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Biomedical subjects

C W Zuppan

Publications and source records attributed to C W Zuppan.

7 recordsLinked to original sources

The effect of preoperative therapy on the histologic features of Wilms' tumor. An analysis of cases from the Third National Wilms' Tumor Study.

Preoperative chemotherapy is being used with increasing frequency in the US for the management of selected children with Wilms' tumor, despite relatively limited knowledge as to the effects of such therapy on tumor histologic features. The authors reviewed pathologic material from all preoperatively treated unilateral Wilms' tumors registered on the third National Wilms' Tumor Study. Preoperative therapy was associated with increased ambiguity of tumor stage. The prevalence of anaplasia in the 83 evaluable specimens was similar to, although slightly increased over, that in comparable high-stage untreated Wilms' tumors (11% versus 5% to 8%), and it appeared to retain its adverse connotations. These data also suggest that alterations of tumor character and distribution by therapy provide useful prognostic information. All 17 children whose residual viable tumor was limited to intrarenal sites at the time of resection were alive and disease-free at last follow-up. Extensive (greater than 90%) tumor necrosis, low mitotic activity, and high degrees of differentiation of residual tumor were also associated with favorable outcomes. Although careful utilization of preoperative chemotherapy can be of value in children with otherwise difficult-to-manage Wilms' tumors, the potential benefits of preoperative tumor bulk reduction must be weighed against the risk of overtreatment or undertreatment as a result of increased ambiguity of tumor stage.

Anaplasia

Riedel's thyroiditis.

Invasive fibrous (Riedel's) thyroiditis is a rare thyroid condition of unknown origin that may be associated with inflammatory fibrosclerosing processes elsewhere in the body. Although the condition is benign and self-limiting, its importance lies in its ability to clinically mimic carcinoma almost completely, necessitating performing an open biopsy to establish the correct diagnosis. In a review of over 700 thyroid operations performed at Loma Linda (Calif) University Medical Center in the past 15 years, we encountered only one documented case of Riedel's thyroiditis. Our patient presented with a firm thyroid mass, vocal cord paralysis, and symptoms of esophageal compression. Surgery was performed to obtain a definitive diagnosis and prevent the possibility of subsequent additional tracheal compression with airway compromise. The pathologic findings, as well as the medical and surgical treatment of this condition, are reviewed.

Adult

Renal neoplasms mimicking rhabdoid tumor of kidney. A report from the National Wilms' Tumor Study Pathology Center.

Fifty-six renal neoplasms reviewed by the National Wilms' Tumor Study Pathology Center presented with histologic features that resulted in confusion with rhabdoid tumor of kidney, a usually lethal childhood renal tumor; all were eventually diagnosed as other entities. Conspicuous filamentous cytoplasmic inclusions or large nucleoli, typical findings in rhabdoid renal tumors, were the usual source of diagnostic difficulty. Most, but not all, tumors occurred in pediatric patients. Sixteen were examples of Favorable Histology Wilms' tumor, which invited confusion with rhabdoid tumors either on the basis of filamentous cytoplasmic inclusions (15 cases) or macronucleoli (one case). In most cases, foci of typical Wilms' tumor blastemal aggregation or evidence of definitive nephrogenic differentiation facilitated the correct diagnosis. All 10 patients for whom information about outcome was available were alive at last follow-up. The other 40 renal lesions mimicking rhabdoid tumor of kidney consisted of a clinically and histogenetically diverse group of neoplasms, including anaplastic Wilms' tumor, congenital mesoblastic nephroma, renal cell carcinoma, transitional cell carcinoma, collecting-duct carcinoma, oncocytoma, rhabdomyosarcoma, malignant neuroepithelial tumors, and lymphoma. Most of these lesions could be separated from renal rhabdoid tumors and correctly classified on the basis of careful attention to light microscopic details, but in several cases electron microscopy or immunocytochemical studies were helpful or essential.

Adenoma

Anaplasia in unilateral Wilms' tumor: a report from the National Wilms' Tumor Study Pathology Center.

Fifty-eight unilateral anaplastic Wilms' tumors entered on the Third National Wilms' Tumor Study (NWTS-3) were reviewed. The favorable outcome of stage I anaplastic tumors was confirmed. In stage II-IV tumors, the extent and severity of anaplasia were not of prognostic importance. However, two histologic features were correlated with extremely poor outcome; anaplasia in extrarenal tumor sites (P = .016) and predominantly blastemal tumor pattern (P = .020). The importance of these features in predicting relapse and death for children with anaplastic Wilms' tumor was supported by the study of 47 additional anaplastic Wilms' tumor cases from NWTS-2. These results emphasize the importance of examining sites of extrarenal spread in anaplastic Wilms' tumors. Cytologic criteria for anaplasia and clues to its recognition are reviewed, and the potential clinical and biological implications of our findings are discussed.

Cell Transformation, Neoplastic

Malignant fibrous histiocytoma in childhood: a report of two cases and review of the literature.

Malignant fibrous histiocytoma (MFH) is a tumor of late adult life not often recognized as occurring in children. Our search of the English literature produced only 27 well-documented examples occurring in the soft tissues, to which we here add 2 additional cases. Both of our cases displayed the classical storiform-pleomorphic histologic pattern typical of such tumors in adults, and both pursued a malignant and rapidly fatal course. Previous reports of this tumor in children are reviewed, and the differential diagnostic considerations briefly discussed. Overall, the clinical features of MFH in children are similar to those reported in adults, and surgical removal remains the key element of successful therapy.

Child

Lipid-rich rhabdomyosarcoma--a potential source of diagnostic confusion.

Rhabdomyosarcoma is an often primitive tumor capable of diverse morphologic manifestations. The article describes three cases of childhood rhabdomyosarcoma in which a significant population of lipid-rich tumor cells was present. The lack of specificity and potentially confusing nature of this feature are discussed, and the ultrastructural diagnosis of rhabdomyosarcoma is briefly reviewed.

Abdominal Neoplasms

Patterns of measles pneumonitis.

As a result of the enlarging pool of unvaccinated children and young adults, there has been an increase in serious measles pneumonitis in our areas. We recently examined autopsy and/or lung biopsy material from five children with fatal measles pneumonitis. Two patients were immunocompromised because of either prematurity or acute leukemia and died 13-16 days following onset of symptoms. Both had classic giant cell pneumonitis, with readily demonstrable intranuclear inclusions. Three other children without known immunocompromise had a more prolonged course. The lungs of these patients lacked the classic pattern and displayed instead a spectrum of less specific findings ranging from organizing diffuse alveolar damage to interstitial pneumonia with giant cells, but without viral inclusions. An accompanying necrotizing bronchiolitis was also present. Electron microscopy and/or detection of elevated measles-specific immunoglobulin M was necessary to confirm the diagnosis in these apparently immunocompetent patients. We conclude that the histologic features of fatal or serious measles pneumonitis are variable and depend to some extent on the immunocompetence of the host as well as the duration and tempo of the disease. Ancillary studies may be necessary to establish the diagnosis in cases lacking classic histopathologic features.

Adult