Primary Intracranial Adenoid Cystic Carcinoma: A Case Report

Abstract

Adenoid cystic carcinoma (ACC) is a rare slow-growing but aggressive malignant tumor arising from the epithelial cells of mucous-secreting glands. Primary intracranial ACC is one of the rarest entity. We report a case of a 61 years old male presenting with difficulty in swallowing, slurring of speech, generalized body weakness. Patient had residual right cerebellopontine angle (CPA) mass causing midline shift and fourth ventricular obstruction on MRI. Patient underwent right retrosigmoid craniotomy with excision of CPA mass. Histopathological examination confirmed the case as primary intracranial ACC.

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CASE REPORT J Nepal Med Assoc 2025;63(282):117-9
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doi: 10.31729/jnma.8890
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______________________________________
Correspondence: Dr. Pralisha Maharjan, Department of
Laboratory Medicine and Pathology, Nepal Mediciti Hospital,
Sainbu, Lalitpur, Nepal. Email: pralisha.mhzn@gmail.com,
Phone: +977-9849113661.
Primary Intracranial Adenoid Cystic Carcinoma: A Case Report
Pralisha Maharjan,1 Gopi Aryal,1 Reena Rana,1
1Department of Laboratory Medicine and Pathology, Nepal Mediciti Hospital, Lalitpur, Nepal.
ABSTRACT
Adenoid cystic carcinoma (ACC) is a rare slow-growing but aggressive malignant tumor arising
from the epithelial cells of mucous-secreting glands. Primary intracranial ACC is one of the rarest
entity. We report a case of a 61 years old male presenting with difculty in swallowing, slurring
of speech, generalized body weakness. Patient had residual right cerebellopontine angle (CPA)
mass causing midline shift and fourth ventricular obstruction on MRI. Patient underwent right
retrosigmoid craniotomy with excision of CPA mass. Histopathological examination conrmed the
case as primary intracranial ACC.
Keywords: adenoid cystic carcinoma; craniotomy; intracranial.
INTRODUCTION
Adenoid cystic carcinoma (ACC) is a rare slow-growing
but aggressive malignant tumor with potential of
perineural invasion. It accounts for less than 1% of all
head and neck malignancies and approximately 10%
of all salivary gland neoplasms. 1 Primary intracranial
ACC is one of the rarest entity. ACC has three main
histological patterns : solid, tubular, and cribriform.
Only eight cases of primary intracranial ACC have been
reported till date (1990-2022). 2,3 Here we report a case
of 61 years male diagnosed with primary intracranial
ACC.
CASE
A 61 years man presented with dysphagia, slurring of
speech, generalized body weakness for three months.
On clinical examination, he was conscious and
oriented, his vitals were stable and on other systemic
examinations no abnormalities were detected . On
neurological examination , higher motor function was
intact but patient had right sided lower motor neuron
facial palsy of grade III and right sided sensorineural
hearing loss, cerebellar signs were positive on right side
and had gait ataxia, gag reflex was absent and there
was no motor and sensory deficits. Hematological and
biochemical parameters were within normal limits. The
patient had significant past history of right CPA mass
for which he was operated at other medical center and
histological report was suggestive of neuroenteric
cyst. Repeated Magnetic Resonance Imaging (MRI)
showed tumor infiltrating the nerve sheath of cranial
nerves 9th , 10th and 12th on right side extending
into exocranial aspect and inferiorly along pterygoid
muscle, lateral wall of nasopharynx and right medial
temporal region for which intervention was not done.
Figure 1 A. Preoperative MRI brain revealing a
hyperintense lesion on T2-weighted image on
axial plane measuring 4.2 cm × 4.0cm × 1.4cm. B.
Postoperative MRI brain revealing heterogenous
collection at tumor resection bed in right lateral
aspect of posterior fossa where central component
of the collection displays mixed signal.

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Maharjan et al. Primary Intracranial Adenoid Cystic Carcinoma: A Case Report
The MRI done on 15th April, 2024 revealed right CPA
mass causing midline shift and fourth ventricular
obstruction (Figure 1). He underwent right retrosigmoid
craniotomy with excision of CPA mass on 23th April,
2024. Peroperatively, the lesion was greyish white to
greyish brown and firm.
On gross examination, the specimen appeared
greyish brown with largest nodular tissue measuring
1.5cmx1.2cm (Figure 2A). Cut sections showed solid
to cystic area. (Figure 2B). Remaining fragmented
tissue altogether measures 2.5x2.2cm. Entire tissue
submitted.
Figure 2. A. Gross image appeared greyish white to
greyish brown nodular tissue. B. Cut section showed
solid to cystic area.
Microscopic examination revealed tumor tissue
predominantly composed of cribriform growth
pattern with nest of tumor cells with discrete, rounded
punched out gland like spaces filled with eosinophilic
to basophilic material (Figure 3A). Area of tubular
pattern with multiple ducts and tubules like structure
are lined by small uniform cuboidal epithelium (Figure
3B). A focus of solid sheets of tumor cells (<30%
solid component) noted. Infiltrating tumor nests are
identified within fibrous stroma. Perineural invasion is
evident. Focal areas of reactive gliosis with dilated and
congested blood vessels are noted.
The tumor cells expressed CD117 positivity in luminal
layer and P40 and CK7 in myoepithelial layer but
was negative for S100 and Ki-67 was nearly 12%
(Figure 4). With all these histological features and
immunohistochemical reports, the case was finally
diagnosed as intracranial ACC.
To look for the primary site of the lesion, further
evaluation like chest X-ray, ultrasonography of
abdomen, and whole-body MRI was performed but
the primary site remained unknown so we concluded
the tumor to be intracranial in origin and diagnosed
it as primary intracranial ACC. Patient was managed
conservatively with intravenous antibiotics and all
other supportive measures. However, the patient
didn’t receive postoperative radiotherapy and he
passed away on 18th post operative day (POD).
Figure 3. A.Tumor tissue predominantly composed
of cribriform growth pattern with nest of tumor
cells with discrete, rounded punched out gland like
spaces filled with eosinophilic to basophilic material
(Hematoxylin and Eosin stain x10). B. Area of tubular
pattern with multiple ducts and tubules like structure
are lined by small uniform cuboidal epithelium
(Hematoxylin and Eosin stain x40).
Figure 3. A. The tumor cells expressed CD117
positivity in luminal layer. B and C.The tumor cells
expressed P40 and CK7 in myoepithelial layer. D. Ki-
67 was nearly 12%
DISCUSSION
ACC is a rare slow-growing but aggressive malignant
tumor. More than half of ACC arises from the minor

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Maharjan et al. Primary Intracranial Adenoid Cystic Carcinoma: A Case Report
salivary glands but it can also affect the major salivary
glands, paranasal sinuses, larynx, and trachea and
hard palate is most commonly affected intraoral
site.4 Though direct extension of the tumor or
invasion along cranial nerves is common but primary
intracranial ACC is extremely rare. Only eight cases of
primary intracranial ACC have been reported till date
(1990-2022) 2,3 where the youngest case was 34 years
and the eldest was 71 years, clinical features varied
from headache, epistaxis to hemianopia and left
facial region numbness and most common location
was cavernous sinus. Cases underwent operation,
radiosurgery and radiotherapy. Among them 3 cases
died after management while 2 cases were alive with
disease and 3 were alive without disease.
In our case, the mass was seen in right CPA causing
midline shift and fourth ventricular obstruction.
Literatures have shown that the tumors arising close
to the skull base may cause ocular dysmotility, and
cranial nerve (CN) palsies involving IX, X, XI, and XII.5,6
It is thought to arise from existing bucconasal cell
rests.7 Among the primary intracranial ACCs reported
in the literature, Gasserian ganglion, middle cranial
fossa, frontal lobe, cavernous sinus and posterior fossa
comprise majority of locations.8 Similar to findings
of these literatures, our case also had multiple nerve
sheath tumors (right IX CN, X CN, XII CN), extending
into exocranial aspect and inferiorly along pterygoid
muscle, lateral wall of nasopharynx and right medial
temporal region as seen on MRI.
Age of the patient, tumour site, clinical stage,
histological type with or without nerve involvement
are the important factors influencing prognosis
however the survival at 5,10, and 20 years around
68%, 52% and 28% respectively after decompression
surgery and postoperative radiotherapy.9The need
for early diagnosis and radical tumor resection, if
possible , coupled with postoperative radiation should
be emphasized so that local disease control and better
long-term survival will be achieved.10 However, the
patient didn’t receive postoperative radiotherapy and
he passed away on 18th POD .
Acknowledgement: I appreciate Dr. Shoshan
Raj Acharya, Dr. Arnav Singh Parmar for their
histopathological insights, and Mr. Ajay Shah for slide
preparations.
Consent: JNMA Case Report Consent Form was signed
by the patient.
Conflict of Interest: None.
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