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Low grade fibromyxoid sarcoma with giant collagen rosettes

MedGen UID:
310942
•Concept ID:
C1708749
•
Neoplastic Process
Synonyms: Hyalinizing Spindle Cell Tumor with Giant Rosettes; hyalinizing spindle cell tumor with giant Rosettes; hyalinizing spindle cell tumour with giant Rosettes; Low Grade Fibromyxoid Sarcoma with Giant Collagen Rosettes; low grade fibromyxoid sarcoma with giant collagen rosettes
 
Monarch Initiative: MONDO:0006273

Definition

A low grade fibromyxoid sarcoma characterized by the presence of prominent collagen rosettes. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLow grade fibromyxoid sarcoma with giant collagen rosettes

Recent clinical studies

Etiology

Low-grade fibromyxoid sarcoma: a clinicopathologic study of 18 cases, including histopathologic relationship with sclerosing epithelioid fibrosarcoma in a subset of cases.
Rekhi B, Deshmukh M, Jambhekar NA
Ann Diagn Pathol 2011 Oct;15(5):303-11. Epub 2011 May 6 doi: 10.1016/j.anndiagpath.2011.02.005. PMID: 21550274
Low-grade fibrosarcoma--report on 39 not otherwise specified cases and comparison with defined low-grade fibrosarcoma types.
Hansen T, Katenkamp K, Brodhun M, Katenkamp D
Histopathology 2006 Aug;49(2):152-60. doi: 10.1111/j.1365-2559.2006.02480.x. PMID: 16879392
Hyalinizing spindle cell tumor with giant rosettes: a distinctive tumor closely resembling low-grade fibromyxoid sarcoma.
Lane KL, Shannon RJ, Weiss SW
Am J Surg Pathol 1997 Dec;21(12):1481-8. doi: 10.1097/00000478-199712000-00011. PMID: 9414192

Diagnosis

Endometrial Stromal Sarcoma With Hyalinizing Giant Rosettes, Mimicking Low-Grade Fibromyxoid Sarcoma.
Kolson Kokohaare E, Strauss DC, Jones RL, Thway K
Int J Surg Pathol 2018 Sep;26(6):525-527. Epub 2018 Apr 6 doi: 10.1177/1066896918767547. PMID: 29623744
Low-grade fibromyxoid sarcoma with prominent giant rosettes and heterotopic ossification.
Hisaoka M, Matsuyama A, Aoki T, Sakamoto A, Yokoyama K
Pathol Res Pract 2012 Sep 15;208(9):557-60. Epub 2012 Jul 20 doi: 10.1016/j.prp.2012.06.002. PMID: 22819758
Low-grade fibromyxoid sarcoma of the perineum with heterotopic ossification: case report and review of the literature.
Lee AF, Yip S, Smith AC, Hayes MM, Nielsen TO, O'Connell JX
Hum Pathol 2011 Nov;42(11):1804-9. Epub 2011 Jun 11 doi: 10.1016/j.humpath.2011.01.023. PMID: 21658743
MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma.
Doyle LA, Möller E, Dal Cin P, Fletcher CD, Mertens F, Hornick JL
Am J Surg Pathol 2011 May;35(5):733-41. doi: 10.1097/PAS.0b013e318210c268. PMID: 21415703
Primary intrathoracic low-grade fibromyxoid sarcoma.
Jakowski JD, Wakely PE Jr
Hum Pathol 2008 Apr;39(4):623-8. Epub 2008 Feb 14 doi: 10.1016/j.humpath.2007.08.017. PMID: 18275982

Prognosis

Low-grade fibromyxoid sarcoma: a clinicopathologic study of 18 cases, including histopathologic relationship with sclerosing epithelioid fibrosarcoma in a subset of cases.
Rekhi B, Deshmukh M, Jambhekar NA
Ann Diagn Pathol 2011 Oct;15(5):303-11. Epub 2011 May 6 doi: 10.1016/j.anndiagpath.2011.02.005. PMID: 21550274
Low-grade fibrosarcoma (hyalinizing spindle cell tumor with giant rosettes) with pulmonary metastases at presentation: case report and review of the literature.
O'Sullivan MJ, Sirgi KE, Dehner LP
Int J Surg Pathol 2002 Jul;10(3):211-6. doi: 10.1177/106689690201000310. PMID: 12232578
Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes: a clinicopathologic study of 73 cases supporting their identity and assessing the impact of high-grade areas.
Folpe AL, Lane KL, Paull G, Weiss SW
Am J Surg Pathol 2000 Oct;24(10):1353-60. doi: 10.1097/00000478-200010000-00004. PMID: 11023096
Hyalinizing spindle cell tumor with giant rosettes: a distinctive tumor closely resembling low-grade fibromyxoid sarcoma.
Lane KL, Shannon RJ, Weiss SW
Am J Surg Pathol 1997 Dec;21(12):1481-8. doi: 10.1097/00000478-199712000-00011. PMID: 9414192

Clinical prediction guides

Low-grade fibromyxoid sarcoma: a clinicopathologic study of 18 cases, including histopathologic relationship with sclerosing epithelioid fibrosarcoma in a subset of cases.
Rekhi B, Deshmukh M, Jambhekar NA
Ann Diagn Pathol 2011 Oct;15(5):303-11. Epub 2011 May 6 doi: 10.1016/j.anndiagpath.2011.02.005. PMID: 21550274
Low-grade fibrosarcoma--report on 39 not otherwise specified cases and comparison with defined low-grade fibrosarcoma types.
Hansen T, Katenkamp K, Brodhun M, Katenkamp D
Histopathology 2006 Aug;49(2):152-60. doi: 10.1111/j.1365-2559.2006.02480.x. PMID: 16879392
Low-grade fibrosarcoma (hyalinizing spindle cell tumor with giant rosettes) with pulmonary metastases at presentation: case report and review of the literature.
O'Sullivan MJ, Sirgi KE, Dehner LP
Int J Surg Pathol 2002 Jul;10(3):211-6. doi: 10.1177/106689690201000310. PMID: 12232578
Hyalinising spindle cell tumour with giant rosettes: report of a case with unusual features including original histological and ultrastructural observations.
Scolyer RA, McCarthy SW, Wills EJ, Palmer AA
Pathology 2001 Feb;33(1):101-7. doi: 10.1080/00313020120034984. PMID: 11280598

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