Editor - Olufemi E. Idowu Copyright- Frontiers of Ikeja Surgeon, 2015 CLINICAL VIGNETTE OF THE MONTH -September 2015 (e- edition, vol 1:1)

Dermatofibrosarcoma Protuberans: Current Management Trend Copyright- Frontiers of Ikeja Surgeon, 2015 Ajani AO; Jinadu

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 2 0 & 3 0 Hospital - Radiotherapy Growth recurred a year ago with ulceration and infection Copyright- Frontiers of Ikeja Surgeon, 2015

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 Copyright- Frontiers of Ikeja Surgeon, 2015

CT Thoraco-lumbar- huge soft tissue mass, not attached to underlying spine or ribs Copyright- Frontiers of Ikeja Surgeon, 2015

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 Copyright- Frontiers of Ikeja Surgeon, 2015

Histology- proliferating spindle cells arranged in storiform pattern in keeping with dermatofibrosarcoma protuberans Adjuvant therapy- oral Imatinib 400mg daily Copyright- Frontiers of Ikeja Surgeon, 2015 Treatment

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 Copyright- Frontiers of Ikeja Surgeon, 2015

Discussion… <0.1% of all malignancies and 1% of soft tissue sarcomas Occur in all ages, most common years Peak incidence recorded in the 4 th 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 Copyright- Frontiers of Ikeja Surgeon, 2015

Differentials Diagnoses Keloid, Hypertrophic scars Dermatofibroma Neurofibroma Sarcoidosis Lymphoma Lipoma Malignant melanoma Cutaneous secondaries fibrosarcoma Copyright- Frontiers of Ikeja Surgeon, 2015

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 Copyright- Frontiers of Ikeja Surgeon, 2015

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 Copyright- Frontiers of Ikeja Surgeon, 2015 Discussion- Prognosis

Conclusion Tumour is locally aggressive with high potential for recurrence Tissue biopsy key to diagnostic evaluation Imatinib likely revolutionises care Copyright- Frontiers of Ikeja Surgeon, 2015