Successful Treatment with 177Lutetium-Dotatate and Maintenance Octreotide in a Patient with Esthesioneuroblastoma with Central Nervous System Invasion: Case Report and Review of the Literature

This article presents a case of a patient with relapsed esthesioneuroblastoma (ENB), an aggressive rare tumor that arises from the specialized sensory epithelial olfactory cells in the skull base area, which was initially treated with endoscopic surgery, followed by adjuvant radiotherapy. After local relapse, new surgical approaches and subsequent lines of platin-based chemotherapy were performed. A PET-CT with 68GALIUM DOTATATOC (PET-DOTATOC) showed intense uptake of disease, compatible with the presence of somatostatin receptors, in the face, nodes, liver, bones, and meningeal area. Treatment with 4 cycles of 177Lutetium-Dotatate was performed, followed by maintenance octreotide, with a major radiological and clinical response that is lasting more than 1 year after treatment. This article describes a rare case of a skull-base tumor, with multiple recurrences, in which disease control was achieved with a targeted Peptide Receptor Radionuclide Therapy (PRRT) with 177Lutetium-Dotatate, and discusses factors that could influence the incorporation of this form of therapy. Previous case reports proved the potential efficacy of this therapy usually given for low-grade neuroendocrine tumors and will be carefully reviewed.

Nasal metastases from neuroblastoma-a rare entity:Two case reports

BACKGROUND Neuroblastoma(NB)is one of the most common malignancies in children.Metastasis in NB is not uncommon.However,nasal metastases are rare.Here,we reported two pediatric cases of nasal metastases.CASE SUMMARY Case 1 was a 3-year-old boy without a history of NB.Case 2 was a 10-year-old girl who had a history of NB for 6 years.Both of them presented with symptoms of nasal and sinus masses such as epistaxis or discharge from the nose.The radiologic imaging results revealed masses in the nasal cavity or nasopharynx in both cases and a mass in the right adrenal gland of case 1.The pathologic examination of biopsy samples of their nasal masses revealed“small round bluecell tumor”along with abundant vascular fibrous septa.The tumor cells expressed synaptophysin,cluster of differentiation 56,chromogranin A,paired like homeobox protein 2B and a very high Ki67 index in both case but were negative for vimentin,desmin,leucocyte common antigen and cytokeratin.Myelocytomatosis viral related oncogene,neuroblastoma derived(MYCN)amplification was detected in both cases.Finally,the two cases were diagnosed as nasal metastases from NB based on the clinical and pathologic findings.The two patients affected by NB were>18 mo old,the primary tumor location was adrenal gland,and they presented with multiple metastases.CONCLUSION It is difficult to differentiate between metastatic NB in the nose and olfactory neuroblastoma in the absence of a history of NB.Paired like homeobox protein 2B can play an important role in the diagnosis and differential diagnosis of this disease.

Exclusively endoscopic surgical resection of esthesioneuroblastoma: A systematic review

Background:Historically sinonasal malignancies were always addressed via open craniofacial surgery for an oncologic resection.Increasingly esthesioneuroblastomas are excised using an exclusively endoscopic approach,however,the rarity of this disease limits the availability of long-term and large scale outcomes data.Objective:The primary objective is to evaluate the treatment modalities used and the overall survival of patients with esthesioneuroblastoma managed with exclusively endoscopic surgery.Methods:In accordance with PRISMA guidelines,PubMed was queried to identify studies describing outcomes associated with endoscopic management of esthesioneuroblastomas.Results:Forty-four out of 2462 articles met inclusion criteria,totaling 399 patients with esthesioneuroblastoma treated with an exclusively endoscopic approach.Seventy-two patients(18.0%)received adjuvant chemotherapy and 331 patients(83.0%)received postoperative radiation therapy.The average age was 50.6 years old(range 6-83).Of the 399 patients,57(16.6%)were Kadish stage A,121(35.2%)were Kadish stage B,145(42.2%)were Kadish stage C,and 21(6.1%)were Kadish stage D.Pooled analysis demonstrated that 66.0%of patients had Hyams histologic GradeⅠorⅡ,while 34.0%of patients had GradeⅢorⅣdisease.Negative surgical margins were achieved in 86.9%of patients,and recurrence was identified in 10.3%of patients.Of those with 5-year follow-up,reported overall survival was 91.1%.Conclusion:Exclusively endoscopic surgery for esthesioneuroblastoma is performed for a wide range of disease stages and grades,and the majority of these patients are also treated with adjuvant chemotherapy or radiation therapy.Reported overall recurrence rate is 10.3%and 5-year survival is 91.1%.