Caring for a patient with an A.I.C.D.
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Arrhythmias, Cardiac↗
Biomedical subjects
C Fisher
Publications and source records attributed to C Fisher.
Abnormalities of epidermal differentiation associated with expression of the human papillomavirus type 1 early region in transgenic mice.
The promoter region of a keratin 6 (K6) gene was used to regulate expression of the early region of human papillomavirus type 1 (HPV-1e) in transgenic mice. In one line of mice the K6-HPV1e transgene was transcribed in several regions of the skin, the predominant transcript being a 1.1 kb RNA including the E4 open reading frame, and E1-E4 protein was detected in the upper suprabasal layers of the skin in paws and tail. A 1.7 kb RNA corresponding to the E6/E7 transcript was also prominent in tails of homozygous transgenic animals. In young homozygous transgenic mice the epidermis of the tail showed dysplasia and hyperplasia of the suprabasal layers with both hyperkeratosis and focal parakeratosis in the stratum corneum. A similar though milder phenotype was also observed sporadically in hemizygous transgenics. Analysis of the pattern of mouse keratins present in the affected tail skin showed strong up-regulation of the endogenous keratins 6 and 16 throughout the basal and suprabasal layers, suggesting a positive feedback mechanism for the strong transgene activation. Expression of the major differentiation-specific keratins 1 and 10 was repressed. The pattern of E1-E4 expression and the perturbation of normal epithelial differentiation parallel many of the characteristics of HPV-1 warts or verrucae, suggesting that HPV transgenic mice could be useful for analysis of the interactions of HPV gene products with cellular regulatory pathways within an otherwise normal epithelium.
Animals↗
Chylous ascites in Kaposi's sarcoma: a case report.
We report a case of a patient with Kaposi's sarcoma (KS), massive chylous ascites and chylous pleural effusions. This association has not been reported previously. The pathogenesis of chylous effusions is discussed with respect to KS.
Chylous Ascites↗
Peripheral nerve sheath differentiation in malignant soft tissue tumours: an ultrastructural and immunohistochemical study.
Thirteen soft tissue sarcomas with ultrastructural evidence of nerve sheath differentiation were investigated by immunohistochemistry. Three arose in a major nerve or nerve trunk and four patients had von Recklinghausen's neurofibromatosis. Ultrastructurally, 10 cases showed variable differentiation towards Schwann cells, two resembled perineurial cells and one tumour had features suggestive of both cell types. Immunostaining for S-100 protein was positive in eight Schwann cell tumours, negative in the other two Schwann cell tumours and negative in those with perineurial-like cells. No cases demonstrated epithelial membrane antigen, so that the existence of perineurial cells in malignant nerve sheath tumours remains immunohistochemically unsubstantiated; it may be that the perineurial-like cells are merely incompletely differentiated Schwann cells, with nerve sheath differentiation manifesting a continuous spectrum. Leu 7 was detected in four Schwann cell tumours, three of which were ultrastructurally well differentiated. Cytokeratin and desmin were demonstrated only in an undifferentiated pleomorphic area of one Schwann cell tumour. Electronmicroscopy can aid diagnosis by revealing nerve sheath differentiation in malignant soft tissue tumours without demonstrable S-100 protein.
Adolescent↗
Proliferating cell nuclear antigen and S phase fraction in endometrial stromal sarcoma.
AIMS: To investigate the value of immunohistochemical staining for the cell cycle protein proliferating cell nuclear antigen (PCNA) and flow cytometric S phase fraction in determining prognosis in endometrial stromal sarcoma, graded according to mitotic count. METHODS: Seventeen endometrial stromal sarcomas from 13 patients treated at the Royal Marsden Hospital were analysed. Serial 5 microns sections were cut for haematoxylin and eosin and immunohistochemical staining for PCNA, performed using the murine monoclonal antibody PC10. PCNA positivity was expressed as a percentage of the total number of cells (PCNA index). Flow cytometric analysis was performed on nuclei extracted from paraffin wax sections. RESULTS: In the five patients who died of disease within five years, PCNA index varied between < 1% and 60% (mean 21%) and S phase fraction ranged from 11.3 and 20.1 (mean 13.8). Four patients who were apparently cured showed PCNA indices ranging from < 1% to 5% (mean 1.75%) and S phase fraction ranging from 1.4 to 3.5 (mean 2.3); and three patients alive with disease showed PCNA indices ranging from 1% to 15% (mean 8.6%) and S phase fraction ranging from 1.4 to 3.5 (mean 2.3). One patient who died from indolent local disease after nine years showed a PCNA of 1 or less and an S phase fraction of 0.9. CONCLUSIONS: PCNA staining was variable and therefore not a reliable prognostic indicator, but a high PCNA index was only found in those patients dying of disease within five years. A stronger association was seen between S phase fraction and prognosis; this also correlated well with histological grade determined by mitotic count. In individual borderline cases that are between low and high grade categories, these procedures may be useful.
Antigens, Neoplasm↗
Review of studies of risk and protective factors for psychopathology in children.
Child psychiatry is increasingly focusing on early identification of children at risk and the importance of prevention. Our review attempts to summarize recent studies regarding risk and protective factors in children and address the areas of difficulty inherent in this type of research. Recommendations for future research study design will be discussed.
Child↗
What to do with the biopsy specimen--practice in an oncology setting.
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Biopsy↗
Biotech International Ltd.
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Antibodies, Monoclonal↗
The diagnosis of soft tissue tumours.
We prospectively analysed methods of diagnosis in 118 patients referred for definitive treatment with documented or presumed soft tissue sarcoma (STS). Of 65 patients with primary STS, 54 were biopsied before referral. Of these, 5 (9%) were biopsied by Tru-cut biopsy, 17 (32%) by incisional biopsy and 32 (59%) by excisional biopsy. The remaining 11 patients with primary STS, referred without biopsy, were all diagnosed by Tru-cut biopsy. An additional eight patients suspected of having STS were referred without biopsy and were found to have malignant tumours other than STS involving soft tissue by Tru-cut biopsy. Nineteen patients were proved to have benign soft tissue tumours; in 13 presumed to have STS, the diagnosis was unknown at referral. In four of these, biopsy was inappropriate. Of nine submitted to Tru-cut biopsy, an unequivocal diagnosis was made in 5 (56%) and incisional biopsy was required in the other four. Therefore, paradoxically, benign soft tissue tumours may be more difficult to diagnose with Tru-cut biopsy than malignant tumours. This study confirms the high degree of accuracy of Tru-cut biopsy in diagnosing malignant soft tissue tumours and highlights the disadvantages of open biopsy techniques.
Adolescent↗
Aggressive angiomyxoma. A report of four cases occurring in men.
Aggressive angiomyxoma is a distinctive, locally aggressive but nonmetastasizing soft tissue tumor of the pelvic soft tissues and perineum. This rare tumor occurs almost exclusively in adult women. We report four cases occurring in adult men, one each located in the scrotum, inguinal region, spermatic cord, and pelvis. The tumors were infiltrative, and were composed of fibromyxoid matrix sparsely populated by bland-looking spindled and stellate cells with delicate cytoplasmic processes. There were haphazardly scattered small and large blood vessels, some of which exhibited hypertrophy or hyalinization of the wall. Immunohistochemically, the stromal cells stained consistently for vimentin and variably for muscle-specific actin, but not alpha-smooth muscle actin, desmin, and S-100 protein. None of the four tumors recurred in follow-up times from 11 months to 6 years, although two previously reported cases in men recurred. This uncommon tumor occurring around the genital region in men merits wider recognition because of its potential for recurrence. It should be distinguished from benign tumors with low risk of recurrence on one hand, and from malignant myxoid tumors with metastatic potential on the other.
Adult↗
The effect of elastic compression on the venous tone in patients with varicose veins.
The long term effect of elastic compression, with an emphasis on the changes in venous wall elasticity, was studied in 10 limbs with varicose veins. By simultaneously measuring venous volume, using the air plethysmograph (APG), and venous pressure, while the venous outflow was occluded, the pressure/volume (P/V) relationship of the venous system of the leg could be studied and the elastic modulus calculated. These measurements were performed prior to the application of elastic stockings and 6 weeks later, one day after the stockings had been removed. Statistically significant improvements were noted in the APG parameters studied and in the P/V curve derived elastic modulus calculations. The limbs could be divided into two groups based on the response of the P/V curves to elastic stockings and the severity of disease. Those limbs with milder disease tended to shift their P/V curves downward and to the right after compression (giving them a more normal pattern). Limbs with more severe disease showed evidence of a similar pattern of improvement at low but not at high venous pressures.
Bandages↗
Endometrioid carcinoma of the prostate: a misnomer?
The origin of this unusual variant of carcinoma of the prostate has provoked discussion ever since its first description in 1967. This is of both embryological interest and therapeutic importance. Four cases have been reviewed, and all have demonstrated immunohistochemical features consistent with an origin from prostatic tissue. In addition, three had evidence of disseminated disease which responded well to androgen ablation. It is concluded that the term endometrioid carcinoma is of descriptive value only, and these tumours are a variant of primary duct prostatic carcinoma. Patients should be treated by androgen ablation when metastases are present.
Adenocarcinoma↗
Limitations of surgery in the treatment of retroperitoneal sarcoma.
A retrospective analysis was undertaken of 120 patients with retroperitoneal sarcoma referred to the Royal Marsden Hospital over a period of 20 years. The actuarial 5-year survival rate of all cases following referral was 29 per cent. On univariate and multivariate analysis the principal factors associated with an unfavourable prognosis were the presence of metastases, poor performance status at presentation, high tumour grade and incomplete excision of the primary tumour. The single most important factor affecting the ability to remove the primary tumour completely was multiple organ involvement. After apparently complete excision, however, the probability of local recurrence by 5 years was 85 per cent (95 per cent confidence interval 56-99). The prognosis of patients with retroperitoneal sarcoma is poor.
Adolescent↗
Factors influencing local recurrence and survival in patients with soft tissue sarcoma of the upper limb.
Fifty-one patients with soft tissue sarcoma of the upper limb were studied to identify risk factors for local recurrence and survival. More than half (53 per cent) of the patients referred had locally recurrent disease. The flexor aspect of the forearm was the most common site of origin. The majority of patients were managed by a combination of conservative surgery and radical radiotherapy. Wide or radical excision was achieved in 49 per cent of cases. One-third of patients required partial resection of bone or neurovascular structures; 75 per cent of them had presented with local recurrence after treatment elsewhere. Skin grafts and flap repairs were used more often in patients with local recurrence (P = 0.013) and 20 (74 per cent) of those referred with locally recurrent disease have had no further local relapse. The overall 5-year survival rate of 80 per cent (95 per cent confidence interval 61-90 per cent) supports a policy of conservative surgery. Factors associated with a lower survival rate were deep fixation, origin in the flexor aspect of the forearm, and previous local recurrence. Deep fixation was also associated with an increased risk of local recurrence. Referral to a specialist unit at the time of initial presentation may result in lower rates of local recurrence and may improve the survival rate.
Adolescent↗
Bilateral malignant phyllodes tumours.
We report a rare example of bilateral primary malignant phyllodes tumours. The diagnosis was supported by the identification of a benign epithelial element in each lesion. The case illustrates the typical dimorphic features of malignant phyllodes tumours. A contralateral tumour should not be regarded as metastatic without histological confirmation.
Adult↗
Primary soft tissue tumours of the pelvis causing referred pain in the leg.
Referred pain in the leg is occasionally due to a pelvic soft tissue tumour. Among 11 patients who presented this way, one had a lymphoma, one had a benign schwannoma, and nine had soft tissue sarcomas. Most patients had undergone a variety of procedures, including laminectomy, before the correct diagnosis was established. In five cases, an accurate diagnosis was obtained by needle biopsy. The lymphoma responded to chemotherapy, and the benign schwannoma was excised. Of the nine patients with soft tissue sarcoma, six underwent marginal/intracapsular excision, three receiving supplementary radiotherapy, and two were treated by nonsurgical means. Hindquarter amputation was technically impossible or inappropriate in these cases. All those with high-grade tumours have died or have metastases. Of four patients with low-grade tumours, three have exhibited only slow disease progression. Careful judgment and a precise histopathological diagnosis are required in planning treatment for patients with pelvic soft tissue tumours causing referred pain in the leg.
Adult↗
On the semantic content of subcategorization frames.
This paper investigates relations between the meanings of verbs and the syntactic structures in which they appear. This investigation is motivated by the enigmas as to how children discover verb meanings. Well-known problems with unconstrained induction of word meanings from observation of world circumstances suggest that additional constraints or sources of information are required. If there exist strong and reliable parallels between the structural and semantic properties of verbs, then an additional source of information about verb meanings is reliably present in each verb's linguistic context. Five experiments are presented which investigate the following hypothesis regarding the scope of these relations: The closer any two verbs in their semantic structure, the greater the overlap should be in their licensed syntactic structures. To investigate this hypothesis, data of two kinds were collected from different groups of subjects: (a) One group of subjects was asked to judge the semantic relatedness of verbs by selecting the semantic outlier in triads presented to them. (b) A second group of subjects was asked to judge the grammaticality of these same verbs in a large range of syntactic environments. These two types of data were then compared to assess the degree of correspondence in the two partitionings (syntactic and semantic) of the verb set. The findings, overall, support the view that the syntax of verbs is a quite regular, although complex, projection from their semantics. In conclusion, we discuss the kinds of features that are formally marked in syntactic structure and relate these to the problem of verb-vocabulary acquisition in young children.
Concept Formation↗
High dose hyperfractionated radiotherapy in the treatment of extremity soft tissue sarcomas.
A hyperfractionated radiotherapy schedule has been evaluated in the treatment of 29 adults with limb or limb girdle soft tissue sarcomas. The objective was to increase the total administered dose and possibly improve local control, without increasing late normal tissue damage. Twice daily 1.25 Gy fractions (with a minimum interval of 6 h) have been given over 5 to 6 weeks to 12 patients pre-operatively, 10 post-operatively and 7 palliatively. Five patients received 62.5 Gy in 5 weeks pre-operatively and 3 palliatively. Nineteen of the remaining 21 patients received 75 Gy in 6 weeks with a field size reduction after 5 weeks. Sixteen of 29 tumours were situated in the thigh and only 2 were in the upper limb. Twenty were of high grade. The mean tumour size of those treated radically was 13.1 cm (range 5 to 40 cm). Sixteen patients (76%) given 75 Gy developed moderate or severe skin erythema maximal at 5 weeks. Despite the large field sizes used (mean phase one of 34.5 cm, and phase two of 22.8 cm) only 2 patients failed to complete the planned treatment because of the severity of these reactions. Two other patients developed partial wound breakdowns after the end of treatment--both healing spontaneously. Fourteen patients developed an area of moist desquamation--11 mild, 2 moderate and one severe. There have been four late wound breakdowns requiring surgical intervention; all have since healed well. The median follow up is short at 556 days. Ten of 19 evaluable patients have developed moderate/severe induration, 5/19 mild and 4/19 none.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗