Composite hemangioendothelioma in the cervical spine with kaposiform hemangioendothelioma features in an elderly patient: a case report

Abstract

Background Composite hemangioendothelioma (CHE) is an intermediate group of tumors with features between hemangioma and angiosarcoma both histologically and biologically. CHE is predominant in young and middle-aged adults, but very infrequently affects the spine. We describe the case of primary CHE in the cervical spine exhibiting kaposiform hemangioendothelioma (KHE)-like components that was associated with cervical myelopathy with vertebral body destruction in an elderly woman. We retrospectively reviewed the case of a primary cervical spinal tumor, diagnosed as CHE with KHE-like components in pathological findings, associated with cervical myelopathy and cervical vertebral body destruction. Case presentation An 80-year-old woman presented with progressive cervical myelopathy caused by a cervical spine tumor. Preoperative cervical MRI revealed a neoplastic lesion invading the cervical spine that strongly compressed the spinal cord, causing right upper-limb paralysis. We performed partial tumor resection along with posterior decompression and fixation. Postoperatively, pathological findings showed that the tumor was CHE with KHE-like features. Following radiotherapy, no recurrences have been observed in 21 months. Conclusions This is the first report of CHE with features of KHE in the spine of an elderly patient. Posterior decompression and fusion of the cervical spine and subsequent radiotherapy resulted in a good outcome.

Dural composite hemangioendothelioma: The first intracranial case

“…Areas of fibrosis and abnormal lymphatic spaces may be seen at the periphery of the lesion. [96] While KHE has recently been reported within a CHE, [60] no histologic evidence of KHE was identified in our patient's neoplasm.…”

“…Less than 100 cases have been reported in the literature [Table 1]. [4,5,8,[11][12][13]15,[18][19][20][21]25,28,31,32,36,39,41,46,[48][49][50][51][52]55,56,58,60,61,69,74,75,77,80,81,87,88,90,91,93,100,102,103] Two patients with primary spinal CHE have been reported. [58,60] e reported median age at diagnosis is 42.5 years, which is nearly identical to our patient's age.…”

“…[4,5,8,[11][12][13]15,[18][19][20][21]25,28,31,32,36,39,41,46,[48][49][50][51][52]55,56,58,60,61,69,74,75,77,80,81,87,88,90,91,93,100,102,103] Two patients with primary spinal CHE have been reported. [58,60] e reported median age at diagnosis is 42.5 years, which is nearly identical to our patient's age. Risk factors for CHE include Maffucci syndrome, lymphedema, and a history of radiation therapy.…”