Management of lacrimal sac tumours.

PURPOSE: To report a series of 15 primary lacrimal sac tumours and present an algorithm in managing this rare condition. DESIGN: Noncomparative interventional case series. METHODS: A retrospective review of the clinical, radiological, and pathological records of 15 patients with primary lacrimal sac tumours. Main outcome measures Histologic evaluation and clinical follow-up, including tumour clearance and recurrence, were assessed. RESULTS: Of the patients, 11 were female and the median age at presentation for the whole group was 59 years (range 22-94 years). The commonest tumour was non-Hodgkins B-cell lymphoma (five cases), followed by two cases each of squamous cell carcinoma and transitional cell carcinoma, one case of benign transitional papilloma, haemangiopericytoma, adenoid cystic carcinoma, mucoepidermoid carcinoma, mixed (squamous/transitional) carcinoma, and a highly malignant undifferentiated tumour. Treatment modalities included surgery, radiotherapy and chemotherapy and, with a median follow-up of 30 months (range 2 months to 17 years), two patients had died from metastatic disease but nine patients remained without evidence of recurrent tumour. CONCLUSIONS: Primary lacrimal sac tumours are extremely rare, require long-term follow-up for recurrence and metastasis, and can be fatal.

Two unusual osteogenic orbital tumors: presumed parosteal osteosarcomas of the orbit.

OBJECTIVE: To report two cases of suspected parosteal osteosarcoma of the orbit, with dedifferentiation into a high-grade liposarcoma occurring in one patient. DESIGN: Two retrospective case reports. METHODS: The clinical, radiologic, and pathologic records of two patients with suspected orbital parosteal osteosarcoma were retrospectively reviewed. MAIN OUTCOME MEASURES: Histologic evaluation and clinical follow-up were measured. RESULTS: The first patient was a 47-year-old male presenting with a 5-month history of painless right lower lid swelling; excision biopsy suggested a well-differentiated parosteal osteosarcoma of the orbital floor, which recurred 3 years later. Six months after excision of the recurrence, the mass demonstrated accelerated growth, and a lid-sparing exenteration was performed; histologic examination showed a high-grade liposarcoma. The patient remains disease-free at 4 years. The second patient, a 40-year-old male, presented with an 8-year history of proptosis and a right superotemporal orbital mass. The mass was excised completely at lateral orbitotomy; histologic examination suggested a well-differentiated parosteal osteosarcoma. The patient remains well 9 months postoperatively. CONCLUSIONS: Parosteal osteosarcoma is an uncommon tumor, usually affecting long bones, that is extremely rare in the orbit. It is a low-grade sarcoma that tends to recur locally after excision but has a favorable prognosis. Dedifferentiation into a high-grade sarcoma occasionally occurs in parosteal osteosarcoma, but transformation into liposarcoma does not seem to have been previously reported. It is important to recognize dedifferentiated parosteal osteosarcoma, because the prognosis is poor, and radical treatment may be required.

Visual prognostic value of the pattern electroretinogram in chiasmal compression.

BACKGROUND/AIMS: The visual loss associated with compression of the optic chiasm by pituitary tumours may be transient or permanent, possibly related to the extent of irreversible retrograde degeneration to the retinal ganglion cells. The pattern electroretinogram (PERG) N95 component is thought to rise in relation to retinal ganglion cell function and hence may be a potential prognostic indicator for visual function following decompressive surgery. METHODS: The notes and electrodiagnostic records of 72 eyes from 36 patients with chiasmal compression were retrospectively analysed. RESULTS: The postoperative change in visual field was found to be associated with the PERG N95:P50 ratio (p=0.01). Improvement in visual field was shown by a greater proportion of eyes with a normal N95:P50 ratio (65%) than with an abnormal ratio (27%). No change in visual field occurred in 26% of the eyes with a normal N95:P50 ratio compared with 67% of those with an abnormal ratio. Only 8% of eyes showed a worsening of visual field following surgery, in similar proportions for eyes with normal and abnormal N95:P50 ratios. There was no significant relationship with visual acuity. CONCLUSION: The PERG is a useful visual prognostic indicator in the preoperative assessment of chiasmal compression.

Transpupillary thermotherapy for retinal capillary hemangioma in von Hippel-Lindau disease.

Retinal capillary hemangiomas are a common manifestation of von Hippel-Lindau disease. We report the treatment of a peripapillary retinal capillary hemangioma in the left eye of a 30-year-old woman with this condition, using infrared diode laser transpupillary thermotherapy (TTT). The hemangioma was evaluated before and after treatment by ophthalmoscopy, fundus fluorescein angiography, and Doppler ultrasonography. Infrared diode laser TTT was delivered over 3 sessions during a period of 22 weeks, resulting in an improvement in visual acuity from counting fingers to 6/24 and a marked decrease in exudates surrounding the hemangioma. Doppler ultrasonography demonstrated a decrease in intralesional blood flow from 7 cm per second to less than 3 cm per second, together with a decrease in the size of the lesion. Infrared diode laser TTT provides a useful modality in the treatment of retinal capillary hemangiomas, and may be particularly favorable for peripapillary lesions because of its relatively nondestructive characteristics.

Cotton gauze foreign body granuloma following microvascular decompression.

We describe a case of a cotton gauze foreign-body granuloma developing 2 months after microvascular decompression for trigeminal neuralgia and hemifacial spasm. This complication has not been previously described. Moreover, the patient's initial clinical and radiological findings were suggestive of an acoustic neuroma or meningioma at the time.