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Adenosarcoma
From Wikipedia, the free encyclopedia

Main page Adenosarcoma (also Mullerian Adenosarcoma) is a rare malignant tumor that occurs in women
Contents of all age groups, but most commonly post-menopause. Adenosarcoma arises from
Current events mesenchymal tissue and has a mixture of the tumoral components of an adenoma, a tumor of
Random article epithelial origin, and a sarcoma, a tumor originating from connective tissue.[1][2] The adenoma,
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or epithelial component of the tumor, is benign, while the sarcomatous stroma is malignant.[3]
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The most common site of adenosarcoma formation is the uterus, but it can also occur in the
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cervix and ovaries. It more rarely arises in the vagina and fallopian tubes as well as primary
Contribute pelvic or peritoneal sites, such as the omentum, especially in those with a history of
Help endometriosis.[3][4] The rare cases of adenosarcoma outside the female genital tract usually
Learn to edit occur in the liver,[5][6] bladder,[7][6] kidney,[6][8] as well as the intestine [4] and are typically
Community portal associated with endometriosis.[9]
Recent changes
Upload file Mullerian adenosarcoma with sarcomatous overgrowth is a very aggressive form of
adenosarcoma that is characterized by post-operative recurrence and metastases even when
Tools diagnosed at an early stage.[10] Sarcomatous overgrowth is diagnosed when the sarcomatous
What links here portion of the adenosarcoma makes up more than 25% of the tumor.[3] Adenosarcomas do not
Related changes typically have distant metastases, but they have a propensity for local recurrence.[3][11][12]
Special pages
Permanent link Contents []
Page information
1 Uterine Adenosarcoma
Cite this page
1.1 Risk Factors
Wikidata item
1.2 Treatment
Print/export 1.3 Recurrence and Survival

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2 Ovarian Adenosarcoma
Printable version 2.1 Risk Factors
2.2 Treatment
Languages 2.3 Recurrence and Survival
‫العربية‬ 3 Cervical Adenosarcoma
Italiano 4 See also
Slovenčina 5 References
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Uterine Adenosarcoma ​[ edit ]

Uterine adenosarcoma are a subtype of uterine sarcomas. Uterine sarcomas account for 3 to
9 % of uterine cancers, and 5.5 to 9 % of uterine sarcomas are adenosarcomas.[6] The most
common presenting symptom is abnormal vaginal bleeding. Other symptoms include pelvic
pain, abdominal mass, or vaginal discharge.[13] Uterine adenosarcoma commonly arise from the
[1]
endometrium.

Uterine adenosarcomas have the highest incidence in perimenopasual and postmenopausal


women with a mean age of 50 years, but some incidence among children. Survival is better
compared to other types of uterine sarcomas. The prognosis of uterine adenosarcoma depends
on the stage and if sarcomatous overgrowth is present.[14]

Risk Factors ​[ edit ]

No definitive causes of adenosarcoma have been identified. Potential risk factors include a
medical history of endometriosis and use of estrogen modulating agents such as tamoxifen.
Other potential risk factors include previous pelvic irradiation and prolonged estrogen exposure
[6][15]

Treatment ​[ edit ]

The standard care of treatment is total abdominal hysterectomy with bilateral salpingo-
oophorectomy. Lymphadenectomy is usually not performed as the incidence of lymph node
metastasis is rare. There is no standardized chemotherapy, hormone therapy, or radiation
therapy.[6][16][17] Because of the rarity of adenosarcoma, there is limited data to guide treatment
decisions, particularly in regard to recurrent or metastatic tumors.[17] Chemotherapy may be
considered in patients with recurrence or tumors unable to be completely removed through
surgery. It has been suggested that uterine adenosarcomas can respond to
doxorubicin/ifosfamide and gemcitabine/docetaxel chemotherapy.[18] The use of hormone
therapy in recurrent or metastatic disease is limited to case reports.[17]

Recurrence and Survival ​[ edit ]

Survival is influenced by the presence of myometrial invasion, sarcomatous overgrowth,


lymphovascular invasion, necrosis, and the presence of heterologous elements, which are
features in the tumor not native to the tissue of origin such as rhabdomyoblastic differentiation [6]
Post-operative recurrence is common in uterine adenosarcomas.[3] Recurrence usually occurs
in the vagina, pelvis, and abdomen, and is seen in up to 30% of cases resulting in a poor
prognosis.[14]

The presence and depth of the sarcoma’s myometrial invasion determines early staging
diagnosis. The FIGO (International Federation of Gynecology and Obstetrics) staging is IA: no
myometrial invasion, IB: inner myometrial half, IC: outer myometrial half. If confined to the
endometrium with no myometrial invasion (IA), the prognosis is good with 7-13% recurrence for
noninvasive tumors.[16] FIGO stage II or greater is considered advanced with overall survival of
approximately 60% with myometrial invasion, but less than 50% if metastases are present. High
grade adenosarcomas tend to have extrauterine spread and rapid recurrence.[14]
Adenosarcoma with myometrial invasion recurred in 36-46% of cases.[14][16] Patients with
sarcomatous overgrowth showed significantly increased risk of recurrence, around 70-77 %, a
risk of metastases around 40%, and a decreased 5-year overall survival, 50 to 60 %. This is
comparable to other high grade uterine sarcomas.[6][16]

​[ edit ]
Ovarian Adenosarcoma
Ovarian adenosarcoma is a very rare tumor effecting the ovaries. 97.5% of ovarian
adenosarcomas are unilateral,[19] affecting only one ovary. It mainly affects women of
reproductive age 30-84, with a mean age of 54. Symptoms of ovarian adenosarcoma include
abdominal or pelvic pain and abdominal swelling. Tumor may present as adnexal mass.[20]

Risk Factors ​[ edit ]

Most of the cases reported have associated endometriosis or an adenosarcoma arising from an
endometriotic area, but the direct relation between this tumor and endometriosis has not been
made clear in the literature.[21]

Treatment ​[ edit ]

Typically, ovarian adenosarcomas are surgically removed via salphingopherectomy or


panhysterectomy. 67% of patients had tumor rupture at or before excision. There is no
standardized chemotherapy, hormone therapy, or radiation therapy due to limited data.[20]

Recurrence and Survival ​[ edit ]

Ovarian adenosarcomas have a worse prognosis than uterine adenosarcomas, presumably


because of the greater ease of peritoneal spread. Many of these ovarian tumors have caused
problems in differential diagnosis.[19] Advanced stage ovarian adenosarcoma is characterized
by extraovarian spreading, sarcomatous overgrowth, and tumor rupture.[20] The presence of
sarcomtous overgrowth is associated with increased risk of recurrence or extraovarian
spreading. Recurrence poses more of a threat than metastases, which appear to be less
prevalent.[19] 5 year survival is 64%, 10 year survival is 46%.[20]

Cervical Adenosarcoma ​[ edit ]

Cervical adenosarcoma is an extremely rare tumor that occurs most often in women of
reproductive age. Typically, adenosarcoma found in the cervix is a result of extrauterine
spreading of the tumor, though adenosarcoma can arise in the cervix.[3] Symptoms of cervical
adenosarcoma, like uterine adenosarcoma, are characterized by abnormal bleeding.[22]
Treatment mainly consists of total abdominal hysterectomy, occasionally with additional
radiotherapy and chemotherapy.[22] Prognosis of cervical adenosarcoma is usually favorable,
with metastasis and recurrence of the tumor being less common. As with uterine
adenosarcoma, the depth of myometrial invasion determines the prognosis, with deeper
invasion being associated with metastases and tumor recurrence.[22]

See also ​[ edit ]

Sarcoma

References ​[ edit ]
1. ^ a b NCI Dictionary of Cancer Terms: Adenosarcoma.” National Cancer Institute, National
Institutes of Health, www.cancer.gov/publications/dictionaries/cancer-terms/def/adenosarcoma.
2. ^ Carroll, A., Ramirez, P. T., Westin, S. N., Soliman, P. T., Munsell, M. F., Nick, A. M., ... &
Fleming, N. D. (2014). Uterine adenosarcoma: an analysis on management, outcomes, and risk
factors for recurrence. Gynecologic oncology, 135(3), 455-461.
3. ^ a b c d e f Podduturi, V., & Pinto, K. R. (2016, January). Mullerian adenosarcoma of the cervix
with heterologous elements and sarcomatous overgrowth. In Baylor University Medical Center
Proceedings (Vol. 29, No. 1, pp. 65-67). Taylor & Francis.
4. ^ a b Mullerian Adenosarcoma of the Female Genital Tract. McCluggage, W. Glenn
MD [Review] Advances in Anatomic Pathology. 17(2):122-129, March 2010.
5. ^ Volkov VP, Lazdin OA, Sadikov ID. Adenosarcoma of the liver in patient with liver cirrhosis.
Klin Med (Mosk). 1979;57(3):105–7.
6. ^ a b c d e f g h Nathenson, M. J., Ravi, V., Fleming, N., Wang, W. L., & Conley, A. (2016).
Uterine adenosarcoma: a review. Current oncology reports, 18(11), 68.
7. ^ Vara AR, Ruzics EP, Moussabeck O, Martin DC. Endometrioid adenosarcoma of the bladder
arising from endometriosis. J Urol. 1990;143(4):813–5.
8. ^ Sameshima N, Marutsuka K, Tsukino H, Kamoto T, Kono S, Asada Y. So-called
‘adenosarcoma’ of the kidney a novel adult renal tumor with a cystic appearance. Pathol Int.
2011;61(5):313–8.
9. ^ Kondi-Pafiti A, Spanidou-Carvouni H, Papadias K, Hatzistamou-Kiari I, Kontogianni K, Liapis
A, et al. Malignant neoplasms arising in endometriosis: clinicopathological study of 14 cases.
Clin Exp Obstet Gynecol. 2004;31(4):302–4.
10. ^ Patrelli, T. S., Gizzo, S., Di Gangi, S., Guidi, G., Rondinelli, M., & Nardelli, G. B. (2011).
Cervical Mullerian adenosarcoma with heterologous sarcomatous overgrowth: a fourth case and
review of literature. BMC cancer, 11(1), 236.
11. ^ Clement PB, Scully RE. Mullerian adenosarcoma of the uterus: a clinicopathologic analysis of
100 cases with a review of the literature. Hum Pathol. 1990;21(4):363–381
12. ^ Verschraegen CF, Vasuratna A, Edwards C, Freedman R, Kudelka AP, Tornos C, Kavanagh
JJ. Clinicopathologic analysis of mullerian adenosarcoma: the M.D. Anderson Cancer Center
experience. Oncol Rep. 1998;5(4):939–944
13. ^ McCluggage, Glenn W. “Uterine Adenosarcoma.” Uterine Adenosarcoma, Department of
Pathology, Royal Group of Hospitals Trust, Belfast, Northern Ireland, 2009,
uscapknowledgehub.org/site~/98th/pdf/companion12h05.pdf.
14. ^ a b c d Andre Pinto and Brooke Howitt (2016) Uterine Adenosarcoma. Archives of Pathology &
Laboratory Medicine: March 2016, Vol. 140, No. 3, pp. 286-290.
15. ^ Stern RC, Dash R, Bentley RC, Snyder MJ, Haney AF, Robboy SJ. Malignancy in
endometriosis: frequency and comparison of ovarian and extraovarian types. Int J Gynecol
Pathol. 2001;20(2):133–9.
16. ^ a b c d Özen Ö. Müllerian adenosarcoma. PathologyOutlines.com website.
http://www.pathologyoutlines.com/topic/uterusadenosarcoma.html . Accessed March 20th,
2019
17. ^ a b c Nathenson, M. J., Conley, A. P., Lin, H., Fleming, N., & Ravi, V. (2017). Treatment of
recurrent or metastatic uterine adenosarcoma. Sarcoma, 2017.
18. ^ M. J. Nathenson, V. Ravi, N. Fleming, W.-L. Wang, and A. Conley, “Uterine adenosarcoma: a
review,” Current Oncology Reports, vol. 18, no. 11, p. 68, 2016.
19. ^ a b c Eichhorn JH, Young RH, Clement PB, Scully RE. Mesodermal (mullerian) adenosarcoma
of the ovary: a clinicopathologic analysis of 40 cases and a review of the literature. Am J Surg
Pathol. 2002;26(10):1243–58.
20. ^ a b c d Gupta N Müllerian adenosarcoma. PathologyOutlines.com website.
http://www.pathologyoutlines.com/topic/ovarytumormullerianadeno.html . Accessed March
25th, 2019.
21. ^ Shakuntala, P. N., Umadevi, K., Usha, A., Abhilasha, N., & Bafna, U. D. (2012). Primary
ovarian adenosarcoma with elevated Ca-125 levels and normal ascitic fluid cytology: a case
report and review of literature. ecancermedicalscience, 6.
22. ^ a b c Jones, M. W., & Lefkowitz, M. (1995). Adenosarcoma of the uterine cervix: a
clinicopathological study of 12 cases. International journal of gynecological pathology: official
journal of the International Society of Gynecological Pathologists, 14(3), 223-229.

Categories: Types of cancer

This page was last edited on 8 September 2020, at 07:01 (UTC).

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