Table 1. Summary of clinicopathological findings of primary neuroendocrine breast carcinoma published in retrospective reviews and case series since 2000.
| Author/Year (Ref.), Study design |
Patients/sex | Age | Size (mm) | Pathological, Immunohistochemical Findings |
Treatment | Follow-up (months) | Outcome |
|---|---|---|---|---|---|---|---|
| OZDIRIK/2020 | 5 F | 67 (49-73) | 12 (9-53) | ER+: 60%, PR +: 80%, | BCS+ALND: 20% | 45.4 (11-130) | Alive: 60% |
| (19), CS | HER2 - : 60% | BCS+SNLB: 40% | |||||
| ER +, PR -, HER2 - : 100% | No Surgery: 40% | Died :40% | |||||
| Syn+: 100%, CgA+ : 80% | Chemo : 40% | ||||||
| N+: 20%, N/A: 40% | RT: 40%, ET:20% | ||||||
| CANBAK/2020 | 11 F | 68 (49-86) | N/A | ER+, PR+: 90% N/A:10% | BCS: 64% | N/A | Alive: 90% |
| (14), RR | HER2 - :100% | ||||||
| Syn+:, CgA+: 100% | MT: 36% | Died :10% | |||||
| N: N/A | |||||||
| HEJJANE/2020 | 2 F | 71 | 22 | ER+, PR+, HER2- | MRM + ALND | 21 | Alive |
| (25), CS | N- | ET | |||||
| 48 | 40 | ER+, PR+, Syn+, CgA+ | BCS+ ALND | 28 | Alive with recurrence | ||
| HER2-, N+ | Chemo, ET | at 12 months | |||||
| ZHANG/2020 | 2 F | 46 | 65 | ER-, PR-, HER2 - | MRM + ALND | N/A | N/A |
| (24), CS | Syn+, CgA-, N:- | Chemo | |||||
| 34 | 30, 20 | ER+, PR+ ,HER2 + | N/A | N/A | N/A | ||
| Syn+, CgA+ N- | |||||||
| LI/2017 | 119 F | 53.2 (N/A) | T1:23% | ER+: 81%, PR+: 72.2%, | MT: 79.4% | 4-144 | Alive without tumor: 80.2% |
| (26), RR | 7 M | T2:31% | HER2+: 15.1%, | BCS: 14.3% | Alive with tumor:7.1% | ||
| T3:19% | N: +15.1%, N/A: 46% | Other: 5.5% | Died of disease: 5.6% | ||||
| T4:6.3% | ALND: 82.5% | Died of other causes:0.8% | |||||
| N/A:20.6% | Chemo: 43.7% | Lost to follow-up: 6.3% | |||||
| RT: 17.5% | |||||||
| ET: 63.5% | |||||||
| ROININEN/2017 | 43F | 66 | 25.3 (18.9-31.7%) | ER+: 97.7%, PR+: 58.1%, | MT+ALND: 44.2% | 35.4 (23.5-47.2) | Local recurrence: 7.6% |
| (3), RR | HER2+: 4.7%, | BCS+ ALND: 25.6% | |||||
| Syn+: 100%, CgA+: 69.8% | Other type: 14% | ||||||
| N+: 44.2% | Chemo: 30.3% | ||||||
| RT: 74.4% | |||||||
| ET:76.7% | |||||||
| YANG/2017 | 19 F | 59.2 (17-82) | ER+: 93.3%, PR+: 73.3%, | MT +ALND: 89% | 59.2 | Alive: 52.6% | |
| (1), RR | HER2+: 4.7%, HER2-: 93% | MT: 5.3% | (15.5-114) | Died: 10.5% | |||
| Syn+: 77.8% CgA+: 42.1% | MT+SLNB: 5.3% | N/A:32% | N/A: 36.8% | ||||
| N+: 22.2% | Chemo: 61% | ||||||
| RT: 16.6% | |||||||
| ET:44%, N/A: 44% | |||||||
| COLLADO-MESA/2017 | 2 F | 58 | 15, 8 | ER+, PR+, HER2- | BCS+SLNB (-) | N/A | N/A |
| (7), CS | Syn+, CgA+, PN- | ||||||
| 62 | 17 | ER+, PR+, HER2- | BCS+SLNB (-) | N/A | N/A | ||
| Syn+, CgA+, N- | |||||||
| LOCURTO/2016 | 5 F | 59.4 (50-75) | 29 (2.3-4.5) | ER+: 100%, PR+: 80% | MRM -ALND: 20% | N/A | Alive: 80% |
| (27), CS | HER2+: 100% | BCS- ALND :60% | Died: 20% | ||||
| Syn+: 80%, CgA+: 60% | BCS+SLNB: 20% (-) | ||||||
| N+: 60% | Chemo: 60%, | ||||||
| RT: 100%, ET:100% | |||||||
| LU/2014 | 85 F | 53.9 (25-83) | 27.5 (0-180) | ER+:59%, PR+: 66% | MRM : 50% | 38.1 | Alive with no evidence of recurrence 79%. |
| (18), LR | 1 M | HER2+: 27% | BCS: 50% | (3-99) | OS: 94%, 86%, 73% in I, II, III stages respectively | ||
| Syn+: 80%, CgA+: 60% | Chemo: 41.7%, | ||||||
| RT: 28.3%, ET:13.3% | |||||||
| PARK/2014 | 84 F | 62.9 (28-89) | 31 (6-110) | ER+:98.9%, PR+: 77% | N/A | N/A | N/A |
| (2), RR | 3 M | HER2+: 2.4% | |||||
| SUHANI/2014 | 4 F | 58 (50-65) | 51 (4-6.5) | ER+:100%, PR+: 80% | MRM: 100% | 27.7 | Alive:100% |
| (15), RR | HER2- : 100% | Chemo: 100% | (9-48) | ||||
| Syn+: 50%, CgA+: 75% | RT: 100% | ||||||
| N+: 75% | ET: 100% | ||||||
| WANG/2014 | 139 F | 64 (26-99) | 31.9 | ER+:54.2%, PR+: 37.3% | Surgery: 76.8% | N/A | Median survival:26 months |
| (6), RR, SEER | 3 M | HER2 : N/A | RT:35.9% | (12-48) | |||
| N+: 28.2% | Chemo: N/A | ||||||
| ET: N/A | |||||||
| JEON/2014 | 11 F | 54.7 (29-79) | 18 (0.5-4) | ER+:100%, PR+: 100% | BCS+SLNB: 55% | 38.6 | Alive free of disease: 100% |
| (5), RR | HER2 -: 55%, N/A in 5 cases | BCS: 9% | (21-76) | ||||
| N+: 28.2% | MRM+SLNB:27% | ||||||
| MRM:9% | |||||||
| Chemo: N/A, | |||||||
| RT: N/A, ET: N/A | |||||||
| ZHU/2013 | 22 F | 52.5 (29-77) | 22 (0.5-4) | ER+:91%, PR+: 95% | MRM:100% | 64.5 | Alive free of disease: 95% |
| (28), RR | HER2 -: 75%, | Chemo: 64% | (4-89) | Alive with recurrence: 5% | |||
| ET: 91% | |||||||
| RT: 0% | |||||||
| ROVERA/2013 | 61 F | 70 (42-87) | 2.05 (0.6-6) | ER+: 90%, PR+: 75% | BCS:49% | 88 | Local or systemic recurrence: 14.8% after a median of 53.7 months |
| (29), RR | HER2- :98% | MRM:48% | (4-242) | ||||
| N+: 21.3% | Chemo:5% | ||||||
| RT: 48% | |||||||
| ET: 72% | |||||||
| ZHANG/2012 | 107 F | 65 (25-95) | 8-50 | ER+: 94.3%, PR+: 85.05% | N/A | 27 | Overall survival: 85.1% |
| (31), RR | HER 2- :97.2% | (3-134) | Local recurrence: 3.7% | ||||
| N+:24.3% | Distant recurrence: 5% | ||||||
| WU/2012 | 13 F | 53 (36-78) | 25.5 (10-40) | ER+, PR+: 100% | N/A | 67.5 | Alive free of disease: 85% |
| (32), RR | HER2+: 100% | (41-89) | Died: 7.5% | ||||
| Syn+, CgA+: 54% | Lost: 7.5% | ||||||
| N+: 7.7% | |||||||
| MENENDEZ/2012 | 4 F | 44 | 20 | ER:+, PR:-, HER2: -, N:+ | BCS+ALND, Chemo,RT | ||
| (33), CS | CgA+, N+ | 48 | Alive disease free | ||||
| 68 | 36 | ER, RP, HER2:N/A, CgA+, | BCS+ALND, Chemo, RT, ET | 24 | Alive disease free | ||
| Syn:N/A, N+ | |||||||
| 58 | 10 | ER:+, PR:-, N: -, HER2:- | BCS+SLNB, Chemo, RT | 8 | Alive with liver metastasis | ||
| 69 | 14 | ER:+, PR:+, HER2:-, | |||||
| Syn:+, CgA: +, N: - | BCS+SLNB, Adjuvant treatment: N/A | 2 | Alive disease free | ||||
| BRASK/2013 | 13 F | 67.8 (42-89) | 15.1 (8-30) | ER+: 100%, PR: N/A, HER2-: 100%, | BCS: 77% | N/A | N/A |
| (30), RR | Syn+: 92%, CgA+:30% | MRM: 23% | |||||
| N+: 23% | Adjuvant treatment: N/A | ||||||
| KAWASAKI/2012 | 24 F | 47.8 (28-74) | 4.9 (1-25) | ER+, PR+, HER2-: 100%, | BCS: 63% | 83.7 | Alive free of disease:100% |
| (34), RR | Syn:+, CgA: +, N: - | MRM: 37% | (64-101) | ||||
| Adjuvant treatment: N/A | |||||||
| GHANEM/2011 | 7 F | 56 (50-82) | 40 | ER+, PR+: 85% | BCS+ALND: 29% | 28 | Alive free of disease: 86% |
| (35), RR | (14-130) | HER2-: 100%, | MRM +ALND: 71% | (0-38) | |||
| Syn+, CgA+: 100%, | Chemo:66%, RT: 86% | Alive with recurrence: 14% | |||||
| N +: 71% | ET: 71% | ||||||
| ADEGBOLA/2005 | 3F | 46 | 10 | ER:-, PR:-, HER2: - | BCS, Chemo, RT | 48 | Alive free of disease |
| (36), CS | Syn:+, CgA: +, PN: - | ||||||
| 60 | 17 | ER:-, PR:-, HER2: - | BCS, Chemo, RT | 20 | Died | ||
| Syn:+, CgA: +, PN: - | |||||||
| 61 | 17 | ER:-, PR:-, HER2: - | BCS, Chemo, RT | 6 | Alive with disease | ||
| Syn: -, CgA: +, N: + | |||||||
| ZEKIOGLU/2003 | 12F | 66.5 (43-79) | 23.5 (8-70) | Syn+, CgA+: 66%, | BCS+ALND: 50% | 24-54 | Alive free of disease |
| (38), RR | MT+ALND:50% | ||||||
| SHIN/2000 | 9F | 54.1 (43-70) | 26 (13-50) | ER+, PR+: 92% | BCS:67% MT: 33% | 3-35 | Metastases:22% |
| (37), RR | HER2-: N/A, | ALND: 89% | |||||
| Syn+:42%, CgA+: 92%, | Chemo:78%, RT:44% | All alive | |||||
| N+: 50% | ET:22% |
CS: case series, RR: retrospective review, LR: literature review, F: female, M: male, ER: estrogen receptors, PR: progesterone receptors, Syn: synaptophysin, CgA: chromogranin A, MT: mastectomy, BCS: breast conserving surgery, SLNB: sentinel lymph node biopsy, ALND: axillary lymph node dissection, N: nodes, Chemo: chemotherapy, RT: radiation therapy, ET: endocrine therapy, N/A: not available.