Ординатура / Офтальмология / Английские материалы / Oculoplasty and Reconstructive Surgery Made Easy_Garg,Touky, Nasralla_2009
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Figures 3A and B: Old man with exophthamos right eye due to slowly growing lymphoma
Clinical Signs and Symptoms
The patient develops a painless proptosis, eyelid edema and the bulb is suppressed downward. The first sign could be diminished vision due to optic neuropathy by compression of the lymphoma at the orbital apex. The growth of the tumor is often very slowly so the patient adapt to the tumor for a long time.
Investigation
MRI (magnetic resonance image) shows a homogeny mass in the superior part of the orbit caste to the bulb and orbital bones. CT (computed tomography) shows a homogeneous texture.
Fine-needle cytology will often give the diagnosis. PET and MRI of the body to look after systemic involvement by the lymphoma.
Differential Diagnosis
The REAL (Revised European-American Classification of Lymphoid Neoplasms) classify the lymphomas in categories based on morphology, immunophenotype, and genotype as indolent, aggressive and highly aggressive.
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Treatment
Radiotherapy is advocated in local low-grade orbital lymphoma only, but if any suspicion on systemic involvement systemic chemotherapy is advocated to control the lymphomas. For the more aggressive type systemic chemotherapy and local radiation therapy is advocated. A new treatment is monoclonal immunotherapy in low-grade lymphomas especially in very old and weak patients.
In the most aggressive lymphomas aggressive chemotherapy followed by stem cells transplantation has been used.
Inference
Orbital lymphomas are the most common tumor of the orbit, often unilateral. The tumor is insidious bringing the patient to the ophthalmologist first after long time. The visual acuity might be reduced, and a painless proptosis is seen. MRI and fine-needle cytology will give the diagnosis in most cases. Systemic involvement should always be looked for.
Squamous Cell Carcinoma
Introduction
Squamous cell carcinoma (SCC) (Figure 4) is seen in 9% of tumors of the eyelids and 10% of carcinomas involving the orbit, but represent one of the most seen skin cancers. SCC inflicts mostly elderly individuals. Seen most often in fairskinned individuals who have been exposured to sunlight as the skin of the face, hands and the scalp. Australian has again as in basal cells cancer the highest incidence in the world: 166 per 100,000 persons. It can metastasize and can locally spread aggressively.
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Figure 4: Squamous cell cancer
Predisposing factors of SCC is ultraviolet lightment treatments, chronic skin inflammation and in immunosuppressed individuals.
Clinical Signs and Symptoms
Often seen as an ulcerated lesion with induration and erythema of the inflicted skin (Figure 5). The spread is locally invasive, deep into the connective tissue. It can spread via the lymphatic glands. The SCC tends to have a more
Figure 5: Recurrence of squamous cell canthus carcinoma
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aggressive spread than the basal cells cancer. Look for involvement of the preauricular nodes and submandibular nodes.
Investigation
The same as with the basal cells cancer: Computed tomography (CT) and magnetic resonance imaging (MRI) (Figure 6). The CT is the best tool for determining the bone destruction and MRI will show the invasion into soft tissue especially if perineural spread of the tumor is suspected. Perineural spread points at a solemn prognosis.
In the SCC the risk for spread via the lymph glands must be investigated.
Patients with the orbital apex syndrome or superior orbital fissure syndrome: pain, complete ptosis, and ophthalmoplegia indicate that the spread of the SCC is far reached.
Differential Diagnosis
Other cancers involving the eyelids, canthus and epicanthus and even the conjunctiva, basal cells cancer, melanoma
Figure 6: Recurrence of squamous cancer right orbita
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especially the amelanotic type even lymphoma, sebaceous gland tumors and metastatic tumors with ulceration can be difficult clinically to different from squamous cell carcinoma.
Treatment
Radical surgery guided by the frozen section to be sure that the margins are free of tumor cells. Exenteration if the SCC is invasive into the orbit followed by radiotherapy. As said perineural spread is a very bad prognostic sign. The mortality rate is as high as 40% in squamous cell cancers involving the eyelids. Delayed diagnosis and inadequate, primary treatment are the factors that lead to invasive growth into the orbit.
Inference
The squamous cell cancer is a very common seen cancer of the skin. As the basal cells cancer seen in a fair-skinned individual exposed for sunlight.
The squamous cell cancers are 9% of the cancers involving the eyelids and 10% of the cancers involving the orbit.
The mortality rate is as high as 40% in squamous cell cancers involving the eyelids due to the risk of metastases and aggressive growth of the tumor. It is of the highest importance that the primary surgery shall be radical as inadequate treatment leaving cancer cells, often leads to recurrence of the tumor in spite of radiotherapy.
Basal Cell Carcinoma
Introduction
Basal cells carcinomas represent 90% of periocular tumors with orbital invasion. The exposure to sunlight is thought to be the major cause particularly in fair-skinned people. Especially seen in a fair-skinned population, which have
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migrated to a sunny climate as the white population in Australia, which have the highest incidence of skin cancers in the world, ranging from 650 to 1560 cases per 100,000 persons.
Clinical Signs and Symptoms
The BCC is classified into 4 types: Nodular ulcerative BCC, sclerosing BCC, superficial BCC and basosquamous BCC.
The nodular ulcerative BBC (Figure 7) is the most common type seen in 75% of the BBC tumors. The tumor start as a little papule, further growing form a central necrosis with a sclerosing round border, a rodent ulcer, which seldom invade the orbit.
The sclerosing BCC present as the name tells an indurated plaque without distinct borders often with a deep invasion into the dermis. This type is invasive into the orbit and accounts for near 15% of all BCC (Figure 8).
The superficial BCC looks like dermatitis of chronic type
(Figure 9).
Figure 7: Nodular basal cells carcinoma
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Figure 8: Basal cells cancer ingrowth orbit
Figure 9: The superficial BCC
The basosquamous type of BCC may clinically look like the nodular type but is most more liable to grow invasive and even to metastasize (Figure 10).
The typical patient is middle-aged person who has been working in the open air or has been exposed to actinic radiation for a long time.
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Figure 10: Highly aggressive BCC morphea basaliom
Figure 11: BCC inferior eyelid
The tumor is often seen in the lower eyelid (Figure 11), medial canthus and more seldom the lateral canthus and upper eyelid.
If the complain is pain and restricted movement of the bulb or proptosis invasion into the orbit should be suspected.
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Investigation
Computed tomography (CT) and magnetic resonance imaging (MRI). The CT is the best tool for determining the bone destruction and MRI will show the invasion into soft tissue especially if perineural spread of the tumor is suspected.
Differential Diagnosis
Melanoma especially amelanotic can be difficult to distinguish from BCC. Squamous cell carcinoma and sebaceous gland tumors can even be difficult to distinguish clinically. Metastatic tumors involving the skin, e.g. breast carcinoma.
Treatment
The best treatment is surgical excision of the tumor with clear margins verified by the pathologist. In orbital invasion exenteration is the preferred technique to be sure that the tumor is totally removed. Radiation is not recommended as a high risk of recurrence of the tumor is seen after radiotherapy. The recurrent tumor is often much more biologically aggressive (Figure 12).
Figure 12: Exenteration of invasive BCC
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Inference
The basal cell carcinoma account for 90% of the periocular skin tumors with orbital invasion. Four subtypes are described: Nodular ulcerative BCC, sclerosing BCC, superficial BCC and basosquamous BCC.
The BCC is seen in a fair-skinned individual who has been exposed for a long time to bright sunlight. The highest incidence of BCC in the world is seen in Australia in the white population. Treatment is surgically excision, excenteration if invasion to the orbit is seen. Radiotherapy is not advocated, as the risk for recurrence of a biologically aggressive BCC is very high.
Melanoma
Introduction
Melanoma affecting the eyelids accounts for 1% of the cancers afflicting the periocular skin and is 1% of melanomas seen elsewhere in the skin of the body.
Three types are described, melanoma arising from a Hutchinson’s melanotic freckle or lentigo maligna (Figure 13), nodular melanoma (Figure 14) and from premalignant melanosis or cutaneous melanoma in situ (Figures 15 and 16).
The incidence of malignant melanoma is the fastest growing in Sweden. The cause to that is the popularity to have tanned skin. The risk for developing melanoma increases if the child has heavy sunburn early in life. The risk is higher in individuals with red or fair hair and freckled skin.
Clinical Signs and Symptoms
Lentigo maligna is seen in an elderly Caucasian that has been exposured to sunlight. Often seen in skin with degeneration