A Rare Case of Pediatric

Primary Spinal Diffuse Large B-

Cell Lymphoma presenting As
Lumbar Radiculopathy
 Objectives
• To report a rare case of Primary Spinal
Diffuse Large B-cell Lymphoma
 Objectives
• To discuss Primary Spinal DLBCL in
terms of:
• Epidemiology
• Clinical manifestation
• Diagnostic work-up
• Treatment
• Prognosis
General Data
J.J.
10 year old Male
Right Handed
Laguna
 Chief Complaint

Weakness of Both Lower

Extremities
 History of Present Illness
LUMBAR PAIN

 Radiating to both thighs

 Graded 4-5/10
 Compressing
 Aggravated by walking
 Relieved by rest and
Paracetamol

1 month PTA
 History of Present Illness
LUMBAR PAIN

 Increased Severity
 Graded 7/10

 Assessment: Muscle Spasm

 Medications: Ibuprofen, Vitamin B
complex

1 month PTA 3 Weeks PTA

 History of Present Illness
LUMBAR PAIN
LOWER EXTREMITY
WEAKNESS

 Increased Severity
 Graded 8/10

 Difficulty in
ambulation
 Assisted in activities
of daily living

1 month PTA 3 Weeks PTA 2 Weeks PTA

 History of Present Illness
LUMBAR PAIN
LOWER EXTREMITY WEAKNESS

Admitted in a Hospital

Assessment: Myalgia vs Septic Arthritis


CBC, Electrolytes, Radiography NORMAL

5 days PTC
 History of Present Illness
LUMBAR PAIN
LOWER EXTREMITY WEAKNESS

Referred to Neurology

Assessment: Incomplete Spinal Cord
Compression, Spinal mass vs Pott’s
Disease


Medications: Celecoxib, Dexamethasone

Transfer

5 days PTC
 BIRTH AND
FAMILY
MATERNAL HISTORY
HISTORY

GROWTH
NUTRITIONAL IMMUNIZATION AND

Non-Contributory
HISTORY HISTORY DEVELOPMENT

SOCIOECONOMIC PAST
HISTORY MEDICAL
HISTORY
 PHYSICAL EXAMINATION

Conscious, not in cardiorespiratory distress

Wheelchair borne
Normal Anthropometric Measurements
Vital Signs:
 BP: 100/70 mmHg
 HR: 92 bpm
 RR: 22 cpm
Normal
 Temp: 37.5ºC
 O2 saturation: 98%
PHYSICAL EXAMINATION

Normal
NEUROLOGIC EXAMINATION
Mental Status Examination Normal
Cranial Nerves Intact
Reflexes Normoreflexive
Babinski or Clonus Negative
Cerebellum Normal
Autonomic Reflexes Good Anal Sphincteric Tone
Sensory Decreased on Levels L5-S5
Straight Leg Test Positive
 NEUROLOGIC EXAMINATION
Upper Right Left Lower Right Left
Extremities Extremities

Shoulder abductors 5/5 5/5 Hip flexors (L1,L2) 3/5 3-4/5

(C5)
Elbow flexors (C5,6) 5/5 5/5 Knee extensors (L3) 5/5 3-4/5

Elbow extensors (C7) 5/5 5/5 Knee flexors (L5) 5/5 3-4/5

Wrist flexors (C8) 5/5 5/5 Ankle dorsiflexor (L4) 5/5 3-4/5

Finger flexors( C8) 5/5 5/5 Toe extensor (L5) 5/5 3-4/5

Finger abductors (T1) 5/5 5/5 Ankle plantar flexor 5/5 3-4 /5
(S1,S2)
 ADMITTING DIAGNOSIS

Lumbosacral Radiculopathy, Rule Out Myositis

 Diagnostic Work-up
Admission
 Complete blood count
 Lactate Dehydrogenase Normal
 Serum Electrolytes
 CKMM CKMB CK total
Radiograph
Pelvis

Normal
Medications
Tramadol
Eperisone
Pregabalin
 Diagnostic Work-up
Course in the Ward

 EMG-NCV
Normal
 Cranial CT scan
 Diagnostic Work-up
Course in the Ward

 CSF Analysis
 High Protein and Hypoglycorrachia
 Coagulase Negative Staphylococcus
 Medications: Oxacillin and Gentamicin
 Diagnostic Work-up
Course in the Ward
PARESIS
AREFLEXIA
DIPLOPIA

Diffuse and Nodular thickening of

cauda equina rootlets from levels
T12 to S1
 Diagnostic Work-up
Course in the Ward
PARESIS
AREFLEXIA
DIPLOPIA

Thickening of the
bilateral trigeminal
nerves and possibly the
infundibulum
LYMPHOMA
OR
LEUKEMIA
 Diagnostic Work-up
LAMINECTOMY with NERVE ROOT BIOPSY
PARESIS
AREFLEXIA

Atypical Lymphoid
Proliferation
 IMMUNOHISTOCHEMICAL STAINING

2/17/19 CD20
199 2/17/19 CD3
198

TdT 2/17/19 KI67

200
2/17/19 201
 FINAL PATHOLOGIC
DIAGNOSIS

PRIMARY SPINAL
DIFFUSE LARGE B CELL
LYMPHOMA
On discharge

 Regular follow-up with the Hematology-Oncology

Service
 Started on R-CHOP Protocol:

Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
 Salient Features

10 year old
Male
Difficulty in ambulation
Pain
Weakness of both lower extremities
Hypoesthesia of bilateral lower extremities
Straight Leg Test Positive
 WEAKNESS

Complete History
Physical Examination

UPPER LOWER
MOTOR MOTOR
NEURON NEURON
 WEAKNESS

UPPER MOTOR LOWER MOTOR

NEURON NEURON
Weakness Weakness
No Atrophy With atrophy
Spastic Flaccid
↑ Deep Tendon Reflexes ↓ Deep Tendon Reflexes
No Fasciculations Fasciculations
(+) Babinski (–) Babinski

Kliegman, R., Stanton, B., St Geme III, J., Schor, N., & Behrman, R. (2016).
Nelson's Textbook of Pediatrics. Philadelphia: Elsevier, Inc.
 Approach to Diagnosis

Pattern of weakness is essential in identifying
underlying pathology

Guillain-Barre Syndrome
 Patient had asymmetric weakness
 Absence of ascending paralysis

Myositis
 Bilateral hip flexor weakness
 Normal CKMM and EMG NCV results
 Approach to Diagnosis
Pain
Weakness Nerve Root
Sensory Deficit

PRIMARY SPINAL
DIFFUSE LARGE B CELL
LYMPHOMA
 LYMPHOMA
11% of Pediatric
Cancers

NON-
HODKIN’S
HODGKIN’S

NODAL
or
EXTRANODAL
Epidemiology of Primary
Spinal Lymphoma

Non-Hodgkin’s Lymphoma
comprises 5% of childhood cancers

Spinal Lymphoma comprises 1-2% of

Extranodal Non-Hodgkin’s
Lymphoma
 Clinical Presentation
 Thoracic Spine is the most common site of Spinal
Lymphoma
 Due to its length and numerous lymphatic drainage
 In contrast, the patient had lumbar spinal lymphoma

Córdoba-Mosqueda, M., Guerra-Mora, J., et al., Primary Spinal Epidural Lymphoma

As a Cause of Spontaneous Spinal Anterior Syndrome: A Case Report and Literature
Review, J Neurol Surg Rep 2017;78:e1–e4.
 Clinical Presentation
 Spinal Lymphoma can be:
 Asymptomatic
 Back Pain
 Spinal Cord Compression is a rare manifestation

Areflexia

Paresis

Córdoba-Mosqueda, M., Guerra-Mora, J., et al., Primary Spinal Epidural Lymphoma

As a Cause of Spontaneous Spinal Anterior Syndrome: A Case Report and Literature
Review, J Neurol Surg Rep 2017;78:e1–e4.
 Diagnosis
 Imaging modalities are necessary to rule out
a metastatic or systemic lymphoma

Magnetic Resonance Imaging

Positron Emission Tomography

Moussaly, E., Nazha, B., et al., Primary Non-Hodgkin’s Lymphoma of the Spine: A Case
Report and Literature Review, World J Oncol. 2015;6(5):459-463.
 Diagnosis
 Gold Standard

Biopsy

Immunohistochemical stains

Moussaly, E., Nazha, B., et al., Primary Non-Hodgkin’s Lymphoma of the Spine: A Case
Report and Literature Review, World J Oncol. 2015;6(5):459-463.
 Management
 In pediatric patients, the first line of treatment is
Multiagent Chemotherapy
 Indications for surgery

Signs of spinal cord compression needing
decompression

Histopathologic diagnosis

Cho, H., Lee, J., et al., A Rare Case of Malignant Lymphoma Occurred at Spinal Epidural
Space: A Case Report, Korean J Spine 12(3):177-180, 2015.
 Prognosis
 Poor Prognostic Factors

Age > 50

Aggressive Histologic type

Paraplegia

Bowel/Bladder involvement

Poor performance status

Elevated LDH

High CSF Protein

Flanagan EP, O'Neill BP, Porter AB, Lanzino G, Haberman TM, Keegan BM. Primary
intramedullary spinal cord lymphoma. Neurology. 2011;77(8):784-791.
 Prognosis
 Favorable factors for the patient

Age < 50

Absent Bowel/Bladder involvement

Normal LDH
 Non-ambulant patients have a 10-30% chance of
being ambulant after therapy

Flanagan EP, O'Neill BP, Porter AB, Lanzino G, Haberman TM, Keegan BM. Primary
intramedullary spinal cord lymphoma. Neurology. 2011;77(8):784-791.
 Summary

10 year old male

Progressive weakness, pain, sensory deficits

Signs of spinal cord compression

Underwent Laminectomy with Biopsy

Primary Spinal Diffuse Large B-cell Lymphoma

Chemotherapy and Surgery
 Summary
 Spinal Lymphoma should be considered as a
differential diagnosis for a patient with lumbar
radiculopathy
 Update
 Still on Chemotherapy
 Improved lower extremity motor and sensory
deficit
 But did not regain full function of his lower
extremities
Thank you!