Overview of embryonal rhabdomyosarcoma of cervix in women over 40-year-old

Table 1 Intergroup Rhabdomyosarcoma Study Group pretreatment clinical staging system

Stage	Sites of primary tumor	Tumor size, cm	Regional lymph nodes	Distant metastases
1	Orbit, non-parameningeal head/neck; genito-urinary non-bladder/prostate; biliary tract	Any size	N0 or N1 or Nx	M0
2	All other sites	≤ 5 cm	N0 or Nx	M0
3	All other sites	≤ 5 cm > 5 cm	N1 N0 or N1 or Nx	M0
4	Any site	Any size	N0 or N1 or Nx	M1

N0: Absence of nodal spread; N1: Regional nodes involved; Nx: Unknown nodal status; M0: No metastases; M1: Distant metastases at diagnosis.

Table 2 Intergroup Rhabdomyosarcoma Study Group surgical grouping system

Group I	Localized disease, completely resected (clear margins, negative regional nodes)
IA	Confined to organ or muscle of origin
IB	Infiltration outside organ or muscle of origin
Group II	Microscopic disease remaining (at margins or in regional nodes)
IIA	Grossly resected tumors with microscopic residual tumor
IIB	Regional disease, completely resected, with nodes involved and/or tumor extension into an adjacent organ
IIC	Regional disease, with involved nodes, grossly resected, but with evidence of microscopic residual tumor
Group III	Incomplete resection or biopsy with gross residual disease remaining
IIIA	After biopsy
IIIB	After major surgical resection
Group IV	Distant metastases present at onset

Table 3 Case-series of cervical embryonal rhabdomyosarcomas reporting on > 4 patients, of whom at least one was > 40-year-old

AuthorYearCases	Women ≤40-year-old				Women > 40-year-old
	n	Age,yr	1st treatment(n)	Follow-up(yr)	n	Age,yr	1st treatment(n)	Follow-up(yr)
Montag et al[5] 1986 n = 4	3	18, 21, 26	TAH (3), BSO (2), LND (2); CT (3)	Alive NED at 1.4 yr, 7 yr, and 8 yr	1	42	TAH-BSO-LND; RT + BT	No local recurrence, but lung metastasis at 9 mo, resection and CT; Alive NED after 10 yr
Brand et al[12] 1987 n = 4	3	1.4, 17, 17	TAH (2), BSO (1), LND (1), no surgery (1); CT (2), RT (1)	Died WD at 0.5 yr (case no RT); Alive NED at 1 yr and 8 yr	1	48	TAH-BSO; CT	Vaginal recurrence at 2 mo, pelvic RT + intravaginal brachytherapy; Alive NED after 22 yr
Ferguson et al[13] 2007 n = 8	3	17, 22, 32	Unknown (1), TAH (2), BSO (1), LND (2); CT (2), RT (2)	Died WD at 1.8 yr (case CT + RT); Alive NED at 0.2 yr and 10 yr (RT)	5	46, 51, 52, 56, 58	TAH-BSO-LND (4); TAH-LND (1); CT (2); RT (2); BT (2)	Dead WD (2) 1 yr and 1.4 yr after; Alive NED (3) at 0.6 yr, 2 yr, and 3 yr
Kriseman et al[14] 2012 n = 11	9	3, 12, 13, 17, 18, 18, 27, 33, 34	No surgery (2), cone biopsy (6), polypectomy (1), TAH-BSO (1), CT (8), RT (1)	No FU (4), Died at date not reported (1) Alive NED (5) at 0.3 yr, 0.5 yr, 2 yr, 3 yr, and 10 yr	2	49	TAH-BSO; CT; RT	No recurrence, died after 6 yr from the second primary tumor, adenocarcinoma of parotid;
						52	Cone biopsy; no adjuvant therapy	Alive NED after 1.5 yr
Li et al[15] 2013 n = 20	7	20, 21, 23, 26, 29, 29, 31	Unknown (2), polypectomy (4), TAH (1) CT (3)	No FU (5); Alive NED (3) at 3.5 yr, 6 yr, and 8 yr	13	54	TAH	Alive WD (vaginal recurrence) at 0.4 yr
						46	TAH; CT	Alive NED at 3 yr
						73	Polypectomy	Died at 0.4 yr with lung metastasis and a second primary tumor, ductal breast carcinoma
						43, 44, 46, 47 48, 49, 51, 56, 56, 89	Polypectomy (7); TAH (2); Unknown (1)	No FU data
Summary n = 47	25	Median age 21 yr (range 1-34 yr)	Surgery: TAH 9/21 (42.9%), CT: 18/20 (90.0%), RT: 4/19 (21.1%)	No FU: 8 (32.0%), Alive: 15/17(88.0%), 0 WD, Median FU, 3.5 yr Died: 3/17 (17.6%), at median 1.1 yr	22	Median age 50 yr (range 42-89 yr)	Surgery: TAH 12/14 (85.7%) CT: 6/11 (54.5%) RT: 4/11 (36.4%)	No FU: 10 (45.5%) Alive: 8/12 (66.7%), 3 WD, Median FU 2.6 yr Died: 4 (33.3%), at median 1.2 yr

TAH: Total abdominal hysterectomy; BSO: Bilateral salpingo-oophorectomy; LND: Lymph node dissection; NED: No evidence of disease; WD: With disease; CT: Chemotherapy; RT: External radiotherapy; BT: Brachytherapy; FU: Follow-up.

Table 4 Intergroup Rhabdomyosarcoma Study Group clinical grouping of the 5 case-series of cervical embryonal rhabdomyosarcomas, overwhelming missing data

IRSG clinicalstage	Women ≤40-year-oldn = 25n (%)	Women > 40-year-oldn = 22n (%)
NA	11 (44.0)	13 (59.1)
I	11 (44.0)	9 (40.9)
II	1 (4.0)	0 (0.0)
III	1 (4.0)	0 (0.0)
IV	1 (4.0)	0 (0.0)

IRSG: Intergroup Rhabdomyosarcoma Study Group; NA: Unknown, not available, or could not be inferred from the reports.