Educational Case: Wilms Tumor

The following fictional case is intended as a learning tool within the Pathology Competencies for Medical Education (PCME), a set of national standards for teaching pathology. These are divided into three basic competencies: Disease Mechanisms and Processes, Organ System Pathology, and Diagnostic Medicine and Therapeutic Pathology. For additional information, and a full list of learning objectives for all three competencies, see http://journals.sagepub.com/doi/10.1177/2374289517715040

Rapidly growing subcutaneous mass in an infant

Fibrous hamartoma of infancy (FHI) is a benign mesenchymal tumor of young children. It has a broad clinical differential diagnosis and is often clinically confused for vascular and malignant soft tissue neoplasms. Recognition of the unique histologic features of FHI, a triphasic population of mature adipose tissue, mature fibrous tissue, and immature mesenchymal tissue, will ensure the correct diagnosis. In this report we present a case of this rare entity, including the associated clinical, radiologic, and histologic findings

Concurrent diagnoses of Hodgkin lymphoma and biclonal myeloma in the bone marrow

The diagnoses of Hodgkin lymphoma and multiple myeloma have rarely been made simultaneously in the same patient. We present a case of an 82-year-old man who rapidly developed pancytopenia and liver failure with coagulopathy. Serum protein electrophoresis and immunofixation revealed an unequivocal immunoglobulin Gκ and immunoglobulin Gλ biclonal gammopathy. Bone marrow biopsy showed involvement by classic Hodgkin lymphoma with an inflammatory background including 49% mature plasma cells. Unfortunately, the patient died 14 days after admission. To our knowledge, a case of concurrent Hodgkin lymphoma and biclonal multiple myeloma has not previously been reported. Detection of severe bone marrow plasmacytosis in the background of Hodgkin lymphoma should alert the pathologist to the possibility of collision with a plasma cell neoplasm, warranting a complete diagnostic workup. © 2010 Elsevier Inc. All rights reserved

Rapidly growing subcutaneous mass in an infant

Fibrous hamartoma of infancy (FHI) is a benign mesenchymal tumor of young children. It has a broad clinical differential diagnosis and is often clinically confused for vascular and malignant soft tissue neoplasms. Recognition of the unique histologic features of FHI, a triphasic population of mature adipose tissue, mature fibrous tissue, and immature mesenchymal tissue, will ensure the correct diagnosis. In this report we present a case of this rare entity, including the associated clinical, radiologic, and histologic findings

Cardiac and Pericardial Neoplasms in Children: Radiologic-Pathologic Correlation

Primary cardiac and pericardial neoplasms are rare in the pediatric population and can include both benign and malignant lesions. Rhabdomyomas, teratomas, fibromas, and hemangiomas are the most common benign tumors. The most common primary cardiac malignancies are soft-tissue sarcomas, including undifferentiated sarcomas, rhabdomyosarcomas, and fibrosarcomas. However, metastatic lesions are more common than primary cardiac neoplasms. Children with primary cardiac and pericardial tumors may present with nonspecific cardiovascular symptoms, and their clinical presentation may mimic that of more common nonneoplastic cardiac disease. The diagnosis of cardiac tumors has recently been facilitated using noninvasive cardiac imaging. Echocardiography is generally the first-line modality for evaluation. Cardiac MRI and CT are used for tissue characterization and evaluation of tumor size, extension, and physiologic effect. The varied imaging appearances of primary cardiac neoplasms can be explained by their underlying abnormality. Treatment of these lesions varies from conservative management, with spontaneous regression of some lesions such as rhabdomyomas, to surgical resection, particularly in patients with associated heart failure. With adequate imaging techniques and knowledge of the pathologic basis of the neoplasm, it is often possible to differentiate benign from malignant tumors, which can greatly affect adequate and timely treatment. RSNA, 2023 Quiz questions for this article are available through the Online Learning Center