| Introduction: |
| A 43 year old man was evaluated for treatment of multiple, recurrent basal cell carcinomas on his scalp and face. |
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| History: |
| For many years he was followed in dermatology for management of multiple new and recurrent basal cell carcinomas of the face and scalp. Tumors have been treated with surgical excision, Moh's micrographic surgery and carbon dioxide laser vaporization.
His past medical history was notable for intraventricular hemorrhage at birth, medulloblastoma at two years of age treated with resection and external beam radiotherapy without recurrence, and a second hemorrhage related to trauma when he was 6 years old that left him with a mild left hemiparesis. He developed a seizure disorder at age 11, and he underwent excision of a large jaw keratocyst when he was 27.
He was also diagnosed with mild mental retardation and depression. |
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| Clinical
Presentation: |
| He was a somewhat confused dysmorphic appearing man who complained of pain in his right foot and walked slowly to the examination room. Examination of the face showed frontal bossing, a protruding jaw, a broad saddle nose, and general coarsening of facial features. The skin exam was notable for multiple 1 mm to 3 cm round pearly nodules and tumors with surrounding telangiectasias throughout the scalp, posterior neck, nose and left temporal region. There were multiple scars on his scalp, nose, cheeks, eyelids, temples, ears, neck and arms. Multiple 1-2 mm pits were scattered on his palms and soles. The oral mucosa was pink and moist and without lesions. He did have a soft compressible subcutaneous cyst at the base of the right thumb and a surgical scar where a similar cyst was excised from the base of the right great toe 3 months earlier. |
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| Pathology: |
| A biopsy taken from a tumor on the left side of the neck shows typical features of basal cell carcinoma including basophilic lobules, nests and strands of tumor cells extending from the basal cell layer of the epidermis. Note the palisading arrangement of the peripheral cell layers separated from the surrounding stroma by cleft formations. |
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| Question
1: |
| What is the most likely diagnosis in this man? |
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| Question
2: |
| In addition to the basal cell tumors, what other lesions are associated with this disorder? |
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| Question
3: |
| How would you manage his tumors? |
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