Biomedical subjects
Publications and source records attributed to F A Jakobiec.
Two patients with orbital vascular malformation ipsilateral facial nevus flammeus were studied. The literature contains one similar case that interestingly shares with our cases the lack of leptomeningeal angiomatosis. Orbitofacial angiomatosis seems to be a distinct entity from meningofacial angiomatosis because of its true vascular malformation of the orbit.
In seven patients who each had either a methyl methacrylate corneal shell, a postenucleation ocular prosthesis, or a keratoprosthesis, giant papillary conjunctivitis of the tarsus of the upper lids developed after prolonged wear. These cases expand the spectrum of disease that was initally described in wearers of hard and soft contact lenses. The papillary changes in prostheses wearers did not always produce symptoms and were not readily reversible. Basophils and mast cells characterized the inflammatory infiltrate, suggestng an antigen-antibody mechanism underlying the response. Increased production of epithelial mucin, in contrast to goblet cell mucin, may play a role in producing symptoms in this syndrome.
Two benign acquired melanocytic lesions of the iris stroma had distinctive clinical and pathologic features. One tumor was loculated and polypoid, suggesting ectopic glandular tissue, while the other was a flat tumor with a rich surface capillarity. Both lesions histologically were composed of mononucleated, binucleated, and multinucleated hypopigmented epithelioid and elongated "myoid" cells, widely separated in a finely fibrillar eosinophilic stroma. The ultrastructural features of one case included hyperconvoluted nuclei, conspicuous cytoplasmic filaments are rare, filamentary-striated (leptomeric) structures, scant organelles and inclusions except for numerous mitochondria and glycogen granules, small and infrequent melanosomes, and a fibrillary extracellular matrix focally organized into banded basement membrane material.
Capillary and cavernous hemangiomas are often grouped together as if they were modulations of the same entity. To clarify this nosologic question, three capillary hemangiomas in children and four cavernous hemangiomas in adults were studied by transmission electron microscopy. The capillary hemangiomas consisted of two variably admixed zones, solid cellular zones and zones with open vascular lumens. However, both zones had similar organization. Each vascular unit was composed of endothelial cells and pericytes, which persisted in a tumor three years after its clinical appearance. Macrophages and degenerating cells were found in the interstitium and may be related to the clinical feature of partial spontaneous regression. The cavernous hemangiomas had much larger lumens, and wider and more heavily collagenized trabeculae. The vascular walls were created by multilaminar smooth muscle cells. Therefore, capillary hemangioma is completely different from cavernous hemangioma, which resembles a venous tumor.
In spite of the fact that balloon cell change has been recognized ophthalmoscopically and histologically in certain posterior choroidal melanomas, the nature and importance of this observation remain controversial. This article provides a clinicopathologic correlation in two cases of ciliary body melanoma with almost total balloon cell transformation. On the basis of special stains and electron microscopic observations, we have concluded that balloon cells in such cases represent spindle melanoma cells that have undergoing extensive cytoplasmic lipid metamorphosis. The possible mechanisms for this change are considered. Melanomas containing an abundant number of balloon cells are probably comparatively dormant and benign tumors that offer a relatively good prognosis. Although clinical recognition may be difficult, the use of needle biopsy coupled with cytologic diagnosis in selected ciliary body tumors may disclose balloon cell change and help to direct therapy.
In five patients, specimens of discrete, subepidermal lid tumors were successfully aspirated with 22- to 24-gauge spinal needles and dianosed cytologically. The simplicity, rapidity of diagnosis, and high accuracy of cytodiagnosis should make it a popular method for the evaluation of clinically puzzling subepidermal lid tumors.
A 70-year-old woman with a long history of nasal and sinus polyps developed bilateral proptosis and left total external ophthalmoplegia. She was known to have hypercalcemia, which was later discovered to be caused by a parathyroid adenoma. X-ray studies, including computerized tomography, revealed increased radiodensity in the ethmoid sinuses (due to dystrophic calcification from the hypercalcemia), partial absence of the orbital walls from earlier surgical procedures, and bilateral rounded, retrobulbar tumors. At surgery, glistening, yellow, transparent, and encapsulated-lobulated masses were removed from the left orbit; they appeared to have prolapsed through a postsurgical dehiscence in the superomedial orbital wall. Light and electron microscopy confirmed that the lesional tissue represented polyps covered by respiratory epithelium; the yellow color was the consequence of secondary lipidization of the stromal fibroblasts.
Explore the source record for details and available documents.
We correlated the light microscopic features of ten ocular adnexal lymphoid neoplasms with the results of selected immunologic and histochemical lymphocyte marker studies. The lesions were divided into three histomorphologic patterns: inflammatory pseudotumor, reactive follicular hyperplasia, and malignant lymphoma. The six inflammatory pseudotumors, and reactive follicular hyperplasia, despite diverse histomorphology, were immunologically polyclonal and consisted of from 45 to 73% T cells and 27 to 60% B cells, the B cells being polyclonal with respect to light chain determinants. The four malignant lymphomas were immunologically monoclonal. Each consisted of a predominant B-cell proliferation, greater than 50% of the cells, which were monoclonal with respect to surface light chain determinants. No T-cell proliferations were observed. Our preliminary data indicate that, as in systemic lymphoid neoplasms, benign reactive lymphoid hyperplasias are immunologically polyclonal whereas malignant lymphomas are immunologically monoclonal. The systematic application of immunologic and histochemical techniques to the study of ocular adnexal lymphoid neoplasms may elucidate the natural history of these lesions.
Explore the source record for details and available documents.
A 36-year-old man had several episodes of left angular blepharoconjunctivitis associated with upper respiratory infections, herpes labialis, or emotional stress. The clinical diagnosis had always been a blepharitis of bacterial origin. During the last attack, cytologic examination of a scraping obtained from an eyelid pustule vesicle revealed multinucleated keratinocytes, diagnostic of a herpetic infection. Virologic studies proved that herpes simplex virus type 1 was the causal agent.
Six solid intraocular tumors were reliably diagnosed by needle biopsies and cytologic examination of the aspirates. All of the tumors had an unusual clinical or diagnostic feature that raised the possibility of a non-melanomatous tumor. Two intraocular lesions that were obscured by opaque media or a retinal detachment were successfully biopsied under B-scan ultrasonographic guidance of the needle. Cytodiagnosis of narrow spindle B, plump spindle B, and eipthelioid cell types, as well as one case of Coats' disease, was possible and correlated closely with the predominant cell types comprising the lesions discovered on histopathologic examination of the globes that were enucleated. The ocular tissues were not significantly disturbed and hemorrhage was not a serious problem. Details of the biopsy and cytologic techniques, the major clinical indications, for the procedure, and the authors' belief that the procedure is not likely to produce local seeding or extraocular metastasis in cases of melanoma are presented and discussed. Considerable profit may attend the use of this technique for the diagnosis of possible metastatic lesions and tumors of the ciliary body. The technique, however, should not be routinely employed and should be restricted to extremely difficult diagnostic problems.
The clinical characteristics and microscopic features of the biopsy specimens of 66 patients with orbital lymphoid tumors were analyzed with respect to clinical outcome (disease remaining localized to the orbit or developing associated extraorbital lesions). The patients' average age was 63 years, and their tumors had a marked tendency to arise in the anterosuperior orbit. No clinical or radiographic findings helped to distinguish the cases of localized disease from disseminated disease. The lesions were divided microscopically into three categories: reactive lymphoid hyperplasia (16), atypical lymphoid hyperplasia (38), and malignant lymphoma (12). Extraorbital lesions developed in 25%, 50%, and 75% of cases, respectively. The five-year mortality rates were also significantly different for each type of lesion: 6%, 19%, and 58%, respectively.
The clinical records of 101 patients (biopsies done on 48) with capillary hemangiomas of the orbit, orbit and lids, and lids were analyzed. Lesions predominated in females and typically appeared at birth or within the first two months of life. The superior orbit and lids were definite sites of predilection. Telltale periocular cutaneous involvement occurred in one third of the cases. Pure proptosis caused by a deep orbital tumor without an anterior component was found in seven cases. Proptosis in general was mild, but in two cases was severe enough to cause corneal exposure. Findings suprortive of a diagnosis of an orbital or periorbital hemangioma include (1) change in tumor size on crying (46%); (2) a dark vascular coloration to the subcutaneous tissue (80%); and (3) the existence of nonperiocular strawberry marks (25). Diagnostic tests of most value were plain radiography, ultrasonography, computed axial tomography, and arteriography. The complication rate in the 50 patients followed up over five years was 80% (60% with amblyopia). Present treatment modalities do not appear to be achieving the desired goals of a good cosmetic and functional outcome. Radiotherapy and corticosteroids are both effective in individual cases, but many lesions are resistant to all forms of present treatment. Amblyopia therapy is difficult and often unsuccessful in these patients.
The clinical and pathological data of 66 patients with orbital cavernous hemangioma are presented. This tumor occurs in females more frequently than in males, and has its peak incidence in early middle age. Visual disability results from a high degree of relative hyperopia or from optic-nerve compression. Postural or temporal variation is proptosis is not characteristic. Multiple cavernous hemangiomas are rare, but may occur simultaneously or separated by long intervals. In this series, incompletely excised lesions did not cause recurrent proptosis. Relative hyperopia may persist, in spite of complete removal of the tumor. Improved preoperative localization with modern techniques appears to be reducing the morbidity associated with surgical excision of the lesion. A local hemodynamic disturbance may initiate proliferation of vascular channels that undergo progressive ectasia. Growth of the lesion may occur intrinsically by the budding-off of capillary channels from cavernous spaces into the interstitium. Clinical and pathological findings fail to demonstrate any relationship between this lesion and capillary hemangioma of childhood.
A ciliary body tumor that was believed clinically to be a malignant melanoma developed in a 28-year-old woman and led to enucleation of her eye. The light microscopic appearance of the tumor was interpreted by light microscopy as a neurogenic neoplasm, possibly a choristomatous astrocytoma. Electron microscopic studies disclosed the smooth muscle origin of the tumor. Results of experimental embryologic studies performed on lower animals have proved that the neural crest contributes most of the connective tissues, "mesectoderm," of the globe and orbit in these species. The occurrence of hybrid neurogenic-myogenic tumors in the human eye supports the applicability of these embryologic discoveries to man. The association of a pigmented nevus with the present tumor is additional evidence of the neural crest origin of mesectodermal leiomyomas, since melanocytes are well-recognized descendants from the neural crest.
Two patients had freely movable epibulbar subconjunctival cysts that occurred in the absence of trauma, inflammation, and infection. Histopathologic examination of these cysts revealed them to be linked by nonkeratinizing epithelial cells and goblet cells. The cysts had little or no stroma and contained PAS-positive mucoid material. Electron microscopy of adjacent conjunctiva indicated that the epithelial cells were actively producing mucous granules and did not form many hemidesmosomes. The mechanical displacement of epithelial cells, as well as their mucous-producing ability, probably contribute to cyst formation. The relative lack of hemidesmosomes may explain why these cysts are freely movable.