Table 1

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

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

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].

**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.

Fadare International Seminars in Surgical Oncology 2008 5:7   doi:10.1186/1477-7800-5-7

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