Biomedical subjects
Publications and source records attributed to J M Kissane.
Renal involvement in the tuberous sclerosis complex includes angiomyolipomas, cysts, and carcinomas. Angiomyolipomas, despite a sometimes frightening histopathologic appearance, are benign. Cystic disease, apparently resulting from tubular epithelial hyperplasia, causes hypertension and renal insufficiency, progressing to end-stage renal disease. The same epithelial hyperplasia predisposes to renal carcinoma.
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Review of current data from the Intergroup Ewing's Sarcoma Study (IESS) shows that Ewing's sarcoma (ES) is rare in bones of the hands and feet. Only 12 of 377 evaluable patients in the first two IESS studies had a primary tumor in these small, distal bones. The age distribution was typical for that seen in patients with ES at other sites. Males were affected twice as often as females, and tumors in the bones of the feet were much more common than those in the hands. All signs and symptoms were local in distribution. As in other sites, the dominant histologic pattern was categorized as diffuse. With the exception of those patients with lesions in the calcaneus, the prognosis for disease-free survival was excellent. A literature review of cases of ES reported in bones of the hands and feet showed generally comparable results.
Roentgenographic review of the first 210 cases of Ewing sarcoma (ES) in the Intergroup Ewing Sarcoma Study revealed that 37.6% of cases had evidence of diffusely increased intraosseous density or diffuse sclerosis (DS). In these cases the sclerosis was usually mixed with various patterns of lysis and/or combined with a periosteal reaction. A radiograph blinded histologic review of selected biopsies showed an 83% incidence of dead bone compared to 23% in those without DS. Ten percent of the cases with DS had appositional new bone formation on dead bone whereas none of the cases without DS showed such reactions. Pathologic explanation of the roentgenographically identified diffuse sclerosis in ES has not been previously well documented in the medical literature.
Subtotal pancreatectomy specimens from 11 pediatric patients with idiopathic hyperinsulinemic hypoglycemia (IHH) were studied by conventional light and electron microscopic methods and by morphometric methods applied to sections immunostained specifically for A, B, D, and PP cells. The results were compared with corresponding studies of pancreata obtained at autopsy of 31 infants and children without abnormalities in carbohydrate homeostasis. In the control tissue, the total volume density of islet cells in live born premature infants (n = 12) was about 20%, in live born term newborn infants (0 to 1 months, n = 9) between 17.5% and 20%, in infants (1 to 7 months, n = 5) about 10%, and in children (1.5 to 11 years, n = 5) about 7.5%. Endocrine tissue was as abundant in the body and head as in the tail of the pancreata. The contribution of PP cells to total islet cell mass increased with age, and for the relative contribution of PP cell compared with total pancreatic parenchyma it remained relatively constant, while that of A, B, and D cells decreased with age. A wide spectrum of islet cell aggregates was a normal feature of development in the control tissue, an observation accentuated by specific immunocytochemical staining. Islet cells of all types were present singly and in small clusters in pancreatic ductal structures and intimately related to acini; nesidioblastosis, therefore, is a feature of normal maturation of the pancreas. Seven of the 11 cases of IHH had pancreata that were morphologically and morphometrically normal for age. No anatomic basis for hyperinsulinism in these cases was apparent. Four pancreata from patients with IHH contained discrete foci of proliferation of islet cells of all types but in which B cells greatly predominated. We conclude that nesidioblastosis as a morphologic diagnosis cannot be viewed as the structural basis of endocrine dysfunction since it can be absent in IHH, or many of its features present in control pancreata.
Clinicopathologic features of 303 cases of Ewing's sarcoma of bone collected by the Intergroup Ewing's Sarcoma Study group are described. Ewing's sarcoma was found to arise predominantly in the inferior segment of the skeleton in young white patients from 10 to 15 years of age. Of the several recognized histologic patterns, the filagree pattern--an organoid pattern consisting of bicellular strands of tissue separated by filmy vascular stroma--was associated with the lowest survival rates. Light microscopic evidence of cytoplasmic glycogen in tumor cells was found to be useful in but not requisite for diagnoses. The most important predictor of clinical behavior, regardless of treatment modality, was the site of primary involvement. Survival among patients with primary tumors in pelvic bones was lowest, whereas survival among patients with lesions in distal bones of the extremities was highest.
Six cases of small-cell osteosarcoma, a tumor that resembles Ewing's sarcoma but produces osteoid matrix, are presented. The patients were young (6-31 years of age) and presented with symptoms of pain and/or swelling of 1-10 months duration. The lesions demonstrated a wide variation in radiographic appearance. Histologically, the tumors were composed of small, round cells that produced variable amounts of osteoid. In three cases chondroid was also present. Two of the six patients were treated with surgery alone and four received radiation and either single- or multiple-agent adjuvant chemotherapy. The two patients who received radiation and multiple-agent adjuvant chemotherapy have no evidence of disease at four and ten years after diagnosis. Recognition of this tumor as distinct from Ewing's sarcoma and from other forms of osteosarcoma is important to determine the incidence, clinical features, and optimal therapy for this tumor.
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Skeletal muscle may be infiltrated by a variety of invasive or metastatic malignant tumors. In 14 out of 291 cases of Ewing's sarcoma, we observed diagnostic biopsies in which local skeletal muscle fibers contained intrasarcolemmal aggregates of tumor cells. Cases demonstrating local intramyofiber invasion have a higher mitotic count, an increased rate of developing distant metastases, and a decreased survival when compared to cases not demonstrating this skeletal muscle "parasitism." The usefulness of skeletal muscle intramyofiber invasion in the differential diagnosis of Ewing's sarcoma remains unknown at this time.
Some sarcomas of bone are not characteristically childhood tumors. Within the context of osteosarcomas, telangiectatic and parosteal (juxtacortical) variants deserve recognition. Among chondrosarcomas, which ordinarily are not tumors that occur in children, the mesenchymal variant should be recognized in children especially, this lesion may be extraosseous. Fibrous histiocytoma and its malignant variant have recently emerged as morphologically specific lesions, occasionally osseous, among other spindle cell tumors. Ewing's sarcoma, which is predominantly of osseous origin, consists of undifferentiated fragile cells. The elaborate spectrum of topographic, cytologic, nuclear, and other morphologic features that fall within the designation Ewing's sarcoma are described.
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The incidental finding of hyalinized glomeruli in otherwise normal infant kidneys is referred to as congenital glomerulosclerosis. Two infants had extensive glomerulosclerosis manifested by nephrotic syndrome, severe oliguria, and progressive renal failure. Both patients were believed to have had intrauterine infections. These two cases have unequivocally identified congenital glomerulosclerosis as one of the causes of nephrotic syndrome in infancy. In addition, they suggest that extensive glomerulosclerosis in some cases may be a result of congenital infections.
Multilocular renal cysts are benign lesions with an unknown histogenesis. We report 3 children and summarize information currently known about these lesions. Capsular veins were seen in two patients during the early phases of intravenous urography. Intravenous urography with sonography and arteriography may suggest the diagnosis prior to surgery.
A newborn girl with respiratory distress due to bilateral pneumothorax was found to be anuric, and died at 18 hours of age. Autopsy revealed a large pancreatic cyst, multiple large hepatic cysts, congenital hepatic fibrosis, bilateral dysplastic kidneys, and dysplasia of the pancreas. These findings constitute a variant of Ivemark's syndrome of dysplasia of the pancreas, liver, and kidneys.