PMC6339750_01
Sagittal and coronal contrast-enhanced CT images of the neck show a large, heterogeneously enhancing and locally aggressive mass centered at the hard palate to the left of midline. Mass erodes the hard palate (yellow arrow) and extends superiorly into the nasal cavity with extensive bony remodeling (blue arrows). Large exophytic component projects into the oral cavity (red arrow).
CRIPA2019-3863270.001.jpg
PMC6339750_01_CRIPA2019-3863270.001.jpg
fig1
Representative images of near-total maxillectomy specimen measuring 9.2 x 6.5 x 6.5 cm overall. Examination revealed a 7.0 x 6.5 x 5.2 cm light tan, solid tumor replacing the majority of the hard palate.
CRIPA2019-3863270.002.jpg
PMC6339750_01_CRIPA2019-3863270.002.jpg
fig2
Sectioning revealed a light tan, solid tumor, measuring 7.0 x 6.5 x 5.2 cm (Figure 2).
Representative images of oral mucosa with changes consistent with a malignant glandular epithelium neoplasm. The neoplastic clear cells were arranged in nests, cords, and anastomosing trabeculae embedded in hyalinized, acellular, predominantly basophilic stroma. The tumor demonstrated a nodular infiltrative growth pattern, lacked overt ductal differentiation, and showed several areas of necrosis, including nests with comedo type necrosis.
CRIPA2019-3863270.003.jpg
PMC6339750_01_CRIPA2019-3863270.003.jpg
fig3
Histologic examination revealed similar findings to those described by the outside hospital: a poorly demarcated lesion consisting of nests of pleomorphic and hyperchromatic clear cells (Figure 3).
Immunohistochemical staining showing tumor positivity for SMA (smooth muscle actin) and calponin. CK7 was overall negative with a small fraction of the tumor showing patchy positivity. EMA was negative.
CRIPA2019-3863270.004.jpg
PMC6339750_01_CRIPA2019-3863270.004.jpg
fig4
Subsequent staining for p40, smooth muscle actin, CK7, EMA, and calponin confirmed a diagnosis of myoepithelial carcinoma, clear cell variant, moderately differentiated (Figure 4).
PET/CT shows a hypermetabolic spiculated lung nodule in the right middle lobe. There is background severe bullous emphysema.
CRIPA2019-3863270.005.jpg
PMC6339750_01_CRIPA2019-3863270.005.jpg
fig5
Twelve weeks after radiation (seven months after the surgery), positron emission tomography/computed tomography (PET/CT) revealed mildly hypermetabolic bilateral pulmonary nodules that were concerning for metastatic disease (Figure 5).
Noncontrast axial and sagittal CT images of the chest show interval enlargement of prior right middle lobe nodule (red circle) and multiple new nodules throughout the lungs (yellow circles).
CRIPA2019-3863270.006.jpg
PMC6339750_01_CRIPA2019-3863270.006.jpg
fig6
Outside CT of the thorax showed bilateral pulmonary nodules of various sizes throughout all lung lobes (Figure 6).
Representative low and high power images of bronchoscopic biopsies seventeen months later. The two lung nodules from the left lower lobe showed morphology consistent with metastatic CCMC.
CRIPA2019-3863270.007.jpg
PMC6339750_01_CRIPA2019-3863270.007.jpg
fig7
Bronchoscopic biopsies of two lung nodules from the left lower lobe showed morphology consistent with metastatic CCMC (Figure 7).