PURPOSE: To report a case where bilateral malignant retrobulbar lymphoma was diagnosed after repetitive intraocular lens dislocation to the anterior chamber. CASE SUMMARY: An 85-year-old male with a history of stroke who had undergone cataract surgery 10 years ago at another hospital presented with repeated intraocular lens (IOL) dislocations of both eyes into the anterior chamber. He had previously undergone IOL scleral fixation once in his left eye and twice in his right eye, but IOL dislocation was still repeatedly occurring. The best-corrected visual acuity was 0.4 in both eyes. Hertel exophthalmetry was 20 mm in his right eye and 18 mm in his left eye. Painless limitation of motion at supraduction was observed in the right eye. Funduscopy showed newly appeared choroidal folding in the right eye, so orbital computed tomography (CT) with contrast was performed. The CT scans showed bilateral homogenously enhancing retrobulbar masses. Biopsy of the masses showed a MALToma. After radiation therapy, the choroidal folds resolved and exophthalmetry improved to 10 mm in both eyes. No additional IOL dislocation occurred. During 2.5 years of follow-up, there was no evidence of recurrence or distant metastasis of the MALToma. CONCLUSIONS: Orbital lymphomas can cause lid edema, exophthalmos, strabismus, and diplopia, and can be diagnosed with imaging modalities such as CT. Final diagnosis involves biopsy and radiation therapy or chemotherapy. If IOL dislocation occurs repeatedly, it may result from an increase in retrobulbar pressure, and concurrent choroidal folding using funduscopy is strongly recommended for imaging to check for the presence of retrobulbar masses.
Aged, 80 and over ; Anterior Chamber ; Biopsy ; Cataract ; Choroid ; Diagnosis ; Diplopia ; Dislocations* ; Drug Therapy ; Edema ; Exophthalmos ; Follow-Up Studies ; Humans ; Lenses, Intraocular* ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Male ; Neoplasm Metastasis ; Orbit ; Recurrence ; Strabismus ; Stroke ; Tomography, X-Ray Computed ; Visual Acuity

PURPOSE: To analyze the clinical and radiologic findings and to evaluate the treatment and prognosis of epithelial tumor patients. METHODS: We retrospectively reviewed clinical and radiologic findings of 36 patients who had been histopathologically diagnosed with epithelial tumors of the lacrimal gland after biopsy and surgery at Seoul St. Mary's Hospital from May 2005 to October 2012. RESULTS: Among the patients with epithelial tumors of the lacrimal gland based on histopathological findings, there were 21 cases of pleomorphic adenoma, seven cases of dacryops, four cases of adenoid cystic carcinoma, two cases of adenocarcinoma, one case of benign oncocytoma, and one case of mucoepidermoid carcinoma. The characteristic clinical finding of epithelial tumors of the lacrimal gland was proptosis (52.7%). In contrast with benign epithelial tumors of the lacrimal gland, the characteristic clinical findings of malignant epithelial tumors of the lacrimal gland were limitation of motion (57.1%), diplopia (57.1%), ocular pain (42.9%), and decreased visual acuity (42.9%). The mean duration of the symptoms of malignant epithelial tumors (5.0 +/- 4.2 months) was shorter than that of benign epithelial tumors (11.2 +/- 11.1 months) (t-test, p = 0.034). In radiologic CT and MRI findings, there was minimal bony destruction in two cases of pleomorphic adenoma and calcification in one case of pleomorphic adenoma. Malignant epithelial tumors of the lacrimal gland, in contrast to benign tumors, showed characteristic bony destruction (57.1%), poorly marginated tumor outline (42.9%) and calcification (14.3%). The 57.1% of patients in this study with malignant tumors were treated with chemotherapy and radiotherapy after surgical treatment, and there was one case (14.3%) of recurrence after treatment. CONCLUSIONS: Careful analysis of clinical and radiologic findings can lead to early diagnosis of malignant tumors.
Adenocarcinoma ; Adenoma, Oxyphilic ; Adenoma, Pleomorphic ; Biopsy ; Carcinoma ; Carcinoma, Adenoid Cystic ; Carcinoma, Mucoepidermoid ; Diplopia ; Drug Therapy ; Early Diagnosis ; Exophthalmos ; Humans ; Lacrimal Apparatus* ; Magnetic Resonance Imaging ; Prognosis ; Radiotherapy ; Recurrence ; Retrospective Studies ; Seoul ; Visual Acuity

Extramedullary plasmacytoma (EMP) is a rare disease that occurs in 3% to 5% of patients with plasma cell disorder. It occurs most commonly in the upper respiratory tract and the oral cavity. Very few EMP cases have been reported in the central nervous system (CNS). We report herein an unusual case of EMP in the nasal cavity that recurred in the CNS without systemic involvement. A 67-year-old man visited our hospital due to a month-long bout with exophthalmos. He was diagnosed with EMP in the nasal cavity, paranasal sinus, and orbital cavity. He received radiotherapy to which he had complete responses. After 2 years, he visited our hospital because of a month-long headache. He was diagnosed with EMP recurrence in the CNS via brain magnetic resonance imaging and cerebrospinal fluid analysis. He was treated with whole brain radiotherapy and intrathecal chemotherapy with methotrexate, but he expired due to pneumonia.
Aged ; Brain ; Central Nervous System* ; Cerebrospinal Fluid ; Drug Therapy ; Exophthalmos ; Headache ; Humans ; Magnetic Resonance Imaging ; Methotrexate ; Mouth ; Nasal Cavity ; Orbit ; Plasma Cells ; Plasmacytoma* ; Pneumonia ; Radiotherapy ; Rare Diseases ; Recurrence ; Respiratory System

Orbital metastasis from hepatocellular carcinoma is very rare, with only 14 biopsy-proven cases from hepa tocellular carcinoma cases reported in English literature and three cases reported in Korea. Common symptoms of orbital metastasis are proptosis, visual loss, ocular pain and oculomotor dysfunction. For its precise diagnosis, we can perform fine needle aspiration biopsy, orbit CT or MRI, and ultrasonography. Radiotherapy is the mainstay in the treatment of orbital metastasis. In addition, chemotherapy, hormonal therapy and surgical intervention can play a role in the treatment of orbital metastasis according to the primary cancer and symptoms. However, the prognosis of orbital metastasis is poor. We report herein a rare case of a patient with orbital metastasis from hepatocellular carcinoma, which was treated with various modalities that included resection, and who had good clinical and radiological responses to radiation therapy and sorafenib (Nexavar, Bayer HealthCare).
Biopsy ; Biopsy, Fine-Needle ; Carcinoma, Hepatocellular* ; Diagnosis ; Drug Therapy ; Exophthalmos ; Eye Neoplasms ; Humans ; Korea ; Magnetic Resonance Imaging ; Neoplasm Metastasis ; Orbit* ; Prognosis ; Radiotherapy ; Ultrasonography

OBJECTIVETo compare the therapeutic effect and side effect of the treatments on hyperthyroid exophthalmos with the combination of acupuncture and medication and with medication only.

METHODSFifty-two cases were randomly divided into an acupuncture and medication group (27 cases) and a medication group (25 cases). Acupuncture in combination of oral taking of Thiamazole and Euthyrox were adopted for the acupuncture and medication group. And acupoints such as Jingming (BL 1), Chengqi (ST 1) and Sizhukong (TE 23) etc. were selected. Western medication for oral taking was applied as the only treatment for the medication group. Objective eye syndrome marks, side effects and accidents were compared between two groups before and after treatment.

RESULTSThe improvement of the objective marks of eye syndrome in the acupuncture and medication group was better than that in the medication group (P < 0.01). There were 4 cases with hypoleucocytosis, 3 cases with rash and 3 cases with aggravated symptom of exophthalmos in the medication group during the treatment, while no case with side effects was observed in the acupuncture and medication group. However, 8 cases were found with hemorrhage and 8 with hematoma in the acupuncture and medication group.

CONCLUSIONTreatment with the combination of acupuncture and medication may not only enhance the therapeutic effect, but also reduce the side effects.

Acupuncture Points ; Acupuncture Therapy ; adverse effects ; Adult ; Combined Modality Therapy ; Drug-Related Side Effects and Adverse Reactions ; Exophthalmos ; Female ; Graves Ophthalmopathy ; drug therapy ; therapy ; Humans ; Male ; Methimazole ; adverse effects ; therapeutic use ; Middle Aged ; Thyroxine ; adverse effects ; therapeutic use ; Treatment Outcome ; Young Adult

Kimura's disease (KD) is a rare, chronic inflammatory disorder, which is characterized by tumor-like masses mainly located in the head and neck region. Extraocular muscle involvement in KD is uncommon. We report a case of KD that involved both the extraocular muscles and buccal area. A 13-year-old male presented to our clinic with a two-year history of exophthalmos of the left eye and facial swelling. Facial CT and MRI showed a 1.5 x 1.5 cm2 soft tissue mass located at the left masticator and buccal area, exophthalmos of the left eye, and diffuse thickening of the left extraocular muscles. We performed a lateral rectus muscle incisional biopsy of the left eye. Oral methylprednisolone therapy was initiated and tapered following the incisional biopsy.
Administration, Oral ; Adolescent ; Angiolymphoid Hyperplasia with Eosinophilia/complications/*diagnosis/drug therapy/surgery ; Cheek/*pathology ; Edema/etiology ; Exophthalmos/etiology ; Face ; Glucocorticoids/administration & dosage ; Humans ; Magnetic Resonance Imaging ; Male ; Methylprednisolone/administration & dosage ; Oculomotor Muscles/*pathology/surgery ; Postoperative Care ; Treatment Outcome

PURPOSE: We report a case of ectopic adenoid cystic carcinoma developed on the nasal orbital side adjacent to the nasal cavity. METHODS: A 60-year-old man developed progressive right ocular proptosis and pain for 3 years. The patient had undergone regular medical examination in a hospital but eventually visited our hospital due to aggravated symptoms. Orbit MRI and CT showed a 24 x 36 x 17 mm-sized lower signal mass on the medial side of the right medial rectus muscle and also showed erosion of the orbital bone in the superior and medial orbital walls. An endoscopic biopsy was done after transferring him to the ENT department. A frozen section of the tissue in the operating room showed no malignant evidence, and the mass was excised by endoscopic surgery. The permanent pathological result was adenoid cystic carcinoma. The authors recommended orbital exenteration, but the patient refused the procedure and was seen in follow-up visits after receiving chemotherapy and radiologic therapy. Some orbital mass remained in the posterior orbital area immediately directly after endoscopic excision. The patient underwent his first session of Cyberknife radiosurgery therapy after concurrent chemotherapy and radiologic therapy. CONCLUSIONS: Ectopic adenoid cystic carcinoma should be considered as a differential diagnosis of nasal orbital mass lesions.
Adenoids* ; Biopsy ; Carcinoma, Adenoid Cystic* ; Diagnosis, Differential ; Drug Therapy ; Exophthalmos ; Follow-Up Studies ; Frozen Sections ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Nasal Cavity ; Operating Rooms ; Orbit* ; Radiosurgery

PURPOSE: To report a case of primary yolk sac tumor of the orbit. METHODS: A 15-month-old girl was referred for evaluation of ptosis and strabismus in the right eye which had developed over the previous two weeks. On examination, ptosis, exotropia, proptosis, and restriction of extraocular muscle movement of the right eye were noted. A computed tomography (CT) scan of the orbit showed a 2.4 cm x 3.4 cm-sized soft tissue mass in the posterior aspect of the right orbit which enveloped the optic nerve and invaded the maxillary sinus, posterior ethmoid sinus, nasal cavity, and intracranial space. Endoscopic biopsy of the nasal cavity mass was performed. RESULTS: Histologic evaluation revealed solid proliferation of germ cells with large, clear cytoplasm and intracellular, extracellular hyaline globules. Immunohistochemical staining for alpha-fetoprotein was positive. Results of further systemic evaluation were all negative. The tumor regressed after combined chemotherapy and radiotherapy. The patient has been disease free for 2 years. CONCLUSIONS: The possibility of yolk sac tumor should be considered in the differential diagnosis in a younger patient for an orbital mass with rapidly increasing size and adjacent bony destruction. Prompt diagnosis and appropriate therapeutic approaches are essential.
alpha-Fetoproteins ; Biopsy ; Cytoplasm ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Endodermal Sinus Tumor* ; Ethmoid Sinus ; Exophthalmos ; Exotropia ; Female ; Germ Cells ; Humans ; Hyalin ; Infant ; Maxillary Sinus ; Nasal Cavity ; Optic Nerve ; Orbit* ; Radiotherapy ; Strabismus ; Yolk Sac*

PURPOSE: To report a case with complete ophthalmoplegia after herpes zoster ophthalmicus. METHODS: A 70-year-old male patient visited a clinic because of vesicular eruptions over the left side of his face with severe pain. Drooping and severe swelling of the left eyelid were present, along with keratitis and uveitis. While the lid swelling and uveitis were improving, external ophthalmoplegia and exophthalmos were discovered. Intramuscular injections of dexamethasone 5 mg were given for 10 days, followed by oral administration of prednisolone at a dosage of 15 mg for two weeks and 10 mg for two weeks. RESULTS: The patient was fully recovered from the complete ophthalmoplegia and exophthalmos six months after the onset of the cutaneous lesion. CONCLUSIONS: Complete ophthalmoplegia is a rare ophthalmic complication of herpes zoster infection. Therefore, an evaluation of extraocular muscle and lid function should be performed during the examination of herpes zoster patients in order to screen for ophthalmoplegia.
Severity of Illness Index ; Prednisolone/therapeutic use ; Ophthalmoplegia/drug therapy/*etiology ; Male ; Humans ; Herpes Zoster Ophthalmicus/*complications/drug therapy ; Glucocorticoids/therapeutic use ; Follow-Up Studies ; Exophthalmos/drug therapy/etiology ; Aged

PURPOSE: Granulocytic sarcoma (GS), an extramedullary tumor consisting of primitive myeloid cells, is a rare manifestation of acute myelogenous leukemia (AML). However, GS can occasionally precede the development of systemic leukemia by weeks to years. The objectives of this study are to describe the frequency, clinical characteristics and survival of AML children with GS from a single Korean institute. METHODS: Retrospective review of all the AML children who presented between January, 1995 and June, 2003 was undertaken. RESULTS: GS developed in 9 children among 118 AML patients (incidence, 7.6%). The median age at diagnosis of AML was 82 months (8 months~13 years) with equal sexual distribution. The sites of GS were scalp (n=4), skull (n=3), paranasal sinuses (n=1), external auditory canal (n=1), spinal epidura (n=1), and spinal intramedulla (n=1). The symptoms related with GS were scalp mass (n=4), paraparesis (n=3), facial nerve palsy (n=3), hearing impairment (n=2), and exophthalmos (n=1). In the case with spinal epidural mass, GS preceded the diagnosis of AML by 15 months. Cytogenetics were available in 8 cases, and t (8; 21) was found in five cases. All cases received systemic chemotherapy, with surgical decompression and radiotherapy for 2 patients involving spine. Seven cases received stem cell transplantations (3, allogeneic bone marrow; 4, autologous peripheral blood). The 5-yr event-free survival was 35.0% by Kaplan-Meier method. All 3 allografted patients are alive (86 mo, 5 mo, 1 mo), while 3 of 4 autografted patients had either died or relapsed. CONCLUSION: GS should be considered in patients with or even without AML who have palpable mass or neurological manifestation. Effective treatment, including allogeneic stem cell transplantation, should be considered to achieve a durable disease control.
Allografts ; Autografts ; Bone Marrow ; Child ; Cytogenetics ; Decompression, Surgical ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Ear Canal ; Exophthalmos ; Facial Nerve ; Hearing Loss ; Humans ; Leukemia ; Leukemia, Myeloid, Acute* ; Myeloid Cells ; Neurologic Manifestations ; Paralysis ; Paranasal Sinuses ; Paraparesis ; Radiotherapy ; Retrospective Studies ; Sarcoma, Myeloid* ; Scalp ; Skull ; Spine ; Stem Cell Transplantation