Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Jun 20:e30504.
doi: 10.1002/pbc.30504. Online ahead of print.

Mammary-analog secretory carcinoma in children: Surgery or TRK inhibitors first?

Eric Moreddu  1 , Nicolas André  2   3 , Romain Appay  4
Affiliations

Mammary-analog secretory carcinoma in children: Surgery or TRK inhibitors first?

Eric Moreddu et al. Pediatr Blood Cancer. .

Abstract

We report two cases of pediatric mammary-analog secretory carcinoma (MASC), a male operated on at age 8 and a female operated on at 12, who are in remission 2 years after surgery. The diagnosis of MASC was challenging and established by identifying the ETV6::NTRK3 fusion transcript in both cases. Given the excellent results of TRK inhibitor treatments in adult MASC and pediatric tumors expressing an ETV6::NTRK3 fusion, they should probably be prescribed as first-line treatment in cases requiring surgery with foreseeable serious sequelae or metastatic disease.

Keywords: NTRK fusion; TRK inhibitor; entrectinib; larotrectinib; mammary analog secretory carcinoma.

PubMed Disclaimer

References

REFERENCES

    1. Kelly GA, Venkatramani R, Quintanilla NM, Chelius DC, Roy A, Mahajan P. Secretory carcinoma of the salivary gland: a rarity in children. J Pediatr Hematol Oncol. 2022;44:167-172.
    1. Ding A, Zuo J, Lv H, et al. Imaging identification of primary mammary analogue secretory carcinoma and acinic cell carcinoma in major salivary glands. Oral Dis. 2022;28:1861-1870.
    1. Parekh V, Stevens TM. Mammary analogue secretory carcinoma. Arch Pathol Lab Med. 2016;140:997-1001.
    1. Skalova A. Mammary analogue secretory carcinoma of salivary gland origin: an update and expanded morphologic and immunohistochemical spectrum of recently described entity. Head Neck Pathol. 2013;7(1):S30-S36.
    1. Blauel ER, Laetsch TW. The promise of TRK inhibitors in pediatric cancers with NTRK fusions. Cancer Genet. 2022;262-263:71-79.

LinkOut - more resources