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I. Case History
A. Patient Demographics: 82 YOWM
B. Chief Complaint: “My left eyelid is shut and I can’t open it”
C. POHX: Acquired left lid ptosis with miosis x 1mo, etiology unknown but Horner’s has
been r/o by CT; Congrous left inferior quadranopsia; Dry AMD OU; Cataract OS;
Pseudophakia OD.
D. PMHx: HTN, Hypothyroid, CAD, CHF, Carotid stenosis with total occlusion left and right
stent, multiple infarcts of left lobe and right medial occipital lobe, basal cell cancer
location unknown, left brow squamous cell carcinoma (SCC) with perineural invasion s/p
MOHS and radiation.
E. Meds: Coumadin, HCTZ, Lisinopril
II. Pertinent Findings (photos and imaging included where appropriate)
A. Clinical
1. Uncorrected DVA: OD 20/20, OS 20/25 while holding lid open
2. EOM: full OD, OS restriction superior and nasal
3. CF: bilateral left inferior quad, full all others
4. Pupils: PERRL-APD, no miosis noted
5. SLEx OS:complete UL ptosis, mass present supnasal 10mmx5mm in size with edema
surrounding, distension of superior tarsal plate, hypoglobus, 2+conjunctival
chemosis, possible sup orbital rim erosion compared to OD.
6. DFEx OS: bullous compression of superior retina from 10:00-1:00 at ora
extending to the CB
B. Lab studies: aspiration biopsy of LUL mass revealed malignant cells consistent with
origin from SCC
C. Radiology Studies
1. MRI: predominantly cystic mass involving the superior left orbit and left orbital rim
which displaces the globe inferiorly
2. PET/CT
a. Cystic rim enhancing mass causing mass effect upon the left globe, mass is
consistent with the given history of left orbital SCC although the tumor does not
demonstrate FDG activity.
b. No evidence of metastatic disease via PET scan
III. Differential Diagnosis
A. Primary/leading: recurrence of SCC vs other tumor
B. Others: traumatic ptosis, third nerve palsy
IV. Diagnosis and Discussion
A. Diagnosis: Recurrent squamous cell carcinoma involving the superior orbit OS
B. Discussion:
This patient had a history of SCC with perineural invasion (PNI) involving the left brow
in 2003. He was treated with MOHS after the original tumor excision and underwent radiation
therapy for PNI. PNI in cutaneous SCC is associated with an increased risk of local recurrence
and distant metastasis. The perineural space is a cleavage plane between the nerve and nerve
sheath which extends from small peripheral nerves to the subarachnoid space. Less than 5%
of excised skin cancers are reported to exhibit PNI and of these only 30-40% present with
clinical manifestations. Patients are typically middle aged-elderly Caucasian males with
previously treated skin cancer of the head and neck. Symptoms such as sensory disturbance
or pain can be brief and episodic and can predate the diagnosis by months to years. Ocular
motility disturbances are present in 76-92% of cases. Over time, diplopia and ophthalmoplegia
can develop and indicate advanced orbital disease. The diagnosis can often be made clinically
with biopsy corroborating the clinical and radiological findings. Evidence of PNI on imaging is
associated with a poorer prognosis (50% 5 yr survival rate) than negative disease (86% 5 yr
survival rate).
V. Treatment, Management
A. The preferred mode of treatment for cutaneous SCC is MOHS, although there is still a
significant recurrence rate.
B. The treatment options for this patient were:
1. Do nothing: extremely poor prognosis with patient facing eminent death
2. Surgical debulking: the potential for tumor clearance was minimal
3. Orbital exenteration: the chance of recurrence is rare, cosmesis may be a factor
4. Radiation: palliative, mainly used for growth restraint, may be of limited use since pt
has hx of radiation.
C. Patient’s decision: orbital exenteration with post-operative radiation after the graft has
healed.
D. Bibliography:
1. Howard GR, Nerad JA, Carter KD, Whitaker DC. Clinical characteristics associated with
orbital invasion of cutaneous basal cell and squamous cell tumors of the eyelid. Am J
Ophthalmol 1992 Feb 15; 113(2):123-33.
2. Leibovitch I, et al. Cutaneous squamous cell carcinoma treated with MOHS
micrographic surgery in Australia II: Perineural invasion. J AM Acad Dermatol 2005
Aug; 53:261-6.
3. Bowyer J, Sullivan T, Whitehead K, Jelly L, Allison R. Management of perineural
spread of squamous cell carcinoma to the ocular adnexae. Ophthal Plast Reconstr
Surg 2003 Jul; 19(4): 275-81.
4. Clouston PD, Sharpe DM, Corbett AJ, Kos S, Kennedy PJ. Perineural spread of
cutaneous head and neck cancer. Its orbital and central neurologic complications.
Arch Neurol 1990 Jan; 47(1):73-7.
5. Veness MJ. Perineural spread in head and neck skin cancer. Australas J Dermatol
2000 May; 41(2):117-9.
6. Veness MJ, Biankin S. Perineural spread leading to orbital invasion from skin cancer.
Australas Radiology August 2000; 44(3):296-302.
7. McNabb AA, Framis IC, Benger R, Crompton JL. Perineural spread of cutaneous
squamous cell carcinoma via the orbit. Clinical features and outcome in 21 cases.
Ophthal 1997 Sep; 104(9):1457-1462.
8. Donaldson MJ, Sullivan TJ, Whitehead KJ, Williamson RM. Squamous cell carcinoma of
the eyelids. Br J Ophthalmol 2002; 86:1161-1165.
VI. Conclusion
A. Clinical Pearls
1. Recognition that periocular skin cancer represents a potentially blinding and life
threatening disease.
2. PNI in SCC is associated with increased risk of recurrence and metastasis.
3. Potential of orbital invasion from skin cancer is well documented.
4. Patients are typically middle-aged-elderly Caucasian males with a previous history of
skin cancer.
5. Symptoms and signs may be subtle and brief and include sensory and oculomotor
disturbance, facial palsy, orbital pain and ophthalmoplegia.
6. Imaging is useful to delineate the extent of disease and determine the prognosis.
7. Once orbital invasion is noted, early intervention, often with radical procedures, is
necessary to prevent intracranial spread.