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Metastastic choriocarcinoma causing cavernous sinus syndrome
  1. E Sharkawi,
  2. K Tumuluri,
  3. J M Olver
  1. Department of Ophthalmology, Charing Cross Hospital, Fulham Palace Road, London, UK
  1. Correspondence to: Eamon Sharkawi MBBS, BSc, MRCP, MRCOphth, Department of Ophthalmology, Charing Cross Hospital, Fulham Palace Road, London W6 8RF, UK; eamonshark{at}doctors.org.uk

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Choriocarcinoma is a rare trophoblastic tumour.1,2 We describe a case of metastatic choriocarcinoma to the cavernous sinus causing complete ophthalmoplegia and ptosis, following an uneventful pregnancy.

Case report

A 28 year old woman was referred to the oculoplastic and orbital service with a 1 week history of right sided ptosis and 6 weeks of right facial pain. Six weeks earlier she underwent evacuation of retained products of conception, following spontaneous vaginal delivery. Facial pain immediately ensued and her dentist extracted a right wisdom tooth, but the pain continued to worsen. The visual acuity was 6/9 right eye, 6/5 left eye, with complete right sided ptosis, total ophthalmoplegia, and a dilated unreactive pupil (fig 1A, B). Reduced sensation in the distribution of right trigeminal branches V1 (including cornea) and V2 was noted. There was no relative afferent pupillary defect, visual field or colour vision defects, or proptosis.

Figure 1

 (A, B) Right sided complete ptosis, fixed dilated pupil, and total ophthalmoplegia. (C, D) Axial and coronal post gadolinium T1 MRI brain scans, showing enhancing right cavernous sinus mass extending to right temporal lobe surface. Arrows illustrate tumour location. (E, F) MRI scans 6 months after chemotherapy, showing reduction in tumour size. Reproduced with permission.

Magnetic resonance imaging (MRI) scans of brain and orbits (fig 1C, D), revealed a right cavernous sinus mass extending to right temporal lobe. Image guided biopsy of the temporal lobe revealed a dural encased highly vascular choriocarcinoma. Subsequent computed tomography (CT) imaging revealed a grossly enlarged uterus and multiple large pulmonary metastases. Serum β-HCG levels were markedly elevated at 2.5×107 IU/l, consistent with choriocarcinoma. She was commenced on systemic and intrathecal chemotherapy. Five months later, β-HCG levels returned to normal, her facial pain and sensation improved, and her ptosis resolved. Between 9 months and 18 months, ocular motility examinations revealed a stable right esotropia with poor abduction and reduced elevation. She underwent lateral transposition surgery to superior and inferior recti (combined with botulinum toxin chemodenervation of ipsilateral medial rectus) to improve ocular alignment.

MRI scans at 6 months revealed a reduced tumour size (fig 1E and F). She remains clinically stable 18 months after presentation with a useful field of binocular single vision.

Comment

This patient had a potentially fatal cavernous sinus and intracranial metastasis from choriocarcinoma. Patients can become symptomatic long after an uneventful pregnancy,1–3 and may present to a variety of specialists, including ophthalmologists, as illustrated by our case. A high index of suspicion, rapid diagnosis, and multidisciplinary care are essential when managing women of childbearing age with neurological or orbital symptoms, as early treatment is curable.

Multiple cranial nerves palsies, particularly 3, 4, 6, and 5 divisions V1 and V2, while sparing optic nerve function are strongly suggestive of cavernous sinus pathology.4,5 Common neoplastic causes of sphenocavernous syndrome include nasopharangeal carcinoma, lymphoma, meningioma, lung and breast metastases (see table 1 for other aetiologies); however, metastatic choriocarcinoma has, to our knowledge, not been reported.

Table 1

 Causes of sphenocavernous or orbital apex syndrome

Although a less favourable prognosis is associated with central nervous system (CNS) metastasis,3 newer intensive chemotherapy regimens such as high dose EMA-CO (etoposide, methotrexate, actinomycin-cyclophosphamide, and vincristine), have encouraging results,6 as highlighted by this patient. In sufficiently chemosensitive tumours, the blood-brain barrier does not prevent treatment from eliminating CNS disease.6 Additional modalities for treating cerebral metatstases include intrathecal chemotherapy and neurosurgery.1–3 Cranial irradiation may be used in selective cases; however, there is no consensus regarding its role.3,6

References

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