Abstract:Objectives Sinonasal neuroendocrine carcinomas (SNECs) are among the rarest paranasal sinus cancers. Consensus guidelines for therapy are difficult to develop due to limited data regarding the natural history and successful treatment of these tumors. This study presents 15 years of experience treating SNEC at a single institution and a review of the literature.
Design Retrospective review.
Setting Academic medical center in the United States.
Participants Patients diagnosed with p… Show more
“…Sinonasal undifferentiated NEC is relevant because the only current factor in response to treatment and survival is histological grade; suspicion is limited to nonspecific symptoms even in advanced stages, and the majority of cases (75.0–84.6%) are diagnosed at stage IV,[ 11 , 17 , 23 ] when there is already invasion to the skull base, orbit, or brain. Its prevalence peaks between the 5 th and 6 th decades of life, with a slight male predisposition;[ 11 ] however, there are no identified risk factors.…”
Section: Discussionmentioning
confidence: 99%
“…Regarding the treatment of persistent high-grade lesions, salvage resection has been shown to be a potentially curative option; one cohort demonstrated a disease-free survival of 60% following salvage surgery. [ 11 ]…”
Section: Discussionmentioning
confidence: 99%
“…Enasidenib is an anti-IDH2 agent that could be incorporated into the therapeutic regimen for recurrent lesions. [ 7 ] In addition, a recent cohort analysis found the use of octreotide, a somatostatin analog, as an effective antiproliferative treatment in residual low-grade lesions after surgery;[ 11 ] however, it has not yet been fully studied.…”
Section: Discussionmentioning
confidence: 99%
“…However, the low degree of histologic differentiation found has been associated with overall survival as low as 30.8–40.0% at 5 years in representative studies. [ 11 , 22 ] Some other negative prognostic factors reported are high TNM stage, lymph node metastasis, Ki-67> 55%, and skull base infiltration[ 18 ] which give greater relevance to the follow-up of this patient.…”
Section: Discussionmentioning
confidence: 99%
“…Sinonasal neuroendocrine differentiation accounts for only 5% of all neoplasms in this region; it was first described 30 years ago, and by 2016, only 242 published cases were found. [ 11 ]…”
Background:
Sinonasal neuroendocrine carcinoma is a rare head and neck tumor that represents only 5% of sinonasal neoplasms. This lesion has a high risk of invasion to adjacent structures such as the orbit, skull base, and soft tissues, with symptoms usually being nonspecific. Most cases are diagnosed in late stages, decreasing overall survival without treatment. To date, there is no consensus on management given its low prevalence; however, it has been shown that multimodal therapy, with the correct surgical approach as the mainstay, offers a better disease-free prognosis.
Case Description:
A 46-year-old woman presented with a 1 year history of nasal symptoms, characterized by obstruction and epistaxis. Imaging studies showed an extra-axial mass causing skull base erosion and displacement of the right fronto-orbital region, without invasion of brain parenchyma or meninges. A biopsy was performed and an unresectable poorly differentiated sinonasal neuroendocrine carcinoma was diagnosed. Treatment with radio and chemotherapy was initiated and, as the tumoral volume decreased, she was referred for neurosurgical intervention; an endonasal endoscopic approach was performed. Gross total resection was achieved and the patient was discharged without postoperative complications and no residual lesion on imaging.
Conclusion:
We describe the evolution of a rare advanced-stage neoplasm. It highlights that despite receiving an initial diagnosis of an unresectable mass, multimodal therapy, and an adequate surgical approach deemed the entire lesion to be resected. Despite the favorable clinical evolution, the follow-up of neuroendocrine carcinoma is prioritized as a neoplasm with a high rate of recurrence and metastasis.
“…Sinonasal undifferentiated NEC is relevant because the only current factor in response to treatment and survival is histological grade; suspicion is limited to nonspecific symptoms even in advanced stages, and the majority of cases (75.0–84.6%) are diagnosed at stage IV,[ 11 , 17 , 23 ] when there is already invasion to the skull base, orbit, or brain. Its prevalence peaks between the 5 th and 6 th decades of life, with a slight male predisposition;[ 11 ] however, there are no identified risk factors.…”
Section: Discussionmentioning
confidence: 99%
“…Regarding the treatment of persistent high-grade lesions, salvage resection has been shown to be a potentially curative option; one cohort demonstrated a disease-free survival of 60% following salvage surgery. [ 11 ]…”
Section: Discussionmentioning
confidence: 99%
“…Enasidenib is an anti-IDH2 agent that could be incorporated into the therapeutic regimen for recurrent lesions. [ 7 ] In addition, a recent cohort analysis found the use of octreotide, a somatostatin analog, as an effective antiproliferative treatment in residual low-grade lesions after surgery;[ 11 ] however, it has not yet been fully studied.…”
Section: Discussionmentioning
confidence: 99%
“…However, the low degree of histologic differentiation found has been associated with overall survival as low as 30.8–40.0% at 5 years in representative studies. [ 11 , 22 ] Some other negative prognostic factors reported are high TNM stage, lymph node metastasis, Ki-67> 55%, and skull base infiltration[ 18 ] which give greater relevance to the follow-up of this patient.…”
Section: Discussionmentioning
confidence: 99%
“…Sinonasal neuroendocrine differentiation accounts for only 5% of all neoplasms in this region; it was first described 30 years ago, and by 2016, only 242 published cases were found. [ 11 ]…”
Background:
Sinonasal neuroendocrine carcinoma is a rare head and neck tumor that represents only 5% of sinonasal neoplasms. This lesion has a high risk of invasion to adjacent structures such as the orbit, skull base, and soft tissues, with symptoms usually being nonspecific. Most cases are diagnosed in late stages, decreasing overall survival without treatment. To date, there is no consensus on management given its low prevalence; however, it has been shown that multimodal therapy, with the correct surgical approach as the mainstay, offers a better disease-free prognosis.
Case Description:
A 46-year-old woman presented with a 1 year history of nasal symptoms, characterized by obstruction and epistaxis. Imaging studies showed an extra-axial mass causing skull base erosion and displacement of the right fronto-orbital region, without invasion of brain parenchyma or meninges. A biopsy was performed and an unresectable poorly differentiated sinonasal neuroendocrine carcinoma was diagnosed. Treatment with radio and chemotherapy was initiated and, as the tumoral volume decreased, she was referred for neurosurgical intervention; an endonasal endoscopic approach was performed. Gross total resection was achieved and the patient was discharged without postoperative complications and no residual lesion on imaging.
Conclusion:
We describe the evolution of a rare advanced-stage neoplasm. It highlights that despite receiving an initial diagnosis of an unresectable mass, multimodal therapy, and an adequate surgical approach deemed the entire lesion to be resected. Despite the favorable clinical evolution, the follow-up of neuroendocrine carcinoma is prioritized as a neoplasm with a high rate of recurrence and metastasis.
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