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Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_I | Pages 38 - 38
1 Mar 2010
Arumilli BRB Crewe C Babu VL Khan T Paul AS Chan A
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Purpose: The literature on management of advanced soft tissue tumours is limited because of the rarity of cases following increased awareness and improved diagnostic resources.

Method: Our experience of managing 18 patients with fungating soft tissue tumours of the extremities and one patient with a sarcoma involving the scapular region (limb girdle) is presented. There were 14 males and 5 females. Average age was 70.6 yrs ranging between 37 – 98 years. 13 tumours involved lower limb and 6 the upper limb.

Results: The follow-up ranged from a minimum of 6 months to 10 years from the initial referral. The histological diagnosis was Sarcoma in 15 patients (Spindle cell sarcoma in 4, Fibrous Histiocytoma in 2, Pleomorphic sarcoma in 3, liposarcoma in 2, leiomyosarcoma in 2, Fibrosarcoma in 1 and 1 Round cell sarcoma). In the remaining 3 patients immunohistochemistry studies confirmed a Metastatic Squamous cell Sarcoma, a Metastatic Malignant Melanoma and a Metastases from a poorly differentiated upper GI malignancy each. Primary wide local excision was performed in 15 patients and primary amputation was performed in two patients. In 2 patients when tumour was unresectable due to the location and local spread, an embolisation was performed in both for palliation. Lung Metastases were present at the time of referral in 6 patients and developed later during follow-up in 4 patients. A histologically proven recurrence occurred in 6 patients after an average of 15.83 (4 to 41) months. Revision surgery was needed in 9 patients for either a positive margin on histology or a recurrence, including 3 secondary amputations. Local adjuvant Radiotherapy was given for 7 patients and a combination of radio and chemotherapy was used in 2 patients for metastases. Mortality was 53 % (9 patients) by the end of 32 months of follow-up.

Conclusion: Fungation in soft tissue tumours is rare and often a sign of locally advanced disease and a high grade nature, patients either have systemic spread by the time or develop later inspite of good local disease control. Primary wide local excision in such patients is difficult and has a high chance of a positive margin hence primary amputation may be better for local clearance. Recurrence of tumour and revision surgery is common and the mortality was > 50% at the end of 3 years from presentation to treatment in our series.


Orthopaedic Proceedings
Vol. 88-B, Issue SUPP_II | Pages 299 - 299
1 May 2006
Nandhara GS Babu L Ng ABY Paul AS
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Purpose: Many cases of Alveolar Soft Part Sarcoma have been reported on the trunk or head and neck region; we present two cases of the tumour in the soft tissues of the extremities.

Case Series: Alveolar Soft Part Sarcoma is a very rare tumour representing only 0.2–1 percent of soft tissue sarcomas. Patients commonly present with distant metastases both at the time of diagnosis and late in the course of disease. Many of the reported cases involve the trunk or head and neck region, especially around the orbit.

We present cases of Alveolar Soft Part Sarcoma, one in the upper limb the other in the lower limb, in two young females. Both were treated in the same centre by the same consultant.

The first is the case of a 33 year old female with a swelling in the right triceps present for 7 months before presentation to our centre. The time between diagnosis of possible tumour and biopsy was less than a month. Biopsy revealed a trojani grade 2 tumour and there were no metastases prior to wide excision. She received radiotherapy and has no metastases on follow up. Post therapy she is left with neurological pain and a rash on her face after radiotherapy.

The second case is that of a 25 year old female presenting, with a swelling in the right thigh, after 12 months including a 6 month history of shortness of breath. She had a family history of pancreatic cancer. Biopsy revealed a trojani grade 3 tumour and imaging revealed lung metastases. She went on to have chemotherapy. She later developed bone metastases.

Conclusion: Alveolar Soft Part Sarcoma must be suspected in all tumours and there aggressive nature must be realised.


Orthopaedic Proceedings
Vol. 88-B, Issue SUPP_II | Pages 299 - 299
1 May 2006
Nandhara GS Babu L Ng ABY Paul AS
Full Access

Purpose: Case to highlight the difficulties and delays in diagnosing pelvic soft tissue tumours.

Introduction: A 32 year old female presented with long standing lower back pain. She had a history of a road traffic accident four years prior which lead to a splenectomy. Over the next few years she had intermittent episodes of increasing pain in the lower back, groin and right leg. These symptoms were attributed to the RTA and she was given a course of physiotherapy.

There were no focal neurological signs and lumbar spine movements were normal. However, she did have some pain inhibition in her proximal muscles and difficulty weight bearing in her leg.

Lumbar spine x-rays showed grade II spondylolytic spondylolisthesis at L5/S1. MR scan of the lumbar spine confirmed this with some facet joint degeneration. She was sent back for more physiotherapy.

Six months later she presented with increasing pain in the right hip and a lump in the right groin. Subsequent MR scan showed a large 20cm x 15cm x 10cm lobulated soft tissue mass within the right obturator space extending through the foramen to lie in the adductor space with infiltration into right hip joint. She had a radical resection but later developed lung metastases.

Conclusion: This case highlights the difficulties in diagnosing pelvic soft tissue tumours especially with lower back pain. We should have a high index of suspicion in all young patients who present with intermittent flare up of groin/pelvic pain and no sign of inflammatory pathology. Once cutaneous signs appear it is often too late.