Treatment and prognostic implications of strong PD-L1 expression in primary hepatic sarcomatoid carcinoma.

Primary hepatic sarcomatoid carcinoma (HSC) is an extremely rare and aggressive subtype of primary liver cancer. HSC has uncertain pathogenesis and dismal prognosis with overall survival of only 8.3 months. The molecular alterations of HSC are also not well understood. In this study, the authors describe a patient who presented with a large liver mass. The patient underwent complete surgical resection and histological examination demonstrated HSC, infiltrating the stomach. PD-L1 was strongly positive in the tumor cells. The patient was started on anti-PD-L1 immunotherapy postsurgery and is doing well 15 months after surgical resection. Tumor whole exome sequencing revealed genetic alterations in TP53, NF2 and MAGEC3 genes, indicating their potential role in tumor development.

Primary sarcomatoid cancer of the liver is a rare type of severe cancer that generally has a very poor prognosis. People diagnosed with primary sarcomatoid of the liver normally survive for only a few months. Surgery is not very effective in treating this type of cancer and recurrence is common even after complete removal. In this paper, the authors report a patient who presented to them with a large liver tumor. The patient underwent operation and the tumor was completely removed from the liver. Pathological testing of the tumor revealed it was severe primary sarcomatoid liver cancer. The patient was started on an immunotherapy treatment. The treatment enhanced the ability of the body's immune system to fight cancer. The patient is doing well 15 months after the operation, which might mean that this type of immunotherapy treatment after surgery helps prolong the life of people diagnosed with primary sarcomatoid cancer of the liver.

Pathologic and Immunophenotypic Characterization of Syncytial Giant Cell Variant of Pediatric Hepatocellular Carcinoma. A Distinct Subtype.

INTRODUCTION: Hepatocellular carcinoma (HCC) in pediatrics has a uniformly poor prognosis. Complete surgical resection or liver transplantation remain the only curative options. In contrast to adult HCC, literature on pediatric HCC is sparse and a majority of the distinct subtypes are undefined with regards to their histology, immunohistochemistry and prognosis. CASE REPORT: Two infants, one with biliary atresia and another with transaldolase deficiency, underwent living donor liver transplants. Explant-liver histopathology revealed tumor with diffuse neoplastic syncytial giant cell pattern. Immunophenotypic characterization highlighted expression of epithelial cell adhesion molecule, alpha fetoprotein and metallothionein. CONCLUSION: HCC with syncytial giant cells variant can occur in infants with underlying liver disease, specifically in our experience, with biliary atresia and another with transaldolase deficiency.

Newly proposed classification of celiac artery variations based on embryology and correlation with computed tomography angiography.

Purpose: We studied the prevalence of celiac trunk and its anatomical variations on diagnostic computed tomography angiography (CTA) studies and have proposed a new classification to define the celiac artery (CA) variations based on embryology. Material and methods: We retrospectively assessed the celiac trunk variations in 1113 patients who came to our department for diagnostic CTA for liver and renal donor workup. The patient data were acquired from the Picture Archiving and Communication System of our institutions. We analysed the celiac trunk's origin and branching pattern, including the superior mesenteric artery (SMA) and inferior phrenic artery (IPA). Results: We evaluated the CTA studies of 1050 patients. A normal trifurcation pattern, the most common type, was observed in 39% of cases. Variation with CA + left IPA was the most common subtype. Other variations noted in the study and their incidences are listed in the table below. We attempted to propose a new classification based on embryo-logy, which comprises 6 main types and their subtypes. We also analysed previous studies from the literature, including cadaveric, post-mortem, CTA, and digital subtraction angiography studies and compared them with the present study. Conclusions: Because variations of CA classifications reported to date do not encompass all CA branching pattern variants, we have proposed a new classification that incorporates most of the variants. We reiterate the clinical importance of anatomical variants of CA, IPA, and SMA in surgical and interventional radiology procedures.

Uncommon presentation of a recurrent diaphragmatic hernia after pediatric liver transplantation.

DH is a rare but well-recognized complication of PLT. However, a recurrent DH in the setting of PLT has not been reported. We report the case of a child who had previously undergone a DH repair early after PLT and presented more than two years later with atypical findings of severe sepsis and a tender abdominal swelling.

Adhesive capsulitis: MRI correlation with clinical stages and proposal of MRI staging.

OBJECTIVE: The purpose of this study was to correlate the magnetic resonance imaging (MRI) findings of adhesive capsulitis with clinical stages and thereby propose a MR staging system. MATERIALS AND METHODS: This study consisted of 74 patients with clinically diagnosed adhesive capsulitis. The edema of the inferior glenohumeral ligament (IGHL), pericapsular edema, thickness of anterior band of IGHL, axillary pouch, thickness of coracohumeral ligament, and obliteration of fat in the subcoracoid triangle were evaluated by MRI. RESULTS: Thickening of the anterior band of IGHL showed most significant correlation with the clinical stages. The distribution of edema of IGHL and pericapsular edema also showed significant correlation with the clinical stages of adhesive capsulitis. Pericapsular edema and IGHL edema was not observed in stage IV. CONCLUSION: MR is a useful tool for evaluation and prediction of clinical stage of adhesive capsulitis.

Role of 3D SPACE sequence and susceptibility weighted imaging in the evaluation of hydrocephalus and treatment-oriented refined classification of hydrocephalus.

OBJECTIVE: The aim of our study was to evaluate the diagnostic utility of three-dimensional sampling perfection with application optimized contrast using different flip angle evolution (3D SPACE) sequence and Susceptibility Weighted Imaging (SWI) in hydrocephalus and to propose a refined definition and classification of hydrocephalus with relevance to the selection of treatment option. MATERIALS AND METHODS: A prospective study of 109 patients with hydrocephalus was performed with magnetic resonance imaging (MRI) brain using standardized institutional sequences along with additional sequences 3D SPACE and SWI. The images were independently read by two senior neuroradiologists and the etiopathogenesis of hydrocephalus was arrived by consensus. RESULTS: With conventional sequences, 46 out of 109 patients of hydrocephalus were diagnosed as obstructive of which 21 patients showed direct signs of obstruction and 25 showed indirect signs. In the remaining 63 patients of communicating hydrocephalus, cause could not be found out in 41 patients. Whereas with 3D SPACE sequence, 88 patients were diagnosed as obstructive hydrocephalus in which all of them showed direct signs of obstruction and 21 patients were diagnosed as communicating hydrocephalus. By including SWI, we found out hemorrhage causing intraventricular obstruction in three patients and hemorrhage at various sites in 24 other patients. With these findings, we have classified the hydrocephalus into communicating and noncommunicating, with latter divided into intraventricular and extraventricular obstruction, which is very well pertaining to the selection of surgical option. CONCLUSION: We strongly suggest to include 3D SPACE and SWI sequences in the set of routine MRI sequences, as they are powerful diagnostic tools and offer complementary information regarding the precise evaluation of the etiopathogenesis of hydrocephalus and have an effective impact in selecting the mode of management.