Rhabdomyosarcoma pathophysiology: Difference between revisions
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**Retroperitoneum (6%) | **Retroperitoneum (6%) | ||
**Other sites such as bladder, vagina, nasopharynx, and middle ear (3%) | **Other sites such as bladder, vagina, nasopharynx, and middle ear (3%) | ||
* Round or spindle-shaped muscle cells are present in embryonal rhabdomyosarcoma (ERMS).<ref name="pmid10666368">{{cite journal| author=Dias P, Chen B, Dilday B, Palmer H, Hosoi H, Singh S et al.| title=Strong immunostaining for myogenin in rhabdomyosarcoma is significantly associated with tumors of the alveolar subclass. | journal=Am J Pathol | year= 2000 | volume= 156 | issue= 2 | pages= 399-408 | pmid=10666368 | doi=10.1016/S0002-9440(10)64743-8 | pmc=1850049 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10666368 }} </ref> | |||
* Alveolar architecture with small round undifferentiated cells which are seprated by dense hyalinized fibrous septa are present in alveolar rhabdomyosarcoma (ARMS). | |||
* Despite all muscle differentiation markers expression in ARMS, mature muscle characteristics is not seen in ARMS histology. | |||
===Pathogenesis=== | ===Pathogenesis=== | ||
*The exact pathogenesis of rhabdomyosarcoma is unclear.<ref name="pmid16651427">{{cite journal| author=Taulli R, Scuoppo C, Bersani F, Accornero P, Forni PE, Miretti S et al.| title=Validation of met as a therapeutic target in alveolar and embryonal rhabdomyosarcoma. | journal=Cancer Res | year= 2006 | volume= 66 | issue= 9 | pages= 4742-9 | pmid=16651427 | doi=10.1158/0008-5472.CAN-05-4292 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16651427 }} </ref> | *The exact pathogenesis of rhabdomyosarcoma is unclear.<ref name="pmid16651427">{{cite journal| author=Taulli R, Scuoppo C, Bersani F, Accornero P, Forni PE, Miretti S et al.| title=Validation of met as a therapeutic target in alveolar and embryonal rhabdomyosarcoma. | journal=Cancer Res | year= 2006 | volume= 66 | issue= 9 | pages= 4742-9 | pmid=16651427 | doi=10.1158/0008-5472.CAN-05-4292 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16651427 }} </ref> |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shadan Mehraban, M.D.[2]
Overview
Rhabdomyosarcoma arises from the skeletal muscle cells. Development of alveolar rhabdomyosarcoma is result of specific genetic mutations. The pathogenesis of rhabdomyosarcoma include t(2;13) and t(1;13) chromosomal translocations. The microscopic pathology of rhabdomyosarcoma depends on the histological subtype.
Pathophysiology
Histology
- The origin of rhabdomyosarcoma is straited muscle cells.[1]
- The presentation sites of rhabdomyosarcoma are:
- Head and neck (28%)
- Extremities (24%)
- Genitourinary tract (18%)
- Trunk (11%)
- Orbit (7%)
- Retroperitoneum (6%)
- Other sites such as bladder, vagina, nasopharynx, and middle ear (3%)
- Round or spindle-shaped muscle cells are present in embryonal rhabdomyosarcoma (ERMS).[2]
- Alveolar architecture with small round undifferentiated cells which are seprated by dense hyalinized fibrous septa are present in alveolar rhabdomyosarcoma (ARMS).
- Despite all muscle differentiation markers expression in ARMS, mature muscle characteristics is not seen in ARMS histology.
Pathogenesis
- The exact pathogenesis of rhabdomyosarcoma is unclear.[3]
- Distruption of skeletal muscle progenitor cell growth and related differentiation may cause rhabdomyosarcoma.[3]
- There is causal association between MET proto-oncogene and macrophage migration inhibitory factor (MIF).[4]
- It is thought that P53 is related to oncogenic transformation and tumor progression.[5]
Genetics
Specific genetic abnormalities have been identified, that are specific for alveolar rhabdomyosarcomas. They include t(2;13) and t(1;13) chromosomal translocations resulting in PAX3-FKHR and PAX7-FKHR gene fusions.
Microscopic Pathology
Characteristic features on microscopic analysis are variable depending on the rhabdomyosarcoma subtype:[6]
Alveolar Rhabdomyosarcoma
- Characterized by Alveolus-like pattern.
- Fibrous septae lined by tumor cells.
- Cells may "fall-off" the septa, i.e. be detached/scattered in the alveolus-like space.
- Space between fibrous septae may be filled with tumor, called solid variant of alveolar rhabdomyosarcoma.
- Rhabdomyoblasts: Essentially diagnostic cells with eccentric nucleus and moderate amount of intensely eosinophilic cytoplasm.
- Nuclear pleomorphism
- Mitoses
Embryonal Rhabdomyosarcoma
- Randomly arranged small cells
- Myxoid matrix
- Strap cells: Tadpole like morphology
- Rhabdomyoblasts: Essentially diagnostic cells with eccentric nucleus and moderate amount of intensely eosinophilic cytoplasm
Botryoid Rhabdomyosarcoma
- Malignant cells in an abundant myxoid stroma.
- Non-proliferating layer deep to the surface called "Cambium layer".
- Cambium layer is defined as cellular region deep to epithelial component.
Spindlecell Rhabdomyosarcoma
- Vesicular growth pattern
- Spindle cells
References
- ↑ Barr FG (1997). "Molecular genetics and pathogenesis of rhabdomyosarcoma". J Pediatr Hematol Oncol. 19 (6): 483–91. PMID 9407933.
- ↑ Dias P, Chen B, Dilday B, Palmer H, Hosoi H, Singh S; et al. (2000). "Strong immunostaining for myogenin in rhabdomyosarcoma is significantly associated with tumors of the alveolar subclass". Am J Pathol. 156 (2): 399–408. doi:10.1016/S0002-9440(10)64743-8. PMC 1850049. PMID 10666368.
- ↑ 3.0 3.1 Taulli R, Scuoppo C, Bersani F, Accornero P, Forni PE, Miretti S; et al. (2006). "Validation of met as a therapeutic target in alveolar and embryonal rhabdomyosarcoma". Cancer Res. 66 (9): 4742–9. doi:10.1158/0008-5472.CAN-05-4292. PMID 16651427.
- ↑ Tarnowski M, Grymula K, Liu R, Tarnowska J, Drukala J, Ratajczak J; et al. (2010). "Macrophage migration inhibitory factor is secreted by rhabdomyosarcoma cells, modulates tumor metastasis by binding to CXCR4 and CXCR7 receptors and inhibits recruitment of cancer-associated fibroblasts". Mol Cancer Res. 8 (10): 1328–43. doi:10.1158/1541-7786.MCR-10-0288. PMC 2974061. PMID 20861157.
- ↑ Xu J, Timares L, Heilpern C, Weng Z, Li C, Xu H; et al. (2010). "Targeting wild-type and mutant p53 with small molecule CP-31398 blocks the growth of rhabdomyosarcoma by inducing reactive oxygen species-dependent apoptosis". Cancer Res. 70 (16): 6566–76. doi:10.1158/0008-5472.CAN-10-0942. PMC 2922473. PMID 20682800.
- ↑ "librepathology".