4/29/22
A Rare Cause of Sphenoid Sinusitis
Brian L. Risavi, DO, MS, FACEP, FAAEM, FACOEP, CEMSO,
PHP1,
brisavi@lecom.edu
Kevin Elwell, DO2,
elwellkt@upmc.edu
Courtney Whiteman, OMS II1,
cwhiteman38651@med.lecom.edu
1Lake Erie College of Osteopathic Medicine, Erie, PA;2UPMC Hamot, Erie, PA
Word Count: 674
Correspondence: Brian L. Risavi, DO, MS, Lake Erie College of
Osteopathic Medicine,
1858 W. Grandview Blvd., Erie, PA 16509, Phone: 814-877-3628; E-mail:
brisavi@lecom.edu
Conflict of Interest Statement: The authors report no conflict of
interest.
Data Availability Statement: Data sharing is not applicable to this
article as no new data were created or analyzed in this study.
Key Clinical Message (Abstract):
Malignancy of the sphenoid sinus is rare. Tumors may extend to adjacent
anatomic structures before detection and may be easily missed. Outcomes
are typically poor and vary by tumor type. Clinicians should maintain
vigilance for neoplastic disease in patients presenting with headache
and ocular/neurological complaints of the face/sinuses.
Key Words: lymphoma, headache, sphenoid sinus
Introduction:
Malignancy of the sphenoid sinus is rare, representing less than one
percent of all cancers [1]. The most common types are squamous cell,
adenocarcinoma, non-Hodgkin’s mature B-cell lymphoma, and unspecified
epithelial neoplasms [1]. Tumors may extend to adjacent anatomic
sites before detection [2]. Each tumor may present with a broad
range of symptoms, such as chronic sinusitis, and may be easily missed
[3]. Outcomes are typically poor [1]. Survival rates, however,
vary by type of tumor [4]. The most common age group affected is
50-59, predominantly white males [1]. Average tumor size is 3.7cm
[1].
Case:
A 54-year-old white male complained of an intermittent, increasingly
severe, headache for two weeks. On the day of presentation to the
emergency department, he awakened at 0600 with increasingly severe right
periorbital pain, described as sharp, radiating down his right cheek and
the right side of his neck. He had two episodes of nausea/vomiting. He
denied any syncope, visual changes, vertigo, slurred speech, extremity
weakness/paresthesias, fever/chills, or trauma. Review of systems was
otherwise negative. Past medical history includes diabetes mellitus,
migraine headaches, and Meniere’s disease. He denied and tobacco,
alcohol, or drug use. Family history includes migraines. On exam the
patient was awake/alert with stable vital signs. Patient was afebrile.
HEENT exam revealed pupils to be equal/round/reactive to light.
Photophobia was noted in the right eye. Extraocular muscles intact. No
cranial nerve deficits. No nasal drainage. Tenderness over the right
temporal artery noted. Uvula midline. No tongue deviation on protrusion.
No evidence of carotid bruits noted. No cervical adenopathy or nuchal
rigidity. The remainder of the exam was unremarkable. Diagnostic results
revealed a white cell count of 4.0 and an ESR of 2. Lumbar puncture was
negative. CT of the head revealed complete opacification of the sphenoid
sinus. CT angiogram of the head revealed no abnormalities. Patient was
admitted for further work-up, antibiotics, and ENT consultation. Patient
initially refused an MRI, however, he ultimately consented to an MRI of
the brain which was negative. MRA demonstrated stenosis of the right
anterior cerebral artery and right middle cerebral artery. Two days
later he developed a third nerve palsy and diplopia in the right eye
secondary to optic nerve involvement. Biopsy of the sphenoid sinus
revealed a large B-cell lymphoma, which was wrapped around the optic
nerve.
Patient treatment consisted of six months of chemotherapy with negative
PET scans on follow-up. Approximately five years thereafter the patient
was diagnosed with a GI bleed, fatty liver, and esophageal varices (for
which banding was completed). Further evaluation revealed malignant
hepatic lesions and metastasis to the spine causing back pain.
Palliative radiotherapy was initiated but the patient passed shortly
thereafter prior to further work-up of the hepatic lesions.
Discussion:
Primary B-cell lymphoma of the sphenoid sinus is very rare, having an
incidence of 0.03 per 100,000 persons between 2000-2012 [1,2,5,6].
B-cell lymphomas remain the most common non-epithelial tumors of the
paranasal sinuses [2,7]. The sphenoid sinus, contained within the
confines of the sphenoid bone, is anatomically contiguous with the
carotid arteries, optic nerves, the maxillary division of cranial nerve
V, brain stem, and sella turcica [3]. Consequently, clinical
presentations vary widely [5,7]. Headaches, often in the temporal,
retroorbital, and retrobulbar regions or, cranial neuropathies, and
facial pain/paresthesias may all occur [3,8,9,10]. Headaches are
thought to be secondary to tumor effect on the sphenopalatine ganglion
and remain the most common presenting symptom [4]. The anatomic
location makes resection challenging due to adjacent structures
[1,4]. Imaging often reveals opacification of the sphenoid sinus
[4,5]. CT and MRI remain the imaging modalities of choice
[10,11]. Fortunately, B-cell lymphomas are rapidly-growing and thus
very sensitive to chemotherapy and radiotherapy [5]. Surgery may be
possible in select cases [1,4]. Prophylactic intrathecal
chemotherapy may be utilized as there is a potential for recurrence in
the CNS [6]. Biopsy of sphenoid sinus opacification must be
considered in chronic sinusitis or when other previously mentioned
signs/symptoms occur [5,10,12,13]. Clinicians should maintain a high
degree of vigilance for neoplastic disease in patients presenting with
headache and ocular/neurological complaints of the face/sinuses.
Ethical Statement:
I certify that this material has not been published elsewhere, either in
whole or part, and is not under consideration for publication in any
other journal. I have personally and actively been involved in
substantive work leading to the revised manuscript and the authors will
hold themselves jointly and individually responsible for its content.
Statement of Acknowledgement:
This case report was published with the written informed consent of the
patient’s wife.
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