Primary orbital Ewing sarcoma in a middle-aged man

A 54-year-old man presented with a painful left proptosis and a soft tissue mass at the inferolateral aspect of the left orbit with bone involvement. There was no clinical or investigational evidence of systemic disease. Both light microscopy and immunohistochemistry were required for the diagnosis of Ewing sarcoma. After failure of chemotherapy alone, management included extensive surgical excision and postoperative adjuvant radiotherapy and chemotherapy. Despite intervention, the patient died of widespread metastatic disease 17 months after initial presentation. Although rare, Ewing sarcoma should be included in the differential diagnosis of uniform round cell orbital tumors in adults.     

Primary Ewing sarcoma of the orbit

PURPOSE: To report the clinicopathologic features of a 17-year-old patient with primary Ewing sarcoma of the orbit. METHODS: The patient was evaluated clinically before surgery with computed tomography scans of the orbit. After surgery, the patient was staged with computed tomography scans and bone scan and was treated with systemic chemotherapy and radiation therapy to the orbit. The orbital biopsy was evaluated with conventional light microscopy and immunohistochemistry. RESULTS: Clinical evaluation revealed proptosis and limited upgaze. Computed tomography scans disclosed a mass involving the superior orbit, anterior cranial fossa, and temporal fossa. Microscopic examination revealed small, poorly differentiated cells with medium-sized nuclei containing finely granular chromatin and small nucleoli. The cytoplasmic borders of the cells were indistinct. A PAS stain revealed modest glycogen in many of the tumor cells. The tumor stained positive for O-13 and vimentin and was negative for neural, skeletal, and lymphoid cell markers. Computed tomography scan, bone scan, and blood chemistries revealed no other site of involvement. After treatment, the clinical symptoms and signs resolved, and there has been no evidence of residual orbital tumor or metastasis. CONCLUSIONS: Primary Ewing sarcoma of the orbit should be considered in the differential diagnosis of children or young adults with proptosis, diplopia, or periorbital swelling. Immunohistochemistry is essential to distinguish Ewing sarcoma from other small round cell tumors.     

Primary Ewing sarcoma of the orbit in an adult

A 42-year-old man presented with visual field loss in the right eye and unintentional weight loss associated with a right orbital mass. Surgical resection and biopsy revealed a diagnosis of Ewing sarcoma, and systemic evaluation showed no metastasis. Subsequent chemotherapy and radiation resulted in tumor resolution without recurrence for 3.5 years. Although systemic symptoms are associated with a poorer prognosis, longer survival may be possible in the absence of metastasis.     

Primary orbital calcified synovial sarcoma: a case report

A 32-year-old woman presented with a progressively increasing recurrent swelling of the left eye. An orbital tumor was detected and histologically classified as a synovial sarcoma, confirmed by immunohistochemistry and electron microscopy. Isolated cases of synovial sarcoma have been reported in the retroperitoneum, mediastinum, pharynx and orofacial region. However, the orbit is an extremely rare site. The authors report a case of recurrent primary orbital calcified synovial sarcoma in a young lady treated with surgery and radiotherapy.     

Primary orbital calcified synovial sarcoma: A case report

A 32-year-old woman presented with a progressively increasing recurrent swelling of the left eye. An orbital tumor was detected and histologically classified as a synovial sarcoma, confirmed by immunohistochemistry and electron microscopy. Isolated cases of synovial sarcoma have been reported in the retroperitoneum, mediastinum, pharynx and orofacial region. However, the orbit is an extremely rare site. The authors report a case of recurrent primary orbital calcified synovial sarcoma in a young lady treated with surgery and radiotherapy.     

Primary orbital neuroblastoma in a neonate

Neuroblastoma is an undifferentiated malignant tumor of the primitive neuroblasts. Orbital neuroblastoma is typically a metastatic tumor. We describe a two-days-old girl, who presented with a large tumor in her left orbit. Magnetic resonance imaging revealed that the tumor originated from the retrobulbar area, extending into the upper and lateral orbit. She was operated on the fifth day of life. A histopathologic diagnosis of neuroblastoma was made. Medical evaluation including chest roentgenogram, ultrasonography of the abdomen, whole body computerized tomogram and bone scintigraphy showed no evidence of systemic involvement or metastasis. Neuroblastoma should be considered in the differential diagnosis of neonatal orbital tumors.     

Primary orbital leiomyosarcoma

An 84-year-old woman presented with a right upper eyelid nodule, first noted 1 month earlier. Incisional biopsy revealed leiomyosarcoma, which was subsequently treated with wide excision and adjunctive radiotherapy. There has been no evidence of tumor recurrence in 3 years of follow-up.     

Primary orbital lymphoma

BACKGROUND/AIMS. Localized orbital non-Hodgkin's lymphoma is a rare form of extranodal lymphoma. The aim of this study was to evaluate the outcome of 48 patients presenting with stage I-E non-Hodgkin's lymphoma (NHL). METHODS. Out of 118 charts of patients with lymphoproliferative lesions, those of 48 patients with stage I-E orbital lymphoma seen over a 22-year period from 1977 through 1999 were reviewed. RESULTS. Twenty-five patients were male and 23 were female. Their mean age was 58.7 (12-85) years. The mass was localized inside the orbit in 37 cases and in the lacrimal gland region in 11 cases; bilateral involvement was observed in two cases. The duration of the symptoms at the time of presentation ranged from 10 days to 10 years with a mean duration of 18 months. The 24 cases with low-grade lymphoma are all alive without disease, whatever therapeutic approach was performed (biopsy, radiotherapy, chemotherapy). In intermediate and high-grade NHL the rate of recurrence after chemotherapy has been 50% and 33%, respectively, compared to 75% and 50% after biopsy alone and 75% after radiotherapy (not administered in high-grade forms). CONCLUSION. The therapeutic approach to localized orbital non-Hodgkin's lymphoma must take the histologic grading into consideration. The 24 cases with low-grade lymphoma had a good prognosis. The 17 cases with the intermediate-grade form and the 7 cases with the high-grade form had a better prognosis when treated with chemotherapy.     

Primary orbital angiosarcoma: a case report

PURPOSE: The pathogenesis, natural history, histopathology, and recommended treatment for orbital angiosarcoma are illustrated and reviewed. METHODS: Case report. RESULTS: A 71-year-old white male presented with bluish discoloration and swelling of the left medial canthal area. A fine needle aspiration and excisional biopsy with histopathologic examination was performed, which showed angiosarcoma. Pattern of growth was demonstrated radiographically and histopathologically, confirming primary orbital angiosarcoma. Subsequent wide surgical resection was carried out, with substantial reconstruction of the left orbital and periorbital area. The patient responded well to the surgery, and was free of tumor after six years of follow-up. CONCLUSION: Angiosarcoma is a rare and highly malignant tumor of epithelial origin. The aggressive nature of this tumor usually results in a high mortality rate despite treatment. However, early diagnosis and wide surgical excision has resulted in successful treatment of these tumors.     

Osteogenic sarcoma after orbital radiation rhabdomyosarcoma

PURPOSE: We describe the occurrence of maxillary and orbital osteogenic sarcoma in a child after treatment of contralateral orbital rhabdomyosarcoma with external beam radiation and chemotherapy. DESIGN: Interventional case report. INTERVENTION: Treatment of a maxillary and orbital rhabdomyosarcoma with surgical resection, systemic chemotherapy, and local radiation. MAIN OUTCOME MEASURES: Occurrence and histology of secondary malignancy after orbital radiation. RESULTS: An eleven year-old male presented for evaluation of left facial swelling, occurring ten years after successful treatment of a right orbital embryonal rhabdomyosarcoma with chemotherapy and 5040 cGY of external beam radiation. Computerized tomography demonstrated a mass involving the left maxillary sinus and orbital floor. Biopsy demonstrated osteogenic sarcoma. Despite attempted total excision with radical maxillectomy, resection margins were found to have microscopic extension of the tumor. Postoperatively he was treated with systemic chemotherapy and local radiation. Eight months postoperatively he remains alive despite local progression. CONCLUSIONS: Osteogenic sarcoma can occur as a secondary malignancy years after the successful treatment of orbital rhabdomyosarcoma with external beam radiation and chemotherapy. After orbital radiation, subjects should undergo routine lifelong examinations.     

Primary orbital ganglioneuroma in a 2-year-old healthy boy

A 2-year-old healthy child presented with progressive unilateral proptosis.Complete work up including: general examination, detailed ophthalmic evaluation and radiological imaging were done. He underwent orbital exploration via anterior orbitotomy incision and debulking of the tumor was done.The histopathological examination confirmed the diagnosis of orbital ganglioneuroma.Ganglioneuroma is an unusual benign tumor of neuroplastic origin with extremely rare orbital involvement with only one prior reported case in a youth. The tumor is slow growing and non-metastasizing. Biopsy is necessary to differentiate it from the malignant neuroblastoma and excision is usually curative.