Pathological clinical data of the chosen studies
Schmidt et al.6 (1986), Germany | Yasui et al.3 (2015), Japan | Rekhi et al.4 (2018), India | |
---|---|---|---|
Study type | Epidemiological | Epidemiological | Epidemiological |
No. of evaluable patients | 64 | 16 | 300 |
Sex, M/F | 43 (67.2)/21 (33.8) | 10 (62.5)/6 (37.5) | 203 (67.7)/97 (32.3) |
Mean age | 5 yr 11 mo | 23 yr | 14 yr 8 mo |
Classification | 64 (100) Embryonal RMS | 10 (62.5) Spindle cell RMS 5 (31.3) Embryonal RMS 1 (6.3) Alveolar RMS |
140 (46.7) Alveolar RMS 90 (30.0) Embryonal RMS 61 (20.3) Spindle cell RMS 9 (3.0) Pleomorphic RMS |
Primary tumor location | • 21 (32.8) Head and neck • 10 (15.6) Orbit • 9 (14.1) Intrathoracic space • 11 (17.2) Genitourinary region • 7 (10.9) Paratesticular • 3 (4.7) Trunk • 3 (4.7) Extremities |
• 10 (62.5) Head and neck • 4 (25.0) Genitourinary region • 1 (6.3) Extremities • 1 (6.3) Trunk |
• 170 (56.7) Soft tissues, including extremities as the most common location in 59 (19.7) • 126 (42.0) Head and neck • 4 (1.3) Unknown |
Histopathologic | Yes | Yes | Yes |
Immunohistological | No | No | Yes |
Main pathological features | Three subtypes of embryonal RMS (primitive and intermediate). The main difference between the three subgroups was the number of rhabdomyoblasts | Mixture of spindle cell and sclerosing features. Only 63% of cases showed rhabdomyoblasts | The most common subtype was alveolar RMS (46.7%), followed by embryonal RMS (30.0%), spindle cell/sclerosing RMS, and pleomorphic RMS |
Treatment | Surgical resection and chemotherapy | Combination of surgery, VAC chemotherapy, and/or radiotherapy | Surgery, chemotherapy, and radiotherapy (singular or combined) |
Surgery | Tumor resection | Local excision, palatectomy, neck resection, orbit resection | Tumor resection |
Chemotherapy protocol | • VCR, actinomycin D • Adriamycin and cyclophosphamide (stages I, II, III) • Stage IV Ifosfamide replaces cyclophosphamide |
• 11 (68.8) VAC • 4 (25.0) VCR, CDDP and THP-ADR • 1 (6.3) GEM+DOC |
Does not mention |
Radiotherapy dose | Does not mention | Eight patients received radiotherapy, two of whom received heavy-particle radiotherapy | Does not mention |
Follow-up (mo) | 48 | 39 | 27.7 |
Relapse | 6 (9.4) | 6 (37.5) | 20 (6.7) |
Five-year overall survival (%) | 92.18 | 56.25 | 69.30 |
(M: male, F: female, RMS: rhabdomyosarcoma, VAC: vincristine, actinomycin, and cyclophosphamide, VCR: vincristine, CDDP: cisplatin, THPADR: tetrahydropyranyl adriamycin, GEM: gemcitabine, DOC: docetaxel)
Values are presented as number (%).