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Biomedical subjects

J L Hungerford

Publications and source records attributed to J L Hungerford.

At least 73 records · Page 4Linked to original sources

Erythrocyte stearic to oleic acid ratio in patients with ocular melanoma.

The main characteristics of malignant cells include increased metabolism and capacity for division which are associated with high membrane fluidity. Membrane fluidity is in turn controlled by multiple factors which include the ratio of saturated to unsaturated fatty acids in the cell wall i.e. the Saturation Index (SI). The SI is known to be reduced in neoplastic cells as well as in the circulating erythrocytes of patients with some malignant tumours but not others. It has been suggested that the SI may be useful as a marker of malignancy.

Adult↗

AgNOR counts in conjunctival malignant melanoma lack prognostic value.

Using a silver staining technique, argyrophilic nucleolar organiser region-associated proteins (AgNORs) have been studied in routinely processed paraffin sections of 46 invasive malignant melanomas (MM) of the conjunctiva. The aim of this retrospective study was to assess the value of the AgNOR method as a prognostic indicator for this neoplasm. The 46 cases were divided into two groups: (A) 14 cases of MM that metastasised and caused death of the patient within 5 years of (histological) diagnosis, and (B) 32 cases of MM that did not metastasise and in which patients survived beyond 5 years. The mean of the AgNOR counts per nucleus was 7.03 (95% CI: 5.81-8.24) in group A, and 7.15 (95% CI: 6.53-7.77) in group B. A comparison using a multifactor analysis of variance (ANOVA) model, which corrected for possible confounding effect of tumour thickness, site, and cell type showed no significant difference in AgNOR counts between groups A and B (p = 0.8). Analysis by the Cox proportional hazards regression model showed that survival was not influenced significantly by the mean AgNOR number (hazard ratio: 0.92). Whereas the AgNOR technique may be used to distinguish benign from malignant melanocytic lesions of the conjunctiva, we conclude it has no value in predicting the outcome for patients with conjunctival MM.

Cell Count↗

Premalignant melanosis of the conjunctiva and the cornea in xeroderma pigmentosum.

Xeroderma pigmentosum is a rare autosomal recessive dermatosis. The neoplastic changes in sunlight-exposed areas of the skin and eyes may be related to the impaired replication of ultraviolet radiation-damaged DNA. A 38-year-old Greek woman is reported with a mild form of xeroderma pigmentosum and primary acquired melanosis with atypia of her right limbal conjunctiva and cornea. The development of this precursor of conjunctival malignant melanoma in a xeroderma pigmentosum patient may support the putative role of sunlight exposure in malignant transformation of conjunctival melanocytes.

Adult↗

Multifocal amelanotic conjunctival melanoma and acquired melanosis sine pigmento.

Clinical and histopathological features of four cases of multifocal amelanotic malignant melanoma of the conjunctiva in association with 'acquired melanosis sine pigmento' are reported. The absence of conjunctival pigmentation in this extremely rare combination of lesions prevented early diagnosis and clinical monitoring. As a result orbital exenteration was required in three cases. This multicentric non-pigmented variety of conjunctival malignant melanoma tends to present later than pigmented forms and may require exenteration of the orbit as a primary procedure.

Adult↗

Impression cytology of conjunctival melanosis and melanoma.

Impression cytology using cellulose acetate paper has been used in various ocular surface disorders as a simple non-invasive diagnostic test. To assess its value in differentiating melanocytic tumours, 24 patients with a range of pigmented lesions of the conjunctiva were examined using this technique. Cytological and histological diagnoses were compared in 23 cases. In 73% of cases impression cytology predicted the histological diagnosis by detection of superficial atypical melanocytes and their proportion relative to benign epithelial cells. This pilot study shows impression cytology to be a useful diagnostic aid in the differentiation of pigmented tumours of the bulbar conjunctiva.

Adult↗

Nasal and orbital recurrence of conjunctival melanoma 21 years after exenteration.

In our experience 5% of invasive malignant melanomas of the conjunctiva arising from areas of primary acquired melanosis with atypia spread to the ipsilateral nasal cavity and paranasal sinuses. Twenty one years after orbital exenteration for multicentric conjunctival melanoma an 82-year-old man was seen with an orbital recurrence, which had extended to the nasal cavity and paranasal sinuses through the nasolacrimal duct without invading the mucosa. This previously undescribed way of spread after the longest symptom-free interval following exenteration ever reported is illustrated.

Aged↗

Recurrent malignant melanoma of the corneal stroma: a case of 'black cornea'.

A 39-year-old Caucasian woman with a history of recurrent conjunctival melanoma of her right eye developed an intrastromal heavily pigmented malignant melanoma, which involved the whole corneal diameter. The patient was treated by corneoscleral lamellar keratoplasty and there has been no evidence of recurrent neoplasm during 4 years of follow-up. This apparently unique presentation of malignant melanoma of the cornea is illustrated and the differential diagnosis of corneal pigmentation is discussed.

Adult↗

Familial aggressive nevi of the iris in childhood.

We report the occurrence of amelanotic melanocytic tumors of the iris arising in the second decade of life in two successive generations. Clinically, both tumors enlarged rapidly, with subsequent enucleation of the eye in one patient. In both cases, histologic examination revealed an aggressive iris nevus composed of spindle A cells. Both lesions had similar argyrophilic nucleolar organizing region counts, which may be of prognostic significance. A possible autosomal-dominant pattern of inheritance for this rare tumor is discussed.

Adolescent↗

Genetic counselling in retinoblastoma: importance of ocular fundus examination of first degree relatives and linkage analysis.

We report an unusual family pedigree segregating the retinoblastoma predisposition gene. Expression of the phenotype in different individuals in this family ranges from asymptomatic gene carriers, regressed tumours, through unifocal to bilateral multifocal lesions. Because of the unusual pattern of inheritance in this family, initial genetic counselling at a local hospital did not take into account the possibility of incomplete penetrance of the gene, and complete ophthalmological examination of unaffected family members was not undertaken. We have used DNA probes from within the retinoblastoma predisposition gene for unequivocal identification of gene carriers. The subsequent demonstration of regressed tumours in founder members of the family confirmed the diagnosis of a dominantly inherited disease. The circumstances of the management of this family emphasises the need for specialist ophthalmic examination of first degree relatives and detailed genetic analysis of all such families with DNA probes.

Alleles↗

Oestrogen receptors in conjunctival malignant melanoma: immunocytochemical study using formalin fixed paraffin wax sections.

In a group of 15 women with conjunctival malignant melanoma six cases (40%) were shown to have oestrogen receptor positivity using a monoclonal antibody to oestrogen receptor related antigen (ER-D5, Amersham RPN.710) on formalin fixed, paraffin wax sections. Receptors were also present in the epithelium of these six cases but not in controls and cases without ER-D5 positivity in their tumours. A subset of these aggressive, intractable unilateral tumours may therefore be amenable to hormone treatment.

Adolescent↗

Radioimmunoscintigraphy of orbital metastases from ovarian carcinoma.

Radioimmunoscintigraphy with I-123 labeled monoclonal antibody HMFG1 has been used for imaging primary and metastatic ovarian neoplasms. Uptake of I-123 labeled HMFG1 is reported in a patient with an orbital metastasis from a primary ovarian adenocarcinoma. Radioimmunoscintigraphy may have a role to play in imaging metastatic orbital neoplasms.

Adenocarcinoma↗

Application of intragenic DNA probes in prenatal screening for retinoblastoma gene carriers in the United Kingdom.

Restriction fragment length polymorphisms (RFLPs) in 55 families affected by retinoblastoma have been studied using recombinant DNA probes derived from within the retinoblastoma predisposition gene. Only six families were uninformative for any of the DNA polymorphisms. The remaining 49 families can be offered prenatal screening. No obligate recombinations between any of the polymorphic loci and the retinoblastoma phenotype were observed. Four previously unknown cases of non-penetrance were identified. Prenatal testing for the inheritance of mutant alleles was performed in two cases and perinatal screening in two additional cases. One fetus inherited the normal allele from the affected parent and is therefore not at risk of retinoblastoma; the second fetus inherited the mutant allele and will require frequent screening for early detection of retinoblastoma. Both perinatal tests showed the absence of the mutant allele.

DNA Probes↗

Postenucleation orbital radiotherapy for the treatment of malignant melanoma of the choroid with extrascleral extension.

The outcome is reported in 17 patients in whom an eye was enucleated for malignant melanoma of the choroid with extrascleral extension and who subsequently underwent adjuvant external beam radiotherapy to the orbit as the primary treatment of the extraocular spread of their tumour. Extrascleral extension was encapsulated in five, non-encapsulated in two, and had been surgically transected at enucleation in 10 cases. All the patients have been followed up from enucleation to the present day. Orbital recurrence occurred in only one patient. The overall actuarial survival rate was 51% at 5 years, 44% at 10, and 33% at 15 years. A low orbital recurrence rate of 6% compares very favourably with published figures for this event after enucleation for melanoma with extrascleral extension but without radiotherapy. Adjuvant orbital radiotherapy may have a place in the treatment of selected cases of extracleral extension of intraocular malignant melanoma.

Adult↗

Orbital recurrence of retinoblastoma successfully treated by combined therapy.

Five children with an orbital recurrence of retinoblastoma have been successfully treated by a combination of excision biopsy of the tumour mass, radical orbital radiotherapy, and systemic chemotherapy. Nine previous children, consecutive with the five presented here, died from disseminated retinoblastoma after failure of earlier treatment programmes for orbital recurrence. An aggressive therapeutic approach is justified by this improvement in survival.

Antineoplastic Combined Chemotherapy Protocols↗

Pigmentation of the eyelid margin accompanying conjunctival melanoma.

In a review of patients with histologically verified malignant melanoma of the conjunctiva, we found 17 patients who had heavy pigmentation of the ipsilateral eyelid margin that was visible on external examination. Among nine patients in whom the histologic characteristics of the skin pigmentation could be studied, the features ranged from an increase in pigmentation of melanocytes within the basal layer to invasive malignant melanoma. The presence of heavy skin pigmentation in association with conjunctival melanoma appeared to be an ominous sign, even when the eyelid did not show histologic features of malignancy. Twelve of the 17 patients (70%) died of metastases after histologic verification of the conjunctival melanoma.

Adult↗

Malignant melanomas of the conjunctiva, nasal cavity, and paranasal sinuses.

Four patients had malignant melanoma involving the conjunctiva, nasal cavity, and paranasal sinuses. In each patient, the conjunctival melanoma preceded recognition of the lesion in the nasal cavity by months or years. Each patient had one or more conjunctival biopsy specimens that documented melanoma, and in each patient the melanomas evolved in primary acquired melanosis of the conjunctiva. Histologic examination of the epithelium adjacent to the tumor in the nasal cavity failed to show either atypia or diffuse melanosis. We believe that these melanomas in the nasal cavity and paranasal sinuses are most likely to evolve as regional metastases, although it is possible that they arise de novo or as related foci in accordance with the multicentric characteristics of conjunctival melanoma. If a patient with a conjunctival melanoma has symptoms referable to the nasal cavity or paranasal sinuses, ophthalmologists should refer the patient to an otolaryngologist.

Conjunctival Neoplasms↗