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C A S E O F M O N T H F E B R U A R Y 2 0 1 0 bpa_382 683..684

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 fora 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 andbilateral 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

centered in andexpanding theexit foramina andindentingthe 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 cmsand furthermultiple 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 infiltratewith 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 1.

Figure 2.

Figure 3.

Figure 4.

doi:10.1111/j.1750-3639.2010.00382.x

683Brain Pathology 20 (2010) 683–684

© 2010 The Authors; Journal Compilation © 2010 International Society of Neuropathology

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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 approximately25 years (6).There is a reported increasedrisk 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 involvementis rare in HL there is no

consensus on therapy although various combinations of surgery,

chemotherapy and radiation therapy were used in the recent seriesreported 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

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 aright-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.

Correspondence

684 Brain Pathology 20 (2010) 683–684

© 2010 The Authors; Journal Compilation © 2010 International Society of Neuropathology