A Report on Kaposiform Hemangioendothelioma in the Cervical Spine
Kaposiform hemangioendothelioma (KHE) is a rare, locally invasive vascular tumor that primarily affects infants and adolescents. With an annual incidence of approximately 0.071 per 100,000 children, KHE is characterized by its aggressive nature and potential for significant morbidity. Although it most commonly presents in soft tissues as mass lesions with associated cutaneous abnormalities and Kasabach-Merritt phenomenon (KMP), KHE can also occur in non-cutaneous sites such as viscera and retroperitoneum. Involvement of the spine is exceptionally rare, with only two previously reported cases in the literature. This report documents a unique case of KHE in the cervical spine of a 40-year-old woman, successfully treated with surgical resection and thalidomide therapy.
The patient presented with chronic, atraumatic pain in the left neck and shoulder region, which had persisted for four years without worsening. Physical examination revealed no neurological deficits or cutaneous lesions. Laboratory tests, including blood routine examination, comprehensive metabolic panel, C-reactive protein, erythrocyte sedimentation rate, and serology tests for tuberculosis, were all within normal limits. Imaging studies were subsequently performed to investigate the cause of the pain.
Unenhanced computed tomography (CT) scan revealed lytic lesions involving the vertebral bodies of C4 and C5, as well as the left pedicle and transverse process of the C4 to C5 levels. These lesions extended into the corresponding intervertebral and transverse foramina. Magnetic resonance imaging (MRI) showed no associated cord compression but revealed hypo-intense signal changes in the C4 and C5 bodies on the sagittal T1-weighted image, which exhibited marked enhancement after gadolinium infusion. These findings raised suspicion of malignancy.
To further evaluate the lesion, 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET)/CT was performed. The study demonstrated mild FDG-avid disease involving C4 and the intervertebral and transverse foramina of the C4 to C5 levels, with a maximum standardized uptake value of 4.6. A CT-guided biopsy of the C4 body was then conducted, and histopathological analysis confirmed the diagnosis of KHE. The histopathological findings showed glomeruloid-like areas characterized by small, thin-walled vascular channels merging with epithelioid cells, consistent with KHE.
Following the diagnosis, the patient underwent surgical resection, which included anterior C4 and C5 corpectomy and autogenous iliac crest grafting with titanium plate fixation. Due to the tumor’s proximity to vital vascular structures, its involvement of multiple vertebrae, and its low-grade nature, the tumors in the intervertebral and transverse processes were not completely removed during the operation. Postoperative histopathological examination of the intraoperative specimen confirmed the diagnosis of KHE.
Postoperatively, the patient was treated with oral thalidomide for eight months, a decision based on previous reports of its efficacy in treating KHE. At the 34-month follow-up, the patient reported no discomfort, and imaging studies showed no evidence of disease progression. The residual tumor in the intervertebral and transverse processes remained stable, and the bony fusion was successful with no displacement of the titanium plate.
KHE is a challenging diagnosis due to its heterogeneous clinical presentations. The most common manifestations include immovable enlarging masses with ill-defined margins. Radiologically, KHE is characterized by heterogeneous hyper-intensity or slight hyper-intensity with speckled hypo-intense signals on T2-weighted MRI. However, these findings are not specific enough to establish a definitive diagnosis. In cases where clinical features and imaging are inconclusive, histopathological examination is essential for confirmation.
The rarity of KHE has resulted in a lack of standardized management strategies. Complete surgical resection is recommended whenever possible to maximize patient outcomes. However, the infiltrative nature of the tumor, the high risk of bleeding, and the anatomic location often make complete resection challenging. In such cases, adjuvant chemotherapy or targeted therapies are considered to achieve local control. Corticosteroids and vincristine have been used with varying success rates, while sirolimus has shown promise due to its ability to inhibit angiogenesis and lymphangiogenesis. Thalidomide, which also inhibits angiogenesis, was chosen in this case based on its reported efficacy in treating spinal KHE.
The use of 18F-FDG PET/CT in KHE is not well-documented in the literature. In this case, although the PET/CT showed mild FDG uptake, the diagnosis remained challenging due to the absence of cutaneous changes, laboratory abnormalities, or soft tissue masses. Nevertheless, PET/CT proved valuable in evaluating the metabolic activity of the lesion and ruling out other pathologies.
This case highlights the importance of a multidisciplinary approach in diagnosing and managing KHE, particularly in rare locations such as the spine. The combination of surgical resection and thalidomide therapy resulted in a favorable outcome, with no evidence of disease progression at the 34-month follow-up. Further research is needed to establish standardized treatment protocols and to explore the role of advanced imaging techniques in the diagnosis and management of KHE.
doi.org/10.1097/CM9.0000000000000217
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