy and present with small lymphocytic lymphoma associated with disseminated histoplasmosis at diagnosis. A female 82-year-old patient was admitted due to an intermittent dry cough, asthenia, and adynamia that had persisted for one month. Multiple studies to detect infections and immuno-rheumatic conditions were performed and an extensive cervical, thoracic and peritoneal adenopathic syndrome was diagnosed. A flow cytometry and a cervical lymph node biopsy were performed reporting CD19+, CD20dim, CD5+, CD45+, CD23+, CD43neg, and CD10neg phenotypes with restriction in the light kappa chain compatible with a small lymphocytic lymphoma. Epithelioid granulomas without necrosis were observed in the lymph node histopathology and special colorations showed no microorganisms. The culture from the lymph node was positive for Histoplasma capsulatum. We initiated treatment with amphotericin B and itraconazole with an adequate response. In the absence of compliance with oncology treatment criteria, the patient was managed on a "watch and wait" basis. Opportunistic infections could be the initial clinical manifestation in patients with low-grade lymphoproliferative syndromes. This case report shows that they can develop even in the absence of chemotherapy.

Lymphomatoid granulomatosis of the brain: A case report.

BACKGROUND: Lymphomatoid granulomatosis is a rare disorder of the central nervous system (CNS) with few cases being reported in literature. We present the case of an adult with an unusual lesion of the CNS who presented with motor seizures and was diagnosed with lymphomatoid granulomatosis, followed by a discussion of the process of evaluation and management. CASE DESCRIPTION: A 42-year-old male presented with motor seizures and loss of consciousness for 10 minutes along with dysarthria and left hemiplegia. Neurological examination and imaging with magnetic resonance imaging (MRI) of the brain revealed a mass in the right striatum. The patient was hospitalized and underwent an image-guided right frontal craniotomy using the Leksell Stereotactic G-Frame. Pathology reported a lymphomatoid granulomatosis. Being immunocompetent, the patient received medical treatment with prednisone and rituximab. Two years after his diagnosis, the patient had no active disease and his brain MRI did not show contrast enhancement. After almost 3 years of follow-up, the patient has a mild weakness in the left-side of his body (4/5), is seizure-free, and can walk and perform daily activities. CONCLUSIONS: This rare lesion in an adult, immunocompetent patient, debuting with motor seizures represents a challenge in terms of diagnosis and treatment. After surgical and medical treatment, the patient had a satisfactory recovery. Clinical features, imaging, differential diagnosis, and pathology are discussed.

Detección rápida de metástasis en ganglio centinela y comparaciónde diferentes técnicas en carcinomas de mama de bajo grado / Rapid detection of sentinel lymph node metastases in different techniques and comparison in low-grade breast carcinomas

Introduction: The role of sentinel node biopsy has revolutionized breast cancer treatment. This determinationreduces the mobility of a complete axillary lymphadenectomy. The aim of our study is to analyze the value of sentinel node in low-grade histological breast tumors, studied with hematoxylin and eosin techniques, mmunohistochemistry,and molecular chain reaction in real-time quantitative polymerase (RT-PCR). Materials and methods: In a pilot study we studied a total of 21 patients with histological diagnosis of mucinous carcinoma, adenoid cystic carcinoma, and medullar carcinoma that underwent the sentinel node technique. Once the lymph node was removed, it was sent to pathology, where it was fragmented and evaluated, using between 25% and 50% of the lymph node for molecular biology laboratory studies. Results: The sentinel nodes studied were 32, corresponding to the 21 patients. Of the 32 lymph nodes analyzed, 29 (90.6%) were negative on histopathological examination and the molecular identification, 2 (6.2%) were positive in both techniques and 1 (3.125%) lymph node was positive with quantitative RT-PCR and negative in histology (H&E), which – subsequently by immunohistochemistry (IHC) – was diagnosed as isolated tumor cells (ITC). Conclusion: When comparing the techniques of hematoxylin and eosin, immunohistochemistry, and molecularRT-PCR technique, we found greater sensitivity of molecular techniques; this can reduce the false negative andimprove diagnosis of sentinel node metastases. Patients with low histological grade carcinomas have high survivalrates, less aggressive tumor behavior, and reduced lymph node at diagnosis.

In situ mantle cell lymphoma: clinical implications of an incidental finding with indolent clinical behavior.

BACKGROUND: Cyclin D1-positive B cells are occasionally found in the mantle zones of reactive lymphoid follicles, a condition that has been called "in situ mantle cell lymphoma". The clinical significance of this lesion remains uncertain. DESIGN AND METHODS: The clinical and pathological characteristics, including SOX11 expression, of 23 cases initially diagnosed as in situ mantle cell lymphoma were studied. RESULTS: Seventeen of the 23 cases fulfilled the criteria for in situ mantle cell lymphoma. In most cases, the lesions were incidental findings in reactive lymph nodes. The t(11;14) was detected in all eight cases examined. SOX11 was positive in seven of 16 cases (44%). Five cases were associated with other small B-cell lymphomas. In two cases, both SOX11-positive, the in situ mantle cell lymphoma lesions were discovered after the diagnosis of overt lymphoma; one 4 years earlier, and one 3 years later. Twelve of the remaining 15 patients had a follow-up of at least 1 year (median 2 years; range, 1-19.5), of whom 11 showed no evidence of progression, including seven who were not treated. Only one of 12 patients with an in situ mantle cell lymphoma lesion and no diagnosis of mantle cell lymphoma at the time developed an overt lymphoma, 4 years later; this case was also SOX11-positive. The six remaining cases were diagnosed as mantle cell lymphoma with a mantle zone pattern. Five were SOX11-positive and four of them were associated with lymphoma without a mantle zone pattern. CONCLUSIONS: In situ mantle cell lymphoma lesions are usually an incidental finding with a very indolent behavior. These cases must be distinguished from mantle cell lymphoma with a mantle zone pattern and overt mantle cell lymphoma because they may not require therapeutic intervention.

Detección inmunomagnética de células tumorales circulantes en cáncer de mama metastásico: nuevas tecnologías / Inmunomagnetic detection of circulating tumor cells in metastatic breast cancer: new technologies

Las metástasis hematógenas son la mayor causa de mortalidad en el cáncer de mama. Está documentado que una vez las células tumorales se diseminan el resultado es, generalmente, letal. Las células tumorales circulantes han sido consideradas por largo tiempo un reflejo de la agresividad de los tumores, y entre ellos uno de los más agresivos es el cáncer de mama metastásico. Los primeros resultados clínicos han permitido determinar una fuerte relación entre la detección y el número de las células tumorales circulantes, como un valor pronóstico y como marcador de la actividad antitumoral del tratamiento. El análisis inmunomagnético utilizando una nueva metodología permite determinar que un recuento de 5 células tumorales circulantes o más en 7,5 ml de sangre, en cualquier fase de la enfermedad, se asocia a un mal pronóstico, y es predictivo de una supervivencia global más corta.

Hematogenous metastasis is the major cause of mortality in breast cancer. Evidence indicates that tumor cells escape from the primary tumor mass into the blood stream and that these disseminated cells are the source of increased lethality. Circulating or metastatic tumour cells have been considered as useful indicators of the aggressiveness of breast cancer tumours. The first clinical results obtained with such assays strongly suggest that in metastatic breast cancer, circulating tumour cells detection and enumeration can be used to estimate prognosis and may serve as an early marker to assess anti-tumour activity of a treatment. Immunomagnetic analysis using a new methodology, determine that a circulating tumour cells count of 5 or more per 7,5 ml of blood, at any time during the course of the disease is associated with a poor prognosis and is predictive of shorter progression and overall survival.

Report of the first case of myxoid liposarcoma in Colombia: a rare tumor / Informe del primer caso de liposarcoma mixoide en Colombia: un tumor raro

Introduction: Primary esophageal myxoid liposarcoma is exceedingly rare. Sarcomas make up 1% of esophageal malignant tumors. There are only five (5) cases reported with this histological variant (myxoid) in previous literature. In Colombia, this is the first case reported and the sixth in the world. Objective: To report the first case in Colombia of myxoid liposarcoma of the esophagus with clinical, radiographic images, histology, surgical and to describe difficulties in the diagnosis. Methodology: We reviewed the clinical history of a 28-year old male patient. He was admitted to Hospital Universitario del Valle in Cali, Colombia with a clinical history of dysphagia, weight loss, and excessive salivation. The initial examination (esophagogram, cervical CAT scan and endoscopy) demonstrated a mass that was reported as a fibrovascular polyp. The finding of the pathological diagnosis was myxoid liposarcoma. Conclusions: The rarity of this condition recommends report of its detailed description. The myxoid liposarcoma of the esophagus can be diagnosed if a patient has a history of a slow-growing esophageal mass with a low tumor density in computed tomography in combination with surgical resection and histological examination.

Introducción: El liposarcoma mixoide es una neoplasia maligna del mesénquima con una presentación muy rara en esófago. Los sarcomas representan 1% de los tumores esofágicos malignos, y este tipo histológico es el menos frecuente. En la actualidad, se encuentran informes en la literatura de cinco (5) casos de esta variante histológica en el esófago. En Colombia, es el primer caso encontrado y el sexto (6º) a nivel mundial. Objetivo: Presentar el primer caso en Colombia de un liposarcoma mixoide en el esófago, sus características clínicas, imagenológicas, histología, manejo quirúrgico y las dificultades en su diagnóstico. Metodología: Se revisó la historia clínica de un paciente masculino de 28 años que ingresa por urgencias al Hospital Universitario del Valle en Cali, Colombia, con historia de disfagia, pérdida de peso y sialorrea. Los estudios imagenológicos como esofagograma, escanografía cervical y endoscopia de vías digestivas altas son consistentes con un pólipo esofágico y el manejo quirúrgico consistió en la resección parcial y luego la resección completa de la lesión. El informe histopatológico de la resección parcial comunicó un pólipo fibrovascular y el informe de la resección completa fue de liposarcoma mixoide. Las dificultades diagnósticas que surgieron en este caso se relacionan con otras encontradas en la literatura. Conclusiones: El liposarcoma mixoide del esófago es una entidad que presenta dificultades en su diagnóstico debido a que la presentación clínica no es específica. Aunque en estos casos las biopsias iniciales pueden suponer lesiones benignas, sólo hasta el procesamiento histológico de todo el espécimen, es posible realizar el diagnóstico de la entidad.