Milanesi U, Mangili F, Milanesi I
Divisione di ORL, Ospedale Civile, Desio, MI.
Acta Otorhinolaryngol Ital. 1994 Jul-Aug;14(4):439-47.
Paragangliomas of the carotid body with an autosomal dominant genetic transmission have been described in a familial presentation in 7-10% of all the cases observed. In less than 10% malignancy is confirmed by secondary metastatic localization rather than by typical histological features of malignant diseases, such as vascular or perineural invasion, nuclear pleomorphism, mitotic activity. The purpose of the study is to present a familial group of paragangliomas of the carotid boy and a flow cytometric analysis of tumor content DNA and to discuss the prognostic value of the results. The paragangliomas of these related patients (father and daughter) are diploid, without regional lymph node or distant metastases; clinical and cytometric findings support a good prognosis even if an accurate follow up of such neuroendocrine tumors is nonetheless mandatory. Considering data in Literature, the Authors also propose a prognostic classification of paragangliomas as follows: noninvasive (capsulated, without vascular or perineural infiltration, diploid); locally invasive (histological signs such as vascular or perineural infiltration, nuclear pleomorphism, abnormal mitoses etc, diploid); potentially malignant (histological signs, non-diploid, expression of few antigens) and malignant (regional or distant metastases).
常染色体显性遗传的颈动脉体副神经节瘤在所有观察到的病例中,有7% - 10%以家族形式出现。不到10%的病例通过继发性转移定位而非恶性疾病的典型组织学特征(如血管或神经周围浸润、核多形性、有丝分裂活性)来确诊恶性。本研究的目的是呈现一组家族性颈动脉体副神经节瘤病例,并对肿瘤细胞DNA进行流式细胞术分析,同时讨论结果的预后价值。这些相关患者(父女)的副神经节瘤为二倍体,无区域淋巴结或远处转移;临床和细胞术结果支持良好预后,即便如此,对这类神经内分泌肿瘤进行精确随访仍是必要的。考虑到文献中的数据,作者还提出了副神经节瘤的预后分类如下:非侵袭性(有包膜,无血管或神经周围浸润,二倍体);局部侵袭性(有血管或神经周围浸润、核多形性、异常有丝分裂等组织学征象,二倍体);潜在恶性(有组织学征象,非二倍体,少数抗原表达)和恶性(区域或远处转移)。