Metastasis of advanced gastric cancer to the extraocular muscle: a case report.

BACKGROUND: Metastatic tumors in the orbit, especially from gastric cancer, are rare. We present a rare case of extraocular muscle metastasis from gastric cancer and raise consideration of metastasis to extraocular muscle as a differential diagnosis of proptosis/lid swelling in a patient with history of malignancy. CASE PRESENTATION: A 54-year-old Japanese woman presented with proptosis, lid swelling, diplopia, and retro-orbital pain in her left eye, which she had been experiencing for 1 day. She had a medical history of poorly differentiated adenocarcinoma of the stomach, which had metastasized to several organs. A computed tomography scan showed enlargement of the medial rectus muscle in her left eye. She was diagnosed as having gastric cancer metastasis to the medial rectus muscle of her left eye, and received a total of 20 Gy radiation therapy to the orbit, which resulted in resolution of her ocular symptoms. She died 3 months after her initial visit to our ophthalmic department. CONCLUSIONS: Metastasis from malignancy should be considered in the differential diagnosis of a patient presenting with proptosis or lid swelling who has a history of gastric cancer. Radiation therapy of metastases in the orbit may be an effective treatment in such cases.

Vulvar Melanoma with Isolated Metastasis to the Extraocular Muscles: Case Report and Brief Literature Review.

BACKGROUND/AIM: Orbital metastasis of systemic cancer is exceedingly rare. This is a case report of a patient treated for locally recurrent vulvar melanoma who later presented with unilateral proptosis and was found to have an isolated biopsy-proven extraocular muscle metastasis. PATIENTS AND METHODS: A 94-year-old female with locally recurrent vulvar melanoma presented with eye discomfort and blurry vision. Patient underwent histopathological, genetic, and imaging studies. RESULTS: All prior work-up, including brain MRI and PET/CT, was negative for disease elsewhere from local recurrence. Orbital MRI demonstrated a mass involving the extraocular muscle, and immunohistochemistry staining of biopsy was consistent with metastasis. The patient underwent radiation therapy and tolerated treatment well. CONCLUSION: This is the first reported case of vulvar melanoma with extraocular muscle metastasis. The absence of findings on imaging as part of the staging work-up underscores the importance of considering extraocular muscle (EOM) metastasis as a differential for patients with vulvar melanoma who present with proptosis.

A case report of unifocal Langerhans' cell histiocytosis or eosinophilic granuloma.

A case of unifocal Langerhans' cell histiocytosis or eosinophilic granuloma in a child is reported where the frontal, zygomatic and maxillary bones of the left orbital wall are affected. As eosinophilic granuloma is a rare disease and the involvement of maxillary bone in orbital eosinophilic granuloma is not well documented in the literature, this case is reported for its unusual involvement of 3 bones at a single site. The diagnosis was established by clinical, radiological and histological findings. As no other system or site was involved and considering the osseous involvement of multiple bones at one site, local radiotherapy was preferred as the mode of treatment. The patient responded favourably to radiotherapy with reduction of proptosis and tumour mass but there was no visual recovery. In the absence of universal agreement over the mode of treatment, the result emphasises the probable benign nature of the tumour and the need to withhold more aggressive treatment modalities for extensive multisystem involvements.

Exoftalmos maligno en hipertiroidismo con crisis hipotiroidea / Malignant exophtalmos in hyperthyroidism with a hypothyroid crisis

Caso clínico: Presentamos un paciente varón de 56 años de edad con un exoftalmos maligno en el contexto de una enfermedad de Graves, que sufrió un claro empeoramiento coincidiendo con su entrada en crisis hipotiroidea, con una evolución tórpida, que requirió de distintas medidas terapéuticas para la reducción de la proptosis. Discusión: En el manejo de este tipo de pacientes hay que tener en cuenta si cumplen o no criterios de gravedad y/o actividad para aplicar las distintas opciones terapéuticas en el momento preciso

Case report: We present the case of a 56-year-old male with a malignant exophthalmos in the context of Graves’ disease. He suffered a significant worsening coincident with a hypothyroid crisis, and various therapeutic approaches were required for reduction of the proptosis. Discussion: When managing this condition, both the severity of the exophthalmos and the activity of the thyroid must be considered when choosing which of the different therapeutic approaches should be employed

Treatment of benign diseases with megavoltage X-ray beams: is there a risk for gonadal damage?

AIMS: To measure the testicular and ovarian doses and to assess the risk for gonadal damage to patients treated with megavoltage X-ray beams for benign diseases. MATERIALS AND METHODS: Radiation therapy of benign diseases was simulated on an anthropomorphic phantom with a 6MV photon beam. The gonadal dose was calculated during the irradiation of heterotopic ossification, liver and vertebra haemangiomas, bone cysts, Graves' ophthalmopathy and gynaecomastia. Dose measurements were carried out using thermoluminescent dosimeters. For the radiotherapy of heterotopic ossification, the effect of using lead blocks to spare lymphatic drainage on the gonadal dose was determined. RESULTS: The ovarian and testicular total doses were found to be 2.00-680 and 2.0-39.0 mGy, respectively, depending on the gonadal location in respect to the treatment volume. The introduction of blocks into the primary beam resulted in an increase in gonadal dose up to a factor of 1.7. The radiation-induced risk of hereditary disorders in future generations was (1.0-40.8) x 10(-4) and (1.0-23.4) x 10(-4) for women and men, respectively. CONCLUSIONS: Radiation therapy of benign diseases always resulted in gonadal doses below 1 Gy and therefore there was no risk for permanent gonadal failure. The excess risk of radiation-induced hereditary disorders in offspring was low in comparison with the natural frequency of these effects. However, there was a considerable excess in risk after irradiation in the hip bone.

[Malignant exophtalmos in hyperthyroidism with a hypothyroid crisis]. / Exoftalmos maligno en hipertiroidismo con crisis hipotiroidea.

CASE REPORT: We present the case of a 56-year-old male with a malignant exophthalmos in the context of Graves' disease. He suffered a significant worsening coincident with a hypothyroid crisis, and various therapeutic approaches were required for reduction of the proptosis. DISCUSSION: When managing this condition, both the severity of the exophthalmos and the activity of the thyroid must be considered when choosing which of the different therapeutic approaches should be employed.

Functional outcome of patients with benign meningioma treated by 3D conformal irradiation with a combination of photons and protons.

PURPOSE: To evaluate efficacy and tolerance of external fractionated combination of photon and proton radiation therapy (RT) for intracranial benign meningiomas. METHODS AND MATERIALS: Between 1994 and 2002, 51 patients with intracranial meningiomas of the base of the skull were treated with a combination of photon and proton RT. Median total dose was 60.6 cobalt Gy equivalent (54-64). One hundred eight eye-related symptoms were collected; 80 other symptoms were noted and followed up. RESULTS: Mean follow-up was 25.4 months. Acute tolerance was excellent. Out of the 108 eye-related symptoms, 106 (96%) were evaluated. Improvements were reported for 73 (68.8%) of them. Out of the 88 other miscellaneous symptoms, 81 (92%) were evaluated. Improvements were reported in 54 cases (67%). Median time to improvement ranged from 1 to 24 months after completion of the radiotherapy, depending on the symptom. We did not observe any worsening of primary clinical signs. Radiologically, 1 patient relapsed 4 months after the end of irradiation. Pathology revealed a malignant (Grade 3) transformation of the initial Grade 1 meningioma. Four-year local control and overall survival rates were, respectively, 98% and 100%. Stabilization of the tumor was observed in 38 cases (72%), volume reduction in 10 cases (20%), and intratumor necrosis in 3 cases. Two patients complained of Grade 3 side effects: 1 unilateral hearing loss requiring aid and 1 case of complete pituitary deficiency. CONCLUSION: These results stressed the clinical efficacy of fractionated-associated photon-proton RT in the treatment of meningiomas, especially on cranial nerve palsies, without severe toxicity in almost all patients.

[Therapy for Graves' ophthalmopathy].

Graves' ophthalmopathy (GO) is also called thyroid-related eye disease, infiltrative ophthalmopathy, which is related with the autoimmunity of thyroid, especially hyperthyroidism. Its morbidity ragnes from five percent to ten percent of hyperthyroidism, and the morbidity of male patients is higher than that of the female patients. The treatment of severe GO is a difficult task for doctors. The therapeutic effect is not always satisfactory. In order to solve this knotty problem, researchers have been devoting themselves to the development of new therapeutic methods. Here, the development of the therapies for GO is introduced, and the trends of treatments are prospected.

Radiotherapy for Graves' orbitopathy: results of a national survey.

BACKGROUND: Graves' orbitopathy (GO) is a widely accepted indication for radiation therapy (RT). In conjunction with the German Cooperative Group on Radiotherapy for Benign Diseases (GCG-BD), a national survey was conducted in order to assess whether or not there is a consensus on the indication for RT and various treatment factors which were studied. MATERIAL AND METHODS: A questionnaire was circulated to 190 RT institutions to obtain relevant data concerning the patients' workload, stage-dependent indication, and diagnostic procedures, which were considered to be necessary. Further questions addressed details on radiation technique and dose-fractionation schedules, the combined use of corticoids, and salvage RT after previous treatment failure following RT. RESULTS: With a response rate of 152/190 (80%), the survey is nationally representative. Based on the case workload, an estimated annual number of 1,600 GO cases are treated in German radiotherapy departments. With an 88% consensus, stages II-V are the typical indications. 85% considered imaging studies necessary for indication and only 48% for laboratory tests. 76% of the institutions used total doses in the range of 15-20 Gy, and conventional fractionation was most common (57%). 82% used a face mask fixation and 67% CT-based treatment planning. Approximately 50% would prescribe salvage RT, and total doses in the range of 20-40 Gy were considered to be acceptable. CONCLUSIONS: The survey revealed a consensus concerning most of the factors studied. We recommend to review the patterns of care for RT of other entities of benign diseases and to implement a quality assurance program both on national and international levels.

[Exophthalmos secondary to a lateral rectus muscle metastasis]. / Exoftalmos secundario a metástasis en músculo recto externo.

CASE REPORT: 60-year-old male, with metastatic adenocarcinoma of the rectum-sigmoid colon, who developed exophthalmos due to a tumour in the lateral rectus muscle, compatible with a diagnosis of metastasis. DISCUSSION: Metastasis in the extraocular muscles are very uncommon, but should form part of the differential diagnosis of any patient presenting exophthalmos, since although the majority of patients with such metastasis have a previously diagnosed malignant tumour, there are cases where they lead to the diagnosis of the primary tumour.

[Ablative therapy for immune hyperthyroidism in patients with ophthalmopathy]. / Die ablative Therapie der Immunhyperthyreose unter spezieller Berücksichtigung der endokrinen Orbitopathie.

The influence of ablative treatment of Graves' disease on the course of endocrine ophthalmopathy is discussed controversially. Data are inhomogeneous, because some studies were performed retrospectively, some prospectively, because of different follow up periods and different patient groups. In principal near total resection seems to influence endocrine ophthalmopathy in a positive way, due to the removal of thyroid antigen. Most studies also demonstrate that treatment using radioiodine has a negative influence on ophthalmopathy, because the course of disease is worsening in up to one third of patients. There is also consensus that treatment of Graves' disease with radioiodine should be performed only by concomitant administration of glucocorticoids. The early administration of L-thyroxine after radio-iodine therapy of Graves' disease seems to have a positive influence on the course of disease, whereas additional treatment with methimazole has no positive influence on endocrine ophthalmopathy.

Radiation response of Rosai-Dorfman disease presenting with involvement of the orbits.

The purpose of this study was to review an interesting case of recurrent orbital sinus histiocytosis. The patient initially failed surgery, chemotherapy, and steroid therapy, only to have a durable response to low-dose radiation therapy of the orbits, lasting 6 and 11 years, respectively. Because there are few documented responses to radiotherapy, we present a case report in conjunction with the clinical, radiographic, and histopathologic information as well as a literature review of similar cases.

Radiotherapy in the management of Graves' ophthalmopathy.

BACKGROUND: To report the results of radiotherapy for patients with failure, adverse reactions or relative contraindications to the use of steroids or immunosuppressants, by using newly developed quantitative indexes. METHODS: Fourteen female and six male patients with Graves' ophthalmopathy were treated with radiotherapy between 1989 and 1996. Prior to radiotherapy, eight patients received treatment with prednisone, four received immunosuppressants and four received a combination of both. Four patients with contraindications to steroids were initially managed with radiotherapy. Most of the patients received a dose of 24-28 Gy in 2 Gy fractions. We used the newly developed motility limitation index to assess extraocular motility. RESULTS: Treatment was well tolerated. There have been no late complications. All 12 patients with soft tissue signs such as edema, irritation, tearing and pain were improved. Proptosis did not improve or improved only slightly, 3 mm at best. However, proptosis in all but two has been stabilized and has not deteriorated in the follow-up period. Most of the patients have experienced an improvement of eye-muscle motility. Extraocular muscles that work for elevation were impaired more severely than the other muscles and this tended to remain. Of the 16 patients using steroids before or when radiotherapy was initiated, 15 were tapered off and only one patient required additional steroids, thus sparing the majority from steroid adverse reactions. CONCLUSION: Radiotherapy was effective in preventing exacerbations of active inflammatory ophthalmopathy in patients with Graves' disease with minimal morbidity and thus eliminated the adverse reactions associated with protracted corticosteroid use. The newly developed motility limitation index was useful in detecting delicate changes in motility of individual extraocular muscles.

[The current role of radiotherapy in immunogenic exophthalmos]. / Il ruolo attuale della radioterapia nell'esoftalmo immunogenico.

In this study, the value of radiation therapy was investigated in 41 patients with Graves' ophthalmopathy treated with orbital irradiation at the Radiotherapy Department of Mauriziano Hospital in Turin, 1986 through 1993. Our series consisted of 41 patients (32 women and 9 men), whose mean age was 53.6 years (range: 26-72 years). All patients had a high index of endocrine ophthalmopathy, based on the American Thyroid Association classification-NOSPECS classes III and IV. Irradiation was administered with two opposed convergent beams tilted posteriorly 5-10 degrees with 20 Gy/12 fractions/2 weeks. The total dose delivered to the lens was than 5%. The follow-up consisted of endocrinologic and ophthalmologic tests and, if possible, of pre/post-irradiation orbital US. A positive result was obtained in 31 patients, which was very good in 22 of them. Severe complications, i.e., 1 corneal ulceration and 3 cataracts, were observed in the patients with associated ocular conditions and were treated simultaneously with high-dose corticosteroids so that no direct and unquestionable correlation can be made between irradiation and complications. To conclude, our data show that radiation therapy can improve the signs and symptoms of Graves' disease, as many authors report.

[Is treatment with corticotherapy or radiotherapy effective for involvement of ocular motility and for reduction of exophthalmos in Basedow's disease?]. / Le traitement par corticothérapie ou radiotherapie est-il efficace pour l'atteinte de la motilité oculaire et pour la réducion de l'exophtalme dans la maladie de Basedow?

It is acknowledged that high-dose corticotherapy, radiotherapy, or surgical decompression of the orbit are often efficient in dysthyroid optic neuropathy. Corticotherapy and radiotherapy are also given to patients with dysfunction of eye-muscle motility and/or proptosis in the absence of visual loss. The latter indication has been retrospectively evaluated in 13 patients. The limited character of our series is partially explainable by strict inclusion criteria (objective evaluation of muscle dysfunction and proptosis). We failed to demonstrate any significant functional improvement after oral corticotherapy and/or radiotherapy. It thus appears that our study sheds doubt on the actual efficacy of these treatments in patients presenting with not very evolutive involvement of eye-muscle motility. A larger study aimed at comparing the benefits and drawbacks of these treatments is consequently to be encouraged.

Supervoltage orbital radiotherapy in 36 cases of Graves' disease.

Thirty-six patients who had clinically progressive Graves' dysthyroid orbitopathy were treated with supervoltage orbital radiotherapy. Twenty-three of the patients had discontinued the use of orally administered prednisone after developing intolerable side effects. Medical contraindications prevented seven patients from receiving systemic corticosteroid treatment. Six additional patients declined to take prednisone and chose orbital radiotherapy as their primary treatment. All patients were treated with one radiotherapy protocol wherein a 6 MV linear accelerator delivered 2,000 cGy to the midplane of both orbits in ten fractions. None of the 36 patients was treated with corticosteroids during the orbital radiotherapy treatment interval. We encountered three patients who failed to respond to orbital radiotherapy and required supplemental immunosuppression to arrest progression of Graves' disease. The remaining 33 patients experienced stabilization or clinical improvement of their condition. None of the patients experienced complications from orbital irradiation. We believe supervoltage orbital radiotherapy is an effective means of treating Graves' dysthyroid orbitopathy.

[Aspects of exophthalmos in relation to treatment for dysthyroid ophthalmopathy].

257 eyes of 135 patients with dysthyroid ophthalmopathy were treated with steroids and/or radiation. Steroid therapy consisted of 100-150 mg betamethasone given intravenously and orally. Radiation therapy was conducted with 1.5 Gy Lineac delivered to the retrobulbar tissue from two directions once a day for a period of 10 days. In combination therapy, the above two therapies were performed simultaneously. Exophthalmos and the new index of the c/o ratio (c/o ratio; defined as the content of the orbit divided by the capacity of the bony orbit) were measured on CT films and followed for 1 or 2 years in order to evaluate the effectiveness of these therapeutic modalities. Combination therapy yielded the best results among these therapies. Steroid therapy yielded good results for 3 or 6 months, but rebound phenomenon was recognized after a course of 1-2 year.