Sir,
Soft-tissue sarcoma of the upper limb is rare tumour.[[1]] Only 15% of soft-tissue sarcomas occur in extremity.[[2]] The most common histologic subtypes of soft-tissue sarcoma seen in the hand are
epitheloid sarcoma, synovial sarcoma and malignant fibrous histiocytoma.[[1]] The synovial sarcoma arising in the arm and that too in the 4-month-old infant
is rare.[[3]
[4]
[5]]
A 4-month-old infant was brought with complaints of rapidly growing swelling in the
right arm with ulceration and bleeding for 35 days [[Figure 1]]. There was no history of any comorbid illness. No other relevant history was elicitable.
Clinical examination revealed an active infant with normal nutrition. There was a
firm ovoid swelling measuring 4.5 cm × 3 cm × 3 cm attached to biceps brachii muscle
with 2 cm × 3 cm ulceration at its summit, which was bleeding on manipulation [[Figure 1]]. There were no regional lymph node enlargements. Incisional biopsy was reported
as synovial sarcoma. Metastatic workup revealed no spread of tumour to distant sites.
Appropriate informed consent for the surgery was obtained from the parents including
consent for the use of clinical data and pictures for research studies.
Figure 1: A 4-month-old baby with synoviosarcoma of the arm (left). Magnetic resonance imaging
showing infiltration into biceps muscle (right)
The surgical oncologists under general anaesthesia did wide local excision of the
tumour with 2.5-cm margin. Resultant defect was measuring 8 cm × 6 cm. There was only
small residuum of the long head of biceps with exposure of radial nerve and musculocutaneous
nerve over the brachialis muscle in the bed of the excised defect [[Figure 2]].
Figure 2: After wide local excision showing the exposure of neurovascular bundles (left). Supermicrovascular
anastomosis (right)
With pattern of the defect, 8.5 cm × 6 cm free anterolateral free flap was harvested
with lateral femoral cutaneous nerve. Recipient vessels were musculocutaneous perforator
from the brachial artery and tributary to brachial vein. The sizes of vessels were
from 0.75 mm to 0.8 mm. Single vein anastomosis was done. Operating microscopes with
×20 magnification were used for anastomosis. Lateral femoral cutaneous nerve was anastomosed
to anterior branch of medial cutaneous nerve of the arm [[Figure 3]]. Donor site was closed primarily.
Figure 3: Vimentin and Transducin like Enhancer split -1 (TLE-1)-positive monophasic synoviosarcoma
Immunohistochemical examination of the excised specimen revealed the spindle cells
in whorls, glandular pattern and vimentin and TLE-1(Transducin Like Enhancer split
-1) positive[[6]] [[Figure 3]] confirming the pre-operative pathology study as synovial sarcoma – monophasic.
It was Stage IA tumour (G1T1bN0 M0). Tumour margins were declared free. Therefore,
tumour board of our institution declared no need for any adjuvant therapy.
The infant was followed for 12 months postoperatively. Flap and donor sites settled
well [[Figure 4]]. There was neither any locoregional recurrence nor any distant metastases. There
was good functional recovery of the right elbow function with no distal deficit.
Figure 4: Late post-operative picture
Surgery is the most common modality of treatment for the synovial sarcoma and advocated
as the primary modality for the locoregional control of the disease.[[7]] The goal of the surgical treatment is excision of tumour with cuff of normal tissue
three dimensionally. Tumour was fixed to biceps brachii and the greatest dimension
was <5 cm (T1b tumour). In this case, it was in the uncommon site as the common site
being the carpus and fingers in the upper limb and also not in relation to any joints,
tendons or bursae.[[8]
[9]] Quite contrast to its grade, it was growing aggressively, and thus, histological
grade was not truly predictive of biological behaviour of the tumour.[[10]] Considering the stage of the disease, it was decided for the limb preservation
surgery. As the tumour in this case was not violating the fascia between the brachialis
and biceps brachii, it was possible for the wide local excision with reasonable margin,
with preservation of brachial neurovascular bundles and radial and musculocutaneous
nerves. However, the surgical oncologists excised the most portions of biceps. Considering
the resultant defect, single-stage free anterolateral thigh flap was planned and was
done with intraoperative consultation confirming the tumour-free margins as per the
current consensus in the management of synovial sarcoma.[[11]
[12]] The donor site morbidity was almost nil as the secondary defect was closed primarily.
The neurotisation, in this case, was done to improve the local proprioception paving
the way for early recovery. One-year disease-free survival was obtained in this case
and baby remained healthy.
Despite the aggressive nature of tumour, a well-executed wide local excision with
single-stage reconstruction with free flap can be a good choice for these uncommon
malignant tumours occurring in the infants. Advancements in super microsurgery and
infant anaesthesia pave the way for achieving the limb preservation in these types
of rare malignant tumours of the infants.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
In the form the patient(s) has/have given his/her/their consent for his/her/their
images and other clinical information to be reported in the journal. The patients
understand that their names and initials will not be published and due efforts will
be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
