BizarreParosteal Osteochondromatous Proliferation (Nora's lesion) with translocation t(1;17)(q32;q21): a case report and role of cytogenetic studies on diagnosis.

Bizarre Parosteal Osteochondromatous Proliferation (BPOP) is a benign tumor-like lesion that has recently been reported to have an association with a specific translocation t(1:17)(q32;q21)[1]. Like other reactive periosteal lesions, BPOP can be diagnostically challenging, with the ever-present possibility of a potentially devastating erroneous diagnosis of malignancy. These lesions are often clinically, radiologically and histopathologically ambiguous, with rapid but circumscribed, non-infiltrative growth patterns, and histological atypia, but without overt features of malignancy. However, recent published reports have better characterized radiological [2] as well as histological features that aid in making an accurate diagnosis. In spite of all these advances, one of the biggest challenges in making the correct diagnosis still remains the inexperience of the practicing pathologist with this lesion, simply due to its rarity. We present a case of Nora's lesion in the distal ulna of an 8 year-old girl, in which, besides the histological features, we were able to demonstrate the translocation t(1:17)(q32;q21). Thus, we would like to emphasize the utility of cytogenetic studies in the correct and rapid diagnosis of clinically and radiologically ambiguous periosteal-based lesion.

Metanephric adenoma in a five-year-old boy presenting with chyluria: case report and review of literature.

Metanephric adenoma and chyluria are rare entities, especially in the pediatric population of North America. To date, no report of chyluria associated with metanephric adenoma has been published. The incidental presentation is most common in the fifth decade of age with this tumor; however, when signs and symptoms are present they include hematuria, palpable mass, flank pain, polycythemia, and hypercalcemia. We present the case of a 5-year-old boy who did not demonstrate the common findings listed, but rather he presented with chyluria.

Primary and metastatic lung tumors in the pediatric population: a review and 25-year experience at a large children's hospital.

CONTEXT: Primary lung neoplasms are rare in children, but they comprise a broad and interesting spectrum of lesions, some of which are familiar from other tissue sites, and some of which are unique to the pediatric lung. OBJECTIVE: To determine the relative incidence of primary and metastatic lung tumors in children and adolescents through a single-institution case series, to compare these data to reports in the medical literature, to discuss the clinical and pathologic features of primary tumors of the tracheobronchial tree and lung parenchyma in children, and to provide recommendations for handling pediatric lung cysts and tumors. DATA SOURCES: A 25-year single institutional experience with pediatric lung tumors, based on surgical biopsies and resections at Texas Children's Hospital from June 1982 to May 2007, an additional 40 lung tumors referred in consultation, and a review of the medical literature. CONCLUSIONS: A total of 204 pediatric lung tumors were diagnosed at our institution, including 20 primary benign lesions (9.8%), 14 primary malignant lesions (6.9%), and 170 secondary lung lesions (83.3%). The ratio of primary benign to primary malignant to secondary malignant neoplasms is 1.4:1:11.6. The common types of lung cancer in adults are exceptional occurrences in the pediatric population. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumor. Other primary pediatric lung tumors include congenital peribronchial myofibroblastic tumor and other myofibroblastic lesions, sarcomas, carcinoma, and mesothelioma. Children with primary or acquired immunodeficiency are at risk for Epstein-Barr virus-related smooth muscle tumors, lymphoma, and lymphoproliferative disorders. Metastatic lung tumors are relatively common in children and also comprise a spectrum of neoplasia distinct from the adult population.

Synoptic reporting in tumor pathology: advantages of a web-based system.

The American College of Surgeons Commission on Cancer (ACS-CoC) mandates that pathology reports at ACS-CoC-approved cancer programs include all scientifically validated data elements for each site and tumor specimen. The College of American Pathologists (CAP) has produced cancer checklists in static text formats to assist reporting. To be inclusive, the CAP checklists are pages long, requiring extensive text editing and multiple intermediate steps. We created a set of dynamic tumor-reporting templates, using Microsoft Active Server Page (ASP.NET), with drop-down list and data-compile features, and added a reminder function to indicate missing information. Users can access this system on the Internet, prepare the tumor report by selecting relevant data from drop-down lists with an embedded tumor staging scheme, and directly transfer the final report into a laboratory information system by using the copy-and-paste function. By minimizing extensive text editing and eliminating intermediate steps, this system can reduce reporting errors, improve work efficiency, and increase compliance.