Ординатура / Офтальмология / Английские материалы / Surgical Atlas of Orbital Diseases_Mallajosyula_2009

Contrast enhancement: Whenever I suspect a vascular lesion or a tumor, I ask for contrast studies. Contrast enhancement shows that the tumor is very vascular. It differentiates a cavernous hemangioma

from a hemangioendothelioma. When the tumors are very strongly enhance on contrast as in the Figures 3.64A to D, I will keep a unit of blood reserved at the time of surgery.

BIBLIOGRAPHY

1.Abou-Rayyah Y, Rose GE, Konrad H, Chawla SJ, Moseley IF. Clinical, radiological and pathological examination of periocular dermoid cysts. Eye. 2002;16(5):507-12.

2.Arger PH The radiologic evaluation of unilateral proptosis. CRC Crit Rev Clin Radiol Nucl Med. 1974;5(1):43-67.

3.Char DH, Sobel D, Kelly WM, Kjos BO, Norman D Magnetic resonance scanning in orbital tumor diagnosis. Ophthalmology. 1985;92(10):1305-10.

4.Forbes G. Radiologic evaluation of orbital tumors. Clin Neurosurg. 1985;32:474-513.

5.Forbes GS, Sheedy PF 2nd, Waller RR. Orbital tumors evaluated by computed tomography. Radiology. 1980;136(1):101-11.

6.Hilal SK, Trokel SL. Computerized tomography of the orbit using thin sections. Semin Roentgenol. 1977;12(2):137-47.

7.Klöppel R, Schulz HG, Ballin R, Lommatzsch P Value of computed tomography in orbital tumors. Radiol Diagn (Berl). 1985;26(6):745-52.

8.Kokemueller H, Zizelmann C, Tavassol F, Paling T, Gellrich NC A comprehensive approach to objective quantification of orbital dimensions. J Oral Maxillofac Surg. 2008;66(2): 401-7.

9.Trokel SL, Hilal SK Recognition and differential diagnosis of enlarged extraocular muscles in computed tomography. Am J Ophthalmol. 1979;87(4):503-12.

10.Urbanik A, Chojnacka I, Herman-Sucharska I, Jele?ska I, Brzozowska-Czarnek A. Computed tomography images of selected retrobulbar orbit tumors. Przegl Lek. 2000;57(6):327-9.

11.Vignaud J, Hasso AN, Lasjaunias P, Clay C Orbital vascular anatomy and embryology. Radiology. 1974 ;111(3):617-26.

12.Wackenheim A, van Damme W, Kosmann P, Bittighoffer B. Computed tomography in ophthalmology. Density changes with orbital lesions. Neuroradiology. 1977;13(3):135-8.

13.Wei R, Cai J, Wang H, Tao X, Zhu H, Zhou H. Analysis of MRI and CT manifestations of paranasal sinuses and orbitocranial disorders with secondary exophthalmos. Zhonghua Yan Ke Za Zhi. 1999;35(3):200-2, 12.

14.Wende S, Aulich A, Nover A, Lanksch W, Kazner E, Steinhoff H, Meese W, Lange S, Grumme T. Computed tomography or orbital lesions. A cooperative study of 210 cases. Neuroradiology. 1977;13(3):123-34.

15.Wende S, Kazner E, Grumme T The diagnostic value of computed tomography in orbital diseases. A cooperative study of 520 cases. Neurosurg Rev. 1980;3(1):43-9.

Cytologic diagnosis of lesions is one of the mainstay of diagnosis for lesions affecting various organs of the body. This speciality has made tremendous impact even in ophthalmic pathology, an upcoming subspeciality, which has been referred by Fredrick Jakobiec as “queen of subspecialities of Ophthlmology.” The techniques of obtaining specimens of ocular cytology have undergone much refinement and with increasing reports of larger series of cases, the learning curve has been crossed by many experts. In general, the techniques used for orbital lesions include: Fine needle aspiration of palpebral lesions, squash and imprint cytology of fresh tissue for rapid intraoperative diagnosis.1-6 The cytology specimens could also be subjected to recently emerging molecular tools of diagnosis,7-8 thus aiding in patient management.

Fine Needle Aspiration/Sampling Technique

The procedure for FNAC at any site in the body is the same. The procedure can be done by the pathologist or the surgeon, directly under vision or under guidance of CT. Usually there is no need of any local anesthesia injection by this technique, except in children where a general anesthesia may be preferred. The technique of obtaining the material could be a “sampling technique” wherein a 23 /24 guage needle is introduced into the lesion and pushed in various directions within the lesion and gently withdrawn.9 By the capillary action, the cells are drawn into the needle. This technique has also been applied to ocular cytology.3,6 The advantages of this technique are: It is easy, simple, less hemorrhagic and causes less apprehension to the

patient. The cytologic material obtained by this method is usually adequate with minimum amount of hemorrhage. A few disadvantages of fine needle aspiration in general include inadequate material, hemorrhagic aspirate, bleeding at the site, especially for highly vascular lesions. At our center, FNAC has yielded diagnostic yield in more than 90% of cases. Tijl et al.10 reported the diagnostic yield of orbital FNAB combined with clinical and radiological features as 80%. Very rare potential complications include globe penetration, retrobulbar hemorrhage, diplopia and ptosis.11

Intraoperative-operative Diagnosis by Squash and Imprint Cytology

There is often a need for a reliable intraoperative diagnosis, specifically in situations where a definitive preoperative tissue diagnosis is lacking and where the tissue diagnosis is likely to influence the immediate surgical management.12 The established methods of intraoperative diagnosis include frozen section diagnosis and intraoperative cytologic diagnosis, each of which has its own merits and demerits.

Squash or Imprint Cytology

The utility of imprint cytology in eye lesions was first described by Fuchs for uveal melanoma in 1988.13 Imprint cytology of fresh unfixed tissue specimens and squash cytology of central nervous system lesions have been extensively used in the last few decades, but rarely applied to ophthalmic pathology practice. The main indications for rapid intraoperative diagnosis are: a) Infiltrative lesions, suspected

86 Surgical Atlas of Orbital Diseases

malignant lesions or deeply located lesions where a preoperative tissue diagnosis is not available; b) Where there is a discrepancy between a preoperative clinical diagnosis and the intraoperative findings and: c) Unusual clinical presentations with diagnostic dilemma.

Fresh unfixed tissue obtained at the time of diagnostic or excision biopsy can be used for making squash preparation and impressions on glass slides. The procedure for making squash or imprint smears is usually based on the size, shape, consistency and crushable properties of the tissue submitted. For soft, easy to spread tissues, tiny bit of the fresh tissue is placed between two clean glass slides and gently drawn apart. For large firm specimens, the imprint smears are prepared by touching the freshly cut surface of the lesion with clean slides, avoiding smearing to retain cell morphology. If the surface is covered with blood or exudates, more number of smears is made, after gently wiping the surface clean. It is preferable to make a minimum of three slides for each case. It is advisable to preserve extra unstained smears for further tests like immunocytochemsitry or for any molecular studies in future.

These smears are either alcohol fixed for rapid hematoxylin and eosin staining, or fixed by air-drying for Diff-quick staining. A provisional cytologic diagnosis can be made by the pathologist based on the cellular and architectural features seen on smears prepared from either or both techniques.

CASE ILLUSTRATIONS

Case 1

A 23-year-old female presented with swelling of left upper lid for 2 months with occasional diplopia. Examination showed non-axial proptosis with downward displacement of globe and fullness of the upper lid sulcus (Figure 4.1A). There was a firm, slightly tender nodular mass in the superolateral orbit. There was limited abduction of the left eye. CT scan shows a soft tissue mass in superolateral orbit, which could not be seen separate from the lateral rectus muscle (Figure 4.1B). Incision biopsy was done. Squash and imprint preparation shows a polymorphous population of inflammatory cells consisting of lymphocytes, neutrophils and also a few eosinophils (Figure 4.1C), suggestive of a nonspecific

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Figures 4.1A to C: (A) Photograph shows the downward displacement of the left eye and fullness of the upper lid sulcus due to the presence of a lacrimal gland mass (B) CT scan (coronal cut) shows a well-circumscribed, homogenous, extraconal, soft tissue mass in the superolateral quadrant of mid-orbit, which is not seen separate from the lateral rectus muscle. There is no orbital fat streaking.

(C) The squash smear shows a polymorphic population of cells consisting of lymphocytes, neutrophils, eosinophils. (Giemsa, x 500)

orbital inflammatory disease. Permanent sections confirmed the diagnosis.

Case 2

A 12-year-old female presented with swelling of left upper lid and cheek for 1 month. There was no history of cough, hemoptysis, fever or weight loss. There was a soft, non-tender swelling in the superior orbit (Figure 4.2A). Preauriclar and submandibular lymph nodes of the left side were enlarged, firm, nontender and mobile. CT scan showed a soft tissue mass in superior orbit with bone destruction. Clinically, a provisional diagnosis of adenoid cystic carcinoma of the lacrimal gland was made. FNAC from the orbital lesion and preauricular lymph node showed lymphocytic infiltrates, epithelioid granulomas and few giant cells (Figure 4.2B). Extensive necrosis was seen in some areas. Acid-fast bacilli staining of the smears showed a few bacilli.

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Figures 4.2A and B: (A) Photograph shows narrowed palpebral aperture and fullness of left superior sulcus due to a soft mass in the superior orbit. (B) The squash smear show cluster of epitheloid cells with slipper shaped nuclei (hematoxylon and eosin, × 200)

Role of Cytology in Orbital Lesions 87

Case 3

A 70-year-old female presented with history of painful vision loss with drooping of the upper eyelid of the right eye for 1 month. Examination showed complete external ophthalmoplegia with ptosis, dilated and fixed pupil and optic atrophy (Figure 4.3A). She was a diabetic on irregular treatment. CT scan showed a large, elongated cystic mass in posterior, superomedial orbit straddling intraconal and extraconal spaces and lying close to the optic nerve (Figure 4.3B). Differential diagnoses were: a parasitic cyst or cystic degeneration in a solid tumor. Preoperatively, an abscess was found, the wall was excised after draining the contents. Squash and imprint preparation of the walls showed chronic inflammatory cells and fibrosis with fungal filaments (Figure 4.3C). Cultures of the specimen confirmed aspergillosis.

Case 4

An 18-month-old male child presented with right sided non-axial proptosis and temporal fossa fullness

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Figures 4.3A to C: (A) Photograph shows complete ptosis on right side. The patient had total external ophthalmoplegia with optic atrophy in the right eye. (B) Axial CT scan shows an elongated mass with cystic changes in the superomedial quadrant of the right orbit close to the optic nerve and soft tissue mass or mucosal thickening in adjoining ethmoidal sinuses. (C) the smears shows prominent branching and septate fungal filaments with inflammatory cells in the background (Giemsa, × 1000)

of 15 days duration (Figure 4.4A). Peripheral blood smear was normal. CT scan showed an extraconal, well circumscribed soft tissue mass temporally with destruction of the greater wing of sphenoid and extending into temporal fossa and middle cranial fossa (Figure 4.4B). Incision biopsy was done from the temporal fossa. The squash and imprint smears showed cellular infiltrates consisting of neutrophils, eosinophils, plasma cells and giant cells. In addition there were large cells with moderate amount of cytoplasm and large vesicular nucleus with prominent grooves and folds (Figure 4.4C). Frequent multinucleated giant cells are seen. Tingible body macrophages and histiocytes with phagocytic activity were noted. Based on the above features on squash and imprint smears, a provisional diagnosis of eosinophilic granuloma was given which was confirmed on histology sections. Intralesional triamcinolone was injected and the lesion resolved completely. Systemic workup included USG abdomen, skeletal survey, bone marrow biopsy and liver function tests were normal. Bone remodeling was complete at 6 months.

Case 5

A 45-year-old male presented with progressive protrusion of the left eye for 3 months (Figure 4.5A)

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Figures 4.4A to C: (A) Photograph shows swelling and erythema of the right upper lid and fullness of the temporal fossa. (B) CT scan

(axial cut) shows a homogenous, well-circumscribed, low intensity soft tissue mass with destruction of the greater wing of sphenoid.

The mass involves extraconal lateral orbit, temporal fossa and middle cranial fossa. (C) The squash smears are cellular with polymorphic population of cells with multinucleated giant cells, neutrophils, eosinophils and histiocytes with prominent nuclear grooves and cleaves (hematoxylin and eosin, × 1000).

and non-tender, smooth, firm subcutaneous nodules in left breast, upper abdomen and thigh for 2 months. CT scan showed a well defined soft tissue intraconal and extraconal mass in the left orbit (Figure 4.5B). He had a previous episode of proptosis on the same side ten years ago, which was diagnosed histopathologically as reactive lymphoid hyperplasia previously, which responded to local radiotherapy after initial poor response to systemic steroids. Incision biopsy performed on the orbital mass two months ago showed sinus histiocytosis.

FNAC was performed from the breast and abdominal nodule at this presentation and smears showed high cellularity with a polymorphous population of cells consisting of lymphocytes, plasma cells, histiocytes and neutrophils. A large number of histiocytic giant cells with abundant cytoplasm extended by the presence of intracytoplasmic plasma cells, lymphocytes and occasional neutrophils were present(Figure 4.5C). This feature, described as emperipolesis, confirmed that the subcutaneous

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Role of Cytology in Orbital Lesions 89

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Figures 4.5A to C: (A) Photograph shows severe chemosis and non-axial proptosis of the left eye. (B). CT scan (axial cut) shows a well defined irregular hypodense soft tissue mass in intraconal and extraconal spaces in the left orbit. (C) The smears show large multinucleated giant cells with multiple lymphocytes, plasma cells within the cytoplasm of the histiocytes-called as emperipolesis, a pathognomonic feature of Rosai-Dorfman disease ( Giemsa, × 500)

nodules were a part of multifocal Rosai-Dorfman Disease.

Case 6

A 48-year-old male presented with a swelling on the surface of the right eye with prominence of that eye for 6 months. Examination showed a pink fleshy conjunctival mass with intrinsic vessels (Figure 4.6A), axial proptosis and limitation of eye movements. CT scan showed a soft tissue mass moulding around the globe and extending into the conjunctiva (Figure 4.6B). Incision biopsy was done from the conjunctiva. Squash and imprint smears were cellular with a monomorphic population of lymphoid cells. These cells showed scant rim of cytoplasm and a round nucleus with moderately coarse chromatin and small nucleoli (Figure 4.6C). The biopsy confirmed a diffuse large cell lymphoma, immuno-phenotyping suggested a B cell lymphoma. Systemic workup did not show any other focus of lymphoma. He was treated with local external beam radiotherapy and was doing well on last follow-up.

Case 7

A 65-year-old male presented with non-axial proptosis of right eye with inferomedial displacement for 15 days. He also complained of low back ache for 3 months. Firm, non-tender, fixed nodular mass in

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Figures 4.6A to C: (A) A closeup photograph of the right eye shows the pink fleshy conjunctival mass in the lateral bulbar conjunctiva with intrinsic vessels which seem to appear from and disappear into the tumor. This mass represents the conjunctival component of the lymphoma. There is also axial proptosis and limitation of eye movements. (B) CT scan shows a soft tissue mass classically moulding around the globe and extending into the conjunctiva. (C) The smears are cellular with monomorphic cells, with scant rim of cytoplasm and a moderately coarse chromatin patter. Note the absence of cohesion, cytoplasm and any differentiation. (Giemsa, x 500)

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Figures 4.7A to D: (A) Photograph shows fullness of upper lid sulcus and downward displacement of the right eye by an orbital mass.(B). CT scan (axial cut) shows a well-circumscribed, homogenous, lobulated soft tissue mass in the orbit with bone destruction and extension into the temporal fossa and middle cranial fossa. (C). Radiograph of skull (lateral view) shows multiple, punched out osteolytic lesions in the skull bones. (D) The cytology smear shows multiple plasma cells with bi and multinucleated forms. The amphophilic cytoplasm and the perinuclear halo is classical of plasma cell lineage ( Giemsa, × 500).

stained smears are helpful in identifying the amphophilic cytoplasm and a perinuclear halo. The Bone marrow shows 15-25% plasma cells in various stages of differentiation.

Case 8

A 17-year-old male presented with gradual inferior displacement of right eye for three months and blurred vision following a trivial sports injury. Firm, fixed, slightly tender superomedial orbital mass observed. CT scan shows a soft tissue mass with patchy enhancement in the superior orbit (Figure 4.8A.) Peripheral blood smear was normal. Incision biopsy revealed a greenish yellow (chloroma) solid tumor in the peripheral surgical space. Squash imprint preparations of tissues showed a monomorphic round cell tumor. However the characteristic feature is the pale staining nucleus, irregular nuclear membrane, and pinkish cytoplasm. The Giemsa stained smears are of great importance in confirming the blast like morphology with cytoplasmic granules and sometime Auer rods can also be identified (Figures 4.8B and C), which confirms the diagnosis of leukemic deposits. Bone marrow biopsy was normal in this patient, at the time of orbital presentation suggesting the extramedullary leukemic deposits.

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Figures 4.8A to C: (A) CT scan orbit (coronal cut) shows a wellcircumscribed, homogenous, hypodense soft tissue mass, with no surrounding bony changes, displacing the orbital contents inferiorly.

(B) The smears show large cells with scant to moderate amount of blue cytoplasm with lack of cohesion. (C) The higher magnification shows the cytoplasmic Auer rod, confirming the diagnosis of granulocytic sarcoma (Giemsa, × 1000)