CASE DESCRIPTION
An 80-year-old female patient presented to our department with a chief
complaint of swelling over the right side of the face in the past 1
year. The swelling gradually increased in size within 10 months. The
patient noticed a rapid increase in swelling over the last two months
which was associated with intermittent pricking pain. The patient also
reported having difficulty in opening her mouth and a lack of sensation
in the lower lip on the right side. She gave a history of exfoliation of
all her teeth by the age of 60, and there was no history of discomfort
or swelling, which made her visit a health practitioner.
On extra oral examination, a solitary oval-shaped swelling was noted
along the right posterior body and ramus of the mandible measuring
approximately 5 cm X 3 cm in the greatest dimensions, roughly oval in
shape with a smooth surface. The skin overlying the swelling appeared to
be smooth and tense. On palpation, the swelling was warm, tender, firm
to hard in consistency, and had a smooth surface and well-defined
margin. Lymph nodes were not palpable [Figure 1].
Intra orally, a solitary irregularly shaped swelling measuring
approximately 4 cm X 2 cm in its largest dimensions was noted along the
posterior third of the lower right edentulous alveolar ridge causing
expansion of the alveolus bucco - lingually. The swelling was pale pink
in color and had a lobulated surface. Obliteration of buccal vestibule
and tense buccal mucosa were also noted [Figure 2]. On palpation,
the swelling was afebrile, firm with areas of tenderness over the
superior aspect of the swelling.
A provisional diagnosis of ameloblastoma was made considering the
history, age, aggressive nature of the tumour, and location.
Differential diagnoses of malignant non-odontogenic tumours, and
metastatic tumours to the jaw were also considered. Basic investigations
were undertaken, including hematological parameters, renal function
tests and liver function tests, an orthopantomogram (OPG), and CT scan.
Panoramic radiography revealed a large radiolucent lesion, located over
the right side of the mandible involving the entire ramus and body up to
the right para-symphysis region. It measured approximately 4 cm X 7 cm
roughly oval in shape with an epicenter at the ramus of the mandible and
had a partially corticated border. The internal structure appeared to be
completely radiolucent. Expansion and thinning of the anterior border,
and thinning of the posterior and inferior border of the mandible were
evident with few areas of perforations at the anterior border along the
ascending ramus of the right side of the mandible [Figure 3].
A computed Tomography scan demonstrated a uniformly expansile,
oval-shaped osteolytic lesion around the right side of the mandible,
involving the ramus, coronoid, and body, measuring approximately 4 cm X
4.84 cm X 7.5 cm in its largest dimension. The internal structure
appeared to be hypodense, with radiodensity equivalent to soft tissue.
The lesion had caused expansion, thinning, and loss of cortex in
bucco-lingual, antero-posterior, and superior-inferior directions.
Irregular bone destruction with bony spicule was noted over the body of
the mandible in relation to the para symphysis region. The inferior
alveolar canal could not be visualized due to lesion invasion [Figure
4].
Screening CT of the thorax and USG abdomen revealed no evidence of any
lesion. Liver function tests and renal function tests showed normal
values.
On histopathological evaluation, Hematoxylin and eosin-stained section
showed a nonencapsulated tissue stroma with proliferating tumour cells
arranged in solid sheets and cords. The tumour cells were uniformly
arranged round cells with round oval-shaped nuclei, scanty pale
cytoplasm, and showing abundant mitotic figures. Spindle-shaped cells
arranged in pagetoid fashion were also noted with few areas showing
blood vessels along with extravasated RBCs, and focal areas of bony
trabeculae infiltrated by tumour cells [Figure 5].
The immunohistochemical staining was performed. The percentage of the
tumour marker Ki-67 was positive and the proportion was assessed to be
90%. Additionally, the markers cytokeratin (AE1/AE3), synaptophysin,
and chromogranin A were found to be positive. Hence, a diagnosis of
small cell neuroendocrine carcinoma was made.
Resection and chemo and radiotherapy were planned for the patient. But
patient passed away due to post covid complications within one month of
diagnosis; hence definitive treatment could not be given.