Presentation
Chronic left facial pain.
Patient Data
The contrast-enhanced CT study shows poorly enhancing mass eroding the left sphenoid sinus and skull base with extension into the left middle cranial fossa. The mass also appears eroding through the left foramen ovale and foramen rotundum into the left infratemporal fossa.
MRI study shows heterogeneously enhancing soft tissue mass measuring about 3.5 X 2.7 cm in the axial plane and 4.1 cm in the craniocaudal aspect, which is seen centered in the lower portion of the left lateral recess of the sphenoid sinus, and appears protruding medially and superiorly into the left sphenoid sinus cavity, while posteriorly and superiorly appears eroding the lower medial portion of the greater wing of the sphenoid, extending into the medial aspect of the left middle cranial fossa, exerting mild mass effect upon the lower medial aspect of the left temporal lobe, without obvious signs of parenchymal infiltration, partially eroding the lateral wall of the left carotid canal, where it becomes closely adherent to the petrous, lacerum and cavernous segments (C2, C3 and C4) of the left internal carotid artery.
This mass also infiltrates the osseous portion of the left Eustachian tube, with subsequent development of secretory otitis media, manifested by fluid signal intensity filling the left middle ear cleft and mastoid air cells. It also appears to obliterate the foramen rotundum and protrudes inferiorly through the left foramen ovale, which appears enlarged and remodeled, into the left infratemporal fossa.
HISTOPATHOLOGY REPORT
CLINICAL DATA:
Nasopharyngeal mass. CT-scan: 3.4 x 3 x2 cm enhanced ill-defined left nasopharyngeal soft tissue mass invading the left para-pharyngeal and prevertebral spaces, destructing the skull base and the left sphenoid sinus, R/O malignancy.
GROSS DESCRIPTION:
Received multiple fragments of white-tan tissue measuring in aggregate 1.7 x 1.7 x 1.5 cm, submitted entirely for examination.
MICRO DESCRIPTION:
Sections show an ill-defined infiltrative tumor composed of atypical basaloid-like cells mostly arranged in tubular and cribriform sieve-like structures with pseudo glandular lumina containing faintly basophilic basement membrane material. Few foci of solid growth pattern - 10% are also noted.
DIAGNOSIS:
NASOPHARYNGEAL MASS, ENDOSCOPIC BIOPSY:
ADENOID CYSTIC CARCINOMA, PREDOMINANTLY CRIBRIFORM AND TUBULAR PATTERNS, GRADE II.
Case Discussion
Adenoid cystic carcinomas (ACC) are considered low-grade rare malignant tumors with a high tendency for perineural spread and local recurrence. It accounts for less than 1% of all head and neck tumors, commonly arise from the salivary glands (10% of all salivary gland neoplasms) and rarely have an origin from the nasal sinuses, such as in this case.
Adenoid cystic carcinoma of the paranasal sinuses is an aggressive tumor that warrants early diagnosis. Imaging is essential to rule out perineural spread, which if present indicates poor prognosis. ACC of the sphenoid sinus are difficult to treat, due to its proximity to vital structures.
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