Soft tissue, pelvic, and urinary bladder leiomyosarcoma as second neoplasm following hereditary retinoblastoma.

This report describes two patients who developed leiomyosarcomas, one involving the subcutaneous tissue of the thigh and the pelvic soft tissues and the other the urinary bladder, following hereditary retinoblastoma 36 and 38 years earlier, respectively. There is an increased risk of the development of sarcoma, most commonly osteosarcoma, as a second malignancy following hereditary retinoblastoma. Leiomyosarcoma developing as a second malignancy has rarely been reported and most have occurred in the field of previous radiotherapy. The literature on leiomyosarcoma occurring as a second neoplasm following retinoblastoma is reviewed.

Morphological effects of chemotherapy on ovarian carcinoma.

AIMS: Traditionally, advanced stage ovarian carcinoma is treated by debulking surgery followed by chemotherapy. However, in some circumstances preoperative chemotherapy may be given before optimal surgical debulking. This study aims to describe the morphological features found in ovarian carcinoma after chemotherapy because these have not been detailed previously. METHODS: Histological sections were examined from 18 cases of ovarian carcinoma that had been treated by preoperative chemotherapy. The morphology was compared with any pre-chemotherapy biopsies that had been performed. Tumours were classified as showing morphological features suggesting a good response to chemotherapy (n = 14) or as showing little or no response (n = 4). Serum CA125 values before and after chemotherapy were compared. In all cases, the mitotic activity index (MAI), volume percentage of epithelium (VPE), and mean nuclear area (MNA) of tumour cells were calculated. RESULTS: The preoperative biopsies were all typical ovarian serous or endometrioid adenocarcinomas. Morphological features present in the group responding to chemotherapy included the presence of small groups or single tumour cells in a densely fibrotic stroma. Tumour cells were characterised by both nuclear and cytoplasmic alteration, making accurate tumour typing and grading impossible. Nuclear features included the presence of bizarre enlargement with hyperchromatism, irregularity of outline, and chromatin clumping or smudging. Cytoplasmic alterations included intense eosinophilia, vacuolation, or foam cell change. There were pronounced stromal changes of fibrosis, inflammation, collections of foamy histiocytes, cholesterol cleft formation, haemosiderin deposition, fat necrosis, and dystrophic calcification, including the presence of many free psammoma bodies. There was no correlation between morphological response and biochemical response, as determined by serum CA125 values. In all nine cases in which pre-chemotherapy and post-chemotherapy biopsies were available, the MNA increased post-chemotherapy (p = 0.007, paired Wilcoxon test) and in six of nine cases the MAI decreased (p = 0.093). CONCLUSIONS: Because preoperative chemotherapy is being used increasingly in the management of ovarian cancer, pathologists should be aware of the resultant morphological effects. Accurate tumour typing and grading is impossible. In some cases, it may be difficult to confirm the presence of residual tumour, making it imperative that pre-chemotherapy tissue biopsies are obtained. Definite confirmation of residual tumour may require the examination of multiple histological sections from areas showing pronounced stromal changes, sometimes with multiple levels and immunohistochemistry. In the absence of definite residual tumour, the report should state that the features are consistent with the prior presence of tumour.

Histiocytic lymphoma presenting as a testicular tumour and terminating in acute monoblastic leukaemia.

A 58 year old man presented in 1995 with a swollen testicle. After orchidectomy, a diagnosis of poorly differentiated lymphoma was made. Lymphoid, epithelial, and seminoma markers were all negative. Six months later he developed a buccal lesion, which was biopsied and reported as a high grade non-Hodgkin's lymphoma. It responded completely to chemotherapy but within a year he developed a forearm swelling, which was biopsied and imprints made before fixation of the material. Immunocytochemistry on the imprints showed positivity with antibodies to CD4, CD68, and muramidase, and the non-specific esterase cytochemical stain was strongly positive, leading to a diagnosis of true histiocytic lymphoma. Despite further treatment, the patient entered a terminal acute leukaemic phase, the blasts marking as monoblasts. Review of all the biopsies, including molecular investigations and further immunohistochemistry studies performed retrospectively on the original biopsy, demonstrated that this was the same malignant cell line throughout, and we conclude that this is a case of histiocytic lymphoma, initially presenting as a testicular tumour and terminating in acute monoblastic leukaemia. A diagnosis of histiocytic lymphoma should be considered when lymphoid markers are negative in an apparent lymphoma, but should not be made without recourse to appropriate immunophenotypic and molecular studies.

Visual recovery in orbital vasculitis.

A 46-year-old woman with polyarteritis nodosa and chronic renal failure developed sudden loss of vision which was associated with orbital vasculitis. Treatment with cyclophosphamide produced rapid improvement in vision, which has been preserved with maintenance doses of cyclophosphamide and prednisolone.

Histological appearances of the levator palpebrae superioris muscle in the Marcus Gunn phenomenon.

The levator palpebrae superioris muscles from 12 cases of Marcus Gunn phenomenon were examined histologically by means of histochemistry and morphometry. The appearances were of a neurogenic atrophy with pathological changes occurring in both the affected side and the clinically normal side, suggesting that the causative lesion was located in the central nervous system.

Ocular larva migrans: a case report.

A case of intraocular toxocara larva migrans occurring in a 12-year-old boy is presented. Motility is inferred from the nature of multiple intraocular lesions observed. Possible mechanisms preventing a conclusive immunological response to the organism are discussed.

Anterior uveal neurilemmoma--a rare neoplasm simulating malignant melanoma.

A 30-year-old woman presented with dilated episcleral vessels in the right eye which were found to be associated with an underlying ciliary body tumour. Contact lens examination, transpupillary transillumination, ultrasonography, computerised tomography, magnetic resonance imaging, and fine needle aspiration biopsy produced apparently conflicting and inconclusive results, and the eye was enucleated. Light and electron microscopy showed the lesion to be a neurilemmoma (schwannoma), of which very few cases have been reported. The clinical and pathological features are described and discussed.

An experimental study of quinine blindness.

An experimental model of quinine induced blindness is presented. Electrophysiological, angiographical and morphological examinations were made. The occurrence of blindness and any recovery from blindness was dependent upon the dose of quinine taken. As no evidence of acute retinal ischaemia was found it is concluded that quinine is retinotoxic.

Iris atrophy in sickle cell disease.

Iris atrophy, of unknown origin and believed to be secondary to the vaso-occlusive process of sickle cell disease, has been observed in 25 eyes of 22 patients (two SS disease, 20 SC disease). The crude prevalence was highest in males with SC disease, in whom 14.7% of patients were affected. Iris atrophy was closely associated with proliferative sickle retinopathy in the same eye. Analysis of haematological indices failed to reveal any significant differences between patients with and without iris atrophy. The characteristics and distribution of iris atrophy are described as well as the histopathology in one 68-year-old male patient with SS disease.

Prognostic factors in malignant melanoma of the choroid: a retrospective survey of cases occurring in Northern Ireland between 1965 and 1980.

In Northern Ireland between 1965 and February 1980 one hundred eyes were enucleated following diagnosis of malignant melanoma of the choroid. The pathology of 87 cases was available for re-examination. Data on those patients still alive were examined together with the post-mortem reports of those deceased. A mathematical model was constructed to assess tumour volume more accurately. Survival was analysed using univariate and multivariate methods. Actuarial survival curves were constructed and two multivariate regression models were used--the proportional hazards model and the log-logistic model. In addition person years at risk analysis was used to compare survival with the Northern Ireland population. When the data were examined univariately, the location of the tumour, cell type, degree of invasion and the parameters of size (height, diameter and volume) were all significant prognostic indicators. When multivariate analysis was employed, the degree of invasion did not contribute significantly to the model, this being accommodated by tumour size. Cell type was also shown to be of less importance in the multivariate models. The various prognostic indicators together with the person years at risk analysis are discussed.