scholarly journals Very Rare Salivary Gland Tumor: Follicular Variant of Acinic Cell Carcinoma

Author(s):  
Betül Demirciler Yavaş
Author(s):  
Mandakini M. Patel ◽  
Bhavna N. Gamit ◽  
Sharmistha M. Patel ◽  
Mubin I. Patel ◽  
Shivangi S. Gandhi

Acinic Cell Carcinoma is a rarest malignant epithelial neoplasm arising from pleuripotent stem cells. ACC constitute 6% of all salivary gland tumor. Most common site is parotid. It is slowly growing tumor with female preponderance most commonly occur during fifth and sixth decades of life. Diagnosis by imaging study are complex, but USG is helpful in evaluating tumor. We are presenting a 9year female, complaint of a painless, freely movable, atraumatic swelling on left cheek measuring 2x1cm2, slowly enlarging since, 8 months. FNAC was performed from the swelling and routine stain (H and E, MGG, Pap) and special stain (PAS) was performed. On cytological smears, overall findings are suggestive of papillary-cystic variant of ACC which is difficult to diagnosed in cytology. PAS stain showed positivity for both intra-cytoplasmic granules and extra-cellular secretions. ACC is a slowly growing multi-potent malignant salivary gland tumor. Management is with surgery.


Cancers ◽  
2020 ◽  
Vol 12 (9) ◽  
pp. 2433
Author(s):  
David Y. Lee ◽  
Kathryn J. Brayer ◽  
Yoshitsugu Mitani ◽  
Eric A. Burns ◽  
Pulivarthi H. Rao ◽  
...  

Acinic cell carcinoma (AcCC) is a morphologically distinctive salivary gland malignancy often associated with chromosome rearrangements leading to overexpression of the NR4A3 transcription factor. However, little is known about how NR4A3 contributes to AcCC biology. Detailed RNA-sequencing of 21 archived AcCC samples revealed fusion reads arising from recurrent t(4;9), t(9;12), t(8;9) or t(2;4) chromosomal translocations, which positioned highly active enhancers adjacent to the promoter of the NR4A3 gene or the closely related NR4A2 gene, resulting in their aberrant overexpression. Transcriptome analyses revealed several distinct subgroups of AcCC tumors, including a subgroup that overexpressed both NR4A3 and MSANTD3. A poor survival subset of the tumors with high-grade transformation expressed NR4A3 and POMC as well as MYB, an oncogene that is the major driver in a different type of salivary gland tumor, adenoid cystic carcinoma. The combination of NR4A3 and MYB showed cooperativity in regulating a distinct set of genes. In addition, the ligand binding domain of NR4A3 directly bound the Myb DNA binding domain. Transformation assays indicated that, while overexpressed NR4A3 was sufficient to generate transformed colonies, the combination of NR4A3 plus Myb was more potent, leading to anchorage-independent growth and increased cellular invasiveness. The results confirm that NR4A3 and NR4A2 are the main driver genes of AcCC and suggest that concurrent overexpression of NR4A3 and MYB defines a subset of AcCC patients with high-grade transformation that display exceptionally poor outcome.


2016 ◽  
Vol 140 (9) ◽  
pp. 997-1001 ◽  
Author(s):  
Todd M. Stevens ◽  
Vishwas Parekh

Mammary analogue secretory carcinoma (MASC) is a recently described salivary gland tumor that shares the same histologic appearance and ETV6 gene (12p13) rearrangement as secretory carcinoma of the breast. Prior to its recognition, MASC cases were commonly labeled acinic cell carcinoma and adenocarcinoma, not otherwise specified. Despite distinctive histologic features, MASC may be difficult to distinguish from other salivary gland tumors, in particular zymogen-poor acinic cell carcinoma and low-grade salivary duct carcinoma. Although characteristic morphologic and immunohistochemical features form the basis of a diagnosis of MASC, the presence of an ETV6-NTRK3 gene fusion is confirmatory. Given its recent recognition the true prognostic import of MASC is not yet clearly defined.


2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Anthony C. Nichols ◽  
Michelle Chan-Seng-Yue ◽  
John Yoo ◽  
Sumit K. Agrawal ◽  
Maud H. W. Starmans ◽  
...  

We describe the presentation, management, and clinical outcome of a massive acinic cell carcinoma of the parotid gland. The primary tumor and blood underwent exome sequencing which revealed deletions in CDKN2A as well as PPP1R13B, which induces p53. A damaging nonsynonymous mutation was noted in EP300, a histone acetylase which plays a role in cellular proliferation. This study provides the first insights into the genetic underpinnings of this cancer. Future large-scale efforts will be necessary to define the mutational landscape of salivary gland malignancies to identify therapeutic targets and biomarkers of treatment failure.


2002 ◽  
Vol 126 (9) ◽  
pp. 1104-1105 ◽  
Author(s):  
Simonetta Piana ◽  
Alberto Cavazza ◽  
Corrado Pedroni ◽  
Rosa Scotti ◽  
Luigi Serra ◽  
...  

Abstract Dedifferentiated acinic cell carcinoma of the salivary gland is an uncommon variant of acinic cell carcinoma, characterized by the coexistence of both an usual low-grade acinic cell carcinoma and a high-grade dedifferentiated component, as well as by an accelerated clinical course. We describe a case of acinic cell carcinoma of the parotid gland in a 67-year-old woman, which recurred 4 times after surgery and radiotherapy. The recurrences consisted of residual foci of acinic cell carcinoma intermingled with a high-grade epithelial proliferation; the latter was focally constituted by cells with morphologic and immunohistochemical features of myoepithelium.


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