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Childhood Undifferentiated Pleomorphic Sarcoma

MedGen UID:
859232
•Concept ID:
C3899638
•
Neoplastic Process
Synonym: Childhood Unclassified Pleomorphic Sarcoma

Definition

An undifferentiated pleomorphic sarcoma that occurs during childhood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood Undifferentiated Pleomorphic Sarcoma

Professional guidelines

PubMed

Intraoperative electron irradiation in the management of pediatric malignancies.
Schomberg PJ, Gunderson LL, Moir CR, Gilchrist GS, Smithson WA
Cancer 1997 Jun 1;79(11):2251-6. PMID: 9179074
Multiplex RT-PCR assay for the differential diagnosis of alveolar rhabdomyosarcoma and Ewing's sarcoma.
Downing JR, Khandekar A, Shurtleff SA, Head DR, Parham DM, Webber BL, Pappo AS, Hulshof MG, Conn WP, Shapiro DN
Am J Pathol 1995 Mar;146(3):626-34. PMID: 7887445Free PMC Article
Soft tissue sarcomas of childhood: the differential diagnostic dilemma of the small blue cell.
Dehner LP
Natl Cancer Inst Monogr 1981 Apr;(56):43-59. PMID: 6272117

Recent clinical studies

Etiology

The proteomic landscape of soft tissue sarcomas.
Burns J, Wilding CP, Krasny L, Zhu X, Chadha M, Tam YB, Ps H, Mahalingam AH, Lee ATJ, Arthur A, Guljar N, Perkins E, Pankova V, Jenks A, Djabatey V, Szecsei C, McCarthy F, Ragulan C, Milighetti M, Roumeliotis TI, Crosier S, Finetti M, Choudhary JS, Judson I, Fisher C, Schuster EF, Sadanandam A, Chen TW, Williamson D, Thway K, Jones RL, Cheang MCU, Huang PH
Nat Commun 2023 Jun 29;14(1):3834. doi: 10.1038/s41467-023-39486-2. PMID: 37386008Free PMC Article
Etiopathogenic role of ERK5 signaling in sarcoma: prognostic and therapeutic implications.
Sánchez-Fdez A, Matilla-Almazán S, Del Carmen S, Abad M, Arconada-Luque E, Jiménez-Suárez J, Chinchilla-Tábora LM, Ruíz-Hidalgo MJ, Sánchez-Prieto R, Pandiella A, Esparís-Ogando A
Exp Mol Med 2023 Jun;55(6):1247-1257. Epub 2023 Jun 19 doi: 10.1038/s12276-023-01008-x. PMID: 37332046Free PMC Article
The role of radiology in paediatric soft tissue sarcomas.
Park K, van Rijn R, McHugh K
Cancer Imaging 2008 Apr 22;8(1):102-15. doi: 10.1102/1470-7330.2008.0014. PMID: 18442956Free PMC Article
The value of adjuvant chemotherapy in the management of sarcomas in children.
Donaldson SS
Cancer 1985 May 1;55(9 Suppl):2184-97. doi: 10.1002/1097-0142(19850501)55:9+<2184::aid-cncr2820551422>3.0.co;2-n. PMID: 3884137
Primary histiocytic dermatoses.
Ringel E, Moschella S
Arch Dermatol 1985 Dec;121(12):1531-41. PMID: 2998286

Diagnosis

Etiopathogenic role of ERK5 signaling in sarcoma: prognostic and therapeutic implications.
Sánchez-Fdez A, Matilla-Almazán S, Del Carmen S, Abad M, Arconada-Luque E, Jiménez-Suárez J, Chinchilla-Tábora LM, Ruíz-Hidalgo MJ, Sánchez-Prieto R, Pandiella A, Esparís-Ogando A
Exp Mol Med 2023 Jun;55(6):1247-1257. Epub 2023 Jun 19 doi: 10.1038/s12276-023-01008-x. PMID: 37332046Free PMC Article
The role of radiology in paediatric soft tissue sarcomas.
Park K, van Rijn R, McHugh K
Cancer Imaging 2008 Apr 22;8(1):102-15. doi: 10.1102/1470-7330.2008.0014. PMID: 18442956Free PMC Article
Malignant bone tumors.
Weber K, Damron TA, Frassica FJ, Sim FH
Instr Course Lect 2008;57:673-88. PMID: 18399615
Tumors of skeletal muscle.
Agamanolis DP, Dasu S, Krill CE Jr
Hum Pathol 1986 Aug;17(8):778-95. doi: 10.1016/s0046-8177(86)80198-8. PMID: 3525381
Primary histiocytic dermatoses.
Ringel E, Moschella S
Arch Dermatol 1985 Dec;121(12):1531-41. PMID: 2998286

Therapy

Defining an embryonal rhabdomyosarcoma endotype.
Ricker CA, Crawford K, Matlock K, Lathara M, Seguin B, Rudzinski ER, Berlow NE, Keller C
Cold Spring Harb Mol Case Stud 2020 Apr;6(2) Epub 2020 Apr 1 doi: 10.1101/mcs.a005066. PMID: 32238403Free PMC Article
Malignant fibrous histiocytoma in childhood.
Cole CH, Magee JF, Gianoulis M, Rogers PC
Cancer 1993 Jun 15;71(12):4077-83. doi: 10.1002/1097-0142(19930615)71:12<4077::aid-cncr2820711245>3.0.co;2-6. PMID: 8389660
Immunohistochemical study of childhood rhabdomyosarcomas and related neoplasms. Results of an Intergroup Rhabdomyosarcoma study project.
Parham DM, Webber B, Holt H, Williams WK, Maurer H
Cancer 1991 Jun 15;67(12):3072-80. doi: 10.1002/1097-0142(19910615)67:12<3072::aid-cncr2820671223>3.0.co;2-z. PMID: 1710539
Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation.
Newton WA Jr, Soule EH, Hamoudi AB, Reiman HM, Shimada H, Beltangady M, Maurer H
J Clin Oncol 1988 Jan;6(1):67-75. doi: 10.1200/JCO.1988.6.1.67. PMID: 3275751
The value of adjuvant chemotherapy in the management of sarcomas in children.
Donaldson SS
Cancer 1985 May 1;55(9 Suppl):2184-97. doi: 10.1002/1097-0142(19850501)55:9+<2184::aid-cncr2820551422>3.0.co;2-n. PMID: 3884137

Prognosis

The proteomic landscape of soft tissue sarcomas.
Burns J, Wilding CP, Krasny L, Zhu X, Chadha M, Tam YB, Ps H, Mahalingam AH, Lee ATJ, Arthur A, Guljar N, Perkins E, Pankova V, Jenks A, Djabatey V, Szecsei C, McCarthy F, Ragulan C, Milighetti M, Roumeliotis TI, Crosier S, Finetti M, Choudhary JS, Judson I, Fisher C, Schuster EF, Sadanandam A, Chen TW, Williamson D, Thway K, Jones RL, Cheang MCU, Huang PH
Nat Commun 2023 Jun 29;14(1):3834. doi: 10.1038/s41467-023-39486-2. PMID: 37386008Free PMC Article
Etiopathogenic role of ERK5 signaling in sarcoma: prognostic and therapeutic implications.
Sánchez-Fdez A, Matilla-Almazán S, Del Carmen S, Abad M, Arconada-Luque E, Jiménez-Suárez J, Chinchilla-Tábora LM, Ruíz-Hidalgo MJ, Sánchez-Prieto R, Pandiella A, Esparís-Ogando A
Exp Mol Med 2023 Jun;55(6):1247-1257. Epub 2023 Jun 19 doi: 10.1038/s12276-023-01008-x. PMID: 37332046Free PMC Article
Neurofibromatosis type 1 and malignancy in childhood.
Varan A, Şen H, Aydın B, Yalçın B, Kutluk T, Akyüz C
Clin Genet 2016 Mar;89(3):341-5. Epub 2015 Jul 14 doi: 10.1111/cge.12625. PMID: 26073032
Malignant bone tumors.
Weber K, Damron TA, Frassica FJ, Sim FH
Instr Course Lect 2008;57:673-88. PMID: 18399615
Primary histiocytic dermatoses.
Ringel E, Moschella S
Arch Dermatol 1985 Dec;121(12):1531-41. PMID: 2998286

Clinical prediction guides

Long-term results with resection of radiation-induced soft tissue sarcomas.
Cha C, Antonescu CR, Quan ML, Maru S, Brennan MF
Ann Surg 2004 Jun;239(6):903-9; discussion 909-10. doi: 10.1097/01.sla.0000128686.51815.8b. PMID: 15166970Free PMC Article
Fas (Apo-1, CD95) receptor expression in childhood astrocytomas. Is it a marker of the major apoptotic pathway or a signaling receptor for immune escape of neoplastic cells?
Bodey B, Bodey B Jr, Siegel SE, Kaiser HE
In Vivo 1999 Jul-Aug;13(4):357-73. PMID: 10586378
Soft tissue tumours in childhood.
Malone M
Histopathology 1993 Sep;23(3):203-16. doi: 10.1111/j.1365-2559.1993.tb01192.x. PMID: 8225238
Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation.
Newton WA Jr, Soule EH, Hamoudi AB, Reiman HM, Shimada H, Beltangady M, Maurer H
J Clin Oncol 1988 Jan;6(1):67-75. doi: 10.1200/JCO.1988.6.1.67. PMID: 3275751
Undifferentiated (embryonal) sarcoma of the liver: ultrastructural and immunohistochemical similarities with malignant fibrous histiocytoma.
Keating S, Taylor GP
Hum Pathol 1985 Jul;16(7):693-9. doi: 10.1016/s0046-8177(85)80154-4. PMID: 2989150

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