Late presentation of Two Rare Sacral Spine
Tumors challenges of Diagnosis and
Treatment in a Low Resource Setting
Alexis DB Buunaaim1,2,*, Anwar Sadat Seidu1,2, Emmanuel Akorli3, Yempabe Tolgou1 and Der EM4
1Orthopedic Unit, Tamale Teaching Hospital, Ghana
2Department of Surgery, University for Development Studies, Ghana
3Department of Radiology, Tamale Teaching Hospital, Ghana
4Department of Pathology, University for Development Studies, Ghana
Submission: September 18, 2020; Published: September 25, 2020
*Corresponding author: Dr. Alexis Dun Boib Buunaaim, Tamale Teaching Hospital, Tamale, Ghana
How to cite this article: Alexis DB B, Anwar S S, Emmanuel A, Yempabe T, Der E. Late presentation of Two Rare Sacral Spine Tumors challenges of
Diagnosis and Treatment in a Low Resource Setting. JOJ Case Stud. 2020; 11(4): 555818. DOI: 10.19080/JOJCS.2020.11.555818.
Lower back pain with or without an associated mass should be examined thoroughly by physical examination aided with radiological imaging. Described here are two young male adults; 29 and 30 years of age with lower back pain associated with lumbar masses. These patients initially treated themselves with herbal preparations without success. Diagnoses of chordoma and myxopapillary ependymoma were made in the 29 and 30-year old patients respectively after physical examination and imaging. These diagnoses were confirmed by histopathological investigation. Both patients requested discharge against medical advice. Unfortunately, they died at home within one month after discharge.
The diagnosis of chordoma and myxopapillary ependymoma can be very challenging and requires a multidisciplinary approach involving a radiologist, pathologist, oncologist and an orthopedic surgeon.
The poor outcomes in both patients could be attributed to ignorance, entrenched cultural beliefs, late diagnosis from low index of suspicion and lack of requisite diagnostic tools. This indicates that patients are still dying of treatable conditions in low- and middle-income countries especially in Africa. We propose awareness creation among clinicians, and a fund to be established both locally and globally to help with the diagnosis and treatment of sacral tumors.
Keywords:Sacral chordoma; Myxopapillary ependymoma; Late presentation; Spinal tumors
Abbreviations: CT: Computed Tomography; MRI: Magnetic Resonance Imaging; CNS: Central Nervous System; MPE: Myxopapillary Ependymoma; CBC: Complete Blood Count; LFT: Liver Function Test; RFT: Renal Function Test; ESR: Erythrocyte Sedimentation Rate
Chordomas are rare slow growing malignant bone tumors that arise from embryonic notochordal remnants [1,2]. They account for 1-4% of all malignant bone neoplasms [3,4]. They occur in various anatomic locations with the sacral region being the most predominant site and lumbar spine being the least (sacrum 60%, spheno-occipital 25%, cervical 10% and thoracolumbar 5%) . Symptoms generally depend on their location but patients typically present with pain at the initial stages [4,6-11]. Some patients may present with constipation, urine and or fecal incontinence due to autonomic nervous system involvement .
Chordomas pose diagnostic challenges to physicians [1,4]. However, diagnosis can be made through the use of CT and MRI, but histopathology is the mainstay of establishing diagnosis [1,2,4,6,12]. Surgical resection (marginal en bloc) with or without adjuvant radiotherapy is the treatment of choice [1,2,6,13,14]. Chordomas have poor prognosis because of local recurrence and metastasis [6,12], hence require yearly follow up even after 20-years of surgical resection .
Ependymomas are primary central nervous system (CNS) tumours that arise from the ependymal cells of the choroid plexus. They account for 2% of all primary CNS tumors .
They are divided into 5 subtypes: sub-ependymoma (grade I),
myxopapillary ependymoma (grade I), ependymoma (grade II),
RELA fusion-positive ependymoma (grade II or III), and anaplastic
ependymoma (grade III) . It is twice as common in men than
women with an average age of presentation of 36yrs .
Myxopapillary ependymoma (MPE) commonly occur in the
conus medullaris, cauda equina and filum terminale of the spinal
cord . MPE are grade I tumours and so they tend to grow
slowly . They have a high survival rate (98.4%) if early surgical
intervention is performed .
We present two cases of rare spinal tumours; chordoma and
myxopapillary ependymoma in Northern Ghana, who presented
late to our institution for care when the initial herbal medical
treatment failed. Clinical examination aided by imaging resulted
in the suspicion of tumor. Histopathological examination of biopsy
samples taken were confirmed as chordoma and myxopapillary
ependymoma. We lost both patients a month after discharge
against medical advice. This case report illustrates how late
presentation and misdiagnosis results in poor outcomes of
patients in low and middle resource settings.
A 30-year old male presented with low back pain of 15 months
duration. He also had a fungating lower back mass of 6-month
duration. The patient initially managed the pain with over the
counter analgesics until 10-months prior to presentation when he
noticed a mass in his lower back. He went to a traditional healer
where he was treated with herbal medications for 6-months
because he thought it was a spiritual ailment. During this period,
he began to experience a gradual loss of power in the lower limbs
and this was associated with urine and faecal incontinence. He
was bedridden and thus developed decubitus ulcers and the mass
became ulcerated. He reported to a primary health facility because
of the fungating mass and inability to move his lower limbs, where
he was referred to our facility (tertiary).
On examination, he was a chronically ill looking man who
had conjunctiva pallor, anicteric and mildly dehydrated. Systemic
examination was essentially normal. He had a fungating lower
back mass measuring 9×6cm, hard with irregular edges. The
power in his lower limbs was 0/5 with lax anal sphincter tone.
Lumbosacral CT scan showed a heterogenous sacral
mass measuring 5.8×6.5×6.2cm with bony distraction of the
sacrococcygeal spine (Figures 1A & 1B). Lumbosacral MRI showed
a 6.8×7.0×6.8cm lobulated heterogenous T1W hypointense and
T2W hyperintense right sided mass involving the vertebral bodies,
laminae and pedicles of S1, S2 and S3. The mass extends across
the sacroiliac joint and involves the right iliac bone. It encases the
right S1-S3 nerve roots with attendant severe canal stenosis. There
is also infiltration of the right erector spinatus muscle posteriorly
(Figures 1C & 1D).
Hematology and biochemistry results
Complete blood count (CBC) showed a low hemoglobin. Renal
function and liver function tests (RFT and LFT) were essentially
normal and the erythrocyte sedimentation rate (ESR) was
Histopathology from an incision biopsy showed a cellular
lesion with bubbly myxochondroid stroma. The cells were illdefined
with round oval nuclei. Some of the cells had vacuolated
cytoplasm and prominent vesicular nuclei while others had small
with inconspicuous nuclei and no visible nucleoli. The stroma
was infiltrated with inflammatory cells. The histopathological
findings confirmed the diagnosis of Chordoma in line with the
clinical diagnosis. Immunohistochemical examination could not
be performed by our laboratory.
While conducting a metastatic workup patient requested to
be discharged against medical advice for care at home and died a
A 29-year old male who presented with a 12-month history
of low back pain and a slowly growing mass in the lower back.
The pain increased in intensity over the period and was associated
with lower limb weakness and urine and fecal incontinence. He
resorted to herbal medications as well as over the counter drugs for
7 months. He presented to a Primary health facility where he was
misdiagnosed as Pott’s disease and treated with antituberculous
drugs for 5 months before referral to our facility because of lower
On examination, he was a chronically ill looking man who was
moderately dehydrated. Systemic examination was essentially
normal. He had a mass in his lower back measuring 7×5cm, it was
hard, nodular, tender with irregular edges. The power in his lower
limbs was 4/5 and anal sphincter tone was good.
Lumbosacral CT scan showed a heterogenous soft tissue
mass involving the sacrococcygeal spine with bony distraction. It
invaded the distal spinal canal and the surrounding soft tissue. The
lumbar vertebral bodies and their intervertebral disc were normal
(Figure 2A, 2B & 2C). Lumbosacral MRI showed an ill-defined
sacrococcygeal tumour measuring 11.6×8.9×15.6cm with bony
distractions of the sacrum and coccyx, extending to L3 vertebra
and invasion of the distal spinal canal. There was severe spinal
canal stenosis from L3 to the sacral elements with destruction of
the involved vertebral bodies (Figure 2D).
Hematology and biochemistry results
CBC, RFT and LFT were essentially normal with elevated ESR.
Histology from a core biopsy showed fragments of
fibrocollagenous tissue infiltrated by an epithelial lesion with
papillary pattern of growth. There were also areas of myxoid
changes. This confirmed the diagnosis of myxopapillary
ependymoma was made and this agreed with the initial diagnosis
made by the clinicians. Immunohistochemical examination could
not be performed by our laboratory.
The patient requested discharge against medical advice on
financial grounds. He succumbed to the disease after 3 months of
Chordomas are rare slow growing malignant bone tumors
accounting for 1-4% of all malignant bone neoplasms [1,6].
Because of their slow growing nature, they are clinically dormant
until later in the course of the disease . The symptoms generally
depend on their location with commonest being pain and
neurologic symptoms [4,6-11]. For these reasons, most patients
present to health facilities when the course of the disease is
advanced. However, in low resource settings where the hospital
is usually not the first point of call for a lot of patients (but the
Traditional healer), patients present much later to the hospital
when all hope is lost. This leaves the surgeon with very few
options to work with. This was demonstrated as it took 15 months
from the onset of symptoms, to presenting to a health facility. The
patient in this current case report presented with very advanced
form of the disease. In poor resource settings, most patients will
resort to the Traditional healer whose services are relatively
affordable compared to Orthodox health facilities, as illustrated in
Mcmaster et al.  in 2001 reported the incidence rate of
chordoma as 0.08 per 100,000 in the United States . They
also reported that chordomas are uncommon in blacks and
people under 40yrs . They observed a male predominance
with an incidence rate of 0.10 as compared to females with 0.06
. Murphey et al reported the male to female ratio of 2-3:1 .
The current case report from Northern Ghana involved a black
male of 39-years of age. Being a male increased his chances of
developing a chordoma, but his race and age reduced his risk. This
accounts for the rarity of such cases in our settings and sometimes
contribute to the low index of suspicion by most physicians. The
young age group and the female sex are more likely to develop
cranial chordoma  which contrast with the sacral presentation
of case 1.
Diagnosing chordoma can be very challenging [1,4] but
radiological imaging (CT and MRI) play a crucial role in
preoperative diagnosis of chordoma [1,2,4,6,12]. Murphy et al
illustrated that, most primary tumors of the spine present with
characteristic radiologic features on CT and MRI . With the
aid of diagnostic images and the appropriate technical human
resource, diagnosis can be made easily.
It is worth noting that plain radiographs cannot be used in
diagnosing myxopapillary ependymoma (MPE). In low resource
settings, primary and secondary health facilities only have access
to X rays, hence the reason for misdiagnosis of case 2. The patient
was misdiagnosed as Pott’s disease and received tuberculosis
treatment regimen for 5 months which delayed time to definitive
diagnosis. He was referred from the primary health facility to a
tertiary facility for specialist care. A lumbosacral CT and MRI was
done and a core biopsy taken for histopathology to confirm the
Both chordoma and MPE have good prognosis when they
present early because they can be completely resected [4,18].
Prognosis is largely poor if both chordoma and MPE are left
In our opinion, poverty and ignorance is the main reason for
late presentation of most patients in low resource settings of rural
Ghana. Patients are more likely to resort to Traditional healers as
their first point of call because their services are cheaper and are
more accessible compared to orthodox health services. Also, the
bureaucracies in the orthodox health services can be frustrating
to patients. Our primary and secondary health services are under
resourced both in diagnostic capability and human resource,
which accounted for the misdiagnosis.
The lesson from these cases is for clinicians to develop a high
index of suspicion and early referral of patients to appropriate
centers for treatment especially when there is a suspicion of
a cancer. Policy makers in low resource settings should work
towards developing the referral and diagnostic capabilities of
their primary and secondary health services. They also need to
reduce the cost of diagnostic investigations especially for cancer
through an established fund to enable patients present early to
health facilities for prompt diagnosis and treatment.
ADBB conceived the idea. ASS and ADBB drafted the
manuscript. EM made the radiological inputs and EMD read the
histopathology. ADBB, ASS, TY critically reviewed the manuscript
for academic content. All authors read and agreed on the final