It is advised that lumps which are greater than 5cm should be referred to a sarcoma centre for management and that small lesions cause less harm with unplanned excision.
Of the hand patients 7 of the 10 patients had wide excision of the lesion with 3 an amputation. 5 of the cases were for inadequate previous excision (50%). 7 of the 17 in the foot and ankle group underwent amputation (41%) the rest wide excision. 29% of cases were for second time surgery due to inadequate previous excision.
All patients underwent staging and follow up at Sarcoma Clinic with functional assessment and TESS evaluation.
Nine patients underwent planned marginal excision. Sciatic nerve involvement was 13–30cm in eight cases and in one case the sciatic nerve was abutting the tumour throughout its length. There was soft tissue reconstruction in three cases using fascial adductor or gracilis graft for sciatic nerve cover and one with superficial femoral nerve and vein resection requiring ipsilateral saphenous reconstruction. The remainder underwent direct primary reconstruction. Four patients underwent radiotherapy 46–60 Gy. There was no local recurrence of disease within 14 – 96m follow-up. There was one patient with post radiation wound breakdown that resolved. Three patients have died of unrelated causes. To date there has been no evidence of local recurrence of disease at FU.
It has a characteristic radiographic appearance with marked periostitis in the involved bone mimicking osteosarcoma and associated focal abnormality in juxtaposed skeletal sites. With the help of two previously reported cases from the literature of aggressive bone forming tumours in children who presented with marked anorexia and cachexia diagnosed as osteomyelitis and osteosarcoma and treated by ablative surgery this tumour was correctly diagnosed with planned subtotal scapulectomy and reconstruction enabling a good functional result. The systemic symptoms fully resolved following surgery with return of appetite and weight gain.