Abstract
IntroductionPrimary neuroendocrine neoplasms (NENs) in the breast are very rare. Until 2011, the prevalence was 0.1% of all breast lesions and 1% of all NENs, whereas metastatic breast NENs represent 1%–2% of all breast tumours. However, it seems that over the last 5 years the diagnostic frequency of breast NENs has increased, probably for more alert specialists and advanced diagnostic tools, leading to a prevalence of 2%–5% of diagnosed breast cancers, mostly in the elderly population. Breast metastases from extramammary malignancies are uncommon and bilateral ones are even more uncommon, with few reported in the literature. We describe four clinical settings of breast metastases from different NENs and the multidisciplinary approach for diagnosis and treatment.MethodsFour patients were found to have NEN primaries metastasised to the breast. A literature review was conducted to identify similar cases and characterise breast metastases from neuroendocrinal tumors (NETs).ResultsTwo patients presented with bilateral breast metastases (one with well-differentiated panNET and another with atypical lung carcinoid) and two had unilateral (one with moderately differentiated lung NET and one with atypical lung carcinoid). There are about 13 cases of NEN breast metastases reported in the English literature. The ileum is the most common primary site, followed by the appendix, duodenum, pancreas and lung.ConclusionBreast lesions from extramammary primary often pose a diagnostic challenge, since a breast nodule can be the first and often the only presentation of the disease. However, differentiating between primary and secondary NEN breast lesions is essential, owing to different clinical management and prognosis.
Highlights
Primary neuroendocrine neoplasms (NENs) in the breast are very rare
The first case is that of a 40-year-old woman with a family history of gastric cancer, who presented at our centre after having a pancreaticoduodenectomy for a pancreatic tumour with liver metastases in April 2006
Post-operative somatostatin receptor scintigraphy (SRS) was negative. She was started on somatostatin analogue 30 mg/die (SSA) for 3 months, when the hepatic lesions showed evidence of progression of disease (PD) in October 2006. She was started on Thalidomide (100 mg/day) and Temozolomide (150 mg/die for 7 days) chemotherapy, followed by loco-regional treatment of liver metastases with transarterial embolisation (TAE) and trans-arterial chemoembolisation (TACE) until February 2008
Summary
Primary neuroendocrine neoplasms (NENs) in the breast are very rare. Until 2011, the prevalence was 0.1% of all breast lesions and 1% of all NENs, whereas metastatic breast NENs represent 1%–2% of all breast tumours. Up until 2011, the prevalence was less than 0.1% of all breast lesions and less than 1% of all NENs, whereas metastatic NENs lesions involving the breast represent only 1%–2% of all breast tumours [1] It seems that over the last 5 years the diagnostic frequency of primary breast NENs has increased, leading to an estimated prevalence of 2%–5% of diagnosed breast cancers, affecting mostly the elderly population aged between 60 and 80 years [12,13,14,15,16]. Recent data suggests that breast secondaries from NENs maybe more frequent than what was thought, based on the idea that many of them have been misdiagnosed in the past as primary NENs or carcinomas [4, 18, 19], they remain a rare phenomenon with no more than 200 cases published in the English literature to date [4, 18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48]
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