A Rare Type of Colorectal Cancer: Mixed Adeno-Neuroendocrine Carcinoma (MANEC)

Abstract

RezumatScopul acestei lucrări este de a raporta datele clinico-patologice aferente unui caz de adenocarcinom neuroendocrin mixt metastatic (MANEC) cecal; mai puţin de zece astfel de cazuri au fost descrise în literatura de specialitate britanică. Un pacient în vârstă de 57 de ani s-a prezentat cu un adenocarcinom neuroendocrin mixt metastatic (MANEC) cecal cu determinări secundare hepatice şi carcinomatoză peritoneală. S-au efectuat hemicolectomie dreaptă şi omentectomie de urgenţă, urmate de mai multe cicluri de chimioterapie paliativă cu rezultate slabe. Progresia metastatică a continuat, iar pacientul a decedat la 10 luni de la prezentarea la Camera de Gardă. MANEC sunt definite ca formaţiuni alcătuite din minimum 30% componentă neuroendocrină şi minimum 30% din cea adenocarcinomatoasă. Localizarea tumorii, precum şi proporţiile şi comportamentul celor două componente ale sale influenţează tratamentul. Totuşi, ghidurile de chimioterapie rămân slab definite, iar prognosticul rămâne unul sumbru, cu supravieţuirea medie sub un an de zile. AbstractThe aim of this paper is to report the clinicopathological data of one case of mixed metastatic adenoneuroendocrine carcinoma (MANEC) in the caecum; less than ten cases of which have been described in the English literature. A 57-year-old male patient presented with a mixed adenoneuroendocrine carcinoma (MANEC) of the caecum with liver metastasis and peritoneal carcinomatosis. An emergency right hemicolectomy and omentectomy were performed, followed by several cycles of unsuccessful palliative chemotherapy. The metastasis developed further, and the patient died 10 months after presenting to the emergency room. MANECs are defined as containing at least 30% of both a neuroendocrine and an adenocarcinomatous component. The location of the tumour and the proportions and behaviour of its two components influence the treatment. However, chemotherapy guidelines remain poorly defined, and prognosis remains sombre, with median survival of less than one year.

Mixed Neuroendocrine Non-Neuroendocrine Neoplasms: A Systematic Review of a Controversial and Underestimated Diagnosis

“…The quantitative composition of the primary tumour was described in 36 studies (n = 294) [12,15,17,19,21,[23][24][25]27,29,30,34,36,[39][40][41]45,46,[48][49][50][52][53][54]56,57,59,65,73,74,80,82,[84][85][86]92]; the two components were present in equal proportion in 27.9% (n = 82) of cases, whereas in the remaining 72.1% one of the two histologies was predominant; the neuroendocrine component in 42.2% (n = 124), the non-neuroendocrine component in 29.9% (n = 88).…”

“…Interestingly, in 43 studies (n = 61) where more than one diagnostic sample was available [11,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28]30,[32][33][34][36][37][38][39][41][42][43][44][45][46][47]49,50,53,55,[59][60][61][62]64,65,96], the initial diagnosis from the first sample collected (either cytological or histological) was in keeping with MiNEN or suspicion of MiNEN in 36.1% (n = 22) of cases, adenocarcinoma in 36.1% (n = 22), poorly differentiated neuroendocrine carcinoma in 21.3% (n = 13), and well differentiated neuroendocrine tumour in 6.6% (n = 4).…”

Management of MANEC of the colon and rectum: A comprehensive review of the literature

“…Included studies. A total of 18 studies (14 case reports and 4 retrospective cohorts) reported outcomes for patients with colorectal MANEC and were finally considered eligible for analysis (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22). Data on 93 patients (62 male and 31 female; age range of 32-96 years) was retrieved from the selected studies.…”

Mixed adenoneuroendocrine carcinoma of the tongue arising within a congenital enteric cyst

“…Higher grade lesions have nuclear signaling in excess of 20% and extensive tumor‐associated necrosis . The histogenesis of MANECs may be related to simultaneous malignant transformation of 2 cell types of different lineage or conversely the existence of pluripotent stem cells, which, under inductive influences, may show divergent bidirectional differentiation . A neuroendocrine cell population is not infrequent in adenocarcinomas of the gastrointestinal tract particularly within the colorectum, however, MANEC should be distinguished from cases of adenocarcinoma in which scattered neuroendocrine cells are present …”