Introduction: Hemangioendothelioma denotes rare vascular tumors of intermediate grade malignancy, locally aggressive but rarely metastasizing. More than 160 cases of head and neck hemangioendothelioma have been reported in the English, Spanish and Korean literature, but its occurrence in a deep compartment of the head and neck, such as the carotid space has not been well documented.

Case report: Hemangioendothelioma appearing as a bulging lateral neck mass of 6–7 years duration in a 50-year-old male, with synchronous submandibular swelling. Radiological evaluation revealed right level II lymph node enlargement and a 3 cm × 3.5 cm well-circumscribed soft tissue mass, posterior and adjacent to the right submandibular gland, displacing the carotid sheath, external jugular vein, sternocleoidomastoid and platysma muscles. Also a 7 mm-long calcification was noted in the distal portion of the right Wharton’s duct, interpreted as sialolith. Excision of the lateral neck mass and removal of the right submandibular gland and sialolith were performed.

Result: Microscopically, solid nests of rounded to slightly spindle endothelial cells were observed. Pleomorphism was noted but mitosis was not identified. Tumor infiltration was detected in the surgical resection margin. The final pathologic diagnoses for the submandibular and neck masses were hemangioendothelioma and sialadenitis with sialolithiasis, respectively. Due to the unpredictable biological behavior and recurrent potential of hemangioendothelioma, the patient has been under close follow-up for 8 months without evidence of local recurrence or metastasis.

Conclusion: In our knowledge, this is the first case to report such rare occurrence of head and neck hemangioendothelioma. Literature review is also summarized and discussed.

Conflict of interest: None declared.