The American journal of surgical pathology
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Hereditary pancreatitis is an autosomal dominant disorder with 80% penetrance and variable expressivity. The vast majority of cases have been linked to mutations within the cationic trypsinogen gene, also referred to as serine protease 1 (PRSS1). Other than inheritance, PRSS1 pancreatitis has been considered clinically and pathologically indistinguishable from other etiologies of chronic pancreatitis. ⋯ These changes were more developed in younger adults (n=2), in whom fatty replacement seemed to extend from the periphery to the central portions of the pancreas. With older patients (n=4), the pancreas showed marked atrophy and extensive replacement by mature adipose tissue with scattered islets of Langerhans and rare acinar epithelium concentrated near the main pancreatic duct. In summary, PRSS1 hereditary pancreatitis is characterized by progressive lipomatous atrophy of the pancreas.
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Am. J. Surg. Pathol. · Mar 2014
Tumor regression grade of urothelial bladder cancer after neoadjuvant chemotherapy: a novel and successful strategy to predict survival.
Histopathologic tumor regression grades (TRGs) after neoadjuvant chemotherapy predict survival in different cancers. In bladder cancer, corresponding studies have not been conducted. Fifty-six patients with advanced invasive urothelial bladder cancer received neoadjuvant chemotherapy before cystectomy and lymphadenectomy. ⋯ In conclusion, among all parameters reflecting tumor regression, the dominant TRG was the only independent risk factor. A favorable chemotherapy response is associated with a high proliferation rate in the initial chemotherapy-naive bladder cancer. This feature might help personalize neoadjuvant chemotherapy.
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Am. J. Surg. Pathol. · Nov 2013
Gastrointestinal stromal tumors with KIT exon 9 mutations: Update on genotype-phenotype correlation and validation of a high-resolution melting assay for mutational testing.
KIT exon 9 mutations in gastrointestinal stromal tumors (GISTs) are highly relevant and have direct therapeutic implications. In this context, we established and validated a fast and sensitive high-resolution melting assay. Analyzing 126 primary and 18 metastatic KIT exon 9-mutated cases from our registry, we demonstrate that the mutational spectrum of exon 9 is broader than previously thought and describe 3 novel mutations. ⋯ Furthermore, we provide first evidence that exon 9-mutated GISTs metastasize significantly more often to the peritoneum than to the liver. Performing extensive statistical analyses on data from our registry and from the literature, we demonstrate that KIT exon 9 mutations are neither associated with intermediate-risk/high-risk status nor overrepresented among metastatic lesions. Thus, we conclude that exon 9 mutations per se do not have prognostic relevance.
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Am. J. Surg. Pathol. · Aug 2013
Perineural invasion in T1 oral squamous cell carcinoma indicates the need for aggressive elective neck dissection.
Observation or elective neck dissection (END) for cN0 neck remains controversial for the treatment of T1-2 oral squamous cell carcinoma (OSCC). Perineural invasion (PNI) has been recognized as a poor prognostic factor for OSCC. However, its significance in T1 OSCC remains unclear. ⋯ In addition, END improved the 5-year DSS of T1 OSCC more in PNI-positive than in PNI-negative patients (16.2% vs. 5.4%). Our results indicate that PNI independently predicts a poor prognosis in T1 OSCC patients who are potentially curable but tend to be treated conservatively. For its efficacy in improving treatment outcomes, aggressive END is indicated for T1 OSCC patients at the presence of PNI.
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Am. J. Surg. Pathol. · Jun 2013
Comparative StudyComparison of WHO Classifications (2004, 2010), the Hochwald grading system, and AJCC and ENETS staging systems in predicting prognosis in locoregional well-differentiated pancreatic neuroendocrine tumors.
It is difficult to predict prognosis in patients with locoregional well-differentiated (WD) pancreatic neuroendocrine tumors (PanNET). We aimed to examine commonly used stratification systems [World Health organization (WHO) 2004 and 2010 classifications, American Joint Committee on Cancer (AJCC) and European Neuroendocrine Tumor Society (ENETS) staging, and the Hochwald grading system] for their power in predicting recurrence-free survival (RFS) in these patients. Seventy-five such patients (mean age 56 y, mean follow-up 79 mo) who underwent resection with sufficient tissue material and follow-up data were studied. ⋯ When these systems were analyzed in conjunction with various additional important pathologic features, combination of the Hochwald grading system and Ki-67 PI achieved the highest CI (0.7946). Therefore, although all these systems predict RFS well in locoregional WD PanNETs, the Hochwald grading system achieves the highest predictive ability. Further predictive power can be achieved by combining the Hochwald grading system and Ki-67 PI.