Baylor University Medical Center Proceedings July 2017 - 325

Ewing's sarcoma of the trachea in an adolescent girl
Jaisankar Puthusseri, MD, Geetha Narayanan, MD, DM, T. R. Preethi, MD, and G. Jayapriya, MD

Primitive neuroectodermal tumors (PNET) are aggressive neoplasms of
neuroectodermal origin. Although they are known to arise in a host of
locations, involvement of the trachea has rarely been reported. We describe an adolescent girl who presented with stridor and was diagnosed
with PNET of the trachea. She is in remission following treatment with
combination chemotherapy and local radiotherapy.

rimitive neuroectodermal tumors (PNET) are a heterogeneous group of highly malignant neoplasms of neuroectodermal origin. Largely a disease of children and
adolescents, PNETs have been diagnosed across all age
groups. Most often, PNETs arise in the chest wall, paravertebral
region, retroperitoneum, and extremities. PNET of the trachea
is exceedingly rare, with only a few reported cases (1-3). Here
we report the case of a 14-year-old girl who presented with
stridor and was eventually diagnosed with PNET of the trachea.
CASE PRESENTATION
A 14-year-old girl presented to us with a 6-month history
of dry cough and dyspnea. She also gave a history of signiﬁcant
weight loss and dysphagia for solids for the past 2 months. On
examination, she had an Eastern Cooperative Oncology Group
performance status of 1, and chest examination revealed bilateral
rhonchi. Computed tomography of the thorax showed a wellenhancing homogenous mass of 16 × 20 × 15 mm arising from
the anterior and left lateral wall of the trachea with intraluminal
extension causing airway narrowing, and it was seen extending
to the left paratracheal region (Figure 1). A ﬁber optic bronchoscope-assisted biopsy was done. Histopathology showed a neoplasm composed of sheets of atypical small round cells beneath
the respiratory epithelium. The tumor cells were positive for
MIC2 membrane staining and for pan cytokeratin (AE1/AE3).
Other markers like desmin, myogenin, and synaptophysin were
negative. The morphological and immunohistochemical proﬁle
was consistent with the diagnosis of PNET (Figure 2). Staging
evaluation including computed tomography scan of the abdomen, Technetium99 bone scan, and bone marrow biopsy were
normal.
The patient was started on chemotherapy with vincristine,
doxorubicin, and cyclophosphamide alternating with ifosfamide
Proc (Bayl Univ Med Cent) 2017;30(3):325-326

Figure 1. CT of the thorax showing a moderately enhancing soft tissue lesion
involving the left anterolateral wall of the trachea at the T3 vertebral level causing luminal narrowing.

and etoposide (VAC/IE). At 12 weeks of chemotherapy, she received intensity-modulated radiation therapy to the primary site
at a dose of 55 Gy given over 30 fractions. She has completed
1 year of chemotherapy and is presently on follow-up.
DISCUSSION
PNETs are aggressive small round cell tumors of neural
crest lineage, coming within the ambit of the Ewing's sarcoma family of tumors (ESFT). The median age at diagnosis
is 19.5 years, and 30% of patients present with metastatic
disease. Compared with patients with skeletal ESFT, patients
with extraskeletal ESFTs are more likely to be older and female
and to have tumors in axial locations (4). Clinical features
are often site speciﬁc, with swelling and pain being the most
common presentations.
There have been very few reported cases of tracheal PNETs.
One case described a young male presenting with stridor and
hemoptysis caused by a PNET arising in the distal trachea.
After initiating chemotherapy with the VAC/IE regimen, the
tumor was resected and adjuvant radiation with 50.4 Gy over
28 fractions was administered, followed by completion of the
planned chemotherapy (1). In another case of tracheal PNET
From the Departments of Medical Oncology (Puthusseri, Narayanan) and
Pathology (Preethi, Jayapriya), Regional Cancer Center, Trivandrum, India.
Corresponding author: Geetha Narayanan, MD, DM, Department of Medical
Oncology, Regional Cancer Centre, Trivandrum 695011, Kerala, India
(e-mail: geenarayanan@yahoo.com).
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