Leiomyosarcoma differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]

Overview

Leiomyosarcoma must be differentiated from other soft tissue tumors, some variants of leiomyoma resembles malignant tumor. Other possible differential diagnosis of leimyosarcoma include, epithelioid endometrial stromal sarcoma, epithelioid angiosarcoma.

Differentiating Leiomyosarcoma from other Diseases

The table below summarizes the findings that differentiate Leiomyosarcoma from other conditions that may cause similar signs and symptoms.[1][2][3]

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histology
Ultrasound CT scan MRI
Uterine Leiomyosarcoma[4][5][6]
  • Gross histology:
  • Large, solitary, poorly circumscribed masses (average 10 cm)
  • Typically display a fleshy variegated cut surface with areas of hemorrhage or necrosis
  • Women with sarcoma vs women with fibroids, tend to be older
  • More likely to be postmenopausal
  • More likely to have a history of another nonuterine malignancy
  • Women with sarcoma were more likely to have masses that are subserosal
  • Solitary rather than multiple uterine mass
Leiomyoma (mitotically active type)[7][8][9][10][11][12]
  • CBC suggestive of anemia
  • Bcl-2 expression is more frequent and strong marker in leiomyomas compared with leiomyosarcoma.
  • Enlarged uterus with multiple whorled mass lesions, largest of size 8.6×7.1 cm, not extending into the abdomen
  • An enlarged uterus and a deformed uterine contour are the most common CT findings of leiomyomas
  • Leiomyomas usually have a uniformly solid consistency

T2-weighted MRI:

  • Hypointense masses
  • Homogeneous

enhancement

  • Red degeneration
  • Mild nuclear atypia, up to 10-20 mitosis
Hemorrhagic cellular leiomyomas (apoplectic leiomyoma)[13][14]
  • Irregularly enlarged uterus, usually hypoechoic but can be isoechoic and even hyperechoic as well.
  • Enlarged uterus with irregular border
T2-weighted MRI:
  • Hypointense masses
On microscopic analysis:

Mitotic figure not exceeding 2/10 HPF, mostly located in the perihemorrhagic areas

On gross examination:

PEComa[15][16][17]
  • Mean tumor diameter of 5 cm, well-defined margins, hypodense mass
  • Low and high signal intensity on T1- and T2-weighted MRI
  • Strong, heterogeneous enhancement
Microscopic analysis:
Epitheloid angiosarcoma[18][19][20]
  • Painful, enlarging soft tissue masses
  • Peritoneal bleeding in some cases
  • Painful, enlarging soft tissue masses
  • Nonspecific
  • Enhanced huge enhanced round heterogenous solid mass with internal multifocal cysts on CT scan
T2-weighted MRI:
  • Focal areas of high signal intensity, known as the “cauliflower-like appearance” on gadolinium-enhanced MRI
Microscopic histology:
Intravenous leiomyomatosis[21][22][23]
  • No specific lab finding

References

  1. Cotton PB, Shorvon PJ (1984) Analysis of endoscopy and radiography in the diagnosis, follow-up and treatment of peptic ulcer disease. Clin Gastroenterol 13 (2):383-403. PMID: 6378443
  2. Wanebo HJ, Kennedy BJ, Chmiel J, Steele G, Winchester D, Osteen R (1993) Cancer of the stomach. A patient care study by the American College of Surgeons. Ann Surg 218 (5):583-92. PMID: 8239772
  3. Kimura T, Kamiura S, Yamamoto T, Seino-Noda H, Ohira H, Saji F (2004) Abnormal uterine bleeding and prognosis of endometrial cancer. Int J Gynaecol Obstet 85 (2):145-50. DOI:10.1016/j.ijgo.2003.12.001 PMID: 15099776
  4. Santos, Pedro; Cunha, Teresa Margarida (2015). "Uterine sarcomas: clinical presentation and MRI features". Diagnostic and Interventional Radiology. 21 (1): 4–9. doi:10.5152/dir.2014.14053. ISSN 1305-3825.
  5. Hata, Kohkichi; Hata, Toshiyuki; Makihara, Ken; Aoki, Showa; Takamiya, Osamu; Kitao, Manabu; Harada, Yuji; Nagaoka, Saburo (1990). "Sonographic Findings of Uterine Leiomyosarcoma". Gynecologic and Obstetric Investigation. 30 (4): 242–245. doi:10.1159/000293278. ISSN 1423-002X.
  6. Rha, Sung Eun; Byun, Jae Young; Jung, Seung Eun; Lee, Soo Lim; Cho, Song Mee; Hwang, Seong Su; Lee, Hae Giu; Namkoong, Sung-Eun; Lee, Jae Mun (2003). "CT and MRI of Uterine Sarcomas and Their Mimickers". American Journal of Roentgenology. 181 (5): 1369–1374. doi:10.2214/ajr.181.5.1811369. ISSN 0361-803X.
  7. Woźniak, Andrzej; Woźniak, Sławomir (2017). "Ultrasonography of uterine leiomyomas". Menopausal Review. 16 (4): 113–117. doi:10.5114/pm.2017.72754. ISSN 1643-8876.
  8. Bodner, Klaus; Bodner-Adler, Barbara; Kimberger, Oliver; Czerwenka, Klaus; Mayerhofer, Klaus (2016). "Bcl-2 Receptor Expression in Patients With Uterine Smooth Muscle Tumors: An Immunohistochemical Analysis Comparing Leiomyoma, Uterine Smooth Muscle Tumor of Uncertain Malignant Potential, and Leiomyosarcoma". Journal of the Society for Gynecologic Investigation. 11 (3): 187–191. doi:10.1016/j.jsgi.2003.10.003. ISSN 1071-5576.
  9. Adaikkalam J (April 2016). "Lipoleiomyoma of Cervix". J Clin Diagn Res. 10 (4): EJ01–2. doi:10.7860/JCDR/2016/16505.7531. PMID 27190823.
  10. Houser, L. Murray; Carrasco, C. H.; Sheehan, C. R. (1979). "Lipomatous tumour of the uterus: radiographic and ultrasonic appearance". The British Journal of Radiology. 52 (624): 992–993. doi:10.1259/0007-1285-52-624-992. ISSN 0007-1285.
  11. Keriakos, Remon; Maher, Mark (2013). "Management of Cervical Fibroid during the Reproductive Period". Case Reports in Obstetrics and Gynecology. 2013: 1–3. doi:10.1155/2013/984030. ISSN 2090-6684.
  12. Coronado GD, Marshall LM, Schwartz SM (May 2000). "Complications in pregnancy, labor, and delivery with uterine leiomyomas: a population-based study". Obstet Gynecol. 95 (5): 764–9. PMID 10775744.
  13. Myles JL, Hart WR (November 1985). "Apoplectic leiomyomas of the uterus. A clinicopathologic study of five distinctive hemorrhagic leiomyomas associated with oral contraceptive usage". Am. J. Surg. Pathol. 9 (11): 798–805. PMID 4073354.
  14. . doi:10.1097/PAS.0000000000000569. Check |doi= value (help). Missing or empty |title= (help)
  15. Tan, Y.; Zhang, H.; Xiao, E.-H. (2013). "Perivascular epithelioid cell tumour: Dynamic CT, MRI and clinicopathological characteristics—Analysis of 32 cases and review of the literature". Clinical Radiology. 68 (6): 555–561. doi:10.1016/j.crad.2012.10.021. ISSN 0009-9260.
  16. Kang, Jeong Ba; Seo, Jin Won; Park, Young-Han; Jang, Pong Rheem (2014). "Malignant Perivascular Epithelioid Cell Tumor of the Uterus with Lung Metastasis". The Korean Journal of Pathology. 48 (6): 454–457. doi:10.4132/KoreanJPathol.2014.48.6.454. ISSN 1738-1843.
  17. "PEComa of the Uterus: A Rare Mesenchymal Tumor Displaying a ≪Snowstorm≫ Pattern at Magnetic Resonance Imaging". Journal of the Belgian Society of Radiology. 100 (1). 2016. doi:10.5334/jbr-btr.926. ISSN 1780-2393.
  18. . doi:10.1043/1543-2165-135.2.268. Check |doi= value (help). Missing or empty |title= (help)
  19. Hwang, Jae Pil; Lim, Sang Moo (2013). "Uterine Epithelioid Angiosarcoma on F-18 FDG PET/CT". Nuclear Medicine and Molecular Imaging. 47 (2): 134–137. doi:10.1007/s13139-013-0191-y. ISSN 1869-3474.
  20. Chen, Innie; Firth, Bianca; Hopkins, Laura; Bougie, Olga; Xie, Ri-hua; Singh, Sukhbir (2018). "Clinical Characteristics Differentiating Uterine Sarcoma and Fibroids". JSLS : Journal of the Society of Laparoendoscopic Surgeons. 22 (1): e2017.00066. doi:10.4293/JSLS.2017.00066. ISSN 1086-8089.
  21. Nakayama Y, Kitamura S, Kawachi K, Kawata T, Fukutomi M, Hasegawa J; et al. (1994). "Intravenous leiomyomatosis extending into the right atrium". Cardiovasc Surg. 2 (5): 642–5. PMID 7820530.
  22. Kang LQ, Zhang B, Liu BG, Liu FH (January 2012). "Diagnosis of intravenous leiomyomatosis extending to heart with emphasis on magnetic resonance imaging". Chin. Med. J. 125 (1): 33–7. PMID 22340462.
  23. Fasih, Najla; Prasad Shanbhogue, Alampady K.; Macdonald, David B.; Fraser-Hill, Margaret A.; Papadatos, Demetrios; Kielar, Ania Z.; Doherty, Geoffrey P.; Walsh, Cynthia; McInnes, Matthew; Atri, Mostafa (2008). "Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations". RadioGraphics. 28 (7): 1931–1948. doi:10.1148/rg.287085095. ISSN 0271-5333.


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