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

advanced regional metastasis or more distant spread. Recent reviews of cases have shown that orbital involvement confers a particularly bad prognosis. The 5 years survival of a recent case series of nasopharyngeal cancer with orbital extension was only 28%.146

Metastatic Orbital Tumors

Metastasis to the orbit occurs secondary to hematological spread of a primary tumor. Of all the orbital cases 1.5 to 3.3 % are metastatic of which 7 % cases are bilateral. In the pediatric age group, the common primaries include neuroblastoma, Wilm's tumor and Ewing's sarcoma. In adults, the common primary sites for metastatic tumors to the orbit are from breast, prostate, lung, gastrointestinal tract, kidney and cutaneous melanoma. Most commonly, the patient has a known primary tumor. The most common malignancies that metastasize to the orbit are carcinoma of the breast in females and carcinoma of the lung in males.147,148 Metastases to the orbit have also been reported to occur from carcinoma of the tongue, pancreas, gallbladder, cervix, penis, urinary bladder, thyroid intestinal carcinoid, renal cell carcinoma and carcinosarcoma of the parotid

gland.149-157

Orbital metastasis may also be the first manifestation of a systemic malignancy. Metastases to fat and bone are twice as common than muscle. Usually the primary tumor is known except in about one quarter of patients. Certain carcinomas like melanoma and breast carcinoma are likely to have a known primary.

Average survival is approximately 9 months from the orbital presentation. The first manifestation of symptoms due to orbital metastasis usually occurs after a mean period of 31-64 months after diagnosis of primary disease, but tumors such as breast cancer and thyroid cancer have a longer delay (3-5 years). Melanoma is intermediate (2 years) and those from the lung and gastrointestinal tract are diagnosed shortly before or after orbital presentation. The mean age of the patients was 64 years in one study.147

These tumors present similarly to others involving the orbit. Symptoms include lid swelling, red eye, pain, proptosis and diplopia and signs include incomitant strabismus with diplopia, blepharoptosis, decreased vision, proptosis and a

Secondary and Metastatic Orbital Tumors 263

palpable mass.158,159 However, motility disturbance out of proportion to the proptosis is characteristic of an orbital metastatic tumor. Unusually, enophthalmos has been reported with schirrous carcinomas of the breast whilst metastases from the prostate to the orbit are usually osteoblastic in nature.160,161 Renal cell carcinoma has a tendency to induce hemorrhage and consequently it may initially be difficult to differentiate from orbital hematoma.156 Diagnosis of metastatic origin follows simple lines. Site of the original tumor may also be ascertained with symptoms and signs suggestive of other systems being involved. Investigations may include nonspecific tests such as carcinoembryonic antigen (CEA) which is elevated in metastatic disease of the bowel. Other specific tests may be refined with regards to the primary tumor suspected. Both CT and MRI have a place in management. Both hyperostotic and spiculated CT appearances have been described.147,149 MRI may assist in soft tissue differentiation and to assess spread. Ultimately, however, a fine needle aspiration biopsy may be required to confirm the diagnosis in situations where an orbitotomy is not possible.162

The survival rate of these patients is poor and consequently treatment is mainly palliative and is aimed at providing symptomatic relief163 with radiotherapy, hormonal therapy, chemotherapy and surgery depending on the type and stage of the disease.

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The etiology of proptosis is multifactorial. As we know it can be congenital, or acquired due to various causes like infections both nonspecific and specific bacterial like tuberculosis,1-4 sarcoidosis,5-7 fungal infections, inflammations, parasitic infestations, vascular malformations, primary orbital tumors arising from various tissues like nerves and their sheaths, vascular tissues, mesenchymal, lymphoproliferative, bony lesions apart from secondary involvement from the globe, paranasal sinuses and the brain. Metastatic lesions from breast,8 thyroid, prostate, GIT and other primary lesions elsewhere were documented in the literature. Involvement of Orbit in generalized lymphoproliferative lesions like systemic lymphomas, leukemia is very well known. It is really surprising to know the wide variety of pathology that can involve such a small structure as orbit. It is not an exaggeration that in the orbit one can come across almost all the types of pathology that can involve human body.

With such a wide spectrum of pathological lesions causing proptosis, it is natural that the management strategies also vary. The options include observation and reassurance, medical management, surgery, radiotherapy and chemotherapy. The surgical procedures include biopsy, debulking, excision of tumor/mass/cyst through various surgical approaches, multi-speciality approaches along with ENT/Neurosurgeon, and exenteration.The decision depends upon accurate diagnosis of the nature of the lesion (infective/cystic/tumor: benign/ malignant/Secondary/metastatic) and its location and extent (intraconal/peripheral space/extension

to paranasal sinuses/intracranial extension). A detailed clinical evaluation, and imaging with CT scan/MRI of Orbit usually give us enough information to plan proper management strategy. Other investigations like serology, FNAC13,14, Squash and IHC (Immuno-Histochemistry) are useful aids in establishing the diagnosis and planning the management. In this chapter I am outlining the different management strategies and which to choose from. The details of the management procedures are described in the following chapters.

Medical management is indicated when proptosis is due to orbital cellulitis, orbital myocysticercosis, idiopathic orbital inflammation, most cases of thyroid associated orbitopathy (the drug-trial for TAO is well known), Wegener's granulomatosis, tuberculosis of the orbit, fungal granulomas, in other words in all the inflammatory and infective conditions. However, drain an orbital or sub-periosteal abscess, along with systemic antibiotics. The role of medical management is discussed in a separate chapter.

Chemotherapy and radiotherapy have a great role in the management of rhabdomyosarcoma, lymphoma and other lymphoproliferative diseases, retinoblastoma. They can also be used occasionally in thyroid orbitopathy, idiopathic orbital inflammation. They are helpful in the treatment of other malignancies like adenoid cystic carcinoma of lacrimal gland, secondary and metastatic lesions. These were discussed in a separate chapter.

For me, it is always very important to get at an accurate diagnosis as early as possible, so that I am not missing any serious condition. That is why, even in a case of clinically suspected orbital cellulitis, I get

a CT scan as early as possible to make sure that I am not missing other conditions like rhabdomyosarcoma, retinoblastoma which can occasionally present as orbital cellulitis. The CT scan also informs me if there is an abscess which I have to drain, or it is only orbital cellulitis which I can manage medically.

When the diagnosis is not definite, I prefer to do a biopsy and get a histopathological diagnosis. The tissue diagnosis can be by way of FNAC (Figures 19.1A and B and 19.2A and B), biopsy or intraoperative Squash smear examination. However I personally don't perform FNAC on a lacrimal gland tumor. Instead I wish to excise the tumor in toto and plan further treatment depending on the histopathological diagnosis.

Biopsy is indicated for histopathological confirmation of clinical diagnosis (Figures 19.3A to C) as in lymphoma, meningioma of optic nerve sheath, rhabdomyosarcoma, so that the patient can be referred to an oncologist. Biopsy is also indicated when the clinical diagnosis is not definite (Figures 19.4A to E).

When I am planning excision of the tumor, the approach depends on its location. If it is involving surrounding structures like PNS, or brain, it will be a multispeciality approach. However, FESS (Functional endoscopic sinus surgery) is the best way to manage fronto-ethmoid mucocele. If the tumor is confined to the orbit, it will be dealt with by me alone, the only exception being a small apical lesion, for which I prefer a transcranial approach.

The orbital approaches to proptosis are lateral orbitotomy approaches and anterior orbitotomy approaches. The anterior orbitotomy approaches are further divided into cutaneous approaches and conjunctival approaches. When choosing a procedure I consider the surgical exposure and also the cosmetic result. Though I wish to hide the scar and give the best cosmetic result, adequate surgical exposure of the lesion is of paramount importance, which can not be over-emphasized. I advise the beginners and the occasional orbital surgeons to choose simpler procedures first, and as they gain experience, they can perform other complicated procedures. I wish

Figures 19.1A and B: FNAC showing malignant cells in whorl pattern, suggestive of neuroblastoma. The details are better seen in higher magnification. This patient presented with painful proptosis and restricted ocular motility. CT scan revealed a mass lesion with bony erosion. Diagnosis of metastatic neuroblastoma was made from FNAC

Figures 19.2A and B: FNAC showing cells with large, peripheral nucleus and vacuolated cytoplasm arranged in clusters, from metastatic ductal carcinoma of breast leading to proptosis