U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Granular cell cancer

MedGen UID:
87276
•Concept ID:
C0334618
•
Neoplastic Process
Synonym: malignant granular cell tumor
SNOMED CT: Malignant granular cell tumor (13238004); Malignant granular cell myoblastoma (13238004); Malignant granular cell tumor (404041003); Granular cell tumor, malignant (13238004); Granular cell myoblastoma, malignant (13238004)
 
Monarch Initiative: MONDO:0003252

Definition

An uncommon granular cell tumor which may metastasize to other anatomic sites. Morphologic characteristics include the presence of spindling neoplastic cells, necrosis, extensive pleomorphism, prominent nucleoli, and increased mitiotic activity. [from NCI]

Professional guidelines

PubMed

Large Granular Lymphocytic Leukemia: Current State of Diagnosis, Pathogenesis and Treatment.
Magnano L, Rivero A, Matutes E
Curr Oncol Rep 2022 May;24(5):633-644. Epub 2022 Feb 25 doi: 10.1007/s11912-021-01159-y. PMID: 35212923
Endoscopic management of subepithelial lesions including neuroendocrine neoplasms: European Society of Gastrointestinal Endoscopy (ESGE) Guideline.
Deprez PH, Moons LMG, OʼToole D, Gincul R, Seicean A, Pimentel-Nunes P, Fernández-Esparrach G, Polkowski M, Vieth M, Borbath I, Moreels TG, Nieveen van Dijkum E, Blay JY, van Hooft JE
Endoscopy 2022 Apr;54(4):412-429. Epub 2022 Feb 18 doi: 10.1055/a-1751-5742. PMID: 35180797
LGL leukemia: from pathogenesis to treatment.
Lamy T, Moignet A, Loughran TP Jr
Blood 2017 Mar 2;129(9):1082-1094. Epub 2017 Jan 23 doi: 10.1182/blood-2016-08-692590. PMID: 28115367

Recent clinical studies

Etiology

Cangfudaotan decoction inhibits mitochondria-dependent apoptosis of granulosa cells in rats with polycystic ovarian syndrome.
Jiang XL, Tai H, Xiao XS, Zhang SY, Cui SC, Qi SB, Hu DD, Zhang LN, Kuang JS, Meng XS, Li SM
Front Endocrinol (Lausanne) 2022;13:962154. Epub 2022 Nov 9 doi: 10.3389/fendo.2022.962154. PMID: 36465612Free PMC Article
Large Granular Lymphocytic Leukemia: Current State of Diagnosis, Pathogenesis and Treatment.
Magnano L, Rivero A, Matutes E
Curr Oncol Rep 2022 May;24(5):633-644. Epub 2022 Feb 25 doi: 10.1007/s11912-021-01159-y. PMID: 35212923
Epstein-Barr virus-associated T- and NK-cell lymphoproliferative diseases: an update and diagnostic approach.
Hue SS, Oon ML, Wang S, Tan SY, Ng SB
Pathology 2020 Jan;52(1):111-127. Epub 2019 Nov 22 doi: 10.1016/j.pathol.2019.09.011. PMID: 31767131
Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article
Benign esophageal tumors.
Ha C, Regan J, Cetindag IB, Ali A, Mellinger JD
Surg Clin North Am 2015 Jun;95(3):491-514. Epub 2015 Mar 21 doi: 10.1016/j.suc.2015.02.005. PMID: 25965126

Diagnosis

Neutrophil extracellular traps and neutrophil-related mediators in human thyroid cancer.
Modestino L, Cristinziano L, Poto R, Ventrici A, Trocchia M, Ferrari SM, Fallahi P, Paparo SR, Marone G, Antonelli A, Varricchi G, Galdiero MR
Front Immunol 2023;14:1167404. Epub 2023 Aug 29 doi: 10.3389/fimmu.2023.1167404. PMID: 37705974Free PMC Article
Not all LGL leukemias are created equal.
Semenzato G, Calabretto G, Barilà G, Gasparini VR, Teramo A, Zambello R
Blood Rev 2023 Jul;60:101058. Epub 2023 Feb 20 doi: 10.1016/j.blre.2023.101058. PMID: 36870881
Epstein-Barr virus-associated T- and NK-cell lymphoproliferative diseases: an update and diagnostic approach.
Hue SS, Oon ML, Wang S, Tan SY, Ng SB
Pathology 2020 Jan;52(1):111-127. Epub 2019 Nov 22 doi: 10.1016/j.pathol.2019.09.011. PMID: 31767131
Granular cell astrocytoma.
Shi Y, Morgenstern N
Arch Pathol Lab Med 2008 Dec;132(12):1946-50. doi: 10.5858/132.12.1946. PMID: 19061297
NK cell lymphoma.
Oshimi K
Int J Hematol 2002 Aug;76 Suppl 2:118-21. doi: 10.1007/BF03165101. PMID: 12430911

Therapy

Effective treatment with the selective cytokine inhibitor BNZ-1 reveals the cytokine dependency of T-LGL leukemia.
Brammer JE, Ballen K, Sokol L, Querfeld C, Nakamura R, Mishra A, McLaughlin EM, Feith D, Azimi N, Waldmann TA, Tagaya Y, Loughran T
Blood 2023 Oct 12;142(15):1271-1280. doi: 10.1182/blood.2022017643. PMID: 37352612Free PMC Article
Cangfudaotan decoction inhibits mitochondria-dependent apoptosis of granulosa cells in rats with polycystic ovarian syndrome.
Jiang XL, Tai H, Xiao XS, Zhang SY, Cui SC, Qi SB, Hu DD, Zhang LN, Kuang JS, Meng XS, Li SM
Front Endocrinol (Lausanne) 2022;13:962154. Epub 2022 Nov 9 doi: 10.3389/fendo.2022.962154. PMID: 36465612Free PMC Article
Single-cell RNA sequencing coupled to TCR profiling of large granular lymphocyte leukemia T cells.
Gao S, Wu Z, Arnold B, Diamond C, Batchu S, Giudice V, Alemu L, Raffo DQ, Feng X, Kajigaya S, Barrett J, Ito S, Young NS
Nat Commun 2022 Apr 11;13(1):1982. doi: 10.1038/s41467-022-29175-x. PMID: 35411048Free PMC Article
A phase 2 biomarker-driven study of ruxolitinib demonstrates effectiveness of JAK/STAT targeting in T-cell lymphomas.
Moskowitz AJ, Ghione P, Jacobsen E, Ruan J, Schatz JH, Noor S, Myskowski P, Vardhana S, Ganesan N, Hancock H, Davey T, Perez L, Ryu S, Santarosa A, Dowd J, Obadi O, Pomerantz L, Yi N, Sohail S, Galasso N, Neuman R, Liotta B, Blouin W, Baik J, Geyer MB, Noy A, Straus D, Kumar P, Dogan A, Hollmann T, Drill E, Rademaker J, Schoder H, Inghirami G, Weinstock DM, Horwitz SM
Blood 2021 Dec 30;138(26):2828-2837. doi: 10.1182/blood.2021013379. PMID: 34653242Free PMC Article
NOX2-dependent regulation of inflammation.
Singel KL, Segal BH
Clin Sci (Lond) 2016 Apr 1;130(7):479-90. doi: 10.1042/CS20150660. PMID: 26888560Free PMC Article

Prognosis

Bladder cancer patients have increased NETosis and impaired DNaseI-mediated NET degradation that can be therapeutically restored in vitro.
Herranz R, Oto J, Hueso M, Plana E, Cana F, Castaño M, Cordón L, Ramos-Soler D, Bonanad S, Vera-Donoso CD, Martínez-Sarmiento M, Medina P
Front Immunol 2023;14:1171065. Epub 2023 May 19 doi: 10.3389/fimmu.2023.1171065. PMID: 37275882Free PMC Article
Not all LGL leukemias are created equal.
Semenzato G, Calabretto G, Barilà G, Gasparini VR, Teramo A, Zambello R
Blood Rev 2023 Jul;60:101058. Epub 2023 Feb 20 doi: 10.1016/j.blre.2023.101058. PMID: 36870881
Malignant granular cell tumor: Clinical features and long-term survival.
Moten AS, Zhao H, Wu H, Farma JM
J Surg Oncol 2018 Nov;118(6):891-897. Epub 2018 Sep 9 doi: 10.1002/jso.25227. PMID: 30196562
Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article
Pancreatic panniculitis.
Rongioletti F, Caputo V
G Ital Dermatol Venereol 2013 Aug;148(4):419-25. PMID: 23900163

Clinical prediction guides

Not all LGL leukemias are created equal.
Semenzato G, Calabretto G, Barilà G, Gasparini VR, Teramo A, Zambello R
Blood Rev 2023 Jul;60:101058. Epub 2023 Feb 20 doi: 10.1016/j.blre.2023.101058. PMID: 36870881
Single-cell RNA sequencing coupled to TCR profiling of large granular lymphocyte leukemia T cells.
Gao S, Wu Z, Arnold B, Diamond C, Batchu S, Giudice V, Alemu L, Raffo DQ, Feng X, Kajigaya S, Barrett J, Ito S, Young NS
Nat Commun 2022 Apr 11;13(1):1982. doi: 10.1038/s41467-022-29175-x. PMID: 35411048Free PMC Article
Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article
Actinic keratosis and squamous cell carcinoma: clinical and pathological features.
Filosa A, Filosa G
G Ital Dermatol Venereol 2015 Aug;150(4):379-84. Epub 2015 Jun 23 PMID: 26099352
The nucleolus.
Schwarzacher HG, Wachtler F
Anat Embryol (Berl) 1993 Dec;188(6):515-36. doi: 10.1007/BF00187008. PMID: 8129175

Recent systematic reviews

Granular Cell Tumors of the Larynx: A Systematic Review.
Luke AS, Mairson TM, Husain IA, Kaplan SE
Laryngoscope 2024 Apr;134(4):1523-1530. Epub 2023 Oct 2 doi: 10.1002/lary.31071. PMID: 37782552
Granular cell tumour of the pancreas: a case report and systematic review.
Tree K, Kotecha K, Mehta S, Fuchs TL, Toon CW, Gill AJ, Samra JS, Mittal A
Langenbecks Arch Surg 2023 Jan 25;408(1):64. doi: 10.1007/s00423-023-02761-3. PMID: 36694023Free PMC Article
Oral granular cell tumour: A multicentric study of 56 cases and a systematic review.
Lafuente Ibáñez de Mendoza I, López Ortega K, Trierveiler M, Oliveira Alves MG, Dias Almeida J, Gándara Vila P, Aguirre Urízar JM, Pérez Sayans M
Oral Dis 2020 Apr;26(3):573-589. Epub 2020 Jan 28 doi: 10.1111/odi.13273. PMID: 31898368
Primary tumors of the posterior pituitary: A systematic review.
Guerrero-Pérez F, Marengo AP, Vidal N, Iglesias P, Villabona C
Rev Endocr Metab Disord 2019 Jun;20(2):219-238. doi: 10.1007/s11154-019-09484-1. PMID: 30864049
Central granular cell odontogenic tumor: a systematic review.
Sarode SC, Sarode GS, Vaidya K
J Oral Pathol Med 2014 Mar;43(3):167-76. Epub 2013 May 13 doi: 10.1111/jop.12085. PMID: 23668324

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...