Coincidence of infectious osteomyelitis and disseminated neuroblastoma: A diagnostic dilemma solved by scintigraphic imaging

Summary The case of a 41/2-year-old girl with disseminated neuroblastoma and concomitant osteomyelitis is reported. Neuroblastoma was detected in the vertebral column, the right suprarenal fossa, the left side of cranium and in bone marrow aspirates. Osteomyelitis was present in the left femur and was due to Salmonella tennessee. For the first time in the literature the two lesions were demonstrated by means of simultaneous ¹³¹I-MIBG and ^99mTc-DPD scintigraphy. The diagnoses were proved by direct histological and microbiological studies.     

Role of surgery in the treatment of patients with high-risk neuroblastoma who have a poor response to induction chemotherapy

Background

In instances of high-risk neuroblastoma that do not show a clinical response to induction therapy, whether it is worth performing surgical resection or not and whether gross total resection (GTR) is more important than subtotal resection (STR) remain controversial.

Methods

We retrospectively analyzed the data of patients with stage 4 neuroblastoma aged 18 months or older at diagnosis. Primary tumor volumes were measured both at diagnosis and at the first tumor response evaluation (after 6 cycles of induction chemotherapy). If the tumor volume at the first response evaluation was > 50% of the initial tumor volume, the patient was categorized as a poor responder. Otherwise, the patient was categorized as a good responder. Only poor responders were included. Patients were evaluated for event-free survival (EFS), overall survival (OS), and complications of surgery based on extent of surgical intervention.

Results

Sixty-five patients were included in this study. The 41 patients who underwent surgical intervention had a higher 3-year OS than the 24 patients who had a biopsy only (55.4% ± 8.1% vs. 31.3% ± 10.2%, P = 0.02). However, there was limited improvement in 3-year EFS following surgical intervention. Three-year EFS rates of BX group (biopsy only) and OP group (surgical resection) were 24.2% ± 9.3% and 37.7% ± 7.9%, respectively (P = 0.063). The extent of resection had no impact on 3-year OS (P = 0.631) and 3-year EFS (P = 0.796). Patients in the GTR group trended to have more severe surgical complications than patients in the STR group (P = 0.105).

Conclusions

For high-risk neuroblastomas that do not show a clinical response to induction therapy, surgical resection is important in predicting outcome, but the extent of resection is not.     

State of the art in oncology: High risk neuroblastoma,alveolar rhabdomyosarcoma,desmoplastic small round cell tumor,and POST-TEXT 3 and 4 hepatoblastoma

Despite advances in the treatment of pediatric cancers during the past few decades, high-risk neuroblastoma, alveolar rhabdomyosarcoma, desmoplastic small round cell tumor, and hepatoblastomas with 3 or 4 sector involvement after chemotherapy continue to present significant challenges. This review summarizes recent research on the management of these diseases, with a special focus on the use of surgical debulking, genetic analysis, immunotherapy, and chemotherapy in improving outcomes of patients with these solid tumors.     

Neuroblastoma: Outcome over a 14 year period from a tertiary care referral centre in India

Purpose

To evaluate the outcome of children with neuroblastoma (NB) from a tertiary care referral centre in India.

Method

All children with NB registered from October 1996 through July 2009 were included in the study. INSS was used for staging. All children included in the study received chemotherapy and radiation therapy appropriate for stage. Tumor resection was done when feasible. The final outcome was overall survival and it was categorized as Complete Response (CR), Partial Response (PR); No Response (NR) and Progressive Disease (PD). Analysis of three-year overall survival was done using Kaplan Meier method and Log Rank test of significance. Multivariate analysis for significance of age, site and stage was performed.

Results

144 children in the age range of 1–132 months (median 36) were enrolled. Only 38 (26.4%) children were below 12 months. 112 (77.8%) of the tumors were abdominal and 32 (22.2%) were extra-abdominal. Stage distribution was 1 + 2 in 6 (4.2%); 3 in 58 (40.3%); 4 in 68 (47.2%); 4 s in 12 (8.3%). 83 (57.6%) underwent gross complete resection. At the time of last follow-up, 100 (69.4%) were alive [60 CR (41.7%); 33 PR; 7 PD/NR] and 44 (30.6%) were dead [1CR; 11PR; 32 PD/NR]. The three-year OS was 60.7% [95 CI 50.4–69.5]. The OS was 69.7% for those < 12 months of age [95 CI 51.8–82.0] and CR was achieved in 57.9%, while for those > 12 months the OS was 55.3% [95 CI 42.2–66.6] and CR was achieved in 35.8% (p = 0.73). All 6 (100%) patients with Stage 1 and Stage 2 disease were alive and disease free. The OS was 71.5% for Stage 3[95 CI 55.3–82.7] and CR was achieved in 56.9%, while for Stage 4 the OS was 35.7%[95 CI 19.3–52.4] and CR was achieved in 17.6% (p = 0.001). The OS was 83.3% for 4 s [95 CI 48.2–95.6] and CR was achieved in 75%.

Conclusion

All the six children with Stage 1 & 2 achieved CR and were alive, while 57% of Stage 3 could achieve CR and had an OS of 71.5%. The OS (35.7%) and CR (17.6%) for Stage 4 were significantly less (p = 0.001).     

“活板门”手术切除儿童颈胸部神经母细胞瘤

目的：由于颈胸部神经母细胞瘤其独特的位置致手术空间局限,加上肿瘤常常包绕血管,使手术操作困难而具有挑战性。本研究探讨以“活板门”前开胸手术法切除颈胸神经母细胞瘤的方法。方法对10例以“活板门”前开胸手术处理的颈胸神经母细胞瘤患儿,经一期手术切除肿瘤组织,并对结果进行分析。结果10例中,男6例,女4例,手术时年龄4个月至7岁10个月（平均3岁8个月）。5例行左侧开胸手术。临床分期[国际神经母细胞瘤分期系统（INSS）]：Ⅰ期（2例）,Ⅱ期（1例）,Ⅲ期（1例）,Ⅳ期（5例）,ⅣS期（1例）。6例术前接受化疗,其中1例手术前接受局部放疗。一期肉眼完全切除率100％。术后并发症包括霍纳氏综合征10例,膈神经麻痹1例,无围手术期死亡病例。中位随访时间为4．3（0．1～7．4）年。结论通过“活板门”前开胸手术方法切除儿童颈胸神经母细胞瘤,能够显著提高一期手术完全切除率,且并发症少。     

儿童神经母细胞瘤染色体分析

目的总结55例神经母细胞瘤（NB）患儿的染色体结果,分析与临床特点及近期治疗效果的关系,提高对伴有染色体异常NB的认识。方法回顾性分析55例NB患儿的临床资料,包括分期分组、肿瘤标记物、染色体结果、治疗方案及近期预后情况。结果55例NB患儿中有4例存在17q获得（7％）,1例患儿同时存在17q获得及1 p缺失（2％）,余50例（91％）患儿染色体检查均正常。伴有染色体异常的5例患儿肿瘤标记物在病初均有不同程度的增高,而且血神经元特异性烯醇化酶（NSE）高于染色体正常组;5例染色体异常患儿均为Ⅳ期、高危组,均伴有MYCN基因获得,其中1例在治疗过程中失访,余4例中有2例肿瘤进展,1例死亡,1例经化疗联合手术切除及自体造血干细胞移植,门诊随访33个月疾病稳定。结论结果提示染色体1 p缺失和17q获得可能是NB的预后不良因素,染色体异常在NB的诊断及预后评估中具有一定临床指导意义。但本研究中的异常染色体检出率偏低,考虑与常规检测的误差有关,方法学有待进一步改进。     

染料木素对神经母细胞瘤细胞增殖的抑制作用

目的：探讨染料木素对儿童神经母细胞瘤有无潜在的治疗价值。方法：应用细胞生长抑制效应和四甲基偶氮唑蓝法（MTT）于体外观察染料木素对神经母细胞瘤细胞株SH—SYSY增殖的影响。结果：10．0～60．0μg／ml的染料木素明显抑制细胞的增殖。结论：染料木素可能成为治疗神经母细胞瘤的有效药物。