Von Hippel-Lindau syndrome (VHL) is a familial neoplastic condition seen in approximately 1 in 36,000 live births. It is caused by germline mutations of the tumor suppressor gene VHL, located on the short arm of chromosome 3. While the majority of the affected individuals have a positive family history, up to 20% of cases arise from de novo mutations. VHL syndrome is characterized by the presence of benign and malignant tumors affecting the central nervous system, kidneys, adrenals, pancreas, and reproductive organs. Common manifestations include hemangioblastomas of the brain, spinal cord, and retina; pheochromocytoma and paraganglioma; renal cell carcinoma; pancreatic cysts and neuroendocrine tumors; and endolymphatic sac tumors. Diagnosis of VHL is prompted by clinical suspicion and confirmed by molecular testing. Management of VHL patients is complex and multidisciplinary. Routine genetic testing and surveillance using various diagnostic techniques are used to help monitor disease progression and implement treatment options. Despite recent advances in clinical diagnosis and management, life expectancy for VHL patients remains low at 40–52 years. This article provides an overview of the major clinical, histological, and radiological findings, as well as treatment modalities.

Intranodal palisaded myofibroblastoma is a rare benign primary mesenchymal neoplasm originating from differentiated smooth muscle cells and myofibroblasts. The precise etiology and pathogenesis has not been adequately explained as yet. Very few series and cases have been reported in the literature. Though inguinal region is the commonest site of this rare tumor, but the tumor at other diverse sites have been reported. Because of its rarity, it can be often misdiagnosed and confused with other disorders and more commonly with metastasis. We report an extensive review of literature about intranodal palisaded myofibroblastoma--its characteristics, presentations, features, and management.

In 2004, A new peculiar subtype of renal cell carcinoma, which later received the name of tubulocystic carcinoma (TCC-RC), was recognized. Though the tumor has distinct macroscopic, microscopic and immunohistochemical features, the tumor was previously considered to have some similarities to various other renal cancers. We did an extensive review of literature using PubMed and CrossRef, which yielded more than 80 cases reported from various parts of the world. We evaluated the epidemiology, tumor presentations, pathological characteristics, treatment, and outcome of TCC-RC.

Tubulocystic renal cell carcinoma of the kidney is a rare entity with less than one hundred cases reported so far. It was previously considered to have some similarities to various other renal cancers although this tumor has distinct macroscopic, microscopic and immuno-histochemical features. It is now a well-established entity in renal neoplastic pathology and has been recognized as a distinct entity in the 2012 Vancouver classification of renal tumors. This review aims to give an overview of tubulocystic renal cell carcinoma after extensive literature search using PubMed and CrossRef.

OBJECTIVE: We sought to determine the clinical and histopathological factors associated with intestinal hemorrhage and its correlation with clinical outcomes in neonates with surgical necrotizing enterocolitis (NEC). METHODS: A retrospective study compared clinical and histopathology information in neonates following surgical NEC with severe hemorrhage and those with mild/moderate hemorrhagic lesions seen on resected intestine pathology. RESULTS: The infants with severe hemorrhage (Grade 3-4, 81/148, 54.7%) had significantly lower exposure to antenatal steroids (52.5 % vs 76.9 % ; p = 0.004), had higher gestational age (28.5 weeks [7.14] vs. 26.58 [2.90]; p = 0.034), lost more bowel length (p = 0.045), had higher CRP levels at 2 weeks (p = 0.035), and had less intestinal failure ([30.3 % vs 52.5 %]; p = 0.014) than mild/moderate (Grade 0–2, 67/148, 45.2%) hemorrhage group. Those with severe hemorrhage had significantly higher mean inflammation score (2.67 [0.94] vs. 1.63 [0.92]; p = <0.001), higher necrosis scores (1.95 [1.28] vs. 1.49 [1.35]; p = 0.037), higher neovascularization (p = 0.01), higher fibroblasts (p = 0.023) and higher lymphocyte percentages up to 48 hours (p <  0.05) following NEC than mild/ moderate hemorrhage group. On multivariable regression, less exposure to antenatal steroids (OR 0.18 [95% CI 0.05–0.58]; p = 0.005), higher inflammation (OR 3.7 [95% CI 2.09–7.32]; p = 0.001), and lymphocyte count on the day of onset/24 hours following NEC (OR 1.06 [95% CI 1.02–1.11]; p = 0.005) were independently associated with a higher odd of severe intestinal hemorrhage. CONCLUSION: The surgical NEC infants with intestinal hemorrhage were less likely to have antenatal steroid exposure but had higher inflammation grade and lymphocyte counts following NEC onset on multivariable regression modeling.

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Objective: To determine clinical correlates of moderate to severe bronchopulmonary dysplasia (BPD) in preterm infants following surgical necrotizing enterocolitis (NEC). Methods: A retrospective, single-center cohort study comparing patients with moderate to severe BPD to patients with non/mild BPD among surgical NEC infants. BPD was defined by NIH 2001 consensus definition Results: Of 92 consecutive neonates with surgical NEC, 77% (71/92) had moderate/severe BPD and 22% (21/92) had non/mild BPD. The patent ductus arteriosus (PDA) was significantly higher in those developing moderate/severe BPD (67.6% [48/71]) than non/mild BPD (28.6% [6/21]; p=0.001). Postoperatively,infants with moderate/severe BPD had more severe AKI (67.6% [48/71] vs. 28.6% [6/21]; p=0.001), were intubated longer (40.5 days [IQR 12, 59] vs. 6 days [IQR 2, 13]; p<0.001), received more parenteral nutrition (109 days [IQR 77, 147] vs. 55 days [IQR 19, 70]; p<0.001), developed higher surgical morbidity (46.5% [33/71] vs. 14.3% [3/21]; p=0.008), had more intestinal failure (62.5% vs. 13.3%; p<0.001), required a longer hospital stay (161 days [IQR 112, 186] vs. 64 days [IQR 20, 91]; p<0.001), and were more likely to need home oxygen. In a multivariable analysis, lower birth weight (OR=0.3, [95% CI 0.1-0.5]; p=0.001), patent ductus arteriosus (OR=10.3, [95%CI 1.6-65.4]; p=0.014) and longer parenteral nutritional days (OR=8.8; [95%CI 2.0 -43.0]; p=0.005) were significantly and independently associated with higher odds of moderate/severe vs. non/mild BPD. Conclusion: Development of moderate/severe BPD occurred in the majority of preterm infants with surgical NEC in this consecutive series. Preterm infants with moderate/severe BPD were more likely to have a PDA before NEC. Development of moderate/severe BPD was associated with significantly greater burden and duration of post-operative morbidity following surgical NEC. Identifying surgical NEC infants at increased risk of moderate/severe BPD and developing lung protection strategies may improve surgical NEC outcomes.

Renal cell carcinoma is known to cause metastasis to unusual sites, which can be both synchronous or metachronous. Thyroid gland is a rare site for metastasis, but when it occurs, renal cell carcinoma is the most common primary neoplasm. We report the case of a 81-year-old female patient who had a significant medical history of right clear cell renal carcinoma with adrenal metastasis. She underwent right radical nephrectomy and adrenalectomy followed by radiofrequency ablation of left adrenal metastasis and systemic chemotherapy with sunitinib. Eleven years later, she presented with dysphagia and was found to have distal esophageal adenocarcinoma. On imaging, there was incidental detection of a left renal mass lesion and a right thyroid nodule, which on histopathology and immunohistochemistry were confirmed to be clear cell carcinoma of renal origin.