Table I.
Summary of patient cases.
| 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