Department of Pathology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India.
Department of Pathology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India.
Ann Diagn Pathol. 2021 Aug;53:151763. doi: 10.1016/j.anndiagpath.2021.151763. Epub 2021 May 26.
Primary mediastinal germ tumours (PMGCT) constitute, a mere 3-4% of all germ cell tumours (GCT). Although they account for approximately 16% of mediastinal tumours in adults and 19-25% in children as per western literature, there is hardly any large series on PMGCT reported from the Indian subcontinent.
We have retrospectively analysed clinicopathological features of 98 cases of PMGCT diagnosed over 10 years (2010-2019) from a tertiary-care oncology centre.
The study group (n = 98) comprised predominantly of males (n = 92) (M:F ratio-15:1), with an age range between 3 months to 57 years (median: 25 years). The tumours were predominantly located in the anterior mediastinum (n = 96). Broadly, Non-seminomatous germ cell tumours (NSGCT) were more common (n = 73, 74%) compared to pure seminoma (n = 25, 26%). Mixed NSGCT was the most common histological subtype (n = 30) followed by pure mature teratoma (n = 18), pure Yolk sac tumour (n = 13), mixed seminoma and NSGCT (n = 5), pure immature teratoma (n = 3) and GCT; NOS (n = 4). Interestingly, all female patients had exclusive teratomas. Nine cases revealed secondary somatic malignancy (5 carcinomas and 4 sarcomas). The majority of patients received neoadjuvant chemotherapy (n = 71). Surgical excision was performed in 60 patients. Follow up was available in 68 patients. NSGCT showed a poor prognosis as compared to seminoma (p value = 0.03) and tumours with somatic malignancies had a more aggressive clinical course.
PMGCT was seen predominantly in young adult males and somatic malignancies were noted in as high as 9% of cases. Patient with somatic malignancy have aggressive clinical course, hence, extensive sampling and careful histopathological evaluation are recommended for the identification and definitive characterization.
原发性纵隔生殖细胞瘤(PMGCT)仅占所有生殖细胞瘤(GCT)的 3-4%。尽管根据西方文献,PMGCT 约占成人纵隔肿瘤的 16%,占儿童纵隔肿瘤的 19-25%,但来自印度次大陆的此类大型系列报道几乎没有。
我们回顾性分析了一家三级肿瘤中心 10 年来(2010-2019 年)诊断的 98 例 PMGCT 的临床病理特征。
研究组(n=98)主要由男性(n=92)(男女比例 15:1)组成,年龄在 3 个月至 57 岁之间(中位数:25 岁)。肿瘤主要位于前纵隔(n=96)。非精原细胞瘤生殖细胞瘤(NSGCT)比单纯精原细胞瘤(n=25,26%)更常见(n=73,74%)。混合 NSGCT 是最常见的组织学亚型(n=30),其次是单纯成熟畸胎瘤(n=18)、单纯卵黄囊瘤(n=13)、混合精原细胞瘤和 NSGCT(n=5)、单纯未成熟畸胎瘤(n=3)和 GCTNOS(n=4)。有趣的是,所有女性患者均为单纯畸胎瘤。9 例患者出现继发性实体恶性肿瘤(5 例癌和 4 例肉瘤)。大多数患者接受新辅助化疗(n=71)。60 例患者行手术切除。68 例患者可获得随访。与精原细胞瘤相比,NSGCT 预后较差(p 值=0.03),伴发实体恶性肿瘤的肿瘤具有更具侵袭性的临床病程。
PMGCT 主要见于年轻成年男性,高达 9%的病例中存在继发性实体恶性肿瘤。伴发实体恶性肿瘤的患者具有侵袭性临床病程,因此,建议广泛取样并进行仔细的组织病理学评估,以识别和明确特征。