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Table 1 Clinical features of the 44 reported cases of uterine PEComa***

From: Uterine PEComa: appraisal of a controversial and increasingly reported mesenchymal neoplasm

REFERENCE

Year

Age (yrs)

TSC

Location

Management

Outcome

Hornick & Fletcher (39)

2008

34

NS

Corpus

NS

NERM at 33 months

Armah & Parwani (40)

2008

59

No

Corpus

Hysterectomy/BSO performed 7-years previously for presumed leiomyosarcoma; Renal and pulmonary metastases at current presentation; all resected

NERM at 15 months

Liang et al (41)

2008

59

Yes

Corpus

Hysterectomy/BSO/Pelvic and paraaortic Lymphadenenctomy, omentectomy, appendectomy, peritoneal biopsies; hormonal therapy

Extension into cervix at presentation; NERM at 10 months

Gan et al (20)

2007

40

NS

Corpus

Hysterectomy

NERM at 16 months

  

33

NS

Corpus

Hysterectomy

NERM at 32 months

  

44

NS

Corpus

Hysterectomy

NERM at 52 months

Rammeh Rommani et al (21)

2006

35

No

Corpus

Excision

NERM at 4 months

Azad et al (22)

2006

25

No

Cervix

Radical hysterectomy, Lymphadenectomy

Unavailable or Recent

Folpe et al (13)

2005

59

No

Corpus

Adjuvant Chemotherapy

Liver and Lung metastases at 30 months. Alive with metastases 30 months

  

56

No

Corpus

Adjuvant Chemotherapy and radiotherapy

Lung and bone metastases at 11 months; Alive with metastases 11 months

  

36

No

Corpus

Hysterectomy, adjuvant chemotherapy

Lung metastases at 12 months, liver metastases 36 months; Death at 39 months

  

42

No

Corpus

No additional surgery or adjuvant therapy

NERM at 11 months

  

28

No

Cervix

Hysterectomy and Lymphadenectomy

NERM at 36 months

  

48

No

Cervix

Adjuvant Radiotherapy

NERM at 21 months

Jeon et al (23)

2005

9

No

Corpus

Neoadjuvant chemotherapy, Hysterectomy with Lymphadenectomy, Adjuvant Chemotherapy/Radiotherapy

Lymph node metastases at presentation, NERM at 18 months

Bernado Vega et al (27)

2005

34

No

Corpus

Hysterectomy/BSO

Unavailable or Recent

Bosincu et al (24)

2005

59

No

Corpus

Hysterectomy/BSO, omentectomy, abdominal exploration

Pelvic recurrence at 6 months; Death at 12 months

  

48

No

Corpus

GnRH analogues, then Hysterectomy

NERM at 36 months

Fukunaga (25)

2005

40

No

Corpus

Hysterectomy/BSO, omentectomy, Adjuvant chemotherapy and radiation.

Ovarian and Omental metastases at presentation, Death at 16 months

  

30

No

Corpus

Hysterectomy

NERM at 36 months

  

36

No

Corpus

Hysterectomy

NERM at 6 months

Fukunaga (25,26)

2004

32

No

Corpus

Excision

NERM at 12 months

Gao et al (28)

2004

60

No

Corpus

Hysterectomy/BSO, staging

Unavailable or Recent

Darai et al (29)

2004

18

No

Corpus

Excision

Pelvic recurrence and lymph node metastases, 6 months, NERM subsequent 24 months

Fadare et al (5)

2004

41

Yes

Cervix

Hysterectomy/BSO

NERM at 35 months

Dimmler et al (32)

2003

61

NS

Corpus

Hysterectomy

Lung metastases at 84 months

Greene et al (30)

2003

79

NS

Corpus

Hysterectomy/BSO

Pelvic and abdominal recurrence at 24 months; Death at 24+ months

Park et al (31)

2003

32

No

Corpus

Hysterectomy/RSO

Right broad ligament/mesovarian extension at presentation; NERM at 18 months

Vang & Kempson (33)

2002

40

No

Corpus

Excision

Unavailable or Recent

  

54

No

Corpus

Hysterectomy/BSO

Unavailable or Recent

  

56

No

Corpus

Hysterectomy/BSO

Unavailable or Recent

  

75

No

Corpus

Hysterectomy/BSO, Adjuvant Radiotherapy

NERM at 31.2 months

  

47

No

Corpus

Hysterectomy/BSO

NERM at 1.5 months

  

49

Yes

Corpus

Hysterectomy/BSO

NERM at 54 months

  

55

No

Corpus

Hysterectomy/BSO

NERM at 2 months

  

58

No

Corpus

Hysterectomy/BSO

Unavailable or Recent

Bonnetti et al (34)

2001

19

No

Corpus

Hysterectomy/BSO/pelvic and inguinal lymphadenectomy, Adjuvant chemotherapy and radiotherapy

Metastases to pelvic/inguinal nodes and vaginal extension at presentation. Local recurrence, bone and lung metastases 18 months; no further follow-up

  

41

Yes

Corpus

Hysterectomy/BSO

Ovarian metastases at presentation, NERM at 6 months

Michal & Zamecnik* (35)

2000

58

No

Corpus

Hysterectomy

NERM at 48 months

  

48

No

Corpus

Hysterectomy

NERM at 48 months

  

46

No

Corpus

Hysterectomy

NERM at 12 months

  

46

No

Corpus

Hysterectomy

NERM at 12 months

Ruco et al** (38)

1998

56

No

Corpus

NS

Ovary and ileum metastases at presentation

Pea et al (19)

1996

57

No

Corpus

NS

NERM at 24 months

  1. TSC tuberous sclerosis complex; BSO bilateral salpingo-oophorectomy; NERM no evidence of tumor recurrence or metastases. *these authors [35] published a subsequent opinion in which they indicated that their cases are better designated epithelioid smooth muscle neoplasia [36].
  2. **Presumes the same case is being described in references 37 and 38. ***Includes cases published in the English literature before 01/31/08 only, and excludes the case reported in reference 53 due to lack of data.