Orbital lympboma: blindness as a presenting symptom M.
Kuriakosc,
Depurtment
SUMMARY. bone erosion
J. Llewelyn,
A. Sugar
of Orul und Muxillofuciul
Surgery,
St Lawrence
llospitul,
Chepstow.
Gwent
A case of isolated orbital lymphoma presenting as blindness is reported. An unusual feature of is highlighted and the dilficulty in the histological interpretation is discussed.
INTRODUCTION The orbit is an uncommon extranodal site for a lymphoid tumour. In an autopsy series of 1269 patients with systemic lymphoma. it was discovered that only 1.3% showed orbital involvement (Kosenbcrg et al., 1961). In a large series of all orbital tumours, lymphoma accounted for only 1% (Calle et al., 1975) to 11% (Kennedy, 1984). Lymphoid tumours of the orbit generally present insidiously with moderate proptosis and mild motility disorders and only occasionally has a decrease in visual acuity been noted (Yco, 1982). Rlindness has been reported as a rarity (Harris, 1981). The following is a case history of a patient with an isolated orbital lymphoma presenting as blindness. Fig. 2- Computcrised tomography showing a high density mass at the apex of the right orbit extending to the ethmoid sinuses.
Case report A S-year-old steel worker was referred from the Departmcnt of Ophthalmology. for the management of a retrobulbar lesion. The patient had originally hccn seen by his General Medical Practitioner, having suddenly developed blurred vision and ‘shadowing’ over the complete field of vision of the right eye. An initial diagnosis of an inflammatory ischaemic right disc was made. and computcrised tomography was carried out to determine the cause. An ill defined soft tissue mass was found lying at the apex of the right orbit.
Fig.
I-
Ophthalmoplcgia
At presentation to the Department of Oral and Maxillofacial Surgery he complained of blurring of vision in the right eye for 2 months, progressing to total blindness. Past medical history indicated hypertension for which hc had been prescribed a combined antihypertensive preparation (Triamterene and Hydrochlorothiazide) and beta adrcnoccptor antagonist (Accbutolol). On examination (Fig. I), the right eye showed partial ptosis, complete ophthalmoplegia and loss of vision. There was absence of direct and consensual pupillary light reflex on the right side, but these were prcscnt on the left. Fundoscopic examination revealed inflammatory changes in the right disc. Exophthalmomctry showed proptosis of 5 mm on the right side. There were no abnormalities on a full physical systemic examination. Computerised tomography of the orbits (Fig. 2) showed a high density mass at the apex of the right orbit with a distinctive moulding pattern around the optic nerve and extraocular muscles. The mass showed extensive invasion medially into the ethmoid sinus and inferiorly into the infratemporal fossa, with a diffuse enlargement involving the lateral pterygoid muscle. Chest radiograph and routine haematological investigations were found to be normal. A biopsy was carried out through a medial canthal incision. The medial wall of the right orbit was explored
of right cyc on upward garc.
IOH
Orbital
and the lesion found extending into the ethmoid air cells. Multiple biopsies were taken at this point. Postoperatively. there was some return of vision to the right eye. Computcrised tomography of the thorax and abdomen did not reveal any significant lymphadenopathy. Following the diagnosis of a non-Hodgkin’s lymphoma of the right orhit, the patient was given multiple fractional radiotherapy with a 5 meV linear accelerator. The total dose was 2340 cGy lasting 8 days. lie developed a marked skin and mucosal reaction which settled in 3 weeks. The patient responded to this trcatmcnt with improvement in ophthalmoplegia. although vision remained poor. A further course of 2600 cGy was prescribed. and the ophthalmoplegia completely resolved with some improvcmcnt in vision. Except for atrophy of the right disc with telangiectasia there was no further sign of disease. Monitoring for tumour regression and potential rc‘currcncc was aided by further computerised tomography.
Ifisrol~~g~: I Iaematoxylin and cosin staining showed a cellular undifferentiated tumour infiltrating the collagen of the fibrous tissue of the orbit (Fig. 3). Morphologically the infiltrate was composed of large lymphocytes with a fairly uniform cytology with very few mltoses. The findings were suggcstivc of low grade malignant lymphoma. Immuno-
Fig. 3-A uniform. undiffcrcntiated cellular infiltracc into the orbital fibrous tissue (H & E. Original magnificationX50).
Fig. 4- Immunocytochcmic;II staining using common Icucocytc antigen showing pcriphcral stainin,c.of the cell clumps (Common Icucocytc antigen. Original m;lgnlfK;ltionX 12.5).
cytochemistry (Fig. 4).
was performed
to confirm
lymphonia
IhO
the diagnosis
DISCUSSION disorders, the ocular system divided into two distinct anatomical regions. In the conjuctivac and acini of lacrimal glands, presence of lymphoid tissue is a normal feature. This tissue can undergo reactive hvperplasia and often presents as localiscd disease (Stgclman & Jakobiec. 197X). However the prcscncc of lymphoid tissue within the orbit is considcrcd abnormal, and is often associated with systemic lymphoprolifcrative disorders (Jakobiec et al., 1979). Orbital lymphoid tumours present insidiously in elderly indtviduals, typically in the 5th and 6th decade. In those under 20 years, leukaemia should be considered (Jakobiec & Font, 1986). The lesion has a predilection for the superior orbit and frequently involves the lacrimal gland. If it has invaded the orbital septum. it may be palpable as a firm or rubbery mass. The diagnosis and staging of primary orbital lymphoma requires a full clinical examination. blood count. ESR and C’I’ scans of the thorax. abdomen and orbit. Bone marrow trephine and liver function tests may bc necessary to recognize systemic spread of tumour. The final confirmation requires histopathological and immunological examination of the biopsy spccimcns (Knowles & Jakobiec, 1982; Medelros & I Iarris, 1989). Extraorbital manifestation of lymphoprolifcrative disease is found in approximately 50% of orbital cases (Knowles & Jakobicc. 1980). If no systemic signs arc present the examination has to be repeated periodically to assess further developments. With computeriscd tomography (CT) and magnetic resonance imaging (MRI) it is possible to make a strongly presumptive diagnosis of these tumours (Flanders er af.. 1987). Cl‘ discloses a mass of low attenuation with characteristic moulding pattern along the orbital walls, globe. extra ocular muscles and optic nerve (Yco ef al., 1982). MRI shows a relatively hypointense image. Mills and Parsons (1989) showed that thoracic and abdominal Cl scanning was invaluable in the staging of orbital lymphoma. Unlike the case presented, even large lymphomas involving the whole orbit may not cause bony erosion. A multifocal lesion is suggestive of inflammatory pseudotumour. IAymphomas almost always are unifocal in nature. Histologically orbital lymphomas arc of two types; true lymphomas and those believed to be reactive lymphoid hyperplasia or pseudolymphoma. Knowles and Jakobiec (1980) pointed out the difficulty in charactcrisation of adncxal lymphoid lesions by conventional staining techniques. AS both types of the lesion show features of infiltration, the detcrmination of malignancy should be based on cytomorphology. The cytologic interpretation is further In lymphoprolifcrative
may
be
170
British Journal of Oral and Maxillofacial
Surgery
complicated by the fact that often the degree of cytologicdifferentiation isnot totallyclearcut. Indeed there may be a mixed population of benign and malignant cells. 50% of orbital lesions fall into this category (Knowles & Jakobiec, 1980), which is often referred to as ‘atypical lymphoid hyperplasia’. A more definitive differentiation can be accomplished by immunocytochemical study (Medciros & Harris, 1989). Benign reactive lymphoid hypcrplasia consists of mixtures of polyclonal T and B lymphocytes, whilst malignant lymphomas generally consists of a monoclonal population of lymphocytes, usually Blymphocytes. Specific lymphocyte surface markers can be used to identify the monoclonal nature of the cells, suggesting malignancy. Isolated orbital lymphoma respond well to radiotherapy. Long term follow up is necessary for the early diagnosis and management of contralateral orbital and systemic involvement of the lesion. The prognosis of the tumour depends on the histologic types. Jakobiec er al. (1979) reported a S-year mortality of 6% with reactive lymphoid hyperplasia, 19% with atypical hypcrplasia and 58% with malignant lymphoma.
Jakobiec. F. A.. McI.ean, J. P. & Font, R. L. (1979). Clinicopathologic characteristics of orbital lymphoid hyperplasia. Ophrhalmology, 86,948. Kennedy, R. E. (1984). An evaluation of 820 orbital cases.
7iansacrions of the American Ophthalmological Society, 82, 134. Knowles. I>. M. & Jakobiec, F. A. (1980). Orbital lymphoid neoplasms: A clinicopathologic study of 60 casts. Cuncer, 46,
S76. Knowles, I). M. & Jakobiec, F. A. (1982). Ocular adncxal lymphoid neoplasms; clinical, histopathological, electron microscopic and immunological characteristics. Ijuman Pathology, 13, 14X. Mcdeiros. L. J. dt flarris, N. 1.. (lYX9). Lymphoid infiltrations of the orbit and conjunctiva. A morphologic and immunophenotypic study of 99 cases. American Journul of
Surgical Purholqqy, 13,459. Mills. P. & Parsons, C. A. (1989). Primary orbital lymphoma; staging by computed tomographic scanning. Hrirish Jownal
of Rudiology, 62,287 CL735. Rosenberg, S. A.. Diamond. H. D., Jaslowitz. B. & Craven. 1.. F. (1961). Lymphosarcoma: A review of 1269 cases.
Medicine, 40,3
I.
Sigelman. J. & Jakobiec. F. A. (1978). Lymphoid lesions of the conjuctiva: relation of histopathology to clinical outcome.
Ophrhulmology, 85,818. Yeo, J. H.. Jakobicc. F. A.. Abbott,
G. F. & Trokcl. S. L. (1982). Combined clinical and computed tomographic diagnosis of orbital lymphoid tumour. American Journal oj
Ophthulmology, 94,235.
Acknowledgements The authors wish to thank Mr A. D. Holt Wilson. Consultant Ophthalmologist. Neville Hall Hospital, Ahergavcnnv. for referring the patient; Dr David Robinson, Consultant ‘Radiologist. Neville llall Ilospital for the radiological reporting and Dr D. Murray Walker. Reader in Oral Pathology, University of Wales College of Medicine. Cardiff for the histology.
The Authors M. Kuriakose BSc, BDS, FFDRCS, FDSRCS Clinical Assistant J. Llewelyn BDS, MBBS, FDSRCS, FRCS Registrar
A. Sugar BChD, FDSRCS
Keferences Callc, R., Zajdela. A.. Hayc, C. & Schlienger. P. (lY75). Primary malignant lymphoma of the orbit, the eye and its adnexa. Eye,-Ear. %ose and Throat Monograph.~. 54, 141. Flanders, A. E.. Esninosa. G. A.. Markicwicz. D. A. Xr I lowell. D. D. (1987). orbital lymphoma: role of fl and MRI.
Consultant Department of Oral and Maxillofacial Surgery Welsh Centre for Plastic. Burns and Maxillofacial St. Lawrence IIospital Chcpstow Gwent NPhSYX
Surgery
Radiological Clinic5 of Norrh America, 25,601. Ilarris.
Cr. J. (1981).
Annuk Jakohicc.
Bilateral
blindness due to orbital lymphoma.
of Ophrhalmoloxy, 13,427.
F. A. & Font, R. L: (lYX6).
Lymphoid
turnours. In: Vol. 3. Philadcphia & London:
Correspondence and requests for offprints to Mr J. Llcwelyn. Senior Registrar in Oral and Maxillofacial Surgery, The City Hospital, 51 Greenbank Drive, Edinburgh EHIO SSB
Onhthalmolo.~ic Parholoev: An Atlus and Tcxhwk, w. H. Spcn& Saunders.
(ed.).
pp&3-2711.
Paper received 19 March IYYI Accepted 2 January lYY2
Kuriakosc,
Depurtment
SUMMARY. bone erosion
J. Llewelyn,
A. Sugar
of Orul und Muxillofuciul
Surgery,
St Lawrence
llospitul,
Chepstow.
Gwent
A case of isolated orbital lymphoma presenting as blindness is reported. An unusual feature of is highlighted and the dilficulty in the histological interpretation is discussed.
INTRODUCTION The orbit is an uncommon extranodal site for a lymphoid tumour. In an autopsy series of 1269 patients with systemic lymphoma. it was discovered that only 1.3% showed orbital involvement (Kosenbcrg et al., 1961). In a large series of all orbital tumours, lymphoma accounted for only 1% (Calle et al., 1975) to 11% (Kennedy, 1984). Lymphoid tumours of the orbit generally present insidiously with moderate proptosis and mild motility disorders and only occasionally has a decrease in visual acuity been noted (Yco, 1982). Rlindness has been reported as a rarity (Harris, 1981). The following is a case history of a patient with an isolated orbital lymphoma presenting as blindness. Fig. 2- Computcrised tomography showing a high density mass at the apex of the right orbit extending to the ethmoid sinuses.
Case report A S-year-old steel worker was referred from the Departmcnt of Ophthalmology. for the management of a retrobulbar lesion. The patient had originally hccn seen by his General Medical Practitioner, having suddenly developed blurred vision and ‘shadowing’ over the complete field of vision of the right eye. An initial diagnosis of an inflammatory ischaemic right disc was made. and computcrised tomography was carried out to determine the cause. An ill defined soft tissue mass was found lying at the apex of the right orbit.
Fig.
I-
Ophthalmoplcgia
At presentation to the Department of Oral and Maxillofacial Surgery he complained of blurring of vision in the right eye for 2 months, progressing to total blindness. Past medical history indicated hypertension for which hc had been prescribed a combined antihypertensive preparation (Triamterene and Hydrochlorothiazide) and beta adrcnoccptor antagonist (Accbutolol). On examination (Fig. I), the right eye showed partial ptosis, complete ophthalmoplegia and loss of vision. There was absence of direct and consensual pupillary light reflex on the right side, but these were prcscnt on the left. Fundoscopic examination revealed inflammatory changes in the right disc. Exophthalmomctry showed proptosis of 5 mm on the right side. There were no abnormalities on a full physical systemic examination. Computerised tomography of the orbits (Fig. 2) showed a high density mass at the apex of the right orbit with a distinctive moulding pattern around the optic nerve and extraocular muscles. The mass showed extensive invasion medially into the ethmoid sinus and inferiorly into the infratemporal fossa, with a diffuse enlargement involving the lateral pterygoid muscle. Chest radiograph and routine haematological investigations were found to be normal. A biopsy was carried out through a medial canthal incision. The medial wall of the right orbit was explored
of right cyc on upward garc.
IOH
Orbital
and the lesion found extending into the ethmoid air cells. Multiple biopsies were taken at this point. Postoperatively. there was some return of vision to the right eye. Computcrised tomography of the thorax and abdomen did not reveal any significant lymphadenopathy. Following the diagnosis of a non-Hodgkin’s lymphoma of the right orhit, the patient was given multiple fractional radiotherapy with a 5 meV linear accelerator. The total dose was 2340 cGy lasting 8 days. lie developed a marked skin and mucosal reaction which settled in 3 weeks. The patient responded to this trcatmcnt with improvement in ophthalmoplegia. although vision remained poor. A further course of 2600 cGy was prescribed. and the ophthalmoplegia completely resolved with some improvcmcnt in vision. Except for atrophy of the right disc with telangiectasia there was no further sign of disease. Monitoring for tumour regression and potential rc‘currcncc was aided by further computerised tomography.
Ifisrol~~g~: I Iaematoxylin and cosin staining showed a cellular undifferentiated tumour infiltrating the collagen of the fibrous tissue of the orbit (Fig. 3). Morphologically the infiltrate was composed of large lymphocytes with a fairly uniform cytology with very few mltoses. The findings were suggcstivc of low grade malignant lymphoma. Immuno-
Fig. 3-A uniform. undiffcrcntiated cellular infiltracc into the orbital fibrous tissue (H & E. Original magnificationX50).
Fig. 4- Immunocytochcmic;II staining using common Icucocytc antigen showing pcriphcral stainin,c.of the cell clumps (Common Icucocytc antigen. Original m;lgnlfK;ltionX 12.5).
cytochemistry (Fig. 4).
was performed
to confirm
lymphonia
IhO
the diagnosis
DISCUSSION disorders, the ocular system divided into two distinct anatomical regions. In the conjuctivac and acini of lacrimal glands, presence of lymphoid tissue is a normal feature. This tissue can undergo reactive hvperplasia and often presents as localiscd disease (Stgclman & Jakobiec. 197X). However the prcscncc of lymphoid tissue within the orbit is considcrcd abnormal, and is often associated with systemic lymphoprolifcrative disorders (Jakobiec et al., 1979). Orbital lymphoid tumours present insidiously in elderly indtviduals, typically in the 5th and 6th decade. In those under 20 years, leukaemia should be considered (Jakobiec & Font, 1986). The lesion has a predilection for the superior orbit and frequently involves the lacrimal gland. If it has invaded the orbital septum. it may be palpable as a firm or rubbery mass. The diagnosis and staging of primary orbital lymphoma requires a full clinical examination. blood count. ESR and C’I’ scans of the thorax. abdomen and orbit. Bone marrow trephine and liver function tests may bc necessary to recognize systemic spread of tumour. The final confirmation requires histopathological and immunological examination of the biopsy spccimcns (Knowles & Jakobiec, 1982; Medelros & I Iarris, 1989). Extraorbital manifestation of lymphoprolifcrative disease is found in approximately 50% of orbital cases (Knowles & Jakobicc. 1980). If no systemic signs arc present the examination has to be repeated periodically to assess further developments. With computeriscd tomography (CT) and magnetic resonance imaging (MRI) it is possible to make a strongly presumptive diagnosis of these tumours (Flanders er af.. 1987). Cl‘ discloses a mass of low attenuation with characteristic moulding pattern along the orbital walls, globe. extra ocular muscles and optic nerve (Yco ef al., 1982). MRI shows a relatively hypointense image. Mills and Parsons (1989) showed that thoracic and abdominal Cl scanning was invaluable in the staging of orbital lymphoma. Unlike the case presented, even large lymphomas involving the whole orbit may not cause bony erosion. A multifocal lesion is suggestive of inflammatory pseudotumour. IAymphomas almost always are unifocal in nature. Histologically orbital lymphomas arc of two types; true lymphomas and those believed to be reactive lymphoid hyperplasia or pseudolymphoma. Knowles and Jakobiec (1980) pointed out the difficulty in charactcrisation of adncxal lymphoid lesions by conventional staining techniques. AS both types of the lesion show features of infiltration, the detcrmination of malignancy should be based on cytomorphology. The cytologic interpretation is further In lymphoprolifcrative
may
be
170
British Journal of Oral and Maxillofacial
Surgery
complicated by the fact that often the degree of cytologicdifferentiation isnot totallyclearcut. Indeed there may be a mixed population of benign and malignant cells. 50% of orbital lesions fall into this category (Knowles & Jakobiec, 1980), which is often referred to as ‘atypical lymphoid hyperplasia’. A more definitive differentiation can be accomplished by immunocytochemical study (Medciros & Harris, 1989). Benign reactive lymphoid hypcrplasia consists of mixtures of polyclonal T and B lymphocytes, whilst malignant lymphomas generally consists of a monoclonal population of lymphocytes, usually Blymphocytes. Specific lymphocyte surface markers can be used to identify the monoclonal nature of the cells, suggesting malignancy. Isolated orbital lymphoma respond well to radiotherapy. Long term follow up is necessary for the early diagnosis and management of contralateral orbital and systemic involvement of the lesion. The prognosis of the tumour depends on the histologic types. Jakobiec er al. (1979) reported a S-year mortality of 6% with reactive lymphoid hyperplasia, 19% with atypical hypcrplasia and 58% with malignant lymphoma.
Jakobiec. F. A.. McI.ean, J. P. & Font, R. L. (1979). Clinicopathologic characteristics of orbital lymphoid hyperplasia. Ophrhalmology, 86,948. Kennedy, R. E. (1984). An evaluation of 820 orbital cases.
7iansacrions of the American Ophthalmological Society, 82, 134. Knowles. I>. M. & Jakobiec, F. A. (1980). Orbital lymphoid neoplasms: A clinicopathologic study of 60 casts. Cuncer, 46,
S76. Knowles, I). M. & Jakobiec, F. A. (1982). Ocular adncxal lymphoid neoplasms; clinical, histopathological, electron microscopic and immunological characteristics. Ijuman Pathology, 13, 14X. Mcdeiros. L. J. dt flarris, N. 1.. (lYX9). Lymphoid infiltrations of the orbit and conjunctiva. A morphologic and immunophenotypic study of 99 cases. American Journul of
Surgical Purholqqy, 13,459. Mills. P. & Parsons, C. A. (1989). Primary orbital lymphoma; staging by computed tomographic scanning. Hrirish Jownal
of Rudiology, 62,287 CL735. Rosenberg, S. A.. Diamond. H. D., Jaslowitz. B. & Craven. 1.. F. (1961). Lymphosarcoma: A review of 1269 cases.
Medicine, 40,3
I.
Sigelman. J. & Jakobiec. F. A. (1978). Lymphoid lesions of the conjuctiva: relation of histopathology to clinical outcome.
Ophrhulmology, 85,818. Yeo, J. H.. Jakobicc. F. A.. Abbott,
G. F. & Trokcl. S. L. (1982). Combined clinical and computed tomographic diagnosis of orbital lymphoid tumour. American Journal oj
Ophthulmology, 94,235.
Acknowledgements The authors wish to thank Mr A. D. Holt Wilson. Consultant Ophthalmologist. Neville Hall Hospital, Ahergavcnnv. for referring the patient; Dr David Robinson, Consultant ‘Radiologist. Neville llall Ilospital for the radiological reporting and Dr D. Murray Walker. Reader in Oral Pathology, University of Wales College of Medicine. Cardiff for the histology.
The Authors M. Kuriakose BSc, BDS, FFDRCS, FDSRCS Clinical Assistant J. Llewelyn BDS, MBBS, FDSRCS, FRCS Registrar
A. Sugar BChD, FDSRCS
Keferences Callc, R., Zajdela. A.. Hayc, C. & Schlienger. P. (lY75). Primary malignant lymphoma of the orbit, the eye and its adnexa. Eye,-Ear. %ose and Throat Monograph.~. 54, 141. Flanders, A. E.. Esninosa. G. A.. Markicwicz. D. A. Xr I lowell. D. D. (1987). orbital lymphoma: role of fl and MRI.
Consultant Department of Oral and Maxillofacial Surgery Welsh Centre for Plastic. Burns and Maxillofacial St. Lawrence IIospital Chcpstow Gwent NPhSYX
Surgery
Radiological Clinic5 of Norrh America, 25,601. Ilarris.
Cr. J. (1981).
Annuk Jakohicc.
Bilateral
blindness due to orbital lymphoma.
of Ophrhalmoloxy, 13,427.
F. A. & Font, R. L: (lYX6).
Lymphoid
turnours. In: Vol. 3. Philadcphia & London:
Correspondence and requests for offprints to Mr J. Llcwelyn. Senior Registrar in Oral and Maxillofacial Surgery, The City Hospital, 51 Greenbank Drive, Edinburgh EHIO SSB
Onhthalmolo.~ic Parholoev: An Atlus and Tcxhwk, w. H. Spcn& Saunders.
(ed.).
pp&3-2711.
Paper received 19 March IYYI Accepted 2 January lYY2
