Esthesioneuroblastoma in Childhood and Adolescence

Eich, Hans Theodor; Müller, Rolf-Peter; Micke, Oliver; Köcher, Martin; Berthold, Frank; Hero, Barbara

doi:10.1007/s00066-005-1362-2

Cited by 41 publications

(29 citation statements)

References 30 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Two-third of our patients had Kadish stage C disease, which is in agreement with prior reports demonstrating that children usually present with advanced stage disease [12]. None of the major published reports in children, including the current report, had a patient with Kadish stage A [8, 9, 13]. …”

Section: Discussionsupporting

confidence: 92%

See 1 more Smart Citation

Multimodality Treatment of Pediatric Esthesioneuroblastoma

Venkatramani

Pan

Furman

et al. 2015

Pediatr Blood Cancer

View full text Add to dashboard Cite

Background Esthesioneuroblastoma (ENB) is a rare cancer of the nasal cavity in children. Radical surgery followed by post-operative radiation is considered the standard of care in adults. A similar approach in children can lead to significant long-term morbidity. Procedure A retrospective multi-institutional review of patients <21 years of age diagnosed with ENB between 1990 and 2014 was performed. Clinical features, treatment and outcome were obtained from the medical records. Results Twenty-four patients were identified; median age at diagnosis was 14 years (range 0.6 – 20 years). The majority (75%) were female. Headache was the most common presenting symptom, followed by nasal obstruction and epistaxis. Eight patients had Kadish stage B tumors and 16 had Kadish Stage C. Nine patients had metastatic disease. Gross total resection was achieved at diagnosis in eight patients and after neoadjuvant chemotherapy in four patients. Twenty-one patients received radiation therapy (45 Gy – 68.4 Gy). Thirteen patients received neoadjuvant chemotherapy with 84% objective response rate. Seven patients experienced disease progression or relapse; five in central nervous system, one local and one in cervical lymph node. Fifteen patients were alive at last follow-up. The 5-year disease free survival and overall survival were 74% and 73% respectively. Late effects were observed in 78% of long-term survivors. Four patients developed subsequent malignant neoplasms. Conclusions Pediatric ENB is a chemosensitive disease. Pre-operative chemotherapy based multimodal approach should be used in patients with advanced stage disease. Radiation therapy is effective for local control but lower doses should be considered in children.

show abstract

Section: Discussionsupporting

confidence: 92%

“…ENB is a small round blue cell tumor and can be difficult to diagnose [13]. Tumor cells are usually immunopositive for synaptophysin, chromogranin, CD56, neuron-specific enolase, and S-100 and immunonegative for desmin, myogenin, and CD99.…”

Section: Discussionmentioning

confidence: 99%

Multimodality Treatment of Pediatric Esthesioneuroblastoma

Venkatramani

Pan

Furman

et al. 2015

Pediatr Blood Cancer

View full text Add to dashboard Cite

show abstract

“…Overall 5-year survival was 73%, and 5-year disease-free survival was 55%. This is in keeping with our overall 5-year survival of 54% [10].…”

Section: Discussionsupporting

confidence: 86%

“…There have been fewer than 100 pediatric patients with this tumor in the literature [10]. It is a malignant neoplasm that arises from olfactory epithelium, most frequently located at the cribriform plate, the upper surface of the superior turbinates, and the upper third of the nasal septum.…”

Section: Discussionmentioning

confidence: 99%