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.

Introduction: Pathological fracture of the clavicle is not such a rare condition. By definition the fracture occurs either without or with minimal trauma. We present a case of pathological fracture where there was a definite history of trauma; clouding the true malignant diagnosis.

Case Report: A 73 year old man first presented to the Accident & Emergency Dept. of our hospital with a history of having fallen on to his left shoulder after throwing a piece of concrete, and to have developed swelling and pain around the shoulder immediately following the fall. On examination the main findings were swelling and bony tenderness over the proximal clavicle and inability to use his left shoulder due to pain. X-ray showed a fracture at the junction between the proximal 1/3 and distal 2/3 of the left clavicle, and he was given a broad arm sling for support and a one week appointment for review in the fracture clinic.

Two clinic appointments later he was still complaining of pain. X-rays taken at that time showed what appeared to be some evidence of callus formation at the fracture site. Six weeks later he had clinical and radiological signs of what appeared to be “huge callus formation”. He was given a 3 month appointment for what was expected to be a final review.

Before his next fracture clinic appointment, however, he became jaundiced and complained about this to his GP who felt it was obstructive jaundice and referred him to the physicians who admitted him to the hospital, and began to investigate him as to the cause of the jaundice. These investigations included an Ultrasound Scan of the abdomen which showed a bulky head of pancreas with biliary and pancreatic ductal dilatation; and a CT scan of the upper abdomen which showed the presence of a cystic mass within the caudate lobe of the liver. Soft tissue vascular encasement around the portal vein and hepatic artery were reported as in keeping with malignant infiltration. Extensive tumour was present within the retroperitoneum involving local vascular structures.

He came down to the fracture clinic for his next clinic appointment from the ward. At this point he was very ill, deeply jaundiced and frail. The swelling of the clavicle was the size of a large orange, firm to touch with dilated veins. X-ray at this point showed complete radiological destruction of the medial 1/3 of the left clavicle. At this point palliative care was the mainstay of his management.

A week later the chest x-ray report came back as showing collapse of the left upper lobe with whiteout appearance and bulky hilum indicating an underlying bronchogenic carcinoma.

Three days later, almost 5 months after initial presentation following a fall, this patient finally succumbed to his disease.

Conclusion: This patient presented with a simple fracture and was given the standard treatment for the condition. However because his treatment was compartmentalized, i.e., orthopaedics treating the orthopaedic condition, radiology doing x-rays, and physicians managing the jaundice; it took some months before the different pieces of the jig-saw puzzle were put together and the malignant diagnosis arrived at.

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.

Background – Limb salvage surgeries for soft tissue sarcomas (STS) leads to significant amount of morbidity and fear psychosis among the affected individual. We looked into the potential complications and its implications.

Patients & Methods – Retrospective review of 96 patients over a period of 5 years from 1999 to 2004 with a minimum follow up of 8 months. Complete data of every patient was reviewed with particular emphasis on post op complications.

Results – 72 patients had STS in their lower limbs while the rest had in the upper limbs. 53 tumours were either at the level of knee or above the knee while 19 were found below the knee. The anterior compartment of the thigh was the most common site (13) with Vastus Lateralis being the commonly involved muscle. Liposarcoma was the commonest tumour (22) followed by Leiomyosarcoma (19), Fibro sarcoma (14), Synovial Sarcoma (12), Rhabdomyosarcoma (10), Histiocytoma (9) and other rare sarcomas. 61 patients had wide local excision, 17 had radical excision and 12 had marginal excision. 6 patients had palliative treatment only due to extensive metastasis (mets). 38 patients had post op radiotherapy. The average interval between presentation and definitive treatment was 28 days. 22 of the 71 patients (31 %) with no mets pre op. developed mets during follow up. The commonest problem in the post op period is inadequate skin cover which required skin grafting in 17 cases and flap cover in 3. Three of the skin grafts got necrosed due to radiotherapy. Local recurrence was the next most common complication (12 patients) along with equal number of patients with lymphaedema. Seroma/Haematoma was noted in 8 patients but none required drainage. There were 7 cases of superficial infection and one deep infection. Fixed Flexion Deformity at knee of > 10 0 was noted in 5 cases. Intractable neurological pain was noted in 3 cases involving the upper limb and one involving the lower limb. 3 developed skin rashes after radiotherapy. 2 had ulnar nerve palsy and one had foot drop. DVT occurred in 3 patients. Stump neuroma created problems in 2 cases and 2 patients ended up with below knee amputation. Another notable feature was the fear psychosis among the patients about benign swellings that were present in other parts of their body either before or after surgery which resulted in 11 surgeries but none proved to be malignant. As on 31-3-2005, 39 were dead and the rest were alive. The average life span of the patients who died from the time of confirmed diagnosis was 23 months.

Discussion – There is a plethora of complications that can occur following extensive resection of huge tumours in the extremities. This may involve sacrificing neuro-vascular structures to achieve adequate clearance. In spite of this, there is evidence of frequent local recurrences and distant metastatic spread. There is still some lack of awareness among the public about innocuous looking swellings and they present late with distant metastasis when the prognosis becomes poor.

Conclusion –

Significant complications can occur after sarcoma surgeries.

Background: To look into the incidence of lymphatic spread in Soft Tissue Sarcomas (STS) of the extremities and its relevance to the patient’s prognosis.

Patients & Methods: Retrospective review of 96 patients over a period of 5 years from 1999 to 2004 with a minimum follow up of 8 months. Complete data of every patient was reviewed with particular emphasis on lymph node and distant metastasis. All the patients were seen by one consultant and the histology reports were given by one Pathologist. 2 consultant radiologists were also involved in giving reports.

Results: There were 39 males and 57 females with an average age of 51 years. The average duration of swelling at presentation was 6 months. There was a strong family history of cancer in first degree relatives in 23 patients (24%). Liposarcoma was the commonest tumour (22) followed by Leiomyosarcoma (19), Fibro sarcoma (14), Synovial Sarcoma (12), Rhabdomyosarcoma (10), Histiocytoma (9) and other rare sarcomas. The Trojani grade of the tumours was Grade 1 = 36, Grade 2 = 39 & Grade 3 = 21. No metastasis (mets) were found during pre op. screening in 71 patients (74%) while 11 (12%) had lung mets, 9 (10 %) had lymph node involvement and 5 had liver involvement (4 %). 4 had multiple organ involvement on presentation. All except 6 patients had either wide local or radical excision of the tumour. The average interval between presentation and definitive treatment was 28 days. 9 of the patients with lymph node mets underwent nodal clearance during primary surgery. 2 turned out to be reactive hyperplasia while 7 proved to be malignant. 22 of the 71 patients (31 %) with no mets pre op. developed mets during follow up at an average duration of 11 months (11 to lungs, 8 to regional lymph nodes, 2 to liver and one to bone. 4 patients had multiple mets) In addition to this, there was local recurrence in 12 patients of whom 9 had incomplete excision during primary surgery. Of the total 15 patients who had proven lymph node mets, 5 came from Rhabdomyosarcoma, 4 from Leiomyosarcoma, 3 each from Lipo & Synovial sarcoma. The average life span in patients with lymph node involvement was 13 months in total when compared to 31 months for others. As on 31-3-2005, 39 were dead and the rest were alive. The average life span of the patients who died from the time of confirmed diagnosis was 23 months. The average life span for Rhabdomyosarcoma was 8 months, Histiocytoma was 12 months, Liposarcoma was 19 months, Leiomyosarcoma was 28 months and Synovial sarcoma was 36 months. Patients with Trojani grade 3 STS died at an average of 9 months when compared to 38 months for grade 1.

Discussion: There is a 16% spread to regional lymph nodes. It appears that lymph node involvement is indicative of micrometastatic disease elsewhere. Excision of the lymph nodes during primary surgery did not improve the life expectancy.

Conclusion:

Lymph node involvement is a poor prognostic sign

Purpose of Study – The unusual presentation of this case posed a diagnostic dilemma between a chronic haematoma and soft tissue sarcoma even after full investigation and biopsy. Salient points to differentiate between the two are discussed along with literature review.

Case Report – A 61 year old gentleman presented with sudden increase in size of an already existing swelling over the mid third of right leg associated with throbbing pain & foot drop of 4 months duration. There was no recent history of trauma or bleeding abnormalities but there was a vague history of injury to his leg during his late teens. Clinical signs showed features suggestive of malignancy with engorged veins and diffuse margins with complete foot drop (Fig 1 & 2). X-rays showed calcifications within the substance of the swelling along with proximal tibiofibular synostosis (Fig 3). MRI scan revealed a well encapsulated mass between the peroneal muscles mechanically compressing the common peroneal nerve (Fig 4). Trucut biopsy showed cholesterol clefts and areas of dystrophic calcification characteristic of chronic haematoma (Fig 5). Patient successfully underwent enucleation of the swelling along with cutaneofascial suture to obliterate the dead space leading to complete recovery of foot drop. Biopsy confirmed a Chronic Haematoma.

Discussion – Reid et al first used the term chronic expanding haematoma for haematomas that persisted and increased in size more than a month after the initiating haemorrhage. The cause of initial haemorrhage is most commonly trauma which results in displacement of skin and subcutaneous fatty tissue from more deeply located fixed fascia with formation of blood filled cysts surrounded by dense fibrous tissue. Factors in the blood-clotting cascade are said to be associated with an inflammatory reaction leading to additional bleeding from fragile capillaries and thus to additional inflammation, hence setting up a self-perpetuating process.

Although the MRI & biopsy results in this case were reassuring, the clinical scenario of sudden foot drop with increase in pain point more towards a malignant process rather than a benign condition. Some salient points to differentiate the two include that sarcoma have no history of trauma and the duration of symptoms is longer in haematoma than sarcoma. Also, sarcomas usually involve deeper structures while haematoma occur in superficial layers. It should also be noted that several soft tissue sarcoma themselves commonly reveal haemorrhagic or cystic changes. Other differential diagnosis includes myositis ossificans and tumoral calcinosis.

Conclusion – It is difficult to differentiate between chronic haematoma and soft tissue sarcoma based on clinical findings alone. X-ray and biochemical tests are always essential to rule out any fracture or bony mass but MRI is the gold standard and biopsy is the only way to rule out a malignant tumour. Surgical excision of the swelling including the fibrous pseudocapsule along with cutaneofascial suture to obliterate the dead space is the treatment of choice for chronic haematoma because aspiration of the fluid or incomplete excision could lead to recurrence, continued growth or a chronic draining sinus with or without infection.