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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

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
T-small lymphocyte disorders.
Bartlett NL, Longo DL
Semin Hematol 1999 Apr;36(2):164-70. PMID: 10319385

Diagnosis

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
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

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
Non-small cell lung cancer in never- and ever-smokers: Is it the same disease?
Tang A, Ahmad U, Toth AJ, Bourdakos N, Raja S, Raymond DP, Blackstone EH, Murthy SC
J Thorac Cardiovasc Surg 2021 Jun;161(6):1903-1917.e9. Epub 2020 Jun 18 doi: 10.1016/j.jtcvs.2020.03.175. PMID: 32893009Free 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
Treatment of pseudo Felty's syndrome: Is there a place for rituximab?
Verhoeven F, Guillot X, Prati C, Wendling D
Joint Bone Spine 2015 May;82(3):196-9. Epub 2015 Jan 23 doi: 10.1016/j.jbspin.2014.12.001. PMID: 25623522

Prognosis

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
Granular cell tumors.
Kershisnik M, Batsakis JG, Mackay B
Ann Otol Rhinol Laryngol 1994 May;103(5 Pt 1):416-9. doi: 10.1177/000348949410300514. PMID: 8179261

Clinical prediction guides

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
Pleomorphic dermal neoplasms: a review.
Brenn T
Adv Anat Pathol 2014 Mar;21(2):108-30. doi: 10.1097/PAP.0000000000000009. PMID: 24508694
The nucleolus.
Schwarzacher HG, Wachtler F
Anat Embryol (Berl) 1993 Dec;188(6):515-36. doi: 10.1007/BF00187008. PMID: 8129175

Recent systematic reviews

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
Granular cell tumor of the infundibulum: a systematic review of MR-radiography, pathology, and clinical findings.
Polasek JB, Laviv Y, Nigim F, Rojas R, Anderson M, Varma H, Kasper EM
J Neurooncol 2018 Nov;140(2):181-198. Epub 2018 Aug 23 doi: 10.1007/s11060-018-2986-2. PMID: 30141059
Granular Cell Tumor of the Neurohypophysis: 3 Cases and a Systematic Literature Review of 98 Cases.
Zhang Y, Teng Y, Zhu H, Lu L, Deng K, Pan H, Yao Y
World Neurosurg 2018 Oct;118:e621-e630. Epub 2018 Jul 11 doi: 10.1016/j.wneu.2018.07.004. PMID: 30017767
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

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