Fibromatosis agresiva de cabeza y cuello en la edad pediátrica. Un caso clínico y revisión de la literatura / Aggressive fibromatosis of the head and neck in pediatric age. A case report and review of the literature

Introducción. El tumor desmoide es una neoplasia mesenquimal benigna rara en la edad pediátrica, originada principalmente en el tejido conectivo de músculos y aponeurosis. Con frecuencia, es mal diagnosticada como fibromas, procesos reactivos o fibrosarcomas de bajo grado. Se caracteriza por un crecimiento lento, localmente agresiva y con una alta tasa de recurrencia sin capacidad de metastatizar. Material y método. Niña de 7 años con tumoración para mandibular derecha de un año de evolución. Se caracterizaba por un crecimiento lento e indoloro, trismus y latero-desviación mandibular a la izquierda durante la apertura oral. Las pruebas de imagen mostraban tumor departes blandas en región maseterina con una reacción perióstica en el cuerpo mandibular. La biopsia informó de tumor desmoide. Resultados. Se realizó abordaje de Risdon para la exéresis tumoral más Split de cortical externa de cuerpo mandibular sin poder preservarla rama marginal del VII par craneal (PC). La anatomía patológica informó de fibromatosis agresiva sin afectación ósea. Actualmente presenta parálisis marginal y está libre de enfermedad. Conclusiones. Los tumores desmoides de cabeza y cuello son difíciles de tratar debido a la proximidad o implicación de estructuras vitales, su naturaleza infiltrativa y la tendencia a la recurrencia local. La cirugía primaria con márgenes quirúrgicos negativos es el tratamiento de elección. Sin embargo, en muchos casos esto implica cirugías (..) (AU)

Background. Desmoid tumor is a rare benign mesenchymal neoplasmin children primarily originated in the muscle connective tissue, fascial sheaths, and musculoaponeurotic structures. It is often misdiagnosed as fibroids, reactive processes or low-grade fibro sarcomas. It is characterized by slow growth, locally aggressive nature, high recurrence rate without metastasize capacity. Material and methods. 7 year old girl with right (..) (AU)

[Aggressive fibromatosis of the head and neck in pediatric age. A case report and review of the literature]. / Fibromatosis agresiva de cabeza y cuello en la edad pediátrica. Un caso clínico y revisión de la literatura.

BACKGROUND: Desmoid tumor is a rare benign mesenchymal neoplasm in children primarily originated in the muscle connective tissue, fascial sheaths, and musculoaponeurotic structures. It is often misdiagnosed as fibroids, reactive processes or low-grade fibrosarcomas. It is characterized by slow growth, locally aggressive nature, high recurrence rate without metastasize capacity. MATERIAL AND METHODS: 7 year old girl with right paramandibular tumor, a year of evolution. It was characterized by slow and painless growth, trismus and mandibular latero-deviation to the left during mouth opening. Imaging tests showed soft parts tumor in masseteric region with a periosteal reaction in the mandibular body. The biopsy reported a desmoid tumor. RESULTS: Risdon approach was performed for tumor resection and external cortical Split mandibular body, without preserving the marginal branch of CP VII. The pathology reports of aggressive fibromatosis without bone involvement. It currently presents marginal paralysis and free-disease. CONCLUSIONS: Desmoid tumors of head and neck are difficult to treat because of the proximity or involvement in vital structures, infiltrative nature and tendency to local recurrence. Primary surgery with negative surgical margins is the treatment of choice. However, in many cases this involves disfiguring surgery. Therefore, in these cases and in those surgical margins affections, the use of chemotherapy/non-cytotoxic drugs can be an alternative. Because they are low incidence tumors prospective multicenter studies are needed to clarify the role of adjuvant treatment in this tumor.

Leiomiosarcoma intraóseo mandibular / Mandibular intraosseous leiomyosarcoma

Los leiomiosarcomas son neoplasias malignas mesenquimalesderivadas del músculo liso. Los leiomiosarcomas intraóseos mandibularesprimarios son muy raros. Se cree que proceden de la musculatura lisa dela pared de los vasos sanguíneos. Pueden presentarse a cualquier edad, sinninguna predilección y afectan preferentemente a hombres, con una relaciónhombre-mujer de 2:1. La excisión quirúrgica completa está asociadacon una menor recidiva y mayor supervivencia. La radioterapia es efectivaen el tratamiento de la enfermedad microscópica residual. Presentamosel caso de un leiomiosarcoma intraóseo mandibular(AU)

Leiomyosarcomas are malignant mesenchymalneoplasms of smooth muscle differentiation. Leiomyosarcomasoccurring in the jawbones are exceedingly rare, andare believed to arise from the musculature of the tunica ofblood vessels. Intraoral cases may present at any age withno predilection for any age group. They occur predominantlyin males, with a male to female ratio of approximately 2:1.Complete surgical excision is associated with low recurrenceand longer survival. Radiotherapy is effective in the treatmentof microscopic disease(AU)

An approach to the rational use of steatotic donor livers in liver transplantation.

Steatosis or fatty change is a common finding in donor liver biopsies during liver transplantation, and seems to be more frequent than in the general population. Fat can be stored in hepatocytes within macrovacuoles (macrosteatosis) or microvacuoles (microsteatosis), with different degrees of severity. Higher degrees of both macro and microsteatosis may increase the severity of the ischemia-reperfusion lesion producing an initial poor function in the recipient. Different pathogenic mechanisms have been investigated. However, only severe macrosteatotic (> 60%) grafts have been associated with primary non-function, and are universally rejected for transplantation. While donor livers with any severity of microsteatosis do not influence recipient survival and can be safely implanted, donor livers with moderate to severe macrosteatosis (30-60%) have a relative risk of primary non-function and should be considered for transplantation in the absence of other known risk factors. A protocol with a rational use of these steatotic livers is suggested.

[Primary graft failure as the result of unsuspected severe steatosis in the donor liver]. / Fallo primario del injerto como resultado de esteatosis severa no sospechada en el hígado donante.

Primary non-function is an serious complication after orthotopic liver transplantation. A relationship between the severity of fatty infiltration in the donor liver and immediate graft function has been described. We present two cases of primary non-function, attributable to the presence of severe steatosis in the donor liver. They were two recipients with a deteriorated clinical status before liver transplantation. In both cases, liver fatty infiltration was not suspected because of the macroscopic aspect of the donor liver at procurement. In time 0 biopsies, hematoxyllin-eosin stained sections evaluated the degree of severity of the fatty infiltration as moderate (30-60% of hepatocytes involved), while the Sudan stained sections revealed more degree of severity (more than 60%). We believe that in case of moderate steatosis in liver donor the graft may be considered when it is not associated to another known risk factors as poor medical status of recipient and retransplantation.

Hepatic steatosis in liver transplant donors: common feature of donor population?

Fatty change in donor livers is a risk factor for poor function after orthotopic liver transplantation. Various prevalences of steatosis have been reported in time 0 biopsies. The aim of this research was to determine, in a longitudinal study, the degree (percent of hepatocytes involved) and type (size of vacuoles) of fatty change shown by various histologic techniques. Four staining methods were used on sections from three liver wedge biopsies--at liver procurement, at the back-table, and after reperfusion--from 83 consecutive donor livers. Results in Sudan III-stained (SS) sections showed the greatest sensitivity (87.1%), negative predictive value (91.8%), and agreement rate (k = 0.77) when compared with results in thin (1 micron) plastic-embedded toluidine blue-stained (TBS) sections. High-grade steatosis (> 30% steatotic hepatocytes) was identified in 49.4% of SS sections, 46.9% of TBS sections, 38.5% of frozen hematoxylin-eosin (H&E)-stained sections, and 20.7% of deparaffinated H&E-stained sections. Microscopic observations disclosed two types of steatotic pattern: (1) A predominantly small-droplet lipid vacuolzation (high-grade microsteatosis), similar to the steatosis associated with Reye syndrome, was seen in 29% of SS sections and 25% of TBS sections--approximately one-fourth of grafts; and (2) a combined pattern of large and small fat drops (high-grade macromicrosteatosis) was seen in 20% of SS sections and 22% of TBS sections. We concluded that moderate to severe steatosis is a frequent finding in donor livers. The difficulty in detecting lipidic microvacuoles in H&E-stained sections may be the reason for underestimating the grade of fatty change or even for diagnosing as normal some biopsies with high-grade microsteatosis.

[Subclinical Crohn disease in acquired immunodeficiency syndrome]. / Enfermedad de Crohn subclínica en el síndrome de inmunodeficiencia adquirida.

We report the case of a patient suffering from acquired immunodeficiency syndrome and hepatitis B and D virus-related cirrhosis of the liver who was diagnosed as subclinical Crohn's disease. We attribute this clinical course to abnormality of intestinal immune system induced by the human immunodeficiency virus. Concomitant hepatitis B and D virus infection may have contributed. This observation supports the hypothesis of helper-inducer T cells (CD4 T cells) having a critical role in the immunopathogenesis of Crohn's disease and its clinical expression.

[Non alcoholic steatohepatitis. Mid-term clinical and histologic course in 10 patients]. / Esteatohepatitis no alcohólica. Evolución clínica e histológica a medio plazo de diez pacientes.

BACKGROUND: The progressive character of the hepatic lesions in nonalcoholic steatohepatitis (NASH) has not been well established. In the present study, the clinical and histological course of this condition was evaluated at medium term. METHODS: Then patients (6 females and 4 males) with NASH, in a non-cirrhotic stage at the time of diagnosis, were followed up during 58 +/- 7 months (range 24 to 88 months). RESULTS: Nine patients were obese, but a significant reduction of body weight was found at the end of the study (p = 0.0072). Other clinical, physical or laboratory changes were not found, although in two cases hepatic biochemical tests were within normal limits when the follow-up biopsy was performed. Changes in the characteristic hepatic features were also absent, although fat infiltrates disappeared in three cases. Six patients had increased fibrosis and a progression of the hepatic architectural distortion; four reached the stage of cirrhosis. The evolution of the hepatic lesion correlated with the interval between diagnostic and follow-up biopsies (r = 0.69; p less than 0.05) and with the reduction in body weight (r = 0.64; p less than 0.05). CONCLUSIONS: In most cases, NASH results in a progressive hepatic distortion with can end in cirrhosis, although the change is slow and silent.

Hepatic myelolipoma: new case and review of the literature.

We present a case of hepatic myelolipoma that was treated by performing a right hepatic lobectomy. These tumors are very uncommon and present great problems for the pre-operative diagnostic evaluation. Currently, the diagnosis is essentially histopathological with a definitive diagnosis being made by immunocytochemical staining. We have found no reports on this type of tumor in the Spanish literature; a review of the published cases confirms our observations and conclusions regarding hepatic myelolipoma.

[Esophageal tuberculosis. Presentation of a case and review of the literature]. / Tuberculosis esofágica. Presentación de un caso y revisión de la literatura.

We describe the case of a 63-year-old woman who had recently developed dysphagia. Oral endoscopy revealed an ulcerated fungating lesion in the middle third of the esophagus. The histologic examination showed granulomas with caseous central necrosis, and the culture in a Lowenstein medium produced M. tuberculosis. There were mediastinic adenopathies, but no other organic involvement with this mycobacterium was demonstrated. We review the other 21 cases reported in the English and Spanish literature over the past ten years.

[Ménétrier disease. Clinical and histological study of 3 cases]. / Enfermedad de Ménétrier. Estudio clínico e histológico de tres casos.

Three cases of Ménétrier's disease diagnosed and treated in our hospital during the last 15 years are presented. We describe the clinical manifestations as well as the pathological findings; one of the cases was complicated by carcinomatous degeneration. On the basis of our experience, we briefly review the controversial clinical, diagnostic and therapeutic aspects of this rare condition.

[Acute submassive cholestatic hepatitis after treatment with carbimazole. Presentation of a new case and review of the literature]. / Hepatitis colestásica aguda submasiva tras tratamiento con carbimazol. Aportación de un caso y revisión de la literatura.

Carbimazole, an antithyroid agent of the thioimidazole group, can induce unpredictable liver alterations, presumably by hypersensitivity. We describe the case of a hyperthyroid woman who suffered acute submassive cholestatic hepatitis in the course of treatment with this drug. We also review the other seven cases of carbimazole hepatotoxicity previously communicated in the literature.