Spontaneously Regressive Multifocal Bone Pseudomyogenic Hemangioendothelioma In A 17-year-old Boy: A Case Report
Maximen, J., Christory, A., Bonneau-Lagacherie, J., Guillin, R., & Ropars, M. (2023). Spontaneously regressive multifocal bone pseudomyogenic hemangioendothelioma in a 17-year-old boy: a case report. Skeletal radiology, 52(1), 119–127. https://doi.org/10.1007/s00256-022-04109-2
View Original Source →Abstract
Pseudomyogenic hemangioendothelioma (PMH) is a rare vascular tumor that occurs in young mostly male patients. Seventy percent of PMH cases are multifocal and 25% involve bones. PMH is an indolent tumor with mild local aggressiveness and an unclear pathology. Only two cases of spontaneous regressive bone PMH have been reported. Here, we report the case of a 17-year-old boy with a multifocal bone PMH diagnosed from a chronic pain in his left knee. The PMH affected the right scapula, both humeri, the right olecranon, the second metacarpal bone, the second and fourth right ribs, the thoracic and lumbar spine, the pelvic ring, the left and right femoral neck, and the left patella. Every lesion presented with a lobulated, lytic pattern, sometimes with a peripheral sclerotic rim. MRI showed a tissue lesion with a low intensity on T1-weighted sequences and high intensity on T2-weighted sequences. Enhancement of T1 gadolinium fat-saturated sequences was bright. After discussion, a national specialized board decided to actively monitor the patient and start general chemotherapy in the case of progression. The disease was stable at 3 and 6 months and showed signs of regression at 1 year, which was further confirmed at 2 years. CT scan and MRI highlighted a progressive filling of the tumor with cancellous bone and a regression of the tissue contingent. This case report highlights to a new therapeutic approach for indolent PMH that does not prevent further treatment in the case of progression.
Case Details
Disease Location
Bones
Personal Characteristics
17-year-old boy
Clinical Characteristics
Progressively worsening pain in his left knee. It worsened progressively and became constant when running. On physical examination, palpation and mobilization of the patella of the left knee were painful. X-ray examination highlighted a lobulated, lytic lesion of the patella with inner cortical bone destruction and geographic outlines. CT-guided biopsy of the lesion reported loose fascicles of spindle-shaped or oval cells with elongated nuclei. Some cells harbored an eosinophilic cytoplasm, giving them a myeloid aspect. The tumor was positive for ckae1/e3, erg, and CD31 antibodies and negative for mdm2, ps100, CD34, and alk-1 antibodies. A final diagnosis of bone pseudomyogenic hemangioendothelioma (pmh) was made. Tc-99 m bone scan highlighted the multifocal extent of the disease within the right scapula; left and right proximal humerus; right olecranon; second metacarpal bone; second and fourth right ribs; sixth, seventh, and twelfth thoracic vertebrae; first lumbar vertebrae; pelvic ring; left and right femoral neck; and left patella.
Remission Characteristics
Progressive ossification of the lesion was further confirmed at the 2-year follow-up. This reduction of lesions was visible on all affected bones. Some locations had almost disappeared at the 2-year fol- low-up, especially in the pelvic ring
Treatment & Mechanisms
Proposed Remission Mechanisms
None reported
Non-Clinical Treatment
None reported