1. Dermatofibrosarcoma Protuberans: Current Management Trend
CLINICAL VIGNETTE
2015; 1:1
Dermatofibrosarcoma Protuberans: Current Management Trend
Editor-in-Chief: Olufemi E. Idowu. Neurological surgery Division, Department of Surgery, LASUCOM/LASUTH, Ikeja, Lagos, Nigeria.
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2. Dermatofibrosarcoma Protuberans: Current Management Trend
Ajani AO; Jinadu
Burns & Plastic Division, Department of Surgery, Lagos State University Teaching Hospital, Ikeja, Lagos, Nigeria.
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Presentation…
O. D.; 30-year-old
Recurrent back lump- 13years
Started as a boil-like swelling, slowly growing initially, then rapid in the last 5months becoming huge and disfiguring
No antecedent trauma, animal or insect bite
Previous treatment
- Surgical excision & 2012 at a 20 & 30 Hospital
- Radiotherapy
Growth recurred a year ago with ulceration and infection
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Presentation…
Not a known hypertensive, diabetic or asthmatic
Previous blood transfusion without history of reactions
Pale, anicteric
Mass- huge fungating mass (45cmx30cm) on mid-back, slough ++, non-tender, differentially warm, engorged subcutaneous veins and not attached to underlying structure
No evidence of regional metastasis/enlarged lymph nodes
Diagnosis: Recurrent Dermatofibrosarcoma Protuberans
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CT Thoraco-lumbar- huge soft tissue mass, not attached to underlying spine or ribs
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Treatment…
Transfused and optimized for surgical excision
Electrolytes, Urea & Creatinine, Chest X-ray, LFT, Abdominal Ultrasound, FBS- Normal
Surgery- Wide local excision with 5cm perimeter margin
Post op recovery uneventful
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Treatment
Histology- proliferating spindle cells arranged in storiform pattern in keeping with dermatofibrosarcoma protuberans
Adjuvant therapy- oral Imatinib 400mg daily
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Discussion…
Rare disease and Unusually locally aggressive
Tumour of dermal origin
‘Annoyingly’ recurrent, Aetiology- largely unknown
85-90% low grade tumours; 1-5% may be malignant
Prior trauma-10-15%; may occur in old burn scars, surgical scars, multiple immunization sites
Possibly hereditary/ familial
Associations- long term arsenic exposure, acanthosis nigricans, acrodermatitis enterohepatica
Chromosome 17q to 22q translocation- collagen type1(COL1A1) to PDGF receptor-B chain gene in some cases
Exact cellular origin controversial
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Discussion…
<0.1% of all malignancies and 1% of soft tissue sarcomas
Occur in all ages, most common years
Peak incidence recorded in the 4th decade
Incidence (France Sweden, USA) per million population/year
Slight male preponderance in most studies, slight female preponderances in congenital cases
Higher incidence in coloured races especially dark-skinned
Bednar tumour variant almost exclusive to blacks
STAGING: German Guidelines
Stage I = Primary tumor, localized disease
Stage II = Lymph node metastasis
Stage III = Distal Metastasis
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11. Differentials Diagnoses
Keloid, Hypertrophic scars
Dermatofibroma
Neurofibroma
Sarcoidosis
Lymphoma
Lipoma
Malignant melanoma
Cutaneous secondaries
fibrosarcoma
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12. Discussion- Treatment Options
Surgical
Resection with wide margin
Moh’s Micrographic Surgery (MMS), particularly recurrent cases
Chemotherapy- as neo-adjuvant therapy
Imatinib Mesylate (Gleevec) monotherapy- small molecular pathway-tyrosine kinase- inhibitor; inhibits PDGF-receptors and effective for tumours positive for the t(17; 22) translocation; may induce tumour regression in recurrent, unresectable or metastatic DFSP especially adult patients; Usual dose 800mg daily
Others-vincristine, adriamycin, cyclophosphamide(VAC) combination also used
Radiation therapy sometimes useful
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13. Discussion- Prognosis
Metastasis in 1-5%, regional lymphatics usually & with poor prognosis
5year survival- 99.2%
Fibrosarcomatous variants poor prognosis
Patient's age >50years- poor prognosis
Follow-up: 3-6monthly
Complete history and review of systems
Complete physical examination; skin, excision site and regional lymph node examination
Extensive work up not needed except metastasis is suspected
Patient education necessary to report any new lesion to dermatologist
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Conclusion
Tumour is locally aggressive with high potential for recurrence
Tissue biopsy key to diagnostic evaluation
Imatinib likely revolutionizes care
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