K Nair Lekha, Das Anitha, Kumar A S Arun, Ramachandran V
Department of Pathology, MES Medical College, Perinthalmanna. Malappuram District. Kerala. India.
Department of Orthopaedics, MES Medical College, Perinthalmanna. Malappuram District. Kerala. India.
J Orthop Case Rep. 2015 Jan-Mar;5(1):37-40. doi: 10.13107/jocr.2250-0685.251.
Epitheloid hemangioendothelioma is a rare vascular soft tissue tumour of intermediate malignant potential. The tumor affects any age group, and liver is the commonest internal organ affected. Bones as calvarium, spine, tibia and femur may also be affected. The lesion can be multifocal in the liver or the same bone itself and can metastasise to lungs. Here we report a rare case of epithelioid hemangioendothelioma involving tibia in a male infant, which regressed by itself without any treatment, after incisional biopsy over a period of one and half years.
A 7 month old male baby was presented with incessant cry, fever and swelling in left upper leg of 2 weeks duration. X-ray of leg showed a well demarcated lytic lesion in the meta diaphysial region of left tibia. Magnetic resonance imaging showed an irregular lytic lesion with intramedullary extension. Incisional biopsy showed a vascular tumor, epithelioid hemangioendothelioma grade 1. Meanwhile the patient became asymptomatic and subsequent follow ups showed regression in the size of the tumor and complete disappearance after one and half years. This points towards the need of a wait and watch policy in such intermediate grade vascular tumours even though the lesion is so extensive. This is the first case report of an extensive self regressing epithelioid hemangioendothelioma of tibia in an infant to our knowledge.
Intermediate grade vascular tumors can undergo spontaneous regression which points towards the need of a wait and watch policy in such tumors thus avoiding extensive surgeries, especially in young patients.
上皮样血管内皮瘤是一种罕见的具有中等恶性潜能的血管软组织肿瘤。该肿瘤可发生于任何年龄组，肝脏是最常受累的内脏器官。颅骨、脊柱、胫骨和股骨等骨骼也可能受累。病变在肝脏或同一骨内可呈多灶性，且可转移至肺部。在此，我们报告一例罕见的男性婴儿胫骨上皮样血管内皮瘤病例，在切开活检后一年半的时间里，未经任何治疗自行消退。
一名7个月大的男婴因持续哭闹、发热和左腿肿胀2周前来就诊。腿部X线显示左胫骨干骺端区域有一个边界清晰的溶骨性病变。磁共振成像显示一个不规则的溶骨性病变并向髓腔内延伸。切开活检显示为1级血管肿瘤，上皮样血管内皮瘤。与此同时，患者症状消失，随后的随访显示肿瘤大小缩小，一年半后完全消失。这表明对于这种中等分级的血管肿瘤，即使病变范围广泛，也需要采取观察等待策略。据我们所知，这是首例婴儿胫骨广泛的自行消退的上皮样血管内皮瘤病例报告。
中等分级的血管肿瘤可自发消退，这表明对于此类肿瘤需要采取观察等待策略，从而避免进行广泛的手术，尤其是在年轻患者中。
