Pleomorphic Rhabdomyosarcoma of the Uterine Corpus: A Clinicopathologic Study of 4 Cases and a Review of the Literature

被引:27
作者
Fadare, Oluwole [1 ,5 ]
Bonvicino, Amanda [1 ,2 ,3 ]
Martel, Maritza [6 ,7 ,8 ,10 ]
Renshaw, Idris L. [4 ]
Azodi, Masoud [8 ]
Parkash, Vinita [8 ,9 ]
机构
[1] Wilford Hall USAF Med Ctr, Dept Pathol, Lackland AFB, TX USA
[2] San Antonio Uniformed Serv Hlth Educ Consortium, Pathol Program, San Antonio, TX USA
[3] Brooke Army Med Ctr, Dept Pathol & Lab Serv, Ft Sam Houston, TX 78234 USA
[4] Vanguard Pathol Associates, Austin, TX USA
[5] Vanderbilt Univ, Med Ctr, Dept Pathol, Nashville, TN 37332 USA
[6] Yale New Haven Med Ctr, Dept Pathol, New Haven, CT 06504 USA
[7] Yale Univ, Sch Med, Dept Pathol, New Haven, CT 06510 USA
[8] Yale Univ, Sch Med, Dept Obstet Gynecol & Reprod Sci, New Haven, CT USA
[9] Bridgeport Hosp, Dept Pathol, Bridgeport, CT USA
[10] Providence Hlth & Serv, Portland, OR USA
关键词
Rhabdomyosarcoma; Pleomorphic; Uterus; Myo-D1; MALIGNANT RHABDOID TUMOR; ENDOMETRIAL STROMAL SARCOMA; FEMALE GENITAL-TRACT; PURE EMBRYONAL RHABDOMYOSARCOMA; ALVEOLAR RHABDOMYOSARCOMA; EPITHELIOID LEIOMYOSARCOMA; POSTMENOPAUSAL PATIENT; FALLOPIAN-TUBE; UTERUS; ADULTS;
D O I
10.1097/PGP.0b013e3181bc98c0
中图分类号
R71 [妇产科学];
学科分类号
100211 ;
摘要
We report the clinicopathologic features of 4 cases of pure pleomorphic rhabdomyosarcoma of the uterine corpus with an emphasis on their frequent expression of CD10 and CD56, review the relevant literature, and discuss differential diagnostic considerations. The patients ranged from 51 to 79 years (mean 68 y). All were FIGO stage IIIC to IV at initial surgical staging, and 3 were dead from the disease at an average of 8.6 months follow-up. In addition to the expected findings, other notable morphologic features included tumor giant cells (4/4), osteoclast-like giant cells (1/4), patchy myxoid stroma (4/4), and only infrequent cytoplasmic cross striations (1/4). The tumors in all 4 cases were positive for myogenin, myo-D1, smooth muscle actin, desmin, muscle-specific actin (HHF-35), and CD10; 3 (75%) of 4 cases were positive for calponin and CD56; all cases were negative for cytokeratin 7, synaptophysin, epithelial membrane antigen, placental-like alkaline phosphatase, chromogranin, and a pan-keratin. Twenty-three cases have been reported earlier in the English-language literature between 1969 and 2009. In combination with the current 4, the 27 patients had an age range of 35 to 87 years (mean 66.33 y). Only 1 patient was deemed inoperable; most had staging operations. Following their initial evaluations, 16 (59%) were found to have extrauterine extension of disease. At follow-up, 73% (19/27) were dead from the disease and 19.2% had no evidence of recurrence. Ten (53%) of the 19 deaths occurred within 6.5 months of initial evaluation. Stage at presentation did not have any significant impact on outcome: 73% of the 11 patients with uterus-confined disease at presentation were dead from the disease at follow-up, a rate of disease-associated death that was nearly identical to the 75% in the 16 patients with extrauterine disease at presentation. A wide variety of neoadjuvant and adjuvant therapies were administered, which did not appear to significantly impact outcomes. These data indicate that pleomorphic rhabdomyosarcoma of the uterine corpus is a highly aggressive, rapidly progressive tumor with a high case-fatality rate.
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收藏
页码:122 / 134
页数:13
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