Neuroendocrine neoplasms of the larynx importance of the correct diagnosis and differences between atypical carcinoid tumors and small-cell neuroendocrine carcinoma

1995 ◽  
Vol 252 (5) ◽  
Author(s):  
F.M. Gripp ◽  
E.K.J. Risse ◽  
H. Leverstein ◽  
G.B. Snow ◽  
C.J.L.M. Meijer
2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Yingying Zhu ◽  
Liming Gao ◽  
Yunxiao Meng ◽  
Wenwen Diao ◽  
Xiaoli Zhu ◽  
...  

Laryngeal neuroendocrine carcinomas (LNECs) are rare and highly heterogeneous which present a wide spectrum of pathological and clinical manifestations. Fourteen patients with histologically demonstrated LNEC were collected and analyzed retrospectively. The 14 cases were classified into 3 subtypes: typical carcinoid in 2, atypical carcinoid in 5, and small cell neuroendocrine carcinoma in 7. The mean survival time of the 14 patients in this study was 112.5 months (95% CI, 81.5–143.6). Surgeries were performed for 2 patients of typical carcinoid, and they were alive with no evidence of recurrence after 24 and 47 months of follow-ups. Patients in the atypical carcinoid group were treated with surgeries and postoperative radiotherapy. After 58.4 months of follow-ups (range: 9–144), 2 patients showed no evidence of disease and 1 was lost to follow-up after 72 months. The other 2 patients died of other unrelated diseases. In the small cell neuroendocrine carcinoma group, a combination of chemotherapy and radiotherapy was applied. The mean survival time was 79.7 months (95% CI, 37.9–121.4), and the 5-year survival rate was 53.6%. In conclusion, the clinical behaviors, treatment protocols, and prognosis are different for each subtype of LNECs.


2006 ◽  
Vol 120 (4) ◽  
pp. 289-297 ◽  
Author(s):  
E Babin ◽  
V Rouleau ◽  
P O Vedrine ◽  
B Toussaint ◽  
D de Raucourt ◽  
...  

Introduction: Small cell neuroendocrine carcinoma (SNEC) of the sinonasal tract is a rare disease.Objective: Report a descriptive study of a relatively large cohort of SNEC of the nasal cavity and paranasal sinuses.Method: The medical records of 21 patients presenting with nasal and paranasal SNEC to various French hospitals, from 1989 to 2003, were analysed to determine the clinical features and current treatment of the disease.Results: Patient data were obtained from eight French hospitals. Twelve of the patients were male and nine were female, with a mean age at presentation of 55 years (range: 27 to 79 years). Patients' staging for nasal cavity malignancy was: T1, four; T2, three; T3, one; T4, 13; N0, 18; N2, three; M0, 20; and M1, one. None of the patients suffered from SNEC of the sinonasal tract with ectopic hormone production. Immunohistochemistry proved useful for diagnosis in 20 cases. Twelve cases were positive for cytokeratin, 14 for chromogranin, eight for neuron-specific enolase and 11 for neuron-specific synaptophysin. One patient had an adenocarcinoma and an inverted papilloma associated with neuroendocrine carcinoma. Patients underwent surgery (11 cases), radiotherapy (14 cases) and chemotherapy (12 cases). Recurrence occurred in 10 cases. Five patients had visceral metastases or cervical lymph node involvement. Nine of the patients died within four years of onset of the disease.Conclusion: Small cell neuroendocrine carcinoma of the sinonasal tract is an uncommon neoplasm with aggressive clinical behaviour. Recurrence is frequent and the prognosis is poor. However, the current treatment of these neuroendocrine neoplasms varies widely.


2021 ◽  
Author(s):  
Yuxiang Shi ◽  
Li Li ◽  
Luyao Fan ◽  
Zheng Huang ◽  
Yonghui Xie ◽  
...  

Abstract Purpose To investigate the histological origin and clinical and pathological features of primary ovarian neuroendocrine neoplasms. Methods We retrospectively analyzed nine cases of ovarian neuroendocrine neoplasms diagnosed at our hospital from January 2009 to January 2021. Results The mean age of the nine patients was 44.9 ± 15.2 years (range, 28–68 years). Six cases manifested ovarian carcinoid cancer, and the pathological types were insular and trabecular carcinoid; microscopic observation showed that the carcinoid components were limited and that stromal reaction was slight. Four cases showed teratomas, with the carcinoid components not displaying adjacent mucinous glands; and the other cases exhibited carcinoid cancer as the only tumor component, without adjacent or migratory epithelial components. The six patients were followed up for 76.6 ± 41.2 (6–123) months after resection, without disease. Two cases manifested adenocarcinoma admixed with neuroendocrine carcinoma, and the intermigration of neuroendocrine carcinoma and adenocarcinoma components could be observed; and one case was an isolated small cell neuroendocrine carcinoma with no epithelial proximity or migration observed. Adenocarcinoma admixed with neuroendocrine carcinoma and small-cell neuroendocrine carcinoma exhibited an obviously promoted interstitial reaction and damaging infiltration: these three patients underwent radical surgery supplemented by postoperative radiotherapy and chemotherapy, and follow-up lasted 34.6 ± 24.2 (7–52) months; two patients died and one showed recurrence. Conclusions Ovarian neuroendocrine neoplasms may reflect multiple tissue origins, carcinoid and simple neuroendocrine neoplasms with no adjacent, transitional epithelium, and may originate from original/transformed neuroendocrine cells or stem cells of the ovarian stroma. In addition, the adenocarcinoma admixed with neuroendocrine carcinoma may originate from dedifferentiated epithelium. The prognosis with carcinoid cancer is favorable, while the prognosis for neuroendocrine carcinoma is poor.


1998 ◽  
Vol 112 (9) ◽  
pp. 827-834 ◽  
Author(s):  
Alfio Ferlito ◽  
Leon Barnes ◽  
Alessandra Rinaldo ◽  
Douglas R. Gnepp ◽  
Christopher M. Milroy

AbstractNeuroendocrine neoplasms of the larynx have been divided into those of epithelial or neural origin. The latter consist of paragangliomas while the epithelial origin group can be divided into the typical and atypical carcinoids and small cell neuroendocrine carcinomata, the latter consisting of the oat cell type, the intermediate cell type and the combined cell type. There are now over 500 cases of neuroendocrine neoplasms of the larynx in the literature.The diagnosis is primarily based on light microscopy, and, in some instances, it may be supported by special histochemical studies. It should be confirmed by immunocytochemical and/or ultrastructural investigation. The different biological behaviour of neuroendocrine neoplasms of the larynx makes a specific diagnosis of paramount importance, since treatment depends on diagnostic accuracy.Typical carcinoid is an extremely rare lesion. It is treated preferably by conservative surgery; elective neck dissection is not necessary because of the lack of lymph node metastases at diagnosis. Chemotherapy and/or radiotherapy have not been effective in the limited number of patients treated thus far. Prognosis is excellent with cure following surgery.Atypical carcinoid is the most frequent non-squamous carcinoma of the larynx. The mainstay of treatment is surgery. Elective neck dissection should be performed because of the high likelihood of cervical lymph node metastases. Primary radiation therapy with adjuvant chemotherapy is not indicated. The survival rate is 48 per cent at five years and 30 per cent at 10 years.Although the larynx is one of its most common extrapulmonary sites, small cell neuroendocrine carcinoma is still a rare tumour. Surgical results for this tumour have been disappointing and is reserved for cases of local relapse with no evidence of metastasis. Chemotherapy and radiotherapy currently appear to offer the least disabling and most effective forms of therapy. The two- and five-year survival rates are 16 per cent and five per cent, respectively.Paraneoplastic syndromes have occasionally been reported in association with carcinoid tumours (typical and atypical) and small cell neuroendocrine carcinoma. There have been also rare reports of an elevated neuropeptide serum level.Paraganglioma is the only laryngeal neuroendocrine neoplasm with a female preponderance (3:1). Confusion with atypical carcinoid has led to incorrect diagnosis and inappropriate classification schemes, erroneously suggesting that laryngeal paraganglioma has the potential for aggressive behaviour. Conservative surgery represents the treatment of choice; elective neck dissection is not necessary, and the prognosis is excellent.


2021 ◽  
Vol 9 ◽  
pp. 2050313X2199920
Author(s):  
Kotaro Inoue ◽  
Kentaro Kai ◽  
Shimpei Sato ◽  
Haruto Nishida ◽  
Koji Hirakawa ◽  
...  

A 65-year-old, gravida 3, para 2 Japanese woman was referred to our hospital for symptomatic thickening of the endometrial lining. Endocervical and endometrial cytology revealed an adenocarcinoma. The endometrial biopsy specimen was mixed, with a glandular part diagnosed as endometrioid carcinoma and a solid part diagnosed as high-grade mixed large and small cell neuroendocrine carcinoma (L/SCNEC). She underwent extra-fascial hysterectomy with bilateral salpingo-oophorectomy, complete pelvic and para-aortic lymphadenectomy, and omentectomy (FIGO IIIB, pT3b pN0 M0). She currently has no deleterious germline mutation, but high tumor mutation burden and high microsatellite instability (MSI) were identified. She underwent six cycles of platinum-based frontline chemotherapy and achieved complete remission. Immune checkpoint blockade therapy is a promising second-line therapy for MSI-high solid tumors. However, the MSI or mismatch repair (MMR) status of endometrial L/SCNEC remains unclear in the literature. Universal screening for MSI/MMR status is needed, particularly for a rare and aggressive disease.


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