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Table 1 Clinical features of 41 patients with extra-genital mullerian adenosarcoma reported in the English literature and of the index case

From: Primary extra-uterine and extra-ovarian mullerian adenosarcoma: case report and literature review

Reference number Author Age (years) Site Size (cm) Tumour markers Signs and Symptoms at presentation Treatment Sarcomatous overgrowth Endometriosis Hormonal therapy Follow-up
4 Douglas 18 gravida Retroperitoneum 5 × 4.5 × 4 NR Anorexia, suprapubic-low back pain, loss of weight, vaginal bleeding, preterm delivery. 24 weeks CHT (MTX) NR No No DOD 10 weeks later with distant metastasis
5a Clement 45 Right Pelvic peritoneum (pelvic mass that extended into the rectum and the bladder) 7x7x5 NR Right lower leg thrombophlebitis, right paravaginal mass Surgery (partial tumour resection) + RT NR No No DOD 9 months later due to pelvic recurrence and visceral metastasis
5b Clement 73 Midline Pelvic peritoneum, displcing the bladder anteriorly 14 NR Large pelvic mass with bilateral hydronephrosis. Inability to void Surgery (complete tumour resection) NR No No DOD 2 months later (postoperatively massive gastric bleeding necessitating a subtotal gastrectomy occurred but after that the patient’s conditions deteriorated gradually-autopsy not done-)
5c Clement 58 Left Pelvic peritoneum 16x15x8 NR Large pelvic mass. Urinary urgency, rectal pressure Surgery (partial tumour resection) NR Yes No AWD local recurrence 15 months later (RT), lung metastases 45 months later (resected)
6 Bard 46 Right pelvic peritonum (the mass was adherent to the right bladder wall and surrounded the right ureter) 10 × 8 NR Weakness and pelvic pain in the right lower extremity. Urinary incontinency Biopsy + RT NR NR No DOD 11 weeks later with distant sepsis and metastasis
7 Kao 42 Left round ligament 10 NR NR Surgery (partial tumour resection) + CHT (Cyt) + RT NR No No DOD after 10 months due to the tumor
8 Russell 29 Left Broad ligament 7x6x5 N.R. Lower abdominal pain for 2 months and occasional dyspareunia Surgery (tumour resection) NR No No Recurrence after 5 months treated with surgery (hysterectomy, bilateral salpingo-oophorectomy) + RT; Died of melanoma after 9 years.
9 Kerner 32 gravida Broad ligament 15x10x6 and 10x7x4 NR Abdominal pain at 28 weeks Surgery (tumour resection) No No No AWD omentum and infundibulopelvic ligament recurrence 22 months later
10 Vara 62 Bladder NR Haematuria, weight loss, suprapubic pain Surgery (radical cystectomy + urethrectomy) NR Yes No FOD 12 months
11 Roman 55 Retroperitoneal NR NR NR Surgery (tumour resection) + RT NR Yes No First abdominal recurrence 3 years later (resection by thoracoabdominal approach + MPA), a 5 cm perihepatic recurrence 5 years later was completely resected, second perihepatic recurrence 7 years from original tumour (resection of recurrence and TMX), 6 months later intrahepatic metastasis (CHT for recurrences (cisplatin-ifosfamide, ifosfamide, doxorubicin), later atrial tumour (resection of cardiac tumour and oral therapy with etoposide). Died after 10 years from original tumour and 70 days after resection of cardiac tumour.
12 De Jonge 16 Pelvic peritoneum and infracolic omentum NR Ca 125 > 190 U/ml Severe Abdominal distension and pain Surgery (tumour resection with extirpation of pelvic mass, left fallopian tube, infracolic omentum and appendix) Yes No NR Three weeks after primary surgery a pelvic mass recurred (CHT with doxorubicin and ifosfamide), first recurrence of the pouch of Douglas 8 months later (CHT cisplatinum, etoposide and ifosfamide), second recurrence of the pouch of Douglas 14 months later the first recurrence (bilateral salpingo-oophorectomy, abdominal hysterectomy, pelvic and para-aortic lymphadenectomy and hormonal therapy) FOD 57 months after the last cycle of chemotherapy
13 Benda 65 Vaginal apex 10x7x8 NR Pelvic pressure and urinary frequency Surgery (tumour resection) NR No No Three years later a 6 cm vaginal recurrence was completely resected; 5 yrs. after first recurrence a 12 cm vaginal recurrence was completely resected and a progesterone therapy was delivered; 7 years after the second recurrence a 17 cm pelvic recurrence was partially resected and a TMX (2 weeks) therapy followed by P (2 weeks) therapy was delivered; 10 months after the third recurrence a fourth pelvic recurrence was partially resected and treated with RT. AWD 16 years
14 Ostor 49 Pouch of Douglas 19x8x3 NR Right Iliac fossa pain Surgery (tumour resection and abdominal hysterectomy and bilateral salpingo-oophorectomy) Radiotherapy and Hormonal therapy (Medroxyprogesterone) NR No No AWD recurrence 5 weeks later (chemotherapy cisplatin and ifosfamide). Persistence of some nodularity on the pelvic floor 18 months later
15 Inoue 54 Left paracolpium 15x11x10 Ca 125: 860 U/ml Brownish vaginal discharge Small ulcer in the left posterior fornix RT+ Surgery (tumour resection+ total abdominal hysterectomy, bilateral adnexectomy, abdominal perineal resection with colostomy) NR Yes No FOD 1 year later
16 Judson 42 Vaginal cuff 6 × 3 NR Endometriosis recurred three times and was treated with surgery, hormonal therapy (megestrol, danazol) and brachytherapy. Lesion coming out from vagina CHT (paclitaxel and carboplatin followed by TMX) NR Yes No 12 months later a 4 cm vagina recurrence was excised and a RT was delivered than was FOD
17 N’Senda 54 Liver 20 × 12 CA 15–3 and CA 19–9, were three and fourfold normal level respectively Right-sided epigastric pain Surgery (tumour resection: A segment -IV enlarged right hepatectomy extended to adjacent diaphragm) No Yes HRT FOD 24 months
18 Kato 20 Abdominopelvic peritoneum 23x23x14 CA 125: 1000 IU/ml Fatigue and constipation Surgery (tumour resection) NR No No FOD 1 years later
19a Yantiss 36 Sigmoid 10,5 NR Hypermenorrhoea, 6 months abdominal pain Surgery (tumour and sigmoid resection) NR Yes NR FOD 36 months
19b Yantiss 2000 50 Colon NR NR NR Surgery (tumour and colon resection) NR Yes NR FOD 24 months
19c Yantiss 2000 83 Small bowel 15 NR Abdominal mass obstruction Surgery (tumour and bowel resection) NR Yes NR NR
19d Yantiss 2000 43 Small bowel 6.5 NR NR Surgery (incomplete tumour resection) NR NR ER T NR
20 Visvalingam 50 Abdominopelvic peritoneum 13 kg NV Painless abdominal swelling Surgery (tumour resection), Hormonal therapy (progesterone) NR No No Ten months later a 50 cm pelvic recurrence was resected (tumour debulking, extrafascial hysterectomy, omentectomy, appendicectomy). DOD 16 months later (Autopsy revealed tumor nodules throughout the abdominal and pelvic cavity limited to the peritoneal surface)
21 Anderon 46 Vagina 10 cm NR   Removal of the vaginal mass, stalk and paravaginal tissue. No Yes Yes FOD after a parametrium recurrence that was treated with external radiotherapy and interstitial brachytherapy.
22 Dincer 50 Perisplenic Peritoneum NR NR Large bowel obstruction in a woman with Endometriosis treated with aromatase inhibitor Surgery (partial tumour resection) + Chemotherapy (anthracycline) + experimental anti-angiogenesis agent Yes Yes No DOD 13 months later due to no regression of the pelvic tumour
23 Hines 43 Peritoneum (from posterior cul-de-sac through the middle to upper abdomen) NR Ca 125: 824 IU/ml Dysmenorrhea and endometriosis Surgery (tumour resection) + Hormonal therapy (medroxyprogesterone acetate) No Yes No FOD 10 months later
24 Murugasu 23 Pouch of Douglas 11 Ca 125: 378 IU/ml; CEA: 13. Right-sided pelvic pain Surgery (tumour resection), Chemotherapy (Mesna, adriamycin, ifosfamide), Radiotherapy Yes Yes No FOD 1 years later
25 Liu 56 Vaginal Vault 16 NR Urinary incontinence and prolapse in a woman with Vaginal endometriosis (TAH, BSO). Surgery (tumour resection with adherent structures including rectum and part of the bladder wall) + Chemotherapy (ifosfamide and cisplatin) + Radiotherapy No Yes ERT FOD
26 Raffaelli 50 Rectovaginal septum Not reported NR Deep dyspareunia, rectal pain, periovulatory pelvic pain in a woman with diagnosis of endometriosis Surgery (Hysterectomy, left salpingo oophorectomy and partial vaginectomy) + Hormonal therapy (megestrol acetate) No Yes No Pelvic recurrence 14 months after first surgery (resection of the mass, chemotherapy with ifosfamide and epirubicin-stopped due to intolerance- and radiotherapy 52,90Gy-stopped due to toxic side effect) FOD 9 months after the last surgery
27 Toyoshima 52 Vaginal cuff 11 cm High level of Ca125   Neoadjuvant therapy and surgical removal of the tumour, the vaginal wall and the greater omentum Yes Yes No After a month the first lung recurrence treated with chemotherapy, and then a second abdominal recurrence treated with chemotherapy. DOD after 9 months from surgery
28 Kanngurn 48 Pelvic peritoneum 26x26x10 cm NR Right lower quadrant pain Surgery (tumour resection, Hysterectomy, bilateral salpingo oophorectomy) + Chemotherapy (Bleomycin, Etoposide and Cisplatinum × 7 cycles). Yes No No Abdominal recurrence 8 months later during the fourth cycle of therapy (13, 5 × 7, 8 × 13 cm), lost at follow-up.
29 Chang 37 cul-de-sac 3.5 NR Vaginal bleeding in a woman with endometriosis (TAH, BSO and hormonal therapy) Surgery (resection of cul-de-sac tumour, left anterior proctectomy, coloanal anastomosis) No yes yes FOD 36 months
30 Milam 47 Right inguinal channel 12 × 4 CA 125: 76,8 Persistent and enlarged groin mass Surgery (tumour resection) No Yes HRT FOD 12 months later
31 Huang 41 Mesentery of the terminal ileum, right colon and pelvic sidewall 10 NV Right lower quadrant pain and nausea Surgery (TAH, bilateral salpingo oophorectomy, omentectomy, resection of cul-de-sac and sigmoid colon nodules, and pelvic and para-aortic lymph node dissection) + Chemotherapy (ifosfamide+ cisplatin) No Yes No Peritoneal recurrence at 1 month and chemotherapy (liposomal doxorubicin) FOD for 18 months
32 Han 34 Vagina 7 × 6 High value   Tumour resection, hysterectomy and bilateral salpingo-oophorectomy No Yes No The patient had 4 vaginal recurrences. The first was treated with chemotherapy, the second was treated with surgery, the third with surgery and adjuvant therapy and the fourth with chemotherapy. FOD
33 Maeda 47 Left pelvic side wall 3 NR (LDH level 993 IU/ml) Acute lower abdominal pain due to pedunculated subserosal myoma Surgery (tumour resection, bilateral salpingo-oophorectomy, total abdominal hysterectomy) Yes Yes TMX Four weeks later 8 cm recurrent tumour in the right pelvis treated with salvage surgery. One month after second surgery recurrent tumour in pelvis and upper abdomen that was treated with salvage chemotherapy (liposomal doxorubicin), 5 cycles). FOD three months after chemotherapy.
34 Patrelli 49 Pouch of Douglas 10 × 6 CA 125 and Ca 19–9 slightly elevated Pelvic pain Surgery (tumour resection) Yes No No Eighteen months later recurrence of posterior vaginal fornix (resected), 6 months after recurrence another posterior vaginal fornix recurrence (radical hysterectomy with bilateral salpingo-oophorectomy, and pelvic lymphadenectomy and Radiotherapy-50Gy-) FOD
35 Clarke 50 Pelvic peritoneum (partially adherent to the posterior uterine serosa) 34x14x7 NR NR Surgery (omentectomy, appendicectomy, removal of small bowel mesenteric implants) NR NR NR  
36 Karateke 26 Pouch of Douglas 18 NR Lower abdominal distention and left lower quadrant pain Surgery (tumour resection) No Yes No FOD 24 months later
37 Yang 36 Rectum 2,5 × 2 NR Loose stool, dysmenorrhea, deep dyspareunia, haematochezia Surgery (tumour resection) No Yes No FOD 60 months
38 Kar 30 Omentum NR NR Abdominal distension due to abdominal mass and free fluid Surgery (hysterectomy, bilateral salpingo oophorectomy, omentectomy) and preoperative chemotherapy Yes Yes No NR
39 Pontrelli 58 Vagina 5 cm NR Bleeding vaginal lesion Vaginectomy and parametrectomy using the laparoscopic approach, total colpectomy and partial cystectomy. After that the patient was candidate for progestin therapy. Yes Yes Yes FOD at November 2016
40 Mandato 79 Abdominal Peritoneum 16 × 11 NV Abdominal distension Surgery (tumour resection, bilateral salpingo-oophorectomy, total hysterectomy) Residual absent Yes No No 4 weeks later had a pelvic recurrence; DOD 4 months after diagnosis
  1. Abbreviations: AWD alive with disease, DOD died of disease, NER no evidence of recurrence, UK unknown, NR not reported, NV normal value, FOD free of disease, CHT chemotherapy, MTX methotrexate, RT radiotherapy, Cyt cyclophosphamide, MPA medroxyprogesterone acetate