Orbital tumours

See the separate article on Eye and Optic Nerve Tumours. Other relevant separate articles include Retinoblastoma and Rhabdomyosarcoma.

Other tumours occurring in the orbit include:

  • Lacrimal adenomas. Locally invasive tumours of the lacrimal gland. These occur in middle age and slightly more often in males. There is gradual, painless proptosis over months or years with a palpable tumour in the superior temporal quadrant of the orbit. Treatment is excision of the tumour and the lacrimal gland, with an excellent prognosis if the removal is complete. Malignant transformation occurs in 10% of tumours over 10 years.
  • Lacrimal carcinoma. A rare malignant tumour of the lacrimal gland, usually occurring in middle age or elderly people. They grow rapidly, causing more rapid proptosis than adenomas, associated with ophthalmoplegia due to perineural spread, and pain. If diagnosed early, surgical removal may be attempted (usually followed by radiotherapy) but the prognosis is very poor.
  • Neuroblastoma. This is one of the most common of childhood malignancies. It arises from the primitive neuroblasts of the sympathetic chain, most commonly in the abdomen. Orbital metastases may be unilateral/bilateral and usually present with abrupt proptosis associated with a superior orbital mass. Prognosis is very poor.
  • Metastases:
    • In children the orbit may be the site of secondary deposits from neuroblastoma or acute leukaemia.
    • In adults, breast or lung carcinoma may give secondary deposits in the orbit.
  • Lymphomas(eg, Burkitt’s lymphoma). These may form orbital deposits (therefore, examine the liver, spleen and lymph nodes, and check FBC).
  • Nasopharyngeal tumours and mucoceles and pyoceles of the ethmoid and frontal sinuses. These occasionally invade the orbit. A frontal mucocele is a cystic swelling originating from the frontal sinus. There is slowly progressive proptosis with a palpable tumour in the superior nasal quadrant of the orbit. Treatment is by surgical drainage. Maxillary carcinoma, ethmoidal carcinoma and nasopharyngeal carcinoma may invade the orbit. See the separate article on Pharyngeal Cancer.

Idiopathic orbital inflammatory disease

This condition, previously known as ‘orbital pseudotumour’, is not a diagnosis but rather the description of an inflammatory response in the orbit, in response to trauma, infection, tissue necrosis, ischaemia, toxins and so on. It is a diagnosis of exclusion which can involve any of the orbital tissues. Its cause is not known.

  • It can affect one or both eyes of relatively young patients (less than 50 years old).
  • Inflammation may be diffuse, anterior, apical, around the optic nerve or selectively affecting the lacrimal gland.
  • Typically it presents with the rapid development of pain, unilateral or bilateral proptosis with or without paralysis of the extra-ocular muscles, and swelling around the eye and orbit.
  • A full blood screen is done to look for evidence of infection, inflammation or autoimmune disease.
  • Ultrasound and CT scans typically show an infiltration of the orbit, and an inflammation of the sclera and optic nerve.
  • Any underlying cause should be managed and the inflammation itself may respond to non-steroidal anti-inflammatory drugs. Failure to respond may prompt a diagnostic biopsy ± systemic steroids. Refractory cases may also be treated with radiotherapy.

 

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