Leiomyosarcoma differential diagnosis: Difference between revisions

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* Association with lymphangiomyomatosis and tuberous sclerosis
* Association with lymphangiomyomatosis and tuberous sclerosis
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Epitheloid angiosarcoma'''<ref>{{cite journal|doi=10.1043/1543-2165-135.2.268.}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Epitheloid [[angiosarcoma]]'''<ref>{{cite journal|doi=10.1043/1543-2165-135.2.268.}}</ref>
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* Painful, enlarging soft tissue masses  
* Painful, enlarging soft tissue masses  
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* Arteriovenous shunting and subsequent high-output cardiac failure symptoms such as dypnea on exertion.
* Arteriovenous shunting and subsequent high-output cardiac failure symptoms such as dypnea on exertion.
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* Peritoneal bleeding in some cases
* Painful, enlarging soft tissue masses
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* Epithelioid angiosarcoma has a male predilection
* Most often arises in the deep soft tissues (usually intramuscular) of the extremities, but a variety of primary sites, including the thyroid gland can be involved.
* Early nodal and solid organ metastasis, especially to the lungs, bone, soft tissue, and skin.
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Revision as of 16:18, 13 March 2019

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 leimyoma 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]
  • Abnormal vaginal bleeding (Postmenopausal or intermenstrual bleeding or bleeding from uterus or vagina)
  • Pressure or pain pelvic or abdomen
  • Vaginal dyscharge
  • Change in bladder or bowel habits
  • Fatigue
  • Fever
  • Malaise
  • Weight loss
  • Lump protruding from vagina
  • Mass in abdomen or pelvic
  • Tendenrness on palpating pelvic or Abdomen
  • Lymphadenopathy
  • ↓ RBC or Hemoglobin may be seen on CBC.
  • In uterine leiomyosarcomas (LMSs) p16 is overexpressed compared with leiomyoma.
  • Deep myometrial invasion
  • Massive uterine enlargement
  • Irregular central zones of low attenuation,
  • Extensive necrosis and hemorrhage
  • Features such as tumor localization, irregular or nodular margins, necrosis, rapid growth, intense contrast enhancement, and restriction at diffusion-weighted imaging can suggest the diagnosis and help differentiate from more common leiomyomas and endometrial carcinoma.
  • Gross histology:
  • Large, solitary, poorly circumscribed masses (average 10 cm)
  • Typically display a fleshy variegated cut surface with areas of hemorrhage or necrosis
Histology and biopsy
  • 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
  • Abscence of cytologic atypia
  • Mild nuclear atypia, up to 10-20 mitosis
  • No tumor cell necrosis
Biopsy and histology -
Hemorrhagic cellular leiomyomas (apoplectic leiomyoma)[13][14]
  • ↓ RBC or Hemoglubin on CBC
- - On microscopic analysis:
  • Zones of recent hemorrhage within nodules of hypercellular smooth muscle.
  • Coagulative necrosis (red degeneration)
  • Mitotic figure not exceeding 2/10 HPF, mostly located in the perihemorrhagic areas On gross examination:
  • Features of multiple hemorrhagic area
  • Necrosis
  • Cyst formation
  • Softening, or color different than the usual leiomyoma
Biopsy specimen and histology
PEComa[15]
  • 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
  • Association with lymphangiomyomatosis and tuberous sclerosis
Epitheloid angiosarcoma[16]
  • Painful, enlarging soft tissue masses
  • Long bone fractures
  • Arteriovenous shunting and subsequent high-output cardiac failure symptoms such as dypnea on exertion.
  • Peritoneal bleeding in some cases
  • Painful, enlarging soft tissue masses
Microscopic histology:
  • Nodules, and trabeculae of infiltrative epithelioid to spindled cells
  • Eosinophilic cytoplasm.
  • Epithelioid angiosarcoma has a male predilection
  • Most often arises in the deep soft tissues (usually intramuscular) of the extremities, but a variety of primary sites, including the thyroid gland can be involved.
  • Early nodal and solid organ metastasis, especially to the lungs, bone, soft tissue, and skin.

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. . doi:10.1043/1543-2165-135.2.268. Check |doi= value (help). Missing or empty |title= (help)


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