doi:10.1111/j.1750-3639.2010.00382.
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CASE OF MONTH FEBRUARY 2010
20 YEAR OLD LADY WITH A PARASPINAL MASS
Orna O’Toole1, Alan O’Hare2, Liam Grogan4, Ciaran Bolger3, Francesca M. Brett1
Departments of 1 Neuropathology, 2 Neuroradiology, 3 Neurosurgery and 4 Oncology Beaumont Hospital, Dublin 9.
CLINICAL HISTORY
A 20 year old woman attended her general practitioner with right
upper limb pain and intermittent paraesthesias for a 4 month period.
She had no neck pain or systemic symptoms and was a non smoker.
There was no family history of note. She was commenced on
pregabalin for pain and an MRI of cervical spine was ordered. The
MRI revealed a right-sided intradural extramedullary mass extend-
ing from C7-T1 that was displacing the spinal cord to the left. She
was reviewed by neurosurgery and was now complaining of paraes-
thesias in the right lower limb also. She had no bowel or bladder
symptoms. Her examination revealed reduced sensation in the right
upper limb but normal tone, power, coordination and reflexes.
Cranial nerves, the left upper limb and bilateral lower limb examina-
tion were documented as normal. Imaging revealed that the lesion
now extended from C5 to T3 and was causing significant cord
compression at C7-T1. An enhancing extradural soft tissue mass
Figure 1.
Figure 2.
Brain Pathology 20 (2010) 683–684
© 2010 The Authors; Journal Compilation © 2010 International Society of Neuropathology
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centered in and expanding the exit foramina and indenting the thecal
sac (arrow) is shown by axial T1 MRI post-contrast (Figure 1). On
the T2 Sagittal MRI (Figure 2) the low signal soft tissue mass is
demonstrated in the right exit foramina at four spinal levels(small
arrows), normal high signal fat is seen in the foramen below (larger
arrow). (Figure 2) The patient underwent emergency resection
of the lesion. A large rubbery, tan piece of tissue measuring
1.5 ¥ 0.8 ¥ 0.5 cms and further multiple pieces of cream grey tissue
measuring 3.5 ¥ 3 ¥ up to 0.3 cm in aggregate were removed.
PATHOLOGY
Paraffin sections showed a nodular and diffuse cellular infiltrate
with intervening fibrous bands. The cellular areas contained sheets
of histiocytoid cells with scattered large pleomorphic cells with
prominent eosinophilic nucleoli (Figure 3). Admixed with these
were eosinophils and plasma cells. No classic Reed Sternberg cells
were identified. Immunocytochemistry for CD30 and CD15 were
positive in the larger cells (Figure 4). These cells were negative for
CD21, CD23 and ALK.
Figure 3.
Figure 4.
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FINAL DIAGNOSIS
Hodgkins disease-mixed cellularity type.
DISCUSSION
This case was complex and surprising in that by pre-operative
radiological studies the patient was felt to have a plexiform neu-
rofibroma. Definitive diagnosis was not made at frozen section. The
differential pathological diagnosis initially included follicular den-
dritic cell sarcoma, anaplastic Large Cell Lymphoma (ALK) or
Hodgkin’s Disease. Post operative scans showed extensive nodular
residual mass extending into the soft tissues of the neck from the
paraspinal region to deep to the sternocleidomastoid muscle. An
incidental superior mediastinal mass lesion was noted adjacent to
the trachea of mixed signal intensity with co-existent lymphaden-
opathy in both supraclavicular fossae. CT of thorax, abdomen and
pelvis was carried out to further characterize the mediastinal lesion
and look for other evidence of lymphadenopathy elsewhere. No
other lesions were identified. A CT guided biopsy of the posterior
mediastinal mass was carried out. This revealed features consistent
with Hodgkin’s lymphoma. Bone marrow biopsy was negative.
Patient was considered to have stage IV Hodgkin’s mixed-cellular
type. The patient has been commenced on ABVD chemotherapy,
she is currently undergoing her fourth cycle.
Primary CNS-Hodgkin’s lymphoma (CNS-HL) is exceedingly
rare with a reported incidence of 0.02–0.5% of all HL cases (6, 9).
The literature available consists of case reports and small case
series. Common presentations include cranial nerve palsies, motor/
sensory deficits, headache, visual disturbance, seizures and coma
(8). Parenchymal disease is most common followed by dural based
disease (6). Paraspinal or spinal masses are not well described.
Involvement of the dura mater may be de novo or may involve
spread from a contiguous parenchymal lesion or metastases from
systemic HL (3, 5, 10). Median age at diagnosis is approximately
25 years (6). There is a reported increased risk of CNS involvement
in HL of a mixed-cellularity type (as in our patient) which accounts
for up to 44% of CNS cases of HL (1). In a recent case series of 16
patients with CNS-HL, 8 patients had CNS-HL occurring as a
relapse of systemic disease, 2 had primary CNS-HL and 6 patients
had evidence of systemic HL although it was initially discovered in
the CNS. 5 cases had dural based non parenchymal tumors (10).
Although dural based HL mimicking meningioma has been
described there are no previous reports of HL mimicking a plexi-
form neurofibroma in the literature (4). In contrast there have been
several cases of NHL mimicking both neurofibromatosis and men-
ingiomas (2, 7). Because CNS involvement is rare in HL there is no
consensus on therapy although various combinations of surgery,
chemotherapy and radiation therapy were used in the recent series
reported by Gerstner et al Chemotherapy regimes varied widely.
Our patient is currently receiving ABVD which was used in 2
patients in the Gerstner cohort with 50% success rate (5). This case
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© 2010 The Authors; Journal Compilation © 2010 International Society of Neuropathology
is unusual because CNS-HL involvement is extremely rare espe-
cially extracranially. When it does present in this fashion there may
be evidence of systemic disease and the most common histological
subtype is mixed cellularity-HL as in our patient. There are no
previous reports of HL radiologically appearing identical to a
paraspinal plexiform neurofibroma in the literature.
REFERENCES
1. Akyuz C, Yalcin B, Atahan IL et al (2005) Intracranial involvement in
Hodgkin’s disease. Pediatr Hematol Oncol 22:589–596.
2. Balsari KR, Kadri PA, Husain M et al (2005) Malignant lymphoma of
the trigeminal region. Case illustration. J Neuro-oncol 73:279–280.
3. Cuttner J, Meyer R, Huang YP (1979) Intracerebral involvement in
Hodgkin’s disease: a report of 6 cases and review of the literature.
Cancer 43(4):1479–506.
4. Figueroa BE, Brown JR, Nscimento A et al (2004) Unusual sites of
Hodgkin’s lymphoma: CASE 2. Hodgkin’s lymphoma of the CNS
masquerading as meningioma. J Clin Oncol 22:4228–4230.
5. Gerstner RG, Abrey LE, Schiff D et al (2008) CNS Hodgkin
lymphoma. Blood 112:1658–1661.
6. Hirmiz K, Foyle A, Wilke D et al (2004) Intracranial presentation of
systemic Hodgkin’s disease. Leuk Lymphoma 45:1667–1671.
7. Koerbel A, Roser F, Psaras T et al (2005) Primary non-Hodhkin
lymphoma of the cranial nerves mimicking neurofibromatosis Type 2.
J Neurosurg 102:1166.
8. Morawa E, Ragam A, Sirota R et al (2007) Hodgkin’s lymphoma
involving the CNS. J Clin Oncol 25(21):3182.
9. Re D, Fuchs M, Schober T, Engert A et al (2007) CNS involvement in
Hodgkin’s lymphoma. J Clin Oncol 25:3181.
10. Sapopzink MD, Kaplan HS (1983) Intracranial Hodgkin’s disease. A
report of 12 cases and review of the literature. Cancer 52:1301–1307.
ABSTRACT
A 20 year old female presented with a 4 month history of right
upper limb pain and paraesthesias. She had no systemic symptoms
and no prior medical or family history of note. MRI revealed a
right-sided intradural extramedullary mass extending from C7-T1
and displacing the spinal cord. While awaiting surgery her symp-
toms progressed to involve the right lower limb. She was re-imaged
and the lesion now extended from C5 to T3 with spinal cord com-
pression at C7-T1. The radiological features and recent rapid
growth were felt to be in keeping with a large plexiform neurofi-
broma. The patient underwent emergency resection of the lesion
and pathology revealed Hodgkin’s Lymphoma (HL)—mixed cellu-
larity type. A mediastinal mass was identified on further imaging
and biopsy confirmed the diagnosis of HL-stage IV. The patient
is currently undergoing treatment with ABVD chemotherapy.
CNS-HL is extremely rare and may occur de novo or in association
with systemic disease. Lesions may be parenchymal or dural based
and are usually intracranial with an increased risk of CNS involve-
ment in HL-mixed-cellularity type as in our patient. This is the first
report in the literature of CNS-HL radiologically mimicking a
paraspinal plexiform neurofibroma.
Brain Pathology 20 (2010) 683–684