Beckwith-Wiedemann syndrome and splenic hemangioma: report of a case.

Surveillance sonography is performed in Beckwith-Wiedemann syndrome because of the risk of malignant tumors. Benign neoplasms, although reportedly less common, also occur. A splenic hemangioma was found in a patient by routine sonography.

Primitive neuroectodermal tumor of the kidney--another enigma: a pathologic, immunohistochemical, and molecular diagnostic study.

Primitive neuroectodermal tumor (PNET), the second most common type of sarcoma in the first two decades of life, rarely presents as an organ-based neoplasm. Rather, it is seen typically in the soft tissues of the chest wall and paraspinal region. We report a case of primary PNET of the kidney in a 17-year-old girl who presented with abdominal pain, hematuria, and an abdominal mass.

Lung transplantation for treatment of infants with surfactant protein B deficiency.

Objective: To evaluate lung transplantation for treatment of surfactant protein B (SP-B) deficiency.

Study Design: We compared surfactant composition and function from pretransplantation and posttransplantation samples of bronchoalveolar lavage fluid, somatic and lung growth, neurodevelopmental progress, pulmonary function, and pulmonary immunohistology in 3 infants with SP-B deficiency who underwent bilateral lung transplantation at 2 months of age and 3 infants who underwent lung transplantation for other reasons.

Results: Two years after transplantation, the 2 surviving infants with SP-B deficiency exhibited comparable somatic growth and cognitive development to the comparison infants.

Colorectal adenocarcinoma as a second malignant neoplasm following Wilms' tumor and rhabdomyosarcoma.

Colorectal carcinoma is one of the most common primary malignancies in adults and occurs in older patients after pelvic radiation. It is rare in children and young adults. We report two cases of colonic adenocarcinoma which were second malignant neoplasms following treatment for early childhood malignancies.

Myocardial extramedullary hematopoiesis following myocardial infarction.

Extramedullary hematopoiesis (EMH) usually accompanies a chronic hematologic disease in adults or prematurity in neonates. We observed a striking case of EMH in the explanted heart of a 13-year-old boy who underwent transplantation after extensive myocardial infarction. The florid myeloid proliferation raised the possibility of a leukemic process.

What is a juvenile polyp? An analysis based on 21 patients with solitary and multiple polyps.

Background: Juvenile polyps, the most common pediatric gastrointestinal polyp, have been typically characterized as either hamartomatous overgrowths or reactive inflammatory proliferations. Recent observations of excessive colonic and gastric carcinoma and dysplasia in juvenile polyposis have prompted reclassification of this entity as a premalignant condition. The relationship between solitary or multiple juvenile polyps and malignancy is less clear.

Juvenile xanthogranuloma: forms of systemic disease and their clinical implications.

Objective: Juvenile xanthogranuloma (JXG) with systemic (extracutaneous) involvement is a rare histiocytic disorder in which significant morbidity and occasional deaths may occur. The objective of this study was to characterize the spectrum of anatomic involvement, associated clinical problems, and management considerations in children with systemic JXG.

Study Design: Two current cases and literature reports of 34 children with various forms of systemic AG were analyzed with respect to age, clinical presentation, site(s) of involvement, therapy, and outcome.

Liver disease in polyglandular autoimmune disease type one: clinicopathologic study of three patients and review of the literature.

We report the findings from liver biopsies of three patients with polyglandular autoimmune disease type 1. The livers in two patients had histologic features of chronic hepatitis that eventuated in bridging fibrosis and cirrhosis over a period of several years. Nonspecific lobular and portal tract inflammation was present in the liver biopsy of the third patient.

Pleuropulmonary blastoma: a marker for familial disease.

Objective: To catalog and evaluate patterns of disease in families of children with pleuropulmonary blastoma (PPB).

Methods: Data have been collected since 1988 on 45 children with PPB and their families. All pathologic materials were centrally reviewed.

Soft tissue gliomatosis. Morphologic unity and histogenetic diversity.

Normal-appearing tissues in non-native sites constitute one of the more common morphologic expressions of abnormal development. The presence of pancreas in the wall or on the serosa of the small intestine and adrenal cortical tissue in a hernia sac are two familiar examples of heterotopias. We report our experience of mature glial tissues in the soft tissues of six children who were between the ages of 4.

Adenocarcinoma of minor salivary gland origin with skeletal metastasis in a child.

Primary epithelial neoplasms of the salivary gland in children are uncommon but are well recognized and occur principally in the major salivary glands. The purpose of this report is to document our experience with an adenocarcinoma of the buccal submucosa (one of several sites of minor salivary gland tissue) that metastasized to multiple bones as the initial sites of distant disease after a local recurrence. The clinical history, imaging studies, and microscopic sections including immunoperoxidase studies were evaluated from the primary tumor, local recurrence, and a metastatic lesion from the femur.

A unique dysembryonic neoplasm of the adrenal gland composed of nephrogenic rests in a child.

A primary neoplasm of the right adrenal gland in a 4-year-old boy was discovered after the patient developed bowel obstruction following an appendectomy. Until the histologic examination, the tumor was thought to be a neuroblastoma. However, the intra-adrenal tumor was composed of blastematous nodules, primitive tubules, and glomeruloid structures whose overall composition resembled a Wilms' tumor.

Cutaneous presentation of juvenile chronic myelogenous leukemia: a diagnostic and therapeutic dilemma.

Annular, erythematous, circinate plaques were the first manifestation of juvenile chronic myelogenous leukemia (JCML) in an otherwise healthy 2.5-year-old boy who had had these lesions since 6 months of age. The lesions showed an atypical hematopoietic infiltrate on biopsy.

Dedifferentiated chondrosarcoma in childhood: report of a case.

Dedifferentiated chondrosarcoma is an unconventional chondrosarcoma of distinctive pathology. The tumor, not previously reported in childhood, is characterized by a very poor prognosis with an average survival of only 6 months. Imaging features include a lytic lesion, focal calcifications, and a soft tissue mass.

Malignant rhabdoid tumors: a clinicopathologic review and conceptual discussion.

The malignant rhabdoid tumor (MRT) has been a controversial lesion since its seminal description. There is no consensus as to whether it represents a distinctive clinicopathological entity or, alternatively, a phenotypic pattern that is potentially common to several disparate neoplasms. MRT of the kidney is a childhood tumor that is associated with uniformly aggressive behavior, but it shows a wide spectrum of histologic, immunophenotypic, and cytogenetic findings.

False-positive interpretations of carcinoma in exfoliative respiratory cytology. Report of two cases and a review of underlying disorders.

The false-positive interpretation of malignancy is a potential pitfall of exfoliative respiratory tract cytopathology. However, the underlying causes of this problem are still relatively under-recognized. The authors herein present two additional examples in which bronchial brushing and washing specimens were misinterpreted as showing carcinoma of the lung.

Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases.

Inflammatory myofibroblastic tumor (IMT) or inflammatory pseudotumor is a spindle cell proliferation of disputed nosology, with a distinctive fibroinflammatory and even pseudosarcomatous appearance. Although the lung is the best known and most common site, inflammatory myofibroblastic tumor occurs in diverse extrapulmonary locations. We report our experience with 84 cases occurring in the soft tissues and viscera of 48 female patients and 36 male patients between the ages of 3 months and 46 years (mean, 9.

Congenital generalized myofibromatosis: a disseminated angiocentric myofibromatosis.

Infantile myofibromatosis occurs in solitary, multiple, and generalized forms, with similar histology but different clinicopathologic and prognostic implications. We report the findings in two male infants with fatal congenital generalized myofibromatosis (CGMF) who presented with multiple dermal and subcutaneous nodules at birth. Imaging studies revealed bony and visceral lesions, which progressed despite chemotherapy.

Anaplastic renal cell carcinoma following neuroblastoma.

Renal cell carcinoma is unusual in children. We report a case of anaplastic renal cell carcinoma arising in a 7-year-old girl following treatment for Stage III neuroblastoma. The renal cell carcinoma has unusual histologic and ultrastructural features, which are discussed.

Pleomorphic hamartoma of the subcutis: a lesion with possible myogenous and neural lineages.

The authors report an example of an unusual subcuticular spindle cell tumor with histologic features which were most like those of "pleomorphic fibroma." However, the tumor cells exhibited bifid differentiation immunohistologically, with conjoint reactivity for vimentin, S-100 protein, Leu-7 antigen (CD57), myelin basic protein, and desmin. A hamartomatous origin was favored for this lesion, given its overall morphologic features, circumscription, and apparently heterogeneous cellular lineages.

Needle localization breast biopsy: a model for multidisciplinary quality assurance.

As part of the quality assurance role of the Cancer Committee at Barnes Hospital, an institutional audit of Needle Localization Breast Biopsy (NLBB) was performed. Mammographic, operative, and surgical pathology reports from 370 consecutive patients at our institution undergoing both mammography and needle localization biopsy over a 34-month interval were reviewed. Carcinoma was diagnosed pathologically in 103 patients (28%), and 27% of these proved to be noninvasive.