Table I.
Age at initiation of propranolol | Diagnosis | Location | Approximate size | KMP at initiation of propranolol | Previously attempted treatments | Highest dose of propranolol | Duration of propranolol treatment | Concurrent treatments with propranolol | Response to propranolol | |
---|---|---|---|---|---|---|---|---|---|---|
Patient 1 | 5 months | KHE with KMP | Right scalp, right face, and right neck | N/A | Unknown | None | 1 mg/kg/day | 4 weeks | None | No response. Propranolol discontinued when KMP was diagnosed. |
Patient 2 | 12 months | KHE with KMP (no biopsy) | Right inferior thigh, right knee, and right superior tibia | N/A | Present | Prednisolone for 10 months (good response until tapered off) | 2 mg/kg/day | 25 months | Prednisolone (variable doses up to 2 mg/kg/day) for 9 of the months | Complete response† to combination then alone. Off all therapy for 9 months without relapse. |
Patient 3 | 4 years | KHE with KMP | Left scalp, left face, left neck, and bilateral chest | 8 × 11 cm | Present | Multiple courses of vincristine ± cyclophosphamide and actinomycin D for 3.5 years. Prednisolone for 2 years. Interferon-α2b for 2 months. Radiation therapy (1000 centiGray total) for 5 days. Thalidomide, celecoxib, etoposide, and cyclophosphamide regimen for 2 months. |
1.3 mg/kg/day | 8 weeks | Prednisolone up to 2 mg/kg/day for entire course Vincristine 0.05 mg/kg/week for first 2 weeks |
No response. Partial response subsequently to repeated vincristine 0.05 mg/kg/week for 4 weeks. |
Patient 4 | 13 months | KHE with KMP | Retroperitonium | 5 × 8 cm | Present | Vincristine for 6 months. IV methylprednisolone and prednisolone for 10 months (good response but recurrent milder KMP when tapered to low doses) |
2 mg/kg/day | 3.5 months | Prednisolone <1.5 mg/kg/day for first 6 weeks. Vincristine 0.025-0.05 mg/kg q1-2 weeks for entire course. |
No response. KHE slightly larger, but no KMP recurrence. Partial response subsequently to sirolimus 0.8 mg/m2/dose twice daily with smaller tumor. Off all therapy for 7 months without worsening, |
Patient 5 | 21 months | TA | Right thigh | 6 × 8 cm | No | None | 2 mg/kg/day | 4 weeks | Aspirin 5 mg/kg/day | No response. Relapsed within 2 days when aspirin discontinued. Aspirin restarted and propranolol discontinued with partial response to aspirin alone. |
Patient 6 | 8 months | KHE with KMP | Left shoulder, left upper arm, and left elbow | 8 × 12 cm | No | None | 0.3 mg/kg/day | 3 days | None | No response. Discontinued after only 3 days due to KMP. Very good response subsequently to prednisolone 2 mg/kg/day and vincristine 0.05 mg/kg/week. |
Patient 7 | 17 days | KHE with KMP (no biopsy) | Right face and neck | 6 × 6 cm | Present | None | 2 mg/kg/day | 19 days | Prednisolone 2 mg/kg/day for last 8 days | No response. Worsened on treatment. Very good response subsequently to prednisolone 2 mg/kg/day and vincristine 0.05 mg/kg/week. |
Patient 8 | 9 days | KHE with KMP (no biopsy) | Left chest | 9 × 9 cm | Present | None | 2 mg/kg/day | 19 days | None | No response. Worsened on treatment. Very good response subsequently to prednisolone 2 mg/kg/day and vincristine 0.05 mg/kg/week. |
Patient 9 | 2 days | TA with KMP | Right thigh | 5 × 6 cm | Present | None | 3 mg/kg/day | 11 months | None | Complete response.† Treated for 3 months, with relapse after discontinuation. Resolution of TA and KMP with second course of propranolol for 8 months. Off all therapy for 6 months without relapse. |
Patient 10 | 3 years | KHE | Right scalp, right face, and right neck | N/A | No | Prednisolone for 2 months Aminocaproic acid for 13 months |
2 mg/kg/day | 4 months | None | Partial response.‡ KHE stable in size and KMP remained quiescent. |
Patient 11 | 26 months | KHE | Right buttock | 5 × 8 cm | No | None | 2 mg/kg/day | 9 months | None | Partial response.‡ Softening of lesion and symptom improvement. |
Complete response defined as complete resolution of tumor, and in cases with KMP, normalization of hematologic parameters.
Partial response defined as stabilization of tumor growth, partial regression of tumor, improvement of KMP, and/or improvement of symptoms.
IV: intravenous; N/A: not available