Metastasizing chordoma of the lumbar spine

Abstract

Chordoma is a dysontogenetic bone tumour that appears in the region of the axial skeleton. Its malignant transformation has been underestimated in the past, since the incidence of metastasis is disputed. This paper describes a chordoma of the fifth lumbar vertebra, which at first was monolocular, but in the course of the disease led to a diffuse metastasis of the spine. Specific histological findings and the differential diagnosis of the chordoma are discussed, as well as previous descriptions in the literature relating to course, diagnosis, and therapy.

Chordoma of the Mobile Spine: Fifty Years of Experience

“…The tumor invaded the whole vertebra at the clinical onset in 10 patients (19%). In 18 patients (34.5%), the tumor occupied only the vertebral body (5)(6)(7)(8). In 10 patients (19%), the tumor occupied the body and 1 pedicle (sectors 4 -8 or 5-9).…”

Metastatic Chordoma: Report of the Two Cases and Review of the Literature

“…Treatment is challenging because of their unique location, close vicinity to key structures, contiguous spread and relapse. The reported incidence of metastases in the literature vary widely from 3 to 48% with the lungs, liver, bone and lymph nodes being the commonly involved sites [1][2][3][4]. We describe two cases of metastatic sacral chordoma, stable on molecular targeted therapy.…”

“…The reported incidence of metastases in chordoma is between 3-48% [3]. Metastases are known to occur more commonly when the primary is located in the sacrococcygeal region, compared to the clivus [1].…”

“…Metastases are known to occur more commonly when the primary is located in the sacrococcygeal region, compared to the clivus [1]. Metastatic disease has been reported in almost every organ: lung, liver, bone, lymph node, brain, solid abdominal organs, peritoneum and even in the breast [1][2][3][4]18]. Occurrence range of the metastases has been reported to be as early as 2-3 months [19,20] to as late as 9-10 years after initial treatment [19].…”

Imaging of the Lumbar Spine Neoplasms

“…They have been described to be more common at the cranial and caudal ends of the spine, that is, clivus and sacrum, respectively, but may occur at any level, with 35% arising in the lumbosacral spine. [8][9][10][27][28][29][30] The basic cell type of a chordoma appears soap bubbly under light microscopy (physaliphorous), thus dictating its imaging properties. It appears as a lytic lesion on radiography and CT scanning.…”