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Biomedical subjects

J Rootman

Publications and source records attributed to J Rootman.

At least 91 records · Page 5Linked to original sources

Orbital manifestations of plasmacytic lymphoproliferations.

Orbital involvement by plasmacytic lymphoproliferative disorders is rare. Presented are four cases, with different clinical onsets and courses, demonstrating the diversity of expression of lymphoproliferative plasma cell lesions of the orbit. The first patient presented with a slowly developing palpable mass in the upper and anterior orbit. This localized lesion proved to be a reactive plasma cell proliferation. The second case had a 3-month history of proptosis, diplopia, and droopy eyelid and a pulsatile temporal mass with bone destruction. Results of biopsy and systemic investigations showed a solitary plasmacytoma of bone. In the third case, a patient with a known multiple myeloma for 2 years presented with a progressive osteolytic mass which proved on biopsy to be a plasmacytoma. At the time, she also had other active bone lesions. The fourth case presented as a possible orbital cellulitis in a man with known multiple myeloma for 2 years. He proved to have disseminated myeloma, the orbital infiltrate being secondary to sinus involvement. The differential diagnosis for each case can be quite involved. Each case required a biopsy for an accurate diagnosis in addition to a complete clinical, radiographic, and laboratory workup. The pathologic features of each tumor can be misleading. These will be discussed as well as the use of ancillary diagnostic methods such as electron microscopy and immunohistochemistry to further characterize each lesion. The clinical course and treatment modalities will be discussed.

Aged↗

Response of an ocular melanoma to subconjunctival injection of 5-thio-D-glucose or cis-platin.

Successful treatment of ocular tumours by chemotherapy and radiotherapy is sometimes limited by the special nature of the eye. Improvement is needed to avoid enucleation. Previous studies using locally administered antineoplastic agents have given promising results in the treatment of experimental ocular melanoma and spontaneous lymphoma. Another approach is use of radiosensitizers to improve radiotherapeutic effect. For the present study, 5-thio-D-glucose and cis-platin were chosen for evaluation in an ocular system because they exhibit antitumour activity, and interaction with radiation, particularly in hypoxia. Ocular absorption, toxicity, and pharmacokinetics after subconjunctival administration in rabbits were determined, and the effect of drug on tumours was measured using a Greene melanoma model. CHO cells were used for complimentary in vitro studies. 5-thio-D-glucose was readily absorbed into the eye (300 mg resulting in 5 mM in the aqueous) with no observable toxicity. When 5TG (300 mg) was administered at implantation, tumours were approximately half the size of controls. 5 mM 5TG is toxic to extremely hypoxic cells and gives measurable radiosensitization. Cis-platin levels as high as 0.68 microM were attained in the aqueous without local toxicity after 400 micrograms injection. This concentration causes toxicity in vitro. Cis-platin (400 mg) had a larger effect on tumour growth than 5TG given at, or one week after, implantation. Cis-platin may have potential for treatment of ocular tumours by local injection.

Absorption↗

Infiltrating orbital granular cell tumour: a case report and literature review.

A surgical biopsy of an infiltrative retrobulbar mass in a 44-year-old man was diagnosed as granular cell tumour. Electron microscopy and immunoperoxidase stains were used to confirm the diagnosis and to study the histogenesis of this rare soft tissue neoplasm. S-100 stain was positive, while neuron-specific enolase and myoglobin stains were negative, suggesting a non-specific neural origin for the cells. The capability of this tumour to invade surrounding tissues has seldom been described in the orbit and is demonstrated by this case.

Adult↗

Orbital dermoids: features on CT.

The computed tomographic (CT) features of orbital dermoids were retrospectively reviewed in 17 patients; 15 of the lesions were proved histologically. On the basis of clinical and CT features, the tumors were classified as superficial or deep. All but one were extraconal in location. Seven lesions appeared cystic; only six showed typical fat density. The presence of a margin or rim, often partially calcified, was identified in ten lesions. Irregular scalloping of adjacent bone was a highly suggestive feature, occurring with 11 dermoids. Other bone changes, such as linear defects, thinning, or sclerosis, also occurred. Superficial dermoids showed less apparent bone changes. An extraconal orbital lesion associated with adjacent bone thinning or notching should raise the possibility of a dermoid, especially if a rim with calcification is seen. The appearance is pathognomonic if fat density is also present.

Adolescent↗

Retinoblastoma.

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Child↗

Ultrasonography of pathologically proved choroidal melanoma with a high-resolution small-parts scanner.

Between March 1983 and March 1985, 13 patients with choroidal melanoma underwent ultrasonography with a high-resolution small-parts scanner. The patients subsequently underwent enucleation, and the specimens were examined histopathologically. We reviewed the ultrasonograms for the presence of choroidal excavation, rupture through Bruch's membrane, retro-orbital fat shadowing, acoustic texture and retinal detachment. Comparison with the histopathological results showed that when conventional diagnostic criteria were used, most cases of rupture through Bruch's membrane were missed owing to the limits of resolution. No patients with homogeneous acoustic texture had pathological evidence of tumour necrosis or hemorrhage, while most of those with inhomogeneous texture did. The results suggest that high-resolution small-parts scanning may lead to reassessment of the conventional criteria for the diagnosis of intraocular melanomas.

Choroid Neoplasms↗

Orbital lymphoproliferative and inflammatory lesions.

We reviewed the records and biopsy specimens of 38 patients with clinically similar orbital lymphoproliferative and low-grade inflammatory lesions referred to one ophthalmologist at the University of British Columbia between 1977 and 1984. Twenty-six patients had lymphoproliferative lesions, the main feature being a densely cellular population of small lymphocytes. This group was further divided into reactive lymphoid hyperplasia, malignant lymphoma, Hodgkin's disease and atypical lymphoid hyperplasia. Symptoms and signs were similar within the subgroups. The mean length of follow-up was 3.7 years. Of the 26 patients 14 had extraorbital lymphoproliferative disease, 9 had orbital recurrences and 2 died of widespread disease. Twelve patients had inflammatory lesions with distinctly different histologic features from those of the lymphoproliferative group. Symptoms and signs were similar to those in the latter group. The mean length of follow-up was 2.1 years. Four of the 12 had orbital recurrences; none had extraorbital disease or died of their disease. We feel that orbital lymphoproliferative lesions can easily be separated from clinically similar low-grade inflammatory lesions histologically and that they should be staged and followed like small lymphocytic lymphomas. Guidelines are given for handling these specimens in the laboratory.

Adult↗

Pathological and clinical study of Müller's muscle in Graves' ophthalmopathy.

Specimens of Müller's muscle from patients with Graves' disease were examined for evidence of morphologic, histochemical, immunohistochemical and electron microscopic changes. No scarring, significant inflammation or shared antigenicity between Müller's muscle and thyroglobulin-related antigens was found. There were moderate numbers of mast cells in 48 of 66 specimens. Computed tomography in 16 patients with Graves' ophthalmopathy showed enlargement of the superior rectus-levator complex in 17 of 28 orbits with lid retraction. However, no correlation was found between the amount of lid retraction and the degree of enlargement of the muscle complex.

Ciliary Body↗

Extensive squamous cell carcinoma of the conjunctiva presenting as orbital cellulitis: the hermit syndrome.

The authors present three cases of extensive squamous cell carcinoma of the conjunctiva invading the orbit and masquerading as orbital cellulitis. All cases were in elderly alcoholic men living in relative social isolation. Total exenteration of the orbit was required in all three cases. Only one patient has had long-term follow-up; he was well 5 years after presentation. The other two patients were well 15 and 5 months after surgery.

Aged↗

Ocular penetration, toxicity, and radiosensitization effects of two hypoxic cell radiosensitizers on retinoblastoma.

Two new radiosensitizing drugs, SR-2508 and SR-2555, were studied for their in vivo toxicity and absorption properties. For both drugs, 100 mg dissolved in 0.5 mL of normal saline resulted in the maximum acceptable level of toxicity when injected subconjunctivally in rabbit eyes as determined by ocular and histopathologic changes. SR-2508 showed higher ocular and systemic absorption than SR-2555. The radiosensitizing ability of these drugs was studied using Chinese hamster ovary cells and the retinoblastoma cell line, V79c6. Results of the in vitro radiation experiments indicate that both drugs are comparable with misonidazole in their radiosensitizing ability, with SR-2508 being slightly more effective than SR-2555. Because of their relative high ocular absorption and low toxicity in comparison with misonidazole, these two drugs, particularly SR-2508, may be of clinical value and could be considered for adjunctive use as radiosensitizers of hypoxic tumors such as retinoblastoma.

Absorption↗

Signet-ring cell lymphoma in the orbit: a case report and review.

The authors report on the first case of signet-ring cell lymphoma involving the orbit. This variant of non-Hodgkin's lymphoma contains either vacuolated or eosinophilic cytoplasmic inclusions that squeeze the nucleus to one side of the cell. In this case, the inclusions were eosinophilic and stained positively for PAS, immunoglobulin M (IgM) heavy chain and kappa light chain. Electron microscopy showed granular electron-dense material trapped within distended loops of rough endoplasmic reticulum. Twenty-two cases of signet-ring cell lymphoma previously reported elsewhere in the body are reviewed, and their clinical significance is discussed.

Cytoplasm↗

The Tolosa-Hunt syndrome: computed tomographic changes and reversal after steroid therapy.

Painful ophthalmoplegia, or Tolosa-Hunt syndrome, is due to nonspecific inflammation of the cavernous sinus. We report two cases of the Tolosa-Hunt syndrome, which, on high resolution fourth-generation CT scan, showed an abnormality in the cavernous sinus consisting of an enhancing soft tissue mass. Both the clinical findings and the CT lesion resolved after high-dosage corticosteroid therapy.

Adrenal Cortex Hormones↗

Orbital-adnexal lymphangiomas. A spectrum of hemodynamically isolated vascular hamartomas.

A retrospective review of 13 cases of lymphangioma has led to a classification of these lesions into categories of superficial, deep, and combined types. The clinical manifestations, prognosis, and management directly correlate with the pathophysiology and the location of the lesions. Superficial lesions consist of isolated multicystic vascular abnormalities of cosmetic significance only. Deep orbital lymphangiomas present as spontaneous, acute proptosis due to retrobulbar hemorrhage. Combined lesions demonstrate both superficial and deep components and are also characterized by spontaneous hemorrhages. Histopathologically, lymphangiomas represent a spectrum of vascular hamartomas with a constellation of features that parallels the clinical progression and histology of similar lesions elsewhere in the head and neck. This may include the presence of diaphanous serous-filled vascular channels, a connective tissue stroma with lymphorrhages, features of old hemorrhage, dysplastic vessels, and random smooth muscle bundles. Both direct and indirect evidence suggests that these lesions are characterized by relative hemodynamic isolation.

Adult↗

Oculocerebrocutaneous syndrome.

A female infant was found at birth to have an oculocerebrocutaneous syndrome in which the main clinical features include orbital cyst, cerebral malformations, accessory skin tags, and focal hypoplasia or "punched-out" skin lesions. This case, like the four previously reported, was sporadic.

Brain↗

Supervoltage radiotherapy for Graves' ophthalmopathy: CCABC technique and results.

A treatment technique employing retrobulbar supervoltage radiotherapy (XRT) in Graves' ophthalmopathy is described. Twenty-eight patients have been treated and followed between 1980-1983. Twenty-six of 28 patients (93%) showed some response, with 19/28 (68%) having a good to excellent response. Four of 28 (14%) had recurrent symptoms post-XRT. Soft tissue signs and symptoms were relieved and disease progression arrested in the majority of patients, but proptosis and ophthalmoplegia responded poorly. High dose corticosteroids have generally been the alternative form of therapy for this disease. Post-XRT, 24/28 (86%) of patients have had no further steroid requirements, and been spared the associated complications. Treatment was well tolerated, with only five patients (18%) demonstrating a transient worsening of symptoms which settled entirely within 2 weeks. No long-term complications have been encountered. Eleven patients had post-XRT CT scans which were compared to pre-treatment scans and clinical response. Nine of 11 patients (82%) had CT changes in general agreement with the observed clinical response, but the degree of involvement seen on the initial CT scan was not predictive of response nor the risk of relapse. XRT with the described technique is felt to be an effective, safe, and practical approach to the disabling and disfiguring complications of severe Graves' ophthalmopathy.

Adult↗

Treatment of ocular leukemia with local chemotherapy.

Two cases of human ocular leukemic recurrence were treated by combined local chemotherapy. Both cases were histologically proven with aspiration needle biopsy and successfully treated with subconjunctival injections of methotrexate, cytarabine, and corticosteroids.

Adrenal Cortex Hormones↗

Risk factors for ocular melanoma: Western Canada Melanoma Study.

Between April 1, 1979, and March 31, 1981, 90 individuals in the four western provinces of Canada were diagnosed as having ocular melanomas. Of 87 age-eligible cases (age 20-79 yr), 65 (75%) were interviewed along with age- and sex-matched controls chosen at random from the provincial populations. Individuals with blue eyes had a significantly greater crude risk of ocular melanoma than those with brown eyes [odds ratio (OR)=3.0, P=.04]. Subjects with red or blonde hair were at higher risk of having ocular melanoma than those with black or dark-brown hair (OR=7.7, P=.03). Indoor workers appeared to be at elevated risk for ocular melanoma even after controlling for eye and hair color (OR=3.5, P=.006).

Adult↗

Effects of subconjunctivally injected antineoplastic agents on three models of corneal inflammation.

Three models of corneal inflammation--acute toxic keratitis, phlyctenular keratitis and corneal graft rejection--were induced in rabbits and treated with subconjunctival injections of antineoplastic agents (methotrexate, cytosine arabinoside, 5-fluorouracil and 6-mercaptopurine) and Solu-Medrol (methylprednisolone sodium succinate). The inflammations responded to the drugs to various degrees when compared with the response in control animals treated with saline. Cytosine arabinoside effected a slight decrease in the clinical features of acute toxic keratitis, methotrexate was superior in decreasing inflammation and neovascularization in phlyctenular keratitis, and Solu-Medrol appeared to be the most useful in the treatment of graft rejection. When injected repeatedly, 5-fluorouracil tended to have significant toxicity in the presence of inflammation.

Animals↗