O. A. Jensen

Malignant melanomas of the human uvea: 25-year follow-up of cases in Denmark, 1943--1952.

All cases (302) of malignant intraocular melanomas in Denmark between 1943--1952 were followed up and evaluated 25 years after enucleation, which was the treatment used immediately after diagnosis during that period. The follow-up percentage was 99, 82% of the total being dead at the time of follow-up, bringing the real course of the malignant uveal melanoma in a traditionally treated series nearer to the truth. The same series was analysed in 1963 and 1970. Three-quarters of the patients were hospitalized immediately before and/or in the process of dying. In the series of choroidal and/or ciliary body melanoma the autopsy frequency was 36. Death with metastases took place in 51% (150). Orbital recurrence was found in 2.5% and 8--12% of cases with extrascleral extension had orbital recurrence. This appeared at an average of 2 years after enucleation and 10% of metastases appeared within the first 6 months post-operatively, more than half within 3 years. The peak incidence was during the first year after enucleation. No second peak was observed. However, metastases appeared even after 23 years. The probability of being alive without metastases after 25 years was found to be 40%, assuming that the patient did not die of another cause. The prognosis was estimated using various clinical and histopathological parameters. Women below the age of 40 had a better prognosis than women above that age. Small tumours, spindle shaped cells, no invasion and low pigment content of tumour were all in favour of a good prognosis. Of the iris melanomas from the period dealt with, only one died with metastasis. The present lively discussion on early enucleation or conservative management is reported and finally the attitude of the Ophthalmic Tumour Centre towards therapy is indicated.

HISTOPATHOLOGY OF EYE, OPTIC NERVE AND BRAIN IN A CASE OF DOMINANT OPTIC ATROPHY

Histopathology of eye, optic nerve and brain was performed in a patient with typical signs and symptoms of dominant optic atrophy. He belonged to a previously‐reported family of 152 members in which optic atrophy was demonstrable in 14 persons, and probably present in a further 8 cases. In the eyes, fibrosis of the retinal ganglion cell layer and disc was found. Ultra‐structural examination showed a few remaining cells in this layer, heavy fibrosis and in particular a highly condensed inner limiting membrane. The optic nerves, the optic chiasm and optic tracts showed an increased content of collagen tissue and a decreased number of neurofibrils and myelin sheaths. In the lateral geniculate body there was massive loss of ganglion cells, fibrillary gliosis and a great quantity of fine granular lipid in the cytoplasm of the ganglion cells. No changes in the calcarine cortex were observed. Examination of the intracranial part of both vestibulocochlear nerves showed a decreased number of neurofibrils and myelin sheaths.

Malignant melanoma of the conjunctiva: occurrence and survival following surgery and radiotherapy in a Danish population

Abstract• Purpose: To describe patient characteristics, risk of recurrence, and survival in patients with premalignant and malignant melanocytic lesions of the conjunctiva. • Methods: In a retrospective study, 55 cases with a minimum follow-up of 10 years were reviewed. • Results: The actuarial 5-and 10-year survival rates were 86% (95% Cl: 76–96%) and 73% (95% Cl: 60–87%) respectively. The 5-year risk of local recurrence was 35% (95% Cl: 21–49%), and the 10-year risk was 43% (95% Cl: 28–58%). Primary acquired melanosis with atypia was the first manifestation in 13 cases, malignant melanoma in 42 cases. No significant difference in survival and recurrence was found between the two groups or by gender, location of primary lesion, histological characteristics, or extent of surgical procedure. A minimum incidence of 0.052 cases a year per 100000 in Denmark was calculated. • Conclusion: The results are estimated to represent a nationwide sample of patients. No prognostic factors of significance were identified.

ANTERIOR CHAMBER CLEAVAGE SYNDROME

An otherwise healthy girl had from birth right‐sided microphthalmia with leukomatous cornea and left‐sided staphyloma centrally on a leu‐komatous cornea, surrounded by a deep blue sclera. The intraocular pressure was raised and the staphyloma increased in size. The eyeball was enucleated at three months of age and showed anterior chamber cleavage syndrome. The corneal stroma was replaced by connective tissue. No Bowmans membrane was found and the Descemets membrane was present only as small coiled remnants centrally. No lens was observed. The iris was seen only in a few sections, as a string attached to the cornea. The posterior part of the eye was normal.

Structure and composition of the inner limiting membrane of the retina. SEM on frozen resin-cracked and enzyme-digested retinas of Macaca mulatta.

After a historical introduction describing previous observations and views on the structure and composition of the internal limiting membrane of the retina (MLI), it is concluded that no definite unifying concept exists concerning the MLI structure. The authors applied frozen resin cracking with hexamethyldisilazane (HMDS) desiccation on normal and enzyme-digested specimens of monkey retinas. Distinct differences were observed between equatorial, macular, and optic disc regions. The MLI in the equatorial and disc regions was about 70 nm thick with an outer dense, basement membranelike part and an inner, loose fibrillar meshwork fusing with the vitreous fibers. The foveal region was 400 nm thick; the dense outer layer was thicker than normal basement membrane, whereas the inner loose meshwork had only a few fibrils. The enzyme-digestion experiments showed that the fibrillar meshwork consisted mainly of collagen fibers surrounded by predominantly hyaluronic acid.

Bilateral Diffuse Malignant Melanoma of the Uvea Associated with Large Cell Carcinoma, Giant Cell Type, of the Lung

A new syndrome consisting of bilateral diffuse malignant uveal melanoma and simultaneous occurrence of another systemic malignant neoplasm was described in 6 patients in 1982. The present study reports a seventh case. Our patient was younger than the patients previously reported. He had large cell carcinoma of the right lung. The bilateral intraocular neoplasm was characterized as a diffuse malignant uveal melanoma of the mixed cell type by light microscopy and by transmission electron microscopy. All parts of the tumor contained S-100 protein. In addition to the two primary neoplasms, the patient had a combination of Otas and Itos naevi on the left side.

BENIGN MUCOUS MEMBRANE PEMPHIGOID

Conjunctival biopsy specimens were taken from the inferior fornix of 16 eyes affected with benign mucous membrane pemphigoid (BMMP). Biopsy revealed in all cases metaplasia changing the normal columnar epithelium into squamous epithelium, with parakeratinization in eight specimens and keratinization as well, in four. Correlation was noticed between keratinization and reduced secretions of both tears and mucus.

Late complications of biopsy in intraocular tumors.

The diagnosis of an intraocular malignant tumor may be very difficult or impossible by clinical methods and the question of performing an intraocular biopsy is naturally raised. Intraocular biopsy has been used since 1868 (Hirschberg) but only in a few cases of tumors of the choroid. As far as we know a follow-up of biopsied cases has never been done. As for the cases of intraocular biopsy published before, the reader is referred to a paper in 1954 by one of us (S. R. A.). In recent years only a few cases have been added. Sunders (1953) made an intraocular biopsy in 3 cases of malignant melanomas of the choroid followed by enucleation three days after the operation. The tumors were of the cell types spindle B., mixed and epithelioid respectively. 2 of the patients died from metastases 2 and 3 years after the enucleation, the 3rd was not followed. Sanders also made a biopsy in 2 cases of neurofibromas. His technique was a sclerotomy or trepanation. Sunders concluded that the value of biopsy in tumors of the ciliary body and choroid is limited, but may be indicated in one-eyed individuals or unusual clinical cases and in patients demanding a histological diagnosis before enucleation. Christensen & Rowen (1955) found a high incidence of free-floating tumor cells in the subretinal fluid in globes with retinal detachment secondary to malignant melanoma. They do not recommend this method because of danger of extraocular extension of unrecognized tumors through the puncture. Other

Spontaneous regression of a malignant melanoma of the choroid.

A 65‐year‐old man had for 12 years a lesion in the right eye, clinically atypical of a melanoma. The lesion and function of the eye was unchanged during the 12 years. Since a melanoma seemed clinically most probable the eyeball was enucleated. Histopathological examination revealed a choroidal lesion with incipient invasion of the sclera, abundant fibrosis, calcifications and scattered neoplastic melanocytes, confirmed by electron microscopy. There was no necrosis and no inflammatory reaction. The diagnosis and the cause of regression are discussed, and it is concluded that this case represents a true spontaneous regression of a primary malignant melanoma of the choroid.

Mucosubstances of the acini of the human lacrimal gland (orbital part). I. Histochemical identification.

The mucosubstances of the acini of the human orbital lacrimal gland were examined by applying a battery of histochemical methods to normal gland tissue. The material was obtained during operation and postmortally from persons 20–50 years old.