Orthopaedics & Traumatology: Surgery & Research (2012) 98, 233—237

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CASE REPORT

Ewing’s sarcoma of the finger: Report of two cases

and literature review
S. Baccari a, M.F. Hamdi b,∗, Z. Mabrouki a, M. Daghfous a, L. Tarhouni a

a
Department of Hand and Reconstructive Surgery, Kassab Institute of Traumatic and orthopaedic surgery, Manouba 2010, Tunisia
b
Department of Trauma and Orthopaedic Surgery, Fattouma Bourguiba University hospital, Monastir 5000, Tunisia

Accepted: 20 June 2011

KEYWORDS Summary Ewing’s sarcoma of the finger is extremely rare. Pain and swelling of the affected
Chemotherapy; finger are the most frequent presenting features. We report two cases of Ewing’s sarcoma
Ewing’s sarcoma; located at ring finger and the thumb in two children aged 14 and 10 years. The first patient died
Finger; of generalised metastases despite surgery, chemotherapy and radiation therapy. The second
Surgery had no localised recurrence or metastases after surgery and chemotherapy at last follow-up
of 4.5 years after tumour resection. The tumour’s surgical accessibility, chemotherapy, and
radiation therapy improve the prognosis of this tumour.
© 2012 Elsevier Masson SAS. All rights reserved.

Introduction Cases report

Ewing’s sarcoma was first described in 1921 [1,2]. It is the Case report 1
second most frequent primary bone cancer of childhood
and adolescence, after osteosarcoma [3]. It usually affects A 14-year-old, right hand dominant boy presented with
the long bones, but in the hand this tumour is rare, and 11 months history of a gradual pain and tumefaction
exceptional in the finger. Different treatments using surgery, involving the proximal part of the left ring finger (Fig. 1).
chemotherapy, radiotherapy, and their combinations are There was no recent trauma or fever or loss of weight.
described. The aim is to save the hand and the patient’s Locally, the swelling was indurate; the range of motion of
life. We report two cases of Ewing’s sarcoma, one in the the metacarpophalangeal joint was restricted.
thumb and the other in the ring finger. The X-ray examination of the affected finger, revealed
bone destruction with sclerosis and soft tissue component of
the proximal phalanx (Fig. 2). The laboratory findings were
within normal limits. At the Magnetic Resonance Imaging
(MRI), the tumour was detected with more precision (Fig. 3);
∗ Corresponding author. Tel.: +216 99 831 236 / 216 98 408 386; and the tumour appeared to be malignant on MRI.
fax: +216 73 460 678. Open surgical biopsy was performed. The histology con-
E-mail address: [Link]@[Link] (M.F. Hamdi). firmed the diagnosis of Ewing’s sarcoma. The preoperative

1877-0568/$ – see front matter © 2012 Elsevier Masson SAS. All rights reserved.
doi:10.1016/[Link].2011.06.012
234 S. Baccari et al.

Figure 1 Clinical view of the left hand showing a proximal

swelling of the ring finger.

assessment including chest tomography, and whole-body

bone scan showed no metastatic disease. Preoperative Figure 3 Magnetic resonance imaging (T1 weighted coronal
chemotherapy (VIDE protocol) was indicated and used for six section) showing large cortical destruction of proximal phalanx
courses using vincristine, ifosfamide, adriamycin, ectopo- of the left ring finger associated with soft tissue involvement.
side. After the parent’s consent, a wide excision of the
tumour and the involved tissues (ray amputation) was car- metastases 41 months after the diagnosis of the tumour was
ried out without local reconstruction (Fig. 4). The surgical established.
margins were negative. The histological findings proved a
poor response to chemotherapy. After surgery, a second
Case report 2
treatment course of chemotherapy using vincristine, actino-
mycine D, ifosfamide, was administered for six courses. The
A 10-year-old, left hand dominant boy presented with a mass
radiotherapy was indicated because the histological study
arising in the distal phalanx of the left thumb for 4 months.
of the excised tumour revealed more than 10% of tumour
This isolated mass was fixed and painful (Fig. 5). The radi-
cells; the area of the tumour was irradiated with 40 Gy. Six
ological examination revealed permeative bone destruction
months later, the patient developed metastases in the lung
and perpendicular spicules of the distal phalanx of the left
and tibia. Unfortunately, the patient died of generalised
thumb (Fig. 6). A biopsy was performed.

Figure 2 A lytic lesion of the proximal phalanx of the ring Figure 4 Postoperative radiograph of the left hand showing
finger with no evidence of periosteal reaction. the ray resection without reconstruction.
Ewing’s sarcoma of the finger: Report of two cases and literature review 235

Figure 7 No local recurrence of the tumour after metacar-

pophalangeal disarticulation.

Discussion

Initially Ewing’s sarcoma was described as a malignant

tumour of bone origin, invading the soft tissue. The ori-
gin of Ewing’s sarcoma is a subject of much debate. Once
Figure 5 Clinical picture of the multinodular tumour with
thought to be derived from primitive neuroectodermal cells
necrotic area involving the distal part of the left thumb.
[4], many now believe it to arise from a mesenchymal stem
cell [5]. We must mention Ewing tumour and not Ewing sar-
coma [4].
The histopathological diagnosis was Ewing’s sarcoma. Genetic factors play a role in its pathogenesis. There is
The chest tomography showed no metastatic lesions. A a form of anomaly chromosomal translocation of two chro-
technetium bone scan revealed increased uptake only in mosomes 22 and 15 [6]. Usually this tumour occurs during
the distal area of the left thumb. Amputation at the the first or second decade of life; it particularly affects long
metacarpophalangeal level was carried out (Fig. 7). The bones and pelvis [7]. In the hand Ewing sarcoma is very rare,
patient had postoperative adjuvant chemotherapy using vin- Kissane JM series of 303 cases of Ewing sarcoma, identified
cristine, ifosfamide, adriamycin, ectoposide for six cycles. one lesion in the hand [8]. The metacarpals are most com-
The patient did not develop metastasis or local recurrence; monly affected and occurrence at the phalanges is quite rare
he was well and alive at 4.5 years after surgery. [9—11]. Regardless of the treatment modality, the location
of the tumour in the hand is an important factor of prognosis
[10]. Because the survival is highest in lesions of the distal
bones of the extremities [8].
The review of literature found 15 cases of Ewing’s sar-
coma of the finger [1,3,9—17]. We report the summary
of those cases (Table 1). According to literature review,
males were more affected (69%); the average age of detec-
tion of the tumour was 18.5 years (5 month—51 years). The
tumour was located at the proximal phalanx in 53% of cases.
The thumb (28%) and the long finger (28%) were the most
affected fingers.
Pain and swelling of the affected fingers were the most
frequent complaints at diagnosis [7], initially the patient had
a general good health, and there were no fever and loss of
weight [4]. Sometimes the tumour is clinically mistaken for a
local infection because of pain and local inflammatory symp-
toms, X-ray findings can also be mistaken for osteomyelitis
because of the bone destruction [1].
Several studies [4,7,9,10,17] have suggested wide radi-
cal resection of the tumour, combined with chemotherapy
as the best therapy. This is easily accomplished in case
of digital localisation. The surgical accessibility of Ewing’s
sarcoma of the phalanx makes them amenable to radical
resection [1,11]. Two cases of Ewing’s sarcoma of the thumb
Figure 6 An X-ray film of the left thumb showing bone [11,14] were treated by primary amputation without radi-
destruction with perpendicular spicules of the distal phalanx. ation therapy or chemotherapy. There was no evidence of
Soft tissue mass visualized in the border of distal phalanx. disease in 26 to 60 months following up.
236 S. Baccari et al.

Table 1 Summary of published cases after literature review.

Case Author Age Sex Finger Phalanx Treatment Alive Dead Follow-up (months)

1 Clifford RH. 1955 [12] NP NP III Proximal S — + 5

2 Ridings GR. 1964 [13] 19 M II Proximal S—Ch - R — + 8
3 Dick MH. 1971 [14] 12 M I Distal S + — 26
4 Wilson KS, 1979 [15] 39 M II Distal S—Ch - R — + 11
5 Kinsella TJ. 1983 [18] 18 M — Proximal Ch - R + — 62
6 Shirley SK. 1985 [16] 11 M II Distal S - Ch + — 41
7 Shirley SK. 1985 [16] 21 M IV Proximal S—Ch - R + — 63
8 Lacey SH. 1987 [9] 16 F III Proximal S - Ch + — 41
9 Strege DW. 1989 [17] 5 months M III Middle S - Ch + — 12
10 Euler E. 1990 [1] 22 — III Proximal S—Ch - R NP NP NP
11 Jones MW. 1993 [10] 12 F I Distal S - Ch + — 30
12 Yamaguchi T. 1997 [11] 51 M I Distal S + — 60
13 Seroussi D. 2004 [4] 10 F I Proximal S - Ch + — 16
14 Jerome TJ. 2008 [7] 13 F V Distal S - Ch + — 24
15 Anakwenze OA. 2009 [3] 13 M II Proximal S - Ch + — 104
F: female; M: male; S: surgery; Ch: chemotherapy; R: radiation; NP: no precision.

Radiotherapy alone is not indicated because it cannot chemotherapy. The histological findings determine the use-
control the primary lesion, and induces major physi- fulness of radiotherapy. Despite the small size of these
cal damage (soft tissue contracture, growth disturbances tumours the preoperative chemotherapy is necessary.
in young patient, secondary radiation-induced malignant
tumour) [4,9]. The radiation is beneficial when wide mar- Disclosure of interest
gins cannot be surgically achieved or when the response to
chemotherapy is incomplete [3].
The authors declare that they have no conflicts of interest
In spite of the adjuvant therapy, most authors agree
concerning this article.
about the benefit from the tumour’s resection, which
remains the most important stage of the management of
Ewing’s sarcoma of the finger. Kinsella [18] suggested the References
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