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Tumorlet

MedGen UID:
690055
•Concept ID:
C1266001
•
Neoplastic Process
Synonyms: Benign Tumorlet; Tumorlet, benign
SNOMED CT: Tumorlet, benign (128876004)

Definition

A tiny localized pulmonary nodule characterized by neuroendocrine cell proliferation. It is usually discovered as an incidental finding during routine histologic examination of tissue sections or during radiologic examination. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTumorlet

Professional guidelines

PubMed

The spectrum of changes in adults with multifocal pulmonary neuroendocrine proliferations: what is the minimum set of pathologic criteria to diagnose DIPNECH?
Marchevsky AM, Wirtschafter E, Walts AE
Hum Pathol 2015 Feb;46(2):176-81. Epub 2014 Nov 12 doi: 10.1016/j.humpath.2014.10.019. PMID: 25532694

Recent clinical studies

Etiology

Pulmonary Carcinoid Tumorlet in the Explanted Lungs for Lung Transplantation: A Case Series of 15 Patients.
Terada Y, Hachem RR, Pasque MK, Kulkarni HS, Witt CA, Byers DE, Guillamet RV, Nava RG, Kozower BD, Meyers BF, Patterson GA, Kreisel D, Puri V, Takahashi T
Transplant Proc 2023 Mar;55(2):446-448. Epub 2023 Feb 12 doi: 10.1016/j.transproceed.2023.01.004. PMID: 36781372
Incidental Microscopic Granulosa Cell Proliferations: Tumorlet or Non-neoplastic?
Menon PD, Valente P, Nazarullah A
Int J Gynecol Pathol 2020 Mar;39(2):199-202. doi: 10.1097/PGP.0000000000000577. PMID: 30676430
Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence.
Wirtschafter E, Walts AE, Liu ST, Marchevsky AM
Lung 2015 Oct;193(5):659-67. Epub 2015 Jun 24 doi: 10.1007/s00408-015-9755-1. PMID: 26104490
Spectrum of pulmonary neuroendocrine cell proliferation: diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, tumorlet, and carcinoids.
Koo CW, Baliff JP, Torigian DA, Litzky LA, Gefter WB, Akers SR
AJR Am J Roentgenol 2010 Sep;195(3):661-8. doi: 10.2214/AJR.09.3811. PMID: 20729444
Pulmonary tumorlets: CT findings.
Ginsberg MS, Akin O, Berger DM, Zakowski MF, Panicek DM
AJR Am J Roentgenol 2004 Aug;183(2):293-6. doi: 10.2214/ajr.183.2.1830293. PMID: 15269015

Diagnosis

Incidental pulmonary carcinoid tumorlet associated with lung cancer.
Tchercansky AN, Buero A, Auvieux R, Korbenfeld E, Mendez J, Chimondeguy D
Medicina (B Aires) 2022;82(2):297-299. PMID: 35417395
COEXISTENCE OF PULMONARY FIBROSIS AND PROLIFERATION OF PULMONARY NEUROENDOCRINE CELLS FORMING TUMORLET.
Panek I, Liczek M, Rakoczy I, Węglarz P, Braun M, Panek M, Kuna P
Wiad Lek 2021;74(7):1767-1769. PMID: 34459785
The Uterine Plexiform Lesions Revisited.
Karpathiou G, Papoudou-Bai A, Clemenson A, Chauleur C, Peoc'h M
Am J Clin Pathol 2020 Jul 7;154(2):178-189. doi: 10.1093/ajcp/aqaa054. PMID: 32459343
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Case series and a review of the literature.
Jin L, Wang Z, Qi X
Medicine (Baltimore) 2018 Dec;97(52):e13806. doi: 10.1097/MD.0000000000013806. PMID: 30593169Free PMC Article
Spectrum of pulmonary neuroendocrine cell proliferation: diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, tumorlet, and carcinoids.
Koo CW, Baliff JP, Torigian DA, Litzky LA, Gefter WB, Akers SR
AJR Am J Roentgenol 2010 Sep;195(3):661-8. doi: 10.2214/AJR.09.3811. PMID: 20729444

Therapy

Pulmonary Carcinoid Tumorlet in the Explanted Lungs for Lung Transplantation: A Case Series of 15 Patients.
Terada Y, Hachem RR, Pasque MK, Kulkarni HS, Witt CA, Byers DE, Guillamet RV, Nava RG, Kozower BD, Meyers BF, Patterson GA, Kreisel D, Puri V, Takahashi T
Transplant Proc 2023 Mar;55(2):446-448. Epub 2023 Feb 12 doi: 10.1016/j.transproceed.2023.01.004. PMID: 36781372
Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence.
Wirtschafter E, Walts AE, Liu ST, Marchevsky AM
Lung 2015 Oct;193(5):659-67. Epub 2015 Jun 24 doi: 10.1007/s00408-015-9755-1. PMID: 26104490
Paraffinoma and carcinoid tumorlet: a hitherto unreported association mimicking lung cancer.
Casali C, Rossi G, Siopis E, Fontana G, Uliano M
J Thorac Oncol 2007 Nov;2(11):1052-3. doi: 10.1097/JTO.0b013e318158ef01. PMID: 17975499

Prognosis

Population-Based Epidemiology and Mortality of Small Malignant Gastrointestinal Stromal Tumors in the USA.
Coe TM, Fero KE, Fanta PT, Mallory RJ, Tang CM, Murphy JD, Sicklick JK
J Gastrointest Surg 2016 Jun;20(6):1132-40. Epub 2016 Mar 29 doi: 10.1007/s11605-016-3134-y. PMID: 27025710Free PMC Article
Neoplastic lesions in CADASIL syndrome: report of an autopsied Japanese case.
Hassan WA, Udaka N, Ueda A, Ando Y, Ito T
Int J Clin Exp Pathol 2015;8(6):7533-9. Epub 2015 Jun 1 PMID: 26261665Free PMC Article
Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence.
Wirtschafter E, Walts AE, Liu ST, Marchevsky AM
Lung 2015 Oct;193(5):659-67. Epub 2015 Jun 24 doi: 10.1007/s00408-015-9755-1. PMID: 26104490
A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with immunohistochemical studies: TTF-1 is expressed in both round and surface cells, suggesting an origin from primitive respiratory epithelium.
Devouassoux-Shisheboran M, Hayashi T, Linnoila RI, Koss MN, Travis WD
Am J Surg Pathol 2000 Jul;24(7):906-16. doi: 10.1097/00000478-200007000-00002. PMID: 10895813
Multifocal nephroblastic neoplasia.
Bove KE, McAdams AJ
J Natl Cancer Inst 1978 Aug;61(2):285-94. PMID: 210288

Clinical prediction guides

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Case series and a review of the literature.
Jin L, Wang Z, Qi X
Medicine (Baltimore) 2018 Dec;97(52):e13806. doi: 10.1097/MD.0000000000013806. PMID: 30593169Free PMC Article
Downregulation of drs tumor suppressor gene in highly malignant human pulmonary neuroendocrine tumors.
Shimakage M, Kodama K, Kawahara K, Kim CJ, Ikeda Y, Yutsudo M, Inoue H
Oncol Rep 2009 Jun;21(6):1367-72. doi: 10.3892/or_00000362. PMID: 19424611
Pituitary gland in hypothyroidism. Histologic and immunocytologic study.
Scheithauer BW, Kovacs K, Randall RV, Ryan N
Arch Pathol Lab Med 1985 Jun;109(6):499-504. PMID: 2986571
Plexiform tumorlet: a clinical and pathologic study of 15 cases with ultrastructural observations.
Kaminski PF, Tavassoli FA
Int J Gynecol Pathol 1984;3(2):124-34. PMID: 6092289
The histogenesis and development of pulmonary tumorlets.
Ranchod M
Cancer 1977 Mar;39(3):1135-45. doi: 10.1002/1097-0142(197703)39:3<1135::aid-cncr2820390319>3.0.co;2-b. PMID: 199344

Recent systematic reviews

The Uterine Plexiform Lesions Revisited.
Karpathiou G, Papoudou-Bai A, Clemenson A, Chauleur C, Peoc'h M
Am J Clin Pathol 2020 Jul 7;154(2):178-189. doi: 10.1093/ajcp/aqaa054. PMID: 32459343
Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence.
Wirtschafter E, Walts AE, Liu ST, Marchevsky AM
Lung 2015 Oct;193(5):659-67. Epub 2015 Jun 24 doi: 10.1007/s00408-015-9755-1. PMID: 26104490

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