Anteroposterior Combined Surgery of a Rare Massive Epithelioid Hemangioendothelioma at the Cervicothoracic Junction

Abstract

Epithelioid hemangioendothelioma is a rare mesenchymal tumor of vascular endothelial origin. Non-soft tissue epithelioid hemangioendothelioma can also be seen in different organs. Although chemotherapy has been used in some patients, complete surgical removal of the tumor tissue has proven to be the most durable solution. A 15-year-old female patient was admitted to our institution with right arm and neck pain. The patient complained of numbness and weakness in the right hand. Computerized tomography indicated an expansile lesion exhibiting osteolytic features located predominantly on the right side of the corpus, pedicle, lamina, and lateral processes of the C7-T1 vertebra. The patient underwent a surgical procedure involving the application of a bilateral C4-5-6 lateral mass screw, left C7-T1 pedicle screw, and bilateral T2-3 pedicle screw and fusion. The complete residual neoplasm was surgically removed during the procedure. Due to the rarity of epithelioid hemangioendothelioma, the existing literature on this topic is confined to case reports, supplemented by a small number of retrospective descriptive case series that aimed to improve our understanding of the clinical, pathological, and molecular features of the condition, as well as to guide potential treatment strategies.

Keywords: combined surgery; craniocervical junction; epitheloid hemangioendothelioma; pediatric neurosurgery; spinal tumor.

Figure 1. Preoperative images of the patient.

(A) The cervical anteroposterior radiograph revealed the presence of an osteolytic lesion that appeared to be casting a shadow on the soft tissue on the right side. (B) The sagittal computed tomography (CT) and (C) axial CT scan revealed that the lesion had infiltrated the C7-T1 region and resulted in osseous destruction. The magnetic resonance imaging (MRI) findings indicated that the lesion exhibited (D) hypointensity on T1-weighted images, (E) hyperintensity on T2-weighted images, and (F) demonstrated significant enhancement on contrast images. The axial MRI images revealed that the neoplasm infiltrated the (G) soft tissue and the (H) right vertebral artery. (I) The patient’s MRI image reveals a tumor with an intradural onset two years ago. *: the tumor. White arrow: right vertebral artery.

Figure 2. (A) Postoperative anteroposterior radiograph. (B) Postoperative three-dimensional computed tomography image.

Figure 3. Histopathological findings of epitheloid hemangioendothelioma.

(A) Hematoxylin-eosin staining (H&E) at 10× magnification: neoplastic proliferation with myxo hyalinized stroma alongside degenerated bone trabeculae. (B) H&E at 40× magnification: black arrow: epithelioid-looking atypical cells with large eosinophilic cytoplasm, red arrow: intracytoplasmic clear vacuoles, some of which contain erythrocytes. (C) Epithelioid appearance with extensive eosinophilic cytoplasm. (D) Area of necrosis.

Figure 4. (A) Pan-CK (-), (B) ERG (+), (C) CD31 (+), and (D) CD34 (+).

Figure 5. Postoperative images of the cervicothoracic junction tumor.

(A) Axial preoperative magnetic resonance imaging (MRI). (B) After the first operation with the posterior approach, the axial MRI image showed wide resection of the tumor in the posterior area. Axial MRI (C) and sagittal MRI (D) showing complete excision of the tumor after anterior surgery, the second surgery. *: the tumor. White arrow: tumor lodge.