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Introduction: Esthesioneuroblastoma is a rare tumour of the sino-nasal tract. One-third cases present with intracranial extension. However, treatment options are limited for such cases., Methodology: We herein report a case with large intracranial extension treated with Induction chemotherapy, de-bulking surgery, and image guided intensity modulated radiotherapy., Results: The patient was treated with IGIMRT technique to a dose of 64 Gy in 32 fractions. Cone bean CT verification was done twice a week to eliminate set up error. The patient achieved complete resolution of the disease and was disease free 6 months after completion of treatment., Conclusion: IGIMRT even after a de-bulking surgery may help to achieve long-term disease control for patients with large intracranial extension with minimal morbidity.

Olfactory neuroblastoma (ONB) is a rare cancer originating in the olfactory epithelium of the nasal vault. The recurrence rate of ONB is high, as the standard treatment of surgery followed by radiotherapy and/or chemotherapy is usually unsuccessful. The use of targeted therapy based on individual genomic variations after cancer relapse has not been reported. Here, we present the case of a 44-year-old man who was diagnosed with recurrent ONB and treated with a regimen developed using whole exome sequencing. Potential targets were first identified and then matched to appropriate drugs. Gene mutations in the genes encoding EGFR, FGFR2, KDR, and RET were discovered in the patient's tumor tissue by whole exome sequencing and the patient was treated with a combination of the targeted drugs cetuximab and sunitinib. Five days after treatment, enhancement magnetic resonance imaging showed a 65% reduction in tumor size, and the Visual analog scale headache scores went down to 2/10 from 10/10. Repeat imaging at 1 month showed a complete response.This study represents the first demonstration of an effective personalized treatment of ONB by targeted drugs, and sheds light on how precision medicine can be used to treat recurrent ONB that fails to respond to routine tumor resection, radiotherapy, and/or chemotherapy.

Objective: To discuss the role of bilateral adrenalectomy in Cushing syndrome, as illustrated in a case of severe hypercortisolism that was unresponsive to combination agent medical therapy., Methods: We report the clinical, laboratory, imaging, and pathologic findings in a patient with ectopic Cushing syndrome attributable to an adrenocorticotropic hormone (ACTH)-secreting neuroblastoma. In addition, we provide a literature review regarding olfactory neuroblastoma and discuss current and emerging therapeutic options for Cushing syndrome., Results: A 59-year-old man presented with nasal congestion and neck swelling and was noted to have hypokalemia, hypertension, and hyperglycemia. A nasal biopsy demonstrated a poorly differentiated carcinoma with neuroendocrine features. He was subsequently diagnosed as having ACTH-dependent Cushing syndrome, but despite high-dose combination medical therapy, his condition rapidly deteriorated. Urgent bilateral adrenalectomy provided rapid control of the hypercortisolism, and the patient was later able to undergo an uncomplicated total macroscopic resection of his locally metastatic primary tumor., Conclusion: This report describes the challenges in the diagnosis and management of ACTH-dependent Cushing syndrome and highlights the important role that bilateral adrenalectomy can still have in patients with severe hypercortisolism causing life-threatening complications.

The development of an esthesioneuroblastoma outside to the region in which olfactory epithelium exists is extremely rare. Only nine cases were reported in the previous literatures. The author presents a 40 years-old man with ectopic esthesioneuroblastoma in sella turcica. In contrast to the previous nine cases, our case presented unusual presentations-CSF rhinorrhea and meningitis. Endoscopic transphenoid approach with removal of tumor and repair of dura defect followed by radiotherapy offered a good result in this case. Moreover, differential diagnosis and the origin of the ectopic esthsioneuroblastoma would be discussed.

Our previous comparative genomic hybridization (CGH) study of Ewing sarcoma and related tumors showed that DNA sequence copy number increases of 1q21-q22 and of chromosomes 8 and 12 were associated with trends toward poor survival (Armengol et al., Br J Cancer 1997, 75, 1403-1409). These trends were not statistically significant. In the present study, we analyzed 28 primary Ewing sarcomas and related tumors by CGH to study whether these (or other) changes have prognostic value in these tumors. Twenty-one tumors (75%) had changes with a mean of 1.9 changes per tumor. The most frequent aberration was gain of chromosome 8 in 10 tumors (36%). Five tumors (18%) had copy number increases at 1q21-22 and 5 had gain of 7q. Copy number increase of 6p21.1-pter, gain of chromosome 12, and loss of 16q were seen in 11%. Copy number increases of 1q21-q22 and of chromosomes 8 and 12 were associated with trends toward worse outcome, but the differences did not reach statistical significance. A novel finding is the association of copy number increase at 6p with worse distant disease-free (P = 0.04) and overall survival (P = 0.004). To confirm this finding and to see whether copy number increases of 1q21-q22 and of chromosomes 8 and 12 have definite prognostic value, a larger number of cases needs to be studied.

Esthesioneuroblastoma (olfactory neuroblastoma) is an unusual tumor of neuroectodermal origin, and initial presentation with symptoms characteristic of frontal lobe dysfunction is quite rare. This article describes the case of a patient who was referred to the Charleston Area Medical Center for "depression" but was found to have a esthesioneuroblastoma.