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Nerve tumours in the upper limb a clinical review
Nerve Tumours in The Upper Limb A Clinical Review B. J. HOLDSWORTH From The University Hospital, Queen’s Medical Centre, Nottingham Tumours arising from the peripheral nerves in the upper limb are rare. This paper reviews eighteen such cases and the clinical outcome. Problems of pre-operative diagnosis are emphasized. Tumours of the nerves are unusual and among the less common group of swellings in the upper limb or hand. Stack (1960) found eight out of three hundred cases. Strickland (1977) quote rates of less than five per cent of all hand tumours. Sir Herbert Seddon (1972) pointed out that less than careful excision of such lumps unsuspecting that major nerve trunks might be involved can result in disastrous loss of function. True neoplasms of nerves are less common in the hand than post-traumatic hyperplasia. There are basically two benign tumours and various types of sarcomatous lesion. The commonest type of benign tumour arises from the nerve sheath, and has been variously named neurilemmoma, Schwannoma, ‘palisaded’ neurinoma and perineural fibroma (Figure 1). The author prefers the term neurilemmoma and this term will be used in this review. They arise eccentrically and are said to be readily excisable without damage to the underlying nerve trunk (Phalen, 1976). They may be recurrent and multiple (Cavanagh, 1977). The other benign tumours are neurofibromata which may be solitary or multiple (Figure 2). When multiple, other features confirming von Recklinghausen’s disease may be present. Solitary neurofibromata were particularly common in the hand in Seddon’s series (1972). Unlike neurilemmomata, neurofibromata arise actually within the fasciculi and thus excision must lead to some damage to the parent nerve. Neurofibrosarcomata occur particularly in von Recklinghausen’s disease-100 out of 129 malignant nerve tumours (Cutler, 1936). This paper reports a review of eighteen patients treated in Nottingham. The aims are to clarify the mode of presentation in the hopes of facilitating diagnosis, and to review the clinical outcome of surgery. Clinical Material and Methods Patients were collected from several sources in Nottingham, viz. a special case file in the hand department, the computerised index in the pathology department of the new University Hospital, and the card index at the older General Hospital pathology department. Any case in which von Recklinghausen’s disease was obvious clinically at initial presentation was Received for publication July, 1984. B. J. Holdsworth, B.Sc., F.R.C.S., Harlow Nom.
236
Wood
Orthopaedic
Hospital,
Near
Fig. 1
Neurilemmona This photomicrograph shows an example of neurilemmoma with a well circumscribed capsule (C) and a non-diffuse arrangement of spindle cells into nodular dense clumps known as Antoni type A (A). There is little intervening loose Antoni type B tissue in this example (B). There is occasional palisading (P). (H and E section mag. x 35).
excluded, as the diagnosis was not in question. This left a total of eighteen patients in which case notes were available. One patient had died. Fifteen patients were re-examined for this review, and follow-up varied between four months and ten years (mean 4.7 years). Of the original eighteen patients, twelve females were aged
Mansfield,
THE JOURNAL
OF HAND SURGERY
NERVE TUMOURS
upper limb. Fourteen out of eighteen were in the hand or wrist rather than more proximally. Twelve were on the volar aspect of the limb. Major nerve trunks were involved in four cases. Mode of presentation. Only two of eighteen mentioned any paraesthesia or tingling. In nine cases the lump was tender or painful. One case presented as trigger finger and has been reported (Robb, 1978). A simple lump was thus the commonest presenting complaint. In thirteen cases the lump was less than 2 ems in size. Several were considerably larger, the biggest, a neurilemmoma was 7 ems x 4 ems. Length of history prior to presentation was from two months to nine years. In eight cases it was simply said to have been present perhaps slowly growing for years. Pre-operative diagnosis. Five cases were correctly suspected to be some form of nerve tumour, but in two malignancy was feared. In nine cases the wrong diagnosis was made. Five were thought to be simple ganglia, one enchondroma, one glomus tumour, and two lipomata. The rest did not specify.
Fig. 2
An example of neurofibroma showing irregularly arranged spindle cells. There is no palisading and the general appearance is homogeneous. The point of entry of the normal nerve trunk into the tumour is demonstrated (N). The tumour is well encapsulated by nerve sheath tissue (S). (H and E section mag. x 35).
Histological diagnosis showed that there were nine neurilemmomas and eight solitary neurofibromas. One case, thought to be a neurofibrosarcoma on initial biposy of a lump on the median nerve in the forearm, recurred after wide excision and interfascicular grafting. The recurrence was treated by above-elbow amputation, Histology of the resected specimen showed poorly differentiated spindle cells indistinguishable from fibrosarcoma without obvious neural elements. This patient subsequently died aged seventy-eight years due apparently to carcinoma of the breast.
between ten and sixty-eight years (average forty-four years), and six males were aged between twelve and fifty-nine years (average thirty-one years). The preoperative diagnosis was noted when mentioned in the case notes.
Status at review Recurrence-One patient had a definite recurrent lump and asked to have it re-excised. Another patient had a dubious thickening in part of a scar which she thought was a recurrence, and a third patient who failed to reattend was said by her general practitioner to have complained of a recurrence.
At clinical review the patients were questioned carefully concerning any residual symptoms and examined for recurrence, other lumps, cafe au lait patches and objective residual neurological loss. As several patients were examined at home, no special equipment was used for sensory testing, but routine clinical testing was carried out with cotton wool, a pin and a paper clip for static two-point discrimination.
Other lumps were discovered elsewhere in two patients which appeared to be similar in nature to the original. One gave ‘electric shocks’ when firmly pressed. No patient gave a family history of similar problems, and no patient had more than one or two cafe au lait spots above the waist.
Results Distribution. All the tumours in this series were in the
Neurological loss was noted in one patient prior to operation. Subjective hypoaesthesia in the patient with the largest neurilemmoma lead to the correct pre-
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B. J. HOLDSWORTH
operative diagnosis-by a general surgeon. At review, seven patients showed absolutely no loss of sensation or power to careful clinical testing. Minute patches of hypoaesthesia or negligible diminution of two-point discrimination were found in a further five patients.
Of the remaining three patients attending for review, one had a symptomatic recurrence with unpleasant hyperaesthesia and reduced two-point discrimination in the affected ring finger pulp. A man with a 7 ems x 4 ems neurilemmoma present for nine years had a persistent patch of hypoaesthesia, and could not tell rough from smooth material, but had well preserved two-point discrimination. Potentially the most disabled patient reviewed was a lady of thirty years who had a neurilemmoma excised (6 ems x 2lYz ems) at mid humeral level from the median nerve four years previously. Three bundles had to be excised and the gap tilled by interfascicular grafts from the sural nerve. In fact her recovery was very good with normal light touch and pin prick. Two-point discrimination was 6-7 mms in the affected thumb. She failed to recover stereognosis and was unable to identify coins correctly.
Discussion The histological and topographic scatter of nerve tumours in this small series are fairly typical (Cutler, 1936; Strickland, 1977; Geschickter, 1935). Sadly our inability to predict the nature of these lesions preoperatively in two-thirds of cases also seems to be typical (Bogumill, 1975). Realisation that neurological loss is not a feature may perhaps help in this regard (Phalen, 1976). The tumours typically displaced other bundles without pressure effects. A very slowly growing lump which perhaps gives rise to tingling when pressed gives the best clue in the absence of other stigmata of von Recklinghausen’s disease. Size is no clue to diagnosis, but rapid growth and apparent or possible involvement of major trunks should warn of possible malignancy. This small group of patients require urgent expert exploration under the operating microscope with facilities for possible nerve grafting procedures.
Classification of the various forms of malignancy involving peripheral nerves is beyond the scope of this brief report. Suffice it to say that reviewing the old literature proves unhelpful due to lack of uniform terminology. They are usually grouped with fibrosarcomas. There are also several other neural swellings which are probably not true neoplasms-for example fatty infiltration. 238
In general our patients were pleased with their treatment despite the fact that patchy hypoaesthesia resulted in one-third about of cases following excision. Surprisingly there was no obvious difference in this series in the degree of neurological loss following excision between neuromata or neurilemmomata. This was presumably because the neuromata by chance involved small superficial nerves, whose territory was taken over by surrounding intact nerves.
Leung (1981) suggested that to achieve better results more supervision and attention were required in dealing with hand tumours. The rare lumps involving nerves continue to escape pre-operative diagnosis. Luckily the nerves involved are usually very small and no serious neurological loss follows excision. The possibility of major nerve trunk involvement should always be borne in mind when soft lumps are being removed from the hand or more proximally in the arm.
Acknowledgements I am grateful to Mr. N. J. Barton for allowing me to review his patients, and to Dr. Stevens and Mr. Brackenbury of the Pathology Department, University Hospital, Nottingham for their help. Mrs. S. A. Blythe gave secretarial assistance.
References BOGUMILL, G. P., SULLIVAN, D. J., and BAKER, G. I. (1975). Tumors of the Hand. Clinical Orthopaedics and Related Research, 108: 214-222. CAVANAGH, N. P. C. and PINCOTT, J. R. (1977). Ulnar nerve tumours of the hand in childhood. Journal of Neurology, Neurosurgery and Psychology, 40: 795-800. CUTLER, E. C. and GROSS, R. E. (1936). The Surgical Treatment of Tumors of the Peripheral Nerves. Annals of Surgery, 104: 436-452. GESCHICKTER, C. F. (1935). Tumors Of The Peripheral Nerves. The American Journal of Cancer, 25: 377-410. LEUNG, P.C. (1981). Tumours of Hand. The Hand, 13: 169-176. PHALEN, G. S. (1976). Neurilemmomas of the Forearm and Hand. Clinical Orthopaedics and Related Research, 114: 219-222. ROBB, J. E. (1978). Trigger Finger Due to Neurilemmoma in the Carpal Tunnel. The Hand, 10: 299-301. SEDDON, H. J. Surgical Disorders of Peripheral Nerves. Edinburgh, London, Churchill Livingstone. Baltimore, Williams and Wilkins 1972: ~158. STACK, H. G. (1960). Tumours of the Hand. British Medical Journal, 1: 919-922. STRICKLAND, J. W. and STEICHEN, J. B. (1977). Nerve tumors of the hand and forearm. The Journal of Hand Surgery, 2: 285-291. THE JOURNAL OF HAND SURGERY
236
Wood
Orthopaedic
Hospital,
Near
Fig. 1
Neurilemmona This photomicrograph shows an example of neurilemmoma with a well circumscribed capsule (C) and a non-diffuse arrangement of spindle cells into nodular dense clumps known as Antoni type A (A). There is little intervening loose Antoni type B tissue in this example (B). There is occasional palisading (P). (H and E section mag. x 35).
excluded, as the diagnosis was not in question. This left a total of eighteen patients in which case notes were available. One patient had died. Fifteen patients were re-examined for this review, and follow-up varied between four months and ten years (mean 4.7 years). Of the original eighteen patients, twelve females were aged
Mansfield,
THE JOURNAL
OF HAND SURGERY
NERVE TUMOURS
upper limb. Fourteen out of eighteen were in the hand or wrist rather than more proximally. Twelve were on the volar aspect of the limb. Major nerve trunks were involved in four cases. Mode of presentation. Only two of eighteen mentioned any paraesthesia or tingling. In nine cases the lump was tender or painful. One case presented as trigger finger and has been reported (Robb, 1978). A simple lump was thus the commonest presenting complaint. In thirteen cases the lump was less than 2 ems in size. Several were considerably larger, the biggest, a neurilemmoma was 7 ems x 4 ems. Length of history prior to presentation was from two months to nine years. In eight cases it was simply said to have been present perhaps slowly growing for years. Pre-operative diagnosis. Five cases were correctly suspected to be some form of nerve tumour, but in two malignancy was feared. In nine cases the wrong diagnosis was made. Five were thought to be simple ganglia, one enchondroma, one glomus tumour, and two lipomata. The rest did not specify.
Fig. 2
An example of neurofibroma showing irregularly arranged spindle cells. There is no palisading and the general appearance is homogeneous. The point of entry of the normal nerve trunk into the tumour is demonstrated (N). The tumour is well encapsulated by nerve sheath tissue (S). (H and E section mag. x 35).
Histological diagnosis showed that there were nine neurilemmomas and eight solitary neurofibromas. One case, thought to be a neurofibrosarcoma on initial biposy of a lump on the median nerve in the forearm, recurred after wide excision and interfascicular grafting. The recurrence was treated by above-elbow amputation, Histology of the resected specimen showed poorly differentiated spindle cells indistinguishable from fibrosarcoma without obvious neural elements. This patient subsequently died aged seventy-eight years due apparently to carcinoma of the breast.
between ten and sixty-eight years (average forty-four years), and six males were aged between twelve and fifty-nine years (average thirty-one years). The preoperative diagnosis was noted when mentioned in the case notes.
Status at review Recurrence-One patient had a definite recurrent lump and asked to have it re-excised. Another patient had a dubious thickening in part of a scar which she thought was a recurrence, and a third patient who failed to reattend was said by her general practitioner to have complained of a recurrence.
At clinical review the patients were questioned carefully concerning any residual symptoms and examined for recurrence, other lumps, cafe au lait patches and objective residual neurological loss. As several patients were examined at home, no special equipment was used for sensory testing, but routine clinical testing was carried out with cotton wool, a pin and a paper clip for static two-point discrimination.
Other lumps were discovered elsewhere in two patients which appeared to be similar in nature to the original. One gave ‘electric shocks’ when firmly pressed. No patient gave a family history of similar problems, and no patient had more than one or two cafe au lait spots above the waist.
Results Distribution. All the tumours in this series were in the
Neurological loss was noted in one patient prior to operation. Subjective hypoaesthesia in the patient with the largest neurilemmoma lead to the correct pre-
VOL. 10-B No.
2 JUNE 1985
237
B. J. HOLDSWORTH
operative diagnosis-by a general surgeon. At review, seven patients showed absolutely no loss of sensation or power to careful clinical testing. Minute patches of hypoaesthesia or negligible diminution of two-point discrimination were found in a further five patients.
Of the remaining three patients attending for review, one had a symptomatic recurrence with unpleasant hyperaesthesia and reduced two-point discrimination in the affected ring finger pulp. A man with a 7 ems x 4 ems neurilemmoma present for nine years had a persistent patch of hypoaesthesia, and could not tell rough from smooth material, but had well preserved two-point discrimination. Potentially the most disabled patient reviewed was a lady of thirty years who had a neurilemmoma excised (6 ems x 2lYz ems) at mid humeral level from the median nerve four years previously. Three bundles had to be excised and the gap tilled by interfascicular grafts from the sural nerve. In fact her recovery was very good with normal light touch and pin prick. Two-point discrimination was 6-7 mms in the affected thumb. She failed to recover stereognosis and was unable to identify coins correctly.
Discussion The histological and topographic scatter of nerve tumours in this small series are fairly typical (Cutler, 1936; Strickland, 1977; Geschickter, 1935). Sadly our inability to predict the nature of these lesions preoperatively in two-thirds of cases also seems to be typical (Bogumill, 1975). Realisation that neurological loss is not a feature may perhaps help in this regard (Phalen, 1976). The tumours typically displaced other bundles without pressure effects. A very slowly growing lump which perhaps gives rise to tingling when pressed gives the best clue in the absence of other stigmata of von Recklinghausen’s disease. Size is no clue to diagnosis, but rapid growth and apparent or possible involvement of major trunks should warn of possible malignancy. This small group of patients require urgent expert exploration under the operating microscope with facilities for possible nerve grafting procedures.
Classification of the various forms of malignancy involving peripheral nerves is beyond the scope of this brief report. Suffice it to say that reviewing the old literature proves unhelpful due to lack of uniform terminology. They are usually grouped with fibrosarcomas. There are also several other neural swellings which are probably not true neoplasms-for example fatty infiltration. 238
In general our patients were pleased with their treatment despite the fact that patchy hypoaesthesia resulted in one-third about of cases following excision. Surprisingly there was no obvious difference in this series in the degree of neurological loss following excision between neuromata or neurilemmomata. This was presumably because the neuromata by chance involved small superficial nerves, whose territory was taken over by surrounding intact nerves.
Leung (1981) suggested that to achieve better results more supervision and attention were required in dealing with hand tumours. The rare lumps involving nerves continue to escape pre-operative diagnosis. Luckily the nerves involved are usually very small and no serious neurological loss follows excision. The possibility of major nerve trunk involvement should always be borne in mind when soft lumps are being removed from the hand or more proximally in the arm.
Acknowledgements I am grateful to Mr. N. J. Barton for allowing me to review his patients, and to Dr. Stevens and Mr. Brackenbury of the Pathology Department, University Hospital, Nottingham for their help. Mrs. S. A. Blythe gave secretarial assistance.
References BOGUMILL, G. P., SULLIVAN, D. J., and BAKER, G. I. (1975). Tumors of the Hand. Clinical Orthopaedics and Related Research, 108: 214-222. CAVANAGH, N. P. C. and PINCOTT, J. R. (1977). Ulnar nerve tumours of the hand in childhood. Journal of Neurology, Neurosurgery and Psychology, 40: 795-800. CUTLER, E. C. and GROSS, R. E. (1936). The Surgical Treatment of Tumors of the Peripheral Nerves. Annals of Surgery, 104: 436-452. GESCHICKTER, C. F. (1935). Tumors Of The Peripheral Nerves. The American Journal of Cancer, 25: 377-410. LEUNG, P.C. (1981). Tumours of Hand. The Hand, 13: 169-176. PHALEN, G. S. (1976). Neurilemmomas of the Forearm and Hand. Clinical Orthopaedics and Related Research, 114: 219-222. ROBB, J. E. (1978). Trigger Finger Due to Neurilemmoma in the Carpal Tunnel. The Hand, 10: 299-301. SEDDON, H. J. Surgical Disorders of Peripheral Nerves. Edinburgh, London, Churchill Livingstone. Baltimore, Williams and Wilkins 1972: ~158. STACK, H. G. (1960). Tumours of the Hand. British Medical Journal, 1: 919-922. STRICKLAND, J. W. and STEICHEN, J. B. (1977). Nerve tumors of the hand and forearm. The Journal of Hand Surgery, 2: 285-291. THE JOURNAL OF HAND SURGERY