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DOI: 10.1016/j.jhsb.2005.05.002
Giant Lipomatous Tumours of the Hand and ForearmFrom Robert Jones and Agnes Hunt Orthopaedic Hospital, Oswestry, Shropshire SY10 7AG, UK Correspondence: Mr WP Cool, MD MMedSc(Res) FRCS(Ed) FRCS(Orth), Consultant Orthopaedic & Oncological Surgeon, Robert Jones & Agnes Hunt Orthopaedic Hospital Oswestry, Shropshire SY10 7AG, UK. Tel.: +44 1691 404088/+44 1691 404107; fax: +44 1691 404268. E-mail: paul.cool{at}rjah.nhs.uk
This study examines the presentation, management and outcomes of a series of 10 patients with giant lipomatous tumours (defined as greater than 5 cm diameter) of the hand and forearm who presented to our orthopaedic oncology service. All patients underwent local staging and were discussed at our multidisciplinary tumour meeting prior to definitive surgery. In all cases, neurovascular structures required mobilization in order to excise the tumour. Seven of the tumours were benign lipomas and one was a neural fibrolipoma. The other two were well differentiated lipoma-like liposarcomas/atypical lipomatous tumours. Giant lipomas and well differentiated lipoma-like liposarcomas/atypical lipomatous tumours of the hand and forearm present infrequently and a multidisciplinary approach is recommended in the investigation and surgical management of these patients.
Key Words: lipoma • liposarcoma • hand
Lipomas are the single commonest soft tissue tumour (McGee et al., 1992). However, their presentation in the hand and forearm is infrequent. Reports in the literature are of individual cases and small series (Babins and Lubahn, 1994; Bui-Mansfield et al., 2002; Fitzgerald et al., 2002; Leffert, 1972; Oster et al., 1989; Yamamoto et al., 2001) and there are few reports of giant lipomas in this location (Huntley and McEachan, 2004). They are generally slow growing and painless which explains their, often, large size at presentation, particularly if deeply located. Liposarcomas are the most common soft tissue sarcomas (Dei Tos, 2000). The largest subgroup is the well-differentiated liposarcoma which accounts for 40–45% of cases (Laurino et al., 2001). The purpose of this paper is to report our experience of the treatment of giant lipomatous tumours of the hand and forearm, emphasising the need for multi-disciplinary investigation and their subsequent management by surgery.
The Department of Musculoskeletal Oncology at the Robert Jones and Agnes Hunt Hospital, Oswestry, has a database with prospective collection of data for all patients referred with benign or malignant tumours. All patients who presented with lipomatous tumours of the hand and forearm which had a diameter greater than 5 cm during 2001–2003 were included in this study. Ten patients were identified and their presentations, clinical findings, investigations, operative details, outcomes and follow-up were reviewed.
All 10 patients with lipomatous tumours of the hand or forearm had them excised. There were six women and four men, with an mean age of 57 years. Five patients had tumours located in the hand, two in the palm, two in the thenar eminence and one in the hypothenar eminence. Two tumours were in the distal forearm and three in the proximal forearm. The duration of symptoms in all cases was between 1 year and 5 years. Six presented with a painless swelling and examination of these patients revealed a large, deep, mass which was not tender. One patient, with a lipoma in the thenar eminence, complained of mild discomfort with activity and examination revealed a large swelling in the first web space. Two patients presented with parasthesiae in the territory of the median nerve and both had a positive Tinel’s test over the swelling. One patient with a proximal forearm tumour presented with weakness of her hand. Examination revealed an obvious swelling in the extensor compartment and a posterior interosseous nerve palsy (Fig 1).
All patients underwent standard radiographs of their tumour and nine had an MRI scan. Two patients had an open biopsy as the MRI suggested a liposarcoma. The MRI scan was not tolerated by one patient, so a combination of a CT scan and an open biopsy was performed. All patients were discussed before surgery at our Multidisciplinary Tumour Meeting, which comprised Orthopaedic Oncological Surgeons, Musculoskeletal Radiologists and a Musculoskeletal Pathologist. All the surgical excisions were performed jointly by an Oncological Orthopaedic Consultant and a Consultant Hand Surgeon. In all cases, neurovascular structures required mobilization in order to excise the tumour. In one case, in the distal forearm, resection was more difficult as the mass was adherent to the median nerve. In the patient with the posterior interosseous nerve palsy, the nerve had been stretched and deflected by the lipoma, and, in one part, was actually running into the lipoma. Only 90% of this lipoma could be removed with preservation of the nerve. Seven patients with benign lipomas underwent marginal excisions and the eighth, as described above, underwent intralesional excision. The well differentiated lipoma-like liposarcomas/atypical lipomatous tumours were both located in the hand, one having presented with median nerve parasthesiae and the other as a painless swelling. One of these patients had an MRI scan which showed some heterogeneity, which was suggestive of a sarcoma. Open biopsy confirmed the diagnosis. Complete excision of this tumour required division of the radial digital artery and nerve of the ring finger and excision of the A1 and A2 pulleys of the flexor sheath of this finger. The second patient with a well differentiated lipoma-like liposarcomas/atypical lipomatous tumour also had a MRI scan preoperatively. This suggested a benign lipoma (Fig 2). She underwent a marginal excision without sacrifice of any neurovascular structures (Fig 3).
Postoperatively, five patients complained of some dysaesthesiae. These settled in four cases but persisted in the patient who had required division of the digital nerve. One patient with a lipoma of the hand experienced some scar tenderness which settled. The patient with the posterior interosseous nerve palsy had improvement of the weakness and was able to return to piano playing. All of the other patients returned to normal function of the involved limb. The average follow-up from surgery has been 36 (range 24–47) months, during which no recurrences have been observed. The patient with the intralesional excision has been followed for 2 years with no clinically detectable recurrence. All the specimens were examined histologically by a specialist Orthopaedic Pathologist. Seven tumours were benign lipomas, of which two were intramuscular and one was a neural fibrolipoma. The other two were well differentiated lipoma-like liposarcomas/atypical lipomatous tumours. The size of tumours varied by location, with those in the hand being the smallest and those in the distal forearm being the largest. The two well differentiated lipoma-like liposarcomas/atypical lipomatous tumours were of similar size to the benign lipomas (Table 1).
Lipomas consist of mature fat cells which may occur in a subcutaneous, intramuscular or intermuscular location. Local recurrence is uncommon after marginal excision. The term well-differentiated liposarcoma has been used to describe low-grade lipomatous neoplasms with a propensity for local recurrence (Kooby et al., 2004). They tend to occur with equal frequency in the retroperitoneum and the limbs and less frequently in the paratesticular region and the mediastinum. Their peak incidence is between the fifth and the seventh decades (Laurino et al., 2001). Well differentiated lipoma-like liposarcomas/atypical lipomatous tumours are one of the more common subtypes of well differentiated liposarcomas. It is doubtful whether liposarcomas ever arise in pre-existing lipomas (Contran et al., 1989). Giant lipomas and well differentiated lipoma-like liposarcomas/atypical lipomatous tumours of the hand and forearm are rare. Any soft tissue lump which is greater than 5 cm should be considered malignant until proved otherwise (Johnson et al., 2001). Good results can be obtained with surgical treatment, but, as with large tumours located elsewhere, these require a thorough preoperative assessment. In our series, MRI was used for local staging in four of the five cases as this has been shown to be an accurate diagnostic technique for patients who present with a palpable mass of the hand and wrist. A previous analysis of 134 cases revealed that MRI gave the correct diagnosis in 94% of instances (Capelastegui et al., 1999). In our series, open biopsy was only performed if MRI was not tolerated or was not diagnostic. The degree of heterogeneity of the tumour can be seen on MRI and this provides an estimate of the risk of recurrence. The greater the heterogeneity, the greater the risk of local recurrence. Biopsy can be helpful in local staging. However, sampling error occurs. All areas of radiological concern also require either needle or open biopsy. Image guided biopsy allows areas of radiological concern to be targeted and we would recommend ultrasound or CT guided biopsy. If the imaging appearances are completely benign (homogenous), then biopsy of the tumour is generally unhelpful. It is, therefore, reasonable, after discussion at a multidisciplinary meeting and explaining the risks to the patient, to proceed to excision. We believe that discussion of each case at a multidisciplinary team meeting is essential in deciding the most appropriate treatment. Marginal excision is usually the procedure of choice for both lipomas and well differentiated lipoma-like liposarcomas/atypical lipomatous tumours. However, if imaging or biopsy show features of a more aggressive pathology, then a more radical approach may be required. It is essential to estimate the risk of local recurrence and balance this against the likely loss of function. The vast majority of these tumours have no, or only a very low, metastatic potential, so extensive surgery can usually be avoided. Although a conservative approach could be offered, its risks should be considered and discussed with the patient. It is obviously important when considering this treatment option to have a histological diagnosis. The risks of surgery relate largely to the location of the tumour and include contractures, infection, nerve damage, scar sensitivity and complex regional pain syndrome. We believe that it is important that this surgery is performed jointly by hand and tumour surgeons, whenever possible, so that the principles of each subspeciality are applied to give the patient the best possible outcome. In respect of this principle and with respect to posterior interosseous nerve compression caused by lipomas, Fitzgerald et al. (2002) reported five cases and recommended Henry’s approach for excision. This was used in our patients and proved satisfactory.
Received for publication September 27, 2004. Accepted for publication May 3, 2005.
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Abstract
INTRODUCTION
