X-linked Inhibitor of Apoptosis Complicated by Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) and Granulomatous Hepatitis.

The X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency characterized by Epstein-Barr virus (EBV)-driven hemophagocytic lymphohistiocytosis (HLH), splenomegaly, and colitis. Here, we present, for the first time, granulomatous hepatitis and granulomatous and lymphocytic interstitial lung disease (GLILD) as manifestations of XIAP deficiency. We report successful treatment of GLILD in XIAP deficiency with rituximab and azathioprine and discuss the role of XIAP deficiency in immune dysregulation.

Follicular dendritic cell tumour/sarcoma: a commonly misdiagnosed tumour in the thorax.

AIMS: Follicular dendritic cell sarcoma is a rare tumour reported to occur occasionally in association with the hyaline-vascular type of Castleman's disease (HVCD). Most cases arise in lymph nodes, although extranodal presentation is described. METHODS AND RESULTS: Clinical, radiological and histological characteristics, including diagnosis on pre-resection material, were assessed in seven intrathoracic cases from five males and two females with a median age of 38 years. Clinical symptoms were related to mass location, six cases presenting within central and/or posterior mediastinal compartments and one within the lungs. Positron emission tomography-computed tomography demonstrated marked fluoro-deoxy-glucose avidity and the prominent vessels traversing the lesions. Four of six cases (67%) were misdiagnosed initially. HVCD was present in three cases. Two cases with high mitotic rates recurred after resection. All were positive for at least one of the follicular dendritic cell markers (CD21, CD35 and CD23). Six of seven cases (86%) show cyclin D1 expression ranging from 5% to 90%. CONCLUSIONS: Follicular dendritic cell sarcoma is often misdiagnosed on biopsy and pathologists need to be aware of the tumour to request the relevant immunohistochemistry, especially in masses presenting in the central/posterior mediastinum with high vascularity and standardized uptake values. Background HVCD appears more common than previously thought.

The reproducibility and predictive value on outcome of renal biopsies from expanded criteria donors.

Reproducibility and predictive value on outcome are the main criteria to evaluate the utility of histological scores. Here we analyze the reproducibility of donor biopsy assessment by different on-call pathologists and the retrospective evaluation by a single renal pathologist blinded to clinical outcomes. We also evaluate the predictive value on graft outcome of both evaluations. A biopsy was performed in donors with any of the following: age≥55 years, hypertension, diabetes, creatinine>1.5 mg/dl, or stroke. Glomerulosclerosis, interstitial fibrosis, tubular atrophy, intimal thickening, and arteriolar hyalinosis evaluated according to the Banff criteria were added to obtain a chronic score. Biopsies were classified as mild (≥3), intermediate (4-5), or advanced (6-7) damage, and unacceptable (≥8) for transplantation of 127 kidneys biopsied. Weighted κ value between both readings was 0.41 (95% CI: 0.28-0.54). Evaluation of biopsies by the renal pathologist was significantly and independently associated with estimated 12-month glomerular filtration rate and a significant composite outcome variable, including death-censored graft survival and time to reach an estimated glomerular filtration rate<30 ml/min per 1.73 m2. Thus, there was no association between readings of on-call pathologists and outcome. The lack of association between histological scores obtained by the on-call pathologists and graft outcome suggests that a specific training on renal pathology is recommended to optimize the use of kidneys retrieved from expanded criteria donors.

[Krukenberg's tumor as the first clinical manifestation of fibrolamellar hepatocarcinoma]. / Tumor de Krukenberg como manifestación inicial de hepatocarcinoma fibrolamelar.

Krukenberg's tumor refers to unilateral or bilateral ovarian metastatic tumors whose origin may be known or unknown. The incidence of this type of tumor is difficult to evaluate but may represent between 3% and 8% of ovarian tumors. In most cases, the primary tumor is identified and is usually digestive (basically gastric or colorectal). There are some references to primary hepatic tumors (gall bladder tumor and hepatocarcinoma) but none to fibrolamellar hepatocarcinoma as the primary tumor. We present the case of a 45-year-old woman who presented with lower hemiabdominal pain and who was diagnosed with Krukenberg's tumor of hepatic origin. Although ovarian involvement in primary digestive tumors is possible, hepatic origin is rare.