Abstract
Background:
Salivary gland secretory carcinoma is usually a low-grade neoplasm. However, high-grade transformation can occur and has important implications for clinical outcome.
Methods:
A patient presented with an enlarging buccal mass. Magnetic resonance imaging (MRI) showed a tumor with a biphasic appearance along the right parotid duct. Local excision and histopathologic examination confirmed the diagnosis of secretory carcinoma with high-grade transformation. ETV6-NTRK3 translocation and loss of CDKN2A/B were identified.
Results:
The patient subsequently presented with cough and dyspnea and was found to have pleural metastases. Carboplatin and paclitaxel exacerbated the symptoms. Crizotinib resulted in initial symptomatic and radiographic improvement; however, the patient soon succumbed to progressive intrathoracic disease.
Conclusions:
High-grade salivary gland secretory carcinoma can have a biphasic appearance on MRI. Diagnosis is confirmed by the histologic appearance and associated ETV6-NTRK3 fusion. Additional molecular genetic events leading to transformation are unknown; however, loss of CDKN2A/B may have contributed. Treatment with multimodal chemotherapy was of limited benefit.
Keywords:
CDKN2A/B; MRI; crizotinib; high grade; metastatic; salivary gland; secretory carcinoma.
MeSH terms
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Adenocarcinoma, Clear Cell / diagnosis
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Adenocarcinoma, Clear Cell / pathology
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Adult
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Antineoplastic Combined Chemotherapy Protocols / pharmacology*
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Crizotinib
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Cyclin-Dependent Kinase Inhibitor p15 / deficiency
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Cyclin-Dependent Kinase Inhibitor p15 / genetics
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Cyclin-Dependent Kinase Inhibitor p16
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Cyclin-Dependent Kinase Inhibitor p18 / deficiency
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Cyclin-Dependent Kinase Inhibitor p18 / genetics
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Diagnosis, Differential
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Disease Progression
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Drug Resistance, Neoplasm
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Fatal Outcome
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Gene Rearrangement
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Humans
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Immunohistochemistry
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Magnetic Resonance Imaging
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Male
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Mammary Analogue Secretory Carcinoma / diagnosis
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Mammary Analogue Secretory Carcinoma / genetics
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Mammary Analogue Secretory Carcinoma / secondary*
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Mammary Analogue Secretory Carcinoma / therapy
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Myoepithelioma / diagnosis
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Myoepithelioma / pathology
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Oncogene Proteins, Fusion / genetics
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Parotid Gland / pathology
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Parotid Gland / surgery
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Pleural Neoplasms / diagnosis
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Pleural Neoplasms / genetics
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Pleural Neoplasms / secondary*
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Pleural Neoplasms / therapy
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Pyrazoles / pharmacology
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Pyrazoles / therapeutic use
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Pyridines / pharmacology
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Pyridines / therapeutic use
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Salivary Gland Neoplasms / diagnosis
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Salivary Gland Neoplasms / genetics
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Salivary Gland Neoplasms / pathology*
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Salivary Gland Neoplasms / therapy
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Translocation, Genetic
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Treatment Outcome
Substances
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CDKN2A protein, human
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CDKN2B protein, human
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Cyclin-Dependent Kinase Inhibitor p15
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Cyclin-Dependent Kinase Inhibitor p16
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Cyclin-Dependent Kinase Inhibitor p18
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ETV6-NTRK3 fusion protein, human
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Oncogene Proteins, Fusion
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Pyrazoles
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Pyridines
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Crizotinib