Orbital plasmablastic lymphoma: a clinico-pathological correlation of a rare disease and review of literature

Figures & data

Figure 1 External photograph and axial CT scan of the same patient. External photograph of the patient showing right eye proptosis with periocular swelling and mild conjunctival congestion (A). CT scan, axial cut, of the same patient showing a large mass occupying the lateral quadrant of the orbit with erosion of the lateral wall and extension into the temporal fossa (B).

Abbreviation: CT, computerized tomography.

Figure 2 Microphotograph and immunohistochemical staining. Microphotograph showing atypical plasmacytoid tumors cells with abundant amphophilic cytoplasm and a large eccentric vesicular nucleus having a prominent nucleolus (HE × 400) (A). Immunohistochemical staining shows lack of immunoreactivity for CD20 (×400) (B). Strong membranous CD138 immunoreactivity (×400) (C). Strong Ki-67 immunoreactivity in almost all tumors cells (×400) (D).

Abbreviation: HE, hematoxylin-eosin.

Figure 3 External photograph and CT scan of the patient showing gross proptosis of the left eye with severe conjunctival chemosis, and a large mass lesion in the superior orbit. External photograph of the patient showing gross proptosis of the left eye with severe conjunctival chemosis (A). CT scan with sagittal reconstruction showing a large mass lesion in the superior orbit with extension into the frontal sinus and intracranial space (B).

Abbreviation: CT, computerized tomography.

Figure 4 External photograph of the patient showing periocular edema with total ptosis, skin induration, and blisters, as well as lip edema and blisters. External photograph of the patient showing left periocular edema with total ptosis, skin induration, and blisters (A). External photograph showing upper lip edema with blisters (B). CT scan coronal cut of the same patient shows a diffuse ill-defined mass involving the entire superior orbit with superolateral bony erosion and extension into the temporal fossa (C). CT scan with sagittal reconstruction showing diffuse mass involving the entire superior quadrant up to the apex with indentation of the globe and optic nerve stretch (D).

Abbreviation: CT, computerized tomography.

Table 1 Summary of clinical, radiological, and histopathologic features of ocular plasmablastic lymphomas reported in the literature

Parameter	Morley et al^Citation9		Barkhuysen et al^Citation12	Valenzuela et al^Citation10	Degnan et al^Citation11	Colomo et al^Citation13
	Case 1	Case 2				Case 1	Case 2
Age	40	49	50	41	43	37	55
Gender	Male	Male	Female	Female	Male	Male	Male
Race	West African	White	NA	Caucasian	Caucasian	NA	NA
Presentation	Nasal congestion and discharge, swelling over the right cheek. Decreased vision	Toothache for 2 months, intermittent nasal discharge, left eye prominence, binocular diplopia on left gaze	Proptosis, visual loss, ophthalmoplegia	Upper lid induration, K/c/o buccal PBL	Proptosis, headache, eye pain. Jaw abscess 3 months ago	NA	NA
Laterality	Right	Left	Left	Right	Left	NA	NA
BCVA	Blind	NA	Reduced	NA	NA	NA	NA
Pupils	Poorly reacting, mid dilated.	NA	Isocore. Direct reflex almost absent	NA	NA	NA	NA
IOP	33 mm Hg	NA	NA	NA	NA	NA	NA
Fundus	Macular folds	NA	NA	NA	NA	NA	NA
Proptosis	Present	Present	Present	NA	NA	NA	NA
Other findings	Chemosis	Swollen lids	Anesthesia of left maxillary sinus and ophthalmic branches of trigeminal nerve	Chemosis, complete ptosis.	NA	NA	NA
Globe motility	Restricted on right side	Restricted in all gazes	NA	Restricted	NA	NA	NA
CT scan	Soft tissue mass in the posterior one-third of the orbit with extension through the superior orbital fissure	Mass in the maxillary and ethmoid sinus with bony erosion and orbital mass	Abscess in the infratemporal fossa with extension to the posterior cranial fossa, maxillary sinus, and orbit. Skull base destruction	Mass in anterior orbit extending from preseptal tissues of the lid to the lacrimal gland involving the extraconal tissues	Retro-orbital mass with erosion of the sphenoid wing. Mass in the left temporal lobe and the right fossa of Rosenmuller	Primary maxillary origin with orbital extension	NA
Extranodal involvement	Liver	Absent	NA	Lungs, GIT, liver	NA	NA	NA
Lymphadenopathy	Absent	Absent	NA	Positive	Positive	Positive	Positive
Histomorphology	Plasmacytoid	Plasmacytoid	NA	Immunoblastic	Medium- to large-sized	Immunoblastic	Immunoblastic
Initial clinical diagnosis	Cellulitis	Cellulitis	Abscess	NA	NA	NA	NA
CD45	NA	Weak	Positive	Positive	NA	Negative	Negative
CD20	Negative	Negative	Negative	Negative	NA	Negative	Negative
CD138	Positive	Positive	Weak	Positive	Positive	Positive	Positive
CD79a	Negative	Negative	Weak	Negative	Weak	Negative	Negative
Vs38c	Positive	Positive	NA	NA	NA	NA	NA
Ki-67	Nearly 100%	Nearly 100%	NA	100%	NA	NA	NA
Other markers	NA	NA	CD56, CD30, CD10 are negative	Weak CD10 and Bcl6; negative CDE, bcl2 ALK-1, CD30, CD56	CD10 positive	NA	NA
EBV-LMP 1	Negative	NA	NA	NA	NA	NA	Negative
HHV-8	Negative	NA	NA	NA	NA	NA	NA
EBER-ISH	Positive	NA	Positive	Positive	NA	Negative	Positive
Bone marrow	Positive	Negative	Negative	Positive	NA	NA	NA
CSF	NA	Positive	NA	NA	NA	NA	NA
Stage	4	1	1	4	NA	NA	4
Dead/alive	Dead at 3 months	Dead at 3 months	Alive at 13 months	Death on second day	Alive at 10 months	NA	Death at 7 months
Treatment	CHOP followed by ERBT	CHOP × 10, DHAP, PmitCEBO	HAART, R-CHOP, 18 × IT-Mtx alternative with cytarabine	Was on HAART	Pegfilgastrim, EPOCH	NA	NA
Follow-up course	Lymphadenopathy, pleural effusion	Mesenteric and paraaortic adenopathy, nasal mass, frontal sinus extension	Skull base reossification and regression of lymphoma	Bleeding and ulceration from GI lymphoma	Size reduced in 2 months; 5 months remission. Lesion in retromandibular and cervical nodes, and new lesion in the fossa of Rosenmueller at 10 months	NA	NA

Abbreviations: NA, not applicable; K/c/o, known case of; PBL, plasmablastic lymphoma; BCVA, best corrected visual acuity; IOP, intraocular pressure; CT, computerized tomography; GIT, gastrointestinal tract; HIV, human immunodeficiency virus; ALK-1, activin receptor-like kinase-1; EBV-LMP 1, Epstein–Barr virus latent membrane protein 1; HHV-8, human herpesvirus-8; EBER-ISH, Epstein–Barr virus-encoded ribonucleic acid in situ hybridization; CSF, cerebrospinal fluid; CHOP, cyclophosphamide, hydroxydanorubicin, oncovin, and prednisone; ERBT, external beam radiotherapy; DHAP, dexamethasone, cytarabine, and cisplatin; PmitCEBO, prednisolone, mitoxantrone, cyclophosphamide, etoposide, bleomycin, and vincristine; HAART, highly active antiretroviral therapy; R-CHOP, rituximab-cyclophosphamide, hydroxydanorubicin, oncovin, and prednisone; IT-Mtx, intrathecal methotrexate; EPOCH, etoposide, doxorubicin, vincristine, prednisone, and cycolophosphamide; GI, gastrointestinal.

Table 2 Clinicopathological features of cases of plasmablastic lymphoma included in the present study

Feature	Present study
	Case 1	Case 2	Case 3
Age	45	45	48
Sex	Female	Male	Male
Race	Asian Indian	Asian Indian	Asian Indian
Clinical presentation	Proptosis, diminished vision	Swelling of upper lid	Trauma, redness, watering, pain
Laterality	Right	Left	Left
Vision	Finger counting	Light perception	Finger counting
Clinical examination	Proptosis, globe motility, restriction, macular folds	Chemosis	Periorbital swelling, upper lip swelling, mechanical ptosis, globe motility restriction, chemosis, proptosis
CT scan	Mass in superolateral orbit with bony erosion and spread to temporal fossa	Mass in anterior orbit with bony erosion and extension into cranial fossa, frontal, and ethmoid sinus	Orbital mass extending to apex with stretching of optic nerve
Systemic examination	No significant findings	Hepatomegaly, multiple liver deposits	Fever
HIV status	Negative	Positive	Positive
Clinical diagnosis	Lacrimal gland tumor, lymphoma	Lacrimal gland tumor, ES/PNET	Orbital cellulitis
Histopathology	Atypical plasmactoid cells	Atypical plasmacytoid cells	Atypical plasmacytoid cells
CD45	Negative	Weak	Negative
CD20	Negative	Negative	Negative
CD79a	N/P	N/P	N/P
CD138	Positive	Positive	Positive
VS38c	N/P	N/P	N/P
Ki-67	98%	Close to 100%	97%–98%
EBER-ISH	N/P	N/P	N/P
HHV-8	N/P	N/P	N/P
BM	Negative	Positive	Negative
Treatment	Could not be initiated	Refused	Adriamycin, vincristine, cyclophosphamide, HAART
F/U course	Rapid worsening	Lost on F/U	Burnt out tumor
Death	2 weeks	6 months	Alive at 6 months

Abbreviations: CT, computerized tomography; HIV, human immunodeficiency virus; ES/PNET, Ewing sarcoma/primitive neuroectodermal tumor; EBER-ISH, Epstein–Barr virus-encoded ribonucleic acid in situ hybridization; HHV-8, human herpesvirus-8; BM, bone marrow; F/U, follow-up; HAART, highly active antiretroviral therapy; N/P, not performed.

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