Secondary orbital melanomas: analysis of 15 cases.

Orbital melanomas comprise a heterogeneous group of pigmented tumours originating from melanocytes of the ophthalmic tissues, or represent distant metastases of cutaneous melanomas to the orbit. They can be classified into primary and secondary orbital melanomas. Whereas primary orbital melanomas are extremely rare, secondary orbital melanomas are seen more often and usually represent massive extrascleral extensions of uveal melanomas. Their diagnosis is difficult and controversy exists both about the treatment policy and regarding the prognosis. In an effort to clarify some of the aspects of the biological behaviour of these intriguing lesions, we retrospectively reviewed all orbital melanomas treated in our departments during the last eight years. The records of 15 patients with massive secondary orbital melanomas treated surgically were reviewed and analysed. Eleven of the patients were female and four were male. The mean age at the time of surgery was 68 years. Presenting features included unilateral orbital mass, often with painless proptosis, conjuctival bleeding, acute glaucoma crisis, decreased visual acuity and intermittent diplopia. The site of origin was the uveal tract in nine cases, the conjunctiva in three, the eyelids in two and the skin of the lower extremity in one patient. All patients were treated surgically with various types of orbital exenteration. Additional treatment included radiotherapy and chemotherapy. Two patients died from intracranial extension of the disease and 10 died from distant metastases. Three patients are alive with no evidence of disease. The longest survival was 33 months and the mean survival was 16.6 months. Early diagnosis and proper management of ocular melanoma prevents orbital extension and prolongs patient survival. Surgical treatment of secondary orbital melanoma with or without adjuvant radiotherapy and/or chemotherapy does not seem to improve patient survival when compared with conservative treatment used in other reports. However, orbital exenteration is effective for local control of the disease.

Topical mitomycin C chemotherapy for conjunctival melanoma and PAM with atypia.

AIM: To evaluate topical mitomycin C (MMC) chemotherapy in the treatment of conjunctival melanoma and primary acquired melanosis with atypia. METHODS: In a phase I clinical trial, 10 patients with conjunctival melanoma and/or primary acquired melanosis with atypia were treated with topical MMC 0.04% four times daily. Four patients were given MMC for 28 days as a primary treatment. Six patients were treated with MMC for 7 days after excision and cryotherapy in an effort to improve local control. In this series, 10 patients have been followed for an average of 29 months. RESULTS: All patients were noted to develop transient keratoconjunctivitis during treatment. One patient also developed a transient corneal epithelial defect. Decreased conjunctival pigmentation was noted in the four patients where topical chemotherapy was used as a primary treatment. Nodular tumours were resistant to topical MMC chemotherapy. Of the six patients treated within 2 weeks after primary excision and cryotherapy, there has been no tumour recurrence or symblepharon formation. Nine of the 10 study patients have maintained within one line of their pretreatment visual acuity. No retinal or optic nerve toxicity was noted. CONCLUSION: Since no complications which might preclude further investigation of topical MMC chemotherapy occurred, it was concluded that topical MMC chemotherapy was tolerated as a treatment for conjunctival melanoma and primary acquired melanosis with atypia.

Ocular granulocytic sarcoma in childhood acute myelogenous leukemia.

We report two children of Greek origin with granulocytic sarcoma of the orbit and acute myelogenous leukemia. In the first case the orbital tumor was the initial manifestation of the disease, while the other occurred during hematological remission.

[Orbital and intracranial complications of paranasal osteomata (author's transl)]. / Complications orbitaires et endocraniennes des ostéomes des sinus paranasaux.

Osteomata of the paranasal frontal and ethmoidal sinuses are benign, slow growing tumors generally asymptomatic. Complications due to orbital or intracranial development of the osteoma are rare and demand neurosurgical treatment. The authors report two cases. In the first a fronto-ethmoidal osteoma first caused exophtalmos and later ophtalmoplegia due to compression of the superior ophtalmic vein. In the second case the posterior development of an osteoma of the frontal sinus resulted in pneumocephalus with epileptic fits and headache and initially homolateral hemiparesis. In both cases CT Scan showed the extent of the osteoma and in the second case the gap in the wall of the sinus. Both osteomata were radically removed through frontal craniectomy. The literature is reviewed, 12 other cases of pneumocephalus due to posterior development of paranasal osteomata have been published during the last 50 years.

Effects of urea treatment in malignancies of the conjunctiva and cornea.

A series of 9 patients with extensive squamous cell carcinomas of the conjunctiva, affecting also the cornea in 5 of them, were treated with local applications of urea. 8 out of these patients were cured. In 1 patient with a concomitant extensive conjunctivitis, the treatment was ineffective. Urea treatment is very simple and without any complications, apart from a transient opacity of the cornea. Recurrences are easily and effectively retreated as in 1 of 2 of our cases. But if the eyeball has been destroyed, as in the second case, retreatment is impossible. At least 5 out of the treated patients would have had an enucleation of the globe or an exenteration of the orbit without the urea treatment.