Case report primary neuroendocrine carcinoma of breast

Osaid Shoail; Alhareth Azaizeh; Mothana Alnawaflh; Duha Al-khamaiseh; Tariq Alomari; Oadi Al-wraikat

doi:10.18535/ijsrm/v13i11.mp01

Abstract

Case report

primary neuroendocrine carcinoma of breast

Abstract

Osaid Mahmoud Awwad Shoail. MD, Alhareth Ali Hasan Azaizeh. MD, Mothana Yousef Al-Nawaflh. MD, Duha Zaidan Abdel Samad Al-Khamaiseh. MD, Tariq Abdullah Mohammad Alomari. MD, Oadi Abduljalel Ibrahim Al-Wraikat. MD

Introduction: Primary neuroendocrine carcinoma (PNEC) of the breast is a rare subtype of breast cancer, often diagnosed in postmenopausal women and typically associated with aggressive clinical behavior. Histological features and the immunohistochemistry expression of neuroendocrine markers constitute the basis for the diagnosis. Due to its rarity, standardized treatment protocols are lacking.

Case Presentation: We report a case of a 38-year-old premenopausal female who presented with a painless left breast mass. Imaging revealed a suspicious lesion, and core biopsy confirmed invasive mammary carcinoma with neuroendocrine differentiation. Immunohistochemistry was positive for chromogranin, synaptophysin, and NSE, confirming the diagnosis. The patient underwent neoadjuvant chemotherapy (dose-dense AC followed by Taxane), followed by breast-conserving surgery and sentinel lymph node biopsy. Due to close surgical margins, she later underwent a nipple-sparing mastectomy with implant reconstruction. Despite systemic therapy, the patient developed liver metastases with a Ki-67 index of 100%, necessitating platinum-based chemotherapy.

Conclusion: This case highlights the aggressive nature of PNEC, particularly in younger patients, and underscores the need for heightened clinical suspicion, multidisciplinary management, and further research to guide treatment strategies for this rare malignancy.

Keywords: Neuroendocrine tumor, breast carcinoma, diagnosis, immunohistochemistry, metastases

Keywords

Neuroendocrine tumorbreast carcinomadiagnosisimmunohistochemistrymetastases

References

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Bigotti G, Coli A, Butti A, del Vecchio M, Tartaglione R, Massi G. Primary small cell neuroendocrine carcinoma of the breast. J Exp Clin Cancer Res 2004;23:691‑6.Google Scholar ↗
Papotti M, Gherardi G, Eusebi V, Pagani A, Bussolati G. Primary oat cell (neuroendocrine) carcinoma of the breast. Report of four cases. Virchows Arch A Pathol Anat Histopathol 1992;420:103‑8.Google Scholar ↗
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Mohamed A, Zeidalkilani J, Asa SL, Trybula M, Montero AJ. Management of Neuroendocrine Breast Carcinoma (NEBC): Review of Literature. Oncol Rev. 2024 Feb 9;18:12114. doi: 10.3389/or.2024.12114. PMID: 38404967; PMCID: PMC10884130.DOI ↗Google Scholar ↗

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[refR-2] Sun H, Dai S, Xu J, Liu L, Yu J, Sun T. Primary neuroendocrine tumor of the breast: current understanding and future perspectives. Front Oncol 2022; 12: 848485.‏Google Scholar ↗

[refR-3] Menéndez P, García E, Rabadán L, Pardo R, Padilla D, Villarejo P. Primary neuroendocrine breast carcinoma. Clin Breast Cancer. 2012 Aug;12(4):300-3. doi: 10.1016/j.clbc.2012.03.001. Epub 2012 Apr 17. PMID: 22513033.DOI ↗Google Scholar ↗

[refR-4] Rindi G, Klimstra DS, Abedi-Ardekani B, Asa SL, Bosman FT, Brambilla E, Busam KJ, de Krijger RR, Dietel M, El-Naggar AK, Fernandez-Cuesta L, Klöppel G, McCluggage WG, Moch H, Ohgaki H, Rakha EA, Reed NS, Rous BA, Sasano H, Scarpa A, Scoazec JY, Travis WD, Tallini G, Trouillas J, van Krieken JH, Cree IA. A common classification framework for neuroendocrine neoplasms: an International Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal. Mod Pathol. 2018 Dec;31(12):1770-1786. doi: 10.1038/s41379-018-0110-y. Epub 2018 Aug 23. PMID: 30140036; PMCID: PMC6265262.DOI ↗Google Scholar ↗

[refR-5] Tan PH, Ellis I, Allison K, Brogi E, Fox SB, Lakhani S, Lazar AJ, Morris EA, Sahin A, Salgado R, Sapino A, Sasano H, Schnitt S, Sotiriou C, van Diest P, White VA, Lokuhetty D, Cree IA; WHO Classification of Tumours Editorial Board. The 2019 World Health Organization classification of tumours of the breast. Histopathology. 2020 Aug;77(2):181-185. doi: 10.1111/his.14091. Epub 2020 Jul 29. PMID: 32056259.DOI ↗Google Scholar ↗

[refR-6] Özdirik B, Kayser A, Ullrich A, Savic LJ, Reiss M, Tacke F, Wiedenmann B, Jann H, Roderburg C. Primary Neuroendocrine Neoplasms of the Breast: Case Series and Literature Review. Cancers (Basel). 2020 Mar 20;12(3):733. doi: 10.3390/cancers12030733. PMID: 32244940; PMCID: PMC7140078.DOI ↗Google Scholar ↗

[refR-7] Jeon CH, Kim SM, Jang M, Yun BL, Ahn HS, Kim SW, Kang E, Park SY. Clinical and radiologic features of neuroendocrine breast carcinomas. J Ultrasound Med. 2014 Aug;33(8):1511-8. doi: 10.7863/ultra.33.8.1511. PMID: 25063418.DOI ↗Google Scholar ↗

[refR-8] Ju H, Liu M. Primary neuroendocrine tumor of the breast: A case report. Oncol Lett. 2024 Nov 27;29(2):79. doi: 10.3892/ol.2024.14825. PMID: 39655273; PMCID: PMC11626422.DOI ↗Google Scholar ↗

[refR-9] Ycong SMC, Kangleon-Tan HLR, Tan KAE. Primary Neuroendocrine Tumor of the Breast: A Rare Case. Case Rep Surg. 2025 Apr 21;2025:5595521. doi: 10.1155/cris/5595521. PMID: 40297647; PMCID: PMC12037242.DOI ↗Google Scholar ↗

[refR-10] Wei B, Ding T, Xing Y, Wei W, Tian Z, Tang F, et al. Invasive neuroendocrine carcinoma of the breast: A distinctive subtype of aggressive mammary carcinoma. Cancer 2010;116:4463‑73.Google Scholar ↗

[refR-11] Shin SJ, DeLellis RA, Ying L, Rosen PP. Small cell carcinoma of the breast: A clinicopathologic and immunohistochemical study of nine patients. Am J Surg Pathol 2000;24:1231‑8.Google Scholar ↗

[refR-12] Murthy V, Geethamala K, Kumar B, Sudharao M. Primary neuroendocrine carcinoma of breast: a rare case report. Ann Med Health Sci Res. 2013 Nov;3(Suppl 1):S35-7. doi: 10.4103/2141-9248.121218. PMID: 24349846; PMCID: PMC3853605.DOI ↗Google Scholar ↗

[refR-13] Yersal O, Barutca S. Biological subtypes of breast cancer: Prognostic and therapeutic implications. World J Clin Oncol. 2014 Aug 10;5(3):412-24. doi: 10.5306/wjco.v5.i3.412. PMID: 25114856; PMCID: PMC4127612.DOI ↗Google Scholar ↗

[refR-14] Morikawa T, Kobayashi S, Yamadori I, Okino T, Hirakawa E, Kurose A, Ohmori M, Kobayashi M. Three cases of extrapulmonary small cell carcinoma occurring in the prostate, stomach, and pancreas. Indian J Cancer. 1994 Dec;31(4):268-73. PMID: 7533131.Google Scholar ↗

[refR-15] Angarita FA, Rodríguez JL, Meek E, Sánchez JO, Tawil M, Torregrosa L. Locally-advanced primary neuroendocrine carcinoma of the breast: case report and review of the literature. World J Surg Oncol. 2013 Jun 5;11:128. doi: 10.1186/1477-7819-11-128. PMID: 23734899; PMCID: PMC3682896.DOI ↗Google Scholar ↗

[refR-16] Park YM, Wu Y, Wei W, Yang WT. Primary neuroendocrine carcinoma of the breast: clinical, imaging, and histologic features. AJR Am J Roentgenol. 2014 Aug;203(2):W221-30. doi: 10.2214/AJR.13.10749. PMID: 25055297.DOI ↗Google Scholar ↗

[refR-17] Rosen LE, Gattuso P. Neuroendocrine Tumors of the Breast. Arch Pathol Lab Med. 2017 Nov;141(11):1577-1581. doi: 10.5858/arpa.2016-0364-RS. PMID: 29072945.DOI ↗Google Scholar ↗

[refR-18] Bigotti G, Coli A, Butti A, del Vecchio M, Tartaglione R, Massi G. Primary small cell neuroendocrine carcinoma of the breast. J Exp Clin Cancer Res 2004;23:691‑6.Google Scholar ↗

[refR-19] Papotti M, Gherardi G, Eusebi V, Pagani A, Bussolati G. Primary oat cell (neuroendocrine) carcinoma of the breast. Report of four cases. Virchows Arch A Pathol Anat Histopathol 1992;420:103‑8.Google Scholar ↗

[refR-20] Bogina G, Munari E, Brunelli M, Bortesi L, Marconi M, Sommaggio M, Lunardi G, Gori S, Massocco A, Pegoraro MC, Zamboni G. Neuroendocrine differentiation in breast carcinoma: clinicopathological features and outcome. Histopathology. 2016 Feb;68(3):422-32. doi: 10.1111/his.12766. Epub 2015 Aug 17. PMID: 26114478.DOI ↗Google Scholar ↗

[refR-21] Fujimoto Y, Yagyu R, Murase K, Kawajiri H, Ohtani H, Arimoto Y, Yamamura T, Inoue T, Moritani S. A case of solid neuroendocrine carcinoma of the breast in a 40-year-old woman. Breast Cancer. 2007;14(2):250-3. doi: 10.2325/jbcs.889. PMID: 17485914.DOI ↗Google Scholar ↗

[refR-22] Özkurt E, Tükenmez M, Güven E, Çelet Özden B, Öner G, Müslümanoğlu M, İğci A, Özmen V, Küçücük S, Cabioğlu N. Favorable Outcome with Close Margins in Patients Undergoing Nipple/Skin Sparing Mastectomy with Immediate Breast Reconstruction: 5-year Follow-up. Balkan Med J. 2018 Jan 20;35(1):84-92. doi: 10.4274/balkanmedj.2017.0029. Epub 2017 Sep 29. PMID: 28958980; PMCID: PMC5820453.DOI ↗Google Scholar ↗

[refR-23] Swanson, Eric. Changing Perspectives in Mastectomy: The Case for Nipple Preservation. Annals of Plastic Surgery 94(3): p 263-268, March 2025. | DOI: 10.1097/SAP.0000000000004239DOI ↗Google Scholar ↗

[refR-24] Lavigne M, Menet E, Tille JC, Lae M, Fuhrmann L, Bonneau C, Deniziaut G, Melaabi S, Ng CCK, Marchiò C, Rouzier R, Bièche I, Vincent-Salomon A. Comprehensive clinical and molecular analyses of neuroendocrine carcinomas of the breast. Mod Pathol. 2018 Jan;31(1):68-82. doi: 10.1038/modpathol.2017.107. Epub 2017 Sep 8. PMID: 28884749.DOI ↗Google Scholar ↗

[refR-25] Inno A, Bogina, G, Turazza M, Bortesi L, Duranti S, Massocco A, Gori, S. Neuroendocrine carcinoma of the breast: current evidence and future perspectives. 2016 The oncologist, 21(1), 28-32.‏Google Scholar ↗

[refR-26] Mohamed A, Zeidalkilani J, Asa SL, Trybula M, Montero AJ. Management of Neuroendocrine Breast Carcinoma (NEBC): Review of Literature. Oncol Rev. 2024 Feb 9;18:12114. doi: 10.3389/or.2024.12114. PMID: 38404967; PMCID: PMC10884130.DOI ↗Google Scholar ↗