Extraocular muscle involvement in sarcoidosis

BACKGROUND: Sarcoidosis is a granulomatous inflammatory disease that may have a variety of ocular and orbital manifestations. The most common ocular manifestation is uveitis, and the most common orbital manifestation is dacryoadenitis. Extraocular muscle involvement in sarcoidosis has rarely been reported. The authors report a case of sarcoidosis involving the extraocular muscles of a 15-year-old boy with bilateral, painful, external ophthalmoplegia and enlargement of all extraocular muscles on computed tomography (CT) scan. RESULTS: Lateral rectus muscle biopsy and transbronchial lung biopsy showed noncaseating granulomas characteristic of sarcoidosis. Cultures and serologic studies excluded fungal and mycobacterial diseases. Treatment with oral corticosteroids improved symptoms and signs. CONCLUSIONS: The authors report the first case of sarcoidosis in a patient with symptomatic extraocular muscle involvement, and only the third case in which extraocular muscle involvement has been shown histologically.     

Gadolinium enhancement: improved MRI detection of retinoblastoma extension into the optic nerve

We performed T1-, T2-, proton density-weighted, and T1-weighted gadolinium-enhanced MRI on 24 patients with retinoblastoma, using a 1.5 T superconducting unit and head and orbital surface coil imaging. All patients underwent a complete ophthalmologic examination, including B-scan ultrasonography. CT was performed on 10 of 24 patients. Pathologic correlation was obtained in 18 patients who required enucleation. Contrast-enhanced T1-weighted MRI with fat suppression was the sequence most sensitive to optic nerve extension and provided the greatest differentiation between tumor and uninvolved extrascleral tissue. Retinoblastoma demonstrated contrast enhancement.     

Specific metal-oligonucleotide binding studied by high resolution tandem mass spectrometry

BACKGROUND: Benign reactive lymphoid hyperplasia is an uncommon uveal tumor. Misdiagnosis may lead to unnecessary enucleations. Clinical evaluation alone is usually not sufficient to diagnose this tumor. OBJECTIVE: To evaluate the utility of standardized echography in the recognition of this disease. METHODS: Retrospective review of the medical and echographic records of 6 patients with choroidal involvement of benign reactive lymphoid hyperplasia. RESULTS: Extraocular episcleral lymphoid nodules were present in 5 of the 6 patients and were usually seen as a late manifestation of the disease. Two patients had repeated echography after treatment of the tumor and showed marked regression of the episcleral nodules. CONCLUSIONS: A combination of clinical and echographic findings can assist in the accurate diagnosis of this rare uveal tumor. The presence of episcleral nodules on standardized echography supports the diagnosis.     

Prevalence and management of elevated intraocular pressure in patients with Graves' orbitopathy

AIMS: To investigate the prevalence and to discuss the necessity of treating elevated intraocular pressures (IOP) in patients with Graves' orbitopathy (GO). In addition, to study the effects of orbital decompression and extraocular muscle surgery on IOP. METHODS: The records of consecutive patients with GO referred in a 5 year period were studied and those selected, in which glaucoma medication had been prescribed, or a diagnosis of primary open angle glaucoma (POAG) or of ocular hypertension (> or = 22 mm Hg) (OH) had been made. The necessity of treating these patients with glaucoma medication was questioned and the effects of corticosteroids, orbital decompression, and extraocular muscle surgery on the IOP were evaluated. RESULTS: Of 482 patients with GO, 23 (4.8%) met the inclusion criteria. Four patients (0.8%) had POAG, four had elevated IOPs and visual field defects consistent with dysthyroid optic neuropathy, and 15 (3.1%) had only elevated IOPs. Five patients with OH showed a permanent drop of IOP after orbital decompression, two had a marked decrease of their IOP after recession of the inferior rectus muscle. CONCLUSIONS: POAG has the same prevalence in the general Dutch population as in the GO subgroup. The combination of elevated IOPs and visual field defects in GO patients may be attributed to other mechanisms than obstructed aqueous outflow in the trabecular meshwork and should be treated accordingly. Orbital decompression and extraocular muscle surgery may lower the IOP in patients with GO.     

MRI in the diagnosis of osteoid osteoma

INTRODUCTION: Osteoid osteoma (OO) is a frequently encountered benign bone tumor, seen in young adults with male predominance. MATERIALS AND METHODS: Nine patients complaining of nonspecific extremity pain underwent MRI examination. The sequences obtained were T1 and T2 weighted spin-echo and T2 weighted gradient echo. A CT scan examination followed in all cases, exploring the region of the abnormal signal seen on MRI. The results of both examinations were compared. RESULTS: In six of the nine patients (66.6%) MRI showed evidence suggestive of osteoid osteoma, comparable that seen on CT scan. In three patients (33.3%), MRI showed a nonspecific and ill-defined bone marrow signal abnormality. CT cuts focused on those areas of signal abnormality showed the nidus. DISCUSSION: MRI is more sensitive than CT scan in detecting soft tissue and bone marrow abnormalities adjacent to an osteoid osteoma. This may produce a misleading aggressive appearance on MR images. CT scanning is more specific than MRI, by showing the nidus. In three patients studied, the nidus was only seen by CT, the other six osteoid osteomas were equally seen by CT and by MRI. In our study, MRI revealed abnormalities in all the cases. It was also highly specific for osteoid osteoma in 66.6%. CONCLUSION: MRI is very sensitive in detecting bone marrow and soft tissue abnormalities, and can suggest the diagnosis of OO in a good number of patients. In the remainder cases MRI guides the CT-scan. CT is more accurate and remains the definite examination for the diagnosis of OO, by showing the nidus.     

Questionability of the benefits of routine laparotomy as the surgical approach for pheochromocytomas and abdominal paragangliomas

BACKGROUND: Improvement of preoperative imaging of pheochromocytomas and abdominal paragangliomas may render routine laparotomy questionable as the surgical approach of choice for these lesions. METHODS: We studied the records of 100 patients with chromaffin tumors who underwent abdominal exploration. The disease was familial in 28 patients and was malignant in 19. Seventy-five patients had intraadrenal disease (bilateral in 13). Computed tomography (CT), metaiodobenzylguanidine (MIBG) scintigraphy, and magnetic resonance imaging (MRI) were performed since 1979, 1984, and 1987 in 97, 73, and 43 patients, respectively. False-positive and false-negative results were defined as any discrepancy between imaging results and surgical findings. RESULTS: Overall accuracy of preoperative localization was 85% with CT scan, 77% with MIBG scintigraphy, and 86% with MRI. In unilateral pheochromocytoma, accuracy was 94% with CT scan, 80% with MIBG scintigraphy, and 96% with MRI. When all three studies were performed (n = 38), overall accuracy was 97% and only one extraadrenal tumor in a patient with familial pheochromocytoma was overlooked. CONCLUSIONS: The outstanding accuracy of available imaging techniques questions the strategy of routine laparotomy for sporadic and seemingly benign pheochromocytomas, favoring more elective approaches such as the posterior approach or laparoscopy.     

Ring recurrence of ciliary body melanoma after proton-beam therapy

A malignant ciliary body melanoma was treated by proton-beam irradiation. Ring-shaped recurrence with extraocular extension appeared thirty-six months later and required enucleation. Histologic study revealed a predominant epithelioid cell type tumor, flattened in previously treated area. Proton-beam irradiation efficiency depends on clinical evaluation, which remains questioned in ciliary body locations where circumferential extension could be misevaluated.     

Orbital extension of Choroidal and ciliary body melanomas

In a review of 432 choroidal and ciliary body melanomas, histopathologic evidence of orbital extension was found in 45 cases (10.4 percent). The occurrence of orbital extension was influenced mainly by the cell type (P less than .05) and by the size of the choroidal mass (P less than .001). Local orbital recurrence occurred in 23 percent of cases with evidence of extrascleral extension. Survival rates were independent of cell type, pigmentation, and size and shape of the extension. Patients treated by exenteration had significantly better prognosis than nontreated patients or patients treated by chemotherapy, radiotherapy, or local excision (P equals .0002). Early exentration is the best treatment for choroidal melanomas with orbital extension.     

Magnetic resonance imaging in 80 cases of orbital tumors

Magnetic resonance imaging (MRI) is a new medical imaging technique which has been used in evaluating ocular and orbital diseases in recent years. Owing to the high resolution of soft tissues and lesions, it is superior to ultrasound and computed tomography in the localization and characterization of an orbital tumor in diagnosis. In this paper, we analysed 80 cases of MR images of orbital tumors by which all the lesions were detected. Benign or malignant disorders were recognized in 59 cases and complete tumors with accurate localization were shown in 79 cases. The principles of MRI, signal intensity of various tumors and the indications of MRI were discussed.     

Renal cell carcinoma: preoperative assessment for enucleative surgery with angiography, CT, and MRI

PURPOSE: Our purpose was to assess various imaging methods in detecting a pseudocapsule of renal cell carcinoma (RCC), which is critical for successful tumor enucleation. METHOD: In 42 patients with histopathologically proven RCC, images obtained at angiography (n = 42), CT (n = 30), and MRI (n = 19) were investigated retrospectively. All patients underwent treatment (enucleation: n = 15; nephrectomy: n = 27). The imaging criteria for the presence of a pseudocapsule were as follows: a surrounding radiolucent rim on angiography, a low or high density rim on CT, and a low intensity rim on MRI. All images were retrospectively reviewed by three radiologists without knowledge of the clinical and histological findings. RESULTS: Thirty-three of 42 RCCs showed a pseudocapsule on the surgical specimen. A pseudocapsule was detected in 67% of tumors (22/33) on angiography, 26% (6/23) on CT, 27% (4/15) on T1-weighted MRI, 93% (14/15) on T2-weighted MRI, 67% (8/12) on dynamic enhanced T1-weighted MRI, and 15% (2/13) on delayed enhanced T1-weighted MRI. CONCLUSION: T2-weighted MR images are superior for visualizing a pseudocapsule of RCC and for providing reliable selection criteria for tumor enucleation.     

Simultaneous enucleation of a pulmonary melanoma metastasis and myocardial revascularization

BACKGROUND: The incidence of late, recurrence of malignant melanoma, is a well known, but very rare clinical experience. CASE REPORT: We report a case of simultaneous myocardial revascularisation and resection of pulmonary melanoma metastasis. In 1963 an enucleation of the right eye was necessary due to an ocular melanoma. In 1993 the patient suffered acute left heart failure and a 3-vessel disease with severe reduced left ventricular function was diagnosed. Chest X-ray examination revealed a singular pulmonary lesion in the right lower lobe with a diameter of 5.5 cm. Myocardial revascularisation and resection of the pulmonary focus was performed simultaneously without complication. The histological examination documented a pulmonary late recurrence of malignant melanoma. Up to this time (3 years later) the patient is free of cardiac symptoms and there is no evidence of further late recurrence of malignant melanoma. CONCLUSION: The appreciable number of patients who, after a disease-free interval of 10 to 20 years, develop a late recurrence of a malignant melanoma, and in particular-as in the present case-a choroidal melanoma, is powerful evidence for a need to keep these patients under lifelong surveillance.     

Clear cell differentiation in choroidal melanoma. COMS report no. 8. Collaborative Ocular Melanoma Study Group

OBJECTIVE: To describe 2 enucleated eyes of patients enrolled in the Collaborative Ocular Melanoma Study that contained primary choroidal melanoma with clear cell features. METHODS: During a 9-year period, 1493 eyes enucleated as part of the Collaborative Ocular Melanoma Study routinely processed for histologic examination were evaluated by the pathology review committee (H.E.G, D.M.A, and W.R.G). Two eyes with unusual variants of choroidal melanoma were identified and immunostained for S100 protein and HMB 45. Portions of the tumors were processed for electron microscopic examination. RESULTS: Results of electron microscopic examination of both tumors displayed malignant melanoma (mixed cell type with many malignant cells with clear cytoplasm). The cytoplasm of the clear cells stained with periodic acid-Schiff and failed to stain when pretreated with diastase. Results of immunohistochemical stains in both tumors were positive for S100 protein and HMB 45 in the tumor cells. Results of electron microscopic examination showed that the cytoplasm of the clear cells contained scattered glycogen granules, premelanosomes, and melanosomes. CONCLUSION: These cases represent a clear cell variant of malignant melanoma of the choroid. This tumor should not be confused with metastatic clear cell carcinoma to the choroid.     

A study to determine how needs assessment is being used to improve health

Progressive, left exophthalmos developed due to a left, retrobulbar mass in a 76-year-old Japanese woman. An open tumor biopsy was carried out, and both macro- and microscopic findings of the mass confirmed that it was a malignant melanoma. Orbital melanomas usually result from distant metastasis of cutaneous melanomas or from secondary extension of ipsilateral intraocular melanomas. Thorough physical, laboratory, and radiological examination, however, did not disclose any primary cutaneous or visceral melanoma, nor had the patient any previous history of excision or spontaneous regression of a pigmented lesion. Histopathologically, the left retrobulbar melanoma was rich in sinusoidal vessels which were surrounded by melanoma cells, oriented in a perpendicular array, a histological feature more characteristic of uveal melanomas than of cutaneous ones. The ophthalmological examination excluded development of a primary intraocular melanoma on the left side. Fundoscopic examination of the right eye was not feasible because of the complete opacity of the right vitreous body which had resulted from previous episode of idiopathic vitreous hemorrhage. Unexpectedly, CT and MR studies depicted retrobulbar masses of non-homogeneous densities in the bilateral orbits. These radiologic studies indicated the metastatic nature of the left retrobulbar melanoma, while suggesting the development of a primary, intraocular melanoma on the right side, extension into the right orbit, and involvement of the right optic nerve. All these clinical, radiological, and histological data suggested the development of a primary melanoma in the right eye and subsequent metastasis to the left orbit producing exophthalmos. The mechanism of such a peculiar mode of metastasis remains entirely unknown. This is a rare case of metastatic orbital melanoma, without visceral involvement, which originated in the contralateral eye. Development of the right ocular melanoma remained unrecognized due to atrophic degeneration of the right eyeball and complete opacity of the right vitreous body, until the contralateral orbital metastasis grew massive enough to cause exophthalmos.     

Value of MRI, CT and findings in staging of gynecologic malignancies

PURPOSE: The present study was designed to compare the value of MRI, CT and clinical examination in local tumor staging of gynaecologic malignancies. PATIENTS AND METHODS: 99 patients with clinically suspected carcinoma of the cervix uteri, ovarian carcinoma or tumor recurrence after gynaecologic cancer underwent all three staging modalities. Furthermore CT and MRI were compared in detecting lymph node metastasis and peritoneal implants. RESULTS: MRI was superior to CT and clinical staging in local tumor staging with an accuracy of 77% for cervical carcinoma and of 88% for recurrent tumors, whereas CT achieved an accuracy of 65% and 55% and clinical staging 60% and 63% accuracy for carcinoma of the cervix and recurrent cancer. Especially for local staging of these two tumor entities MRI is very useful. MRI and CT reached comparable results in the detection of ovarian tumors with an accuracy of 73% for MRI and 69% for CI. Both imaging modalities also showed equal results in the detection of lymph node metastasis, so that primary the cost saving use of CT tumor staging for ovarian lesions and lymph node metastasis should be favoured.     

The effects of prostaglandin E1 and tyrosine kinase inhibitors on energy status and protein synthetic ability in hepatic ischemia-reperfusion injury

AIM: Preseptal and orbital cellulitis are rare presenting features of intraocular retinoblastoma. The objectives of this study were to determine the frequency of retinoblastoma associated cellulitis, as well as to review its clinical and histopathological features. METHODS: The medical records of 292 retinoblastoma patients in the King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia were reviewed. Those indicating a history of, or presenting with, cellulitis were retrieved and their clinical, radiological, and histopathological variables were assessed. Patients with definite extraocular tumour extension on clinical or radiological examination were excluded. RESULTS: 14 patients were found to have retinoblastoma associated cellulitis (4.8%); nine had bilateral and five had unilateral retinoblastoma. Conjunctival and blood cultures were performed in 10 cases and were negative. 10 children were treated with intravenous steroids, often in conjunction with antibiotics, resulting in a prompt decrease in inflammation. Three other children were treated with antibiotics alone and one received no treatment. Computed tomographic scanning depicted large intraocular tumours occupying between 80% and 100% of the globe in each case. In eight patients, periocular inflammation was radiologically interpreted as possible extraocular extension. In one patient serial computed tomographic scanning showed a reduction in intraocular calcification over time which occurred in the presence of cellulitis, 12 patients underwent enucleation and histopathological examination revealed large necrotic, poorly differentiated tumours associated with uveal involvement and early optic nerve invasion. Focal perilimbal destruction was seen in one patient, and in another peripapillary extrascleral extension was present. 12 patients are alive with a mean follow up of 56.4 months. CONCLUSIONS: Radiological evaluation of scleral integrity may be hindered by periocular inflammatory changes. The orbital cellulitis correlated well with the presence of advanced intraocular retinoblastoma with massive necrosis and anterior chamber involvement. In the majority of patients, cellulitis was not indicative of an extension of retinoblastoma into the orbit. Intravenous steroid treatment reduced orbital inflammation, facilitating examination and subsequent enucleation.     

Invasive squamous cell carcinoma arising from asymptomatic choristomatous cysts of the orbit. Two cases and a review of the literature

BACKGROUND: Epithelial choristomatous cysts are common orbital lesions, the most frequent of which are dermoid or epidermoid tumors. Massive enlargement or extraorbital extension of these benign lesions may occur. Malignant transformation of the epithelial lining of epidermoid cysts is rarely reported. METHODS: Two patients are presented in whom the epithelial lining of a previously asymptomatic choristomatous cyst of the orbit underwent malignant transformation to produce invasive squamous cell carcinoma. The unusual origin and clinical presentation of the lesions caused a delay in the diagnosis and therapy in both patients. Metastatic workup was negative in both patients, who underwent resection of their tumors via orbital exenteration with craniofacial resection. Reported cases of malignant transformation in analogous choristomatous cysts elsewhere in the cranium are reviewed. RESULTS: One patient is alive and well without recurrent disease 40 months postoperatively. The second patient died of a pulmonary embolus 2 months postoperatively. Autopsy showed no residual tumor. Overall, only 3 of 18 reported patients with epidermoid choristoma of the head and orbit with malignant transformation were alive when reported. CONCLUSIONS: Malignant squamous metaplasia is believed to be a rare complication of orbital dermoid or epidermoid cysts, with only two previously reported cases. However, malignant transformation is relatively frequent in analogous epidermoid cysts found elsewhere in the cranial vault, especially after incomplete excision. These reports encourage the complete removal of epithelial choristomas of the orbit.     

Choroidal malignant melanoma

Choroidal nevi are found on routine fundus evaluations in 1% to 2% of patients. It is felt that these usually benign lesions are the precursors of choroidal malignant melanomas. The incidence of choroidal malignant melanoma in the United States is approximately 6 cases per 1 million persons per year. Diagnosis is multifactorial, based on the tumor's funduscopic, angiographic, and ultrasonographic appearance. Once a melanoma is diagnosed in a patient, a complete metastatic workup is performed. A small tumor is observed for change. Medium-sized tumors are treated with radioactive plaque therapy, external beam radiation, or enucleation. Large tumors are treated with enucleation with or without external beam radiation. Studies are underway to establish the optimum form of treatment.     

Neuroligand receptor heterogeneity among astroglia

The clinical and histopathological findings are described in a 39-year-old female patient with two different primary ophthalmic cancers, involving the adnexa and ocular globe of the same eye. The first primary tumor was a malignant fibrous histiocytoma of the left lower eyelid aggressively invading the nasogenian region, bulbar conjunctiva, episclera, and the cornea over a 36-year follow-up period. The second primary cancer was an unsuspected choroidal malignant melanoma unexpectedly found at histology. The possible correlations between these two malignancies are discussed.     

Optic nerve involvement in acute lymphoblastic leukemia

Leukemic infiltration of the optic nerve is rare. We describe the diagnostic assessment and the outcome in two adult patients suffering from acute lymphoblastic leukemia with T phenotype. In both cases the leukemic involvement of the eye was observed as an isolated extramedullary relapse followed after several months by hematological relapse. CT and MRI scans were negative, while an A-scan echography of the eye clarified the diagnosis. Early radiotherapy caused recovery of visual acuity in one case. A-scan echography is the most sensitive investigation for the early recognition of ocular localization in acute leukemias.     

Melanoma in Togo. A retrospective study over 20 years

INTRODUCTION: The malignant melanoma is a rare malignant tumour in black patients, but it is common in white patients. MATERIAL AND METHODS: Medical records were reviewed retrospectively during 20 years (1973-1992) to determine the epidemiological features of malignant melanoma in Togo. RESULTS: During this period 63 cases (31 males and 32 females) of malignant melanoma were histologically diagnosed; an average of 3.15 cases each year. The tumor takes place preferably on the feet 40 cases (63.49%), hands 15 cases (19.04%), legs and thigh 10 cases (15.87%). CONCLUSION: The results of this study confirm the particularity of malignant melanoma in black subjects: its rarity and the feet localizations.