Askin tumor: a rare chest wall sarcoma in an Asian
adult patient—a literature review
and a suggested treatment protocol
Chong Han Pek
Samuel Yew Ming Ho
Ee Cherk Cheong
Received: 13 April 2014 /Accepted: 27 July 2014 / Published online: 26 August 2014
Springer-Verlag Berlin Heidelberg 2014
Abstract Askin tumor is a sarcoma of the chest wall of
neuroectodermal origin, and is part of Ewing’s sarcoma family
of tumors. It is a rare disease with high recurrence rates and
poor prognosis. Management involves wide resection of the
tumour with adjuvant/neo-adjuvant chemotherapy and radio-
therapy. Advances in reconstructive options allow for wider
resection margins that hopefully help to reduce local recurrence.
Much of what we know about Askin tumors was extrapolated
from Ewing’s sarcoma, and there is a lack of clear evidence and
guidelines to the management of these tumors. We report a rare
case of Askin tumor of the anterior chest wall in a 50-year-old
Malay man and suggest a treatment protocol based on a review
of current literature.
Level of Evidence: Level V, therapeutic study.
The first documented report of a primitive neuroectodermal
tumor (PNET) was by Stout, who described it in the ulnar
nerve . Subsequently, Ewing reported a radiosensitive
osteolytic tumor of the radius which was later referred to by
the eponym Ewing’ssarcoma. Askin tumor was coined by
Askin et al.  in 1979 when they reported a case series of
malignant small, round cell tumors of the thoraco-pulmonary
region of neuroectodermal origin. These tumors form a dis-
tinct entity, distinguishing them from other malignant small
round cell tumors such as neuroblastoma, Ewing’ssarcoma,
lymphoma, and rhabdomyosarcoma.
Ewing’s sarcoma family of tumors (ESFTs) was later
conceptualised as a manifestation of a single neoplastic entity
with common phenotypic and molecular features, but
differing in location and differentiation of the tumors. ESFTs
comprised of PNET, Ewing’s Sarcoma, extra-osseous Ewing’s
tumors, and Askin tumor. Pathognomonic to ESFTs are the
non-random translocations involving the EWS gene and one
of the ETS family of transcription factors .
Even though ESFTs are the second most common malig-
nant neoplasm of the bone among children and adolescents
with an incidence of about 3 per 1,000,000, Askin tumors are
very rare and even more so in adulthood. Most literature on
this condition is in the Caucasian population, with few reports
on Asian patients [5–8].
We report a rare case of Askin tumor of the chest wall in an
adult Malay male, review the current literature on this tumor,
and propose a simple treatment protocol.
A 50-year-old Malay man presented with the complaint of a
painless left infra-clavicular-lobulated lump of 6 months duration
(Fig. 1). He was referred with provisional diagnosis of a lipoma.
Ultrasound of the lump revealed a well-defined, hypoechoic
solid subcutaneous lesion measuring 4.1×3.9 cm, with internal
septation and vascularity. Magnetic resonance imaging (MRI)
revealed a complex lesion with enhancing solid-cystic compo-
nents and marginal irregularity, suggestive of a soft tissue sarcoma
(Fig. 2). The lesion was abutting the overlying skin and underly-
ing pectoralis muscle without radiological evidence of invasion.
Fine needle aspiration (FNA) biopsy yielded cores of tissue
with fibrous septae and nests of atypical-looking cells. Immuno-
histochemical analysis stained positive for synaptophysin and
CD99 and negative for TTF-1, chromogranin, cytokeratin,
EMA, S100, LCA, and CD 34. Further gene testing of the
specimen using gene testing kit (Vysis LSI EWSR1 Dual Color,
Break Apart Rearrangement Probe) was positive for EWS
(22q12) gene translocation. Staging computed tomography
(CT) scan of the thorax showed probable right hilar lymph node
involvement. Radionuclide bone scan was negative.
C. H. Pek (*)
S. Y. M. Ho
E. C. Cheong
Plastic, Reconstructive and Aesthetic Surgery Section, Department of
General Surgery, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng,
Singapore 308433, Singapore
Eur J Plast Surg (2014) 37:627–630