Primary extramedullary plasmacytoma of the orbit

Background: We report a case of extramedullary plasmacytoma of the orbit. This is a rare tumour which has uncommonly presented in the orbit.
Case report: A 58-year-old woman presented with painless proptosis, upper lid swelling and ptosis. Incisional biopsy was performed. Clinical findings and investigations were correlated to establish the diagnosis. Treatment consisted of 35 Gy external beam radiotherapy.
Conclusions: This condition is considered to be of low grade malignancy and as such can be regarded as a separate entity to multiple myeloma involving the orbit or solitary myeloma of bone in terms of treatment and prognosis.     

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 bone tumors of the orbit

Primary tumors of orbital bone constitute 0.6% to 2% of all orbital tumors. Our experience over a 24-year period in the Orbital Clinic at the University of British Columbia yielded 62 (1.9%) cases from a total of 3,340 orbital tumors. Although a heterogeneous group, primary orbital bone tumors may be classified on a clinicopathologic basis into benign fibro-osseous or cartilaginous, reactive, neoplastic and vascular disorders. Presentation is usually a gradual mass effect, with infiltration and acute hemorrhage being features of malignant and reactive lesions respectively. The two most commonly encountered entities were fibrous dysplasia and osteoma, accounting for 22 cases. Although both these conditions rarely present a diagnostic challenge, nonspecific histologic and radiologic appearances can result in poor characterization of several of the rarer lesions. Hence, close cooperation between clinician, radiologist and pathologist is essential for accurate diagnosis.     

Primary dermatofibrosarcoma protuberans invading the orbit

A 38-year-old man presented with a slow-growing, firm cutaneous mass beneath his left eyebrow. Histopathology and immunohistochemistry confirmed the diagnosis of dermatofibrosarcoma protuberans. The mass infiltrated the medial canthal tendon and anterior orbital fat and could not be completely excised with Mohs micrographic surgery. The patient underwent exenteration and dacryocystectomy with margin-controlled excision and remained free of disease 9 months after surgery. To our knowledge, no prior case of primary dermatofibrosarcoma protuberans involving the orbit has been reported.     

Primary localised amyloidosis of the orbit.

AIMS/BACKGROUND: Primary localised amyloidosis is rarely encountered in the orbit. The typical clinical and radiological appearances have not been clearly established, in particular the single site deposition of amyloid has never been proved. METHODS: Six cases were reviewed in detail and their clinical and radiological characteristics are described here. Four patients had scintigraphy with 123I serum amyloid P component and one patient had typing of the amyloid fibrils. RESULTS: All the patients had a firm mass in the upper orbit with a predilection for the region of the lacrimal gland. Computed tomography showed a homogeneous mass with thickening and irregularity of the adjacent bone and/or calcification within the mass. None was associated with systemic disease. Scintigraphy with 123I serum amyloid P component demonstrated that the amyloid was confined to the orbit. In one patient the amyloid fibrils were derived from an IgG4 heavy chain constant domain. The lesions were partially excised with subsequent clinical stability over 6 months to 18 years in all but one patient who had continued enlargement of the lesion. CONCLUSION: There is a characteristic clinical and radiological pattern for primary localised amyloidosis of the orbit. The disease process is truly local and not part of a systemic process. A majority respond to simple debulking with subsequent observation.     

Primary primitive neuroectodermal tumor of the orbit

Primitive neuroectodermal tumor (PNET) is a small round cell malignant tumor of neuroectodermal origin. Most of the PNETs occur in the central nervous system (CNS). PNETs recognized outside of CNS are diagnosed as peripheral PNET (pPNET). This tumor which expresses MIC-2 gene (CD99) seems to be least aggressive after complete tumor resection. We describe a rare case of PNET in a young girl.     

Primary conjunctival epithelial cyst of the orbit

Background A primary conjunctival epithelial cyst is an uncommon lesion that consists of nonkeratized, stratified squamous epithelium without dermal appendages. Methods Case report. Results A 57-year-old woman presented with a soft mass in the anterior portion of the nasal orbit. Magnetic resonance imaging demonstrated a rounded, cystic lesion between the right eye and the nasal bone. Histopathological examination following complete surgical excision disclosed a conjunctival epithelial cyst lined by nonkeratinized cuboidal epithelium without goblet cells and adnexal structures. Conclusion A conjunctival epithelial cyst should be considered in the differential diagnosis of a mass in the anterior portion of nasal or superonasal orbit.     

Primary myxoid liposarcoma of the orbit.

Orbital liposarcoma is a rare and usually unsuspected neoplasm. Over a five-year period three female patients aged 22, 71, and 77 years presented with primary myxoid liposarcoma of the orbit. The management of one patient was complicated by a history of orbital decompression for suspected thyroid eye disease. The tumour infiltrates locally beyond a deceptive pseudocapsule, and surgery has to be radical to be effective.     

Primary malignant ectomesenchymoma of the orbit.

Malignant ectomesenchymoma (MEM) is a rare soft tissue tumour believed to arise from a pluripotential migratory neural crest cell and composed of both a mesenchymal element (most often rhabdomyosarcoma) and a neuroectodermal element (often neuroblastoma). Reported sites of origin are the abdomen, perineum or scrotum, the extremities, the middle ear, nasopharynx, face, and neck. We report the first case of an orbital MEM, with a review of the 17 cases previously reported from other sites.     

Primary intraosseous cavernous hemangioma of the orbit

PURPOSE: Primary orbital intraosseous hemangioma is a rare, benign neoplasm presenting most frequently in patients in their fourth or fifth decade of life. We describe an elderly patient affected by this tumor. METHODS: Case report. RESULTS: A 75-year-old man presented with a slowly growing, bony mass in the left orbital rim inferolaterally. He had a history of nephrectomy because of a renal carcinoma. Computed tomography showed a bony lesion with internal radiating trabeculations. A biopsy was performed. Histopathologically, the tumor was an intraosseous cavernous hemangioma. CONCLUSION: Primary intraosseous cavernous hemangioma of the orbit may infrequently affect elderly patients. One indication for surgical removal of these tumors in the absence of visual disturbances is to rule out metastatic disease in patients with a history of malignancy.     

Primary peripheral primitive neuroectodermal tumour of the orbit

CASE REPORT: We present the clinical, radiologic, and histopathologic features of an orbital mass in a 10-year-old boy. Immunohistochemistry confirmed the diagnosis of primary peripheral primitive neuroectodermal tumour. COMMENTS: This recently recognized rare tumour of the orbit should be considered in the differential diagnosis of hypercellular small round cell tumour of the orbit.     

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 liposarcoma of the orbit: a clinicopathological study

Primary liposarcoma of the orbit is a rare tumour. There are very few cases of orbital liposarcoma reported in the literature, mostly of the myxoid variety. In this paper, the authors report the clinical presentation, histopathological features, results of diagnostic studies and management of a case of orbital low grade myxoliposarcoma with local recurrence, together with a review of the literature.     

Primary liposarcoma of the orbit: a clinicopathological study

Primary liposarcoma of the orbit is a rare tumour. There are very few cases of orbital liposarcoma reported in the literature, mostly of the myxoid variety. In this paper, the authors report the clinical presentation, histopathological features, results of diagnostic studies and management of a case of orbital low grade myxoliposarcoma with local recurrence, together with a review of the literature.