Rib desmoplastic fibroblastoma: Video-assisted resection.

A 46-year-old man was referred to our department due to chronic chest pain. A computed tomography showed an exophytic image arising from 5th rib that was projected on the middle lobule. The patient underwent an exploratory videothoracoscopic because we couldn't discard lung compromise. A corneal-like lesion emerging from the inner side of the 5th rib was revealed. Complete video-assited resection was done. Histopathology examination revealed a desmoplastic fibroblastoma. To our knowledge this is the first case of desmoplastic fibroblastoma arising from a rib and the second case published in all literature of chest wall involvement.

[Fatal interstitial lung disease associated with maximum androgen blockade]. / Neumonía intersticial inducida por bloqueo androgénico máximo como tratamiento de cáncer de próstata avanzado: Report of one case.

Maximum androgen blockade is the standard endocrine treatment for advanced prostate cancer. Interstitial lung disease in different degrees of severity, with low mortality and excellent response to treatment may appear with its use. We report a 77 years old patient with advanced prostate cancer who developed severe and progressive respiratory failure associated to bilateral pulmonary infiltrates, attributed to the direct effect of maximum androgen blockade. Despite the therapeutic efforts, the patient died. Lung pathology revealed Usual Interstitial Pneumonia.

[Intravascular lymphoma: Report of one case]. / Linfoma intravascular, un desafio diagnóstico: Caso clínico.

Intravascular lymphoma is a rare subtype of extranodal diffuse large B-cell lymphoma characterized by clonal proliferation of lymphocytes inside of small and medium caliber vessels. Its incidence is estimated at one case per million. The clinical picture is very variable, but frequently has skin and central nervous system involvement. It is diagnosed by demonstrating pathological blood vessel infiltration by lymphoma cells. We report a 44 years old male presenting with fever, malaise and erythematous lesions in the abdominal wall. An abdominal wall biopsy showed dilated vascular vessels with atypical cells in their lumen, compatible with large B-cell intravascular lymphoma. He was treated with rituximab, cyclophosphamide, adriamycin, vincristine and prednisone and an autologous hematopoietic stem cell transplantation, achieving a complete remission that has lasted two years.

[Colonic pseudotumoral involvement as an expression of severe plasminogen deficiency]. / Compromiso pseudotumoral colónico como expresión de déficit grave de plasminogéno.

Plasminogen deficiency is a very rare multisystem entity that affects different tissues of the economy through the deposition of fibrin-rich pseudomembrane and determines a heterogeneous and diverse clinical presentation. It is transmitted in an autosomal recessive manner by mutations of the PLG gene on chromosome 6 and can be divided into hypoplasminogenemia or type I and dysplasminogenemia or type II, the latter not related to clinical pathology. Severe plasminogen deficiency has a prevalence of 1.6 individuals per million inhabitants and although it can be diagnosed in adulthood, the most severe symptoms are observed in infants and children. The most common form of onset is the so-called woody conjunctivitis, characterized by fibrin membranes that are deposited on the eyelids since childhood, causing exophytic lesions that affect vision. It can also affect other mucous membranes such as the gingival, respiratory, oropharyngeal, digestive and genital mucosa, among others. We present a rare case of severe plasminogen deficiency with conjunctivitis and woody cervicitis who was admitted with clinical acute abdominal symptoms, associated with a tumor mass due to pseudomembranous deposition in the ascending colon that simulated inflammatory bowel disease and resolved spontaneously.

La deficiencia de plasminógeno es una entidad multisistémica, muy infrecuente, que afecta diferentes tejidos de la economía mediante el depósito de pseudo membranas ricas en fibrina y que determina una presentación clínica heterogénea y diversa. Se transmite en forma autosómica recesiva por mutaciones del gen PLG del cromosoma 6 y se puede dividir en hipoplasminogenemia o tipo I y displasminogenemia o tipo II, esta última no relacionada con patología clínica. El déficit grave de plasminógeno tiene una prevalencia de 1.6 individuos por millón de habitantes y si bien puede diagnosticarse en edad adulta, los síntomas más graves se observan en lactantes y niños. La forma de inicio más común es la denominada conjuntivitis leñosa, caracterizada por membranas de fibrina que se depositan en los parpados desde la infancia, provocando lesiones exofíticas que afectan la visión. También puede afectar otras mucosas como la gingival, respiratoria, orofaríngea, digestiva y genital entre otros. Presentamos un raro caso de deficiencia grave de plasminógeno con conjuntivitis y cervicitis leñosa que ingresó con un cuadro de abdomen agudo clínico, asociado a una masa tumoral por depósito de pseudomembranas en el colon ascendente que simuló una enfermedad inflamatoria intestinal y que se resolvió espontáneamente.

Intradiaphragmatic bronchogenic cyst: report of a rare new case.

Bronchogenic cysts (BCs) are congenital abnormalities commonly found within the mediastinum. Diaphragmatic location is extremely rare. We present a woman in her 60s with an asymptomatic intradiaphragmatic BC. The patient underwent complete surgical resection with primary closure of the left hemidiaphragm. BCs should remain a differential diagnosis of any soft-tissue mass in the region of the diaphragm. Complete surgical resection is recommended even in asymptomatic patients to avoid complications such as infection, rupture, bleeding or malignant transformation.

Laparoscopic transgastric resection of a gastric leiomyosarcoma: report of a rare case.

Gastric leiomyosarcoma is a rare type of tumour that is far less prevalent than gastrointestinal stromal tumours. We describe a case of a 42-year-old male patient who consulted for upper abdominal pain. Blood work revealed low haemoglobin levels, requiring red blood cell transfusions. An esophagogastroduodenoscopy was performed, showing a submucosal tumour with central ulceration in the greater gastric curvature. The patient underwent an endoscopic ultrasound with fine needle biopsy and the sample showed a spindle cell neoplasia. Computed tomography scan demonstrated absence of distant metastases. Upon multidisciplinary consensus, it was decided to perform surgery. A laparoscopic approach was conducted, where no peritoneal lesions were observed. Transgastric resection of the tumour was performed. Free tumour margins were achieved following oncologic criteria (minimum tumour manipulation and one-piece resection without damaging the tumour capsule). After exhaustive sampling, the final pathology report informed an 11 × 9 × 5 cm gastric leiomyosarcoma. Immunohistochemical examination showed positivity with smooth muscle actin, muscle-specific actin, calponin and desmin. The patient had an uneventful recovery, and 6 post-operative months' clinical, tomographic and endoscopic control informed no disease recurrence. To the best of our knowledge, there are less than 20 published cases of patients with diagnosis of gastric leiomyosarcoma. This study highlights the importance of reporting this entity, in order to contribute to the available literature concerning this topic.

Histamine H4 Receptor Expression in Triple-negative Breast Cancer: An Exploratory Study.

Triple-negative breast cancer (TNBC) is an aggressive breast cancer subtype. There are neither universally accepted prognostic markers nor molecular targets related to TNBC. The histamine H4 receptor (H4R) has been characterized in TNBC experimental models, demonstrating its critical role in tumor development and progression. In this study, H4R expression was compared in breast cancer subtypes and correlated with clinical features using The Cancer Genome Atlas data (Pan-Cancer Atlas). The H4R status was further evaluated by immunohistochemistry in 30 TNBC human samples in relation to clinicopathological parameters. Results indicate that H4R was downregulated in basal-like/TNBC compared with luminal A and normal breast-like tumors. The higher expression of H4R was associated with improved progression-free and overall survival outcomes in basal-like/TNBC. H4R immunoreactivity was detected in about 70% of tumors, and its expression was positively correlated with the levels in the histologically normal peritumoral tissue. High H4R expression in peritumoral tissue correlated with reduced number of lymph node involvement and unifocal TNBC, while it was associated with increased patient survival. In conclusion, the H4R might represent a potential prognostic biomarker in TNBC. Further studies in large cohorts are needed to better understand the significance of H4R in breast cancer biology.

Pseudotumoral Presentation of Cerebral Amyloid-Beta Angiopathy: Case Report and Review of Literature.

OBJECTIVE: Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare and potentially treatable encephalopathy that usually affects people older than 50 years old and has an acute or subacute clinical presentation characterized by rapidly evolving cognitive decline, focal deficits and seizures. In a small subset of patients the disease can adopt a pseudotumoral form in the neuroimages that represents a very difficult diagnostic challenge. METHODS: Here in we report a patient with a tumour-like presentation of histopathologically confirmed CAA-RI. RESULTS: We also conducted a search and reviewed the clinical and radiological features of 41 cases of pseudotumoral CAA-RI previously reported in the literature in order to identify those characteristics that should raise diagnostic suspicions of the disease, there by avoiding unnecessary surgical treatments. CONCLUSION: The therapy of CAA-RI with steroids is usually effective and clinical and radiological remission can be achieved in the first month in approximately 70% of cases.

Compromiso pseudotumoral colónico como expresión de déficit grave de plasminogéno / Colonic pseudotumoral involvement as an expression of severe plasminogen deficiency

Resumen La deficiencia de plasminógeno es una entidad multisistémica, muy infrecuente, que afecta diferentes tejidos de la economía mediante el depósito de pseu do membranas ricas en fibrina y que determina una presentación clínica heterogénea y diversa. Se trans mite en forma autosómica recesiva por mutaciones del gen PLG del cromosoma 6 y se puede dividir en hipoplasminogenemia o tipo I y displasminogenemia o tipo II, esta última no relacionada con patología clínica. El déficit grave de plasminógeno tiene una prevalencia de 1.6 individuos por millón de habitan tes y si bien puede diagnosticarse en edad adulta, los síntomas más graves se observan en lactantes y ni ños. La forma de inicio más común es la denominada conjuntivitis leñosa, caracterizada por membranas de fibrina que se depositan en los parpados desde la infancia, provocando lesiones exofíticas que afectan la visión. También puede afectar otras mucosas como la gingival, respiratoria, orofaríngea, digestiva y genital entre otros. Presentamos un raro caso de deficiencia grave de plasminógeno con conjuntivitis y cervicitis leñosa que ingresó con un cuadro de abdomen agudo clínico, asociado a una masa tumoral por depósito de pseudomembranas en el colon ascendente que simu ló una enfermedad inflamatoria intestinal y que se resolvió espontáneamente.

Abstract Plasminogen deficiency is a very rare multisystem entity that affects different tissues of the economy through the de position of fibrin-rich pseudomembrane and determines a heterogeneous and diverse clinical presentation. It is trans mitted in an autosomal recessive manner by mutations of the PLG gene on chromosome 6 and can be divided into hypoplasminogenemia or type I and dysplasminogenemia or type II, the latter not related to clinical pathology. Severe plasminogen deficiency has a prevalence of 1.6 individuals per million inhabitants and although it can be diagnosed in adulthood, the most severe symptoms are observed in infants and children. The most common form of onset is the so-called woody conjunctivitis, characterized by fibrin membranes that are deposited on the eyelids since child hood, causing exophytic lesions that affect vision. It can also affect other mucous membranes such as the gingival, respiratory, oropharyngeal, digestive and genital mucosa, among others. We present a rare case of severe plasminogen deficiency with conjunctivitis and woody cervicitis who was admitted with clinical acute abdominal symptoms, associated with a tumor mass due to pseudomembranous deposition in the ascending colon that simulated inflam matory bowel disease and resolved spontaneously.

Primary retroperitoneal mucinous cystadenoma.

Mucinous cystadenomas and carcinomas of the ovary are well-established and common tumors. However, Primary retroperitoneal mucinous cystadenomas (PRMCs) are very rare. We present a 50-year-old woman referred to our institution with history of intermittent abdominal pain and palpable mass in her left flank. On CT-scan an unilocular cystic mass with thin wall measuring 171×155×108mm in the retroperitoneum was shown. Patient underwent surgical excision of the tumor. The histopathology examination showed a cystic mass with a fibrous wall and an epithelium composed of a single layer of columnar cells with mucin vacuoles compatible with PRMC.

Hepatic metastasis of thymoma: case report and immunohistochemical study.

Thymomas are rare tumours characterised by their slow growth and capacity to invade directly by contiguity. While distant dissemination is infrequent, all sub-types of thymoma have the capacity to metastasise to extrathoracic organs. We present here the case of a female patient with a liver mass discovered 13 years after the removal of a mediastinal thymoma and after ten years from thyroidectomy for papillary carcinoma. The histopathological study showed that the lesion contained an epithelial component, which was immunohistochemically positive for pankeratin. It was accompanied by numerous small lymphocytes testing positive for TdT, CD3, CD4, CD5, CD8, CD99, and CD43. The result was consistent with hepatic metastatic thymoma sub-type B1, according to the World Health Organisation classification (WHO). Our case highlights the importance of morphological and immunohistological examinations in the differential diagnosis of visceral masses in patients with a history of thymoma. Given the infrequency of its metastasis and the increased risk of developing other primary tumours that these patients have, these studies play a significant role.

Proteinosis alveolar secundaria: reporte de un caso / Proteinosis alveolar secundaria: case report

La proteinosis alveolar es un enfermedad caracterizada por el acúmulo anormal de surfactante en el espacio alveolar lo que conlleva a disrupción en el intercambio gaseoso. Su fisiopatogenia es variada y en los adultos la forma secundaria es la más frecuente. Presentamos el caso de una paciente con leucemia mieloide aguda en tratamiento compasivo con gemtuzumab ozogamicina, e infecciones respiratorias a repetición que se presenta con disnea progresiva y tos seca. Luego de descartar causa embólica y cardíaca, se realiza tomografía que evidencia opacidades en vidrio esmerilado de distribución difusa y engrosamiento de septos, por lo que ante la sospecha de infección se realiza lavado broncoalveolar que revela líquido blanquecino evidenciando en la citología inclusiones en macrógrafos PAS positivo. Con el diagnóstico de proteinosis alveolar secundaria a enfermedad de base, se reinició tratamiento quimioterápico. Presentó progresión de infiltrados y mayor requerimiento de oxígeno falleciendo pocos días después. (AU)

Neumonía intersticial inducida por bloqueo androgénico máximo como tratamiento de cáncer de próstata avanzado: Report of one case / Fatal interstitial lung disease associated with maximum androgen blockade

Maximum androgen blockade is the standard endocrine treatment for advanced prostate cancer. Interstitial lung disease in different degrees of severity, with low mortality and excellent response to treatment may appear with its use. We report a 77 years old patient with advanced prostate cancer who developed severe and progressive respiratory failure associated to bilateral pulmonary infiltrates, attributed to the direct effect of maximum androgen blockade. Despite the therapeutic efforts, the patient died. Lung pathology revealed Usual Interstitial Pneumonia.

Linfoma intravascular, un desafio diagnóstico: Caso clínico / Intravascular lymphoma: Report of one case

Intravascular lymphoma is a rare subtype of extranodal diffuse large B-cell lymphoma characterized by clonal proliferation of lymphocytes inside of small and medium caliber vessels. Its incidence is estimated at one case per million. The clinical picture is very variable, but frequently has skin and central nervous system involvement. It is diagnosed by demonstrating pathological blood vessel infiltration by lymphoma cells. We report a 44 years old male presenting with fever, malaise and erythematous lesions in the abdominal wall. An abdominal wall biopsy showed dilated vascular vessels with atypical cells in their lumen, compatible with large B-cell intravascular lymphoma. He was treated with rituximab, cyclophosphamide, adriamycin, vincristine and prednisone and an autologous hematopoietic stem cell transplantation, achieving a complete remission that has lasted two years.

La displasia como factor pronóstico en los pólipos de colon y recto / Dysplasia as a prognostic factor in colon and rectal polyps

Introducción: La presencia de displasia en el tejido adenomatoso es el primer indicio de su potencialidad maligna y su correcta identificación permite determinar su gravedad y la conducta. Objetivos: Analizar la frecuencia, clasificación, conducta terapéutica, potencialidad maligna y seguimiento de los adenomas colorectales en relación con sus distintos grados de displasia. Pacientes y métodos: Fueron evaluados retrospectivamente 2380 pacientes a los que se les realizaron estudios endoscópicos del colon y/o recto. Se hallaron 307 casos (12,89 por ciento) de pólipos de colon y recto. Las displasias fueron clasificadas en de bajo y de alto grado. Según el grado de displasia se pautó el régimen de controles con videocolonoscopía. En los adenomas con displasia de bajo grado se controló al año y en los adenomas con displasia de alto grado cada 3 meses en el primer año. Resultados: Se hallaron 184 casos (59,93 por ciento) de pólipos únicos y 123 casos (40,06 por ciento) de pólipos múltiples. Correspondieron a adenomas 183 casos (59,6 por ciento). Entre ellos, 205 casos (66,77 por ciento) fueron sésiles y 102 casos (33,22 por ciento) fueron pediculados. Según el tamaño se los clasificó en pólipos menores de 1 cm (214 casos; 69,70 por ciento), pólipos entre 1 y 2cm (55 casos; 17,91 por ciento) y pólipos mayores de 2cm (38 casos; 12,37 por ciento). Según su histología fueron adenomas vellosos (49 casos; 15,96 por ciento). Las displasias fueron clasificadas en dos grupos: Displasias de Bajo Grado y Displasias de Alto Grado. Hubo 4 casos de carcinoma intramucoso (6,55 por ciento) a los cuales se les realizó polipectomía y 4 casos con áreas de carcinoma invasor que recibieron resección oncológica. Conclusiones: La categorización de los adenomas según los distintos grados de displasias y el seguimiento normatizado de las mismas mediante controles endoscópicos, evita la aparición de lesiones avanzadas.

Enfermedad de Bowen perianal / Perianal Bowen disease

Antecedentes: La Enfermedad de Bowen es un carcinoma intraepitelial, no queratinizado de células escamosas de la piel. La localización perineal es poco frecuente. Objetivos: Analizar su forma de presentación, el tratamiento implementado y los resultados obtenidos. Diseño: Trabajo retrospectivo. Población: Pacientes con Enfermedad de Bowen perianal asistidos en nuestro Hospital en los años 2000 y 2001. Método: En todos los casos el diagnóstico se realizó por biopsias de áreas condilomatosas; en 1 caso se realizó resección amplia de la lesión. Conclusiones: La escasa sintomatología de esta patología retrasan su diagnóstico. Ante su sospecha es fundamental el minucioso examen de toda la región anogenital y la biopsia de toda lesión sospechosa. En la actualidad el consenso general mantiene como tratamiento de elección a la resección ampliada. El curso clínico de la enfermedad de Bowen es relativamente benigno con un riesgo a desarrollar un carcinoma invasor que no supera el 2 al 6 por ciento. El seguimiento en el tiempo es imprescindible para prevenir recurrencias.