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Dermatology · Skin tumours

Rare skin tumours

Merkel cell carcinoma, dermatofibrosarcoma, cutaneous lymphomas, Kaposi’s sarcoma… Rare skin tumours are uncommon skin cancers that are often more aggressive or more difficult to diagnose than basal cell and squamous cell carcinomas, and they require specialist care.

Written by Dr A. Turcarelli, dermatologist and allergist Updated 5 January 2026 6 min read

Tumeurs cutanées rares

Introduction

Because they are rare, they can be difficult to diagnose, which is why specialist dermatological expertise and early multidisciplinary care are so important.

Symptoms

  • Rapidly growing skin nodule or plaque
  • Firm, infiltrated lesion, sometimes painful
  • Unusual colour: purplish, dark red, skin-coloured
  • Possible ulceration or bleeding
  • Single or multiple lesions
  • No improvement despite standard treatment
  • Possible associated swollen lymph nodes

Causes

Weakened immune system (organ transplant, immunosuppressive treatment)
Older age
Chronic sun exposure (for certain types)
Cancer-causing viral infections (e.g. polyomavirus)
Particular genetic background
Merkel cell carcinoma (aggressive, fast-growing neuroendocrine cancer)
Skin sarcomas (angiosarcoma, dermatofibrosarcoma, etc.)
Skin adnexal tumours (sweat glands, sebaceous glands)
Cutaneous lymphomas (primary T-cell or B-cell lymphomas of the skin)
Skin metastases from an internal cancer

Diagnosis

  1. Expert dermatological examination
  2. Dermoscopy (helpful but not specific)
  3. Deep skin biopsy (essential)
  4. Histological and immunohistochemical analyses
  5. Routine staging investigations
  6. Review at a specialist multidisciplinary team meeting

Treatments

Wide surgical excision (when possible)
Adjuvant or primary radiotherapy
Specialist systemic treatments (depending on the tumour type)
Personalised combined protocols
Supportive dermatological care
Lasers and local techniques only in very selected cases
Medical LED possible to support healing, never as a cure

Management

Rapid referral to a specialist dermatologist

Accurate histological diagnosis

Coordination with oncology, surgery and radiotherapy

Close, long-term follow-up

Clear information and support for the patient

Frequently asked questions

Consultation for rare skin tumours

Consultation with a confirmed diagnosis and a written treatment plan.

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