Shah, Usman, MD

Tseytlin, Daniel, DO

English

Lehigh Valley Health Network: LVHN Scholarly Works

Lehigh Valley Health NetworkLVHN Scholarly WorksDepartment of MedicineBurkitt Lymphoma Presenting as Left LateralRectus PalsyDaniel Tseytlin DOLehigh Valley Health Network, Daniel.Tseytlin@lvhn.orgUsman Shah MDUsman.Shah@lvhn.orgFollow this and additional works at: http://scholarlyworks.lvhn.org/medicinePart of the Hepatology Commons, Medical Sciences Commons, and the Oncology CommonsThis Poster is brought to you for free and open access by LVHN Scholarly Works. It has been accepted for inclusion in LVHN Scholarly Works by anauthorized administrator. For more information, please contact LibraryServices@lvhn.org.Published In/Presented AtTseytlin, D., & Shah, U. (April 29, 2014). Burkitt Llmphoma presenting as left lateral rectus palsy. Poster presented at: LVHN ResearchDay, Allentown PA.Lehigh Valley Health Network, Allentown, PennsylvaniaBurkitt Lymphoma Presenting as Left Lateral Rectus PalsyDaniel Tseytlin, DO and Usman Shah, MDREFERENCES:1.	 	Kased,	N	et	al.	Niederhuber:	Abeloff’s	Clinical	Oncology,	5th	Ed:	725.2.	 	Dunleavy,	Kieron	and	Wilson,	Wyndham	H.	Diagnosis	and	Treatment	of	Diffuse	Large	B-Cell	Lymphoma	and	Burkitt	Lymphoma.		Hoffman:	Hematology:	Basic	Principles	and	Practice,	6th	Ed:	1241-3.	3.	 	Wilson,	Wyndham	H.	et	al.	Niederhuber:	Abeloff’s	Clinical	Oncology,	5th	Ed:	2033,	2046.4.	 	Vaphiades,	MS	et	al.	J	Neuroopthalmol	2005;	25	(3):249-250.5.	 	N	Engl	J	Med.	1993;329:987-994.© 2014 Lehigh Valley Health NetworkCase:•	 	A	43	year	old	male	approximately	2	weeks	prior	to	initial	hospital	presentation	had	some	left	eye	pain	while	shoveling	snow.	The	following	day	he	woke	up	with	left	eye	pain	and	double	vision.	He	was	seen	by	his	primary	doctor	who	prescribed	him	eye	drops	to	reduce	the	pain,	but	still	had	double	vision.		On	physical	exam	he	was	found	to	have	impaired	left	eye	abduction	and	ptosis	consistent	with	palsy	of	cranial	nerves	VI	and	III,	respectively.		He	did	not	have	other	neurologic	pertinent	findings	such	as	slurred	speech,	weakness,	numbness,	facial	weakness.	He	was	evaluated	in	the	emergency	department.	Initially,	his	CT	scan	did	not	show	any	acute	stroke.	His	first	MRI	showed	no	evidence	of	acute	infarct	but	showed	abnormal	soft	tissue	fullness	in	the	cavernous	sinus,	especially	on	the	left	with	concern	for	lymphoma	or	leukemia	with	metastatic	disease	or	sarcoidosis.		Basic	laboratories	including	CBC	and	CMP	were	within	normal	limits	apart	from	a	mildly	elevated	AST	of	42.•	 	CT	scan	of	chest,	abdomen,	and	pelvis	to	uncover	the	source	of	cavernous	sinus	masses.		Results	showed	gastric	antral	wall	thickening	(Fig	1),	extensive	peritoneal	carcinomatosis,	reticulonodular	opacities	within	the	lungs,	a	pancreatic	tail	mass,	small	bilateral	renal	lesions	and	upper	abdominal	lymphadenopathy.	The	patient	was	initially	treated	with	palliative	brain	radiation	until	diagnosed	with	lymphoma.Figure 1. Gastric antral wall thickening on CT. Figure 2. Peritoneal carcinomatosis on CT.Table 1. Diferential Diagnosis of Visual DisturbancePresentation DifferentialUnilateral  Painful Vision Disturbance•	Corneal	abrasion	or	infection•		Acute	angle-closure	glaucoma•		Iritis/uveitis•		Optic	neuritis•		Endophthalmitis•		Cavernous	sinus	thrombosis/massDiplopia•		Thyroid	disease•		Cranial	Neuropathyy	(CN	III,	IV,	VI)•		Neuromuscular	disease	(Myasthenia	gravis)•		Botulism,	Miller-Fisher	Syndrome•		Encephalitis,	Basilar	meningitis•		Brianstem	Stroke•	 	EGD	and	biopsy	of	the	gastric	lesion	(Fig	3)	as	well	as	bone	marrow	biopsy	(Fig	4,	5)	of	the	iliac	spine.	Ki-67	index	was	very	high	>90%,	c-MYC	rearrangement	was	present,	and	his	disease	was	determined	to	be	stage	IVB.	Immunohistochemical	studies:	Discussion:•	 	Malignant	metastasis	to	the	central	nervous	system	affects	as	many	as	25%	of	patients	with	cancer.1•	 	Burkitt	lymphoma	(BL)	is	an	uncommon2	aggressive	B-cell	Lymphoma	composed	of	rapidly	proliferating	B	cells;	it	is	considered	the	most	highly	aggressive	NHL,	with	doubling	times	of	24	to	48	hours	in	some	cases.3•	 	BL	is	subdivided	into	Endemic,	Sporadic,	and	immunodeficiency-associated.•	 	Diagnosis	of	BL	requires	IHC	staining	for	CD20+,	CD10+	and	TDT-	with	delineation	from	DLBCL	by	negativity	for	BCL-2.	•	 	Translocation	between	MYC	on	chromosome	8	and	one	of	three	immmuoglobulin	chain	loci	results	in	overexpression	of	the	MYC	protein	causing	deregulation	of	cellular	growth.3•	 	Initial	presentation	of	BL	as	cavernous	sinus	syndrome	is	uncommon.4	•	 	Patient	underwent	treatment	with	HyperCVAD	with	intrathecal	Methotrexate	for	CNS	prophylaxis.•	 	The	patient’s	prognosis	was	calculated	using	the	International	Prognostic	Index	(IPI)	for	aggressive	lymphoma5.		The	IPI	is	a	model	for	predicting	outcome	based	on	patient	clinical	characteristics	prior	to	treatment.		Patients	are	stratified	according	to	risk	with	implications	for	treatment	modalities.		Those	at	high	risk	who	are	not	effectively	treated	with	current	regimens	may	benefit	from	new	clinical	trial	therapeutics.	There	are	5	adverse	prognostic	risk	factors	based	on	features	of	tumor	growth	potential,	patient	response	to	tumor,	and	ability	to	tolerate	therapy:	1)	age	>	60	yo,	2)	Ann	Arbor	Stage	III/IV,	3)	>	1	extranodal	site,	4)	Serum	LDH	>	normal,	5)	ECOG	performance	status	>/=	2.	(Score	range	0	-	5).•	 	The	patient’s	IPI	:	2	(Stage	IVB	and	>1	extranodal	site)	indicated	a	5	year	overall	survival	of	51%	and	complete	response	rate	of	67%.Table 2. Ann Arbor Staging System for Lymphomas2Stage Cotswold	Modification	of	Arbor	ClassificationI Involvement	of	a	single	LN	region	or	lymphoid	structure.IIInvolvement	of	two	or	more	LN	regions	on	the	same	side	of	the	diaphragm	(Mediastinum	is	considered	a	single	site,	but	the	hilar	LNs	are	considered	bilaterally);	the	number	of	atomic	sites	should	be	indicated	by	a	subscript	(e.g.,	II3).IIIInvolvement	of	LN	regions	on	both	sides	of	the	diaphragm:	III1	(with	or	without	involvement	of	splenic	hilar,	celiac,	or	portal	nodes)	and	III2	(with	involvement	of	paraaortic,	iliac,	and	mesenteric	nodes.IV Involvement	of	one	or	more	extranodal	sites	in	addition	to	a	site	for	which	the	designation	E	has	been	used.73%0.510.430.260%25%50%75%100%0-1 2 3 4-5OverallSurvival(OS)International Prognostic Index (IPI)International Prognostic Index (1993)Figure 6. Prognosis Variables for NHL Subtypes5CD20:	(+)CD10:	(+)BCL-6:	(+)Ki-67:	(+)	inapprox.	90%of	nucleiBCL-2:	(-/	dim	focal+)BCL-1:	(-)TdT:	(-)Figure 3. Gastric biopsy showing “starry sky” pattern.Figure 4. Bone marrow showing lymphocytic vacuolization.Figure 5. Bone marrow showing extensive necrosis. Risk Group IPI	Score%	Patients5-year	OSComplete	Response	RateLow 0-1 35% 73% 87%Low-Intermed 2 27% 51% 67%High-Intermed 3 22% 43% 55%High 4-5 16% 26% 44%

Burkitt Lymphoma Presenting as Left Lateral Rectus Palsy

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Burkitt Lymphoma Presenting as Left Lateral Rectus Palsy

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