Neuroendocrine Tumor of the Breast in a Young Woman With MEN2A Syndrome.

Dhru Vaghashia, Peggy Sullivan, Masha J Livhits, Ines Donangelo, Israa Laklouk, Mediget Teshome, Marla Lipsyc-Sharf

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Article in AACE endocrinology and diabetes. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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7 authors.

Dhru VaghashiaDivision of Hematology-Oncology, Department of Medicine, University of California Los Angeles, Los Angeles, California.

Peggy SullivanDepartment of Pathology, David Geffen School of Medicine at UCLA, Los Angeles, California.

Masha J LivhitsDepartment of Surgery, Section of Endocrine Surgery, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California.

Ines DonangeloDepartment of Medicine, Division of Endocrinology, Diabetes & Metabolism, David Geffen School of Medicine at UCLA, Los Angeles, California.

Israa LakloukDepartment of Pathology, David Geffen School of Medicine at UCLA, Los Angeles, California.

Mediget TeshomeDepartment of Surgery, Division of Surgical Oncology, David Geffen School of Medicine at UCLA, Los Angeles, California.

Marla Lipsyc-SharfDepartment of Medicine, Division of Hematology-Oncology, David Geffen School of Medicine at UCLA, Los Angeles, California.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background/Objective: Breast neuroendocrine tumors (BNETs) comprise <1% of breast cancers. The coexistence of BNET with multiple endocrine neoplasia (MEN)2A syndrome has not been previously reported. We present a unique case of BNET in a young woman with MEN2A syndrome. Case Presentation: A 20-year-old woman presented with headaches, nausea, and palpitations. Evaluation revealed bilateral adrenal nodules and thyroid mass with elevated calcitonin and metanephrines. Genetic testing confirmed MEN2A syndrome (RET p.C634R mutation). She underwent adrenalectomy and thyroidectomy for pheochromocytoma and medullary thyroid carcinoma. Incidental PET imaging identified a 14 mm breast mass. Biopsy revealed grade 2 ER+/PR+/HER2-BNET. Breast-conserving surgery showed stage I disease with negative lymph nodes. Discussion: BNETs carry worse prognosis than conventional breast cancers. The RET C634R mutation requires lifelong surveillance for recurrent endocrine malignancies. Whether this mutation influences BNET behavior remains unknown. Conclusion: This case highlights diagnostic complexities of BNET in MEN2A syndrome, emphasizing comprehensive surveillance in hereditary endocrine syndromes and multidisciplinary management for rare neuroendocrine presentations.

Indexed as

breast neoplasmMEN2A syndromeneuroendocrine tumor

Identifiers

PMID42491478

DOI10.1016/j.aed.2026.02.007

PMCPMC13377883

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