Case Report


Unusual presentation of incidental atypical carcinoid tumor masquerading as carcinoid tumorlets arising from diffuse neuroendocrine cell hyperplasia (DIPNECH): A case report

,  ,  ,  ,  

1 MD, Department of Pathology, Mercy Medical Center, Springfield, MA, USA

2 PA (ASCP), Department of Pathology, Mercy Medical Center, Springfield, MA, USA

3 MD, Department of Thoracic Surgery, Mercy Medical Center, Springfield, MA, USA

Address correspondence to:

Sandy Liu

Mercy Medical Center, 271 Carew Street, Springfield, MA,

USA

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Article ID: 100080Z11SL2024

doi: 10.5348/100080Z11SL2024CR

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How to cite this article

Liu S, Lynn A, Grunes D, Drake J, Rousou L. Unusual presentation of incidental atypical carcinoid tumor masquerading as carcinoid tumorlets arising from diffuse neuroendocrine cell hyperplasia (DIPNECH): A case report. J Case Rep Images Pathol 2024;10(2):17–21.

ABSTRACT


Introduction: Carcinoid tumorlets are usually incidental findings in lung excisions. These neuroendocrine proliferations should be less than 5 mm, and resemble typical carcinoid (TC) lacking necrosis and elevated mitotic activity. We report a case of atypical carcinoid (AC) presenting as multiple tumors all measuring less than 5 mm, mimicking carcinoid tumorlets.

Case Report: Our patient underwent a right upper lobectomy for multifocal invasive lepidic adenocarcinoma. Multiple nodules of neuroendocrine cells less than 5 mm in greatest dimension were noted within the lobectomy. Unlike carcinoid tumorlets, these nodules showed >2 mitotic figures per square millimeter and punctate necrosis. The ACs masqueraded as multiple microscopic tumor foci (<5 mm) resembling “carcinoid tumorlets” in adjacent background lung tissue next to the adenocarcinoma. However, increased mitoses and punctate necrosis were diagnostic of AC. A background of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) was noted, appeared to be a precursor lesion to AC, and demonstrated the DIPNECH’s preneoplastic potential to evolve directly into an AC.

Conclusion: The rare possibility of AC in “tumorlet”-appearing multiple micronodules should be considered in presence of elevated mitotic activity and necrosis in background of DIPNECH.

Keywords: Atypical carcinoid, DIPNECH, Tumorlets

SUPPORTING INFORMATION


Author Contributions

Sandy Liu - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published

Amy Lynn - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published

Dianne Grunes - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published

Jamie Drake - Acquisition of data, Analysis of data, Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published

Laki Rousou - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published

Guarantor of Submission

The corresponding author is the guarantor of submission.

Source of Support

None

Consent Statement

Written informed consent was obtained from the patient for publication of this article.

Data Availability

All relevant data are within the paper and its Supporting Information files.

Conflict of Interest

Authors declare no conflict of interest.

Copyright

© 2024 Sandy Liu et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.