VOLUME 23 䡠 NUMBER 18 䡠 JUNE 20 2005

JOURNAL OF CLINICAL ONCOLOGY D I A G N O S I S I N O N C O L O G Y

CNS Manifestations of Malignancies

Conjunctival Relapse of Acute
CASE 1.
Lymphoblastic Leukemia Heralding
Pituitary and CNS Disease
A 64-year-old man presented with acute lymphoblastic
leukemia (ALL) and achieved complete remission with che-
motherapy (doxorubicin, vincristine, prednisolone, and cy-
clophosphamide). He completed consolidation therapy with
cranial radiotherapy, intrathecal methotrexate (MTX 12 mg ⫻
6).1 One year later, while on oral maintenance, he presented
with bilateral blurring of vision (visual acuity, 0.2), generalized
bone pain, and weight loss. Physical examination showed bi-
lateral lower conjunctiva fleshy “salmon patch” infiltrates,
which were biopsied (Fig 1A, arrows). Slit lamp and fundo-
scopic examinations were normal. A complete blood picture
was normal, but a bone marrow biopsy and lumbar puncture
showed blast cells. This was followed by sudden development
of diabetes insipidus (daily urine output, 6 L) and panhypopi-
tuitarism (cortisol, 16nmol/L; thyroxine 7.2 pmol/L; thyroid-
stimulating hormone, follicle-stimulating hormone, leutenizing
hormone, adrenocorticotropic hormone, all undetectable).
He was treated with intrathecal MTX (12 mg) and systemic
MOD chemotherapy (MTX, 1g; vincristine, 2 mg; and dexa-
methasone, 40 mg).2 This resulted in overnight resolution of
the conjunctival infiltrate (Fig 1B) and bilateral visual recovery
(acuity, 0.9). The conjunctival histology showed extensive

Fig 1.

Journal of Clinical Oncology, Vol 23, No 18 (June 20), 2005: pp 4225-4230 4225

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 Weil, Lonser, and Quezado
subconjunctival lymphoblast infiltration (Fig 1C, arrows).
Magnetic imaging scanning showed leukemic infiltration of
the pituitary sella and clivus, abutting the optic chiasm (Fig 1D
arrow), together with deposits in the spleen and right kidney.
Three more courses of intrathecal MTX and MOD were ad-
ministered, with pituitary irradiation and thyroxine, cortisol,
and desmopressin acetate replacement, and the patient
achieved a good partial remission.
Conjunctival relapse of ALL is reported rarely for both
pediatric3 and adult patients.4 Most reported cases presented
as isolated asymptomatic masses and were treated with radio-
therapy. This is the first description of concomitant conjunc-
tival, cerebral and pituitary relapse of ALL. Anatomically, the
striking conjunctival infiltrate is unlikely to cause the visual
impairment. Rather, the CSF and pituitary disease suggested
direct ALL involvement of the optic nerve and tracts. Visual
complaints in ALL patients often herald CNS disease and re-
quired urgent lumbar puncture and imaging. Compared with
conjunctival radiotherapy, the use of systemic chemotherapy
carried the advantage of speed and ease of administration, and
also covers inevitable systemic disease. Despite extensive dis-
ease, our case showed good symptomatic response. The choice
of MTX, which has therapeutic CSF5 and eye globe6 penetrat-
ing ability, may also be of benefit.
Charmaine Hon and Ricky W. Law
Departments of Ophthalmology and Visual Sciences, Prince of Wales
Hospital, Hong Kong
■ ■ ■
CASE 2. Skull and Brain Metastasis From
Tibial Osteosarcoma
A 26-year-old man presented with a history of right-leg
pain, first with exercise, then, over the course of several
weeks, at rest. Physical examination revealed a 3 ⫻ 2 cm
firm mass along the tibia of the right leg and plain x-rays
suggested an osteolytic lesion. Simultaneously, chest x-ray
revealed multiple small nodules in both lungs suggestive of
metastatic cancer. Needle biopsy of the tibial lesion demon-
strated an osteosarcoma (OS). The patient was treated with
two cycles of methotrexate and cisplatin, followed by resec-
tion of the right tibial lesion and cadaveric bone graft, then
video-assisted thoracoscopic wedge resection of one re-
maining left pulmonary tumor. He was then treated with
cisplatin, methotrexate, etoposide, and ifosfamide for 36
weeks. The patient had an asymptomatic recurrence in the
left femur 18 months after initial presentation treated with
resection of the femoral neck and left hip arthrodesis, from
which he recovered well. Methotrexate and cisplatin were
resumed for 12 months, with no evidence of recurrent disease.
The patient did well until 3 years after initial presentation, at
which point he bumped his head firmly against a car door.
Later that day he noticed a 3 ⫻ 3 cm swelling under his
4226
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Copyright © 2005 American Society of Clinical Oncology. All rights reserved.
Tony W. Shek
Department of Pathology, Queen Mary Hospital, Hong Kong
Wing Y. Au
Medicine, Queen Mary Hospital, Hong Kong
© 2005 by American Society of Clinical Oncology
Authors’ Disclosures of Potential
Conflicts of Interest
The authors indicated no potential conflicts of interest.
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MOAD: A non-anthracycline containing regimen. Leukemia 7:1236-1241,
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3. Campagnoli MF, Parodi E, Linari A, et al: Conjunctival mass: An unusual
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4. Cook BE Jr, Bartley GB: Acute lymphoblastic leukemia manifesting in
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DOI: 10.1200/JCO.2005.04.133
scalp at that location. It was soft, minimally tender, and
moved with the scalp. Two weeks later, the scalp mass
had not resolved and he sought medical attention. On
examination, it measured 4 ⫻ 4 cm and was elevated 2 cm
above the scalp contour. The patient’s neurological ex-
amination was normal. Needle biopsy of the lesion was
inconclusive. Neuroimaging imaging (Fig 1) demon-
strated a 6 ⫻ 6 ⫻ 3 cm extra-axial mass with extension
through the inner and outer tables of the skull, with
compression of the underlying brain. A lateral skull x-ray
shows thinning of calvarium (Fig 1A, arrow) and a
soft tissue mass above. On T1-weighted, gadolinium-
enhanced magnetic resonance (MR) images (Fig 1B, ax-
ial; Fig 1C, coronal view) one sees the full extent of the
mass, which goes beneath the scalp, penetrates the dura,
and compresses the brain; the small bars at the bottom of
the frames of Figure 1B and C are 1-cm markers. Chest
x-ray demonstrated new bilateral pulmonary nodules
that were felt to be surgically accessible. The patient
underwent craniotomy and gross total resection of the
tumor, which arose from the calvarium and elevated but
did not invade the scalp. A 1 to 2 cm rim of normal bone
was removed circumferentially around the bone tumor.
The tumor invaded through the dura and compressed the
JOURNAL OF CLINICAL ONCOLOGY