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Biomedical subjects

D Harms

Publications and source records attributed to D Harms.

At least 127 records · Page 7Linked to original sources

[Cystic space occupying lesion of the upper pole of the kidney in a newborn infant. Case report and differential diagnosis].

In a newborn female infant a cystic lesion in the upper pole of the right kidney was detected by sonography 4 weeks before an uneventful delivery. In the postnatal period the sonographic examination, IVP, CT, and the biochemical data as well were not able to classify definitely this lesion as a benign cyst, adrenal hemorrhage or cystic malignoma. Therefore a laparatomy was performed, which revealed a cystic neuroblastoma.

Diagnosis, Differential↗

[Frequency of odontogenic tumors in the growth period].

424 odontogenic tumors were diagnosed at the Department of Oral Pathology in Hamburg Eppendorf and the Department of Maxillofacial Surgery in Kiel between 1965 and 1989. 106 of these odontogenic tumors were found in patients of 16 years and under. More often than not the odontogenic tumors of this age group were complex and compound odontomas, totalling 67%. When comparing these with other odontogenic tumors it must be taken into consideration that--although included in the WHO classification--odontomas are often referred to as hamartomas and not real tumors, because they lack the typical features of tumors. Compared with the adult age group, the relative frequency of ameloblastic fibromas, ameloblastic fibro-odontomas and odontogenic myxomas was remarkable. Nevertheless, because of the great differences between the two age groups, direct comparisons of these findings with each other should be made with reservation.

Adolescent↗

Neuroblastoma screening: arguments from retrospective analysis of three German neuroblastoma trials.

The justification for a neuroblastoma screening program has been discussed controversially. The analysis of 701 patients of the German neuroblastoma trials NB 79, 82, and 85 provides additional information on this subject. The basis of our investigation was the good prognosis of stage I and II patients (92% survival 5-10 years after diagnosis) compared with 66% in stage III and 11% in metastatic disease. The correlation of age and stage (p less than 0.0001), a median progression time of 14.6 months (range 3.4-33.5 mo) from localized to metastatic disease as observed in 18 patients, the high incidence of asymptomatic diseases in stages I (49%) and II (30%) patients and the cost-benefit estimation arguments in favor of a screening program. The key problem for the lab part is the lower incidence of abnormal catecholamine metabolite excretion in stage I and II patients. The origin of 89% of metastatic disease from intraabdominal sites suggests that ultrasonography may be of additional value.

Abdomen↗

[Impulse cytophotometry determination of the DNA content of paraffin-fixed neuroblastoma].

The aim of the study was to develop a method to obtain a single cell suspension from paraffin fixated neuroblastoma tissue for measuring the DNA content by flow cytometry, and to detect possible correlation between the histological grading and the DNA stemline, and to relate these findings to the patients' prognosis. 51 samples of the fixated neuroblastoma tissue were measured. It was possible to determine the DNA content in 38 probes of the material. 79% of the evaluated samples showed aneuploid DNA stemlines, 21% showed euploid stemlines. Despite the small number of probes it was found, that in relation to the histological grading of neuroblastoma a lower grade of malignancy correlated with an euploid DNA content and a higher grade of malignancy correlated with an aneuploid DNA content. A correlation between DNA stemlines and the patients' prognosis was not found.

Adolescent↗

Simultaneous palsy of facial and vestibular nerve in a child with Lyme borreliosis.

We describe a boy with borreliosis characterized by lymphocytic meningitis and simultaneous palsy of facial and vestibular nerves on the left side. A mild sensoneural affection was also confirmed by brainstem evoked response audiometry. While symptoms of vestibular dysfunction quickly decreased during antibiotic treatment, facial palsy improved only slowly.

Child↗

Undifferentiated sarcoma of the liver in childhood: morphology, flow cytometry, and literature review.

Nine cases of undifferentiated sarcoma of the liver in childhood were investigated by conventional light microscopy, immunohistochemistry, and DNA flow cytometry. Different histologic patterns were discernible. In some cases, areas resembling poorly differentiated rhabdomyosarcoma were present. This histologic diversity was supported by the immunohistologic detection of desmin and cytokeratins in five cases. When evaluated by flow cytometry, four of five cases investigated were diploid; only one was aneuploid, and this patient had the most aggressive course. Follow-up investigations of our patients and those described in the literature revealed that the prognosis of this type of tumor is not as bad as is generally assumed. Of the patients reported in the literature with detailed follow-up information, 37.5% survived without evidence of disease for an average of 37.5 months, death occurred in 47.5% after an average of 11.9 months, and 15.0% were alive with disease.

Adolescent↗

Cytokeratin expression in malignant Triton tumor.

A malignant Triton tumor in a 9-year-old boy is described. The first biopsy which was taken from the thenar prominence was diagnosed as a monophasic fibrous synovial sarcoma based on the finding of a spindle cell neoplasm with plump nuclei and cytokeratin expression. The true nature of the tumor became apparent when a second biopsy was investigated. In this specimen a rhabdomyosarcomatous component was found in association with a spindle cell sarcoma fulfilling the criteria of a malignant schwannoma. Immunohistochemical staining using antibodies against vimentin, desmin, muscle-specific actin, cytokeratin, glial fibrillary acidic protein, protein S-100, Leu 7 and myoglobin served to distinguish the two tumor components and documented the possible reactivity of malignant Triton tumor for cytokeratins.

Biopsy↗

Neuroendocrine differentiation in hepatoblastoma. An immunohistochemical investigation.

Hepatoblastoma exhibits a wide range of epithelial and mesenchymal lines of differentiation. Neuroendocrine differentiation in this tumor has not previously been reported. We investigated seven hepatoblastomas of different subtypes (five pure epithelial hepatoblastomas, including one small-cell hepatoblastoma, and two mixed hepatoblastomas) using a broad panel of antibodies against epithelial, mesenchymal, neural, and neuroendocrine markers, alpha-1-antitrypsin (alpha 1-AT), alpha-1-antichymotrypsin (alpha 1-ACT), alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA), serotonin, and 14 regulatory peptides. Chromogranin A-immunoreactive neuroendocrine tumor cells, some of which also exhibited immunoreactivity for serotonin and somatostatin, were found in the fetal and embryonal parts of the mixed hepatoblastomas. The osteoid-like material in the mixed hepatoblastomas contained cells with immunoreactivity for chromogranin A, neuron-specific enolase, keratin, and alpha 1-AT, alpha 1-ACT, AFP, and CEA, in addition to S-100 protein and vimentin. Parallels to the neuroendocrine differentiation in hepatoblastomas are found in tumors of the gastrointestinal tract and bronchopulmonary tree. These tumors may also exhibit a neuroendocrine component; that is, multidirectional differentiation may occur, as in hepatoblastoma. The immunoreactivity of some of the cells of the osteoid-like material for keratin, alpha 1-AT, alpha 1-ACT, AFP, CEA, and chromogranin A suggests that these cells--and probably the surrounding material--are of epithelial origin.

Antibodies↗

[Plasma separation in acute, intravenous phenytoin poisoning].

A two year old boy suffered an acute phenytoin intoxication following intravenous treatment of seizures. After two days in coma detoxification with plasma separation was started. The course of clinical symptoms and phenytoin elimination is described and the efficacy of the method in treating phenytoin intoxication is discussed.

Dose-Response Relationship, Drug↗

Soft tissue sarcomas in infants younger than 1 year of age: a report of the German Soft Tissue Sarcoma Study Group (CWS-81).

Of 357 patients with soft tissue sarcomas (STSs) who were registered between 1981 and 1986 in the CWS-81 study, 21 (5.8%) were younger than 1 year of age. Six cases were probably of congenital origin since they were diagnosed in the 1st month of life. There was no difference in clinical grouping (staging and primary site) between infants and older patients. The major histological subsets comprised 12 patients with embryonal rhabdomyosarcoma (57% vs. 44% in older patients) and five patients with fibrosarcoma (23% vs. 2% in older patients). Of 21 patients, 17 received chemotherapy. The response rate (tumor reduction more than 1/3) to exclusive cytostatics therapy within 7-9 weeks, of ten evaluable patients, was 100% (vs. 86% in older patients). Eight of the responders achieved complete remission after 16 weeks of therapy and are in continuous complete remission (CCR). Most patients received 20-50% of the recommended chemotherapy dose for older children, which was calculated using body surface area. No fatal or life-threatening complications were observed. The event-free survival rate for children younger than 1 year of age with rhabdomyosarcoma was 70% vs. 47% in older children (no significant difference). Now, 2-5 years after therapy all five fibrosarcoma patients are alive and free of disease. Our data indicate that children under 1 year of age with rhabdomyosarcoma have a slightly better prognosis than older children, when therapy modalities are adapted to age.

Age Factors↗

Combination chemotherapy in malignant non-seminomatous germ-cell tumors: results of a cooperative study of the German Society of Pediatric Oncology (MAKEI 83).

In January 1983, the German Society of Pediatric Oncology started a cooperative trial (MAKEI 83) for non-testicular germ-cell tumors. The pilot phase closed in December 1985. The treatment regimen was stratified according to histology, tumor site and tumor stage. In malignant non-seminomatous germ-cell tumors (mNSGCTs), chemotherapy consisted of four courses of 3 mg/m2 vinblastine, on days 1 and 2 and 15 mg/m2 bleomycin on days 1-3, given by continuous infusion, and 20 mg/m2 cisplatin on days 4-8 with mannitol diuresis. Courses were repeated every 3 weeks. In mNSGCT patients with ovarian FIGO stages III-IV or extragonadal primaries, second-look surgery was carried out, followed by four additional courses of chemotherapy with 100 mg/m2 VP-16 on days 1-3, 1.5 g/m2 ifosfamide on days 1-5 with mesna uroprotection and 20 mg/m2 cisplatin on days 1-5 with mannitol diuresis. In patients with sacrococcygeal germ-cell tumors, en bloc resection of the tumor, including the coccygeal bone, was mandatory. During the registration period, 57 patients with mNSGCTs were entered: 37 protocol patients and 20 follow-up patients. The event-free survival for protocol patients at 57 months was 78% +/- 6% and that for follow-up patients was 40% +/- 10% (Kaplan-Meier): the crude survival for both groups was 83% +/- 6% and 54% +/- 12%, respectively. After a review by a panel of pathologists, the histological diagnoses in 7% of all registered cases of germ-cell tumors were changed. The results of the present studies show that the histological subclassification of mNSGCTs, tumor site and tumor stage no longer had prognostic value.

Adolescent↗

Abdominal, retroperitoneal and sacrococcygeal tumours of the newborn and the very young infant. Report from the Kiel Paediatric Tumour Registry.

We examined 226 abdominal, retroperitoneal and sacrococcygeal tumours in newborns and infants aged 6 months or less. Most frequent were neuroblastomas (n = 83) followed by germ cell tumours (n = 76), 37 of which were immature and 32 were mature teratomas. Fully malignant germ cell tumours and malignant germ cell tumour components were much rarer in this age group (9.2%) than in older children (58.1%). The majority of germ cell tumours were localized in the sacrococcygeal region (72.4%). Next in frequency were tumours of the kidney (n = 54), including 28 congenital mesoblastic nephromas. In contrast to children over 6 months of age, kidney tumours were frequently low grade malignant with a favourable prognosis. Among liver tumours there were 19 infantile haemangioendotheliomas and 9 hepatoblastomas. By contrast, at ages 7-24 months hepatoblastomas (n = 28) were much more frequent than haemangioendotheliomas (n = 2). Tumours in the newborn and very young infant differ from those in older children in many respects: (1) the relative frequency; (2) localization; (3) distribution of histological types; (4) degree of differentiation, often associated with a tendency toward maturation but occasionally also with increasing malignancy; and (5) prognosis, which is comparatively favourable.

Abdominal Neoplasms↗

Gliomatosis peritonei in childhood and adolescence. Clinicopathological study of 13 cases including immunohistochemical findings.

Gliomatosis peritonei (GP) can be defined as the metastatic implantation of neural tumor tissue on surfaces of the peritoneum in patients with immature ovarian teratomas. Data from 13 patients with GP were evaluated. The average age at time of biopsy was 11 years (median: 11.5 years; range: 2.9 to 18.6 years); average time of follow-up was 26.6 months (median: 22 months; range: 4 to 77 months). In 11/13 patients the GP was discovered at the same time as the primary tumor, in two patients it was detected at second-look surgery. Degree of maturity according to scheme of Robboy and Scully was 8 x G 1, 4 x G 2, and 1 x G 3 for the ovarian teratomas (x = 1.46), and 11 x G 0 and 2 x G 3 for the GP implants (x = 0.46). Light microscopically, the mature glial nodules consisted almost exclusively of GFAP and S-100 protein positive astroglia. Twelve of the 13 patients are alive and clinically healthy. One patient with metachronic immature GP is receiving chemotherapy. In general, the prognosis for GP is good: it depends chiefly on the degree of maturity of the implants. In mature GP, usually no additional chemotherapy is necessary; in immature GP, chemotherapy can induce maturation of the implants.

Adolescent↗

Malignant rhabdoid tumor. A morphological and flow cytometric study.

Sixteen cases of malignant rhabdoid tumor (MRT) were studied by conventional light microscopy, immunohistochemistry, electron microscopy and flow cytometry. The age of the 16 patients varied from two months to 25.9 years. There were 11 males and five females. Eleven tumors were located in the kidney. The remaining five were found in the chest wall (n = 2) and the head and neck (n = 3). Particular histopathological findings included myxoid, pseudoalveolar and hyalinized areas. By immunohistochemistry, 15/15 cases stained positively for vimentin, 9/14 for cytokeratin, 6/15 for desmin, 9/14 for epithelial membrane antigen (EMA), 10/14 for neuron specific enolase (NSE) and 10/15 for protein S-100. Stains for neurofilaments, myoglobin and Ulex europaeus aggl. I (UEA I) were negative. The characteristic finding by electron microscopy in three cases were large numbers of intermediate filaments arranged either randomly or in concentric whorls. None of the 11 cases studied revealed aneuploid DNA stem lines as determined by flow cytometry. Of the 16 patients 12 died, one is living with disease and three are living without evidence of disease. Postoperative treatment consisted of chemotherapy, in some cases combined with radiotherapy. Two patients developed a medulloblastoma in addition to a renal and extrarenal MRT, respectively. Our findings demonstrate that MRT may present more histopathological patterns than hitherto recognized. In addition, they show that MRT may express a wide range of antigenic "markers", similar to epithelioid sarcoma with which it may be confused on cytological grounds. Despite aggressive postoperative chemotherapy prognosis is still poor.

Adolescent↗

[Dysgerminomas. Clinical aspects, therapy and prognosis with reference to the cooperative therapy study MAIKEI 83/86 for non-testicular germ cell tumors of the Society of Pediatric Oncology].

In comparison to other malignant germ cell tumors, dysgerminomas of the ovary show a high sensitivity to radiotherapy. According to histology and biological behaviour, dysgerminomas of the ovary are similar to testicular seminomas, which seem to be very sensitive to chemotherapy. Therefore, in the protocol for non-testicular germ cell tumors of the German Society of Pediatric Oncology (GPO) a treatment for dysgerminomas is established, in which the indication for primary tumor resection and a supplementary radiotherapy, or, in selected cases, primary chemotherapy and delayed tumor resection after the spreading, has been graded. After a median follow-up of 26 months, 17 of 18 patients were alive and disease-free, from which 3 patients with tumor progression after initial ovariectomy are in the second remission phase following relapse therapy. One patient died of tumor progression despite combined chemotherapy and irradiation. With a stratified therapy regime, fertility could be preserved with high probability in 13 of 17 patients.

Adolescent↗

[New aspects of nephroblastoma (Wilms tumor) and other metanephrogenic neoplasms].

We differentiate (continuing the scheme of the National Wilms' Tumor Study) three groups of Wilms' tumors (WT), which for practical reasons also encompass WT variants: 1. a group of low-grade malignant tumors comprising 9.3% of cases (congenital mesoblasticnephroma; cystic, partially differentiated nephroblastoma); 2. the main group of tumors with histologically standard malignancy and constituting 77.7% of cases, against which a combined therapy is used depending upon age and stage of spread; and 3. a small group of tumors of high-grade malignancy (anaplastic WT, clear cell sarcoma of the kidney; malignant rhabdoid tumor of the kidney). This latter group constitutes only 13% of cases but is responsible for a high percentage of total deaths due to WT. To the tumor group with standard malignancy belong the classic triphasic WT (without anaplasia) as well as WT "with quantitative deviations", in which either the blastemic, the epithelial, or the stroma component dominates. In the differential diagnosis the relatively frequent blastemic WT must be differentiated from other so-called small, round, and blue cell tumors of childhood, especially the undifferentiated neuroblastomas. Pseudo-rosettes and cytokeratin expression are signs of an "early" epithelial differentiation. Anaplastic WT (comprising 6.1% of our cases) are diagnosed according to the criteria of BECKWITH and PALMER (1978). They are aneuploid tumors and occur predominantly in children over two years of age. By contrast, the histogenetic still undefined clear cell sarcomas and malignant rhabdoid tumors of the kidney occur chiefly in children under two. Both tumors are diploid, notwithstanding their high-grade malignancy. Clear cell sarcoma, which contains a high content of vessels and comprises 3.7% of our cases, consists of cells with weakly stained and partially vacuolized cytoplasm. In contrast to normal WT, clear cell sarcoma often progresses to bone metastases. Malignant rhabdoid tumor (2.7% of our cases) possesses cells with large, roundish nuclei, pale chromatin, very prominent nucleoli and characteristic spherical intermediate filament condensations. Cross striation and myoglobin are never present. Malignant rhabdoid tumor is not a genuine kidney tumor; it may also occur extrarenally. Low-grade congenital mesoblastic nephroma is a spindel cell tumor often exhibiting high cellularity and characterized by fingerlike projections extending into the adjacent kidney tissue. Nephroblastomatosis, with preferential perilobular localization, is a potential WT precursor found in 25% to 40% of all nephrectomy specimens containing WT and in all cases of bilateral and multifocal WT. Nevertheless, only a small proportion of the usually very small "nephrogenic remnants" lead by way of an adenomatous proliferation to manifestation of WT.(ABSTRACT TRUNCATED AT 400 WORDS)

Adenocarcinoma↗