ABSTRACT
Cicatricial pemphigoid (CP) is a rare blistering autoimmune disease. Esophageal involvement occurs in widespread disease and rarely appears as the only affected organ. We report a 67-year-old Caucasian female with esophageal dysphagia and weight loss. Several oral panendoscopies showed multiple exudative ulcerations with fibrin and webs in mid- and proximal esophagus and a peeling mucosa. There were no lesions in other organs. We established the diagnosis performing a direct immunofluorescence (DIF), demonstrating IgG3 and complement deposition along the basement membrane. As initial treatment the patient received prednisone 60 mg and 1 gr twice daily of mycophenolate mofetil (MMF) as a steroid-sparing agent due to its lower toxicity and its selective mechanism of action. Six months later there was a significant clinical improvement and the esophageal ulcerations had disappeared, developing cicatricial fibrous rings, although no stenosis was present. Four years later, the patient remains asymptomatic with a low maintenance dose of MMF.
ABSTRACT
Retrorectal cystic hamartomas (tailgut cysts) are rare congenital lesions thought to arise from remnants of the embryonic postanal gut. They predominantly occur as asymptomatic retrorectal multicystic masses in women. The treatment of choice is by complete surgical excision. The most important complications of these cysts are infection with a secondary fistula and malignant degeneration. The differential diagnosis includes a wide variety of conditions that occur in the retrorectal space. In this article, 3 cases showing different surgical technical aspects of treatment are presented. In addition, the aetiopathogenic features and histopathological appearance, clinical presentation and complications, imaging features and differential diagnosis of tailgut cysts are described.
Subject(s)
Cysts , Hamartoma , Rectal Diseases , Adult , Cysts/diagnosis , Cysts/diagnostic imaging , Cysts/surgery , Diagnosis, Differential , Female , Follow-Up Studies , Hamartoma/diagnosis , Hamartoma/diagnostic imaging , Hamartoma/surgery , Humans , Magnetic Resonance Imaging , Middle Aged , Rectal Diseases/diagnosis , Rectal Diseases/diagnostic imaging , Rectal Diseases/surgery , Sacrococcygeal Region , Time Factors , Tomography, X-Ray Computed , UltrasonographyABSTRACT
Los hamartomas quísticos retrorrectales (tailgut cysts) son tumores congénitos poco frecuentes derivados de remanentes embrionarios postanales del intestino. La mayoría de los hamartomas quísticos son multiquísticos y aparecen como masas asintomáticas en mujeres de mediana edad. El tratamiento de elección es la extirpación completa. Las complicaciones más frecuentes son la infección y el desarrollo de fístulas cutáneas y la degeneración maligna. El diagnóstico diferencial incluye una extensa variedad de patologías que pueden existir en el espacio retrorrectal. En este artículo presentamos 3 pacientes con hamartomas quísticos y realizamos una revisión de su etiopatogenia, las manifestaciones clínicas, las técnicas de diagnóstico, sus complicaciones y los diagnósticos diferenciales. Asimismo, se discuten las diferentes técnicas quirúrgicas posibles para su abordaje quirúrgico (AU)
Retrorectal cystic hamartomas (tailgut cysts) are rare congenital lesions thought to arise from remnants of the embryonic postanal gut. They predominantly occur as asymptomatic retrorectal multicystic masses in women. The treatment of choice is by complete surgical excision. The most important complications of these cysts are infection with a secondary fistula and malignant degeneration. The differential diagnosis includes a wide variety of conditions that occur in the retrorectal space. In this article, 3 cases showing different surgical technical aspects of treatment are presented. In addition, the aetiopathogenic features and histopathological appearance, clinical presentation and complications, imaging features and differential diagnosis of tailgut cysts are described (AU)
Subject(s)
Female , Adult , Humans , Hamartoma/surgery , Rectal Neoplasms/surgery , Hamartoma/congenital , Hamartoma/complications , Hamartoma/diagnosis , Cutaneous Fistula/etiology , Diagnosis, Differential , Sacrum/surgery , Constipation/etiology , Rectal Neoplasms/congenital , Rectal Neoplasms/diagnosisABSTRACT
UNLABELLED: This study was undertaken to evaluate an image processing method for assessing liver fibrosis in conventional computed tomography (CT) scans in patients with chronic hepatitis C. Two cohorts (designated "estimation," n = 34; and "validation," n = 107) of chronic hepatitis C patients were assessed using digitized conventional helical CT. Weighted CT mean fibrosis (Fibro-CT) was calculated as a nonlinear weighted mean F-score for each sample. Fibrosis was defined according to Scheuer on the F0 to F4 scale by 2 pathologists blinded regarding the Fibro-CT data. Fibrosis according to Fibro-CT correlated with histology-determined fibrosis (r = 0.69; P < 0.001) and with increasing F-stage: F0 = 0.23 +/- 0.39; F1 = 0.90 +/- 0.99; F2 = 1.41 +/- 0.94; F3 = 2.79 +/- 0.55; F4 = 3.15 +/- 0.35 [analysis of variance: P < 0.0001). The receiver operating characteristics curve to diagnose significant fibrosis (>/=F2) was 0.83; 95% confidence interval (95%CI), 0.75 to 0.91; and, to diagnose advanced fibrosis (>/=F3), was 0.86, 95%CI: 0.80 to 0.93. The correlation between Fibro-CT and fibrosis was higher in patients with homogeneous distribution of fibrosis than in patients with heterogeneous distribution (r = 0.77 versus r = 0.43; P < 0.05). CONCLUSION: Optical digital analysis of CT images of the liver is effective in determining the stage and distribution of liver fibrosis in chronic hepatitis C. In patients with homogeneous fibrosis distribution, the correlation between Fibro-CT and histology was better than in patients with heterogeneous distribution. Fibro-CT is a simple to use, readily available, and useful method for the diagnosis of fibrosis in patients with chronic hepatitis C.
Subject(s)
Hepatitis C, Chronic/complications , Image Processing, Computer-Assisted/methods , Liver Cirrhosis/diagnostic imaging , Tomography, X-Ray Computed/methods , Adult , Aged , Cohort Studies , Female , Hepatitis C, Chronic/pathology , Humans , Liver Cirrhosis/pathology , Liver Cirrhosis/virology , Male , Middle Aged , PrognosisABSTRACT
Colorectal cancer is one of the best studied of all malignant diseases in terms of genetics and/or molecular prognostic factors. These factors, and relationships with prognosis, may have important implications especially in the design of surgical and adjuvant chemo-radiotherapy options. However, the true prognostic significance of all known factors has yet to be realised. We have reviewed the literature with specific focus on the role of molecular markers involved in prognosis and the prediction of response to adjuvant treatment.
Subject(s)
Colorectal Neoplasms/genetics , Colorectal Neoplasms/therapy , Apoptosis/genetics , Biomarkers, Tumor/analysis , Combined Modality Therapy , Humans , Mutation , Oncogenes , PrognosisABSTRACT
El cáncer colorrectal es uno de los tumores mejor conocidos de todas las enfermedades malignas en términos genéticos y/o moleculares. Su conocimiento y su relación con el pronóstico puede tener importantes implicaciones, fundamentalmente en el diseño de estrategias quirúrgicas y de quimiorradioterapia adyuvante. Sin embargo, aún desconocemos el verdadero significado pronóstico de todos los factores hasta ahora estudiados. En este trabajo hemos revisado la literatura acerca del papel específico de los marcadores moleculares en cáncer colorrectal y su probable respuesta al tratamiento
Colorectal cancer is one of the best studied of all malignant diseases in terms of genetics and/or molecular prognostic factors. These factors, and relationships with prognosis, may have important implications especially in the design of surgical and adjuvant chemo-radiotherapy options. However, the true prognostic significance of all known factors has yet to be realised. We have reviewed the literature with specific focus on the role of molecular markers involved in prognosis and the prediction of response to adjuvant treatment
