The patient is a 19-year-old man with a 10-month history of nasal congestion and worsening epistaxis for the past 3 months. Previous medical history is significant for bilateral retinoblastoma at age 3 that was treated with left eye enucleation and ocular implant, and conventional radiation treatment of the right globe. Computed tomography (CT), enhanced magnetic resonance (MR) imaging, MR angiography, and conventional catheter angiography were performed.
Prepared by Lewis Shin, BS and Glenn Tung, MD of the
Department of Diagnostic Imaging, Brown University School of
Medicine, Providence, RI.
The patient is a 19-year-old man with a 10-month history of
nasal congestion and worsening epistaxis for the past 3 months.
Previous medical history is significant for bilateral
retinoblastoma at age 3 that was treated with left eye enucleation
and ocular implant, and conventional radiation treatment of the
right globe. Computed tomography (CT), enhanced magnetic resonance
(MR) imaging, MR angiography, and conventional catheter angiography
Juvenile nasopharyngeal angiofibroma (JNA) of the nasal
A left eye prosthesis and calcification of the right globe is
consistent with treated bilateral retinoblastoma (figure 1). A
large, enhancing nasal cavity mass extending into the nasopharynx
with feeding vessels from branches of the left external carotid
artery was found (figures 2 and 3).
Juvenile nasopharyngeal angiofibroma constitutes < 0.5% of
all head and neck neoplasms. It is a histologically benign, yet
locally aggressive, vascular tumor that occurs most frequently in
males from 5 to 25 years of age. Complications occur when there is
local invasion of surrounding tissues. The most common route of
local spread is medially through the sphenopalantine foramen to the
posterior oropharynx and nasal cavity. Consequently, JNA should be
suspected in a boy or adolescent male with a history of chronic
nasal obstruction or recurrent, atraumatic epistaxis and a
soft-tissue mass in the nose or nasopharynx.
Diagnostic imaging should be performed prior to an invasive
procedure since it may suggest the diagnosis, thereby reducing the
risk of catastrophic hemorrhage associated with biopsy. In a study
of 72 patients, Lloyd et al
report three findings on CT and MR imaging that should suggest a
diagnosis of JNA: 1) a soft-tissue mass in the nasopharynx or nasal
cavity; 2) a mass in the pterygopalatine fossa; and 3) erosion of
posterior osseous margin of the sphenopalantine foramen extending
to the base of the medial pterygoid plate. In addition, JNA can
grow into the maxillary and sphenoid sinuses, orbit, and central
There have been rare reports of JNA originating in the maxillary
and sphenoid sinuses.
On CT, bone involvement can best be seen on thin-section axial or
coronal images. On MR imaging, JNA appears as a heterogeneous mass
with signal voids that are consistent with the highly vascular
tumor; intense enhancement with gadopentetate contrast material is
typical (figure 2).
The differential diagnosis for this large nasal mass includes
antrochoanal polyp; inflammatory sinonasal polyp; neurofibroma;
adenoidal hypertrophy; and malignant neoplasm, such as
nasopharyngeal carcinoma, lymphoma, or rhabdomyosarcoma. CT and MR
imaging of nasopharyngeal carcinoma show an inhomogeneous mass
arising from the nasopharyngeal mucosa or submucosal space with
skull-base erosion or intracranial extension.
Lymphoma may arise from adenoidal tissues of the nasopharynx or
Waldeyer's ring and can be associated with lymphadenopathy. Imaging
of rhabdomyosarcoma reveals a soft-tissue mass with frequent bone
destruction and mild enhancement on CT, but marked enhancement on
The carcinogenic effects of radiation therapy have been
confirmed since the classic study of radiogenic osteosarcoma by
Cahan et al.
Four criteria were cited in this study for a tumor to be considered
radiation-induced. These criteria include: 1) absence of
microscopic and radiographic evidence of malignancy prior to
radiation treatment; 2) development of the tumor in the field of
radiation treatment; 3) a relatively long latency (exceeding 5
years) between treatment and discovery of tumor; and 4) histologic
confirmation of the tumor.
Sarcoma is the most common malignant, radiation-induced neoplasm;
most often, osteosarcoma in bone and malignant fibrous histiocytoma
in soft tissue. The most common benign radiogenic tumor is
This case of JNA fulfills the criteria for radiogenic tumor, since
it arose in the treatment field 16 years following conventional
radiation therapy for retinoblastoma. Furthermore, there was no
radiologic or histologic evidence of a naso-pharyngeal tumor prior
Interestingly, patients with retinoblastoma are at increased
risk for developing a second primary tumor even without radiation
treatment. Even though the majority of cases of retinoblastoma are
unilateral, the majority of second neoplasms arise in patients with
This risk of second tumors is increased even further in patients
receiving radiation therapy. In a study of 688 patients with
retinoblastoma, tumors that developed in the field of radiation
treatment included 24 cases of osteosarcoma, 6 cases of
fibrosarcoma, and 5 cases of soft tissue sarcoma; no cases of
juvenile angiofibroma were reported.
In conclusion, we believe that this may represent a rare case of
radiogenic angio-fibroma following radiation treatment of bilateral
retinoblastoma. However, we cannot exclude the alternative that it
represents the rare occurrence of two unusual diseases in the same
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