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Editor,—Basal cell carcinoma (BCC) is the most common malignant tumour of the eyelids and face. Factors which increase the chance of orbital invasion include a medial canthal location, slow indolent growth, morpheaform growth pattern, surgical recurrence, advanced presentation, and neglect. Orbital invasion predisposes to intracranial involvement by direct or perineural spread. Management of orbital invasion is difficult and requires a multidisciplinary team approach for radical surgery and/or radiotherapy.
We present a 76 year old Ukranian man with a neglected tumour on his forehead which had spread over several years to involve the upper eyelids, anterior orbits, and ethmoid sinuses.
CASE REPORT
A 76 year old man with a long history of untreated forehead BCC complained of deteriorating vision in his right eye and ocular discharge. He had been admitted by the care of the elderly unit, for social reasons. He was a known paranoid schizophrenic treated with psychotropic drugs. Four years previously his physician had measured the lesion as being 2 cm × 4 cm and recommended plastic surgery, which was declined.
There was a massive, fungating lesion of the forehead, superior orbits, and nasal bridge, at least 15 cm × 17 cm (Fig 1). The right uncorrected visual acuity was 6/24 and left only light perception (unable to use a pin hole). There was fixed ptosis and right lower eyelid involutional ectropion. There was an opaque left cornea. The right fundus was normal. He did not permit intraocular pressure measurement.
Oblique view of massive basal cell carcinoma of the forehead, nasal bridge, and upper eyelids showing right upper eyelid involvement, superomedial ulceration, and lower eyelid ectropion.
Investigations included a computed tomograph (CT) scan to evaluate the extent of tumour invasion and an incisional biopsy for histopathological diagnosis. The CT scan showed extensive soft tissue destruction, loss of the nasal bone, frontal sinuses, and bilateral anterior ethmoid, and extraconal orbital invasion (Fig 2). Histopathology confirmed extensive solid basal cell carcinoma.
Computed tomograph scan shows destruction of the left nasal bone, frontal sinus, and bilateral anterior ethmoid involvement, bilateral anterior orbital extraconal involvement, as well as extensive soft tissue destruction.
He received symptomatic care with lid cleansing and topical chloramphenicol. A low visual aid assessment was arranged. Palliative treatment with radiotherapy was recommended but he refused all treatment including the low vision assessment.
The patient underwent a thorough mental status examination, which confirmed well controlled schizophrenia. We were not empowered to detain him for treatment against his will. He discharged himself from hospital with an untreated fungating BCC.
COMMENT
This patient had a neglected BCC which had caused destruction of soft tissue and bone, with orbital invasion and was threatening the vision in his remaining eye.
Where advanced scalp cancer displays deep invasion, radical excision and reconstruction are indicated. Some authors suggest that aggressive surgical management of advanced skin neoplasia is the only treatment to produce long term survival. In malignant cutaneous tumours involving the anterior skull base, invasion of the dura mater significantly affects survival. In particular, spread along the medial orbital wall can lead to meningeal infiltration by direct invasion. As with squamous cell carcinoma, large basal cell carcinomas can invade the central nervous system by perineural spread. Our patient risks spread of the tumour along the supraorbital and supratrochlear nerves.
When local surgical therapy fails to prevent recurrence or definitive surgical resection is not possible, as in this case, alternative therapies must be considered. Opinions vary on the roles and efficacy of radiation therapy and chemotherapy for extensive lesions. Cisplatin and doxorubicin have been reported to achieve complete remission of recurrent invasive BCC of the medial canthus and orbit at 5 years. Using adjunctive radiotherapy, large BCCs of the head showed partial to complete response but no cures achieved. A complete response was defined as disappearance of all measurable lesions (but cancer cells are still present microscopically) and a partial response was 50% reduction in all lesions.
Patients with large or aggressive skin cancer are fortunately uncommon and management should be individualised following discussion with both the patient and his/her family. The options include a combination of surgery, radiotherapy, and chemotherapy with every effort made to preserve vision.
Our elderly, schizophrenic patient declined treatment and in these circumstances symptom relief is all that can be offered. Legal issues prevent forced treatment.
Informed consent includes providing adequate information about the treatment to make a reasoned decision. Obtaining consent must be free of coercion or threats, which would affect the patient's decision. The patient must be presumed competent unless shown otherwise. Psychiatric assessment confirmed that this patient was competent to make his own decisions. Exceptions include if immediate treatment is needed and the patient is unable to provide it (in coma or insufficient time to obtain it), the patient is legally incompetent to make a treatment decision, or decides to waiver the right to be fully informed.
Untreated, the outlook for this patient is grim.