This case is the first reported intraconal PNET of the orbit, and the first orbital case that expresses the MIC2 gene. In spite of the aggressive malignant features of peripheral PNET, the orbital variety seems to be the least aggressive since most of the reported patients are still alive.

ABSTRACT.Purpose: To report a patient with a history over 25 years of a slowly growing, large, invasive crateriform tumour filling the anterior part of the orbit. Methods: A 61-year-old male presented with a large tumour of the left orbit. Exenteration was performed with subsequent histological analysis of the excised mass. Results:The main tumour showed the characteristic features of a keratoacanthoma. However, the posterior aspect of the tumour disclosed the morphology of a squamous cell carcinoma. Six months later, the patient presented with metastases to lymph nodes, lung and mediastinal tissue. A leukemoid reaction was diagnosed by fine needle biopsy. Conclusion:The giant variety of keratoacanthoma may fail to regress and can transform into a squamous cell carcinoma. In our patient, the development of a chronic lymphoid leukemia raises the possibility that it may be the underlying cause for the transformation of the posterior part of the keratoacanthoma into a frank squamous cell carcinoma.

ABSTRACT.Purpose: Melanoma associated spongiform scleropathy (MASS) describes areas within the sclera where collagen bundles appear to have disintegrated into loose fibres. It is found adjacent to choroidal and ciliary body melanoma. This study aimed to characterize these changes histopathologically. Methods: Sections of 218 eyes that had been enucleated for malignant melanoma between January 1994 and June 2000 were examined for MASS, using data collected from the files of the Eye Pathology Institute, University of Copenhagen. Findings were correlated to patient characteristics (age, gender and pre-enucleation radiation) and tumour characteristics (location, morphology and invasion). Staining was applied using haematoxylin-eosin (HE), periodic acid-Schiff (PAS), haematoxylin-phloxine-saffron (HPS), alcian blue, colloidal iron and Masson's trichrome. Sixteen normal eyes and 29 eyes with diseases other than choroidal and ciliary body melanoma served as controls.Results: Melanoma associated spongiform scleropathy was observed in 73 of the 218 eyes (33%) with melanoma. No changes were found in normal eyes or in eyes with other intraocular diseases. A significantly higher incidence of MASS was found in older age groups. Thirty-six (49%) of the 73 cases of MASS were observed within a patient age range of 71-90 years, whereas only 14 cases (19%) were observed within an age range of 41-60 years. Pre-enucleation radiation significantly reduced the incidence of MASS. Twenty-two (10%) of the total 218 eyes had received pre-enucleation radiation and in only two (1%) of these was MASS observed. Melanoma associated spongiform scleropathy was found only in areas of contact between the tumour and the sclera, and the degree of MASS correlated directly with the extent of this contact. No significant correlation with tumour cell type was found. Conclusions: In eyes with MASS, the severity of the changes correlated to the extent of direct contact between the tumour and sclera. Melanoma associated spongiform scleropathy showed a significant predilection towards older age groups. Pre-enucleation radiation significantly reduced the incidence of MASS. Melanoma associated spongiform scleropathy is probably caused by a deposition of amorphous material splitting the scleral collagen fibrils. This material may be secreted either directly from the adjacent tumour or from scleral fibroblasts stimulated by the tumour cells.

ABSTRACT.Purpose: To analyse clinical referral diagnoses and the location of lesions with histologically verified lacrimal gland tissue occurring outside the fossa of the lacrimal gland. Methods: Sections of lesions excised from areas outside the fossa of the lacrimal gland containing lacrimal gland tissue on histological examination were collected from the files of the Eye Pathology Institute, Copenhagen, Denmark. Specimens spanned a period of 50 years. Sections were re-examined and referral data on location and clinical diagnosis were compared with histological findings. Results: A total of 120 lesions were collected. Of these, 59 (49%) consisted of prolapsed lacrimal gland. The remaining 61 (51%) lesions contained ectopic lacrimal gland tissue, either as part of a complex choristoma in 38 (32%) cases, or as solitary ectopic lacrimal gland tissue in 23 (19%) cases. The majority (97; 81%) of lesions had been located at the temporal epibulbar conjunctiva and included mainly prolapsed lacrimal gland and complex choristoma. The clinical referral diagnoses covered a wide spectrum of lesions. The most frequent clinical diagnoses were non-specific tumour (35%), non-specific cyst (18%) and dermoid (11%). Of the 61 lesions containing ectopic lacrimal gland tissue, only two had been preoperatively diagnosed as such and only two of the 59 lesions with prolapsed lacrimal gland had been correctly diagnosed. Conclusions: Prolapsed palpebral lobe of the lacrimal gland was the most common lesion and, as expected, the prime location was the temporal conjunctiva. Despite this location, the referring clinical diagnosis was often wrong or non-specific. Surgeons seem to have been unaware of the various clinical manifestations of extrafossal glandular tissue, particularly when excising lesions in the upper temporal region of the conjunctiva. Surgical intervention in this location may jeopardize the excretory ducts of the lacrimal gland and may consequently lead to dry eye and thus should be avoided when the typical clinical appearance of prolapsed lacrimal gland is encountered.

ABSTRACT.Purpose: To investigate biochemical changes of the sclera in eyes with melanomaassociated spongiform scleropathy (MASS), and to analyse possible relationships between these changes and tumour extension. Methods: Sections from 364 eyes, enucleated for choroidal and ciliary body melanoma, were examined for MASS and scleral tumour extension. Biochemical analysis was also performed on eight scleral specimens with MASS and eight specimens (controls) from morphologically normal sclera of the same eyes. The scleral thickness of each specimen was measured. Samples were delipidized, dried and weighed. The weight ratios of collagen-related amino acids were calculated based on quantitation by liquid chromatography. Amounts of glycosaminoglycans (GAGs) were determined by electrophoresis. Results: Melanoma-associated spongiform scleropathy was seen in 140 eyes (38.5%). Tumour scleral extension was observed in 82 eyes. Of these 82 eyes, 75 (91.5%) had MASS (p < 0.05). Biochemically, the majority of the main amino acids of the scleral collagen and total proteins were significantly lower in areas with MASS than in the control specimens. Specific GAGs and total GAGs were found in significantly higher concentrations in areas with MASS than in the control specimens. Scleral thickness was also significantly higher in areas with MASS than in the control specimens. Conclusions: The reduced content of collagen manifested by decreased amino acids and total proteins indicates collagen degradation in the vicinity of the tumour. The concomitant excessive deposition of GAGs accumulates water and may cause loosening of the already degraded collagen bundles, giving a histopathological picture of MASS. These changes could facilitate tumour cell migration and may explain the high incidence of MASS in eyes with scleral tumour extension.