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PubMed2005

Pediatric renal tumors: practical updates for the pathologist.

Pediatric renal tumors were targeted by the National Wilms Tumor Study Group for 4 decades with extraordinary success. Within this historic context, this review provides a summary of the new Children's Oncology Group renal tumor protocols that will be opening in the very near future, focusing on their pathologic requirements. All renal tumors must first be registered on the Renal Tumor Classification and Banking Protocol, followed by registration on 1 of 4 primary therapeutic protocols based on histology, stage, and molecular analysis. This requires prompt submission of samples for molecular analysis and central pathologic review. Changes in staging criteria include classification of all tumor spillage as stage III, and requirement of regional lymph node evaluation for eligibility for stage I Wilms tumors (WTs) weighing less than 550 g in infants younger than 24 months and for stage I clear cell sarcoma. Patients with unilateral favorable histology WT with loss of heterozygosity for chromosomes 1p and 16q will receive more aggressive chemotherapy at each stage. Patients with bilateral WT and patients with diffuse hyperplastic perilobar nephroblastomatosis will be eligible for a novel therapeutic protocol requiring pathologic classification based on response of tumor to previous therapy. Stage I anaplastic WT will be targeted with more aggressive chemotherapy than in the past. For the first time, pediatric renal cell carcinoma will be eligible for a cooperative group protocol. All rhabdoid tumors outside the central nervous system will be eligible for a single protocol. In conclusion, these new protocols bring considerable change in their overall organization, in eligibility, and in therapy.

Antineoplastic Protocols↗
PubMedDate not supplied

National Wilms Tumor Study: an update for pathologists.

The National Wilms Tumor Study (NWTS)-5, begun in August 1995, incorporates some important new definitions and concepts that have critical importance in determining therapy and prognosis. The criteria for stage 1 were refined to accommodate an important subset of stage 1 Wilms tumors that are being managed by nephrectomy alone, without the use of adjuvant therapy. The distinction between stages 1 and 2 in the renal sinus is no longer defined by the hilar plane but by the presence or absence of venous or lymphatic invasion. Specific problems encountered in the interpretation of the renal sinus are discussed here. The topographical definition of focal anaplasia has proven to be of prognostic value and is incorporated into the NWTS-5 study design.

Anaplasia↗
PubMedDate not supplied

Pediatric germ cell tumors: protocol update for pathologists.

The therapy for pediatric germ cell tumors has historically been widely variable and institution dependent. The efforts to provide consistent, biology-driven therapy through Intergroup protocols has been a relatively recent phenomenon that has provided the framework for future protocol designs. The first Intergroup protocols confirmed that stage I malignant testicular germ cell tumors and immature teratomas at all sites in children could be treated with surgery alone followed by close observation. Future protocols currently in the planning stages may extend this low-risk category to stage I ovarian germ cell tumors of all histologic types. Low-stage extragonadal germ cell tumors may be placed in a new intermediate risk category. Particular pathologic issues that were raised and reported during the first protocols and that may impact on future protocol design include the presence and size of foci of endodermal sinus tumors within low-stage immature teratomas at all sites. Accurate staging will grow more critical in future protocols. The ability to recruit international cooperative groups will determine the success of chemotherapy tailored to specific subgroups that now must be lumped for statistical purposes. Lastly, companion biologic studies will be critical to defining the different subtypes of germ cell tumors and to determining predictors of biologic behavior.

Adolescent↗
PubMed1996

Practice guideline development task force of the College of American Pathologists. Hereditary hemochromatosis.

Hereditary hemochromatosis is an autosomal recessive disorder, the gene for which occurs in approximately 10% of Americans, most of whom are unaffected heterozygotes. Approximately 5/1000 white Americans are homozygous and at risk of developing severe and potentially lethal hemochromatosis. The disorder affects numerous organ systems, but the most common symptoms are fatigue, palpitations, joint pains, and impotence; the most common signs are those that relate to hypothalamic, cardiac, hepatic or pancreatic dysfunction, including poor cold tolerance, impotence in males, amenorrhea in females, cardiac arrhythmias, dyspnea, edema, hepatosplenomegaly, spider telangiectases, ascites, deformity, swelling or limitation of motion of joints, weight loss, hyperpigmentation. Characteristic abnormalities of laboratory tests include elevated serum iron concentration, high transferrin saturation, elevated serum ferritin concentration, elevated serum transaminases, hyperglycemia and low values for thyroid-stimulating hormone (TSH) and gonadotropins. Death may be the result of cardiac arrhythmia, congestive heart failure, liver failure or liver cancer. Since many of these complications cannot be reversed once they have developed, early diagnosis and treatment are essential. In view of the high prevalence in the American population (prevalence varies with ethnic background), the low cost of diagnosis and treatment, the efficacy of treatment if begun early, and, on the other hand, high costs and low success rate of late diagnosis and treatment, systematic screening for hemochromatosis is warranted for all persons over the age of 20 years. The initial screening should be by measurement of serum iron concentration and transferrin saturation. The practice guideline provides a diagnostic algorithm for cases in which the serum transferrin saturation is 60% or greater. It also provides guidelines for clinical management.

Female↗