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A retrospective review of undifferentiated malignancy in childhood
1Department of Pediatrics, Children's Hospital of Pittsburgh, Pennsylvania 15213.
Insights
Pediatric cancer diagnoses were re-evaluated, significantly reducing the incidence of "undifferentiated malignancy" with modern pathology. This highlights the importance of thorough diagnostic evaluation for accurate childhood cancer treatment.
Area of Science:
- Pediatric Oncology
- Pathology
- Cancer Research
Background:
- The diagnosis of
Purpose of the Study:
- To re-evaluate the incidence and diagnostic accuracy of "undifferentiated malignancy" in pediatric cancer patients.
- To assess the impact of modern diagnostic methods on childhood cancer classification.
Main Methods:
- Retrospective review of pediatric cancer cases diagnosed between 1971-1987 at The Children's Hospital of Pittsburgh.
- Re-evaluation of initial pathology reports using current diagnostic techniques, including immunostaining.
- Analysis of clinical features and management of initially diagnosed undifferentiated tumors.
Main Results:
- Initial diagnosis of undifferentiated malignancy in 22 of 2,095 pediatric cancer patients (1.1%).
- Re-evaluation using current methods reassigned specific diagnoses in 15 of 20 cases, reducing incidence to 0.23%.
- Two rhabdoid tumors remained undifferentiated; clinical features and management were heterogeneous.
Conclusions:
- The incidence of undifferentiated malignancy in children is significantly lower with current diagnostic standards.
- Thorough pathologic evaluation with sufficient biopsy material is crucial for accurate childhood cancer diagnosis.
- Accurate diagnosis impacts treatment strategies, potentially improving patient outcomes.
Abstract:
We reviewed the experience of children with "undifferentiated" cancer at The Children's Hospital of Pittsburgh (CHP) from 1971 through 1987. Of 2,095 patients 0-18 years old with diagnoses during that time of any cancer, the initial CHP pathology report rendered a diagnosis in 22 children (1.1%) of "undifferentiated malignancy" or "malignant tumor" (15 cases), "undifferentiated carcinoma" (two cases) or "anaplastic tumor or carcinoma" not otherwise specified (five cases). A review of pathologic findings using current methods and immunostaining led to the assignment of a specific diagnosis in 15 of the 20 cases so studied. Thus, the incidence of undifferentiated cancer by current criteria was reduced to no more than 0.23%. Two of the five tumors for which an alternative diagnosis could not be established were described as "rhabdoid," but because it is not clear that these tumors fit into a single category they were still considered to be undifferentiated. Clinical features and management of the 22 cases including the five persistently diagnosed as undifferentiated malignancy were heterogeneous. In the face of reassigned diagnoses, a number of patients would likely have received different chemotherapy as well as radiation. Nonetheless, seven patients with malignancy show no evidence of disease (NED), including several whose therapy, given the current best diagnosis, would not be considered to have been optimal. We recommend that for patients undergoing biopsy of a tumor, sufficient material be obtained for extensive pathologic evaluation. In this way, the diagnosis of undifferentiated malignancy in children can be almost eliminated.(ABSTRACT TRUNCATED AT 250 WORDS)