[Histology of solid tumors and its significance for therapy].
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Child↗
Biomedical subjects
D Harms
Publications and source records attributed to D Harms.
Flow cytometric analysis of nephroblastomas and related neoplasms.
A total of 59 cases of nephroblastoma and related neoplasms were studied by flow cytometry of paraffin-embedded tissue. According to clinical prognosis, cases were subdivided into three groups: Group 1 (low risk) consisted of congenital mesoblastic nephroma (n = 13) and cystic, partially differentiated nephroblastoma (n = 2). Group 2 (intermediate risk) comprised the various subtypes of "typical" nephroblastoma (n = 24) including cases of fetal rhabdomyomatous nephroblastoma (n = 4). In group 3 (high risk) there were cases of anaplastic nephroblastoma (n = 3), clear cell sarcoma of the kidney or "bone metastasizing renal tumor of childhood" (n = 7), and malignant rhabdoid tumor of the kidney (n = 6). The three clinically different groups of tumors also varied in the proportion of cases with aneuploid tumor DNA stemlines, in S-phase fractions, and in proliferation indices (PI = S + G2 + M). Group 1 was generally characterized by a small number of cases with aneuploid tumor DNA stemlines and low values for S-phase fractions and PI, whereas Group 3 showed the largest number of cases with aneuploid tumor DNA stemlines and high values for S-phase fractions and PI. Group 2 was in between. It is concluded that flow cytometry on paraffin-embedded tissue from pediatric tumors may be a useful adjunct in determining prognosis, and that the subdivision of nephroblastomas and related neoplasms into three prognostically different groups is warranted.
Aneuploidy↗
[Embryonal malformation following etretinate therapy of Darier's disease in the mother].
A 22-year-old woman suffering from dyskeratosis follicularis Darier became pregnant 4 months after the last dose of etretinate (Tigason). The pregnancy was terminated in the 10th week because of teratogenic risk. The pathological examination of the fetus revealed severe malformation of the left leg with aplasia of tibia and fibula, as well as hypoplasia of the left femur. This observation emphasises the necessity of effective contraception even after discontinuation of etretinate therapy.
Abnormalities, Drug-Induced↗
Cellular differentiation and prognosis in embryonal rhabdomyosarcoma. A report from the Cooperative Soft Tissue Sarcoma Study 1981 (CWS 81).
Sixty-four cases of embryonal rhabdomyosarcoma (eRMS) were investigated for cellular differentiation by light microscopy. Of these 64 cases 20 were studied by means of immunohistochemistry. Histologically, three subgroups could be distinguished: primitive (less than 10% rhabdomyoblasts), intermediate (10-50% rhabdomyoblasts) and well differentiated (greater than 50% rhabdomyoblasts) eRMS. Vimentin-positive cells predominated in the primitive eRMS. Intermediate eRMS showed large proportions of desmin-positive cells but vimentin containing cells were also numerous. Myoglobin could only be demonstrated in well differentiated eRMS. Primitive and well differentiated eRMS mainly occurred in the head and neck area, whereas intermediate eRMS were predominantly located in the abdomen. Stage III and IV tumours predominated in cases of primitive eRMS, whereas lower stages were noted in cases of intermediate and well differentiated eRMS. Response to chemotherapy, evaluated after seven weeks of treatment, was achieved in 10/15 (66%) cases of primitive, in 16/19 (84%) cases of intermediate and 5/5 cases of well differentiated eRMS. It is concluded from the current study that the three subgroups of eRMS differ not only by cytological differentiation but also by site of predilection, stage at time of diagnosis and response to chemotherapy.
Abdominal Neoplasms↗
Germ cell tumours of childhood. Report of 170 cases including 59 pure and partial yolk-sac tumours.
170 germ cell tumours of childhood and adolescence were studied by light microscopy and immunohistochemistry. The male-to-female ratio was 1:1.3. 52 (30.6%) tumours were benign (mature teratoma), 30 (17.6%) potentially malignant (immature teratoma), and 88 (51.8%) unequivocally malignant. The main locations were ovary, testis and sacrococcygeal region. 92 tumours were located in a gonad, 78 tumours in extragonadal sites (ratio: 1.2:1). Of the frankly malignant tumours 40 were yolk-sac tumours (YST) and an additional 19 tumours of more than one histological type contained a YST component. Therefore, 67% of the malignant tumours had a YST component. Children with immature teratoma and pure YST showed the lowest median age (5 and 24 months, respectively), while children with germinomas of various locations had the highest median age (153 months). A festoon pattern was the predominant histological feature in all YST and in the YST component of mixed germ cell tumours. Hyaline globules were found in 33/37 YST and in 16/17 YST components. Immunohistochemically, alpha 1-fetoprotein (AFP) was demonstrated in 18/22 YST and in 6/7 YST components of mixed germ cell tumours. Hyaline globules were mostly AFP-negative (only 5 cases with AFP-positive globules in addition to many AFP-negative globules). In 3 cases beta-HCG-positive giant cells were seen. In most YST prekeratin intermediate filaments could be demonstrated in the epithelial cells. Follow-up data, available from 51 cases of YST and tumours with YST components showed disease-free survival in 37 cases (72.5%). 10 patients (19.6%) died of disease, and 4 patients (7.8%) are living with disease. The comparably high rate of survivors reflects the effectiveness of modern therapy, particularly polychemotherapy, in addition to surgery.
Adolescent↗
Abdominal recurrences in Wilms' tumours: a report from the SIOP Wilms' tumour trials and studies.
The Wilms' tumour trials and studies conducted from 1971 to 1980 registered 1042 patients. Of these, 82 patients developed an abdominal recurrence. Particulars of these were studied. Half of the recurrences occurred in stage III patients. Often several untoward prognostic factors could be identified, such as large tumour size, difficult operation, incomplete excision, peritoneal adhesions or metastases, tumour extending to renal vein or vena cava. A tumour rupture increases the chance for an abdominal recurrence, especially if appropriate radiotherapy is not given. In many of these cases, postoperative radiotherapy seems to have been insufficiently tailored to the operative findings. For stage III cases, a careful discussion between surgeon, radiotherapist, and pathologist should lead to the optimal radiotherapy field size and dose for each individual patient, so that the risk of abdominal recurrence can be reduced.
Abdominal Neoplasms↗
[Special tumors of childhood].
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Antigens, Neoplasm↗
Bone metastasizing renal tumor (clear cell sarcoma) of childhood with epithelioid elements.
A case of bone metastasizing renal tumor (clear cell sarcoma) of childhood (BMRTC) is presented. Histologic examination demonstrated the typical histopathologic features of this type of tumor. In addition, epithelial cells were present which occurred in groups, either around a central eosinophilic core or occasionally around a small central lumen. To the authors' knowledge, the presence of epithelial cells in BMRTC has not been reported before. The histogenetic implication of this finding is discussed.
Bone Neoplasms↗
Malformation of fetus conceived 4 months after termination of maternal etretinate treatment.
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Abnormalities, Drug-Induced↗
Visual reaction times may be improved by certain beta-blockers.
The effects of the beta-blocker atenolol on vigilance, concentration, and psychophysiological condition were determined by measuring visual reaction time and choice reaction time in volunteers subjected to combined physical and mental stress. Atenolol 50 mg or placebo were administered for three days in a double-blind crossover design. Forty male subjects aged 26 +/- 6 years with a mean blood pressure of 126/79 mmHg and increased activity of the sympatheticus nerve were admitted to the first study. Physical workload was imposed using a training ergometer for ten minutes and mental workload was introduced by asking the subjects to calculate additions and subtractions on mechanical equipment. Electrooculography was used to measure visual reaction time. Fifty subjects aged 32 +/- 9 years with a mean blood pressure of 141/90 mmHg were admitted to a second study. The same crossover design was followed, but a different mental workload was imposed; subjects were required to respond to coloured light signals which were presented at intervals of 0.8 seconds, by pressing the appropriate buttons. In this second study visual reaction time was also assessed by electrooculography whereas choice reaction time was defined as the time between the lighting of a signal (in one of five colours on 1-25 different places on a screen) and its extinction by pressing an appropriate coloured button. As expected, blood pressure and heart rate decreased with atenolol. In the first study, in both periods, visual reaction time was higher with placebo than with atenolol. The effect of treatment was small but highly significant (p = 0.004).(ABSTRACT TRUNCATED AT 250 WORDS)
Adrenergic beta-Antagonists↗
Malignant peripheral neuroectodermal tumours of childhood and adolescence.
Seventeen cases of malignant peripheral neuroectodermal tumour (MPNT) were studied by means of light microscopy, immunohistochemistry and electron microscopy. There were nine males and eight females. The mean age of the 17 patients was 10 years with a range of seven months to 20 years. The vast majority of tumours was located in the trunk. Histologically, they closely resembled Ewing's sarcoma, although minor differences were obvious. Special findings included ganglion cells and Flexner rosettes. In 10/11 cases positive staining for neuron-specific enolase (NSE) was obtained. Five of 10 tumours were positive for protein S-100. Three contained vimentin, two neurofilaments and one vimentin, neurofilaments and GFAP. Neurosecretory granules were noted in the three cases studied. Five patients died, three are alive with disease and five patients are alive without evidence of disease. It is concluded that these tumours form a homogeneous group, although the grade of differentiation varies. The prognosis in most cases is poor. Distinction from Ewing's sarcoma is possible by staining for NSE and by electron microscopy.
Adolescent↗
Primary malignant hepatic tumours in childhood.
Twenty-four cases of hepatoblastoma, 14 cases of hepatocellular carcinoma and three cases of malignant mesenchymoma out of a total of 54 primary liver tumours were studied by light microscopy and immunohistochemistry. A remarkable finding in one case of hepatoblastoma and one case of hepatocellular carcinoma was a sarcoid-like reaction in the tumour tissue. Three cases of hepatoblastoma presented a macrotrabecular pattern. Among hepatocellular carcinomas, three cases corresponded to the fibrolamellar variant. By immunohistochemistry, the proportion of cases with positive staining for alpha 1-fetoprotein was higher in hepatoblastoma than in hepatocellular carcinoma. HBs-antigen could be demonstrated in non-neoplastic liver cells in two cases of hepatocellular carcinoma, but not in the tumour cells. No strong correlation between histological pattern and prognosis could be established in hepatoblastoma. However, there was a tendency to more aggressive biological behavior in cases with pronounced mitotic activity. The number of mitoses in hepatoblastoma varied widely. As in previous studies, patients with the fibrolamellar variant of hepatocellular carcinoma fared better than those with the classical type of this tumour. Prognosis in malignant mesenchymoma was not as poor as suggested from previous studies.
Carcinoma, Hepatocellular↗
Immature teratomas of childhood. Report of 21 cases.
The benign or malignant nature and the biological behaviour of immature teratomas of childhood are difficult to predict. The age of the patient at diagnosis, the anatomical site of the tumour and the degree of immaturity are considered to be important prognostic parameters. In this study the pathological-anatomical findings and the most important clinical features of 21 patients with immature teratoma (including two with supposedly malignant monodermal teratoma with immature neuroectodermal structures) were evaluated. Significant results were: Twelve tumours occurred in infancy or infants who died in the perinatal period, the other nine tumours in children between the ages of 7 and 16 years. The most frequent anatomical sites were the ovary (6 tumours), sacrococcygeal region (4), testis (4) and mediastinum (3). In contrast to most of the tumours of other localizations, immature ovarian teratomas did not occur in children under 7 years of age (in four cases in association with gliomatosis peritonei). The immature tissue components of the tumours were mostly neuroectodermal structures. Eight tumour specimens showed grade 1, four grade 2 and nine grade 3 malignancy. Grade 3 tridermal teratomas chiefly occurred in young children, whereas two grade 3 monodermal tumours developed in older children. Immunohistochemical analysis of the neuroectodermal components showed that mature astrocytes contained glial fibrillary acid protein, whereas mature nerve cells, nerve fibres and a few groups of immature cells reacted with an antibody to neuron-specific enolase. Six of the 21 patients died; two were stillborn immature infants, two were premature infants, one died postoperatively and one died of metastatic disease. One patient with metastatic disease was alive. None of the 19 children with tridermal immature teratoma showed distant metastases. Metastatic disease was observed in only two patients with presumptive monodermal malignant teratoma. In early childhood the biological behaviour of immature teratomas is evidently similar to that of mature teratomas (provided that the tumour can be totally excised). In older children malignancy must be assumed when the tumour is located in the ovary and/or grade 3 immaturity is determined.
Adolescent↗
Fibromatosis of infancy and childhood. Histology, ultrastructure and clinicopathologic correlation.
Fifty-nine cases of fibromatosis in children were investigated. To our knowledge, this is the largest serie with clinicopathologic datas reported so far in German literature. The most frequent type was infantile (desmoid-type) fibromatosis. More than 50% of the tumours developed in the first five years of life. The overall male-to-female ratio was 1.36:1. The lower extremities were the most frequent site of manifestation. Vimentin could be demonstrated in the tumour cells by immunohistochemical methods. Positive staining for dipeptidylaminopeptidase (DAP) IV suggested a myofibroblastic nature of some of the neoplastic cells. These results were supported by ultrastructural examination. Two cases demonstrated the unpredictable biological behaviour of fibromatosis. It is emphasized that clinical behaviour depends on number, size, location and histologic appearance of the lesions as well as age of the patient.
Arm↗
Soft-tissue sarcomas in childhood. A study of 262 cases including 169 cases of rhabdomyosarcoma.
Although numerous investigations of soft-tissue sarcomas of childhood have been done, there are still many unsolved problems in the diagnosis, histogenesis, and biological behaviour of such tumours. In the present study, the soft-tissue sarcomas collected at the Paediatric Tumour Registry in Kiel were investigated. There was a total of 262 cases, including 169 (64.5%) in which a definite diagnosis of rhabdomyosarcoma had been made. An analysis of the age distribution showed that rhabdomyosarcoma is more than three times as frequent as non-rhabdomyosarcomatous soft-tissue tumours in the first quinquennium. Rhabdomyosarcoma exhibited male predominance (male-to-female ratio 1.45:1), whereas non-rhabdomyosarcomatous tumours showed an approximately equal sex distribution. Among the cases of rhabdomyosarcoma, the embryonal type clearly predominated (approx. 70%); the alveolar type was less common (approx. 20%). Correlation of histology with prognosis revealed that even tumours showing tiny foci with an alveolar pattern in otherwise solid, undifferentiated rhabdomyosarcoma, must be classified as alveolar rhabdomyosarcoma. Cases of alveolar rhabdomyosarcoma showed a higher incidence of local recurrence, lymph node metastasis, distant metastatic spread, and death, than did cases of embryonal rhabdomyosarcoma. It is concluded that "cytological differentiation" does not improve the prognosis of rhabdomyosarcoma.
Adolescent↗
[Studies using Ulex europaeus agglutinin I (UEA I) on normal tissue and synovial sarcomas, mesotheliomas and carcinomas].
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Adenocarcinoma↗
Acute hemorrhagic conjunctivitis in Central America: first enterovirus epidemic in the western hemisphere.
Acute hemorrhagic conjunctivitis (AHC) is an eye infection that was first noted in 1969 and became a pandemic disease in Africa, Southeast Asia, and Japan in the 1970s. It was not noted in the western hemisphere until 1981. This report describes the clinical experience with AHC in Honduras where it was first seen in the western hemisphere. Clinical presentation includes acute onset with symptoms of foreign body sensation, burning, and photophobia. Signs include lid edema, periauricular lymphadenopathy, conjunctival injection, follicular reaction, and typically subconjunctival hemorrhages. Since AHC appears to be a very contagious conjunctivitis, it can spread rapidly and result in a high proportion of the population becoming infected. There is no known specific treatment for this disease, and containment includes increased attention to hygiene.
Acute Disease↗
Light- and electron-microscopic studies in congenital pseudarthrosis.
This study presents the results of light- and electron-microscopic and enzyme histochemical investigations in ten cases of congenital pseudarthrosis of the lower limb. At the time of surgery, six of the ten patients had not been operated on previously. The characteristic histological feature of the "sclerotic type" of congenital pseudarthrosis was a marked fibromatous reaction consisting of cellular connective tissue. The constituent cells were arranged in bundles and had elongated nuclei. The number of nuclei per visual field was considerably higher in pathological specimens than in specimens from the uninvolved leg. In places, the histological appearance resembled somewhat that of palmar fibromatosis (Dupuytren's disease). Destruction and absorption of bone were always found. Electron-microscopic analysis showed that a large number of the cells represented myofibroblasts. These findings were supported by the positive reaction of the cells for the enzyme diaminopeptidase IV, a marker enzyme for myofibroblasts [30]. As yet it is not possible to decide whether the constriction of the pseudarthritic bone is caused by a thickened myofibroblast-containing periosteum [40] or by the aggressive osteolytic component of the fibromatosis [12, 19, 41]. Furthermore, the relationship of congenital pseudarthrosis to fibrous dysplasia of bone is still unknown. Obviously, there are histological similarities between the two diseases, including the presence of osteolytic fibrous tissue in the medullary cavity and C-shaped bone trabeculae. However, the pattern of bone involvement and prognosis are different. Irrespective of the type of congenital pseudarthrosis, focal angiomatous hyperplasia was noted in some cases. This proliferation of blood vessels is most likely a reactive change.(ABSTRACT TRUNCATED AT 250 WORDS)
Bone and Bones↗