This case report presents a 56-year-old man with right upper limb weakness which arose 22 years after initial local radiation treatment for a grade III fibrosarcoma. Nerve conduction studies revealed impairment of all three major upper limb nerves compared with the left, with particular impairment of the median and ulnar nerves in the most fibrotic area that had been irradiated. In addition, the patient received multiple courses of chemotherapy. The occurrence of radiation-induced brachial plexopathy should be considered in patients presenting with limb pain or weakness even many years after radiation therapy

Al-Benna, Sammy

Schubert, Cornelius

Steinau, Hans Ulrich

Steinstraesser, Lars

English

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Case report
Open Access
Brachial neuropathy 22 years after radiation therapy
for fibrosarcoma: a case report
Sammy Al-Benna, Cornelius Schubert, Hans Ulrich Steinau
and Lars Steinstraesser*
Address: Department of Plastic and Reconstructive Surgery, Soft Tissue Tumour Reference Centre, BG University Hospital Bergmannsheil,
Ruhr University Bochum, Buerkle-de-la Camp Platz 1, 44789 Bochum, Germany
Email: SAB - sammy.al-benna@ruhr-uni-bochum.de; CS - cornelius-schubert@gmx.de; HUS - hans-ulrich.steinau@bergmannsheil.de;
LS* - lars.steinstraesser@ruhr-uni-bochum.de
*Corresponding author
Received: 20 July 2009 Accepted: 25 August 2009 Published: 15 September 2009
Cases Journal 2009, 2:6838 doi: 10.4076/1757-1626-2-6838
This article is available from: http://casesjournal.com/casesjournal/article/view/6838
© 2009 Al-Benna et al.; licensee Cases Network Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
This case report presents a 56-year-old man with right upper limb weakness which arose 22 years
after initial local radiation treatment for a grade III fibrosarcoma. Nerve conduction studies revealed
impairment of all three major upper limb nerves compared with the left, with particular impairment
of the median and ulnar nerves in the most fibrotic area that had been irradiated. In addition, the
patient received multiple courses of chemotherapy. The occurrence of radiation-induced brachial
plexopathy should be considered in patients presenting with limb pain or weakness even many years
after radiation therapy.
Introduction
1-3% of all adult soft tissue sarcomas are fibrosarcomas.
The fact that soft tissue sarcomas constitute 1% of all adult
malignancies underlines the rareness of fibrosarcomas.
They most commonly occur in middle-aged and older
adults with no sex-related differences. There are no specific
predisposing factors, although some fibrosarcomas may
develop after previous therapeutic irradiation. The
tumours tend to be located in the deep soft tissues of
extremities, trunk, head and neck and classically present as
a well defined mass and cause local symptoms due to
compression and invasion of local structures. The major
prognostic factors include grade, tumour size and depth
from the surface. 9-63% of all patients suffer from
metastases which commonly occur in lungs and bones
and rarely in lymph nodes. The overall 5-year-survival rate
ranges between 39% and 54% and this emphasises the
aggressiveness of this tumour.
Case presentation
A 56-year-old retired Caucasian male presented in 2008
with weakness and discomfort which progressed over
3 months to involve his entire right forearm and hand.
The symptoms were present at rest or with activity, and
there were no exacerbating or releiving factors. He noted
intermittent paresthesias of his entire hand with sensory
loss in the lateral 3 and a half fingers of his right hand and
slowly progressing arm weakness.
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In 1985 he received neoadjuvant chemotherapy with
Farmorubicin (total dose: 1200 mg) and Holoxan (total
dose: 100 g) prior to surgical ablation of a G3 fibrosar-
coma with clear margins in his right arm. In 1986 he
underwent postoperative radiotherapy to a dose of 60 Gy
in 30 fractions. In 1991, he developed a local recurrence
and again received neoadjuvant chemotherapy with
Farmorubicin (total dose: 1200 mg) and Holoxan (total
dose: 100 g) followed by radical surgical ablation. This
operation included partial resection of the biceps brachii,
brachialis and triceps brachii. He was treated with further
radiotherapy at single doses of 2 Gy up to a total dose of
20 Gy, followed by adjuvant chemotherapy with Farm-
orubicin (total dose: 300 mg) and Holoxan (total dose:
50 g). In 1997, he presented with a pathological fracture of
his right humerus which was treated with an intramedul-
lary nail.
Physical examination demonstrated slightly reduced radial
and ulnar pulses compared to the left. Findings on a
cranial nerve examination were normal with no Horner’s
syndrome. Motor examination showed generalised M3
motor function of his right forearm and hand in contrast
to normal M5 motor function of his contralateral upper
limb. There was non-pitting oedema throughout his right
forearm and dorsum of his hand. Biceps and triceps
reflexes were reduced. Findings on a sensory examination
demonstrated global superficial cutaneous S3 sensation in
the forearm and hand with S2 senation in the lateral three
and a half fingers of his hand and thenar eminence.
Electrophysiologic studies were performed to evaluate the
brachial plexus. Nerve conduction studies revealed
reduced amplitude of median, ulnar and radial nerves
compared with the left (Table 1). Needle electromyograhy
(Table 2) showed large polyphasic motor unit potentials
in the muscles innervated by the superior, middle and
inferior trunks of the brachial plexus with no abnormal
spontaneous activity or myokymic discharges. Magnetic
Figure 1. The clinical picture of patient’s right upper limb
showing marked changes in appearance including fibrosis of
the arm and oedema of the forearm 22 years after initial
radiotherapy.
Figure 2. Magnetic resonance imaging demonstrating showed
fibrotic infiltrate from radiotherapy.
Figure 3. 22 years after initial radiotherapy there is reduced
function of the right hand and particularly of the right thumb
which is unable to oppose against the little finger.
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resonance imaging (MRI) of the right arm showed fibrotic
infiltrate and volume loss in the right arm and nodular ill-
defined median, radial and ulnar nerves with no focal
masses, consistent with postradiation changes. The clinical
findings and supporting investigations indicated radia-
tion-induced neuropathy involving the median, radial and
ulnar nerves of the right upper limb. These injuries
occurred within the field of prior radiation therapy. The
patient was treated with compression therapy and
physiotherapy. Surgical decompression was discussed,
however, the patient elected to continue with conservative
treatment only.
Discussion
Radiation therapy is a common adjunctive modality used
in the treatment of soft tissue sarcomas after surgical
ablation. Although radiotherapy has shown benefit in
reducing sarcoma recurrence and improves survival, it has
Table 1. Nerve conduction studies: summary of sensory nerves
Nerve Site Variable Right Left Normal
Median Digit 2 Amplitude (μV) 13.1 22.7 > 15.0
Distal latency (ms) 3.4 2.8 < 3.6
Ulnar Digit 5 Amplitude (μV) 14.3 19.2 > 15.0
Distal latency (ms) 2.7 2.9 < 3.1
Radial Anatomical snuffbox Amplitude (μV) 20.9 NR > 14.0
Distal latency (ms) 4.1 < 2.7
Lateral antebrachial Forearm Amplitude (μV) NR 9.3
Distal latency (ms) 2.1
Medial antebrachial Forearm Amplitude (μV) NR NR
Distal latency (ms) NR
mV = millivolts, ms = milliseconds, m/s = metres per second, μV = microvolts, NR = no response.
Table 2. Electromyographic needle examination
Side Muscle Nerve Root INS FIBS PSW FAS AMP DUR CONFIGURATION REC PAT REC INT
R 1st Dor Int Ulnar C8-T1 Nml 1+ 2+ 0 Nml Nml Di/Tri Phasic Full Normal
R APB Median C8-T1 Nml 1+ 2+ 0 Nml Nml Di/Tri Phasic Full Normal
R Abd Dig Min Ulnar C8-T1 Nml 1+ 2+ 0 Nml Nml Di/Tri Phasic Discrete Dec
R Biceps Musc C5-6 Nml 0 1+ 0 Nml Nml Di/Tri Phasic Full Normal
R Brachialis Musc C5-6 Nml 0 0 0 Nml Nml Di/Tri Phasic Full Normal
R Cerv para Low Rami C6-7 Nml 0 0 0 Nml Nml Di/Tri Phasic Full Normal
R Cerv para Up Rami C5-6 Nml 0 0 0 Nml Nml Di/Tri Phasic Full Normal
R Deltoid Axilla C5-6 Nml 0 0 0 Nml Nml Di/Tri Phasic Full Normal
R Ext Car Rad Radial C7-8 Nml 2+ 2+ 0 Nml Nml Di/Tri Phasic Dec Normal
R Ext Dig Com Radial C7-8 Nml 1+ 2+ 0 Nml Nml Di/Tri Phasic Dec Normal
R Ext Ind Pro Radial C7-8 Nml 1+ 2+ 0 Nml Nml Di/Tri Phasic Dec Normal
R Flex Car Rad Median C6-8 Nml 2+ 3+ 0 Nml Nml Di/Tri Phasic Discrete Dec
R Flex Dig Prof Ulnar C8-T1 Nml 1+ 2+ 0 Nml Nml Di/Tri Phasic Full Nml
R Flex Car Uln Ulnar C8-T1 Nml 1+ 2+ 0 Nml Nml Di/Tri Phasic Full Nml
R Infraspinatus Supra C5-6 Nml 0 0 0 Nml Nml Di/Tri Phasic Full Normal
R Lat triceps Radial C6-7-8 Nml 0 0 0 Nml Nml Di/Tri Phasic Full Normal
R LongHdTricep Radial C6-7-8 Nml 0 0 0 Nml Nml Di/Tri Phasic Full Normal
R PalmarisLong Median C8-T1 Nml 1+ 1+ 0 Nml Nml Di/Tri Phasic Full Normal
R Pronator teres Median C6-7 Nml 2+ 2+ 0 Nml Nml Di/Tri Phasic Discrete Dec
R Rhomboids DorsS C5 Nml 0 0 0 Nml Nml Di/Tri Phasic Full Normal
R Serrat Ant LnTho C4-6 Nml 0 0 0 Nml Nml Di/Tri Phasic Full Normal
R Supraspinatus Supra C5-6 Nml 0 0 0 Nml Nml Di/Tri Phasic Full Normal
R Trapezius Spin C3-4 Nml 0 0 0 Nml Nml Di/Tri Phasic Full Normal
Ins = insertional activity, Fibs = fibrillation potentials, 0 = absent, 1+ minimal to 4+ maximal, PSW = positive sharp wave, Fas = fasciculation, Amp = amplitude,
Dur = duration, Rec Pat = recruitment pattern, Rec Int = recruitment interval, Nml = normal, Dec = decreased, 1st Dor Int = first dorsal interosseus, APB =
abductor pollicis brevis, Abd Dig Min = abductor digiti minimi, Cerv para = cervical paraspinal, Ext Car Rad = extensor carpi radialis, Ext Dig Com = extensor
digitorum communis, Ext Ind Pro = extensor indicis proprius, Flex Car Rad = flexor carpi radialis, Flex Dig Prof = flexor digitorum profundus, Flex Car Uln =
flexor carpi ulnaris, Lat triceps = lateral head pf triceps, LongHdTricep = long head of triceps, Serrat Ant = serratus anterior.
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associated risks. Complications of radiation therapy may
involve the skin, lymphatics, peripheral vasculature, and
peripheral nervous system. The most common symptoms
in the arm include skin changes, reduced mobility of the
arm, or secondary lymphedema, and they usually occur
after local high-dose radiation. A less common but
potentially more serious complication is radiation-
induced brachial neuropathy. The clinical manifestations
include gradually progressive paresthesias and sensory
loss, weakness and atrophy, and pain. Symptom onset
ranges from 1 month to 18 years after radiation exposure
[1]. The main theme of this case is the progressive late
polyneuropathy after two courses of therapeutic radio-
therapy. Hand function has progressively deteriorated. As
the first course of radiotherapy was in 1986, 22 years ago,
and the second course in 1991, 17 years ago, this case
illustrates the severe and progressive late effects many
years after radiotherapy. Multiple surgeries, the humeral
fracture and three courses of chemotherapy may also have
contributed to the neuropathy. The key evidence in favour
of radiation-induced injury is the fibrosis located within
the field of prior radiation therapy. During sarcoma
surgeries, there was no evidence of infiltration of any
major nerves and MRI of his right arm showed fibrotic
infiltrate and volume loss in the right arm with nodular ill-
defined median, radial and ulnar nerves with no focal
masses, consistent with postradiation changes. Che-
motherapy is known to increase the the risk of neuropathy.
Olsen et al. observed that patients receiving additional
chemotherapy have a higher incidence of neuropathy than
patients receiving radiotherapy alone [2]. The develop-
ment of fibrosis is a gradual process with a median interval
between radiotherapy and occurrence of brachial neuro-
pathy ranging between 1-4 years [3]. In most patients,
electrophysiologic studies show abnormal motor and
sensory nerve conduction and large motor unit potentials
with reduced recruitment. Myokymic discharges are found
in up to 63% of patients and are helpful in distinguishing
radiation-induced neuropathies from neoplastic neuropa-
thies [4]. Predisposing factors for the development of
radiation-induced neuropathy include the total radiation
dose and dose per fraction with a total tolerance dose of 60
Gy and with doses greater than 2 Gy per fraction [5]. Size
and localisation of the irradiated area also play a crucial
role. In this case the total dose given in two therapies was
80 Gy and thus exceeded the accepted tolerance dose of 60
Gy [5]. The dose per fraction was 2 Gy and within accepted
tolerance limits [5]. Side effects of radiotherapy can be
subdivided into early and late effects. Early effects occur
two days after nerve irradiation and include bioelectrical
alterations, enzyme changes, abnormal microtubule
assembly and altered vascular permeability. The late
effects occur between 1 year and decades post-irradiation
and they can be split into two phases. The first phase
includes changes in electrophysiology and histochemistry
of neurones and glial cells and the second phase includes
fibrosis of the tissue surrounding the nerves. Indirect
ischaemic damage due to microvascular injury also harms
neurones and glial cells. It is important though to stress that
the major damage caused in neurones, DNA damage, takes
place immediately at the instant of ionization. As the
mitotic rate of these cells is very slow, the damage is
normally not expressed until the cell tries to divide.
Therefore, this damage is latent for periods ranging from
days to decades. These changes lead to the the clinical
manifestations that this patient suffered from, including
gradually progressive paresthesias, hypoaesthesias and
dysaesthesias, paresis and atrophy, hyporeflexia, pain and
oedema. Adverse complications in bones include fracture,
osteoradionecrosis with potential creation of sequestra. It is
always important in individual patients to balance the
aggressiveness of the radiotherapy with its potential and
severe complications against the risk of potential recur-
rences. The occurrence of radiation-induced brachial
plexopathy should be considered in patients presenting
with upper extremity pain or weakness even many years
after radiation therapy. The course of the radiation-induced
neuropathy is one of steady progression or stabilisation in
90%of patients, although cases of improvement have been
reported [7-9]. No treatment is available to reverse or
improve the nerve injury, although surgical intervention,
such as neurolysis or neurolysis with omental grafting, has
been performed in some patients with variable improve-
ment in symptoms [10,11]. Radiation-induced brachial
plexopathy is an uncommon complication of radiation
treatment of fibrosarcoma, which can occur years after the
initial exposure. The risk of these complications is expected
to decrease with the use of modern state-of-the-art
radiation techniques. In patientswith symptoms, including
pain, sensory loss, or weakness, who have received prior
radiation, injury to nerves should be considered.
This case report reminds us that de novo symptoms and
signs of radiation-induced brachial plexopathy can arise
even after twenty two years of satisfactory upper limb
function. It is important for clinicians to inform patients
that complications of radiation therapy, which may
involve the skin, lymphatics, peripheral vasculature, and
peripheral nervous system can arise very late. The most
common complications in the first year post radiation
therapy include skin changes, reduced mobility and
secondary lymphedema but the onset of radiation-
induced neuropathy can occur as a complication from
early to very late time periods.
Consent
Written informed consent was obtained from the patient
for publication of this case report and accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
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Competing interests
The authors declare that they have no competing interests.
Authors’ contributions
All authors participated in the management of the patient
presented. SAB wrote and finalised the manuscript. All
authors read and approved the final form of the
manuscript.
Acknowledgements
We are grateful to the Department of Photography and the
Department of Radiology for the images.
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Brachial neuropathy 22 years after radiation therapy for fibrosarcoma: a case report

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