Fellowship training in pediatric pathology: a guide for program directors.

ABSTRACT The Accreditation Council for Graduate Medical Education (ACGME) has provided guidance for specialty and subspecialty fellowship training programs by defining 6 core competencies that must be met. Furthermore, the ACGME has defined several program requirements for pathology training, including those applicable to several pathology subspecialties. However, the requirements are broad and lack specific details, particularly as they pertain to the unique nature of pediatric pathology. The Fellowship Committee of the Society for Pediatric Pathology examined the ACGME requirements and interpreted the guidelines with respect to their application to training in pediatric pathology. The Committee worked within the ACGME guidelines to provide an expanded and more comprehensive set of guidelines for use by pediatric pathology fellowship directors and trainees. The resultant document lists the educational goals, core competencies, and program requirements with specific application to pediatric pathology. In addition, methods for assessing and documenting the progress of the individual trainees as they progress through each requirement are provided. It is to be emphasized that many of the guidelines set forthwith are flexible, and allowances should be made for individual differences of each training program.

Aggressive osteoblastoma: a case report involving a unique chromosomal aberration.

Osteoblastomas are rare bone-producing neoplasms that generally occur in the young and can be misdiagnosed as an osteosarcoma if correlation with clinical history, radiology, and histology is not carefully considered or if the several variants of osteoblastoma are not recognized. These variants lie on a morphologic spectrum between conventional osteoblastoma and osteosarcoma. Aggressive osteoblastoma is one such subtype. As the name implies, the histologic features of aggressive osteoblastoma may appear malignant, and its biologic behavior may separate it from conventional osteoblastoma. We report a case of aggressive osteoblastoma occurring in the femoral diaphysis of a 12-year-old girl; this osetoblastoma was dyssynchronous from the radiologic appearance and a diagnostic challenge. Cytogenetic evaluation of the neoplasm revealed a pseudodiploid clone with a balanced translocation involving chromosomes 4, 7, and 14. Using the premise that cytogenetics might be useful as a diagnostic tool for a more specific classification, we reviewed the literature in order to compare our findings with known chromosomal aberrations.

Lymphangiomatosis: a case report.

Lymphangiomatosis is a rare congenital malformation that can involve visceral organs, soft tissue, and bone. This report describes a 5-year-old female with this disorder who presented with respiratory distress and subsequently died. Clinical, diagnostic, and treatment aspects of this entity are discussed.

Medication errors in pediatrics--the octopus evading defeat.

Medication errors have come to the forefront in healthcare and oversight organizations as well as to the public over the past several years. There has been an increasing focus on this area of patient care requiring more intensive evaluation and intervention to prevent these errors. Although it is difficult to ascertain the true occurrence of medication errors, they may occur as frequently as once in every 20 orders. Children are at higher risk for medication errors and adverse drug events for numerous reasons. Not only is there great variability in weight and body surface area in this population, there is also significant differences in the pharmacokinetics and pharmacodynamics of many medications when compared to adults. In addition, our knowledge of pharmacogenetics and phenotypic ontogeny must be applied. Sources of medication errors are identified, and specific examples and solutions to improve medication use in children are provided. It is critical to have 1) personnel trained in pediatrics to prescribe, prepare, dispense and administer medications, 2) a quality review system in place to review drug use and medication errors, and 3) to implement computerized physician order entry with decision support and other tools in the next decade to improve pharmacologic therapy for pediatric patients.

Sentinel lymph node biopsy in juvenile secretory carcinoma.

A 9-year-old girl presented with a 3-year history of a right breast mass. Excisional biopsy showed a secretory carcinoma. A treatment plan of simple mastectomy and axillary sentinel lymph node biopsy was chosen. She remains free of disease at 3-year follow-up. Sentinel lymph node biopsy offers an approach to stage the axillary lymphatic basin with a lower complication rate than formal dissection. The authors advocate its use in the treatment of pediatric breast cancer.

Childhood obesity: a risk factor for omental torsion.

PURPOSE: To determine the risk factors and clinical presentation of primary omental torsion (POT) in children. METHODS: Histopathology records of a pediatric hospital from January 1993 to March 2003 were reviewed to identify cases of POT. Hospital charts of patients diagnosed with POT were reviewed for demographic data and clinical presentation. RESULTS: A diagnosis of POT was recorded in 12 of 41,987 pathology records reviewed. Most of the patients were white (92%), male (75%), and 9 to 16 years old (75%). Weight percentiles were >or=95th in 11 (92%) of 12 patients. Body mass index was calculated in 9 of the 12 cases with 8 >95th percentile. Clinical presentation including right-sided abdominal pain, tenderness, and anorexia closely mimics acute appendicitis. CONCLUSIONS: Obesity seems to be an important risk factor for POT in children. The presentation for POT seems to be less acute than with other causes of surgical abdomen.

Fatal intravenous injection of potassium in hospitalized patients.

We describe 4 cases of fatal intravenous injection of potassium in the hospital setting. These cases illustrate the subtlety of findings in such deaths and remind the forensic pathologist to consider this type of event in sudden, unexpected death of hospitalized patients.

Sinus histiocytosis with massive lymphadenopathy and Langerhans cell histiocytosis express the cellular adhesion molecule CD31.

Context.-We investigated expression of the adhesion molecule CD31 in sinus histiocytosis with massive lymphadenopathy (SHML) and Langerhans cell histiocytosis (LCH) because (1) SHML and LCH cells express a variety of cellular adhesion molecules and (2) SHML has been characterized as a reactive histiocytic proliferation, and tissue macrophages (histiocytes) are known to express CD31. Objective.-The purpose of this study was to determine whether SHML and LCH cells express CD31 and whether dual staining with CD31 and S100 facilitates diagnosis of these disease states. Methods.-Formalin-fixed, paraffin-embedded archival tissues were immunohistochemically stained via the labeled streptavidin-biotin method using antibodies against CD31 and S100 protein after heat-induced epitope retrieval. Archival tissues included SHML (n = 2), LCH (n = 10), malignant melanoma (n = 5), sinus hyperplasia (n = 4), granulomas (n = 4), granular cell tumor (n = 6), and normal skin (n = 4). Results.-Normal Langerhans cells in the epidermis were CD31(-)/S100(+); neoplastic Langerhans cells in LCH were CD31(+)/S100(+). Histiocytes in granulomas and in sinus hyperplasia were CD31(+)/S100(-); abnormal histiocytes in SHML were CD31(+)/S100(+). S100(+) tumors (malignant melanoma and granular cell tumor) were CD31(-). Conclusions.-The spectrum of cell types that express CD31 is expanded to include SHML and LCH. We speculate that up-regulation of CD31 in neoplastic Langerhans cells contributes to the migratory capability of LCH cells. CD31 may be a useful nonlysosomal marker of macrophages and their neoplastic counterparts (true histiocytic sarcomas). An immunohistochemical staining panel that includes CD31 and S100 facilitates the diagnosis of SHML and LCH.