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1. Naresh KN, Rice AJ, Bower M: Lymph nodes involved by multicentric Castleman disease among HIV-positive individuals are often involved by Kaposi sarcoma. Am J Surg Pathol; 2008 Jul;32(7):1006-12
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  • Coexistence of MCD and KS in the same tissue sample is a common phenomenon and we hypothesise that the association is due to lytic HHV8 infection of B-lymphoid cells exposing susceptible endothelial cells at vulnerable subsites within the LNs to extremely high levels of HHV8 resulting in formation of KS tumorlets in MCD-LNs.


2. Chan CC, Collins AB, Chew EY: Molecular pathology of eyes with von Hippel-Lindau (VHL) Disease: a review. Retina; 2007 Jan;27(1):1-7
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  • Increases of vascular endothelial growth factor (VEGF), hypoxia induced factor (HIF), and ubiquitin are found in ocular hemangioblastomas.Interestingly, tumorlet cells, which are composed of poorly differentiated small cells with prominent dark nuclei and little cytoplasm, as well as several stem cell markers, such as erythropoietin (Epo), Epo receptor (EpoR), and CD133, are present in ocular VHL lesions.

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  • (PMID = 17218907.001).
  • [ISSN] 0275-004X
  • [Journal-full-title] Retina (Philadelphia, Pa.)
  • [ISO-abbreviation] Retina (Philadelphia, Pa.)
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 EY000222-22; United States / Intramural NIH HHS / / Z99 EY999999
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Review
  • [Publication-country] United States
  • [Chemical-registry-number] EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
  • [Number-of-references] 56
  • [Other-IDs] NLM/ NIHMS22186; NLM/ PMC1971131
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3. Chan CC, Chew EY, Shen D, Hackett J, Zhuang Z: Expression of stem cells markers in ocular hemangioblastoma associated with von Hippel-Lindau (VHL) disease. Mol Vis; 2005;11:697-704
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  • RESULTS: Tumorlet-like cells were identified in retinal and optic nerve hemangioblastomas.

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  • (PMID = 16163267.001).
  • [ISSN] 1090-0535
  • [Journal-full-title] Molecular vision
  • [ISO-abbreviation] Mol. Vis.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 EY000222-22; United States / Intramural NIH HHS / / Z99 EY999999
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / RNA, Messenger; 0 / Receptors, Erythropoietin; 11096-26-7 / Erythropoietin
  • [Other-IDs] NLM/ NIHMS3773; NLM/ PMC1876780
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4. Klöppel G, Dege K, Remmele W, Kapran Y, Tuzlali S, Modlin IM: Siegfried Oberndorfer: a tribute to his work and life between Munich, Kiel, Geneva, and Istanbul. Virchows Arch; 2007 Aug;451 Suppl 1:S3-7
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  • In 1907 Siegfried Oberndorfer published his observations and interpretations on tumorlets ("Geschwulstchen") in the small intestine, which he called carcinoids ("karzinoide Tumoren").

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  • (PMID = 17684760.001).
  • [ISSN] 0945-6317
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Biography; Historical Article; Journal Article; Portraits; Review
  • [Publication-country] Germany
  • [Number-of-references] 14
  • [Personal-name-as-subject] Oberndorfer S
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5. Casali C, Rossi G, Siopis E, Fontana G, Uliano M: Paraffinoma and carcinoid tumorlet: a hitherto unreported association mimicking lung cancer. J Thorac Oncol; 2007 Nov;2(11):1052-3
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  • [Title] Paraffinoma and carcinoid tumorlet: a hitherto unreported association mimicking lung cancer.

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  • (PMID = 17975499.001).
  • [ISSN] 1556-1380
  • [Journal-full-title] Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer
  • [ISO-abbreviation] J Thorac Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 8002-74-2 / Paraffin
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6. Gläsker S, Tran MG, Shively SB, Ikejiri B, Lonser RR, Maxwell PH, Zhuang Z, Oldfield EH, Vortmeyer AO: Epididymal cystadenomas and epithelial tumourlets: effects of VHL deficiency on the human epididymis. J Pathol; 2006 Sep;210(1):32-41
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  • [Title] Epididymal cystadenomas and epithelial tumourlets: effects of VHL deficiency on the human epididymis.
  • The observations also indicate that ECA formation evolves from a variety of microscopic epithelial tumourlets, and that these tumourlets are confined to the efferent ductular system.

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  • [Copyright] Copyright (c) 2006 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
  • (PMID = 16841375.001).
  • [ISSN] 0022-3417
  • [Journal-full-title] The Journal of pathology
  • [ISO-abbreviation] J. Pathol.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Basic Helix-Loop-Helix Transcription Factors; 0 / Hypoxia-Inducible Factor 1; 0 / Neoplasm Proteins; 0 / Transcription Factors; 0 / endothelial PAS domain-containing protein 1
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7. Liu B, Pu Q, Liu L, Che G: Facial flushing due to multifocal tumorlets in the lung with bronchiectasis. Ann Thorac Surg; 2009 Aug;88(2):641-2
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  • [Title] Facial flushing due to multifocal tumorlets in the lung with bronchiectasis.
  • Facial flushing attacks as a Carcinoid syndrome are quite a rare presentation in pulmonary tumorlets and bronchiectasis.
  • A left lower lobectomy was performed, and multiple tumorlets were observed on microscopic examination among the bronchiectasis.
  • Histologic and immunohistochemical examination revealed findings consistent with diffuse tumorlets and bronchiectasis in the lung.

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  • (PMID = 19632427.001).
  • [ISSN] 1552-6259
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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8. Bucella D, Engohan-Aloghe C, Noël JC: Multiple epitheloid plexiform tumourlet leiomyoma of the uterus with focal vascular invasion. Eur J Gynaecol Oncol; 2010;31(4):443-5
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  • [Title] Multiple epitheloid plexiform tumourlet leiomyoma of the uterus with focal vascular invasion.
  • We report the first case of multiple plexiform tumourlets showing focal vascular invasion suggesting that, unlike what the common and accepted opinion would advocate, these tumours may have a more aggressive behaviour.

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  • (PMID = 20882891.001).
  • [ISSN] 0392-2936
  • [Journal-full-title] European journal of gynaecological oncology
  • [ISO-abbreviation] Eur. J. Gynaecol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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9. Lebras L, Arpin D, Collardeau-Frachon S, Isaac S, Guerin JC, Perol M: [Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a rare preneoplastic condition]. Rev Mal Respir; 2008 Nov;25(9):1131-5
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  • In both cases histological studies of the pulmonary biopsies revealed: a proliferation of neuroendocrine cells dispersed in the bronchial and bronchiolar epithelium, more specifically superficial to the basement membrane; some tumourlets; a typical carcinoid tumour was also found in the first patient's biopsy.


10. Parambil JG, Yi ES, Ryu JH: Obstructive bronchiolar disease identified by CT in the non-transplant population: analysis of 29 consecutive cases. Respirology; 2009 Apr;14(3):443-8
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  • Other causes included hypersensitivity pneumonitis, multiple carcinoid tumorlets, Sjögren's syndrome, paraneoplastic pemphigus, inflammatory bowel disease and Swyer-James syndrome.


11. Mehta GU, Shively SB, Duong H, Tran MG, Moncrief TJ, Smith JH, Li J, Edwards NA, Lonser RR, Zhuang Z, Merrill MJ, Raffeld M, Maxwell PH, Oldfield EH, Vortmeyer AO: Progression of epididymal maldevelopment into hamartoma-like neoplasia in VHL disease. Neoplasia; 2008 Oct;10(10):1146-53
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  • [Title] Progression of epididymal maldevelopment into hamartoma-like neoplasia in VHL disease.
  • Inactivation of the von Hippel-Lindau (VHL) gene and activation of the hypoxia-inducible factor (HIF) in susceptible cells precedes formation of tumorlets and frank tumor in the epididymis of male VHL patients.
  • Four epididymides from two VHL patients were serially sectioned to allow for three-dimensional visualization of morphologic changes.
  • Furthermore, we clarify that that there is a pathogenetic continuum between microscopic tumorlets and formation of tumor.
  • In multiple locations, three-dimensional reconstruction revealed papillary growth to extend deeply into ductular lumina, indicative of progression into early hamartoma-like neoplasia.

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  • (PMID = 18813354.001).
  • [ISSN] 1476-5586
  • [Journal-full-title] Neoplasia (New York, N.Y.)
  • [ISO-abbreviation] Neoplasia
  • [Language] ENG
  • [Grant] United Kingdom / Cancer Research UK / / ; United States / Intramural NIH HHS / / ; United Kingdom / Medical Research Council / /
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
  • [Other-IDs] NLM/ PMC2546591
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12. Greiner B, Schulz C, Pfeifer M, Heiss P, Völk M, Feuerbach S, Hamer OW: [A 74-year-old female patient with histologically proven carcinoid of the lungs and pulmonary mosaic pattern]. Radiologe; 2009 Jun;49(6):538-41
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  • DIPNECH is thought to be a precursor of tumorlets and carcinoids and usually runs a benign course.

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13. Warth A, Krysa S, Zahel T, Hoffmann H, Schirmacher P, Schnabel PA, Herpel E: [S100 protein positive sustentacular cells in pulmonary carcinoids and thoracic paragangliomas: differential diagnostic and prognostic evaluation]. Pathologe; 2010 Sep;31(5):379-84
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  • In order to get more insight into the prevalence as well as the prognostic and differential diagnostic value of this cell population in pulmonary carcinoids, we investigated a panel of 26 tumorlets, 147 typical and atypical pulmonary carcinoids and ten thoracic paragangliomas immunohistochemically.

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  • (PMID = 20567830.001).
  • [ISSN] 1432-1963
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / S100 Proteins
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14. Galván JA, González MV, Crespo G, Folgueras MV, Astudillo A: Snail nuclear expression parallels higher malignancy potential in neuroendocrine lung tumors. Lung Cancer; 2010 Sep;69(3):289-95
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  • METHODS: The studied series consisted of formalin-fixed-paraffin-embedded tissue samples from 70 patients diagnosed with NELT (2000-2006) including tumors of low malignancy potential (3 tumorlets, 33 typical carcinoids), intermediate malignancy potential (3 atypical carcinoids) and tumors of high malignancy potential (10 large cell neuroendocrine carcinomas-LCNEC and 21 small cell carcinoma-SCLC).

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  • [Copyright] Copyright (c) 2009 Elsevier Ireland Ltd. All rights reserved.
  • (PMID = 20089328.001).
  • [ISSN] 1872-8332
  • [Journal-full-title] Lung cancer (Amsterdam, Netherlands)
  • [ISO-abbreviation] Lung Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Ireland
  • [Chemical-registry-number] 0 / Cadherins; 0 / Transcription Factors; 0 / beta Catenin; 0 / snail family transcription factors
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15. Haap M, Gallwitz B, Meyermann R, Mittelbronn M: Cushing's disease associated with both pituitary microadenoma and corticotroph hyperplasia. Exp Clin Endocrinol Diabetes; 2009 Jun;117(6):289-93
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  • Neuropathological autopsy revealed nodular proliferations of corticotropic cells in the pituitary gland that are assumed to be morphological entities between diffuse hyperplasias and adenomas, termed as tumorlets.

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  • [CommentIn] Exp Clin Endocrinol Diabetes. 2010 Jan;118(1):68 [20127571.001]
  • (PMID = 19085700.001).
  • [ISSN] 1439-3646
  • [Journal-full-title] Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
  • [ISO-abbreviation] Exp. Clin. Endocrinol. Diabetes
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 9002-60-2 / Adrenocorticotropic Hormone; WI4X0X7BPJ / Hydrocortisone
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16. Francia G, Davì MV, Montresor E, Colato C, Ferdeghini M, Lo Cascio V: Long-term quiescence of ectopic Cushing's syndrome caused by pulmonary neuroendocrine tumor (typical carcinoid) and tumorlets: spontaneous remission or therapeutic effect of bromocriptine? J Endocrinol Invest; 2006 Apr;29(4):358-62
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  • [Title] Long-term quiescence of ectopic Cushing's syndrome caused by pulmonary neuroendocrine tumor (typical carcinoid) and tumorlets: spontaneous remission or therapeutic effect of bromocriptine?
  • Nests of neuroendocrine cells (tumorlets) were also demonstrated in the surrounding lung tissue.


17. Saluja R, Pomplun S, Eisen T, Sheppard MN, Nicholson AG: Multiple combined sclerosing haemangiomas and tumourlets. A report of two patients with bilateral disease. Histopathology; 2008 Nov;53(5):614-7
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  • [Title] Multiple combined sclerosing haemangiomas and tumourlets. A report of two patients with bilateral disease.
  • [MeSH-major] Histiocytoma, Benign Fibrous / pathology. Lung / pathology. Lung Neoplasms / pathology

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  • (PMID = 18983474.001).
  • [ISSN] 1365-2559
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] England
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18. Rigopoulou A, Vlychou M, Ostlere SJ, Gibbons CL, Athanasou NA: A primary leiomyosarcoma of bone containing pseudoepithelial plexiform elements. Skeletal Radiol; 2007 Aug;36(8):791-6
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  • A rare but distinctive variant of smooth muscle tumours that occurs almost exclusively in the uterus is characterised by the presence of plexiform tumourlets, which are composed of clumps and cords of tumour cells that form a discrete pseudoepithelial component.
  • We report on a case of a primary leiomyosarcoma of the proximal humerus, which, in addition to characteristic histological and immunophenotypic features of leiomyosarcoma, contained plexiform tumourlets.

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  • (PMID = 17483943.001).
  • [ISSN] 0364-2348
  • [Journal-full-title] Skeletal radiology
  • [ISO-abbreviation] Skeletal Radiol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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19. Vortmeyer AO, Tran MG, Zeng W, Gläsker S, Riley C, Tsokos M, Ikejiri B, Merrill MJ, Raffeld M, Zhuang Z, Lonser RR, Maxwell PH, Oldfield EH: Evolution of VHL tumourigenesis in nerve root tissue. J Pathol; 2006 Nov;210(3):374-82
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  • We have recently shown that 'tumour-free' spinal cord from patients with VHL disease contains microscopic, poorly differentiated cellular aggregates in nerve root tissue, which we descriptively designated 'mesenchymal tumourlets'.
  • We show that a small subset of mesenchymal tumourlets extends beyond the nerve root to form proliferative VHL-deficient mesenchyme and frank haemangioblastoma.
  • We thus demonstrate that tumourlets present potential, but true precursor material for haemangioblastoma.
  • We further show that intraradicular tumourlets consist of scattered VHL-deficient cells with activation of HIF-2alpha and HIF-dependent target proteins including CAIX and VEGF, and are associated with an extensive angiogenic response.

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  • [Copyright] Copyright 2006 Pathological Society of Great Britain and Ireland.
  • (PMID = 16981244.001).
  • [ISSN] 0022-3417
  • [Journal-full-title] The Journal of pathology
  • [ISO-abbreviation] J. Pathol.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD31; 0 / Basic Helix-Loop-Helix Transcription Factors; 0 / Hypoxia-Inducible Factor 1, alpha Subunit; 0 / Neoplasm Proteins; 0 / Vascular Endothelial Growth Factor A; 0 / endothelial PAS domain-containing protein 1; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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20. Pelosi G, Scarpa A, Puppa G, Veronesi G, Spaggiari L, Pasini F, Maisonneuve P, Iannucci A, Arrigoni G, Viale G: Alteration of the E-cadherin/beta-catenin cell adhesion system is common in pulmonary neuroendocrine tumors and is an independent predictor of lymph node metastasis in atypical carcinoids. Cancer; 2005 Mar 15;103(6):1154-64
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  • METHODS: E-cadherin and beta-catenin immunoreactivity was evaluated in 10 hyperplastic neuroendocrine tumorlets and 210 neuroendocrine tumors, including 96 typical carcinoids (CTs), 35 atypical carcinoids (ACTs), 49 large cell neuroendocrine carcinomas (LCNECs), and 30 small cell lung carcinomas (SCLCs).


21. Ge Y, Eltorky MA, Ernst RD, Castro CY: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Ann Diagn Pathol; 2007 Apr;11(2):122-6
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  • The patient is a 41-year-old man with a 5-year history of progressive dyspnea, cough, and wheezing.
  • These cells also breached the basement membrane, forming tumorlets.

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  • (PMID = 17349572.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers; 0 / Chromogranins; 333DO1RDJY / Serotonin
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22. Soga J: [The life of S. Oberndorfer: the proposer of the term "carcinoid"--the outcome of a seed in the past 100 years]. Nihon Rinsho; 2009 Nov;67(11):2201-6
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  • In 1876, he was born at Munich and, in 1907 reported a series of 6 cases of "carcinoids" in the small intestine as benign tumorlets.
  • Carcinoids are now included in a corner of the GEP-NET classification with a somewhat confusing expression of "benign biological(clinical) behavior" vs. histological malignancy.

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  • (PMID = 19899541.001).
  • [ISSN] 0047-1852
  • [Journal-full-title] Nihon rinsho. Japanese journal of clinical medicine
  • [ISO-abbreviation] Nippon Rinsho
  • [Language] jpn
  • [Publication-type] Biography; English Abstract; Historical Article; Journal Article
  • [Publication-country] Japan
  • [Personal-name-as-subject] Oberndorfer S
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23. Czapiewski P, Majewska H, Tomaszewski D, Biernat W: Coexistence of tumorlet and marginal zone B-cell lymphoma in the lung. Pathol Res Pract; 2010 Jul 15;206(7):508-10
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  • [Title] Coexistence of tumorlet and marginal zone B-cell lymphoma in the lung.
  • We report a case of a 70-year-old female with the simultaneous presence of MALT lymphoma and tumorlet in the middle lobe of the right lung.

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  • [Copyright] Copyright 2010 Elsevier GmbH. All rights reserved.
  • (PMID = 20189728.001).
  • [ISSN] 1618-0631
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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24. Masuko H, Satoh H, Miyazaki K, Kikuchi N, Kondo Y, Ohtsuka M, Hizawa N: Pulmonary sequestration with elevated serum level of progastrin-releasing peptide. Intern Med; 2008;47(3):157-60
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  • In the resected lobe, there was no malignant finding, but there were neuroendocrine tumorlet cells, which were positive for proGRP.

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  • (PMID = 18239324.001).
  • [ISSN] 1349-7235
  • [Journal-full-title] Internal medicine (Tokyo, Japan)
  • [ISO-abbreviation] Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Peptide Fragments; 0 / Peptides; 0 / Recombinant Proteins; 0 / pro-gastrin-releasing peptide (31-98)
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26. Sekhon HS, London CA, Sekhon M, Iversen PL, Devi GR: c-MYC antisense phosphosphorodiamidate morpholino oligomer inhibits lung metastasis in a murine tumor model. Lung Cancer; 2008 Jun;60(3):347-54
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  • METHODS AND RESULTS: Subcutaneous administration of AVI-4126 at local tumor site (50 microg/day) for 3 cycles of 5 days a week starting day 1 post-tumor cell implantation showed significantly decreased tumor burden, number of tumorlets formed in the lung in comparison to saline or control PMO treatment groups, although no significant reduction of the subcutaneous tumor was observed.

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  • (PMID = 18096271.001).
  • [ISSN] 0169-5002
  • [Journal-full-title] Lung cancer (Amsterdam, Netherlands)
  • [ISO-abbreviation] Lung Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Ireland
  • [Chemical-registry-number] 0 / Morpholines; 0 / Morpholinos; 0 / Oligonucleotides, Antisense; 0 / Proto-Oncogene Proteins c-myc
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27. Cavazza A, Paci M, Rossi G: Pulmonary hamartoma associated with typical carcinoid/tumorlet. Virchows Arch; 2006 Sep;449(3):392-3
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  • [Title] Pulmonary hamartoma associated with typical carcinoid/tumorlet.

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  • (PMID = 16912881.001).
  • [ISSN] 0945-6317
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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28. Travis WD: Advances in neuroendocrine lung tumors. Ann Oncol; 2010 Oct;21 Suppl 7:vii65-71
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  • Nodular NE proliferations ≥ 0.5 cm are classified as carcinoid tumors and smaller ones are called tumorlets.
  • When NE cell hyperplasia and tumorlets are extensive they represent the rare preinvasive lesion for carcinoids known as diffuse idiopathic pulmonary NE cell hyperplasia.
  • Multiple endocrine neoplasia type I can be found in TC and AC patients but not those with LCNEC and SCLC.

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  • (PMID = 20943645.001).
  • [ISSN] 1569-8041
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
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29. Nogales FF, Nicolae A, García-Galvis OF, Aneiros-Fernández J, Salas-Molina J, Stolnicu S: Uterine and extrauterine plexiform tumourlets are sex-cord-like tumours with myoid features. Histopathology; 2009 Mar;54(4):497-500
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  • [Title] Uterine and extrauterine plexiform tumourlets are sex-cord-like tumours with myoid features.

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  • (PMID = 19309407.001).
  • [ISSN] 1365-2559
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Muscle Proteins
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30. Ogo A, Eto T, Hiramatsu S, Watanabe A, Sakai Y, Yoshizumi H, Uesugi N, Nakajima O: Autopsy of a patient with Cushing's Syndrome who was revealed to have pulmonary tumorlets producing ectopic ACTH. Endocr J; 2007 Dec;54(6):863-70
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  • [Title] Autopsy of a patient with Cushing's Syndrome who was revealed to have pulmonary tumorlets producing ectopic ACTH.
  • At autopsy, a histological examination of the periphery of the right middle lobe of the lung revealed the presence of tumorlets.
  • Immunohistochemical staining of the tumorlets revealed scattered cells containing ACTH and many cells containing chromogranin A that were positive for Grimelius staining.
  • The ectopic hormonal production observed in the present case suggests that pulmonary tumorlets should thus be considered in the differential diagnosis of Cushing's syndrome, and medical treatment to inhibit steroidogenesis should be started immediately to reduce the risk of complications from hypercortisolism.


31. Kuhnen C, Winter BU: [EGFR-expression in pulmonary neuroendocrine cell hyperplasia]. Pathologe; 2006 Mar;27(2):147-51
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  • 15 cases of pulmonary neuroendocrine cell hyperplasia (carcinoid-tumorlets, diffuse idiopathic pulmonary neuroendocrine cell hyperplasia/DIPNECH) and 20 neuroendocrine pulmonary tumors (10 carcinoid tumors, 5 large cell neuroendocrine, and 5 small cell neuroendocrine lung carcinomas) were immunohistochemically analyzed for the expression of epidermal growth factor receptor (EGFR, = HER-1).

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  • [Cites] Am J Physiol Lung Cell Mol Physiol. 2000 Jun;278(6):L1256-63 [10835332.001]
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  • (PMID = 16435094.001).
  • [ISSN] 0172-8113
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] EC 2.7.10.1 / Receptor, Epidermal Growth Factor
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32. Dogan R, Kara M, Gundogdu AG, Firat P: An underrated potential risk of bronchiectasis: lymph node metastasis of a pulmonary tumorlet. Acta Chir Belg; 2009 Jan-Feb;109(1):109-11
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  • [Title] An underrated potential risk of bronchiectasis: lymph node metastasis of a pulmonary tumorlet.
  • Pulmonary tumorlets occur as the result of extensive scar caused by bronchiectasis or other inflammatory processes.
  • Histologic examination showed cystic bronchiectasis with areas of multiple neuro-endocrine tumorlets in addition to metastatic peribronchial lymph nodes.

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  • (PMID = 19341210.001).
  • [ISSN] 0001-5458
  • [Journal-full-title] Acta chirurgica Belgica
  • [ISO-abbreviation] Acta Chir. Belg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Belgium
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33. Davies SJ, Gosney JR, Hansell DM, Wells AU, du Bois RM, Burke MM, Sheppard MN, Nicholson AG: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax; 2007 Mar;62(3):248-52
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  • Tumourlets and associated typical carcinoids (n = 9) showed weak positivity for thyroid transcription factor-1.
  • Three patients had atypical carcinoids, one with multiple endocrine neoplasia type 1 syndrome.


34. Agaimy A, Wünsch PH, Hofstaedter F, Blaszyk H, Rümmele P, Gaumann A, Dietmaier W, Hartmann A: Minute gastric sclerosing stromal tumors (GIST tumorlets) are common in adults and frequently show c-KIT mutations. Am J Surg Pathol; 2007 Jan;31(1):113-20
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  • [Title] Minute gastric sclerosing stromal tumors (GIST tumorlets) are common in adults and frequently show c-KIT mutations.
  • Small hyalinizing stromal tumors of the proximal stomach (referred to in this study as GIST tumorlets) were collected prospectively from 98 consecutive autopsies and additional cases were retrieved from surgical pathology files (total n=57).
  • GIST tumorlets were grossly detectable in 22.5% consecutive autopsies performed in individuals older than 50 years.
  • Sporadic GIST tumorlets of the proximal stomach are common in the general population over the age of 50 years and frequently show somatic c-KIT mutations.
  • GIST tumorlets probably represent the grossly recognizable counterpart of sporadic ICC hyperplasia caused by somatic c-KIT or PDGFRA mutations.
  • GIST tumorlets likely represent preclinical (preneoplastic) lesions that need additional stimuli to evolve into clinical GISTs, raising the possibility of a hyperplasia-neoplasia sequence in the development of sporadic GISTs.

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  • (PMID = 17197927.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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35. Aubry MC, Thomas CF Jr, Jett JR, Swensen SJ, Myers JL: Significance of multiple carcinoid tumors and tumorlets in surgical lung specimens: analysis of 28 patients. Chest; 2007 Jun;131(6):1635-43
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  • [Title] Significance of multiple carcinoid tumors and tumorlets in surgical lung specimens: analysis of 28 patients.
  • BACKGROUND: The clinical significance of multiple carcinoid tumorlets in surgical lung specimens has not been systematically analyzed.
  • METHODS: We reviewed clinical records, available imaging, and pathology materials from patients evaluated at Mayo Clinic Rochester (from 1987 to 2000) with two or more carcinoid tumors or tumorlets in lung specimens.
  • RESULTS: Twenty-eight of 294 patients with a diagnosis of carcinoid tumor or tumorlet had two or more lesions.
  • CONCLUSIONS: Our series represents the largest compilation of multiple carcinoid tumors or tumorlets.
  • Our analysis reveals that multiple carcinoid tumors or tumorlets occur most commonly in patients with multiple nodules resembling metastatic disease.

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  • (PMID = 17400673.001).
  • [ISSN] 0012-3692
  • [Journal-full-title] Chest
  • [ISO-abbreviation] Chest
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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36. Koo CW, Baliff JP, Torigian DA, Litzky LA, Gefter WB, Akers SR: Spectrum of pulmonary neuroendocrine cell proliferation: diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, tumorlet, and carcinoids. AJR Am J Roentgenol; 2010 Sep;195(3):661-8
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  • [Title] Spectrum of pulmonary neuroendocrine cell proliferation: diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, tumorlet, and carcinoids.
  • OBJECTIVE: The objectives of this article are to review the radiologic, pathologic, and clinical features of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, tumorlet, and carcinoids and to discuss the possible role of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and tumorlet in the development of carcinoids.


37. Rizvi SM, Goodwill J, Lim E, Yap YK, Wells AU, Hansell DM, Davis P, Selim AG, Goldstraw P, Nicholson AG: The frequency of neuroendocrine cell hyperplasia in patients with pulmonary neuroendocrine tumours and non-neuroendocrine cell carcinomas. Histopathology; 2009 Sep;55(3):332-7
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  • METHODS AND RESULTS: Random blocks without tumour from resected typical carcinoids (TCs, n = 46), atypical carcinoids (ACs, n = 14), large cell neuroendocrine carcinomas (LCNECs, n = 18), small cell carcinomas (SCLCs, n = 22), adenocarcinomas (ADENOs, n = 26) and squamous cell carcinomas (SCCs, n = 18) were stained for CD56 and evaluated for linear proliferations, cell aggregates (>4 CD56+ cells), and tumourlets (<5 mm with basement membrane invasion).

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  • [ErratumIn] Histopathology. 2011 Apr;58(5):809. Abdel-Ghani, Syed [corrected to Selim, Abdel-Ghani]
  • (PMID = 19723148.001).
  • [ISSN] 1365-2559
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD56; 0 / Biomarkers, Tumor
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38. Shimakage M, Kodama K, Kawahara K, Kim CJ, Ikeda Y, Yutsudo M, Inoue H: Downregulation of drs tumor suppressor gene in highly malignant human pulmonary neuroendocrine tumors. Oncol Rep; 2009 Jun;21(6):1367-72
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  • Four cases of normal lung and bronchial epithelia, 8 samples of normal brain tissue, and 2 cases of tumorlets in the lung were also examined.
  • The drs mRNA was definitely expressed in all normal tissues of the lung and brain, and 3 TC and 2 tumorlet tissues.

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  • (PMID = 19424611.001).
  • [ISSN] 1021-335X
  • [Journal-full-title] Oncology reports
  • [ISO-abbreviation] Oncol. Rep.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Membrane Proteins; 0 / RNA, Messenger; 0 / SRPX protein, human
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39. Armbruster C, Bernhardt K, Setinek U: Pulmonary tumorlet: a case report of a diagnostic pitfall in cytology. Acta Cytol; 2008 Mar-Apr;52(2):223-7
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  • [Title] Pulmonary tumorlet: a case report of a diagnostic pitfall in cytology.
  • BACKGROUND: Pulmonary tumorlets are usually an incidental pathologic curiosity of no clinical importance, but may be mistaken for epithelial and nonepithelial neoplasms.
  • We describe a pulmonary tumorlet associated with bronchocentric granulomatosis presenting as a tumorous consolidation on chest radiograph.
  • Definitive histologic examination confirmed the intraoperative diagnosis of necrotizing granulomatosis and tumorlet.
  • CONCLUSION: Knowledge of the cytomorphologic presentation of tumorlets in FNA and consideration of the appropriate differential diagnoses combined with ancillary studies might have prevented lung resection.


40. Li F, Wang X, Xu H, Roggli VL: Small neuroendocrine lesions in intrathoracic lymph nodes of patients with primary lung adenocarcinoma: real metastasis? Am J Surg Pathol; 2010 Nov;34(11):1701-7
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  • One patient had three ILNs positive for neuroendocrine proliferation measuring 1.7, 1.8, and 4.0 mm, respectively and a minute tumorlet less than 1.0 mm in the lung.
  • Neuroendocrine cells in ILNs often formed nests of varying size with similar morphology to carcinoid tumorlet in the lung.

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  • (PMID = 20871390.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers
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41. Carmichael MG, Zacher LL: The demonstration of pulmonary neuroendocrine cell hyperplasia with tumorlets in a patient with chronic cough and a history of multiple medical problems. Mil Med; 2005 May;170(5):439-41
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  • [Title] The demonstration of pulmonary neuroendocrine cell hyperplasia with tumorlets in a patient with chronic cough and a history of multiple medical problems.
  • Lung biopsy revealed pulmonary neuroendocrine cell hyperplasia with tumorlets that stained positive for neuroendocrine tissue.
  • We present the case of a woman with chronic cough, multiple medical problems, and pulmonary neuroendocrine cell hyperplasia with tumorlets.

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  • (PMID = 15974215.001).
  • [ISSN] 0026-4075
  • [Journal-full-title] Military medicine
  • [ISO-abbreviation] Mil Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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42. Warren WH, Welker M, Gattuso P: Well-differentiated neuroendocrine carcinomas: the spectrum of histologic subtypes and various clinical behaviors. Semin Thorac Cardiovasc Surg; 2006;18(3):199-205
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  • This umbrella term encompasses a variety of tumors previously described by a variety of terms including "atypical" carcinoids, "malignant tumorlets," peripheral stage I small-cell carcinoma, as well as neoplasms described simply as "undifferentiated carcinoma" (prior to the recognition of their neuroendocrine properties).

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  • (PMID = 17185179.001).
  • [ISSN] 1043-0679
  • [Journal-full-title] Seminars in thoracic and cardiovascular surgery
  • [ISO-abbreviation] Semin. Thorac. Cardiovasc. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 59
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43. Ferolla P, Daddi N, Urbani M, Semeraro A, Ribacchi R, Giovenali P, Ascani S, De Angelis V, Crinò L, Puma F, Daddi G, Regional Multidisciplinary Group for the Diagnosis and Treatment of Neuroendocrine Tumors, CRO, Umbria Region Cancer Network, Italy: Tumorlets, multicentric carcinoids, lymph-nodal metastases, and long-term behavior in bronchial carcinoids. J Thorac Oncol; 2009 Mar;4(3):383-7
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  • [Title] Tumorlets, multicentric carcinoids, lymph-nodal metastases, and long-term behavior in bronchial carcinoids.
  • BACKGROUND: The clinical significance of lymph-node metastases, multicentric forms, and tumorlets in bronchial carcinoids is still a matter of debate.
  • PATIENTS AND METHODS: Nodal dissection and serial sections of resected lung parenchima for research of multicentric forms and tumorlets were performed in most patients.
  • Multicentric forms (syncronous carcinoids or tumorlets) were found in 11.3% of the total with a negative impact on survival (p = 0.021).
  • Multiple tumorlets were found in 7.3% of all cases at the standard pathologic examination, but whenever accurate palpation and serial sections of the surgical specimen were performed, the percentage reached 24% of the cases.
  • CONCLUSIONS: A high prevalence of tumorlets, multiple carcinoids, and lymph-nodal involvement was found in our series.

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  • (PMID = 19247084.001).
  • [ISSN] 1556-1380
  • [Journal-full-title] Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer
  • [ISO-abbreviation] J Thorac Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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44. Darvishian F, Ginsberg MS, Klimstra DS, Brogi E: Carcinoid tumorlets simulate pulmonary metastases in women with breast cancer. Hum Pathol; 2006 Jul;37(7):839-44
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  • [Title] Carcinoid tumorlets simulate pulmonary metastases in women with breast cancer.
  • A pulmonary carcinoid tumorlet (PCT) is a nodular proliferation of neuroendocrine cells smaller than 0.5 cm.
  • Pulmonary carcinoid tumorlets were identified within 5 months from diagnosis of the breast malignancy in 7 patients and at follow-up (range, 57-162 months) in the remaining 5.
  • Pulmonary carcinoid tumorlets are radiologic and histologic mimickers of pulmonary metastases in patients with a history of breast cancer.


45. Chetty R: Small and microscopically detected gastrointestinal stromal tumours: an overview. Pathology; 2008 Jan;40(1):9-12
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  • Several appellations have been employed to describe these small GISTs: minute GISTs, GIST tumourlets, interstitial cell of Cajal (ICC) hyperplasia, microscopic GISTs, minimal GISTs, and sclerosing stromal tumourlets.

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  • (PMID = 18038309.001).
  • [ISSN] 0031-3025
  • [Journal-full-title] Pathology
  • [ISO-abbreviation] Pathology
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
  • [Number-of-references] 8
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