Review Article

Pediatric Sclerosing Rhabdomyosarcomas: A Review

Table 1

Showing clinicopathological features of 21 cases of pediatric sclerosing rhabdomyosarcomas reported in the literature (including present case).

Case numberAuthorYearAge/
sex
SiteSize (cm)Mets (1) Primary RxAdj.
Rx
Local recurrence Mets (2)TTR/TTM
(mos)
Rx of Rec/Mets F/U
(mos)
Outcome

1Folpe et al. [8]200218/M Orbit NA (−) Excision (−) (−) Intra-abdominal and lymph nodes 48 CT 60 Died

2Vadgama et al. [9]20043/F Ischiorectal fossa/buttock 3.2 (−) Excision CT (−) (−) (−) (−) 4 AWD

3Chiles et al. [7] 20046/M Arm NA NA NA NA (+) (−) 20 NA 39Alive with disease
411/M Elbow NA (−) NA NA (−) (+) 20 NA 37 Dead
57/F Pharynx NA (−) NA NA (+) (−) 4 NA 16Alive with disease
610/F Orbit NA (−) NA NA (−) (−) (−) (−) 18 AWD
73/M Leg NA (−) NA NA (−) (−) (−) (−) 10 AWD
88/F Sinus NA (−) NA NA (−) (−) (−) (−) <1 AWD
916/F Thigh NA (−) NA NA (−) (−) (−) (−) 25 AWD
104/F Scapula NA (−) NA NA (−) (−) (−) (−) NA NA
1112/M Masseter NA (−) NA NA (−) (−) (−) (−) <1 AWD
129/M Orbit NA (−) NA NA (−) (−) (−) (−) 43 AWD
134/M Neck NA (−) NA NA (−) (−) (−) (−) 37 AWD
140.3/MRetroperitoneum NA (−) NA NA (−) (−) (−) (−) NA NA
155/M Scrotum NA (−) NA NA (−) (−) (−) (−) 24 AWD

16 Zambrano et al. [11] 20068/F Intra-abdominal10.5 (−) CT f/b Excision (−) NA NA NA NA NA NA
1718/M Thigh NA NA NA NA NA NA NA NA NA NA
1817/M Lower leg 9.0 NA CT f/b Excision NA NA NA NA NA NA NA

19Wang et al. [10] 200812/F Nasal cavity 4.0 (−) Excision (−) (+) (−) 3Excision + CT 5 AWD

20Bouron-Dal Soglio et al. [6]20097/M Deltoid 3.7 (−) Excision CT (+)Lung, Lymph nodes 24Excision + SCT 72 Alive with Disease

21Present case201311/M Skull base 9.3 (−) ExcisionCT + RT (+) Spinal leptomeningeal 14 RT + CT 16 Alive with Disease

AWD: alive without disease; CT: chemotherapy; RT: radiotherapy; NA: not available; Mets (1): metastases at presentation; Mets (2): metastases during follow-up; Adj.: adjuvant; Rx: treatment; f/b: followed by; SCT: stem cell transplantation; mos: months; Rec: recurrence; TTR: time to recurrence; TTM: time to metastases.