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Isolated Sphenoid Sinus Lesions: Experience with a Few Rare Pathologies

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Introduction The sphenoid sinus is often neglected because of its difficult access. The deep position of the sphenoid sinus hinders early diagnosis of pathologies in that location. Delayed diagnosis can cause serious complications due to proximity to many important structures. Objectives The aim of this study is to demonstrate different pathologies which can affect the sphenoid sinus and elucidate the findings. Methods Cases of isolated sphenoid sinus lesions encountered in the neurosurgical setting which had rare pathologies are discussed. Pathologies such as Langerhans cell histiocytosis, solitary plasmacytoma, chordoma, pituitary adenoma, leiomyosarcoma, fungal infection, and mucocele which appeared primarily in sphenoid sinus are discussed along with their imaging features and pathological findings. Conclusion Multitude of different pathologies can occur in sphenoid sinus. Detailed preoperative imaging is very helpful, but transnasal biopsy and histological study are required often for definitive diagnosis. The possible advantages of early diagnosis before spread of pathology for prognosis cannot be overemphasized.
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Volume 8 Issue 1 January - March 2017
Journal of Neurosciences in Rural Practice • • April-June Volume 7Issue 22016 Pages 00-00
Spine
8 mm
Sadashiva N, Nandeesh BN, Shukla D, Bhat D,
Somanna S, Devi BI. Isolated sphenoid sinus lesions:
Experience with a few rare pathologies.
J Neurosci Rural Pract 2017;8:107-13.
107
© 2017 Journal of Neurosciences in Rural Practice | Published by Wolters Kluwer - Medknow
Introduction: The sphenoid sinus is often neglected because of its difcult access. The deep
position of the sphenoid sinus hinders early diagnosis of pathologies in that location. Delayed
diagnosis can cause serious complications due to proximity to many important structures.
Objectives: The aim of this study is to demonstrate different pathologies which can affect the
sphenoid sinus and elucidate the ndings. Methods: Cases of isolated sphenoid sinus lesions
encountered in the neurosurgical setting which had rare pathologies are discussed. Pathologies
such as Langerhans cell histiocytosis, solitary plasmacytoma, chordoma, pituitary adenoma,
leiomyosarcoma, fungal infection, and mucocele which appeared primarily in sphenoid sinus are
discussed along with their imaging features and pathological ndings. Conclusion: Multitude
of different pathologies can occur in sphenoid sinus. Detailed preoperative imaging is very
helpful, but transnasal biopsy and histological study are required often for denitive diagnosis.
The possible advantages of early diagnosis before spread of pathology for prognosis cannot be
overemphasized.
Keywords: Chordoma, Langerhans cell histiocytosis, leiomyosarcoma, mucocele,
pituitary adenoma, plasmacytoma, sphenoid sinus
Isolated Sphenoid Sinus Lesions: Experience with a Few Rare
Pathologies
Nishanth Sadashiva, B. N. Nandeesh1, Dhaval Shukla, Dhananjaya Bhat, Sampath Somanna, Bhagavatula Indira Devi
Address for correspondence: Dr. Dhaval Shukla,
Department of Neurosurgery, National Institute of Mental Health
and Neurosciences, Bengaluru ‑ 560 029, Karnataka, India.
E‑mail: neurodhaval@rediffmail.com
CaseIllustrations
A 12‑year‑old male child had headache for 2 months and
diplopia for 15 days. Child had right 6th nerve paresis. CT
showed a well‑dened homogenously enhancing sphenoid
sinus lesion. Extending superiorly and on both sides.
MRI showed T1‑hypo‑isointemse, T2‑hyper‑isointense,
and heterogeneously enhancing mass in sphenoid sinus.
Patient underwent endoscopic transnasal decompression of
lesion. Lesion was grayish white granular with moderate
vascularity. Histopathology showed polymorphous cellular
lesion with multiple conglomerate conuent histiocytic cells,
with giant cells and mixed inammatory cells. Hemorrhage
and necrosis were noted. Histiocytes had a moderate amount
of cytoplasm with oval nucleus and central constriction
and groves. Immunohistochemistry (IHC) showed CD1a
positive with MIB‑1 labeling index of 10%–12% [Figure 1].
Diagnosis of Langerhans cell histiocytosis (LCH) was
made. Patient was conservatively managed, and the child is
asymptomatic at 18 months follow‑up
A 43‑year‑old male had headache for 1 year, double vision
for 8 months, and diminution of vision in both eyes for
Introduction
As more and more intracranial lesions are approached
through the endoscopic transnasal route, neurosurgeons
nowadays come across a lot of lesions in the sphenoid sinus
with or without extension to intracranial cavity. We hereby
describe our experience with few of the lesions which were
in the sphenoid sinus. Isolated sphenoid sinus lesions are
uncommon, accounting for 1%–2.7% of all paranasal sinuses
lesions.[1] They usually present with nonspecic symptoms
and are inaccessible by physical examination. The clinical
presentation involves vague headaches and may be associated
with purulent rhinorrhea, retropharyngeal drip, nasal
obstruction, abnormal vision, and nerve decits.[2]
The sphenoid sinus is anatomically in close relationship with
the brain, meninges, optic nerve, internal carotid artery (ICA),
cavernous sinus, and cranial nerve (oculomotor, trochlear,
ophthalmic, and maxillary branches of the trigeminal nerve
and abducens).[3] Delayed diagnosis and treatment can result in
serious complications. Conventional radiographs do not allow
a good view of the sphenoid sinus due to its location in the
central skull base. Due its deep position, imaging technologies
such as computed tomography (CT), magnetic resonance
imaging (MRI), and endoscopic biopsy play a signicant role
in understanding different pathologies affecting this region.
The aim of this study is to demonstrate different pathologies
which can affect the sphenoid sinus and elucidate the ndings.
Departments of Neurosurgery
and 1Neuropathology,
National Institute of Mental
Health and Neurosciences,
Bengaluru, Karnataka, India
ABSTRACT
Case Series
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DOI:
10.4103/0976-3147.193540
How to cite this article: Sadashiva N, Nandeesh BN, Shukla D, Bhat D,
Somanna S, Devi BI. Isolated sphenoid sinus lesions: Experience with
a few rare pathologies. J Neurosci Rural Pract 2017;8:107-13.
Sadashiva, et al.: Isolated sphenoid sinus lesions
108 Journal of Neurosciences in Rural Practice ¦ Volume 8 ¦ Issue 1 ¦ January - March 2017
1 month. He was on antiretroviral therapy for human
immunodeciency virus (HIV) infection since 3 years.
CD4 count was 320 cells/mm3. He was previously treated
for pulmonary tuberculosis 1 year back. The visual
acuity was 6/18 bilaterally with left lateral rectus palsy.
CT scan showed a sphenoid‑sellar lesion. MRI showed
well‑dened lobulated homogeneous lesion involving
the sphenoid sinus, sella, and clivus. The lesion was
homogeneously isointense to gray matter on T2‑weighted
image (T2WI) and hyperintense on T2‑weighted
image (T1WI) without any diffusion restriction. There
was no evidence of blooming on gradient images. Strong
homogeneous postcontrast enhancement was noted. The
lesion was seen invading bilateral cavernous sinuses.
Patient underwent endoscopic transnasal decompression
of the lesion. Histopathology showed cellular lesion with
monomorphic small to medium cells in sheets, inltrating
bone which was strongly positive for lambda light chain
and few cells for kappa chain, negative for cytokeratin,
chromogranin, and pituitary hormones [Figure 2]. Features
were suggestive of plasmacytoma. Systemic workup for
myeloma was negative. Patient underwent radiotherapy,
and MRI performed 1 year later showed no recurrence
A 41‑year‑old male had headache and blurring of vision
for 1 year, which increased in last 10 days. There was
no perception of light in the left eye, and vision in the
right eye was 6/9. CT showed well‑dened sphenoid
sinus lesion with sellar and suprasellar extension. MRI
showed an expansile mass lesion arising from the
sphenoid sinus expanding the sinus uniformly. Pituitary
was seen separately and displaced superiorly. Lesion
showed hypointense signal on T1WI, hyperintense
on T2WI with scattered areas of microbleeds within.
Diffusion‑weighted imaging showed predominantly
facilitated diffusion. On postcontrast study, variegated
pattern of enhancement was seen within the lesion. Optic
nerves appeared to be compressed at the optic canal level
with superior displacement of the optic chiasm. Bilateral
ICA was splayed by the mass lesion without any luminal
narrowing. Patient underwent endoscopic transnasal
transsphenoidal decompression of soft suckable vascular
lesion inside sphenoid sinus. Histopathology showed a
cellular neoplasm with prominent myxoid stroma, small
epithelial‑like dispersed cells with eosinophilic cytoplasm
arranged in sheets, and trabeculae, interspersed with
characteristic vacuolated physaliphorous cells [Figure 3].
Impression was chordoma. MRI at 3 months after surgery
showed small residue for which radiotherapy was given
A 52‑year‑old male had headache for 5 years, and nasal
bleeding for 1 month. He is a known case of Parkinson’s
disease on treatment. Although his visual acuity was
normal, he had right temporal eld defect. MRI was
suggestive of primary sphenoid sinus lesion, with sellar
extension. Patient underwent transnasal decompression of
soft suckable vascular lesion. There was no bony defect
seen in the sellar oor [Figure 4]. Histopathology was
suggestive of ectopic pituitary adenoma. He underwent
radiotherapy for residual tumor. MRI performed at
7 months after surgery showed very small residual lesion,
which was managed conservatively
A 53‑year‑old male had headache for 8 months,
decreased vision in the right eye, and numbness of face
for 1 month. Visual acuity was limited to perception of
light. MRI showed a sphenoid sinus lesion T1‑isointense,
T2‑hypointense, and well enhancing on contrast. CT scan
showed lesion extending till posterior nasal cavity with
bone erosion. Patient underwent endoscopic transnasal
Figure 1: (a) Magnetic resonance imaging T1‑weighted image sagittal view showing an
isointense mass lesion in the sphenoid sinus, (b) the lesion is enhancing on contrast on
gadolinium contrast sagittal view, and (c) coronal view. (d) Shows a microphotograph
with clusters of histiocytoid cells with a convoluted, indented nucleus admixed with
giant cells and mixed inammatory cells (H and E, ×400). Inset showing the tumor
cells positive for CD1a immunostain (×400) suggestive of Langerhans cell histiocytosis
ab
cd
Figure 2: (a) Showed a T1‑weighted sagittal view showing an isointense mass lesion
located in the sphenoid sinus with (b) showing postgadolinium contrast enhancement of
the lesion in T1‑weighted sagittal images and (c) coronal images. (d) Microphotograph
showing respiratory mucosa (*) with a submucosal cellular inltrating neoplasm (#) which
is inltrating the bone ($) as well (H and E, ×100). Inset shows the tumor composed of
plasmacytoid cells (H and E, ×200) suggestive of plasmacytoma
ab
cd
Sadashiva, et al.: Isolated sphenoid sinus lesions
109
Journal of Neurosciences in Rural Practice ¦ Volume 8 ¦ Issue 1 ¦ January - March 2017
decompression of lesion which was seen growing
predominantly posterior and inferior, occluding choana
superiorly till cranial base and nasal cavity. Lesion was
rm, moderately vascular with cartilaginous feel. Dura was
not involved. Histopathology of lesion showed variably
cellular lesion composed of fragments of interlacing and
whorled fascicles of spindle cells having oval at ended
nuclei and moderate eosinophilic cytoplasm. There
were signicant anisonucleosis and scattered mitosis
(2–3/10 hpf) and evidence of bone invasion. IHC showed
tumor cells to be strongly positive for vimentin and
smooth muscle actin, negative for desmin and S‑100.
MIB‑1 labeling was 8%–10% [Figure 5]. Features were
suggestive of leiomyosarcoma (LMS). Patient was referred
to oncologist for adjuvant therapy and is not available for
follow‑up
A 48‑year‑old diabetic female had double vision for
2 days. She had isolated left 6th nerve paresis. MRI
showed a T1‑hyperintense, T2‑hypointense right‑sided
sphenoid sinus lesion, which was showing peripheral
contrast enhancement. Patient underwent transsphenoidal
biopsy. Histopathological examination revealed fungal ball
with admixture of broad aseptate fungi with thin septate
acute angled hyphae with chains of spores and conidia
indicative of Aspergillus. Some hyphae were pigmented.
There were no eosinophilic inltrate or Charcot‑Leyden
crystals [Figure 6]. Final impression was combined
zygomycosis and Aspergillus infection without any
host response. Patient was put on voriconazole and was
asymptomatic at 13 months follow‑up
A 61‑year‑old male presented with progressive visual
deterioration in the left eye, and ptosis for 1 month. He
was previously operated for a mucocele about 5 years back.
Patient underwent transnasal resection of lesion [Figure 7].
Diagnosis was sphenoid sinus mucocele.
Discussion
The sphenoid sinus has often been neglected because of its
isolated location and difcult access. The deep position of
the sphenoid sinus prevents and hinders early diagnosis of
pathologies in that location.[4] Delayed diagnosis and treatment
can result in serious complications due to proximity of
different vital structures. Imaging technologies such as CT
and MRI along with the endoscopic surgical techniques have
revolutionized the treatment strategies for lesions involving
sphenoid sinus.
As more and more neurosurgical procedures are done through
endoscopic transsphenoidal route, there is an increase in the
number of sphenoid sinus lesions managed by neurosurgeons.
Isolated sphenoid sinus lesions usually present with headache,
followed by ophthalmological and nasal symptoms.[5] Of these
lesions, 72% are inammatory, 16% are neoplastic, and about
12% are because of other causes such as cerebrospinal uid
leak and brous dysplasia.[6] Although CT is investigation
of choice, it is difcult to distinguish tumor from soft tissue
swelling and secretions. MRI is thought to be complementary,
but bony septae and malignant osseous lesions are poorly
distinguished. Cases seen in neurosurgery are commonly
evaluated by MRI. We presented imaging and histopathological
ndings of seven cases of isolated sphenoid sinus lesion
with different diagnosis. In the above‑illustrated cases, only
lesions involving the sphenoid sinus were included. Patients
were evaluated by objective ear, nose, and throat examination
Figure 4: (a) Shows a T2 weighted sagittal magnetic resonance imaging image with
a heterogeneously iso‑hyperintense mass located in the sphenoid sinus, (b) the lesion
is enhancing heterogeneously on postgadolinium contrast sagittal magnetic resonance
imaging and (c) axial magnetic resonance imaging. (d) Microphotograph showing the
neoplasm composed of closely packed acinar clusters of uniform round to polyhedral
neuroendocrine cells (H and E, ×400) suggestive of pituitary adenoma
ab
cd
Figure 3: (a) Shows a T2‑weighted sagittal magnetic resonance imaging with a
hyperintense mass lesion located in the sphenoid sinus which is enlarged. (b) Shows
the lesion in not enhancing on T1‑weighted postgadolinium contrast compared to
(c) axial T1‑weighted image with a iso‑hypointense mass located in the sphenoid sinus.
(d) Microphotograph showing a myxoid neoplasm with diffusely scattered polyhedral
cells having a vacuolated bubbly cytoplasm (Physaliphorous cells) in a highly myxoid
stroma (H and E, ×400) suggestive of chordoma
ab
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Sadashiva, et al.: Isolated sphenoid sinus lesions
110 Journal of Neurosciences in Rural Practice ¦ Volume 8 ¦ Issue 1 ¦ January - March 2017
followed by imaging using MRI and a few cases with a CT as
well. Isolated sphenoiditis due to infection is a common cause
of such lesions, and hence a course of antibiotics for 3 weeks
was given in cases where there was no suspicion about some
other lesion. Such lesions were not included. We now discuss
each pathological entity seen in sphenoid sinus.
LCH is a rare disorder with excessive proliferation of
pathologic Langerhans cells. It is mainly seen in children and
adolescents, but may occur in any age group.[7] It is commonly
found in males, with a ratio of 2.5:1. The etiology of LCH
is not well dened, and there is a lack of understanding with
inconsistencies in the literature.[8] LCH is seen as hyperintense
lesion on T2 and hypointense on T1‑weighted MRI. LCH
occurring in sphenoid sinus is very rare, and only a few
cases have been described, especially involving neighboring
structures.[9‑11] When involving only skull base, various
modalities such as direct radiotherapy, only biopsy, biopsy
and radiotherapy, or excision and radiotherapy have been
reported with comparable efcacy. Our reported case was a
child; hence he underwent decompression of lesion followed
by observation.
Plasmacytomas are localized plasma cell tumors which can
occur either in tissues (extramedullary) or in bones (solitary
bone plasmacytomas).[12] Intracranial plasmacytomas usually
present in the skull base and arise from the bones or from
the soft tissue of the nasal cavity and paranasal sinuses.[13]
Extramedullary plasmacytomas constitute approximately 3%
of all neoplasms originating from plasma cells. They generally
display a destructive course.[14] Till date, only twenty cases
of sphenoid sinus plasmacytomas have been reported.[14]
Our reported case was HIV positive leading to possibility
of fungal, which was ruled out by IHC. There was no
systemic involvement, and diagnosis of isolated sphenoid
plasmacytoma was conrmed. Although the outcome of
solitary plasmacytoma is variable, our patient was recurrence
free after adjuvant therapy at 12 months follow‑up.
Chordomas are midline tumors of the central nervous system,
which arise from remnants of the primitive notochord where
the heterotopic rests are usually situated extradurally within the
bones of the axial skeleton. They are equally distributed in the
skull base (32%), mobile spine (32.8%), and sacrum (29.2%),
and of all intracranial tumors, skull base account for only
0.1%–0.2% of all chordomas.[15] Although clival chordoma is a
well‑known entity, our case was unique as it was predominantly
intrasphenoidal leading to possibility of alternate diagnosis.
There are only a few cases where chordoma has been reported
to be arising predominantly from sphenoid sinus.[16‑19] Although
the role of radiotherapy is controversial, we still chose to treat
this patient for residual lesion.
Ectopic sphenoid sinus pituitary adenoma by denition is the
tumors centered within the sphenoid sinus and demonstrated,
by imaging studies or intraoperative examination, a normal
sella turcica without a concurrent pituitary adenoma.[20]
Ectopic pituitary adenomas are derived from embryologic
remnants along the path of migration of Rathke’s pouch,
and more than fty ectopic pituitary adenomas have been
reported.[21] Ectopic pituitary adenomas may be found in the
sphenoid sinus region, clivus, parapharyngeal space, nasal
cavity and nasopharynx, hypothalamus, third ventricle, and in
the suprasellar locations.[22] About two‑thirds are hormonally
active adenomas.[23] Our reported case was a nonfunctional
pituitary adenoma which was managed surgically and with
radiotherapy for the residual lesion.
Figure 6: (a) Shows a T2‑weighted sagittal magnetic resonance imaging with a
hypointense mass lesion located inside the sphenoid sinus. (b) The lesion is not
enhancing peripherally on postgadolinium T‑weighted images (c) and in coronal
images. (d) Microphotograph showing ulcerated mucosa with a colony of fungal hyphae
morphologically resembling Aspergillus (H and E, ×100) inset shows the fungal colony
with slender septate and branching hyphae (H and E, ×400)
ab
cd
Figure5: (a) Showing a T2‑weighted sagittal view with a heterogeneously iso‑hypointense
mass lesion located in the sphenoid sinus. (b) Shows heterogonous enhancement of lesion
after gadolinium administration, (c) lesion is isointense on plain T1‑weighted images.
(d) Microphotographs showing a cellular spindle cell tumor with interlacing fascicles;
inset (1) showing the increased MIB‑1 labeling and inset (2) showing positive staining for
smooth muscle actin. Vimentin positive but negative for S100, desmin, creatine kinase,
and CD99 suggestive of leiomyosarcoma
ab
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Sadashiva, et al.: Isolated sphenoid sinus lesions
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Journal of Neurosciences in Rural Practice ¦ Volume 8 ¦ Issue 1 ¦ January - March 2017
LMS is a disease of the gastrointestinal and genitourinary
tracts. It has been associated with a history of irradiation,
retinoblastoma, chemotherapy, HIV, and AIDS. Approximately,
fty cases of primary sinonasal LMS have been reported so
far, but only one case is described where LMS originated in
sphenoid sinus.[24] LMS arises most frequently from smooth
muscle cells within the walls of blood vessels but may also
come from undifferentiated mesenchymal cells. Pathologically,
the tumors may range from extremely well differentiated
to anaplastic. Prognosis for primary LMS remains poor,
with overall 5‑ and 10‑year survival rates of 20% and 6%,
respectively.[25] Approximately 8%–56% of patients will develop
metastatic disease.[26] LMS of the nasal cavity and paranasal
sinuses has a more favorable clinical course, with small tumors
limited to the nasal cavity having the better prognosis.[27] Wide
local excision is treatment of choice. Tumor size at the time
of diagnosis and complete excision appear to be the most
signicant prognostic factors. Adjuvant chemotherapy with or
without radiation therapy is recommended.[24] We referred our
patient for adjuvant therapy to oncologist.
Fungal sinusitis is classied into allergic, chronic
noninvasive (fungus ball), chronic invasive, granulomatous
invasive, and acute fulminant invasive fungal sinusitis based
on histological features according to the diagnostic criteria
of deShazo et al.[28,29] The rarest occurrence of invasive
fungal sinusitis is seen in the sphenoid sinus.[30] Clinical
manifestations are dependent on the immune status of patients
given the ubiquitous nature of these organisms. The presence
of neurological ndings due to intracranial complication
should be investigated immediately. Although bone destruction
is one of the radiologic ndings of invasive fungal sinusitis,
mucor extension along the blood vessels can with intact bony
sinus walls.[31] MRI is superior modality of investigation when
intracranial symptoms are present as it reveals soft tissue
involvement.[32] Even though imaging can give a clue about
fungal sinusitis, the microbiological identication of fungus
is of paramount in the management of the infection[33] and
differentiation from allergic fungal rhinosinusitis. Advanced
invasive fungal infection of the sphenoid sinus carries
signicant mortality. Hence, early diagnosis and appropriate
treatment are crucial.[34] Although noninvasive fungal sphenoid
sinusitis rarely presents with serious complications. Diplopia
and transient vision loss have been reported in up to 3% of
cases.[30] Three most common fungal infections in the nasal
sinuses are the opportunistic genera of Aspergillus, Mucor, and
Candida, of which Aspergillus is the most prevalent and Mucor
is most invasive.[35] It is considered that surgical treatment
should aim more at radically removing the mycotic infected
lesion, rather than draining it though there is no signicant
difference in outcome even if drainage is done, especially
in invasive fungal sinusitis.[36] Postoperative progression
of disease even after antimycotic therapy may lead to fatal
outcomes.[37] Our reported case was of primary focal fungal
ball with combined zygomycosis and Aspergillus infection
without invasion, successfully treated with decompression
followed by antifungal therapy.
Mucocele is dened as the accumulation and retention
of mucoid secretion within a paranasal sinus, leading to
thinning and distension and erosion of one or several of
bony walls.[38] While primary mucoceles occur as retention
cysts of the mucous glands of sinus epithelium, secondary
mucoceles arise from the obstruction of the sinus ostium.
Sphenoid mucocele comprises 1%–2% of all mucoceles.[39]
Less than 150 cases of sphenoid sinus mucoceles have been
described in the literature.[40,41] Headache is the most common
symptom, and visual disturbance is the second most common
symptom.[38] Usually, the cranial nerves involvement brings
the patient to the physician. Our reported patient had 2nd and
3rd nerve involvement which improved after surgery, but the
exact cause of recurrent mucocele could not be found out.
Other common pathologies such as meningocele/
meningoencephalocele,[42] osteomas,[43] lymphoma,[44]
metastasis,[45] other cancers,[46,47] and rare pathologies such
as inverted papillomas,[48] epidermoids,[49] melanoma,[50]
myxoma,[51] esthesioneuroblastoma,[52] trigeminal
schwannoma,[53] and many others have been described. The
sphenoid sinus may be the starting point for primary malignant
tumors of different histological types.[54] Although CT and
MRI can help suspect a malignancy, only transnasal biopsy
and histological study allow the diagnosis. The possible
advantages of early diagnosis before spread of pathology for
prognosis cannot be overemphasized.
Financial support and sponsorship
Nil.
Conicts of interest
There are no conicts of interest.
Figure 7: (a) Shows a T2‑weighted sagittal magnetic resonance imaging with a
hyperintense mass lesion in the sphenoid sinus with enlargement of sphenoid sinus.
(b) The lesion is hyperintense on plain T1‑weighted images and (c) not enhancing on
postgadolinium injection axial and (d) coronal images
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Sadashiva, et al.: Isolated sphenoid sinus lesions
112 Journal of Neurosciences in Rural Practice ¦ Volume 8 ¦ Issue 1 ¦ January - March 2017
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... En promedio la duración de los síntomas antes del diagnóstico es de unos ocho meses 5,9-12 . El seno esfenoidal está anatómicamente en íntima relación con el cerebro, las meninges, el nervio óptico, la arteria carótida interna, el seno cavernoso y pares craneales (oculomotor, troclear, oftálmico y ramas maxilares del nervio trigémino y abducens), por lo que el diagnóstico y el tratamiento a destiempo pueden derivar en serias complicaciones que involucran a estos elementos 13,14 . La infección del seno esfenoidal en comparación con otras localizaciones se ha visto asociada a un peor pronóstico debido a que puede extenderse de forma precoz a nivel intracraneal provocando incluso meningoencefalitis, lo cual hace de la aspergilosis una enfermedad potencialmente letal 15 . ...
... En el caso de estudio se detalla que las manifestaciones clínicas de la paciente son: cefalea moderada a intensa acompañada de periodos intermitentes de secreción nasal mucopurulenta y epistaxis. Estas expresiones han sido descritas por varios autores 5,9,[12][13][14] , sin embargo, resultan altamente inespecíficas, pues se han documentado casos en los cuales la cefalea no se acompaña de rinorrea, fiebre, ni de otro síntoma que haga sospechar un cuadro de características infecciosas 15 . Así mismo, otras manifestaciones reportadas en revisiones sistemáticas que contrastan con nuestra paciente puntualizan el aumento de volumen facial, oftalmoplejía, disminución de agudeza visual, algia facial, disfunción del nervio facial, dolor dental, isquemia del paladar, alteración de conciencia y convulsiones 5,11,12 . ...
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Aspergilosis hace referencia a las enfermedades causadas por una de las casi 50 especies patógenas de Aspergillus. El compromiso fúngico por Aspergillus del seno esfenoidal se torna un desafío diagnóstico. El abordaje quirúrgico y estudio micótico del material obtenido constituirá la pauta diagnóstica-terapéutica. El avance en pruebas diagnósticas por imagen y las microbiológicas ayudan en el reconocimiento oportuno de esta entidad. Aunque se presenta principalmente en pacien-tes inmunocomprometidos, también se pueden presentar en pacientes sin morbilidad añadida. Una terapéutica eficaz es la evacuación del tejido infectado por abordaje transnasal del seno esfenoidal en combinación con medicación antifúngica adecuada. Presentamos un caso atípico de sinusitis de etio-logía fúngica en una paciente femenina de 60 años que cursó con cefalea, rinorrea mucopurulenta y epistaxis. Palabras Clave: aspergilosis, infecciones fúngicas, sinusitis del esfenoides, intervención quirúrgica, terapia combinada Aspergillosis refers to diseases caused by one of the almost 50 pathogenic species of Aspergillus. Aspergillus fungal involvement of the sphenoid sinus becomes a diagnostic challenge. The surgical approach and fungal study of the material obtained will constitute the diagnostic-therapeutic guideline. Advances in diagnostic imaging tests and microbiological tests help in the timely recognition of this entity. Although it can occur mainly in immunocompromised patients, they can also occur in patients without added morbidity. An effective therapy is the evacuation of infected tissue by transnasal approach to the sphenoid sinus in combination with adequate antifungal medication. We present an atypical case of sinus-itis of fungal etiology in a 60-year-old female patient who had headache, mucopurulent rhinorrhea and epistaxis. Introducción Aspergilosis hace referencia a las enfermedades causadas por las especies patógenas de hongos filamentosos sapró-fitos y cosmopolitas del género Aspergillus que son oportu-nistas, siendo las más comunes A. fumigatus, A. flavus, A. terreus y A. niger 1-4. Producen esporas o conidias que se pro-pagan por el aire, se adhieren a las partículas de polvo y son inhaladas por las personas depositándose en la mucosa de los senos nasales y paranasales que constituyen un ambien-te ideal (por su humedad y calidez) para su proliferación, sin embargo, la enfermedad por estos hongos es poco común, excepto en condiciones de crecimiento favorables en indivi-duos altamente susceptibles con compromiso inmunológico o metabólico (SIDA, neoplasias, neutropenia, diabéticos, pa-cientes con insuficiencia renal crónica, desnutrición crónica, estancia en UCI mayor a 21 días, y personas en tratamiento prolongado con corticosteroides y antibióticos) 3-6. El objetivo de este estudio es describir la fisiopatología, diag-nóstico y tratamiento de una enfermedad poco frecuente como la aspergilosis esfenoidal, mediante la revisión de la literatura científica y la presentación de un caso. Entre las enfermedades causadas por el género Aspergillus se encuentran las infecciones de los senos paranasales, evi-denciándose mayor prevalencia en personas con compro-Summary Resumen
... However, characteristic CT and MRI findings helped to reach the diagnosis of fungal mucocele. Since the sinus was expansile and had T1 hyper intensity, other differentials were also ruled out based on imaging findings which have been tabulated in Table 1 [7][8][9]. ...
... Differentials based on T1 hyperintensity of sphenoid sinus[7][8][9] Sphenoid sinus aspergilloma in our case Expansile, T1 hyperintense, T2 hypointense, No restriction on DWI, no enhancement on post-contrast study, hyperdense on CT Usually T1 hypointense and T2 hyperintense. Can be T1 hyperintense, however, the epicenter of the lesion is usually sellar/suprasellar while in our case, it is in sphenoid sinus. ...
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Background:Fungal sphenoid sinusitis mimicking as malignant tumor and invading the pituitary fossa is anuncommon entity. This report aims to highlight radiological salient features to help differentiate fungal lesion frommalignant tumor in sphenoidal sinus mass lesions. Case presentation:We intend to report a case of middle-aged female who presented with gradual progressivediminution of vision since 3 years complicated with acute attack of unilateral headache and numbness. Computed tomography (CT) showed hyperdense lesion involving the sphenoid sinus extending into pituitary fossa andbilateral cavernous sinuses with smooth bony remodeling. Lesion appeared hypointense on T2-weighted andhyperintense on T1-weighted images on magnetic resonance imaging (MRI). Surgical excision of the lesion wasdone and pathological examination showed fungal hyphae and aspergillus fumigatus species on culture after 2weeks of incubation. Post-operative CT revealed empty sinuses with surrounding bone remodeling. Conclusion:Combination of T1 hyperintensity, T2 hypointensity, and hyperdense sinus is a strong predictor offungal mass lesion involving sphenoid sinus. Keywords:Fungal, CT, MRI, Sphenoid sinus, Pituitary, Aspergillus
... 1 For isolated sphenoidal masses rare pathologies like Langerhans cell histiocytosis, solitary plasmacytoma, chordoma, pituitary adenoma, leiomyosarcoma, fungal infection, and mucocele should be considered in the list of differential diagnosis. 9 Histopathology shows a tumour having cells in cords and nests which are dispersed in a characteristic myxoid stroma separated by fibrous bands. 5 The cells are polyhedral with eosinophilic cytoplasm and are interspersed with a variable number of classic physalipherous cells, containing abundant vacuolated bubbly cytoplasm and a bland nucleus. ...
... Cellular atypia, mitotic figures and areas of necrosis may be present as was seen in the given case. 9 To differentiate chordoma from its mimics having chordoid morphology, positive immunohistochemistry with S100, EMA and panCK are useful. 1,10 Despite the limitations posed by the deep seated location, surgery remains the primary modality for debulking. ...
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p class="abstract">Though the parapharyngeal space is sites of primary involvement by neoplastic process, they can rarely house masses descending from a base of skull tumour. Chordoma is an uncommon tumour of the skull base and sacrococcyx. Originating from the notochordal remnants, they are locally aggressive causing lytic destruction of the adjacent bony structures, particularly in the base of the skull. The use of surgery and adjuvant high-dose proton RT is documented to produce best results. Here we report a diagnostic challenge posed by a chordoma occurring as a parapharyngeal mass in a 68 year old male.</p
... To our knowledge, only three cases of such rhinoliths were reported 2,9 , none of these occurring bilaterally. The sphenoidal sinoliths are not usually listed as isolated sphenoidal sinus lesions 10,11 . A possible reason should relate to the conventional radiographs, which do not allow a good view of the sphenoid sinus due to its location in the central skull base 10 . ...
... The sphenoidal sinoliths are not usually listed as isolated sphenoidal sinus lesions 10,11 . A possible reason should relate to the conventional radiographs, which do not allow a good view of the sphenoid sinus due to its location in the central skull base 10 . On other hand, CT, as well as Cone Beam CT, allows a good visualization of the sphenoidal sinus anatomy and pathology. ...
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Sinoliths are rarely found calculi of paranasal sinuses. The most rarely they were found in the sphenoidal sinuses. At a routine Cone Beam CT exam of a 52-year-old male patient clinically silent small sinoliths were found bilaterally in the sphenoidal sinuses and a larger posterior ethmoidal sinolith was found on the right side. To our knowledge, such multiple sinuses involvement has not been previously reported.
... Several prior studies also report immunostaining of the cellular proliferation marker MIB-1(Ki-67). Most cases had a proliferation index above 15%, with the highest at 40%, suggesting a role when histologic features are confounding [25,29,41,43]. Few cases in our review showed unusual immunohistochemical findings. ...
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Sinonasal leiomyosarcoma (LMS) is a rare and aggressive mesenchymal tumor with smooth muscle differentiation. The sinonasal tract is an unusual primary site for LMS, as scant smooth muscle exists in this location, with only 75 cases reported in the English literature including the case presented herein. Sinonasal LMS is considered an aggressive head and neck tumor with significant potential for recurrence and metastasis. Since recurrence is high and the potential for late metastasis exists, lifelong follow-up in these patients would be beneficial, especially among those with previous history of RB.
... Для хронического сфеноидита характерно латентное (скрытое) течение, которое не всегда диагностируется на ранних стадиях [13,14]. По данным ряда исследователей, частота патологических находок в клиновидной пазухе при аутопсии лиц, прижизненный диагноз «сфеноидит» у которых не был установлен, составляет от 10% до 68% [15,16]. Очень важно изучение вопросов ремоделирования в клиновидной пазухе, в том числе явлений остеита при грибковых формах [17 ]. ...
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Objectives - to analyze the cases of isolated lesions of the sphenoid sinuses and to identify the main errors in the differential diagnosis in the prehospital and treatment periods. Material and methods. The study includes the data on the treatment of 58 patients with an isolated lesion of the sphenoid sinus in the period 2015-2018. The patients' age varied from 18 to 68 years. Gender distribution: men - 21 (30.7%), women - 37 (69.3%). Results. The following surgical approaches were used: transnasal access, extended transnasal access, transetmoid access, access according to the Bolger Box technique. In one case, the transpterygoid approach was used. When performing extended access, it was possible to achieve the formation of persistent, epithelized anastomosis in all the cases. In polypous-purulent forms of sphenoiditis, the relapses of the disease were most often observed, however, only anti-inflammatory therapy was required, including a sinus irrigation through the formed anastomosis. Conclusion. Further studies are required, to examine the features/ safety and clinical efficacy of the various endosurgical approaches.
... Optic neuropathy from sphenoid sinus may arise from the spread of sinus inflammation and infection, compression by an expansible lesion, neoplasm or ischemia. [1][2][3] The clinical presentation of sphenoid sinus lesions involves vague headaches and may be associated with purulent rhinorrhoea, retropharyngeal drip, nasal obstruction, abnormal vision and nerve deficit. Inflammatory conditions appear to be the major cause of sphenoid sinus lesions accounting for 65-72% of cases, followed by neoplasm (benign and malignant) accounting for 16 to 17.5%. ...
Article
Langerhan's cell histiocytosis is an uncontrolled proliferation of dendritic cells. The involvement of skull base is rare. Variable clinical presentation and multi organ involvement often warrant a multidisciplinary approach for a successful diagnosis. We are reporting a case of 16-year-old male with sphenoid sinus Langerhan's cell histiocytosis which presented as a sudden and painless loss of vision. It is a rare entity in the diagnosis of blindness. Delayed diagnosis and treatment can result in serious complications. The radiological features and management options are discussed with a review of the pertinent literature. Keywords: : blindness; histiocytosis; langerhans-cell; sphenoid sinus.
... Optic neuropathy from sphenoid sinus may arise from the spread of sinus inflammation and infection, compression by an expansible lesion, neoplasm or ischemia. [1][2][3] The clinical presentation of sphenoid sinus lesions involves vague headaches and may be associated with purulent rhinorrhoea, retropharyngeal drip, nasal obstruction, abnormal vision and nerve deficit. Inflammatory conditions appear to be the major cause of sphenoid sinus lesions accounting for 65-72% of cases, followed by neoplasm (benign and malignant) accounting for 16 to 17.5%. ...
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Langerhan's cell histiocytosis is an uncontrolled proliferation of dendritic cells. The involvement of skull base is rare. Variable clinical presentation and multi organ involvement often warrant a multidisciplinary approach for a successful diagnosis. We are reporting a case of 16-year-old male with sphenoid sinus Langerhan’s cell histiocytosis which presented as a sudden and painless loss of vision. It is a rare entity in the diagnosis of blindness. Delayed diagnosis and treatment can result in serious complications. The radiological features and management options are discussed with a review of the pertinent literature.
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The clivus is a midline anatomical structure in the central skull base. It is affected by a wide range of non-neoplastic, benign and malignant pathologies, some of which typically affect the clivus because of its strategic location and embryological origins. Clival lesions may often be asymptomatic with occasional complaints like headache or cranial neuropathy in few. Cross-sectional imaging techniques, namely, computed tomographic scan and magnetic resonance imaging, thus, play a key role in approximating to the final diagnosis and estimating the disease extent. In this article, we highlight the important imaging features of various clival and paraclival pathologies to facilitate effective diagnosis, therapeutic planning, and management.
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A 74-year-old immunocompetent man admitted for severe retro-orbital headache was diagnosed with isolated sphenoiditis. At the time of scheduled surgery, the patient was mildly obtunded, and a head CT revealed a temporal lobe abscess. The patient underwent a left temporal craniectomy and a bilateral endoscopic sphenoid sinusotomy, which revealed gross fungal debris. The patient made a full recovery with resolution of abscess and sinus findings. Suspicion for intracranial infection should be raised in any sinus patient with neurological changes. Early diagnosis with imaging studies is extremely important for surgical drainage before permanent neurological sequelae.
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Clinical reports of primary sphenoid sinus melanoma and isolated gastric metastatic melanoma are rare. Thus, to the best of our knowledge, the present study reports the first case of isolated gastric metastasis from a sphenoid sinus melanoma. The aim of the present study was to discuss the clinicopathological and radiographic characteristics, the treatment strategy and the prognosis of sphenoid sinus metastatic malignant melanoma of the stomach. Although almost 60% of patients who succumb to melanoma exhibit gastrointestinal metastases at autopsy, antemortem diagnosis is uncommon; this is predominantly due to gastric metastatic melanoma presenting with non-specific symptoms similar to other common gastrointestinal diseases. Gastrectomy may prolong overall survival and improve the quality of life for gastric metastatic melanoma patients, and the present case emphasizes the importance of palliative surgery in such cases.
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Adenocarcinomas of the sphenoid sinus are exceptional. In this paper, we report a new case with a review of the literature. Our patient was a 45-year-old man who presented with isolated retro orbital headache. CT and MRI suspected a malignat tumor of the sphenoid sinus. The patient underwent a debulking surgery. The final pathology carried out the diagnosis of primary adenocarcinoma. The patient died several months later from radiotherapy complications. Even if adenocarcinomas of the sphenoid sinus are exceptional, they should be considered in the differential diagnosis of sphenoid sinus masses. The prognosis is poor.
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Objective This study was conducted to present the clinical outcome of invasive fungal sinusitis of the sphenoid sinus and to analyze clinical factors influencing patient survival. Methods A retrospective review of 12 cases of invasive fungal sphenoiditis was conducted. Results Cases were divided into acute fulminant invasive fungal spheonoidits (n=4) and chronic invasive fungal sphenoiditis (n=8). The most common underlying disease was diabetes mellitus (n=9). The most common presenting symptoms and signs included visual disturbance (100%). Intracranial extension was observed in 8 patients. Endoscopic debridement and intravenous antifungals were given to all patients. Fatal aneurysmal rupture of the internal carotid artery occurred suddenly in two patients. The mortality rate was 100% for patients with acute fulminant invasive fungal sphenoiditis and 25% for patients with chronic invasive fungal sphenoiditis. In survival analysis, intracranial extension was evaluated as a statistically significant factor (P=0.027). Conclusion The survival rate of chronic invasive fungal sphenoiditis was 75%. However, the prognosis of acute fulminant invasive fungal sphenoiditis was extremely poor despite the application of aggressive treatment, thus, a high index of suspicion should be required and new diagnostic markers need to be developed for early diagnosis of invasive fungal sinusitis of the sphenoid sinus.
Article
Chordomas of the skull base are extremely rare intracranial malignancies that arise from ectopic remnants of embryonal notochord. Chondroid chordoma is a subtype of chordoma possessing elements of both chordoma and cartilaginous tissue with better prognosis than classic (nonchondroid) chordomas. We herein report a case of chondroid chordoma of sphenoid sinus in a 46 years old female. We believe this is the first case of chondroid chordoma involving only the sphenoid sinus.
Article
Objective: Aimed to analyse the clinical features of the patients with sphenoid sinus mucocele, achieve earlier diagnosis and more timely intervention and decrease the occurrence of misdiagnoses. Method: A retrospective study was first conducted in patients with sphenoid sinus mucoele treated in Xiangya hospital from Jan 2000 to Jan 2015. Then literature reports on this disease were collected and analyzed from China National Knowledge Infrastructure (CNKI) and Wan Fang database. Result: We collected 82 patients with sphenoid sinus mucocele treated in Xiangya hospital. There were 52 patients presented with headache, 31 patients presented with visual impairment, 10 patients presented with cranial nerve palsy, 2 patients presented with exophthalmos, 15 patients presented with nasal symptoms, and 5 patients with no obvious symptoms. There was no significant difference for symptoms distribution between male and female patients (P > 0.05). Among 45 patients with headache as first symptom and 10 patients with ethmoid sinus mucocele, there were 18 patients and 8 patients subsequently suffering from visual impairment, respectively. We also collected 161 patients in literature except for enrolling, the 82 patients treated in Xiangya hospital, and found that headache was the most common symptom, followed by visual impairment, in the two independent cohorts. Conclusion: To the best of our knowledge, this is the study of maximum sample for sphenoid sinus mucocele in China. Headache and visual impairment are the most common symptoms for sphenoid sinus mucocele. Surgical treatment should be early performed when the desease accompanied with headache or ethmoid sinus mucocele, to avoid other complications such as visual impairment and even blindness.
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Introduction: Invasive fungal rhinosinusitis (IFR) is one of the most important causes of morbidity and mortality in immunocompromised patients, principally those with cellular immunodeficiency, with mortality ranging from 50 to 80%. Prophylaxis and early diagnosis increase the chances of successful treatment. Study design: Clinical prospective randomized study. Aim: To present cases of IFR and to compare them with data reported in the literature. Material and methods: Analysis of eleven cases of IFR confirmed by pathologist examination. Results: Aspergillus was found to be the most prevalent pathogen. Symptoms ranged from high fever in most cases to nasal discharge, ulceration of the nasal mucosa, headache and periorbital edema. Conclusion: The combination of amphotericin B and endoscopic surgery, associated or not with Caldwell-Luc surgery, showed good results. The use of liposomal amphotericin B also presented a satisfactory outcome.
Article
Introduction: Mucocele is a pseudo-cystic tumor of the paranasal sinuses. Despite its benign histological nature, it is aggressive towards neighboring structures (orbit and brain). Our aim was to study the epidemiological, clinical, therapeutic, and evolution aspects of this pathology. Patient and methods: We conducted a retrospective study over a period of 9 years on 32 patients operated on and followed for mucocele in our department. Results: Mean age was 43.28 years with a sex ratio to 1. Mucoceles were located in the fronto-ethmoid sinus (27 cases), the maxillary sinus (3 cases) and the sphenoid sinus (2 cases). The most common symptoms were periorbital swelling and exophthalmia. CT scan confirmed the diagnosis in the majority of cases. MRI was performed in 3 patients. Surgery consisted in a large marsupialization by endonasal approach in 30 cases, and by a combined approach in two cases. A recurrence was observed in two patients after a mean period of 18 months. Discussion: Mucocele is a benign and expansive pseudo-cystic tumor, affecting mostly adults and developing in the paranasal sinuses. Clinical symptoms are not specific. It may reveal itself by ophthalmic or intracranial complications. Diagnosis is based on imaging (CT and MRI). Endonasal surgery has become the gold standard for the treatment of mucoceles and is endowed with low morbidity.
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The purpose is to report a case of chordoma, which orignated from sphenoid sinus and encroach on sella, metasella and clivus. We comprehensively analyzed the CT and MRI information and diffrentiated the illness from the commonly encountered diseases of sphenoid sinus and sellato improve the accuracy before surgery.