Sarcoma of the Uterus. Guideline of the DGGG, OEGGG and SGGG (S2k-Level, AWMF Registry No. 015/074, April 2021)

Abstract

Purpose This is an official guideline, published and coordinated by the Germany Society for Gynecology and Obstetrics (Deutsche Gesellschaft für Gynäkologie und Geburtshilfe, DGGG). Because of their rarity and heterogeneous histopathology, uterine sarcomas are challenging in terms of their clinical management and therefore require a multidisciplinary approach. To our knowledge, there are currently no binding evidence-based recommendations for the appropriate management of this heterogeneous group of tumors.

Methods This S2k guideline was first published in 2015. The update published here is once again the result of the consensus of a representative interdisciplinary committee of experts who were commissioned by the Guidelines Committee of the DGGG to carry out a systematic search of the literature on uterine sarcomas. Members of the participating professional societies achieved a formal consensus after a structured consensus process.

Recommendations 1.1 Epidemiology, classification, staging of uterine sarcomas. 1.2 Symptoms, general diagnostic workup, general pathology or genetic predisposition to uterine sarcomas. 2. Management of leiomyosarcomas. 3. Management of low-grade endometrial stromal sarcomas. 4. Management of high-grade endometrial stromal sarcoma and undifferentiated uterine sarcomas. 5. Management of adenosarcomas. 6. Rhabdomyosarcomas of the uterus in children and adolescents. 7. Follow-up of uterine sarcomas. 8. Management of morcellated uterine sarcomas. 9. Information provided to patients.

Key words

guideline - uterine sarcoma - leiomyosarcoma - endometrial stromal sarcoma - adenosarcoma - morcellation

Publication History

Received: 05 July 2022

Accepted after revision: 11 July 2022

Article published online:
01 December 2022

• References/Literatur

• 1 Wittekind C, Meyer HJ. TNM-Klassifikation maligner Tumoren. Weinheim: Wiley-VHC Verlag; 2010
• 2 Oliva E, Carcangiu ML, Carinelli SG, Ip P, Loening T, Longacre TA, Nucci MR, Prat J, Zaloudek CJ. Mesenchymal Tumors of the Uterus. In: Kurman RJ, Carcangiu ML, Herrington CS, Young RH. eds. WHO Classification of Tumours of female reproductive Tract. Lyon: IARC Press; 2014: 135-147
• 3 Conklin CM, Longacre TA. Endometrial stromal tumors: the new WHO classification. Adv Anat Pathol 2014; 21: 383-393
• 4 Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. WHO Classification of soft Tissue and Bone. Lyon: IARC Press; 2013
• 5 Halaska MJ, Haidopoulos D, Guyon F. et al. European Society of Gynecological Oncology Statement on Fibroid and Uterine Morcellation. Int J Gynecol Cancer 2017; 27: 189-192
• 6 Skorstad M, Kent A, Lieng M. Preoperative evaluation in women with uterine leiomyosarcoma. A nationwide cohort study. Acta Obstet Gynecol Scand 2016; 95: 1228-1234
• 7 Sizzi O, Manganaro L, Rossetti A. et al. Assessing the risk of laparoscopic morcellation of occult uterine sarcomas during hysterectomy and myomectomy: Literature review and the ISGE recommendations. Eur J Obstet Gynecol Reprod Biol 2018; 220: 30-38
• 8 Kohler G, Vollmer M, Nath N. et al. Benign uterine mass-discrimination from leiomyosarcoma by a preoperative risk score: a multicenter cohort study. Arch Gynecol Obstet 2019; 300: 1719-1727
• 9 Park JY, Park SK, Kim DY. et al. The impact of tumor morcellation during surgery on the prognosis of patients with apparently early uterine leiomyosarcoma. Gynecol Oncol 2011; 122: 255-259
• 10 Amant F, Coosemans A, Debiec-Rychter M. et al. Clinical management of uterine sarcomas. Lancet Oncol 2009; 10: 1188-1198
• 11 Coffey D, Kaplan AL, Ramzy I. Intraoperative consultation in gynecologic pathology. Arch Pathol Lab Med 2005; 129: 1544-1557
• 12 Otis CN, Ocampo AC, Nucci MR. et al. Protocol for the Examination of Specimens From Patients With Sarcoma. 2013. Accessed August 26, 2022 at: https://uatcap.objects.frb.io/protocols/cp-uterine-sarcoma-2016-v3100.pdf
• 13 McCluggage WG, Fisher C, Hirschowitz L. Dataset for histological reporting of uterine sarcomas. 2014 Accessed August 26, 2022 at: https://www.rcpath.org/uploads/assets/c146b1c0-0e0e-47a4-a6ab29b3230d69f8/G106-Dataset-for-histopathological-reporting-of-uterine-sarcomas.pdf
• 14 Ognjanovic S, Olivier M, Bergemann TL. et al. Sarcomas in TP53 germline mutation carriers: a review of the IARC TP53 database. Cancer 2012; 118: 1387-1396
• 15 Li FP, Fraumeni jr. JF, Mulvihill JJ. et al. A cancer family syndrome in twenty-four kindreds. Cancer Res 1988; 48: 5358-5362
• 16 Koivisto-Korander R, Martinsen JI, Weiderpass E. et al. Incidence of uterine leiomyosarcoma and endometrial stromal sarcoma in Nordic countries: results from NORDCAN and NOCCA databases. Maturitas 2012; 72: 56-60
• 17 Zaloudek CJ, Hendrickson MR, Soslow RA. Mesenchymal Tumors of the Uterus. In: Kurman RJ, Hedrick Ellenson L, Ronnett BM. eds. Blausteinʼs Pathology of the female genital Tract. 6th ed. New York, Dodrecht, Heidelberg, London: Springer; 2011
• 18 Ip PP, Cheung AN. Pathology of uterine leiomyosarcomas and smooth muscle tumours of uncertain malignant potential. Best Pract Res Clin Obstet Gynaecol 2011; 25: 691-704
• 19 Prayson RA, Goldblum JR, Hart WR. Epithelioid smooth-muscle tumors of the uterus: a clinicopathologic study of 18 patients. Am J Surg Pathol 1997; 21: 383-391
• 20 [Anonym] WHO Classification of Tumours Editorial Board. WHO Classification of Tumours of Soft Tissue and Bone. 5th ed. Lyon: IARC Press; 2020
• 21 Oliva E. Practical issues in uterine pathology from banal to bewildering: the remarkable spectrum of smooth muscle neoplasia. Mod Pathol 2016; 29 (Suppl. 01) S104-S120
• 22 Pelmus M, Penault-Llorca F, Guillou L. et al. Prognostic factors in early-stage leiomyosarcoma of the uterus. Int J Gynecol Cancer 2009; 19: 385-390
• 23 Iasonos A, Keung EZ, Zivanovic O. et al. External validation of a prognostic nomogram for overall survival in women with uterine leiomyosarcoma. Cancer 2013; 119: 1816-1822
• 24 Garg G, Shah JP, Kumar S. et al. Ovarian and uterine carcinosarcomas: a comparative analysis of prognostic variables and survival outcomes. Int J Gynecol Cancer 2010; 20: 888-894
• 25 Pritts EA, Parker WH, Brown J. et al. Outcome of occult uterine leiomyosarcoma after surgery for presumed uterine fibroids: a systematic review. J Minim Invasive Gynecol 2015; 22: 26-33
• 26 Kapp DS, Shin JY, Chan JK. Prognostic factors and survival in 1396 patients with uterine leiomyosarcomas: emphasis on impact of lymphadenectomy and oophorectomy. Cancer 2008; 112: 820-830
• 27 Nasioudis D, Chapman-Davis E, Frey M. et al. Safety of ovarian preservation in premenopausal women with stage I uterine sarcoma. J Gynecol Oncol 2017; 28: e46
• 28 Leitao MM, Sonoda Y, Brennan MF. et al. Incidence of lymph node and ovarian metastases in leiomyosarcoma of the uterus. Gynecol Oncol 2003; 91: 209-212
• 29 Seagle BL, Sobecki-Rausch J, Strohl AE. et al. Prognosis and treatment of uterine leiomyosarcoma: A National Cancer Database study. Gynecol Oncol 2017; 145: 61-70
• 30 Bogani G, Fuca G, Maltese G. et al. Efficacy of adjuvant chemotherapy in early stage uterine leiomyosarcoma: A systematic review and meta-analysis. Gynecol Oncol 2016; 143: 443-447
• 31 Chae SH, Shim SH, Chang M. et al. Effect of adjuvant therapy on the risk of recurrence in early-stage leiomyosarcoma: A meta-analysis. Gynecol Oncol 2019; 154: 638-650
• 32 Pautier P, Floquet A, Gladieff L. et al. A randomized clinical trial of adjuvant chemotherapy with doxorubicin, ifosfamide, and cisplatin followed by radiotherapy versus radiotherapy alone in patients with localized uterine sarcomas (SARCGYN study). A study of the French Sarcoma Group. Ann Oncol 2013; 24: 1099-1104
• 33 Hensley ML, Ishill N, Soslow R. et al. Adjuvant gemcitabine plus docetaxel for completely resected stages I–IV high grade uterine leiomyosarcoma: Results of a prospective study. Gynecol Oncol 2009; 112: 563-567
• 34 Hensley ML, Wathen JK, Maki RG. et al. Adjuvant therapy for high-grade, uterus-limited leiomyosarcoma: results of a phase 2 trial (SARC005). Cancer 2013; 119: 1555-1561
• 35 Hensley ML, Enserro D, Hatcher H. et al. Adjuvant Gemcitabine Plus Docetaxel Followed by Doxorubicin Versus Observation for High-Grade Uterine Leiomyosarcoma: A Phase III NRG Oncology/Gynecologic Oncology Group Study. J Clin Oncol 2018;
• 36 Reed NS, Mangioni C, Malmström H. et al. European Organisation for Research and Treatment of Cancer Gynaecological Cancer Group. Phase III randomised study to evaluate the role of adjuvant pelvic radiotherapy in the treatment of uterine sarcomas stages I and II: an European Organisation for Research and Treatment of Cancer Gynaecological Cancer Group Study (protocol 55874). Eur J Cancer 2008; 44: 808-818
• 37 Bernstein-Molho R, Grisaro D, Soyfer V. et al. Metastatic uterine leiomyosarcomas: a single-institution experience. Int J Gynecol Cancer 2010; 20: 255-260
• 38 Leitao MM, Brennan MF, Hensley M. et al. Surgical resection of pulmonary and extrapulmonary recurrences of uterine leiomyosarcoma. Gynecol Oncol 2002; 87: 287-294
• 39 Levenback C, Rubin SC, McCormack PM. et al. Resection of pulmonary metastases from uterine sarcomas. Gynecol Oncol 1992; 45: 202-205
• 40 Weiser MR, Downey RJ, Leung DH. et al. Repeat resection of pulmonary metastases in patients with soft-tissue sarcoma. J Am Coll Surg 2000; 191: 184-190 discussion 190–191
• 41 Giuntoli RL2nd Garrett-Mayer E, Bristow RE. et al. Secondary cytoreduction in the management of recurrent uterine leiomyosarcoma. Gynecol Oncol 2007; 106: 82-88
• 42 Anraku M, Yokoi K, Nakagawa K. et al. Metastatic Lung Tumor Study Group of Japan. Pulmonary metastases from uterine malignancies: results of surgical resection in 133 patients. J Thorac Cardiovasc Surg 2004; 127: 1107-1112
• 43 Chen H, Pruitt A, Nicol TL. et al. Complete hepatic resection of metastases from leiomyosarcoma prolongs survival. J Gastrointest Surg 1998; 2: 151-155
• 44 Cybulska P, Sioulas V, Orfanelli T. et al. Secondary surgical resection for patients with recurrent uterine leiomyosarcoma. Gynecol Oncol 2019; 154: 333-337
• 45 Sutton GP, Blessing JA, Barrett RJ. et al. Phase II trial of ifosfamide and mesna in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Am J Obstet Gynecol 1992; 166: 556-559
• 46 Look KY, Sandler A, Blessing JA. et al. Gynecologic Oncology Group (GOG) Study. Phase II trial of gemcitabine as second-line chemotherapy of uterine leiomyosarcoma: a Gynecologic Oncology Group (GOG) Study. Gynecol Oncol 2004; 92: 644-647
• 47 Thigpen T, Blessing JA, Yordan E. et al. Phase II trial of etoposide in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Gynecol Oncol 1996; 63: 120-122
• 48 Rose PG, Blessing JA, Soper JT. et al. Prolonged oral etoposide in recurrent or advanced leiomyosarcoma of the uterus: a gynecologic oncology group study. Gynecol Oncol 1998; 70: 267-271
• 49 Miller DS, Blessing JA, Kilgore LC. et al. Phase II trial of topotecan in patients with advanced, persistent, or recurrent uterine leiomyosarcomas: a Gynecologic Oncology Group Study. Am J Clin Oncol 2000; 23: 355-357
• 50 Gallup DG, Blessing JA, Andersen W. et al. Gynecologic Oncology Group Study. Evaluation of paclitaxel in previously treated leiomyosarcoma of the uterus: a gynecologic oncology group study. Gynecol Oncol 2003; 89: 48-51
• 51 Sutton G, Blessing JA, Malfetano JH. Ifosfamide and doxorubicin in the treatment of advanced leiomyosarcomas of the uterus: a Gynecologic Oncology Group study. Gynecol Oncol 1996; 62: 226-229
• 52 Hensley ML, Maki R, Venkatraman E. et al. Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol 2002; 20: 2824-2831
• 53 Gupta AA, Yao X, Verma S. et al. Sarcoma Disease Site Group and the Gynecology Cancer Disease Site Group. Systematic chemotherapy for inoperable, locally advanced, recurrent, or metastatic uterine leiomyosarcoma: a systematic review. Clin Oncol (R Coll Radiol) 2013; 25: 346-355
• 54 Maki RG, Wathen JK, Patel SR. et al. Randomized phase II study of gemcitabine and docetaxel compared with gemcitabine alone in patients with metastatic soft tissue sarcomas: results of sarcoma alliance for research through collaboration study 002 [corrected]. J Clin Oncol 2007; 25: 2755-2763
• 55 Pautier P, Floquet A, Penel N. et al. Randomized multicenter and stratified phase II study of gemcitabine alone versus gemcitabine and docetaxel in patients with metastatic or relapsed leiomyosarcomas: a Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) French Sarcoma Group Study (TAXOGEM study). Oncologist 2012; 17: 1213-1220
• 56 Seddon B, Strauss SJ, Whelan J. et al. Gemcitabine and docetaxel versus doxorubicin as first-line treatment in previously untreated advanced unresectable or metastatic soft-tissue sarcomas (GeDDiS): a randomised controlled phase 3 trial. Lancet Oncol 2017; 18: 1397-1410
• 57 Demetri GD, Chawla SP, von Mehren M. et al. Efficacy and safety of trabectedin in patients with advanced or metastatic liposarcoma or leiomyosarcoma after failure of prior anthracyclines and ifosfamide: results of a randomized phase II study of two different schedules. J Clin Oncol 2009; 27: 4188-4196
• 58 van der Graaf WT, Blay JY, Chawla SP. et al. EORTC Soft Tissue and Bone Sarcoma Group; PALETTE study group. Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet 2012; 379: 1879-1886
• 59 Oliva E. Cellular mesenchymal tumors of the uterus: a review emphasizing recent observations. Int J Gynecol Pathol 2014; 33: 374-384
• 60 Chew I, Oliva E. Endometrial stromal sarcomas: a review of potential prognostic factors. Adv Anat Pathol 2010; 17: 113-121
• 61 Chang KL, Crabtree GS, Lim-Tan SK. et al. Primary uterine endometrial stromal neoplasms. A clinicopathologic study of 117 cases. Am J Surg Pathol 1990; 14: 415-438
• 62 Barney B, Tward JD, Skidmore T. et al. Does radiotherapy or lymphadenectomy improve survival in endometrial stromal sarcoma?. Int J Gynecol Cancer 2009; 19: 1232-1238
• 63 Park JY, Baek MH, Park Y. et al. Investigation of hormone receptor expression and its prognostic value in endometrial stromal sarcoma. Virchows Arch 2018; 473: 61-69
• 64 Chu MC, Mor G, Lim C. et al. Low-grade endometrial stromal sarcoma: hormonal aspects. Gynecol Oncol 2003; 90: 170-176
• 65 Einstein MH, Barakat RR, Chi DS. et al. Management of uterine malignancy found incidentally after supracervical hysterectomy or uterine morcellation for presumed benign disease. Int J Gynecol Cancer 2008; 18: 1065-1070
• 66 Nasioudis D, Ko EM, Kolovos G. et al. Ovarian preservation for low-grade endometrial stromal sarcoma: a systematic review of the literature and meta-analysis. Int J Gynecol Cancer 2019; 29: 126-132
• 67 Chan JK, Kawar NM, Shin JY. et al. Endometrial stromal sarcoma: a population-based analysis. Br J Cancer 2008; 99: 1210-1215
• 68 Bai H, Yang J, Cao D. et al. Ovary and uterus-sparing procedures for low-grade endometrial stromal sarcoma: a retrospective study of 153 cases. Gynecol Oncol 2014; 132: 654-660
• 69 Shah JP, Bryant CS, Kumar S. et al. Lymphadenectomy and ovarian preservation in low-grade endometrial stromal sarcoma. Obstet Gynecol 2008; 112: 1102-1108
• 70 Si M, Jia L, Song K. et al. Role of Lymphadenectomy for Uterine Sarcoma: A Meta-Analysis. Int J Gynecol Cancer 2017; 27: 109-116
• 71 Cui R, Cao G, Bai H. et al. The clinical benefits of hormonal treatment for LG-ESS: a meta-analysis. Arch Gynecol Obstet 2019; 300: 1167-1175
• 72 Gadducci A, Cosio S, Romanini A. et al. The management of patients with uterine sarcoma: a debated clinical challenge. Crit Rev Oncol Hematol 2008; 65: 129-142
• 73 Yamaguchi M, Erdenebaatar C, Saito F. et al. Long-Term Outcome of Aromatase Inhibitor Therapy With Letrozole in Patients With Advanced Low-Grade Endometrial Stromal Sarcoma. Int J Gynecol Cancer 2015; 25: 1645-1651
• 74 Amant F, De Knijf A, Van Calster B. et al. Clinical study investigating the role of lymphadenectomy, surgical castration and adjuvant hormonal treatment in endometrial stromal sarcoma. Br J Cancer 2007; 97: 1194-1199
• 75 Sampath S, Schultheiss TE, Ryu JK. et al. The role of adjuvant radiation in uterine sarcomas. Int J Radiat Oncol Biol Phys 2010; 76: 728-734
• 76 Piver MS, Rutledge FN, Copeland L. et al. Uterine endolymphatic stromal myosis: a collaborative study. Obstet Gynecol 1984; 64: 173-178
• 77 Nam JH. Surgical treatment of uterine sarcoma. Best Pract Res Clin Obstet Gynaecol 2011; 25: 751-760
• 78 Cheng X, Yang G, Schmeler KM. et al. Recurrence patterns and prognosis of endometrial stromal sarcoma and the potential of tyrosine kinase-inhibiting therapy. Gynecol Oncol 2011; 121: 323-327
• 79 Dahhan T, Fons G, Buist MR. et al. The efficacy of hormonal treatment for residual or recurrent low-grade endometrial stromal sarcoma. A retrospective study. Eur J Obstet Gynecol Reprod Biol 2009; 144: 80-84
• 80 Maluf FC, Sabbatini P, Schwartz L. et al. Endometrial stromal sarcoma: objective response to letrozole. Gynecol Oncol 2001; 82: 384-388
• 81 Pink D, Lindner T, Mrozek A. et al. Harm or benefit of hormonal treatment in metastatic low-grade endometrial stromal sarcoma: single center experience with 10 cases and review of the literature. Gynecol Oncol 2006; 101: 464-469
• 82 Thanopoulou E, Aleksic A, Thway K. et al. Hormonal treatments in metastatic endometrial stromal sarcomas: the 10-year experience of the sarcoma unit of Royal Marsden Hospital. Clin Sarcoma Res 2015; 5: 8
• 83 Weitmann HD, Knocke TH, Kucera H. et al. Radiation therapy in the treatment of endometrial stromal sarcoma. Int J Radiat Oncol Biol Phys 2001; 49: 739-748
• 84 Kortmann B, Reimer T, Gerber B. et al. Concurrent radiochemotherapy of locally recurrent or advanced sarcomas of the uterus. Strahlenther Onkol 2006; 182: 318-324
• 85 Benson C, Miah AB. Uterine sarcoma – current perspectives. Int J Womens Health 2017; 9: 597-606
• 86 Gremel G, Liew M, Hamzei F. et al. A prognosis based classification of undifferentiated uterine sarcomas: identification of mitotic index, hormone receptors and YWHAE-FAM22 translocation status as predictors of survival. Int J Cancer 2015; 136: 1608-1618
• 87 Hardell E, Josefson S, Ghaderi M. et al. Validation of a Mitotic Index Cutoff as a Prognostic Marker in Undifferentiated Uterine Sarcomas. Am J Surg Pathol 2017; 41: 1231-1237
• 88 Malouf GG, Lhomme C, Duvillard P. et al. Prognostic factors and outcome of undifferentiated endometrial sarcoma treated by multimodal therapy. Int J Gynaecol Obstet 2013; 122: 57-61
• 89 Tanner EJ, Garg K, Leitao jr. MM. et al. High grade undifferentiated uterine sarcoma: surgery, treatment, and survival outcomes. Gynecol Oncol 2012; 127: 27-31
• 90 Meurer M, Floquet A, Ray-Coquard I. et al. Localized high grade endometrial stromal sarcoma and localized undifferentiated uterine sarcoma: a retrospective series of the French Sarcoma Group. Int J Gynecol Cancer 2019; 29: 691-698
• 91 Schick U, Bolukbasi Y, Thariat J. et al. Outcome and prognostic factors in endometrial stromal tumors: a Rare Cancer Network study. Int J Radiat Oncol Biol Phys 2012; 82: e757-e763
• 92 Baniak N, Adams S, Lee CH. et al. Extrapelvic Metastases in Endometrial Stromal Sarcomas: A Clinicopathological Review With Immunohistochemical and Molecular Characterization. Int J Surg Pathol 2018;
• 93 Fleming NA, Hopkins L, de Nanassy J. et al. Mullerian adenosarcoma of the cervix in a 10-year-old girl: case report and review of the literature. J Pediatr Adolesc Gynecol 2009; 22: e45-e51
• 94 Zhang Y, Li Y, Huang H. et al. Low-Grade Endometrial Stromal Sarcoma and Uterine Adenosarcoma: A Comparison of Clinical Manifestations and Outcomes. J Cancer 2019; 10: 3352-3360
• 95 Tate K, Watanabe R, Yoshida H. et al. Uterine adenosarcoma in Japan: Clinicopathologic features, diagnosis and management. Asia Pac J Clin Oncol 2018; 14: 318-325
• 96 McCluggage WG. Mullerian adenosarcoma of the female genital tract. Adv Anat Pathol 2010; 17: 122-129
• 97 Wells M, Oliva E, Palacios J, Prat J. WHO Classification of Tumours of female reproductive Tract – mixed epithelial and mesenchymal Tumors of the Uterus. Lyon: IARC Press; 2014
• 98 McCluggage WG. A practical approach to the diagnosis of mixed epithelial and mesenchymal tumours of the uterus. Mod Pathol 2016; 29 (Suppl. 01) S78-S91
• 99 Parra-Herran C, Howitt BE. Uterine Mesenchymal Tumors: Update on Classification, Staging, and Molecular Features. Surg Pathol Clin 2019; 12: 363-396
• 100 Seagle BL, Kanis M, Strohl AE. et al. Survival of women with Mullerian adenosarcoma: A National Cancer Data Base study. Gynecol Oncol 2016; 143: 636-641
• 101 Nathenson MJ, Conley AP, Lin H. et al. Treatment of Recurrent or Metastatic Uterine Adenosarcoma. Sarcoma 2017; 2017: 4680273
• 102 Tanner EJ, Toussaint T, Leitao jr. MM. et al. Management of uterine adenosarcomas with and without sarcomatous overgrowth. Gynecol Oncol 2013; 129: 140-144
• 103 Minard-Colin V, Walterhouse D, Bisogno G. et al. International Society of Pediatric Oncology Sarcoma Committee, the Childrenʼs Oncology Group, the Italian Cooperative Soft Tissue Sarcoma Group, and the European pediatric Soft tissue sarcoma Study Group. Localized vaginal/uterine rhabdomyosarcoma-results of a pooled analysis from four international cooperative groups. Pediatr Blood Cancer 2018; 65: e27096
• 104 Kirsch CH, Goodman M, Easiashvili N. Outcome of female pediatric patients diagnosed with genital tract rhabdomyosarcoma based on analysis of cases registered in SEER database between 1973 and 2006. Am J Clin Oncol 2014; 37: 47-50
• 105 [Anonym] Cooperative Weichteilsarkom Study Group CWS der GPOH in cooperation with the European pediatric Soft Tissue Sarcoma Study Group EpSSG: CWS-guidance. 24.05.2014. Accessed August 26, 2022 at: https://www.gpoh.de/kinderkrebsinfo/content/fachinformationen/studienportal/pohkinderkrebsinfotherapiestudien/cws_guidance/index_ger.html
• 106 Martelli H, Oberlin O, Rey A. et al. Conservative treatment for girls with nonmetastatic rhabdomyosarcoma of the genital tract: A report from the Study Committee of the International Society of Pediatric Oncology. J Clin Oncol 1999; 17: 2117-2122
• 107 Beckmann MW, Juhasz-Boss I, Denschlag D. et al. Surgical Methods for the Treatment of Uterine Fibroids – Risk of Uterine Sarcoma and Problems of Morcellation: Position Paper of the DGGG. Geburtshilfe Frauenheilkd 2015; 75: 148-164
• 108 Brohl AS, Li L, Andikyan V. et al. Age-stratified risk of unexpected uterine sarcoma following surgery for presumed benign leiomyoma. Oncologist 2015; 20: 433-439
• 109 Kundu S, Zachen M, Hertel H. et al. Sarcoma Risk in Uterine Surgery in a Tertiary University Hospital in Germany. Int J Gynecol Cancer 2017; 27: 961-966
• 110 Bojahr B, De Wilde RL, Tchartchian G. Malignancy rate of 10,731 uteri morcellated during laparoscopic supracervical hysterectomy (LASH). Arch Gynecol Obstet 2015; 292: 665-672
• 111 Cao H, Li L, Yang B. et al. Unexpected uterine sarcomas after hysterectomy and myomectomy for presumed leiomyoma: a retrospective study of 26,643 patients. Cancer Manag Res 2019; 11: 7007-7014
• 112 [Anonym] Statement of the Society of Gynecologic Oncology to the Food and Drug Administrationʼs Obstetrics and Gynecology Medical Devices Advisory Committee Concerning Safety of Laparoscopic Power Morcellation. 2014. Accessed August 26, 2022 at: https://www.sgo.org/resources/statement-of-the-society-of-gynecologic-oncology-to-the-food-and-drug-administrations-obstetrics-and-gynecology-medical-devices-advisory-committee-concerning-safety-of-laparoscopic-power-morc/
• 113 [Anonym] AAGL Advancing Minimally Invasive Gynecology Worldwide. Morcellation During Uterine Tissue Extraction. 2014. Accessed August 26, 2022 at: https://www.aagl.org/wp-content/uploads/2014/05/Tissue_Extraction_TFR.pdf
• 114 US Food and Drug Administration. FDA Updated Assessment of The Use of Laparoscopic Power Morcellators to Treat Uterine Fibroids. 2017. Accessed April 04, 2017 at: https://wayback.archive-it.org/7993/20170404182209/https:/www.fda.gov/MedicalDevices/Safety/AlertsandNotices/ucm424443.htm
• 115 Singh SS, Scott S, Bougie O. et al. SOGC CLINICAL PRACTICE–GYNAECOLOGY COMMITTEE; GOC EXECUTIVE COMMITTEE; SPECIAL CONTRIBUTOR. RETIRED: Technical update on tissue morcellation during gynaecologic surgery: its uses, complications, and risks of unsuspected malignancy. J Obstet Gynaecol Can 2015; 37: 68-78
• 116 Bogani G, Cliby WA, Aletti GD. Impact of morcellation on survival outcomes of patients with unexpected uterine leiomyosarcoma: a systematic review and meta-analysis. Gynecol Oncol 2015; 137: 167-172
• 117 George S, Barysauskas C, Serrano C. et al. Retrospective cohort study evaluating the impact of intraperitoneal morcellation on outcomes of localized uterine leiomyosarcoma. Cancer 2014; 120: 3154-3158
• 118 Raspagliesi F, Maltese G, Bogani G. et al. Morcellation worsens survival outcomes in patients with undiagnosed uterine leiomyosarcomas: A retrospective MITO group study. Gynecol Oncol 2017; 144: 90-95
• 119 Raine-Bennett T, Tucker LY, Zaritsky E. et al. Occult Uterine Sarcoma and Leiomyosarcoma: Incidence of and Survival Associated With Morcellation. Obstet Gynecol 2016; 127: 29-39
• 120 Lin KH, Torng PL, Tsai KH. et al. Clinical outcome affected by tumor morcellation in unexpected early uterine leiomyosarcoma. Taiwan J Obstet Gynecol 2015; 54: 172-177
• 121 Nemec W, Inwald EC, Buchholz S. et al. Effects of morcellation on long-term outcomes in patients with uterine leiomyosarcoma. Arch Gynecol Obstet 2016; 294: 825-831
• 122 Lee JY, Kim HS, Nam EJ. et al. Outcomes of uterine sarcoma found incidentally after uterus-preserving surgery for presumed benign disease. BMC Cancer 2016; 16: 675
• 123 Gao Z, Li L, Meng Y. Correction: A Retrospective Analysis of the Impact of Myomectomy on Survival in Uterine Sarcoma. PloS One 2016; 11: e0153996
• 124 Gao Z, Li L, Meng Y. A Retrospective Analysis of the Impact of Myomectomy on Survival in Uterine Sarcoma. PloS One 2016; 11: e0148050
• 125 Wasson M, Magtibay 2nd P, Magtibay 3rd P. et al. Incidence of Occult Uterine Malignancy Following Vaginal Hysterectomy With Morcellation. J Minim Invasive Gynecol 2017; 24: 665-669
• 126 Zhang J, Li T, Zhang J. et al. Clinical Characteristics and Prognosis of Unexpected Uterine Sarcoma After Hysterectomy for Presumed Myoma With and Without Transvaginal Scalpel Morcellation. Int J Gynecol Cancer 2016; 26: 456-463
• 127 Ebner F, Friedl TW, Scholz C. et al. Is open surgery the solution to avoid morcellation of uterine sarcomas? A systematic literature review on the effect of tumor morcellation and surgical techniques. Arch Gynecol Obstet 2015; 292: 499-506
• 128 Zhang G, Yu X, Zhu L. et al. Preoperative clinical characteristics scoring system for differentiating uterine leiomyosarcoma from fibroid. BMC Cancer 2020; 20: 514
• 129 Nagai T, Takai Y, Akahori T. et al. Highly improved accuracy of the revised PREoperative sarcoma score (rPRESS) in the decision of performing surgery for patients presenting with a uterine mass. SpringerPlus 2015; 4: 520
• 130 Wickerham DL, Fisher B, Wolmark N. et al. Association of tamoxifen and uterine sarcoma. J Clin Oncol 2002; 20: 2758-2760
• 131 Schmeler KM, Lynch HT, Chen LM. et al. Prophylactic surgery to reduce the risk of gynecologic cancers in the Lynch syndrome. N Engl J Med 2006; 354: 261-269
• 132 Leal MA, Pinera A, De Santiago J. et al. Novel Technique for Contained Power Morcellation through Umbilicus with Insufflated Bag. Gynecol Obstet Invest 2017; 82: 205-207
• 133 McKenna JB, Kanade T, Choi S. et al. The Sydney Contained In Bag Morcellation technique. J Minim Invasive Gynecol 2014; 21: 984-985
• 134 Frasca C, Degli Esposti E, Arena A. et al. Can In-Bag Manual Morcellation Represent an Alternative to Uncontained Power Morcellation in Laparoscopic Myomectomy? A Randomized Controlled Trial. Gynecol Obstet Invest 2018; 83: 52-56
• 135 Venturella R, Rocca ML, Lico D. et al. In-bag manual versus uncontained power morcellation for laparoscopic myomectomy: randomized controlled trial. Fertil Steril 2016; 105: 1369-1376
• 136 Zullo F, Venturella R, Raffone A. et al. In-bag manual versus uncontained power morcellation for laparoscopic myomectomy. Cochrane Database Syst Rev 2020; (05) CD013352