Montalto Shana, Sertorio Fiammetta, Podda Marta, Sorrentino Stefania, Di Cataldo Andrea, Provenzi Massimo, Nonnis Antonella, D'Ippolito Carmelita, Corrias Maria Valeria, De Bernardi Bruno
Oncology Unit, IRCCS Istituto Giannina Gaslini, Genova, Italy.
Department of Radiology, IRCCS Istituto Giannina Gaslini, Genova, Italy.
Pediatr Hematol Oncol. 2022 Aug;39(5):441-452. doi: 10.1080/08880018.2021.2013368. Epub 2022 Feb 10.
Bilateral adrenal neuroblastoma (NB) is rare and is mainly stage 4S. Its incidence, presenting features, management, and outcome have not been fully defined yet. We searched the Italian NB Registry (RINB) for stage 4S NB infants with bilateral adrenal primary tumor to compare them with stage 4S NB with unilateral tumor. Between 1979 and 2016, the RINB enrolled 3731 NB patients aged 0-18 years including 317 infants (8.5%) diagnosed with stage 4S NB. Eleven/317 (3.5%) had a bilateral adrenal primary tumor (Group 1) and 190/317 (59.9%) had a unilateral tumor (Group 2). Group 1 infants were significantly younger (51 vs. 89 days) but were comparable with Group 2 for any other presenting features. In the absence of specific protocols, upfront treatment was based on symptoms, size of adrenal tumors, and biology, and consisted of observation in 5 cases, radiotherapy in one, chemotherapy in 2, and surgery in 3. Five/11 developed progression and 2 of them, both with amplification, died. The 5-year EFS rates of Group 1 and 2 were 54.5% vs. 73.3% (P=.14) and 5-year OSs were 81.8% and 89.4%, respectively (P=.44). Our data support the hypothesis that 4S NB infants with bilateral adrenal tumors can have favorable outcome with personalized therapeutic approach. The three patients with amplified tumor benefited from upfront aggressive chemotherapy, in accordance with current protocols. Because of the rarity of this intriguing form of neuroblastoma, collaborative prospective studies are warranted, especially in view of gaining a better insight on its biological and genetic features.
双侧肾上腺神经母细胞瘤（NB）较为罕见，主要为4S期。其发病率、临床表现、治疗方法及预后尚未完全明确。我们在意大利神经母细胞瘤登记处（RINB）中搜索了患有双侧肾上腺原发性肿瘤的4S期NB婴儿，以将他们与患有单侧肿瘤的4S期NB婴儿进行比较。1979年至2016年间，RINB登记了3731例年龄在0至18岁的NB患者，其中包括317例被诊断为4S期NB的婴儿（8.5%）。317例中有11例（3.5%）患有双侧肾上腺原发性肿瘤（第1组），190例（59.9%）患有单侧肿瘤（第2组）。第1组婴儿明显更年幼（51天对89天），但在任何其他临床表现方面与第2组相当。在没有特定方案的情况下，初始治疗基于症状、肾上腺肿瘤大小和生物学特性，5例采用观察，1例采用放疗，2例采用化疗，3例采用手术。11例中有5例病情进展，其中2例均伴有 扩增，死亡。第1组和第2组的5年无事件生存率分别为54.5%对73.3%（P = 0.14），5年总生存率分别为81.8%和89.4%（P = 0.44）。我们的数据支持这样的假设，即患有双侧肾上腺肿瘤的4S期NB婴儿通过个性化治疗方法可获得良好预后。3例肿瘤有 扩增的患者根据当前方案从初始积极化疗中获益。由于这种有趣形式的神经母细胞瘤罕见，因此有必要进行协作性前瞻性研究，尤其是为了更好地了解其生物学和遗传学特征。
