Rhabdomyosarcoma pathophysiology: Difference between revisions
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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 translocation|chromosomal translocations]]. The microscopic pathology of rhabdomyosarcoma depends on the histological subtype. | 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 translocation|chromosomal translocations]]. The microscopic pathology of rhabdomyosarcoma depends on the histological subtype. | ||
==Pathophysiology== | |||
==Histology== | ===Histology=== | ||
* The origin of rhabdomyosarcoma is muscle cells. | * The origin of rhabdomyosarcoma is muscle cells.<ref name="pmid9407933">{{cite journal| author=Barr FG| title=Molecular genetics and pathogenesis of rhabdomyosarcoma. | journal=J Pediatr Hematol Oncol | year= 1997 | volume= 19 | issue= 6 | pages= 483-91 | pmid=9407933 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9407933 }} </ref> | ||
* The presentation sites of rhabdomyosarcoma are: | * The presentation sites of rhabdomyosarcoma are: | ||
** Head and neck (28%) | ** Head and neck (28%) |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [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 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%)
Genetics
Alveolar rhabdomyosarcoma
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:[2]
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.
- ↑ "librepathology".