Epithelial-Myoepithelial carcinoma of the lacrimal gland - Case report and review of literature

Objective@#To describe the clinicopathologic features and outcome of a patient with epithelial-myoepithelial carcinoma of the lacrimal gland who underwent modified lateral orbitotomy with en toto removal of the lesion and adjuvant radiotherapy.@*Methods@#This is a case report.@*Results@#A 31-year-old Filipino male seafarer presented with a 2-month history of an enlarging left superotemporal orbital mass and inferonasal displacement of the globe. Magnetic resonance imaging revealed a superotemporal extraconal mass within the lacrimal sac fossa with evidence of osseous infiltration of the superolateral orbital rim. Modified lateral orbitotomy was performed with en toto removal of the lesion and the clinically infiltrated adjacent lateral bony margin. Histopathologic diagnosis of epithelial-myoepithelial carcinoma of the lacrimal gland was made based on the classic and distinct biphasic morphology and was confirmed with immunohistochemistry studies (cytokeratin-7, S-100, and p63). Systemic surveillance using positron emission tomography and computed tomography scan with contrast revealed no evidence of regional or distant metastasis. Adjuvant radiotherapy of the orbital area was performed for increased local control. Twelve months postoperatively, the patient showed no evidence of tumor recurrence.@*Conclusion@#Epithelial-myoepithelial carcinoma of the lacrimal gland is a rare condition, and this is the first documented case from the Philippines. Accurate diagnosis is necessary for appropriate treatment. It should be included in the differential diagnosis of infiltrative lesions in the lacrimal gland fossa.

Primary chondromyxoid fibroma of the orbit: An orbital mass with calcification

Primary orbital chondromyxoid fibroma is a rarely reported entity. A 34-year-old lady presented with painless, non-axial proptosis of the left eye of 6 months duration. Orbital imaging showed a supero-temporal mass with calcific foci and bone erosion. The mass caused globe compression resulting in choroidal folds. Anterior orbitotomy with complete mass excision was performed. The histopathology revealed a chondromyxoid fibroma. At 12-months follow-up, the patient is doing fine with no clinical recurrence. Chondromyxoid fibroma is an important differential diagnosis for bony orbital tumors.

Características clínicas y patológicas de los tumores orbitarios / Clinical and pathological characteristics of orbital tumors

Objetivo: describir las características clínico-patológicas de los tumores orbitarios. Métodos: se realizó un estudio descriptivo y retrospectivo de serie de casos en el Instituto de Oncología y Radiobiología desde enero del año 2011 a diciembre de 2017. La muestra se conformó con 241 pacientes sometidos a cirugía de la órbita y fue caracterizada según la edad, el sexo, el origen y la naturaleza de la lesión, el diagnóstico histopatológico y los abordajes quirúrgicos utilizados. Resultados: el 52,7 por ciento de la muestra estudiada fue del sexo masculino; el 36,5 por ciento se encontró en el grupo de 60 a 79 años. El 62,7 por ciento de los casos correspondió a tumores malignos, el 51,9 por ciento a lesiones primarias de la órbita y el 26,6 por ciento a linfoma No Hodgkin. Las lesiones secundarias originadas en los párpados representaron el 58,7 por ciento y el carcinoma epidermoide el 56,5 por ciento. Hubo 11 casos correspondientes a metástasis; las de mama representaron el 72,7 por ciento. El abordaje anterior transpalpebral fue utilizado en el 54,8 por ciento de los casos. Conclusiones: los tumores orbitarios se presentan con mayor frecuencia a partir de los 40 años de edad y con predominio del sexo masculino. Las lesiones malignas son más frecuentes, así como las primarias de la órbita, donde el linfoma No Hodgkin representa el mayor número de casos. Los párpados son la principal estructura de origen de las lesiones secundarias, y el carcinoma epidermoide es la variedad histopatológica predominante. Las metástasis de mama son las que más afectan el espacio orbitario. La vía de abordaje fundamental es la anterior transpalpebral (transeptal), seguido de la exenteración orbitaria(AU)

Objective: describe the clinicopathological characteristics of orbital tumors. Methods: a retrospective descriptive case-series study was conducted at the National Institute of Oncology and Radiobiology from January 2011 to December 2017. A sample of 241 patients undergoing orbital surgery was characterized according to age, sex, origin and type of lesion, histopathological diagnosis and surgical procedures used. Results: 52.7 percent of the study sample was male and 36.5 percent was in the 60-79 age group. 62.7 percent of the cases were malignant tumors, 51.9 percent were primary orbital lesions, and 26.6 percent were non-Hodgkin lymphomas. Secondary lesions originating in the eyelids represented 58.7 percent, and epidermoid carcinomas 56.5 percent. Eleven cases were metastases, 72.7 percent were of the breast type. The anterior transpalpebral approach was used in 54.8 percent of the cases. Conclusions: orbital tumors are more common as of age 40 with a predominance of the male sex. Malignant lesions are more frequent, as well as primary orbital lesions, of which the largest number of cases are non-Hodgkin lymphomas. The eyelids are the main structure of origin of secondary lesions, and epidermoid carcinoma is the prevailing histopathological variety. Breast metastases are the type most commonly affecting the orbital area. The main approach is anterior transpalpebral (transeptal), followed by orbital exenteration(AU)

Orbital Chondroma: A rare mesenchymal tumor of orbit.

While relatively common in the skeletal system, cartilaginous tumors are rarely seen originating from the orbit. Here, we report a rare case of an orbital chondroma. A 27‑year‑old male patient presented with a painless hard mass in the superonasal quadrant (SNQ) of left orbit since 3 months. On examination, best‑corrected visual acuity of both eyes was 20/20, with normal anterior and posterior segment with full movements of eyeballs and normal intraocular pressure. Computerized tomography scan revealed well defined soft tissue density lesion in SNQ of left orbit. Patient was operated for anteromedial orbitotomy under general anesthesia. Mass was excised intact and sent for histopathological examination (HPE). HPE report showed lobular aggregates of benign cartilaginous cells with mild atypia suggesting of benign cartilaginous tumor ‑ chondroma. Very few cases of orbital chondroma have been reported in literature so far.

Orbital malignant melanoma associated with nevus of Ota.

Nevus of Ota (oculodermal melanosis) is a dermal melanocytic hamartoma with bluish hyperpigmentation along the first and second branches of the trigeminal nerve. Extracutaneous involvement, especially ocular, has been reported. A 45-year-old male presented with malignant melanoma of the left orbit in association with nevus of Ota. Being locally invasive, a modified exenteration with frontal flap repair was done on left eye. Adjuvant chemotherapy was given after wound healing. All pigmented lesions of the eye require close monitoring to help in the early diagnosis. Since malignant transformation has been reported in oculodermal melanosis, close follow-up and patient education will facilitate early diagnosis and prompt management. This case is reported for its rarity and unusual presentation.

Lymphatic Malformations of the Orbit

OBJECTIVES: Lymphatic malformations of the orbit are rare lesions that constitute approximately 1% to 8% of all orbital masses. They are difficult to treat since they do not remain within anatomic boundaries and tend to penetrate into normal orbital structures. The aim was to analyze clinical courses and therapy options in patients with lymphatic malformations of the orbit. METHODS: Thirteen patients with orbital lymphatic malformations confirmed by magnetic resonance imaging between 1998 and 2009 were enrolled in this study. Patients' charts were retrospectively reviewed to analyze clinical courses and treatment options. RESULTS: Four patients suffered from isolated intraorbital lymphatic malformations without conjunctival involvement, in three of them the masses were completely resected, in one patient close controls were performed. Three patients had isolated intraorbital lymphatic malformations with conjunctival involvement. Surgical volume reduction of the exterior parts of the lymphatic malformation were performed without any complications and satisfying outcome in these cases. Six patients suffered from intra- and periorbital lymphatic malformations. In 3 patients a watch-and-wait strategy was initiated. In the other 3 patients a surgical therapy was performed, one patient additionally received sclerotherapy with OK-432; however, these 3 patients suffered from residual lymphatic malformations. CONCLUSION: The presented cases underline the inconsistencies in the malformations behavior and underscore the inability to make specific recommendations regarding treatment. The treatment decision should be based on the size and location of the lymphatic malformation. The untreated patient must be watched for signs of visual detoriation, which may signal the need for therapeutic intervention.

Transconjunctional approach for orbital cavernous hemangiomas: A report of 39 cases / 第二军医大学学报

Objective: To evaluate the efficacy of transconjunctival approach for patients with orbital cavernous hemangiomas, and to understand its relationship with imaging localization. Methods: We retrospectively studied the clinical data of 39 patients with orbital cavernous hemangioma who were treated with transconjunctival orbitotomy. The clinical and radiologic characteristics of orbital cavernous hemangiomas, the surgical outcomes, and the selection criteria were analyzed. Results: We noticed that all the cavernous hemangiomas in this study were just behind the globe or compressing the globe. CT and MRI showed that all the 39 hemangiomas were located in the muscle cone, round in shape and with well-defined margins. The hemangiomas of 36 patients, which showed slight adhesion and no large draining vein by image examination, were completely resected. One patient with large draining vein was also extirpated completely, but only with visual loss. In the other 2 patients with tight adhesion, most fragments of tumors were removed. The successful rate of operation was 92.3%. The visual acuity increased in 6 of 9 cases with primary visual impairment. All the 32 patients with primary proptosis had their symptoms resolved completely. The treatment outcomes were obvious in 32 (82.1%) patients, remained unchanged in 5 (12.8%) patients, and worsened in 2 (5.1%) patients. Conclusion: Retrobulbar intraconal cavernous hemangiomas can be removed successfully through a transconjunctival approach. However, other approaches must be recommended if the tumor is closely attached to the orbital apex, adheres tightly to the retrobulbar tissues, or with large draining veins.

Surgical Outcomes of Transconjunctival Anterior Orbitotomy for Intraconal Orbital Cavernous Hemangioma

PURPOSE: To describe surgical outcomes for transconjunctival anterior orbitotomy for intraconal cavernous hemangiomas. METHODS: The medical records of 9 consecutive patients with intraconal cavernous hemangiomas who underwent surgical removal by transconjunctival anterior orbitotomy were retrospectively reviewed. The conjunctiva was incised and retracted with a traction suture. For large tumors, a rectus muscle was temporarily disinserted. Tenon's capsule was separated and the tumor was removed with a cryoprobe or clamp. Surgical outcomes, positions of the tumors, methods of approach, and intra- and post-operative complications were evaluated. RESULTS: The mean follow-up period was 33 +/- 6.8 months. No bony orbitotomy was used in this technique and the cosmetic results were very satisfactory. All tumors were removed intact. In 4 patients, tumors were extirpated with the aid of a cryoprobe. No patients had residual proptosis or limitation of ocular movement. No signs of recurrence were noted in any cases at 33 months follow-up. No serious or permanent complications were observed during or after the operation. CONCLUSIONS: Transconjunctival anterior orbitotomy is an important surgical procedure in the treatment of intraconal cavernous hemangiomas. It can produce an excellent result, even if the posterior border of the tumor abuts the orbital apex.

Intraorbital Cysticercosis Treatment with Lateral Orbitotomy

A 33-year-old female from Uzbekistan visited our hospital with symptoms of right blurred vision, ocular pain which were exacerbated by ocular movement, and exophthalmosis for 2 months. Preoperative facial CT scan showed 3.2x2.4cm-sized cystic mass at the right retrobulbar area. The mass was located at temporal aspect of retrobulbar area and displaced optic nerve medially. Right eyeball was anteriorly displaced at the degree of 7mm than left one and intra-ocular pressure was raised as 32 mmHg compared with left one which estimated at 15 mmHg. Removal of cytic mass was performed using lateral orbitotomy. After incision was made through Stellard- Wright incision, dissection was done to lateral outer orbital periosteum then the periosteum each side of lateral orbit were dissected for lateral orbitotomy. Removal of lateral orbital wall, which was enough to removal cystic mass, by lateral orbitotomy, was done then lateral rectus muscle was divided without cutting. Cystic mass could be resected after lateral rectus muscle was divided and resected lateral orbital bone piece was re-located and fixed by absorbable miniplate. A satisfactory result could be obtained by this procedure. Postoperative intra-ocular pressure was lowerd as 15mm Hg and exophthalmosis was corrected at the degree of 1mm on Hertel's exophthalmometery and visual acuity improved at the degree of 1.0 on optomety compared with preoperative one which estimated 0.04. Preoperative symptoms such as ocular pain, foreign body sensation, headache were disappeared without any complication just like retrobulbar hemorrhage, infection etc.

Removal of Deep Orbital Tumor through Small Incision Approach

PURPOSE: We report on the effectiveness of the small incision anterior approach for the deep orbital tumor. METHODS: The authors operated on 5 patients with deep orbital tumor using the small incision anterior approach. RESULTS: Five cases , 2 neurilemmomas, 1 orbital dermoid cyst, 1 angiofibroma, and 1 osteoma, were treated surgically with the small incision anterior approach and had good cosmetic result, early recovery, and showed no serious complications. CONCLUSIONS: The small incision anterior approach for the deep orbital tumor has a number of advantages over lateral orbitotomy, including shorter operation time, early recovery and good cosmetic results. Nevertheless, this technique requires a precise preoperative diagnosis.

A case of Retrobulbar Intramuscular Hematoma

PURPOSE: To report a case of retrobulbar hematoma within the lateral rectus muscle which resulted in sight-threatening complication. METHODS: A 40-year-old male presented with a chief complaint of painless mass and exophthalmos. Exicional biopsy via lateral orbitotomy and direct decompression of the muscle were performed to the diagnosis and treatment. RESULTS: After decompression, improvement of the exophthalmos was achieved. This case illustrated a potentially serious complication of retrobulbar hematoma following minor periorbital trauma. The successful outcome may be achieved by rapid diagnosis and intervention.

Primary Angiofibroma Developed in the Orbit

PURPOSE: Angiofibroma usually affects the nasopharynx, but primarily developed angiofibroma in the orbit has not yet been reported in Korea. Thereby we report a case of angiofibroma in the orbit. METHODS: A-5-year-old girl, who had been followed up for intraorbital mass which was incidentally detected, was operated with superior orbitotomy approach due to progressive proptosis and dislocated eyeball. RESULTS: The patient was diagnosed angiofibroma in the orbit with light microscopy and immunochemistry. The symptom was improved and she wore the artificial eye due to corneal opacity 6 months after the operation. CONCLUSIONS: We diagnosed and managed the primary angiofibroma in the orbit of a young girl. In diagnosing the orbital tumor, the possibility of angiofibroma should be considered.

Case Report of Retained Intraorbital Metallic Foreign Body Removal

Retained intraorbital foreign body after penetrating orbital injury may affect adjacent tissue and lead to many ophthalmological sequeles such as visual disturbance, EOM limitation, diplopia, strabismus, orbital cellulitis and orbital abscess. If intraorbital foreign body intrudes into adjacent CNS system, it may lead to fatal complications such as meningitis and brain abscess. We should diagnose and treat intraorbital foreign body early and accurately to prevent complications, and rehabilitate damaged tissue anatomically and functionally. A patient with retained intraorbital foreign body after penetrating lower lid injury was transferred to our hospital for lid swelling and periocular pain. We diagnosed and confirmed intraorbital foreign body with use of plain X ray and orbital CT, and removed it with lateral orbitotomy surgical approach.

Case Report of Retained Intraorbital Metallic Foreign Body Removal

Retained intraorbital foreign body after penetrating orbital injury may affect adjacent tissue and lead to many ophthalmological sequeles such as visual disturbance, EOM limitation, diplopia, strabismus, orbital cellulitis and orbital abscess. If intraorbital foreign body intrudes into adjacent CNS system, it may lead to fatal complications such as meningitis and brain abscess. We should diagnose and treat intraorbital foreign body early and accurately to prevent complications, and rehabilitate damaged tissue anatomically and functionally. A patient with retained intraorbital foreign body after penetrating lower lid injury was transferred to our hospital for lid swelling and periocular pain. We diagnosed and confirmed intraorbital foreign body with use of plain X ray and orbital CT, and removed it with lateral orbitotomy surgical approach.