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. 2021 Dec;43(12):3788-3795.
doi: 10.1002/hed.26865. Epub 2021 Sep 15.

Therapeutic approaches and outcomes in patients with larynx or hypopharynx high-grade neuroendocrine carcinoma: A single-center retrospective analysis

Luana Guimaraes Sousa  1 , Felippe Lazar Neto  1 , Danice Karagiannis Torman  1 , Eduardo M Diaz  2 , David I Rosenthal  3 , Bonnie S Glisson  1 , Diana Bell  4 , Renata Ferrarotto  1
Affiliations

Therapeutic approaches and outcomes in patients with larynx or hypopharynx high-grade neuroendocrine carcinoma: A single-center retrospective analysis

Luana Guimaraes Sousa et al. Head Neck. 2021 Dec.

Abstract

Background: High-grade neuroendocrine carcinoma of the larynx (HG-NECL) is rare and aggressive with limited data regarding response to systemic therapy. We evaluated clinicopathological features, therapeutic approaches, and outcomes in patients with laryngeal or hypopharyngeal HG-NECL.

Methods: Data were retrospectively collected through 1997-2020. Median disease-free (mDFS), progression-free (mPFS), and overall survival (mOS) were estimated using the Kaplan-Meier method.

Results: Fifteen patients were identified; most had locoregional (N = 7) or metastatic disease (N = 5). The main curative-intent treatment was chemoradiation concurrent with platinum-based chemotherapy; the rate of complete response was 78%. Most patients (80%) developed recurrence; the mDFS was 13.1 months. For the first-line palliative therapy, the ORR and mPFS were 50% and 3.1 months, respectively. For all patients, the mOS was 17.8 months, and 8.6 months for metastatic disease.

Conclusion: Laryngeal HG-NEC is associated with high relapse rates and dismal prognosis for those with recurrent/metastatic disease. Novel therapeutic strategies are needed.

Keywords: larynx; neuroendocrine carcinoma; palliative therapy; small cell carcinoma; smoking.

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Conflict of interest statement

Conflict of Interest Disclosures: None

Figures

Figure 1.
Figure 1.
Left arytenoid high-grade neuroendocrine carcinoma, small cell type. A-C. Hematoxylin-eosin-stained sections at 2x (A), 4x (B), and 10x (C) magnification with salient cytomorphologic features, including nuclear molding, high nuclear/cytoplasm ratio, mitotic activity, apoptosis, and rosetting. D-E. Ancillary studies were supportive of the neuroendocrine phenotype and included immunoperoxidase studies with anti-synaptophysin antibodies (D) and anti-cytokeratin cocktail (E).
Figure 2.
Figure 2.. Swimmer plot.
Time 0 represents the date of diagnosis. An arrow indicates that the patient was alive at end of the graph period (60 months) and had the indicated outcome outside this range.
Figure 3.
Figure 3.. Overall survival.
Shown are Kaplan-Meier curves for the patients overall (A) and according to tumor stage (B). Shaded are indicate a 95% confidence interval. OS, overall survival.
See this image and copyright information in PMC

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