Idiopathic noncirrhotic portal hypertension: current perspectives

The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis

Total thyroidectomy associated to chemotherapy in primary squamous cell carcinoma of the thyroid

Primary squamous cell carcinoma of the thyroid (PSCCT) is a rare malignant disease with rapid fatal prognosis. The onset is generally characterized by sudden bilateral latero-cervical lymphadenopathy. The Authors report patient of 58-year-old who referred for evaluation of rapidly aggravating bilateral latero-cervical lymphadenopathy. The US highlighted the presence of a hypoechoic nodular lesion characterized by peri and intra-nodular vascularization. Multilayer CT showed diffused involvement of mediastinal and bilateral latero-cervical lymph nodes, with no evidence of primary pulmonary neoplasia or elsewhere. The patient underwent total thyroidectomy. The peri-isthmic tissue was removed due to the presence of a small roundish formation, that was due to lymph node metastasis at histological examination. Histological diagnosis: PSCCT. The immunohistochemical panel of the thyroid lesion was indispensable for the differential diagnosis between PSCCT, medullary carcinoma, anaplastic carcinoma, and thyroid metastasis of neoplasia with unknown primitiveness. The patient underwent chemotherapeutic treatment with Carboplatin and Paclitaxel with modest improvement of dysphagia symptoms and reduction of 10-15% of the target lesions. The clinical course was characterized by loco-regional progression of the disease with exitus in 10 months after diagnosis. Survival and quality of life after surgical therapy and chemotherapy were like that of patients undergoing only chemotherapy. Due to the extreme rarity of the neoplasia, 60 cases described in Literature, no exclusive guidelines are reported for PSCCT. More extensive case studies are needed to evaluate the effects of total thyroidectomy with intent R0/R1 on improving survival and quality of life of patients with PSCCT

Posterior cricoid region fluoroscopic findings: the posterior cricoid plication.

The region posterior to the cricoid cartilage is challenging to assess fluoroscopically. The purpose of this investigation is to critically evaluate the posterior cricoid (PC) region on fluoroscopy and describe patterns of common findings. This was a case control study. All fluoroscopic swallowing studies performed between June 16, 2009, and February 9, 2010, were reviewed for features seen in the PC region. These findings were categorized into distinct patterns and compared to fluoroscopic studies performed in a cohort of normal volunteers. Two hundred patient studies and 149 healthy volunteer studies were reviewed. The mean age of the referred patient cohort and the volunteer cohort was 57 years (±19) and 61 years (±16), respectively (p > 0.05). The patient cohort was 53% male and the control cohort was 56% female (p > 0.05). Four groups were identified. Pharyngoesophageal webs were seen in 7% (10/149) of controls and 14% (28/200) of patients (p = 0.03). A PC arch impression was seen in 16% of patients (32/200) and controls (24/149) (p = 1). A PC plication was demonstrated in 23% (34/149) of controls and 30% (60/200) of patients (p = 0.13). No distinctive PC region findings were seen in 54% (81/149) of controls and 42% (84/200) of referred patients (p = 0.02). Four patients (2%) had both a web and a PC plication. Four categories of PC region findings were identified (unremarkable PC region, web, PC arch impression, and PC plication). Both patients referred for swallowing studies and healthy volunteers demonstrated esophageal webs, PC arch impressions, and PC plications. Only webs were more common in patients than in control subjects (p = 0.03). The PC impression and PC plication are likely to represent normal variants that may be identified on fluoroscopic swallow studies

Esophageal Squamous Cell Carcinoma in Cats

Background: Esophageal neoplasms are rarely reported in cats. The frequency rate is less than 0.5% and those neoplasms are usually malignant. Esophageal squamous cell carcinoma (SCC) is an idiopathic epithelial neoplasm, invasive and metastatic that can induce partial or complete obstruction of the esophageal lumen. There is no breed or sex predisposition, and it is more common in cats over 8-years-old. Esophageal SCC is more frequent in the middle third of the esophagus. The prognosis is poor, as the cats are usually diagnosed at an advanced stage. This report aims to describe clinical, endoscopic, radiographic, and pathological features of two cases of esophageal squamous cell carcinoma in cats.Cases: A 11-year-old neutered male cat presenting regurgitation, weight loss, anorexia and dyspnea was referred to veterinary internal medicine care. Simple and contrast-enhanced radiographic images of the cervical and thoracic regions showed an alveolar pattern in the cranial lung lobes and signs of esophageal lumen irregularity and dilatation in the mediastinum topography. The upper digestive endoscopy showed a dilated esophageal lumen, and an irregular mass was observed in the thoracic esophagus involving the entire esophageal circumference. Biopsy fragments were collected, and the histopathological result was compatible with squamous cell carcinoma. The second case was a 10-year-old neutered male cat presenting hyporexia, regurgitation, dyspnea, tachypnea, and abnormal breath sounds. The ultrasound of the chest showed 3 amorphous hypoechogenic and heterogeneous areas in the right and left hemithorax between parietal and visceral pleura. The cytological examination was compatible with a malignant epithelial tumor. The patient died 3 months after the onset of clinical signs. At gross exam, it was observed a friable, irregular, and ulcerated mass of 5.0 x 3.0 cm in the middle third of the esophagus. Metastatic foci in the lungs and liver were also observed. The histopathological diagnosis was esophageal squamous cell carcinoma, with metastases to liver and lungs. Microscopically, in both cases, were seen aproliferation of polyhedral epithelial cells in the mucosa, arranged in nests or trabeculae with central keratinization. These cells presented oval to rounded nuclei, loose chromatin, prominent nucleolus, and abundant eosinophilic cytoplasm, with marked anisocytosis and anisokaryosis, supported by a thin fibrovascular stroma. In the second cat the neoplastic cells infiltrated the esophageal submucosa, including lymphatic vessels and muscle layer. Lung and liver metastases from theSCC had a cellular pattern similar to the primary neoplasm.Discussion: Esophageal squamous cell carcinoma is extremely rare in cats. The SCC begins in the squamous layer of the mucosa and can infiltrate the muscular layer or protrude into the esophageal lumen, leading to clinical signs, as seen in these 2 cats. The differential diagnoses for esophageal SCC include foreign bodies, esophageal strictures, and infiltrative or compressive non-esophageal tumors. Although uncommon, esophageal tumors should be considered when evaluatingelderly cats with regurgitation and weight loss. The diagnosis of esophageal SCC was confirmed by histopathological findings collected endoscopically or during necropsy. As noted in both cases, the prognosis of SCC is generally unfavorable, usually due to the difficulty in treatment and diagnosis in a late stage of the disease.Keywords: feline, esophagus, neoplasms, metastasis, cancer

Nonesophageal Eosinophilic Gastrointestinal Disorders: Clinical Care and Future Directions

Eosinophilic gastrointestinal disorders are a set of conditions with a wide range of clinical manifestations and treatment modalities. The disorders are suspected to result from an abnormal inflammatory response to allergen(s), and individuals may develop a relapsing or chronic disease, if the allergen is not eliminated. Mechanisms of disease pathogenesis, including the humoral immune response, need to be fully elucidated. A variety of therapies are used, though there is a lack of well-defined randomized, prospective studies. Other therapeutic options are needed as the current treatments have potential concerns; elimination diets may impair a child’s quality of life, and corticosteroids have adverse risks with long-term use. We review what is known about non-esophageal eosinophilic gastrointestinal disorders, and discuss research investigations which need to be conducted to facilitate diagnosis and enhance treatment methods