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Biomedical subjects

Jerry A Shields

Publications and source records attributed to Jerry A Shields.

At least 73 records · Page 4Linked to original sources

Sebaceous carcinoma of the eyelids: personal experience with 60 cases.

OBJECTIVE: To describe clinical features, management, and prognosis of sebaceous carcinoma of the eyelid and adjacent structures. DESIGN: Single-center retrospective interventional case series. PARTICIPANTS: Sixty consecutive patients with sebaceous carcinoma of the eyelid and adjacent structures. METHODS: Retrospective chart review and literature review. MAIN OUTCOME MEASURES: Presenting features, sites of origin, location, growth patterns, management, histopathologic findings, incidence of recurrence, metastasis, and mortality. RESULTS: The median age at referral was 72 years, with 73% female. Four patients had prior irradiation to the area where the sebaceous carcinoma developed. Initial clinical diagnoses elsewhere were sebaceous carcinoma (32%), blepharoconjunctivitis (25%), chalazion (20%), basal cell carcinoma (13%), and squamous cell carcinoma (10%). Initial histopathologic diagnoses elsewhere were sebaceous carcinoma (50%), squamous cell carcinoma (18%), basal cell carcinoma (8%), and others or not available (24%). Initial anatomic sites were upper eyelid (75%), lower eyelid (22%), caruncle (2%), and bulbar conjunctiva (2% [1 case]). Orbital exenteration was necessary in 13%. Recently introduced techniques of posterior lamellar resection of the eyelids with reconstruction (7%) hopefully will decrease this incidence in the future. Pathologically, 47% showed intraepithelial (pagetoid) involvement, 27% of sebaceous carcinomas arose from the meibomian glands, and 18% arose from both meibomian and Zeis glands. Local recurrence developed in 18%, metastasis in 8%, and death from metastasis in 6%. CONCLUSIONS: Despite the fact that the clinical features of sebaceous carcinoma have been widely reported, the diagnosis was suspected initially in only 32% of patients at first examination elsewhere and in only 50% at histopathologic examination elsewhere. Orbital exenteration was necessary in 13%, mostly patients seen in the earlier years of the study. With more recently employed treatment methods, there is a tendency to avoid exenteration and to use more conservative methods of treatment. It is hoped that these modern therapeutic approaches will result in fewer cases of recurrence and metastasis.

Adenocarcinoma, Sebaceous↗

Clinical factors in the identification of small choroidal melanoma.

The detection and treatment of choroidal melanoma early in its natural course is critical to providing the patient with the best prognosis. Studies of tumour doubling time have indicated that metastasis from choroidal melanoma can occur quite early in the course of the disease, when the tumour is about 3.0 mm in basal dimension and 1.5 mm in thickness. Clinical studies have shown that, at 5 years, metastasis occurs in 16% of patients with small choroidal melanomas (less than 4 mm thick), compared with 32% of those with medium-sized (4-8 mm thick) choroidal melanomas and 53% of those with large (more than 8 mm thick) choroidal melanomas. The difficulty with early detection of choroidal melanoma relates to its clinical similarity to benign choroidal nevus. Factors that assist in differentiating small choroidal melanoma from choroidal nevus can be remembered using the mnemonic "TFSOM" (to find small ocular melanoma), where T = thickness greater than 2 mm, F = subretinal fluid, S = symptoms, O = orange pigment and M = margin touching optic disc. Choroidal melanocytic tumours that display none of these factors have a 3% risk of growth into melanoma at 5 years and most likely represent choroidal nevi. Tumours that display one factor have a 38% risk of growth, and those with two or more factors show growth in over 50% of cases. Most tumours with two or more risk factors probably represent small choroidal melanomas, and early treatment is generally indicated. Therefore, ophthalmologists should be aware of the clinical factors that identify small choroidal melanoma so that early treatment and better prognosis can be achieved for their patients.

Choroid Neoplasms↗

Continuing challenges in the management of retinoblastoma with chemotherapy.

The management of retinoblastoma has gradually changed over the past 10 years. Over 95% of children with retinoblastoma in the United States are cured with modern techniques. The challenge remains, however, in maintaining the eye and vision. There is a trend away from enucleation and external beam radiotherapy toward focal conservative treatments involving primary chemoreduction in conjunction with thermotherapy and cryotherapy. This is related to earlier detection of the disease, recognition of more effective chemotherapeutic agents, more focused local treatment modalities, and, most importantly, knowledge of the long-term risks of external beam radiotherapy. Enucleation is still preferable for retinoblastoma that fills most of the eye, especially when the disease is unilateral or when there is concern for tumor invasion into the optic nerve, choroid, or orbit. The orbital integrated implant is placed after enucleation and provides acceptable prosthesis motility and appearance. External beam radiotherapy is still vital for treating advanced retinoblastoma, especially when there is diffuse vitreous or subretinal seeding after failure of other methods and preservation of vision is a priority. The most important recent advance in the management of retinoblastoma is the use of intravenous chemotherapy for tumor reduction, a technique of neoadjuvant chemotherapy termed "chemoreduction." This is followed by tumor consolidation with focal measures such as thermotherapy, cryotherapy, and plaque radiotherapy. This strategy provides reduced tumor volume and often permits consolidation with methods other than radiotherapy. It appears that vision can be preserved in some cases with these methods, avoiding some of the local complications like radiation cataract or macular edema that have been found with radiotherapy techniques. External beam radiotherapy and enucleation can now be avoided in most cases of Reese-Ellsworth groups I (minimal disease) through IV (moderate disease) retinoblastoma. The most advanced stage of retinoblastoma, Reese-Ellsworth group V, continues to provide the greatest difficulty for management, and external beam radiotherapy and enucleation are often employed in addition to chemoreduction to save the child's life. A collaborative prospective study in North America is currently under way to further study the benefits and risks of chemoreduction for minimal, moderate, and advanced retinoblastoma.

Antineoplastic Agents↗

Recent developments in the management of choroidal melanoma.

PURPOSE OF REVIEW: To describe recent advances in the management of choroidal melanoma. RECENT FINDINGS: During the past 10 years, there has been a trend in the management of choroidal melanoma from enucleation to conservative nonenucleation treatments such as thermotherapy, plaque radiotherapy, charged-particle radiotherapy, and local resection. Recently published information regarding conservative management has revealed that plaque radiotherapy combined with thermotherapy offers 97% tumor control, similar to charged-particle radiotherapy. Additionally, patient survival after treatment of medium-size melanoma is similar when comparing plaque radiotherapy versus enucleation. Risk factors identifying small choroidal melanoma include patient symptoms, tumor thickness more than 2 mm, presence of associated subretinal fluid, presence of orange pigment on the tumor surface, and location of the tumor margin at the optic disc. These factors assist in early detection of choroidal melanoma. SUMMARY: The management of choroidal melanoma continues to improve with earlier detection of tumors and better treatment methods. Local tumor control is excellent with radiotherapeutic methods. Future studies should investigate control of subclinical metastasis.

Choroid Neoplasms↗

Contralateral blindness from chiasmal extension of unsuspected choroidal melanoma.

An 80-year-old woman with a prior diagnosis of age-related macular degeneration in her left eye had rapidly progressive visual loss in her right eye. Orbital MRI revealed a mass involving the left optic nerve and chiasm, interpreted by a radiologist as optic nerve sheath meningioma. Further review of the MRI revealed a mass inside the left eye, characteristic of choroidal melanoma, with apparent extension through the optic nerve to the chiasm and adjacent tissues. Orbital biopsy revealed mixed cell-type melanoma. This case demonstrates that unsuspected choroidal melanoma can invade the optic nerve and chiasm, causing contralateral visual loss. Contralateral blindness as the initial complaint from an unsuspected choroidal melanoma is extremely unusual if not unique.

Aged↗

Calcified orbital cyst simulating a malignant lacrimal gland tumor in an adult.

A 56-year-old woman with left periocular pain was discovered on CT to have a partially calcified, circumscribed cystic lesion in the lacrimal gland fossa, suggestive of a malignant lacrimal gland tumor. Adjacent bone fossa formation without erosion was noted. Histopathologic examination of the excised mass disclosed a cyst with chronic granulomatous inflammation and calcification of the cyst wall. The combination of chronic pain, cystic changes, and calcification in a lacrimal gland fossa mass in an adult should suggest a malignant neoplasm, but a long-standing orbital cyst with calcification of its rim can produce the same features.

Calcinosis↗

Chemoreduction for retinoblastoma: analysis of tumor control and risks for recurrence in 457 tumors.

PURPOSE: To evaluate individual tumor control following chemoreduction for retinoblastoma. METHODS: Prospective nonrandomized single-center case series of 457 retinoblastomas managed with six cycles of chemoreduction (vincristine, etoposide, and carboplatin). The tumors were then managed with chemoreduction alone (group A) or chemoreduction combined with thermotherapy (group B), cryotherapy (group C), or both thermotherapy and cryotherapy (group D). The main outcome measure was development of tumor recurrence. RESULTS: Of 457 retinoblastomas, 63 (14%) were in group A, 256 (56%) in group B, 127 (28%) in group C, and 11 (2%) in group D. The tumor was located in the macula in 33 (52%) of group A, 109 (43%) of group B, 3 (2%) of group C, and 1 (9%) of group D. Using Kaplan-Meier analysis, recurrence of the individual retinoblastoma at 7 years was found in 45% of group A and in 18% of combined groups B, C, and D. Treatment of the 93 tumor recurrences included thermotherapy, cryotherapy, or plaque radiotherapy in 62 cases (67%) and external beam radiotherapy or enucleation in 31 cases (33%). Risk factors predictive of tumor recurrence by multivariate analysis included macular tumor location for all groups and, additionally, female sex for group A and increasing tumor thickness for groups B, C, and D. CONCLUSIONS: Chemoreduction alone or combined with cryotherapy and/or thermotherapy is effective for treatment of retinoblastoma, but tumor recurrence is greatest for those located in the macula and those with greater thickness. Globe salvage is usually achieved despite tumor recurrence.

Adolescent↗

Aggressive retinal astrocytomas in four patients with tuberous sclerosis complex.

OBJECTIVE: To report the clinical and histopathologic findings of retinal astrocytic tumors that showed progressive growth in four patients with tuberous sclerosis complex (TSC). METHODS: Four young children each developed an enlarging retinal neoplasm that eventually necessitated enucleation of the affected eye. The systemic findings, clinical course, and histopathologic findings were reviewed. RESULTS: Each patient had a progressively enlarging retinal mass associated with a total exudative retinal detachment and neovascular glaucoma. Enucleation was necessary in each case because the affected eye became blind and painful. The mean patient age at enucleation was 7 years, and the median age was 3 years. At the time of enucleation the tumors ranged from 10 to 20 mm in basal diameter and from 10 to 25 mm in thickness. Histopathologic studies of each eye revealed a giant cell astrocytoma that had produced a total exudative retinal detachment. The tumor cells showed positive immunoreactivity to neuron-specific enolase and glial fibrillary acidic protein. The retinal neoplasms in these cases were identical histopathologically to the subependymal giant cell astrocytoma that typifies TSC in the brain. One tumor filled the entire eye and perforated the globe. Although the lesions simulated retinoblastoma clinically, each patient had ocular and systemic findings of TSC, supporting the diagnosis of astrocytic hamartoma. CONCLUSIONS: Although retinal astrocytic lesions of TSC generally are stationary, they can sometimes grow relentlessly and cause severe ocular complications. Patients with retinal astrocytic hamartomas should have serial ophthalmic evaluations because of this possibility.

Astrocytoma↗

Magnetic resonance imaging of unilateral lacrimal gland lesions.

PURPOSE: To report the findings on magnetic resonance imaging (MRI) of various benign and malignant unilateral lacrimal gland lesions. METHODS: This is a retrospective noncomparative interventional case series. Thirty-one consecutive patients with a unilateral lacrimal gland lesion were analyzed. The preoperative MRI findings were correlated with the pathology results. The main outcome measures were anatomic extent, configuration, margins, angulation, internal features on T1- and T2-weighted images (with respect to extraocular muscles and cerebral gray matter), contrast enhancement of the lesion and adjacent bone change on MRI. RESULTS: Of the 31 patients, 21 had chronic dacryoadenitis, 3 had lymphoid tumors, and 7 had epithelial tumors including pleomorphic adenoma (3), adenoid cystic carcinoma (3), and pleomorphic adenocarcinoma (1). The results of the patients with chronic dacryoadenitis demonstrated involvement of the orbital lobe alone in 13 patients (62%), involvement of both orbital and palpebral lobes in 8 (38%), a molded configuration with ill-defined margins, sharp angles in 13 (62%), round angles in 8 (38%), lack of bone change, an isointense internal signal on T1-weighted images, a hypointense signal on T2-weighted images, and moderate contrast enhancement. The patients with lymphoid tumors demonstrated involvement of the orbital lobe, a molded configuration with ill-defined margins and sharp angles, lack of bone change, an isointense internal signal on T1-weighted images, an isointense signal on T2-weighted images, and moderate contrast enhancement. Those with epithelial tumors showed involvement of the orbital lobe, a well-circumscribed oval configuration, and round angles. Pleomorphic adenoma demonstrated smooth margins, bone expansion in two patients, and no bone change in one. Adenoid cystic carcinoma and pleomorphic adenocarcinoma showed irregular margins and bone destruction. All epithelial tumors demonstrated an isointense internal signal on T1-weighted images, a hyperintense signal on T2-weighted images, and moderate contrast enhancement. CONCLUSIONS: It is difficult to uniformly correlate the MRI features and histopathologic findings in lacrimal gland lesions. However, MRI seems to be useful in determining the etiology of a unilateral lacrimal gland lesion. Internal tissue features on T1- and T2-weighted images of MRI are most helpful in categorizing these lesions. Although the number of patients is small, our findings suggest that there are differences in orbital MRI findings of inflammatory lesions and lymphoid tumors as compared to benign and malignant epithelial tumors in the lacrimal gland fossa.

Adenoma, Pleomorphic↗

Orbital schwannoma: correlation of magnetic resonance imaging and pathologic findings.

PURPOSE: To report the correlation between magnetic resonance imaging (MRI) findings and histopathology of orbital schwannomas. METHODS: This study is a retrospective noncomparative interventional case series of three consecutive patients with orbital schwannoma. After preoperative MRI, all patients underwent orbitotomy and tumor removal. Histopathologic and immunopathologic studies of the excised tumors were performed. The main outcome measures were MRI features, histopathology, and their relationship to the cohesiveness of the tumor. RESULTS: The patients' ages were 56 years (patient 1), 43 years (patient 2), and 18 (patient 3) years. MRI demonstrated that the tumors were well-circumscribed, intraconal in patient 1, superior extraconal in patient 2 and inferotemporal extraconal in patient 3. The tumor in patient 1 had a partly cavitary appearance, while the tumors in patients 2 and 3 had a solid appearance on MRI. The tumor had heterogeneous signal intensity in patients 1 and 2 and homogeneous signal intensity in patient 3 on T2-weighted images. All patients underwent orbitotomy and tumor removal. In patients 1 and 2, the tumor was gelatinous and fragmented during removal. The fragments were completely excised in patient 1 and an incomplete piecemeal tumor excision was made in patient 2. In patient 3, complete excision without fragmentation was performed. Pathologic examination revealed that the tumor was of Antoni B pattern in patient 1, mixed Antoni A and B patterns in patient 2, and marked Antoni A pattern in patient 3. At a mean follow-up of 23.7 months (range: 22-26 months) after surgery, all patients retained preoperative visual acuity. Patient 2 developed recurrence at 21 months follow-up and underwent Krönlein orbitotomy for excision of the recurrent tumor. CONCLUSIONS: MRI is useful in depicting both the anatomic location and internal consistency of orbital tumors. Schwannomas with MRI evidence of cavitary change or heterogeneous signal intensity on T2-weighted images were found to have marked Antoni B pattern on histopathology, imparting a friable structure and fragmented excision.

Adolescent↗

Development of new retinoblastomas after 6 cycles of chemoreduction for retinoblastoma in 162 eyes of 106 consecutive patients.

OBJECTIVE: To evaluate the occurrence of new retinoblastomas in patients treated with 6 cycles of chemoreduction. DESIGN: Prospective nonrandomized single-center case series. SETTING: Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University, Philadelphia, Pa, in conjunction with the Division of Oncology at The Children's Hospital of Philadelphia. PARTICIPANTS: A total of 162 eyes of 106 patients with retinoblastoma treated with 6 cycles of chemoreduction between January 1, 1995, and May 31, 2002. INTERVENTION: All patients received intravenous chemoreduction with vincristine sulfate, etoposide, and carboplatin, combined with focal treatment (cryotherapy or thermotherapy) to each retinal tumor. MAIN OUTCOME MEASURE: Development of new intraretinal retinoblastoma during or after treatment with chemoreduction. Recurrent subretinal tumor seeds or vitreous seeds were excluded from this analysis, and only primary new intraretinal tumors were included. RESULTS: Of 28 patients with unilateral retinoblastoma, new intraretinal tumor development was found during or after chemoreduction in 2 (9%) of the 23 patients with sporadic disease and 4 (80%) of the 5 patients with familial disease. The new tumor was located in the macula in none, between the macula and equator in 7 (54%), and between the equator and ora serrata in 6 (46%). Of the 78 patients with bilateral retinoblastoma, new tumor development was found during or after chemoreduction in 11 (19%) of the 57 patients with sporadic disease and 8 (38%) of the 21 patients with familial disease. The new tumor was macula in 2 (4%), between the macula and equator in 30 (55%), and between the equator and ora serrata in 23 (42%). Overall, according to Kaplan-Meier analysis, new tumor development occurred in 23% of patients by 1-year follow-up and 24% by 5-year follow-up. By multivariate analysis, the most important risk factors for the development of new tumors was younger age at presentation (median age, 2 months with new tumor vs 9 months without new tumor) and family history of retinoblastoma (12 [48%] of patients with new tumor vs 14 [17%] without new tumor). CONCLUSIONS: Children with retinoblastoma treated with chemoreduction should be followed for new intraretinal tumor development, as it peaks at a mean interval of 5 months after initiation of chemoreduction and affects 24% of patients by 5 years of follow-up. New tumors are most commonly found in those who develop disease as young infants and those with a family history of retinoblastoma.

Antineoplastic Combined Chemotherapy Protocols↗