Table 1.
Summary of the pulmonary epithelioid hemangioendothelioma cases reported in the current literature.
| Author/Reference | No. | Gender M/F | Age at detection | Pulmonary nodules | Therapy | Metastatic spread | Survival years | Clinical presentation |
|---|---|---|---|---|---|---|---|---|
| Dail4 | 20 | 4/16 | 36 | Bilateral | Surgery | Liver (3/20) | 4.6-6.4 | Incidental in many cases |
| Amin12 | 93 | 25/68 | 40.1 | Multiple bilateral or unilateral | Surgery CHT None |
Liver Pleural Lymph node metastases Bone |
5-year survival curves 30-90% | No symptoms 49.5% Dyspnea 18.3% Cough 18.3% Chest pain 16% Hemoptysis 6.5% Weight loss 6.5% Others 9.7% |
| Bagan13 | 80 | 31/49 | 39.7 | Multiple bilateral, unilateral | Surgery Surgery+CHT Radiation None |
Liver Bone Brain Bowel |
5-year survival 60% (47-71%) | Incidental 48.7% Respiratory symptoms 42.5% Hemoptysis 11.2% Weight loss 6.2% Abdominal symptoms 2.5% Anemia |
| Kitaichi14 | 21 | 8/13 | 44 | Bilateral or unilateral | Surgery CHT (mitomycin C, 5FU, cyclophosphamide, vincristine, tegafur) None |
Liver Bone |
0.5-12 years (81%) Partial spontaneous regression (14.3%) | Dyspnea 9.5% Cough 28.6% Sputum 9.5% Chest pain 19% No symptoms 76% |
| Rock15 | 1 | 0/1 | 7 | Bilateral | Not stated | Liver | Not stated | Pleural thickening |
| Einsfelder16 | 11 | 4/7 | 49.8 | Multiple bilateral | Surgery CHT Interferon-2a None |
Liver Pleural Bone Meningeal Peritoneum |
6-105 months | Not stated |
| Schattenberg17 | 3 | 1/2 | 47.3 | Multiple bilateral | Surgery CHT (ifosfamide+ adriamycin) Radiation None | Liver (1/3) | 24 months (suicide) 36 months (alive) 16 months (alive) |
Chronic thoracic pain 3/3 Sickling sensations of the left arm 1/3 |
| Radzikowska18 | 1 | 0/1 | 62 | Multiple bilateral | Interferon-2 | None | 6 months (then lost at observation) | Cough Osteo-articular pain |
| Ye19 | 1 | 0/1 | 55 | Multiple bilateral | None (refused) | Liver | 7 months | Pleuritic pain and irritable cough |
| Sicilian20 | 1 | 0/1 | Young | Bilateral | Not stated | None | Not stated | Not stated |
| Tec21 | 1 | 0/1 | 40 | Radiological abnormalities | Not stated | Not stated | 20 years | Not stated |
| Miettinen22 | 1 | 0/1 | 17 | Bilateral | Surgery | Retroperitoneum | 24 years | Not stated |
| van Kasteren23 | 1 | 1/0 | 10 | Bilateral | Radiation CHT (doxorubicin) | Bone Liver |
21 years | Dyspnea |
| Gaur24 | 1 | 1/0 | 35 | Multiple bilateral | Palliative radiation CHT (bevacizumab + nab-paclitaxel +zolendronic ac.) | Superior and anterior mediastinum Bone | Not stated (stable disease at 6-month-follow up) | Cough Shortness of breath Chest wall, lower back and both legs pain Weight loss (10 kg) |
| Nakatani25 | 1 | 1/0 | 68 | Bilateral | Not stated | Liver | Not stated | Not stated |
| Yanagawa26 | 1 | 0/1 | Not stated | Not stated | Not stated | Skin | Not stated | Not stated |
| Coulibaly27 | 2 | 1/1 | 41 | Multiple bilateral | Not stated | None | 9 months (alive) | Chronic cough |
| 7 months | Worsening dyspnea Expectoration Hemoptysis |
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| Hanada28 | 1 | 1/0 | 45 | Unilateral | None | None | 66 months | No symptoms |
| Cronin29 | 1 | 0/1 | 35 | Bilateral | - | None | 9 months | Shortness of breath and dry cough |
| Sakamoto30 | 2 | 1/1 | 36 | Multiple bilateral | Not stated | Liver (2/2) | Not stated | Not stated |
| Iwashima31 | 1 | 0/1 | 51 | Bilateral multiple nodular shadows | None | None | 24 months (alive) | No symptoms |
| Chen32 | 1 | 0/1 | 58 | Bilateral | None Radiation CHT |
Mediastinum | 20 years | Dyspnea |
| Carretero33 | 1 | 1/0 | 62 | Multiple bilateral Infiltrates | Not stated | None | 3 weeks | Increasing dyspnea |
| Anagnostou34 | 1 | 0/1 | 36 | Multiple bilateral | None | None | 24 months | Progressive dyspnea and fever because of associated nocardiosis |
| Saleiro35 | 1 | 1/0 | 39 | Multiple bilateral | Interferon-2 | None | 9 months | Pleuritic and right-sided chest pain |
| Bahrami36 | 1 | 1/0 | 37 | Left hilar mass | Unresectable Radiation CHT |
Pleura Pericardium Diaphragm Skin of the thoracoabdominal wall |
11 months | Back pain |
CHT, chemotherapy.