Cervical tumor
- Skin tumours
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The clinical case
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Now it's your turn!
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Co-prescription and advice
Clinical case presentation
This 13-year-old girl presented with a median cervical skin lesion that had appeared three years earlier and was still evolving. She had no family or personal history and had never traveled outside mainland France.
The lesion was a reddish-brown, well-defined nodule, 1.5 cm in diameter, tender to the touch, mobile on the deeper planes, covered by a slightly thickened epidermis.
Darier’s sign was negative. No peripheral adenopathy was palpable, and the rest of the dermatological examination revealed no other distant lesions.
In the absence of a definitive clinical diagnosis, a biopsy was performed: beneath a hyperplastic epidermis, there was a dermal proliferation of large cells with granular eosinophilic cytoplasm and small central nuclei, dissecting through collagen fibers. There was no cytologic atypia or mitotic activity.
Immunostaining for S100 protein and NSE was positive, but negative for desmin and smooth muscle alpha-actin.
Your turn
What is your diagnosis?
Select 1 answer(s) from the following choices:
Wrong answer!
Good answer!
Selected diagnosis
Granular cell tumor (Abrikossoff tumor) is relatively atypical in terms of age of onset and cutaneous location. This tumor, most often of Schwann cell origin, mainly affects adults and has a mucosal distribution, particularly lingual (70%). Genital (vulva), ENT and digestive mucosa may also be involved.
Involvement of the skin is rarer and does not present any specific or suggestive clinical features. It is therefore a histological diagnosis, confirmed by immunostaining.
Explanation of wrong answers
- Benign cutaneous lymphocytoma is a benign lymphoproliferation in response to infection with Borrelia burgdorferi (Lyme disease), most commonly occurring in children and in the cephalic region as a painless, non-itchy pink, red or brown nodule. Diagnosis may be facilitated by the presence of a tick bite and/or erythema migrans in the preceding weeks or months, serology, or a biopsy with PCR if necessary.
- Mastocytoma is a localized form of cutaneous mastocytosis, common in children. In this case, it can be ruled out by a negative Darier sign.
- Cutaneous B-cell lymphoma in its solitary nodular form is sometimes clinically and histologically indistinguishable from a pseudolymphoma, particularly from a borrelial lymphocytoma; a trial of antibiotic therapy is sometimes necessary to clarify the situation.
Treatment
This tumor is not syndromic and is benign in the vast majority of cases. Surgical excision without margins is the only recommended treatment.
Message from the expert
Abrikossoff tumor should be suspected in the presence of a skin nodule, even when it occurs in children and elsewhere than on the tongue!
References
Lahmam Bennania and col Childhood cutaneous Abrikossoff tumor. Archives de Pédiatrie 2011 ;18 :778-782
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