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Malignant glandular tumor of peripheral nerve sheath

MedGen UID:
232558
•Concept ID:
C1333821
•
Neoplastic Process
Synonyms: Glandular malignant peripheral nerve sheath tumor; Glandular Malignant Peripheral Nerve Sheath Tumor; glandular malignant peripheral nerve sheath tumor; glandular malignant peripheral nerve sheath tumour; Glandular MPNST; glandular MPNST; Malignant Glandular Neoplasm of Peripheral Nerve Sheath; malignant glandular neoplasm of peripheral nerve sheath; Malignant Glandular Neoplasm of the Peripheral Nerve Sheath; malignant glandular neoplasm of the peripheral nerve sheath; Malignant Glandular Peripheral Nerve Sheath Neoplasm; malignant glandular peripheral nerve sheath neoplasm; Malignant Glandular Peripheral Nerve Sheath Tumor; malignant glandular peripheral nerve sheath tumor; malignant glandular peripheral nerve sheath tumour; Malignant Glandular Schwannoma; malignant glandular schwannoma; Malignant Glandular Tumor of Peripheral Nerve Sheath; malignant glandular tumor of peripheral nerve sheath; Malignant Glandular Tumor of the Peripheral Nerve Sheath; malignant glandular tumor of the peripheral nerve sheath; malignant glandular tumour of the peripheral nerve sheath
SNOMED CT: Glandular malignant peripheral nerve sheath tumor (699659007); Glandular malignant peripheral nerve sheath tumor (699658004)
 
Monarch Initiative: MONDO:0004559

Definition

A variant of malignant peripheral nerve sheath tumor characterized by the presence of glandular epithelium. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant glandular tumor of peripheral nerve sheath

Recent clinical studies

Etiology

Malignant peripheral nerve sheath tumors: clinicopathological profile of 63 cases diagnosed at a tertiary cancer referral center in Mumbai, India.
Rekhi B, Ingle A, Kumar R, DeSouza MA, Dikshit R, Jambhekar NA
Indian J Pathol Microbiol 2010 Oct-Dec;53(4):611-8. doi: 10.4103/0377-4929.71998. PMID: 21045379
HMGA proteins in malignant peripheral nerve sheath tumor and synovial sarcoma: preferential expression of HMGA2 in malignant peripheral nerve sheath tumor.
Hui P, Li N, Johnson C, De Wever I, Sciot R, Manfioletti G, Tallini G
Mod Pathol 2005 Nov;18(11):1519-26. doi: 10.1038/modpathol.3800464. PMID: 16056249
Neoplasms with neural differentiation: a review. Part II: Malignant neoplasms.
Sangüeza OP, Requena L
Am J Dermatopathol 1998 Feb;20(1):89-102. doi: 10.1097/00000372-199802000-00018. PMID: 9504678
Glandular peripheral nerve sheath tumours.
Robbins P, Papadimitriou J
Pathol Res Pract 1994 Apr;190(4):412-5. doi: 10.1016/S0344-0338(11)80418-8. PMID: 8078812
Malignant peripheral nerve-sheath tumors (malignant schwannomas). An immunohistochemical study of 29 cases.
Daimaru Y, Hashimoto H, Enjoji M
Am J Surg Pathol 1985 Jun;9(6):434-44. doi: 10.1097/00000478-198506000-00005. PMID: 3937453

Diagnosis

A rare case of cutaneous pseudoglandular schwannoma.
Doan L, Cassarino DS
J Cutan Pathol 2023 Sep;50(9):798-800. Epub 2023 Jun 13 doi: 10.1111/cup.14477. PMID: 37312417
CDX2 expression in malignant peripheral nerve sheath tumour: a potential diagnostic pitfall associated with PRC2 inactivation.
Odeyemi OO, Ozawa MG, Charville GW
Histopathology 2022 May;80(6):995-1000. Epub 2022 Feb 28 doi: 10.1111/his.14626. PMID: 35122289Free PMC Article
Radiation-Induced Glandular Malignant Peripheral Nerve Sheath Tumor.
John I, Bartlett DL, Rao UNM
Int J Surg Pathol 2017 Oct;25(7):635-639. Epub 2017 May 22 doi: 10.1177/1066896917710718. PMID: 28530162
Malignant Peripheral Nerve Sheath Tumor With Divergent Glandular Differentiation.
Miki Y, Thway K
Int J Surg Pathol 2017 Jun;25(4):310-313. Epub 2017 Mar 1 doi: 10.1177/1066896917696749. PMID: 28381143
Synovial sarcoma of nerve.
Scheithauer BW, Amrami KK, Folpe AL, Silva AI, Edgar MA, Woodruff JM, Levi AD, Spinner RJ
Hum Pathol 2011 Apr;42(4):568-77. Epub 2011 Feb 4 doi: 10.1016/j.humpath.2010.08.019. PMID: 21295819

Therapy

Radiation-Induced Glandular Malignant Peripheral Nerve Sheath Tumor.
John I, Bartlett DL, Rao UNM
Int J Surg Pathol 2017 Oct;25(7):635-639. Epub 2017 May 22 doi: 10.1177/1066896917710718. PMID: 28530162

Prognosis

Synovial sarcoma of nerve.
Scheithauer BW, Amrami KK, Folpe AL, Silva AI, Edgar MA, Woodruff JM, Levi AD, Spinner RJ
Hum Pathol 2011 Apr;42(4):568-77. Epub 2011 Feb 4 doi: 10.1016/j.humpath.2010.08.019. PMID: 21295819
Malignant peripheral nerve sheath tumors: clinicopathological profile of 63 cases diagnosed at a tertiary cancer referral center in Mumbai, India.
Rekhi B, Ingle A, Kumar R, DeSouza MA, Dikshit R, Jambhekar NA
Indian J Pathol Microbiol 2010 Oct-Dec;53(4):611-8. doi: 10.4103/0377-4929.71998. PMID: 21045379
Glandular malignant peripheral nerve sheath tumor: an unusual case showing histologically malignant glands.
Nagasaka T, Lai R, Sone M, Nakashima T, Nakashima N
Arch Pathol Lab Med 2000 Sep;124(9):1364-8. doi: 10.5858/2000-124-1364-GMPNST. PMID: 10975940
Glandular peripheral nerve sheath tumours.
Robbins P, Papadimitriou J
Pathol Res Pract 1994 Apr;190(4):412-5. doi: 10.1016/S0344-0338(11)80418-8. PMID: 8078812
Glandular peripheral nerve sheath tumors.
Woodruff JM, Christensen WN
Cancer 1993 Dec 15;72(12):3618-28. doi: 10.1002/1097-0142(19931215)72:12<3618::aid-cncr2820721212>3.0.co;2-#. PMID: 8252477

Clinical prediction guides

CDX2 expression in malignant peripheral nerve sheath tumour: a potential diagnostic pitfall associated with PRC2 inactivation.
Odeyemi OO, Ozawa MG, Charville GW
Histopathology 2022 May;80(6):995-1000. Epub 2022 Feb 28 doi: 10.1111/his.14626. PMID: 35122289Free PMC Article
Claudin-4 expression distinguishes SWI/SNF complex-deficient undifferentiated carcinomas from sarcomas.
Schaefer IM, Agaimy A, Fletcher CD, Hornick JL
Mod Pathol 2017 Apr;30(4):539-548. Epub 2017 Jan 13 doi: 10.1038/modpathol.2016.230. PMID: 28084340
Glandular peripheral nerve sheath tumours.
Robbins P, Papadimitriou J
Pathol Res Pract 1994 Apr;190(4):412-5. doi: 10.1016/S0344-0338(11)80418-8. PMID: 8078812
A biphasic tumor of the mediastinum with features of synovial sarcoma. A report of four cases.
Witkin GB, Miettinen M, Rosai J
Am J Surg Pathol 1989 Jun;13(6):490-9. doi: 10.1097/00000478-198906000-00005. PMID: 2471414
Neuroendocrine differentiation in the glandular peripheral nerve sheath tumor. Pathologic distinction from the biphasic synovial sarcoma with glands.
Christensen WN, Strong EW, Bains MS, Woodruff JM
Am J Surg Pathol 1988 Jun;12(6):417-26. doi: 10.1097/00000478-198806000-00001. PMID: 2837100

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