Department of General Surgery, General Surgery Unit, Rare Hormonal Tumors Group, AO Istituti Ospitalieri, Cremona, Italy.
Eur Rev Med Pharmacol Sci. 2010 Jul;14(7):577-88.
Neuroendocrine tumors are rare neoplasms, with an incidence of about 1/100,000/year. The association between digestive neuroendocrine tumors and epithelial tumors is known, accounting for about 10% of cases, whilst in a very small number of other cases an association with other low incidence tumors has been observed.
During the past 19 years the Rare Hormonal Tumors Group of the Istituti Ospitalieri in Cremona, Italy has observed 300 patients affected by neuroendocrine tumors. We report here on four cases in which there was an unusual association with other rare neoplasms.
Overall, four of the 300 observed cases (1.3%) showed an unusual association with rare nonepithelial neoplasms: (1) gastric carcinoid and glioblastoma multiforme; (2) Merkel cell tumor and squamous cell carcinoma of the skin; (3) medullary thyroid carcinoma, yolk sac tumor of the testis and gastrointestinal stromal tumor (GIST); (4) gastric carcinoid and gastrointestinal stromal tumor (GIST).
There cases are of interest not only from an epidemiological point of view, but also offer insight into possible geno-phenotypical implications. The c-kit expression, typical of GISTs but observed also in other epithelial and neuroendocrine tumors, not only broadens the possibility to gain insight into the carcinogenesis of these neoplasms, but also opens the field to possible new therapeutic opportunities using multitargeted molecules. The contemporaneous presence of other lesions, such as the Merkel cell tumor and the squamous cell carcinoma of the skin can be interpreted as an answer by the cell to the same mutagenic stimulus. In other cases, where a possible link is not yet found which could explain the synchronism or metachronism of low incidence neoplasms, it remains possible that the associations are entirely coincidental. We await for new instruments which could help us demonstrate the possible relationships between low incidence neoplasms.
神经内分泌肿瘤是罕见的肿瘤，其发病率约为 1/100000/年。已知消化系统神经内分泌肿瘤与上皮肿瘤相关，占病例的约 10%，而在极少数其他情况下，还观察到与其他低发生率肿瘤的关联。
在过去的 19 年中，意大利克雷莫纳的 Istituti Ospitalieri 的罕见激素肿瘤小组观察了 300 名患有神经内分泌肿瘤的患者。我们在此报告了四个罕见肿瘤异常关联的病例。
在 300 例观察病例中，有 4 例（1.3%）出现了与罕见非上皮性肿瘤的不寻常关联：（1）胃类癌和胶质母细胞瘤；（2）Merkel 细胞癌和皮肤鳞状细胞癌；（3）甲状腺髓样癌、睾丸卵黄囊瘤和胃肠道间质瘤（GIST）；（4）胃类癌和胃肠道间质瘤（GIST）。
这些病例不仅从流行病学角度具有意义，而且还提供了对可能的基因表型影响的深入了解。c-kit 表达，GIST 的典型特征，但也观察到其他上皮和神经内分泌肿瘤，不仅拓宽了对这些肿瘤发生的深入了解的可能性，而且还为使用多靶点分子开辟了新的治疗机会。同时存在其他病变，如 Merkel 细胞癌和皮肤鳞状细胞癌，可以解释为细胞对相同诱变刺激的反应。在其他情况下，尚未发现可能的联系来解释低发生率肿瘤的同步或异时性，仍然有可能这些关联完全是巧合。我们等待新的工具来帮助我们证明低发生率肿瘤之间可能存在的关系。
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