Adult metastatic orbital disease

Although in adults systemic tumours will metastasise more commonly to the uveal tract, orbital metastases represent 2-3% of all orbital tumours and occasionally will be the first manifestation of an occult primary tumour. In children, however, distant tumours most frequently metastasise to the orbit. As the orbit does not contain any lymphatic channels, it is presumed that haematological spread is responsible for metastatic disease.

In adults the commonest primary sites are breast, prostate, lung, kidney and gastrointestinal tract and they may present with painful proptosis and diplopia, such that a misdiagnosis of orbital inflammation or abscess may be made (Figure 13.10). It is important that the possibility of orbital malignancy is considered, and open biopsy undertaken, whenever a disease progresses despite treatment.

Figure 13.10 Patient referred after exploration for presumed orbital abscess; subsequent biopsy of the inferior rectus muscle demonstrated necrosis of a metastatic carcinoma.

The mainstay of therapy is local treatment with 55-60cGy fractionated radiotherapy, although in some cases there may be a role for surgical debulking prior to radiotherapy. Patients with a solitary orbital metastasis from a carcinoid or renal adenocarcinoma should be considered for exenteration.

The prognosis is variable, but generally the main goal of treatment is palliative with avoidance of discomfort and preservation of vision if possible. Dry eye is a major problem after the high dosage of radiation required for treatment of malignancy.

Rare primary malignant orbital tumours of adulthood

Malignant neurilemmoma, extremely rare and of Schwann cell origin, may arise de novo or in association with neurofibromatosis. It presents as a slowly progressive lid mass or proptosis and CT scan shows an ill-defined mass with possible destruction of adjacent bone. Management involves wide surgical clearance, often requiring orbital exenteration, and adjunctive radiotherapy or chemotherapy. The prognosis is poor as these malignant tumours tend to spread from the orbit into the middle cranial fossa and metastasise to the lungs.

Orbital haemangiopericytoma is a tumour that has a spectrum of invasiveness, from well-circumscribed, benign disease to infiltrative malignancy. Treatment of the benign lesions is intact excision, which is curative, whereas the malignant variants require wide clearance, usually by orbital exenteration. Local and remote recurrence are fairly common with malignant haemangiopericytoma.

Further reading

Affeldt JC, Minchler DS, Azen SP, Yeh L. Prognosis in uveal melanoma with uveal extension. Arch Ophthalmol 1980; 98:1975-9.

Bartley GB, Garrity JA,Waller RR et al. Orbital exenteration at the Mayo clinic. 1967-1986. Ophthalmology 1989; 96:468-73.

Ferry AP, Font RL. Carcinoma metastatic to the eye and orbit. A clinicopathologic study of 227 cases. Arch Ophthalmol 1974; 92:276-86. Fratkin JD, Shammas HF, Miller SD. Disseminated Hodgkin's disease with orbital involvement. Arch Ophthalmol 1978; 96:102-4.

Goldberg RA, Rootman J, Cline RA. Tumours metastatic to the orbit: a changing clinical picture. Surv Ophthalmol 1990; 35:1-24.

Mamalis N, Grey AM, Good JS, McLeish WM, Anderson RL. Embryonal rhabdomyosarcoma of the orbit in a 35-year-old man. Ophthalmic Surgery 1994; 25:332-5.

Meekins B, Dutton JJ, Proia AD. Primary orbital leiomyosarcoma: a case report and review of the literature. Arch Ophthalmol 1988; 106:82-6.

Rose GE,Wright JE. Exenteration for benign orbital disease. Br J Ophthalmol 1994; 78:14-18.

Rose GE, Wright JE. Pleomorphic adenomas of the lacrimal gland. Br J Ophthalmol 1992; 76:395-400.

Schworm HD, Boergen KP, Stefain FH. The initial clinical manifestations of rhabdomyosarcoma. Ophthalmology 1995; 92:362-5.

Shields CL, Sheilds JA, Peggs M. Metastatic tumours of the orbit. Ophthal Plast Reconstr Surg 1988; 4:73-80.

Sohaib SA, Moseley I,Wright JE. Orbital rhabdomyosarcoma - the radiological characteristics. Clin Radiol 1998; 53:357-62.

Weber AL, Jakobiec FA, Sabates NR. Lymphoproliferative disease of the orbit. Neuroimaging Clin N Am 1996; 1:93-111.

Wright JE, Rose GE, Garner A. Primary malignant neoplasms of the lacrimal gland. Br J Ophthalmol 1992; 76:401-7.

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