Case record...Intramedullary cystic spinal cord metastasis
48 Years old female patient- who was operated upon for grade III breast cancer (Adenocarcinoma)- presented clinically a with
rapidly progressive quadriplegia over 4 months- 6 month after the mastectomy operation- with a C3 sensory level and brown
Sequard spinal cord affection. The patient's condition rapidly deteriorated and she died 6 months following the start of
neurological symptoms. (To inspect the patient's full radiological study, click on the attachment icon (The paper clip icon in the left
pane) of the acrobat reader then double click on the attached file) (Click here to download the attached file)
Figure 1. Precontrast MRI T1 images showing mild dilatation of the spinal cord in cross section with intramedullary cystic
changes. The spinal cord appears irregular in cross section with linear precontrast peripheral hyperintensity (D) probably
representing blood products.
Figure 2. Postcontrast MRI T1 images showing densely enhanced well defined intramedullary oval nodules. Intramedullary T1
hypointensities probably represent cystic changes or cord edema. Notice the linear peripheral meningeal enhancement (A) which
probably represent a concomitant meningeal carcinomatosis.
CASE OF THE WEEK
PROFESSOR YASSER METWALLY
Figure 3. Precontrast MRI T1 image (A) and postcontrast MRI T1 images (B,C) showing irregular intramedullary patchy
enhancement with central MRI T1 hypointensities which probably represent cord edema or cystic changes. The spinal cord is
moderately enlarged from C2-D2. Notice the linear anterior peripheral meningeal enhancement which is -most probably- due to
concomitant meningeal carcinomatosis.
DIAGNOSIS: INTRAMEDULLARY CYSTIC CERVICAL SPINAL CORD METASTASIS
Metastasis to the intramedullary spinal cord rarely occurs with systemic cancer. Autopsy series have reported its prevalence in
cancer patients as ranging from 0.9 to 2.1% (3,6), whereas clinically significant intramedullary spinal cord metastasis have been
reported in 0.1 to 0.4% of cancer patients (5). Lung cancer is the most common source of intramedullary spinal cord metastasis,
comprising approximately 50% of reported cases (5,10,13,14,18). Breast carcinoma is another less frequently seen source,
accounting for 8 to 14% of intramedullary spinal cord metastasis. Other primary sources for intramedullary spinal cord metastasis
include renal cell carcinoma, melanoma, colorectal carcinoma, and lymphoma (5,13,14).
Figure 4. Intramedullary cystic metastasis: Notice the
asymmetric bulging of the spinal cord with multiple
nodular enhancing lesions.
Figure 5. Intramedullary spinal cord metastasis
Clinical presentation with rapid progression of symptoms is highly suggestive of a metastatic lesion (2,15). The most common
symptom associated with intramedullary spinal cord metastasis is weakness (5,14). In their series of 40 patients with
Intramedullary spinal cord metastasis, Schiff and O’Neill (13) reported that 92.5% of patients had motor deficits at the time of
diagnosis. In addition, 45% of patients had either a true Brown-Séquard syndrome or a pseudo-Brown-Séquard syndrome.
Patients with intramedullary spinal cord metastasis often present with a true Brown-Séquard syndrome, a finding that often
portends an intramedullary spinal cord metastasis in cancer patients (11,13). In addition to intramedullary spinal cord metastasis,
patients might also have evidence of brain metastases, an occurrence that Schiff and O’Neill (13) observed in 57.5% of patients
with intramedullary spinal cord metastasis. Other series have reported even higher rates of brain metastases (10). Although the
vast majority of patients with intramedullary spinal cord metastasis often have other locations of systemic spread, only 8 to 11% of
patients have been found to have multiple intramedullary lesions (13,14).
Neuroimaging of intramedullary metastasis
Figure 6. Metastatic involvement of the spine.
Intramedullary metastases reach the cord through
hematogenous dissemination and grow within the
cord parenchyma (1). Leptomeningeal metastases
involve the meningeal membranes of the
subarachnoid space, which are extramedullary
and intradural (2). Epidural metastases usually
arise from the highly vascular posterior aspect of
the vertebral body and produce compression of
the anterior aspect of the spinal cord (3). Epidural
compression can also result from paravertebral
tumors that invade the vertebral foramina (4) and,
less often, from metastases arising in the epidural
space itself (5).
Intramedullary metastasis, in general, produce segmental enlargement of the spinal cord and are often 3-4 spinal segments in
length. However, and and unlike primary spinal cord neoplasms, intramedullary spinal cord metastasis produce asymmetric
nodular bulging of the spinal cord in cross-section. Intramedullary multiple enhancing nodules might be demonstrated in cross
section. Intramedullary metastasis are often cystic and might show hemorrhagic components.
Management of Intramedullary spinal cord metastasis
As no prospective trials on treatment have been performed, treatment of intramedullary spinal cord metastasis has primarily been
based on anecdotal experience. External beam radiation therapy is the most effective treatment option, but its effectiveness
depends primarily on the radiosensitivity of the tumor, the duration of symptoms, and the degree of preoperative neurological
deficit (17). To avoid the sequelae of external beam radiation therapy, stereotactic radiosurgery can be used as an alternative to
treat individual metastatic lesions. However, the current literature only reports effectiveness of this treatment modality with
primary vascular tumors (19).
Surgical management has led to improved neurological function in patients with rapidly progressive neurological deficits (9).
However, as aggressive metastatic lesions typically have unidentifiable margins, the goal of surgery must be to decompress the
spinal cord while, at the same time, maintaining residual neurological function (4). Focal radiation can then be used as an adjunct
to treat residual disease within the resection cavity (13).
Intramedullary spinal cord metastasis are rare complications of systemic cancer. Patients with Intramedullary spinal cord
metastasis experience the rapid onset of a Brown-Séquard syndrome in the setting of recently treated cancer. A clinical
presentation such as this should be considered highly suggestive of an intramedullary spinal cord metastasis.
Spinal cord tumors may be either primary or metastatic, and are further categorized by location: extradural, intradural-
extramedullary or intramedullary. Intramedullary spinal cord lesions comprise only 1-5% of spinal cord tumors (1, 2) . The
majority are primary gliomas, while intramedullary spinal cord metastases (ISCM) account for only 1-3% of all intramedullary
neoplasms (1,2,3,4,5). ISCM are associated primarily with lung carcinoma (50%), especially small cell type, breast carcinoma
(13%), melanoma (9%), lymphoma (5%) and renal cell cancer (4%) and are usually diagnosed well into the course of primary
disease (1,2,4) . As many as 2% of patients in the end stages of disseminated cancer are found to have ISCM. This number is
expected to grow with the increasing availability of improved diagnostic imaging, predominantly MRI, which has become the gold
standard for diagnosis and evaluation of these rare tumors. A biopsy is often required for definitive diagnosis of the lesion as
metastasis versus primary tumor and can help tailor therapeutic approach (5) .
Presenting symptoms of ISCM usually consist of weakness, followed in frequency by sensory loss, pain and bowel and bladder
disturbance. Weakness and pain present early, as compared to sensory loss, with sphincter disturbance having the latest onset.
ISCM are therefore difficult to distinguish clinically from extradural tumors, which often present with a similar constellation of
symptoms. Unlike extradural lesions, and exemplified by this case report, intramedullary lesions may first present with asymmetric
or unilateral symptoms 6 . ISCM can be distinguished clinically from primary tumors by the rapidity of onset of symptoms. A
cause of the sudden onset of motor symptoms may be a vascular event in the tumor bed, such as a venous infarct. Neurological
deficits tend to be irreversible, especially if severe motor weakness and bladder/bowel involvement are noted at the time of first
Prognosis of these metastases is guarded, compounded by both treatment modality and tumor type, with lung and breast
metastases correlating with the shortest survival (4) . Treatment of choice for ISCM consists of immediate steroid bolus, radiation
and chemotherapy with subtotal cytoreductive surgery considered on a case by case basis. High dose dexamethasone may allow for
limited and transient neurological improvement, while radiotherapy often results in stasis of the deficit without improvement.
However, in combination with chemotherapy, radiation correlates with an increased length of survival. In one review of 177 cases,
steroids gave approximately 5 weeks of survival, compared to radiation and radiation with chemotherapy allowing for
approximately 15 and 29 weeks of survival, respectively (21) . Cytoreductive surgery is another treatment option for lesions early
in the course of neurological deficits, and is associated with a 75% increase in time of “high quality of life” survival (3) . It has not
been found to increase the length of survival as compared to other treatment modalities 4 . Our patient presented late in her
course, with a high lesion in the cervical cord. Her surgery carried the specific risks of increasing cord edema, resulting in the need
for long-term mechanical ventilation and worsening neurological deficits. In the face of these risks and the poor temporal prognosis
associated with her breast cancer, it was felt by us that the risks of such surgery were too great. It is likely that the surgical risks
would have been minimized had the patient presented with a smaller lesion prior to the precipitous event that caused her such
sudden quadriparesis. Such early presentation was precluded in our case due to the patient's perceived exclusion from the
healthcare system because of her immigrant status and her lack of insurance.
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