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 Table of Contents  
ORIGINAL ARTICLE
Year : 2019  |  Volume : 7  |  Issue : 2  |  Page : 181-185

Mélange of orbital lesions – A histomorphologic study of 135 cases


1 Kempegowda Institute of Medical Sciences, Bengaluru, Karnataka, India
2 Department of Pathology, Kempegowda Institute of Medical Sciences, Bengaluru, Karnataka, India
3 Department of Pathology, BGS Global Institute of Medical Sciences, Bengaluru, Karnataka, India

Date of Submission18-Jul-2019
Date of Decision09-Aug-2019
Date of Acceptance13-Aug-2019
Date of Web Publication16-Dec-2019

Correspondence Address:
Dr. Aparna Muralidhar
No. 137, 5th Main, Padmanabhanagar, Bengaluru - 560 070, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/amhs.amhs_104_19

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  Abstract 


Background: The orbit is an important site for primary and secondary diseases. Various tissue types such as osseous, vascular, neural, muscular, and glandular may be involved with specific pathologies. Tumors can secondarily invade the orbit from the periorbital regions including the paranasal sinuses, eyelids, and intracranial region. Objectives: (i) To assess the histomorphology of various orbital lesions. (ii) To determine the frequency, age, and sex distribution of various orbital lesions in our study population and compare them with the other studies. Materials and Methods: The study involved 135 patients of either sex presenting with orbital lesions reporting to a tertiary care hospital. Results: Among the 135 cases, we observed a slight female predominance, with a female-to-male ratio being 1.17:1. Most of the patients were in their fifth decade and presented with exophthalmos. Histopathologically, cystic lesions were most frequent followed by lymphoproliferative lesions. About 25% of lesions were malignant and included lymphomas, lacrimal gland malignancies, and rhabdomyosarcomas predominantly. We came across a rare case of intravenous papillary endothelial hyperplasia. Conclusion: Orbital lesions arise primarily from soft tissues and bones. The frequency of orbital lesions varies among different series depending on age group, source institution, medical specialty, and geographic areas. Histopathology remains the mainstay of diagnosis. In addition to determining the malignant potential of a lesion, it reveals its exact nature and structure, thereby influencing management and prognosis.

Keywords: Exophthalmos, orbit, rhabdomyosarcoma


How to cite this article:
Gupta R, Venugopal SB, Muralidhar A, Geethamani V, Malhotra A. Mélange of orbital lesions – A histomorphologic study of 135 cases. Arch Med Health Sci 2019;7:181-5

How to cite this URL:
Gupta R, Venugopal SB, Muralidhar A, Geethamani V, Malhotra A. Mélange of orbital lesions – A histomorphologic study of 135 cases. Arch Med Health Sci [serial online] 2019 [cited 2020 Aug 6];7:181-5. Available from: http://www.amhsjournal.org/text.asp?2019/7/2/181/273046




  Introduction Top


The orbit is a site for a wide variety of diseases, majority of which represent primary conditions or may be associated with systemic diseases. The orbital contents are also a rich terrain for the development of a spectrum of neoplasms often referred to as soft-tissue tumors.[1] Some lesions invade the orbit secondarily from ocular adnexa and periorbital structures. In addition, metastases to the orbit originate from various organs.[2]

The classification of primary tumors of the orbit is based mainly on the line of differentiation of the tumor. Each type is further subdivided into benign and malignant groups. In some categories, an intermediate group is incorporated.[3]

Among the pediatric tumors, about half are cytics. Others include capillary hemangiomas, mesenchymal lesions, histiocytic lesions, and leukemias.[3],[4] In adults, inflammatory pseudotumors, lymphoproliferative disorders, and vascular lesions are the most frequent causes of intraorbital mass.[5] The incidence of malignant tumors increases with age, from approximately 22% in children to 63% in older adults.[6]


  Materials and Methods Top


We undertook a 2-year retrospective and 2-year prospective hospital-based study of 135 patients from July 2010 to June 2014 presenting with orbital lesions. Informed consent was obtained from all the study participants. The study was approved by the ethical committee of our institute. Purposive sampling was done. Appropriate clinical history was taken.

The surgically resected specimens were received in 10% formalin. Thorough gross examination of each specimen for its size, shape, and consistency was done. Representative areas were sampled and subjected to routine paraffin embedding. Five-microns thick sections were taken, stained with hematoxylin and eosin, and evaluated. Immunohistochemistry (IHC) was done whenever required.


  Results Top


Of the 135 cases, 63 (46%) were male and 72 (54%) were female. The age distribution ranged from 6 months to 90 years, with a maximum number of cases involving 41–50 years (30%) and minimum involving 81–90 years (2%). Clinically, most patients presented with proptosis [Figure 1], followed by palpable mass and restricted ocular motility.
Figure 1: Clinical photo showing proptosis

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The distribution of the lesions is depicted in [Table 1]. Malignant tumors accounted for 25% of the cases. Among these, lymphoma was most common. About 75% of lesions were benign, of which cystic lesions were most frequent.
Table 1: Distribution of orbital lesions

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Of the 43 cystic lesions, 22 cases (51.1%) were dermoid cysts, followed by 15 cases (34.8%) of epidermal inclusion cysts and 6 cases (13.9%) of mucocele. Majority of the patients were under 20 years of age with a male predominance (65.1%).

Microscopically, dermoid cysts showed the keratinized stratified squamous epithelial lining and pilosebaceous units in the subepithelial stroma. Few cases showed mixed inflammatory infiltrate (infected dermoid cyst) and granulation tissue response. Mucoceles showed cyst cavity lined by columnar epithelium with occasional goblet cells resting on a fibrocollagenous wall.

We encountered 25 lymphoproliferative lesions. Among these, 15 cases showed reactive lymphoid hyperplasia. Out of the ten cases of lymphomas, mucosa-associated lymphoid tissue lymphomas were most common. The mean age of involvement in reactive lymphoid hyperplasia was 41 years, while that in lymphoma was 56 years.

Microscopically, reactive lymphoid hyperplasia was characterized by a polymorphous population of lymphoid cells comprising predominantly of mature small lymphocytes, centrocytes, centroblasts admixed with plasma cells, and histiocytes embedded in a scant fibrous stroma [Figure 2]a. Lymphomas showed monotonous population of atypical lymphoid cells with scant cytoplasm and pleomorphic hyperchromatic nuclei and atypical mitosis. (IHC) was done in all cases. Nine cases showed diffuse cytoplasmic positivity for CD20 and hence were diagnosed as extranodal marginal zone B-cell lymphoma [Figure 2]b. One case was CD10 and CD23 positive and was diagnosed as follicular lymphoma.
Figure 2: (a) Photomicrograph of reactive lymphoid hyperplasia, H and E, ×400. (b) Photomicrograph of lymphoma showing sheets of monomorphic cells, H and E, × 400. Inset shows CD20 positivity

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Vascular lesions were more common in the age group of 21–30 years. There were 16 cases of cavernous hemangioma, two cases each of lymphangioma and hemangiopericytoma, and one case each of malignant hemangiopericytoma and intravenous papillary endothelial hyperplasia. Cavernous hemangioma histopathologically showed large dilated vascular channels lined by flat endothelial cells separated by fibrous septae.

Hemangiopericytomas were microscopically characterized by a rich vascular pattern of dilated branching vessels and interspersed round-to-ovoid cells. IHC showed positivity for vimentin [Figure 3]a and negativity for CD34, differentiating them from a solitary fibrous tumor. We also encountered a case of malignant hemangiopericytoma, characterized by pleomorphic hyperchromatic atypical cells in solid sheets and many mitotic figures [Figure 3]b.
Figure 3: (a) Photomicrograph of hemangiopericytoma, H and E, ×100. Inset – H and E, ×400 IHC – Vimentin positivity. (b) Photomicrograph of malignant hemangiopericytoma, H and E, ×100. (c) Photomicrograph of IPEH, H and E, ×100. Inset – H and E, ×400

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Intravascular papillary endothelial hyperplasia (IPEH) showed small delicate papillae lined by a single layer of endothelium with a collagenized core [Figure 3]c.

Among the lacrimal gland lesions, there were nine cases of pleomorphic adenoma, four cases of adenoid cystic carcinoma, three cases of lymphomas, and two cases of benign lymphoid hyperplasia. Patients were mainly females in their fourth decade of life.

Pleomorphic adenomas were characterized by proliferation of epithelial and stromal components. Epithelial component comprised duct-like structures embedded in a chondromyxoid to fibromyxoid stroma. Adenoid cystic carcinoma showed neoplastic cells arranged in cribriform and solid patterns with pseudoglandular spaces containing eosinophilic material and mucin [Figure 4]a. Lymphomas were characterized by a monomorphic population of atypical lymphoid cells with scant cytoplasm, pleomorphic hyperchromatic nuclei, and atypical mitoses. IHC showed diffuse cytoplasmic positivity for CD20 in all the cases, and a diagnosis of extranodal marginal zone B-cell lymphoma was made.
Figure 4: (a) Photomicrograph of adenoid cystic carcinoma, H and E, ×100. (b) Photomicrograph of ancient schwannoma, H and E, ×100. (c) Photomicrograph of neurofibroma, H and E, ×100. (d) Photomicrograph of angiomatous meningioma, H and E, ×100

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Of the peripheral nerve sheath tumors, we had four cases of schwannoma and two cases of neurofibroma. The mean age at presentation was 50 years with a female predominance. Schwannomas showed solid cellular areas (Antoni A pattern) alternating with areas of loose stellate cells in a mucinous matrix (Antoni B pattern). Nuclear palisading and highly oriented polar cytoplasmic processes (Verocay bodies) were noted. One case showed cystic degeneration with thickened blood vessels and was diagnosed as ancient schwannoma [Figure 4]b. Neurofibromas comprised wavy bundles of spindle cells with kinked nuclei in a fibrillary background, as depicted in [Figure 4]c.

There were three cases of embryonal rhabdomyosarcomas with a mean age at presentation of 3.5 years. Microscopically, they showed sheets of small round cells with scant cytoplasm and hyperchromatic angulated nuclei [Figure 5]a. The cells were strongly positive for myogenin on IHC.
Figure 5: (a) Photomicrograph of embryonal rhabdomyosarcoma, H and E, ×100. (b) Photomicrograph of retinoblastoma showing sheets of cells with scant cytoplasm, round-to-oval hyperchromatic nuclei, and intervening thin fibrous septae. H and E, ×400

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Lipomas were more common around 30 years of age and were characterized by mature adipocytes with univacuolated cytoplasm and peripherally pushed nuclei separated by thin fibrovascular septae.

Two patients in their fifth decade had meningioma. Microscopically, one was a transitional variant with meningothelial cells in bundles, fascicles, and tight whorls. The other was angiomatous with prominent variably sized and variably hyalinized vascular channels [Figure 4]d.

Among the miscellaneous lesions, seven were inflammatory pseudotumors characterized by fibroblasts in a chronic inflammatory background. Two were poorly differentiated tumors. Four cases were diagnosed as metastatic lesions, two were metastatic deposits of adenocarcinoma, and the other two were secondaries from retinoblastoma [Figure 5]b.


  Discussion Top


Orbital lesions can occur in various age groups. Due to their myriad presentation, diagnosis poses a great challenge. Although imaging studies graphically illustrate tissue definition, pathological conditions can be assessed definitely only on histopathologic examination.

The distribution pattern in our study was slightly different from other studies and is depicted in [Table 2]. Cystic lesions were more common in our study and that by Nath and Gogi.[7] However, Shields et al.[4] and Kennedy[5] found vascular lesions to be the most common.
Table 2: Comparison of distribution of lesions with other studies

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We found benign lesions to be more common, among which dermoid cyst was the most common. By far, it is the most common orbital cystic lesion in children, accounting for over 40% of all orbital lesions.[8] It can occur at any age and is usually located in the anterior superior aspect of the orbit. It may protrude through the orbital septum and present as a subcutaneous eyelid mass.

Orbital lymphomas account for 8% of all extranodal lymphomas.[6] Lymphoproliferative lesions in the orbit encompass a wide spectrum of disorders, ranging from benign reactive lymphoid hyperplasia to lymphoma. Lymphomas are the most common orbital malignancy in adults. Eighty percent of lymphomas are B-cell type.[9] They are mostly seen in the fifth to seventh decades of life with a female predominance.[10]

In our study, reactive hyperplasia was the most common lymphoid lesion with 15 cases, while in other studies, lymphomas were the more common. The range of age distribution was 20–80 years which was wider than other studies.[4],[5] Females were more commonly affected in our study, whereas Shields et al.[4] and Kennedy[5] observed a male predominance.

Vascular lesions are the second most common orbital tumors in children and the most common orbital tumors in adults. On the basis of their natural history, growth pattern, and histologic composition, they can be classified into capillary hemangiomas, venous vascular malformations, venous lymphatic malformations, arterial and arteriovenous lesions, or neoplasms.[11] Hemangiopericytomas were first described in 1942 by Stout and Murray. These are rare, slow-growing, highly vascular tumors, relatively common in the musculoskeletal system (lower extremities), pelvis, and retroperitoneum. Approximately 15% of these tumors arise in the extracranial part of the head and neck, usually in the nasal cavity or paranasal sinuses. Origin within the orbit is rare.[11] In ours as well as other studies,[4],[6],[7] cavernous hemangiomas were most common. We also observed three cases of hemangiopericytoma, of which one was malignant.

Intravascular papillary endothelial hyperplasia (IPEH) is another rare vascular tumor, first described by Masson in 1923 under the name of “Vegetant intravascular hemangioendothelioma.” IPEH is a reactive proliferative lesion of endothelial cells in blood vessels.[12] We came across one such case.

Pleomorphic adenoma was the most common lacrimal gland lesion with a female predominance. Kennedy[5] observed similar findings. However, the age distribution was wider (20–80 years).

Peripheral nerve sheath tumors originate from sensory nerves in the orbit. They are most frequent in the superior and medial orbital compartments and comprise 4% of all primary orbital tumors.[13] In our study, schwannoma was most common and affected females predominantly, which correlated with the study by Kennedy[5] The range of age distribution was between 20 and 50 years. Kennedy[5] recorded a wider range of age distribution between 4 months to 78 years.

Orbital rhabdomyosarcoma usually presents as a space-occupying lesion in the orbit during the first decade and may mimic other neoplastic or inflammatory masses.[14] The tumor has a predilection for the superior nasal quadrant of the orbit. Embryonal rhabdomyosarcoma was the most common type which correlated with the other studies.[4],[5] Females were more commonly affected which correlated with the study done by Kennedy.[5]

Among the lipomatous lesions, we found lipoma to be most common with a female predilection, correlating with the study by Kennedy.[5]

Secondary involvement of orbit from adjacent structures arises most commonly from an orbital extension of uveal melanoma. In our study, four cases of metastasis, i.e., two cases each of adenocarcinoma and retinoblastoma, were diagnosed.

A study done by Kennedy.[5] showed 36 cases of metastases, of which 13 cases were from breast carcinoma, 10 were adenocarcinomas, and the rest were unclassified. Shields et al.[4] showed 91 cases of metastases, mostly from the breast followed by the prostate, melanoma, and lungs.

Inflammatory pseudotumors accounted for 5.1% of all orbital lesions. It is a distinct entity with many clinical and histopathologic guises. Hence, it is a diagnosis of exclusion. It can occur in any age and sex.[6] We came across seven cases of inflammatory pseudotumors.


  Conclusion Top


A large spectrum of lesions can occur in the orbit. Orbital symptoms can be of great concern to the patient, family, and clinician. Lesions may be developmental, inflammatory, and associated with general or metabolic disease, traumatic, neoplastic, vascular, or of unknown etiology.

Clinically, orbital lesions can be challenging to diagnose. The core of a good approach to orbital tumors hinges on the good histopathologic study and diagnosis, thereby playing a crucial role in patient management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Jakobiec FA, Font RL. Ophthalmic Pathology, An Atlas and Textbook. 3rd ed. Philadelphia: Saunders; 1986. p. 2459-60.  Back to cited text no. 1
    
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Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. 4th ed. St Louis: Mosby; 2001.  Back to cited text no. 2
    
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Font RL, Croxatto JO, Rao NA. Tumors of the Eye and Ocular Adnexa (AFIP Atlas of Tumor Pathology). Series 4. Washington, DC: American Registry of Pathology; 2006.  Back to cited text no. 3
    
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Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery lecture, part 1. Ophthalmology 2004;111:997-1008.  Back to cited text no. 4
    
5.
Kennedy RE. An evaluation of 820 orbital cases. Trans Am Ophthalmol Soc 1984;82:134-57.  Back to cited text no. 5
    
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Radha J, Sreedhar A. Orbital tumors a clinic pathological study. Kerala J Ophthalmol 2005;3:261-5.  Back to cited text no. 6
    
7.
Nath K, Gogi R. Primary orbital tumors. Indian J Ophthalmol 1977;25:10-6.  Back to cited text no. 7
[PUBMED]  [Full text]  
8.
Shields JA, Shields CL. Orbital cysts of childhood – Classification, clinical features, and management. Surv Ophthalmol 2004;49:281-99.  Back to cited text no. 8
    
9.
Stefanovic A, Lossos IS. Extranodal marginal zone lymphoma of the ocular adnexa. Blood 2009;114:501-10.  Back to cited text no. 9
    
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Ghasemi M, Amoli FA, Gransar A. A clinicopathologic study of orbital and ocular adnexal lymphoproliferative lesions with immunohistochemical staining of indeterminate cases. Acta Med Iran 2003;41:11-4.  Back to cited text no. 10
    
11.
Smoker WR, Gentry LR, Yee NK, Reede DL, Nerad JA. Vascular lesions of the orbit: More than meets the eye. Radiographics 2008;28:185-204.  Back to cited text no. 11
    
12.
Weber FL, Babel J. Intravascular papillary endothelial hyperplasia of the orbit. Br J Ophthalmol 1981;65:18-22.  Back to cited text no. 12
    
13.
Kron M, Bohnsack BL, Archer SM, McHugh JB, Kahana A. Recurrent orbital schwannomas: Clinical course and histopathologic correlation. BMC Ophthalmol 2012;12:44.  Back to cited text no. 13
    
14.
Jurdy L, Merks JH, Pieters BR, Mourits MP, Kloos RJ, Strackee SD, et al. Orbital rhabdomyosarcomas: A review. Saudi J Ophthalmol 2013;27:167-75.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1], [Table 2]



 

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