Costello Syndrome and Umbilical Ligament Rhabdomyosarcoma in Two Pediatric Patients: Case Reports and Review of the Literature

Table 2

Chemotherapy of patients with Costello syndrome and rhabdomyosarcoma.

PT number	Chemotherapy protocol
1	Initially with VCR, ACD, and CTX. After debulking continued on ChT using IFOS, VCR, and ACD. After the second course, when she developed IFOS induced encephalopathy, she was changed back to pulses of VCR, ACD, and CTX given every 3 weeks for 9 courses [7]
2	Initially IFOS, ACD, and VCR. After 4 courses, CTX was substituted for IFOS because hemorrhagic cystitis. Tx complicated by recurrent episodes of moderately severe diarrhoea and febrile neutropenia requiring readmission to hospital. Tx was completed after six courses of ChT [7]
3	Tx consisted of below the knee amputation and ChT (DOX, ACD, VCR, and CTX) [10]
4	Tx was implemented according to the Italian protocol for pediatric soft tissue sarcoma. Three courses of ChT, including VCR (0.025 mg/kg day 1), IFOS (50 mg/kg days 1 and 2), and alternating ACD (0.025 mg/kg day 1) and DOX (0.7 mg/kg day 1) were administered. Symptoms regressed, and the CT scan showed a reduction in tumor volume of more than two-thirds. After 3 more courses of ChT, the patient experienced abdominal pain. A repeat abdominal CT scan showed an increase in the pelvic mass. Despite the administration of a different ChT with CBDCA (11 mg/kg day 1) and VP-16 (3 mg/kg for 3 doses), the tumor continued to grow, and the child died 7 months after the diagnosis of RMS [12]
5	NDF [13]
6	The first cycle of ChT with VCR, ACD, and CTX and topotecan reduced the tumor size [16]. The tumor was surgically removed after the first ChT [8]. Debulking S was required because of rapid growth and tumor necrosis. The protocol was continued and was deemed sufficiently successful to withhold RT. One month after completion of the first protocol, tumor recurrence necessitated a second round of ChT with irinotecan and DOX. The latter drug was administered about 1 week before he died, during which time he had tachycardia (heart rate 180–20 bpm) [14]
7	The tumor did not respond to ChT and she died shortly after her 4th birthday [8]
8	Tx was a combination of S, ChT, and RT, continued until age of 3.5 years [8]
9-10	NDF [8]
11	PT had later generalized tonic-clonic seizures during her Tx for RMS [17]
12	Despite extensive ChT, the tumor progressed rapidly and the PT died 2 months later [18]
13	Induction ChT was commenced with the initial 12-week course of VCR, ACD, and CTX based on the Intermediate Risk Protocol D9803 of the COG [19]
14	ChT did not result in tumor shrinkage; therefore, surgical resection was performed [20]
15–22	NDF [5, 6, 9, 21–23]
23	ChT according to the cooperative soft tissue sarcoma protocol (CWS-2002 P) was well tolerated by the PT [24]
24	NDF [25]
25	ChT in the standard risk group protocol EpSSG RMS 2005 subgroup D, 3 preoperative cycles of IFOS (2 doses at 100 mg/kg), VCR (0.05 mg/kg), and ACD (0.05 mg/kg)
26	ChT (postoperative 15 days) in the standard risk group protocol EpSSG RMS 2005 subgroup B (Stage I), cycles of IFOS (2 doses of 3 g/m^2/d), VCR (1.5 mg/m^2), and ACD (1.5 mg/m^2), was initiated

ACD, actinomycin D; CBDCA, carboplatin; ChT, chemotherapy; COG, children’s oncology group; CTX, cyclophosphamide; CT, computed tomography; CWS, Cooperative Weichteilsarkom Studie (Cooperative Soft Tissue Sarcoma Study); DOX, Doxorubicin; EpSSG, European pediatric Soft tissue sarcoma Study Group; IFOS, ifosfamide; NDF, no data found; PT, patient; RMS, rhabdomyosarcoma; RT, radiotherapy; S, surgery; Tx, treatment; VCR, vincristine; VP-16, etoposide.