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Nasopharyngeal carcinoma presenting with rapidly progressive severe visual disturbance: a case report.

Kamio Y, Sakai N, Takahashi G, Baba S, Namba H - J Med Case Rep (2014)

Bottom Line: The histopathological diagnosis was nonkeratinizing undifferentiated nasopharyngeal carcinoma.Epstein-Barr virus was detected by in situ hybridization.Although focal radiotherapy induced remarkable tumor shrinkage and relieved ocular motor disturbance and facial pain, his visual acuity did not improve.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Neurosurgery, Hamamatsu University School of Medicine, Handayama 1-20-1, Higashiku, Hamamatsu, Shizuoka 431-3192, Japan. nsakaineurosurg@gmail.com.

ABSTRACT

Introduction: Nasopharyngeal carcinoma is one of the most difficult tumors to diagnose correctly at the initial phase because of the occasional lack of nasal symptoms. The perineural spread of the trigeminal nerve is one of the most common and important routes in the intracranial paracavernous extension of nasopharyngeal carcinoma, but visual loss is very rare.

Case presentation: We report the case of a 54-year-old Japanese man with nasopharyngeal carcinoma, who presented with rapid and severe disturbance of left monocular visual acuity and eye movement with a 10-month history of ipsilateral otitis media and facial pain. Magnetic resonance imaging revealed a lesion in the left fossa of Rosenmüller, pterygopalatine fossa, sphenoid and ethmoid sinus, and the left cavernous sinus extending to the orbital apex through the superior orbital fissure. The histopathological diagnosis was nonkeratinizing undifferentiated nasopharyngeal carcinoma. Epstein-Barr virus was detected by in situ hybridization. Although focal radiotherapy induced remarkable tumor shrinkage and relieved ocular motor disturbance and facial pain, his visual acuity did not improve.

Conclusion: The awareness of cranial nerves in addition to intracranial and orbital apex involvement, as in this case, is important for appropriate diagnosis and treatment planning of nasopharyngeal carcinoma.

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Related in: MedlinePlus

Hematoxylin-eosin staining (A) and immunohistochemical staining for Ep-CAM/Ber-EP4 (B), CD117/c-Kit (C), Epstein–Barr virus-encoded ribonucleic acid in-situ hybridization (D), and Ki-67 (×400 magnification) (E).
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Figure 2: Hematoxylin-eosin staining (A) and immunohistochemical staining for Ep-CAM/Ber-EP4 (B), CD117/c-Kit (C), Epstein–Barr virus-encoded ribonucleic acid in-situ hybridization (D), and Ki-67 (×400 magnification) (E).

Mentions: A 54-year-old Japanese man was admitted to our neurosurgical department for the investigation and treatment of acute visual loss of his left eye. He had been treated for left serous otitis media and left facial pain mimicking trigeminal neuralgia for 10 months by the otolaryngologist of a local hospital. Neurological examination on admission showed left visual acuity of no light perception and left eye movements disturbed in all directions. Gadolinium-enhanced, fat-suppressed, T1-weighted magnetic resonance (MR) imaging revealed a lesion in the left fossa of Rosenmüller, pterygopalatine fossa, sphenoid and ethmoid sinus, and the left cavernous sinus extending to orbital apex through the superior orbital fissure (Figure 1). With a tentative diagnosis of malignant skull base tumor, an endoscopic tumor biopsy from nasopharyngeal tissue was performed, but failed because the nasopharyngeal tissue appeared normal and the tumor could not be identified. Subsequently, the intraorbital tumor was explored by performing a left frontotemporal craniotomy with an anterior clinoidectomy. There was no clear plane between the tumor and the extraocular muscles; therefore, an infiltration was suspected. A biopsy of the tumor was performed and an intraoperative diagnosis of a malignant tumor was obtained. Histopathological findings were characterized by small round blue cells that were immunohistochemically positive for Ep-CAM/Ber-EP4 (epithelial marker) and CD117/c-Kit. Ki-67 positive cells totaled more than 90%. EBV was detected by EBV-encoded ribonucleic acid in situ hybridization (EBER-ISH; Figure 2). Considering the MR imaging appearance, the clinical course, and the histopathological findings, the patient was diagnosed with nonkeratinizing undifferentiated NPC, which originally occurred in the fossa of Rosenmüller and was perineurally invading along the V3 to the cavernous sinus.


Nasopharyngeal carcinoma presenting with rapidly progressive severe visual disturbance: a case report.

Kamio Y, Sakai N, Takahashi G, Baba S, Namba H - J Med Case Rep (2014)

Hematoxylin-eosin staining (A) and immunohistochemical staining for Ep-CAM/Ber-EP4 (B), CD117/c-Kit (C), Epstein–Barr virus-encoded ribonucleic acid in-situ hybridization (D), and Ki-67 (×400 magnification) (E).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4227451&req=5

Figure 2: Hematoxylin-eosin staining (A) and immunohistochemical staining for Ep-CAM/Ber-EP4 (B), CD117/c-Kit (C), Epstein–Barr virus-encoded ribonucleic acid in-situ hybridization (D), and Ki-67 (×400 magnification) (E).
Mentions: A 54-year-old Japanese man was admitted to our neurosurgical department for the investigation and treatment of acute visual loss of his left eye. He had been treated for left serous otitis media and left facial pain mimicking trigeminal neuralgia for 10 months by the otolaryngologist of a local hospital. Neurological examination on admission showed left visual acuity of no light perception and left eye movements disturbed in all directions. Gadolinium-enhanced, fat-suppressed, T1-weighted magnetic resonance (MR) imaging revealed a lesion in the left fossa of Rosenmüller, pterygopalatine fossa, sphenoid and ethmoid sinus, and the left cavernous sinus extending to orbital apex through the superior orbital fissure (Figure 1). With a tentative diagnosis of malignant skull base tumor, an endoscopic tumor biopsy from nasopharyngeal tissue was performed, but failed because the nasopharyngeal tissue appeared normal and the tumor could not be identified. Subsequently, the intraorbital tumor was explored by performing a left frontotemporal craniotomy with an anterior clinoidectomy. There was no clear plane between the tumor and the extraocular muscles; therefore, an infiltration was suspected. A biopsy of the tumor was performed and an intraoperative diagnosis of a malignant tumor was obtained. Histopathological findings were characterized by small round blue cells that were immunohistochemically positive for Ep-CAM/Ber-EP4 (epithelial marker) and CD117/c-Kit. Ki-67 positive cells totaled more than 90%. EBV was detected by EBV-encoded ribonucleic acid in situ hybridization (EBER-ISH; Figure 2). Considering the MR imaging appearance, the clinical course, and the histopathological findings, the patient was diagnosed with nonkeratinizing undifferentiated NPC, which originally occurred in the fossa of Rosenmüller and was perineurally invading along the V3 to the cavernous sinus.

Bottom Line: The histopathological diagnosis was nonkeratinizing undifferentiated nasopharyngeal carcinoma.Epstein-Barr virus was detected by in situ hybridization.Although focal radiotherapy induced remarkable tumor shrinkage and relieved ocular motor disturbance and facial pain, his visual acuity did not improve.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Neurosurgery, Hamamatsu University School of Medicine, Handayama 1-20-1, Higashiku, Hamamatsu, Shizuoka 431-3192, Japan. nsakaineurosurg@gmail.com.

ABSTRACT

Introduction: Nasopharyngeal carcinoma is one of the most difficult tumors to diagnose correctly at the initial phase because of the occasional lack of nasal symptoms. The perineural spread of the trigeminal nerve is one of the most common and important routes in the intracranial paracavernous extension of nasopharyngeal carcinoma, but visual loss is very rare.

Case presentation: We report the case of a 54-year-old Japanese man with nasopharyngeal carcinoma, who presented with rapid and severe disturbance of left monocular visual acuity and eye movement with a 10-month history of ipsilateral otitis media and facial pain. Magnetic resonance imaging revealed a lesion in the left fossa of Rosenmüller, pterygopalatine fossa, sphenoid and ethmoid sinus, and the left cavernous sinus extending to the orbital apex through the superior orbital fissure. The histopathological diagnosis was nonkeratinizing undifferentiated nasopharyngeal carcinoma. Epstein-Barr virus was detected by in situ hybridization. Although focal radiotherapy induced remarkable tumor shrinkage and relieved ocular motor disturbance and facial pain, his visual acuity did not improve.

Conclusion: The awareness of cranial nerves in addition to intracranial and orbital apex involvement, as in this case, is important for appropriate diagnosis and treatment planning of nasopharyngeal carcinoma.

Show MeSH
Related in: MedlinePlus