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Hemangiopericytoma, malignant

MedGen UID:
90803
Concept ID:
C0334542
Neoplastic Process
Synonyms: Hemangiopericytoma, Malignant
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Sources: HPO, OMIM, Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in homozygotes. In the context of medical genetics, autosomal recessive disorders manifest in homozygotes (with two copies of the mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
Autosomal recessive inheritance (HPO, OMIM, Orphanet)
SNOMED CT: Malignant hemangiopericytoma (84664004); Hemangiopericytoma, malignant (84664004)
 
OMIM®: 234820

Definition

An uncommon malignant neoplasm arising from pericytes. Distinction between benign and malignant hemangiopericytoma may be difficult or even impossible on morphologic grounds alone. [from NCI]

Clinical features

Disorder of cardiovascular system
MedGen UID:
2848
Concept ID:
C0007222
Disease or Syndrome
Any abnormality of the cardiovascular system.

Recent clinical studies

Etiology

Zhang P, Hu J, Zhou D
Turk Neurosurg 2014;24(6):948-53. doi: 10.5137/1019-5149.JTN.8299-13.1. PMID: 25448214

Diagnosis

Patrick A, Soares-de-Almeida L, Heinz K
Am J Dermatopathol 2016 Apr;38(4):307-11. doi: 10.1097/DAD.0000000000000463. PMID: 26863056
Ishizawa K, Tsukamoto Y, Ikeda S, Suzuki T, Homma T, Mishima K, Nishikawa R, Sasaki A
Brain Tumor Pathol 2016 Apr;33(2):151-6. Epub 2016 Jan 8 doi: 10.1007/s10014-015-0247-z. PMID: 26746203
Siniscalchi C, Basaglia M, Cunzi D, Merla S, Mattioli M, Gaibazzi N, Volpi R
Acta Biomed 2015 Sep 14;86(2):189-92. PMID: 26422435
Nakamura T, Ito H, Sakata K, Kobayashi Y
Asian Cardiovasc Thorac Ann 2015 Nov;23(9):1056-9. Epub 2014 Apr 2 doi: 10.1177/0218492314530979. PMID: 24696103
Ren K, Zhou X, Wu S, Sun X
Clin Neuropathol 2014 Sep-Oct;33(5):364-70. doi: 10.5414/NP300741. PMID: 24887400

Prognosis

Nakamura T, Ito H, Sakata K, Kobayashi Y
Asian Cardiovasc Thorac Ann 2015 Nov;23(9):1056-9. Epub 2014 Apr 2 doi: 10.1177/0218492314530979. PMID: 24696103
Liu J, Cao L, Liu L, Guo S, Tai H, Chen Z
Tumour Biol 2014 Nov;35(11):11655-8. Epub 2014 Aug 20 doi: 10.1007/s13277-014-2455-2. PMID: 25139096
Ren K, Zhou X, Wu S, Sun X
Clin Neuropathol 2014 Sep-Oct;33(5):364-70. doi: 10.5414/NP300741. PMID: 24887400

Clinical prediction guides

Patrick A, Soares-de-Almeida L, Heinz K
Am J Dermatopathol 2016 Apr;38(4):307-11. doi: 10.1097/DAD.0000000000000463. PMID: 26863056
Nakamura T, Ito H, Sakata K, Kobayashi Y
Asian Cardiovasc Thorac Ann 2015 Nov;23(9):1056-9. Epub 2014 Apr 2 doi: 10.1177/0218492314530979. PMID: 24696103

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