Behavior |
Malignant |
Malignant |
Malignant |
Malignant |
Mixed |
Differentiation3434 Rawish KR, Fadare O. Dedifferentiated leiomyosarcoma of the uterus with heterologous elements: a potential diagnostic pitfall. Case Rep Obstet Gynecol. 2012;2012:534634. http://dx.doi.org/10.1155/2012/534634. PMid:23119198. http://dx.doi.org/10.1155/2012/534634...
35 Özen ÖI, Ayhan A. Uterus: mixed epithelial and mesenchymal tumors - Müllerian adenosarcoma. [place unknown]; 2021 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/cervixadenosarcoma.html https://www.pathologyoutlines.com/topic/...
36 Iihara K, Hirano K, Fujioka Y, Sakamoto A. Leiomyosarcoma with dedifferentiation in a premenopausal patient discovered after uterine artery embolization. Pathol Int. 2007;57(10):681-7. http://dx.doi.org/10.1111/j.1440-1827.2007.02157.x. PMid:17803657. http://dx.doi.org/10.1111/j.1440-1827.20...
37 Nicolas MM, Tamboli P, Gomez JA, Czerniak BA. Pleomorphic and dedifferentiated leiomyosarcoma: clinicopathologic and immunohistochemical study of 41 cases. Hum Pathol. 2010;41(5):663-71. http://dx.doi.org/10.1016/j.humpath.2009.10.005. PMid:20004935. http://dx.doi.org/10.1016/j.humpath.2009...
-3838 Shankar V. Soft tissue: smooth muscle - Leiomyosarcoma; Leiomyosarcoma-general[place unknown]; 2022 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/softtissueleiomyosarcoma.html https://www.pathologyoutlines.com/topic/...
|
Skeletal muscle |
Mesenchymal tumor with no specific lineage |
Endometrial stroma |
Uterine myometrium |
Benign epithelial component with malignant stromal component |
Age group most commonly affected3333 Han L, Benett J. Uterus Stromal tumors: high grade endometrial stromal sarcoma. [place unknown]; 2021 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/uterusESShighgrade.html https://www.pathologyoutlines.com/topic/...
,3535 Özen ÖI, Ayhan A. Uterus: mixed epithelial and mesenchymal tumors - Müllerian adenosarcoma. [place unknown]; 2021 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/cervixadenosarcoma.html https://www.pathologyoutlines.com/topic/...
36 Iihara K, Hirano K, Fujioka Y, Sakamoto A. Leiomyosarcoma with dedifferentiation in a premenopausal patient discovered after uterine artery embolization. Pathol Int. 2007;57(10):681-7. http://dx.doi.org/10.1111/j.1440-1827.2007.02157.x. PMid:17803657. http://dx.doi.org/10.1111/j.1440-1827.20...
37 Nicolas MM, Tamboli P, Gomez JA, Czerniak BA. Pleomorphic and dedifferentiated leiomyosarcoma: clinicopathologic and immunohistochemical study of 41 cases. Hum Pathol. 2010;41(5):663-71. http://dx.doi.org/10.1016/j.humpath.2009.10.005. PMid:20004935. http://dx.doi.org/10.1016/j.humpath.2009...
38 Shankar V. Soft tissue: smooth muscle - Leiomyosarcoma; Leiomyosarcoma-general[place unknown]; 2022 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/softtissueleiomyosarcoma.html https://www.pathologyoutlines.com/topic/...
-3939 Allanson ER, Cummings M, Kuchel A, Wastney T, Nicklin J. Undifferentiated uterine sarcoma: a multidisciplinary challenge. Int J Gynecol Cancer. 2022;32(2):198-202. http://dx.doi.org/10.1136/ijgc-2021-003215. PMid:35131912. http://dx.doi.org/10.1136/ijgc-2021-0032...
|
1st decade of life and adolescence |
Post - menopausal; median age 7th decade |
Mostly 4th -5th decade of life |
Post-menopausal |
Peri and post-menopausal. Mean age is 50 years |
M:F ratio2727 Song JS, Song DE, Kim KR, Ro JY. Cellular pseudosarcomatous fibroepithelial stromal polyp of the vagina during pregnancy: a lesion that is overdiagnosed as a malignant tumor. Korean J Pathol. 2012;46(5):494-8. http://dx.doi.org/10.4132/KoreanJPathol.2012.46.5.494. PMid:23136578. http://dx.doi.org/10.4132/KoreanJPathol....
,3535 Özen ÖI, Ayhan A. Uterus: mixed epithelial and mesenchymal tumors - Müllerian adenosarcoma. [place unknown]; 2021 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/cervixadenosarcoma.html https://www.pathologyoutlines.com/topic/...
,3636 Iihara K, Hirano K, Fujioka Y, Sakamoto A. Leiomyosarcoma with dedifferentiation in a premenopausal patient discovered after uterine artery embolization. Pathol Int. 2007;57(10):681-7. http://dx.doi.org/10.1111/j.1440-1827.2007.02157.x. PMid:17803657. http://dx.doi.org/10.1111/j.1440-1827.20...
,3838 Shankar V. Soft tissue: smooth muscle - Leiomyosarcoma; Leiomyosarcoma-general[place unknown]; 2022 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/softtissueleiomyosarcoma.html https://www.pathologyoutlines.com/topic/...
,3939 Allanson ER, Cummings M, Kuchel A, Wastney T, Nicklin J. Undifferentiated uterine sarcoma: a multidisciplinary challenge. Int J Gynecol Cancer. 2022;32(2):198-202. http://dx.doi.org/10.1136/ijgc-2021-003215. PMid:35131912. http://dx.doi.org/10.1136/ijgc-2021-0032...
|
Conventional embryonal RMS predominates males, genital RMS - females |
Female predominant |
Female predominant |
Female predominant |
Female predominant |
Common site of presentation2020 Gibson MC, Nasiri N. Rhabdomyoma. Vienna: WikiDoc; 2019 [cited 2023 Jan 4]. Available from: https://www.wikidoc.org/index.php/Rhabdomyoma https://www.wikidoc.org/index.php/Rhabdo...
,3535 Özen ÖI, Ayhan A. Uterus: mixed epithelial and mesenchymal tumors - Müllerian adenosarcoma. [place unknown]; 2021 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/cervixadenosarcoma.html https://www.pathologyoutlines.com/topic/...
,3636 Iihara K, Hirano K, Fujioka Y, Sakamoto A. Leiomyosarcoma with dedifferentiation in a premenopausal patient discovered after uterine artery embolization. Pathol Int. 2007;57(10):681-7. http://dx.doi.org/10.1111/j.1440-1827.2007.02157.x. PMid:17803657. http://dx.doi.org/10.1111/j.1440-1827.20...
,3838 Shankar V. Soft tissue: smooth muscle - Leiomyosarcoma; Leiomyosarcoma-general[place unknown]; 2022 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/softtissueleiomyosarcoma.html https://www.pathologyoutlines.com/topic/...
,4040 Zhang Y, Chen C, Ren M, Cong X, Li Z, Yang L. Treatment of uterine high-grade endometrial stromal sarcoma with apatinib combined with chemotherapy: a case report. Medicine (Baltimore). 2019;98(13):e15050. http://dx.doi.org/10.1097/MD.0000000000015050. PMid:30921232. http://dx.doi.org/10.1097/MD.00000000000...
|
Head and neck region |
Uterus |
Uterine corpus |
Retroperitoneum |
Commonly in Uterine corpus Sites of previous endometriosis: Cervix, ovary, fallopian tube, vagina, peritoneum |
Etio -pathogenesis2020 Gibson MC, Nasiri N. Rhabdomyoma. Vienna: WikiDoc; 2019 [cited 2023 Jan 4]. Available from: https://www.wikidoc.org/index.php/Rhabdomyoma https://www.wikidoc.org/index.php/Rhabdo...
,3535 Özen ÖI, Ayhan A. Uterus: mixed epithelial and mesenchymal tumors - Müllerian adenosarcoma. [place unknown]; 2021 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/cervixadenosarcoma.html https://www.pathologyoutlines.com/topic/...
,3636 Iihara K, Hirano K, Fujioka Y, Sakamoto A. Leiomyosarcoma with dedifferentiation in a premenopausal patient discovered after uterine artery embolization. Pathol Int. 2007;57(10):681-7. http://dx.doi.org/10.1111/j.1440-1827.2007.02157.x. PMid:17803657. http://dx.doi.org/10.1111/j.1440-1827.20...
,3838 Shankar V. Soft tissue: smooth muscle - Leiomyosarcoma; Leiomyosarcoma-general[place unknown]; 2022 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/softtissueleiomyosarcoma.html https://www.pathologyoutlines.com/topic/...
,4141 Shi Y, Liu Z, Peng Z, Liu H, Yang K, Yao X. The diagnosis and treatment of Mullerian adenosarcoma of the uterus. Aust N Z J Obstet Gynaecol. 2008;48(6):596-600. http://dx.doi.org/10.1111/j.1479-828X.2008.00914.x. PMid:19133051. http://dx.doi.org/10.1111/j.1479-828X.20...
|
Cervical Embryonal RMS: Germline mutations involving the DICER1 gene Conventional embryonal RMS: Overactive IGF2 gene on chromosome 11, promoting tumorigenesis. Also associated with defects in RAS or Hedgehog pathways |
Overexpression of specific genes |
Genetic alterations such as YWHAE-NUTM2A/B fusions, BCOR fusions, BCOR ITD and high-grade transformation of low grade endometrial stromal sarcoma |
Primary etiology is unknown. It is mostly associated with EBV in HIV positive patients. |
Endometriosis, previous pelvic radiotherapy, long term unopposed estrogen therapy and tamoxifen use may be risk factors. Pathogenesis remains largely unknown. |
Gross2020 Gibson MC, Nasiri N. Rhabdomyoma. Vienna: WikiDoc; 2019 [cited 2023 Jan 4]. Available from: https://www.wikidoc.org/index.php/Rhabdomyoma https://www.wikidoc.org/index.php/Rhabdo...
,3535 Özen ÖI, Ayhan A. Uterus: mixed epithelial and mesenchymal tumors - Müllerian adenosarcoma. [place unknown]; 2021 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/cervixadenosarcoma.html https://www.pathologyoutlines.com/topic/...
,3636 Iihara K, Hirano K, Fujioka Y, Sakamoto A. Leiomyosarcoma with dedifferentiation in a premenopausal patient discovered after uterine artery embolization. Pathol Int. 2007;57(10):681-7. http://dx.doi.org/10.1111/j.1440-1827.2007.02157.x. PMid:17803657. http://dx.doi.org/10.1111/j.1440-1827.20...
,3838 Shankar V. Soft tissue: smooth muscle - Leiomyosarcoma; Leiomyosarcoma-general[place unknown]; 2022 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/softtissueleiomyosarcoma.html https://www.pathologyoutlines.com/topic/...
,4141 Shi Y, Liu Z, Peng Z, Liu H, Yang K, Yao X. The diagnosis and treatment of Mullerian adenosarcoma of the uterus. Aust N Z J Obstet Gynaecol. 2008;48(6):596-600. http://dx.doi.org/10.1111/j.1479-828X.2008.00914.x. PMid:19133051. http://dx.doi.org/10.1111/j.1479-828X.20...
|
Poorly circumscribed mass, white, soft or firm, infiltrative |
Generally, a large tumor with fleshy, pink-tan cut surface with surface hemorrhage and necrosis. |
Soft, fleshy yellow or tan pink polypoid mass, rubbery in consistency. |
Large, soft masses, often with necrosis, hemorrhage and cystic degeneration. Osseous differentiation may present with bony foci. |
Usually forms polypoid masses ranging from 1 - 20 cm. Typically fills the endometrial cavity and may project into the endocervical canal. Cut surface is solid, white to tan in color with multiple small soft and fleshy cysts containing watery or mucoid fluid. |
Microscopy2020 Gibson MC, Nasiri N. Rhabdomyoma. Vienna: WikiDoc; 2019 [cited 2023 Jan 4]. Available from: https://www.wikidoc.org/index.php/Rhabdomyoma https://www.wikidoc.org/index.php/Rhabdo...
,3535 Özen ÖI, Ayhan A. Uterus: mixed epithelial and mesenchymal tumors - Müllerian adenosarcoma. [place unknown]; 2021 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/cervixadenosarcoma.html https://www.pathologyoutlines.com/topic/...
36 Iihara K, Hirano K, Fujioka Y, Sakamoto A. Leiomyosarcoma with dedifferentiation in a premenopausal patient discovered after uterine artery embolization. Pathol Int. 2007;57(10):681-7. http://dx.doi.org/10.1111/j.1440-1827.2007.02157.x. PMid:17803657. http://dx.doi.org/10.1111/j.1440-1827.20...
37 Nicolas MM, Tamboli P, Gomez JA, Czerniak BA. Pleomorphic and dedifferentiated leiomyosarcoma: clinicopathologic and immunohistochemical study of 41 cases. Hum Pathol. 2010;41(5):663-71. http://dx.doi.org/10.1016/j.humpath.2009.10.005. PMid:20004935. http://dx.doi.org/10.1016/j.humpath.2009...
-3838 Shankar V. Soft tissue: smooth muscle - Leiomyosarcoma; Leiomyosarcoma-general[place unknown]; 2022 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/softtissueleiomyosarcoma.html https://www.pathologyoutlines.com/topic/...
|
Composed of primitive mesenchymal cells that show variable degrees of skeletal muscle differentiation with marked cytological atypia and presence of undifferentiated or fetoid cells in a background of hypocellular and hypercellular, often myxoid stroma. |
Spindle, epithelioid or polygonal cells with marked nuclear atypia and pleomorphism, frequent multinucleation and macronucleoli. |
Permeative tumors with vaguely nested growth of round cells with scant to moderate eosinophilic cytoplasm and uniform nuclear atypia with presence of brisk mitosis associated with LVI and tumour necrosis. |
Low-grade to high grade leiomyosarcoma associated with a discrete undifferentiated component lacking morphological or immunophenotypic features of myogenic differentiation |
Biphasic tumor with admixed glands and prominent stroma showing marked atypia. |
IHC features1717 Clay MR. Soft tissue; Skeletal muscle Rhabdomyosarcoma; Embryonal rhabdomyosarcoma. [place unknown]; 2022 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/softtissueembryonalrhabdo.html https://www.pathologyoutlines.com/topic/...
,3434 Rawish KR, Fadare O. Dedifferentiated leiomyosarcoma of the uterus with heterologous elements: a potential diagnostic pitfall. Case Rep Obstet Gynecol. 2012;2012:534634. http://dx.doi.org/10.1155/2012/534634. PMid:23119198. http://dx.doi.org/10.1155/2012/534634...
35 Özen ÖI, Ayhan A. Uterus: mixed epithelial and mesenchymal tumors - Müllerian adenosarcoma. [place unknown]; 2021 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/cervixadenosarcoma.html https://www.pathologyoutlines.com/topic/...
-3636 Iihara K, Hirano K, Fujioka Y, Sakamoto A. Leiomyosarcoma with dedifferentiation in a premenopausal patient discovered after uterine artery embolization. Pathol Int. 2007;57(10):681-7. http://dx.doi.org/10.1111/j.1440-1827.2007.02157.x. PMid:17803657. http://dx.doi.org/10.1111/j.1440-1827.20...
,3838 Shankar V. Soft tissue: smooth muscle - Leiomyosarcoma; Leiomyosarcoma-general[place unknown]; 2022 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/softtissueleiomyosarcoma.html https://www.pathologyoutlines.com/topic/...
,3939 Allanson ER, Cummings M, Kuchel A, Wastney T, Nicklin J. Undifferentiated uterine sarcoma: a multidisciplinary challenge. Int J Gynecol Cancer. 2022;32(2):198-202. http://dx.doi.org/10.1136/ijgc-2021-003215. PMid:35131912. http://dx.doi.org/10.1136/ijgc-2021-0032...
,4141 Shi Y, Liu Z, Peng Z, Liu H, Yang K, Yao X. The diagnosis and treatment of Mullerian adenosarcoma of the uterus. Aust N Z J Obstet Gynaecol. 2008;48(6):596-600. http://dx.doi.org/10.1111/j.1479-828X.2008.00914.x. PMid:19133051. http://dx.doi.org/10.1111/j.1479-828X.20...
42 Juhasz-Böss I, Gabriel L, Bohle RM, Horn LC, Solomayer EF, Breitbach GP. Uterine Leiomyosarcoma. Oncol Res Treat. 2018;41(11):680-6. http://dx.doi.org/10.1159/000494299. PMid:30321869. http://dx.doi.org/10.1159/000494299...
-4343 Chen J, Liu X, Lan J, et al. Rhabdomyosarcoma in adults: case series and literature review. Int J Womens Health. 2022;14:405-14. http://dx.doi.org/10.2147/IJWH.S352143. PMid:35370426. http://dx.doi.org/10.2147/IJWH.S352143...
,4141 Shi Y, Liu Z, Peng Z, Liu H, Yang K, Yao X. The diagnosis and treatment of Mullerian adenosarcoma of the uterus. Aust N Z J Obstet Gynaecol. 2008;48(6):596-600. http://dx.doi.org/10.1111/j.1479-828X.2008.00914.x. PMid:19133051. http://dx.doi.org/10.1111/j.1479-828X.20...
,4444 American Cancer Society. Uterine sarcoma early detection, diagnosis, and staging. [place unknown]; 2022 [cited 2022 Oct 22]. Available from: https://www.cancer.org/content/dam/CRC/PDF/Public/8860.00.pdf https://www.cancer.org/content/dam/CRC/P...
|
Desmin +ve Vimentin +ve Myo D1 +ve Myogenin +ve |
Nonspecific CD10+ve p53 +ve in 50% cases Focal SMA +ve Strong p16 may be seen ER and PR -ve |
CD10+ve Cyclin D1 +ve BCOR +ve ER/PR +ve Desmin -ve SMA -ve |
HHF 35, αSMA, Vimentin, Desmin, H-Caldesmon positivity in the low to high grade Leiomyosarcoma component, while the dedifferentiated component may not show any of the myogenic marker reactivity. |
Glandular component shows AE1 / AE3, ER and PR; variable CD10 reactivity; while the mesenchymal component shows vimentin, CD10, WT1, ER and PR reactivity with variable reactivity to SMA. Desmin, CD34 and Cytokeratin |
Management.3232 Chapell DB. Uterus: other mesenchymal tumors - Undifferentiated uterine sarcoma. [place unknown]; 2021 [cited 2022 Oct 22]. Available from: https://www.pathologyoutlines.com/topic/uterussarcoma.html https://www.pathologyoutlines.com/topic/...
,4242 Juhasz-Böss I, Gabriel L, Bohle RM, Horn LC, Solomayer EF, Breitbach GP. Uterine Leiomyosarcoma. Oncol Res Treat. 2018;41(11):680-6. http://dx.doi.org/10.1159/000494299. PMid:30321869. http://dx.doi.org/10.1159/000494299...
43 Chen J, Liu X, Lan J, et al. Rhabdomyosarcoma in adults: case series and literature review. Int J Womens Health. 2022;14:405-14. http://dx.doi.org/10.2147/IJWH.S352143. PMid:35370426. http://dx.doi.org/10.2147/IJWH.S352143...
44 American Cancer Society. Uterine sarcoma early detection, diagnosis, and staging. [place unknown]; 2022 [cited 2022 Oct 22]. Available from: https://www.cancer.org/content/dam/CRC/PDF/Public/8860.00.pdf https://www.cancer.org/content/dam/CRC/P...
-4545 Varghese M, Bruland O, Wiedswang AM, et al. Metastatic mesenteric dedifferentiated leiomyosarcoma: a case report and a review of literature. Clin Sarcoma Res. 2016;6(1):2. http://dx.doi.org/10.1186/s13569-016-0042-6. PMid:26913180. http://dx.doi.org/10.1186/s13569-016-004...
|
Chemotherapy, radiation therapy and surgery |
Hysterectomy with bilateral salpingo- oophorectomy with adjuvant radio and/or chemotherapy |
Chemotherapy after surgery |
Surgery aiming at complete resection. |
Surgery is the primary treatment; chemotherapy can be somewhat beneficial |
5-year survival4646 Seagle BLL, Kanis M, Strohl AE, Shahabi S. Survival of women with Mullerian adenosarcoma: a national cancer data base study. Gynecol Oncol. 2016;143(3):636-41. http://dx.doi.org/10.1016/j.ygyno.2016.10.013. PMid:27771166. http://dx.doi.org/10.1016/j.ygyno.2016.1...
,4747 Zhang YY, Li Y, Qin M, Cai Y, Jin Y, Pan LY. High-grade endometrial stromal sarcoma: a retrospective study of factors influencing prognosis. Cancer Manag Res. 2019;11:831-7. http://dx.doi.org/10.2147/CMAR.S187849. PMid:30697075. http://dx.doi.org/10.2147/CMAR.S187849...
|
27% (in adults) 61% (children) |
43% |
Stage I -90% Stage III - IV -50% |
20-30% |
79% for early stage 48% for stage III cases |