Journal of Otology & RhinologyISSN: 2324-8785

All submissions of the EM system will be redirected to Online Manuscript Submission System. Authors are requested to submit articles directly to Online Manuscript Submission System of respective journal.
bahis siteleri bahis siteleri bahis siteleri casino siteleri

Case Report, J Otol Rhinol Vol: 1 Issue: 2

Metastatic Pancreatic Neuroendocrine Carcinoma Presenting as a Sphenoid Sinus Mass in a Juvenile

Lori A. Lemonnier1, Ozlem E. Tulunay-Ugur2, Imad T. Zak3 and Michael A. Carron4*
1Department of Otolaryngology, Head & Neck Surgery, Temple University, 3440 N. Broad Street, Philadelphia, PA 19140, USA
2Department of Otolaryngology, Head and Neck Surgery, University of Arkansas for Medical Sciences, 4301 W. Markam Street, Slot 543, Little Rock, AR 72205, USA
3Department of Radiology, Wayne State University/Detroit Medical Center, Detroit Receiving Hospital 3L-8, 4201 St. Antoine Detroit, MI, 48201, USA
4Department of Otolaryngology, Head and Neck Surgery, Wayne State University, 5E-UHC, 4201 St. Antoine Detroit, MI 48201, USA
Corresponding author : Michael A. Carron, MD
Associate Professor, Department of Otolaryngology, Head and Neck Surgery, Wayne State University, 5E-UHC, 4201 St. Antoine Detroit, MI 48201, USA
Tel: (313) 577-0805; Fax: (313) 577-8555
E-mail: [email protected]
Received: June 08, 2012 Accepted: August 06, 2012 Published: August 08, 2012
Citation: Lemonnier LA, Tulunay-Ugur OE, Zak IT, Carron MA (2012) Metastatic Pancreatic Neuroendocrine Carcinoma Presenting as a Sphenoid Sinus Mass in a Juvenile. J Otol Rhinol 1:2. doi:10.4172/2324-8785.1000102


Metastatic Pancreatic Neuroendocrine Carcinoma Presenting as a Sphenoid Sinus Mass in a Juvenile

Primary neoplasms of the sphenoid sinus are rare. Instead, these lesions typically arise by local tumor extension from surrounding structures or are non-neoplastic in origin. Metastatic lesions are encountered even less frequently. We present a case of metastasis of pancreatic neuroendocrine carcinoma to the sphenoid sinus in an 18 year old male and to document this rare but potentially confusing radiologic diagnosis.



Sphenoid sinus; Neoplasm; Neuroendocrine carcinoma; Metastasis


5-FU: Fluorouracil; VP16: Etoposide Phosphate; MRI: Magnetic Resonance Imaging; EGD: Esophagogastroduodenoscopy; CT: Computed Tomography


Primary neoplasms of the sphenoid sinus are rare. Instead, these lesions typically arise by local tumor extension or invasion from surrounding structures, including the ethmoid sinus, nasopharynx, and sella turcica. Occasionally, non-neoplastic entities, including fungal and inflammatory disease, such as mycetoma and mucocele are present. Primary sphenoid neoplasms represent only 2-3% of all paranasal sinus cancers [1]. Further, in our review of the English language literature, reports of metastatic disease to the paranasal sinuses are extremely rare, and few note involvement of the sphenoid sinus. Using PubMed without limits on the timeframe and using the keywords metastatic pancreatic neuroendocrine tumor, sphenoid sinus neoplasm, sphenoid sinus metastasis and paranasal sinus metastasis there were no reported cases in the English language literature. Therefore, it was felt that pancreatic neuroendocrine carcinoma, a rare disease in itself with an incidence of 4/1,000,000/ year [2], metastasizing to the sphenoid sinus should be documented in the literature. This is, to the best of knowledge, the first reported case of a pancreatic neuroendocrine carcinoma with metastasis to the sphenoid sinus.

Case Report

The patient is an 18 year old African American male with a history of stage IV metastatic neuroendocrine carcinoma of the pancreatic head to the liver, retroperitoneum, and bone, status post chemotherapy treatment with 5-FU (Fluorouracil), Streptozocin, Carboplatin, and VP16 (Etoposide phosphate), to which he was unresponsive. He presented to the Otolaryngology service with complaints of blurred vision. An MRI (magnetic resonance imaging) scan of the brain revealed a heterogeneous lesion of the sphenoid sinus extending intracranially with cortical discontinuity and meningeal enhancement along the inferior aspect of the right frontal lobe just above the sphenoid sinus (Figures 1A and 1B). In light of his chemotherapeutic regimen and the potential for a reduction in host defense mechanisms these findings were described as being consistent with fungal sinusitis with intracranial extension. MRI scan also demonstrated that the lesion was not an extension from the pituitary gland into the sphenoid and was not a pituitary primary lesion (Figure 2). At the time of examination he was started on dexamethasone therapy and shortly thereafter reported return of visual acuity to normal.
Figure 1a: T2 weighted MRI axial image shows an anterior skull base “dark” mass suggesting bony sclerosis (black arrows).
Figure 1b: T1 weighted MRI coronal image following intravenous gadolinium shows focal sclerosis of the planum sphenoidale (white open arrow) with a soft tissue component filling the sphenoid sinus (asterisk) and an overlying dural reaction (white solid arrows).
Figure 2: Sagittal T1 weighted image shows normal sella and pituitary gland (long white arrow), a separable tumor in the sphenoid sinus (asterisk), and overlying bony hyperostosis of the planum sphenoidale (double short arrows). Notice the inspissated secretions in the sphenoid sinus posterior to the tumor and inferior to the sella.
His past medical history was otherwise significant for peptic ulcer disease, diagnosed on EGD (esophagogastroduodenoscopy) during a workup of abdominal pain and weight loss, his initial presenting symptoms that led to his current diagnosis. He denied alcohol, tobacco, and illicit drug use, and had no known family history of malignancy.
In pre-operative planning for biopsy of the sphenoidal tissue to rule out fungal sinusitis, a CT (computed tomography) scan of the paranasal sinuses showed near complete opacification of the left sphenoid sinus with hyperdense, enhancing material, without fluid. There was an abnormal appearance of the planum sphenoidale, which was sclerotic with a spiculated periosteal reaction and an extraaxial soft tissue extension into the anterior cranial fossa with enhancement of the overlying dura (Figure 3). These findings were described as being most consistent with a metastatic neoplasm with overlying chronic inflammatory mucosal disease of the paranasal sinuses.
Figure 3: Reformatted sagittal image from a CT of the skull base shows sclerosis of the planum sphenoidale (white arrow) and soft tissue tumor filling and causing blistering of the sphenoid sinus (asterisk).
A transseptal approach to the sphenoid sinus was used for sphenoidotomy and removal of the sphenoid contents, which were described as a smooth mass, pink to tan in color. The pathology was reported as intermediate grade neuroendocrine carcinoma. Immunohistochemistry was positive for chromogranin, synaptophysin, and keratin. Significantly, the specimen was morphologically similar to the neuroendocrine carcinoma previously observed on liver biopsy (Figures 4-6). Gram stain, anaerobic cultures and fungal cultures were negative. Aerobic tissue culture grew only rare staphylococcus aureus.
Figure 4: Low-power view of the tumor, which consists of a uniform population of spindle and epithelioid cells organized in a nested pattern (Hematoxylin-eosin, 100X).
Figure 5: High-power view of the tumor, which shows a finely granular (so-called ‘salt-and-pepper’) chromatin pattern in the nuclei. A mitotic figure is present at the arrow (Hematoxylin-eosin, 600X).
Figure 6: Calcitonin immunostain demonstrates positive staining in the cytoplasm, confirming the diagnosis of neuroendocrine carcinoma (400X).
Post-operatively the patient received radiation therapy to the sphenoid sinus and skull base. Post treatment he denied symptoms of visual changes and headache. However, the patient’s overall condition declined, and he eventually expired six months later due to an obstructive hepatic lesion with secondary hepatic failure. The time from initial diagnosis to the expiration of the patient was approximately fourteen months.
The last CT image of the paranasal sinuses obtained was one week prior to the patient’s death and demonstrated a calcified mass in the region of the planum sphenoidale extending inferiorly into the roof of the sphenoid sinus and opacification of the sinus chamber with air-fluid levels.


The reported pathology of primary sphenoid sinus malignancies include neuroendocrine carcinoma, sinonasal undifferentiated carcinoma, mucoepidermoid carcinoma, giant cell tumor, and melanoma [1,3]. Metastatic lesions known to arise in the sphenoid sinus include papillary and follicular carcinoma of the thyroid, hepatocellular carcinoma, renal cell carcinoma, transitional vesical cell carcinoma, endometrial clear cell carcinoma, and rectal and pancreatic adenocarcinoma among others [4-7]. In a case review by Mickel and Zimmerman, the most common tumor sites from which sphenoid metastases arose included the prostate and the lung [8]. After review of the English language literature, we have found that the only previous report specific to neuroendocrine tumor metastasis to the head and neck was a pancreatic primary malignancy presenting with slowly enlarging cervical lymphadenopathy accompanied by watery diarrhea [9]. Therefore this is the first report of a neuroendocrine pancreatic malignancy with metastasis to the sphenoid sinus.
In our patient the initial MRI interpretation raised concern for fungal sinusitis with intracranial extension especially when considering the regimen of chemotherapy the patient underwent prior to his diagnosis. However, CT scan of the paranasal sinuses was consistent with a lesion of metastatic origin. With uncommon findings on his initial imaging studies and taking into consideration his immunocompromised state, biopsy of the sphenoid sinus contents in this patient was thought to be imperative and indeed detected a rare occurrence of metastasis of pancreatic neuroendocrine carcinoma.
In light of the above case, we would suggest that given abnormal findings isolated to the sphenoid sinus on imaging of the paranasal sinuses, patient history should be heavily weighted in decisions regarding evaluation and management, and when appropriate, such rare etiologies should be considered among the differential diagnosis.


Track Your Manuscript

Media Partners