Key-words:
Diffuse large B-cell lymphoma - epidural mass lesion - epigastric pain - non-Hodgkin's
lymphoma - spinal tumor
Introduction
Non-Hodgkin's lymphoma (NHL) compromises the vast majority of lymphomas. Thirty percent
of all newly diagnosed cases of NHL in the United States are of the Diffuse Large
B-cell Lymphoma (DLBCL) type, making it the most prevalent form of NHL in the US.
DLBCL is an aggressive tumour which is fatal if left untreated.[[1]]
Primary Central nervous system (CNS) lymphoma is rare, but when diagnosed it presents
as a DLBCL in 90% of patients.[[2]]
We present a case of an elderly male with unexpected initial presentation and rapid
neurological deterioration. However, he was treated in time and fortunately showed
an excellent recovery.
Case Report
A 60-year-old Middle Eastern male, with no significant medical history, presented
to the emergency room complaining of acute nonradiating epigastric pain started about
6–7 h earlier. There was no vomiting, nausea, or anorexia. There was no jaundice,
pallor, or history of loss of weight. The temperature was 37.5°C. Abdominal examination
was negative for hepatomegaly, splenomegaly, abdominal tenderness, or rebounding pain.
There were no signs of lymphadenopathy. A clinical diagnosis of acute gastritis was
made, and the patient was given proton pump inhibitor and discharged.
Two days later, the pain started to radiate toward the back, and the patient started
to suffer from severe thoracic back pain. Four days later, the pain started to radiate
toward both lower limbs with the subtle beginning of the weakness of the lower limbs
and progressed within a few hours later to inability to walk, with intact neurological
function of the upper extremities.
He denied any history of trauma, recent history of viral illness, or illicit drug
use. On neurological examination, the patient was found to have bilateral numbness
and motor impairment in the lower limbs with muscle strength of 2/5. Hyperactive patellar
and Achilles reflexes were observed. Pain and light touch sensation were objectively
normal; there was no bowel or urinary incontinence. The straight leg raise test was
negative bilaterally.
Magnetic resonance imaging revealed a space-occupying lesion in the spinal canal at
the T6/T7 level without detection of any lesions elsewhere [[Figure 1]]. Because of suspected metastasized tumor, further investigations were undertaken.
Computed tomography (CT) scan of the neck, thoracic, abdomen, and pelvis were performed
and did not reveal any masses, enlarged visceral organs, or lymph nodes. A bone marrow
biopsy presented no marrow involvement.
Figure 1: (a) Magnetic resonance imaging T2. (b) Magnetic resonance imaging T1. (c) Magnetic
resonance imaging T1 with contrast
In view of the rapid progression of the neurological deficit, the patient emergently
underwent a neurosurgical decompression through partial laminectomy with total resection
of the extradural mass.
The pathohistological examination of the biopsy revealed diffuse malignant infiltration
of large atypical lymphoid cells, large vesicular nuclei, prominent nucleoli, and
coarse chromatin.
Numerous mitotic cells were also present [[Figure 2]]a. Immune stains were positive for CD20 and leukocyte common antigen [[Figure 2]]b and negative for CD3 [[Figure 2]]c and CD30 [[Figure 2]]d. These findings are consistent with diffuse large B-cell lymphoma. After the diagnosis
was made, the patient underwent chemotherapy.
Figure 2: (a) Pathohistological examination of the biopsy revealed diffuse malignant infiltration
of large atypical lymphoid cells, large vesicular nuclei, prominent nucleoli, and
coarse chromatin. Numerous mitotic cells were also present. (b) Immune stains were
positive for CD20 and leukocyte common antigen. (c) Immune stains were negative for
CD3. (d) Immune stains were negative for CD30
Day after day, the patient gradually regained the strength in all muscle groups and
was able to walk 1 month after surgery. Furthermore, the patient has been followed
up for over 2 years without any signs of recurrence both clinically and radiographically
[[Figure 3]].
Figure 3: Magnetic resonance imaging showing no tumor or any signs of recurrence after 2 years
Discussion
Non-Hodgkin's lymphoma (NHL) usually presents with a fast-growing and symptomatic
mass, usually located in the neck, chest, or abdomen. However, it may present as a
mass lesion anywhere in the body, including the spinal canal.[[3]] Lymphoma in the epidural space of the spine is very rare and clinically presents
as back pain of the thoracic spine, followed by neurologic deterioration. In addition,
NHL presenting for the first time as spinal cord compression is rare and occurs in
between 1% and 5% of cases.[[2]]
The most prominent notification in our case is how the patient's initial presentation
of epigastric pain for 2 days; then, the pain started to radiate toward the back,
and finally, the symptoms and signs of the spinal cord appeared. Neurological deterioration
usually occurs over several days or weeks. In our case, it progressed very rapidly
within very few hours. Therefore, the surgical intervention has been done as an emergency.
Interestingly, abdominal pain could be more frequent as thought an initial presentation
of spinal epidural mass lesions such as a spontaneous spinal epidural hematoma[[3]] or a spinal epidural abscess.[[4]]
It indeed does not initially come one's mind to consider NHL in the differential diagnosis
of a patient with acute epigastric pain that converts later to a thoracic back pain;
however, in the context of an appropriate constellation of sudden onset and localized
thoracic back pain in an elderly patient, which significantly increases despite the
treatment with analgesics, mass lesion warrants careful consideration as an etiologic
entity and further investigation is needed to confirm or rule out any suspected tumor
mass. If a mass is present, especially in the epidural space, CT-guided biopsy is
needed to determine the underlying etiology.
The treatment for spinal cord compression associated with NHL lacks definitive evidence
but includes chemotherapy, radiotherapy, corticosteroid, and surgery. One approach
proposed immediate surgery for patients presenting with profound neurology cord compression
symptoms. Chemo- and radiotherapy should be started after surgery unless the patients
were not eligible for such additional therapy modality due to poor condition. For
those with mild-to-moderate cord compression symptoms, an initial conservative trial
of chemoradiotherapy may be initiated. If symptoms deteriorated or failed to improve
within 2 weeks of initiating conservative treatment, surgery is definitely indicated.[[5]]
A study conducted by Chang et al. reported complete recovery of neurologic symptoms
in all patients who underwent decompressive surgery compared with 20% of those in
the nonsurgical group. Furthermore, they noted a trend for improved median survival
in the surgical group.[[6]]
Conclusion
In this case, the patient presented complaining of epigastric pain. This pain radiated
later to the back, and after a few days, the patient started to have bilateral weakness
of the lower extremities. Therefore, the decompressive intervention must have been
done as an emergency. According to the symptoms in our case report, maybe, we should
keep in mind that the epigastric pain associates sometimes with the epidural spinal
masses at the thoracic spine. Physicians should include NHL in the differential diagnosis
of a newly diagnosed epidural spinal tumor to expedite diagnosis before the development
of neurological deterioration. An early diagnosis, in the time when chemoradiation
could be sufficient, could save the surgical intervention.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
In the form, the patient has given his consent for his images and other clinical information
to be reported in the journal. The patient understands that name and initials will
not be published and due efforts will be made to conceal identity, but anonymity cannot
be guaranteed.
