Size was available in 26 patients; three patients were scar excisions. The mean size of the resected lesions was 5.5 cm (2–15 cm). Five patients required additional soft tissue cover for wound closure. The average nodal yield was 3 (range = 1–4). Six patients had microscopic margin positive resection, and all received post-operative radiation. Overall, 22 patients received radiation (2 — pre-operative radiation, 2 — brachytherapy, 18 — post-operative radiation). None of the patients received chemotherapy.

Two patients were lost to follow-up at 7 and 35 months, respectively. At a mean follow-up of 45 months (12–118 months), 15 patients have died and 12 are alive.

Six of 27 patients developed local recurrence at a mean of 36 months (15–58 months). Three patients had isolated local recurrence and underwent surgical excision, all developed unresectable distant metastasis. Two succumbed to disease at 52 and 58 months from index surgery and one is on supportive care. Three patients developed both local and pulmonary relapse (19–50 months). Of these 3, two underwent surgery at both sites and are alive at last follow-up (46 and 108 months). One patient had disseminated disease and was treated with best supportive care.

Of 6 margin positive resections, only one patient developed local recurrence. He underwent a wide excision of the recurrence but developed multiple lung metastasis 8 years from index surgery. He is currently alive with the disease. The other five developed distant recurrences and only one patient is alive after subsequent surgical resection.

The local recurrence free survival of the entire cohort was 88% and 56% at 3 years and 5 years, respectively. It was significantly worse in cases with upper limb lesions compared to lower limb lesions (31% vs. 67% p = 0.04) (Table 1).

Of 19 non-metastatic patients, one was lost to follow-up at 35 months. Fourteen developed distant recurrence (2–64 months, mean: 28 months). Various sites of recurrence were nodes (8), lungs (4), brain (1), bone (1), and soft tissue (3). Three of these patients had distant recurrence at two sites synchronously. Of the 14, nine were treated surgically. Six of 9 patients are alive at last follow-up (27–107 months), two died due to disease progression and one was lost to follow-up. In the other five, complete surgical resection at all sites was not possible and they eventually died due to disease.

Ten patients were metastatic at presentation: 3 had lung metastasis, 6 had nodal metastasis, and 1 had both. Of the 3 patients who had isolated lung metastasis, one patient underwent metastectomy along with resection of primary lesion but subsequently developed recurrence in the lung 4 months after index surgery and died 11 months later. Two underwent only resection of primary lesion and were kept under surveillance, in view of small size of the lung lesions. In both patients, pulmonary lesions progressed on follow-up. One patient had unresectable disease and died after 10 months and another patient underwent metastectomy and developed multiple lung metastasis 8 years from index surgery and is currently alive with the disease.

The patient with both, pulmonary and nodal (axillary nodes) metastasis underwent metastectomy and nodal dissection along with resection of primary lesion and developed recurrence in the lung after 10 months and died of the disease 15 months post index surgery.

All six patients with isolated nodal metastasis underwent nodal dissection along with resection of primary lesion. Of these, one had recurrence in the lung after 1 year, underwent metastectomy and is alive after 10 years of follow-up. Five patients developed unresectable recurrences at various sites (3–23 months) and died of disease progression (12–36 months).

Only 1 out of 10 patients metastatic at presentation are disease-free and alive at last follow-up.

5-year local recurrence free survival for patients who received radiation was 52% vs 67% (p = 0.49).

The mean time after surgery till recurrence was 20 months, ranging from 2 to 64 months (27 months for non-metastatic patients, 17 months for patients metastatic at presentation).

Twenty-four patients had distant recurrences at a mean of 22 months (3–64 months) (lung metastasis in 6, bone in 1, brain in 1, only nodal in 9, soft tissue in 1, both lung and nodal in 2, lung and soft tissue in 2, nodal and soft tissue in 1, soft tissue lung and bone in 1). Of 24 distant recurrences, 12 patients underwent surgical resection, the rest were all treated with best supportive care due to extensive disease. Of 12 patients who had surgical resection 3 patients died. Thus, 15 of 24 patients died after distant recurrence. The distant recurrence free survival of the entire cohort was 41% and 35% at 3 years and 5 years, respectively. 5-year survival after surgical resection of recurrence was 65% as compared to 20% (p = 0.016) in those that did not have resection.

The overall survival of the entire cohort was 63% and 53% at 3 years and 5 years, respectively (Fig. 1). Survival rates were significantly poorer in patients with metastasis at presentation, 66% vs. 25% (p = 0.016) (Fig. 2).

Overall survival of entire cohort in this series

Overall survival – Metastatic vs Non metastatic in this series

Patients with a tumor less than 5 cm had a 5-year overall survival of 64% as against 34% for those with a tumor greater than 5 cm (p = 0.34).

The event free survival of the entire cohort was 39% and 14% at 3 years and 5 years, respectively. Event free survival for metastatic patients at presentation was 0%, compared to 19% in non-metastatic patients (p = 0.05).