Ординатура / Офтальмология / Английские материалы / Oculoplasty and Reconstructive Surgery Made Easy_Garg,Touky, Nasralla_2009
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Figure 13: Lentigo maligna
Figure 14: Nodular melanoma
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Figure 15: Melanoma in situ
Figure 16: Nodular melanoma in conjunctiva
after solar exposure. Look as a nodular invasive area with pigmentation and an irregular outline. Is most likely to be inflicting the lower eyelid and canthus. Low grade of malignancy is seen, but the risk for developing into malignant melanoma is estimated to be 25 to 30%. Suspicion for malignant transformation should be when a change in color or shape is seen.
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The premalignant melanosis is seen in the skin and the conjunctiva and is often more demarcated than the lentigo maligna indicating invasion of the dermis. The tumor is seen both in sun-exposed and unexposed skin.
Nodular melanoma can develop from a pre-existing junctional nevus. The melanoma can be no pigmented (Figure 17), but is usually pigmented. Seen in sun-exposed and unexposed skin, conjunctiva and mucosal membranes. Has a rapid growth and is highly invasive.
Investigation
CT scans and MRI is essential as shown in the other tumor types. As the melanoma has a high risk of sending metastases, a systemic screening especially investigation of the lungs, liver and lymph node mapping. The prognosis depends on the thickness of the melanoma the degree of invasion and metastases found.
Differential Diagnosis
Basal cells cancer can be pigmented (Figure 18) and difficult to distinguish from melanoma. Squamous cell carcinoma can
Figure 17: Amelanotic melanoma
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Figure 18: Pigmented BCC
be confused with an amelanotic melanoma as the tumor can be bleeding and the surrounding skin showing inflammation. A very rare type of melanoma can be confused with recurrent chalazion: desmoplastic malignant melanoma, which has an aggressive growth with potential to invade the orbit.
Treatment
Complete surgical excision with wide margins is recommended as soon as possible; do not wait for answer from a biopsy. If orbital invasion is found, the treatment is exenteration. If suspicion of spread to the regional lymph glands a neck dissection followed by chemotherapy or radiation. In superficial conjunctival melanomas topical therapy with mitomycin C can be tried.
Early detection of the melanoma is very important, if the melanomas has reached an invasive growth and send metastases, the prognosis for cure is very poor.
Inference
Melanoma of the periocular skin and conjunctiva is a rare tumor. Three subtypes are described, lentigo maligna, nodular
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melanoma and premalignant melanosis. Melanomas of the eyelids occur in Caucasian. The melanomas have a high risk of distant metastases. Early detection is very important for a good prognosis.
Surgical excision of the melanoma with a broad margin as soon as possible is mandatory.
Merkel Cell Carcinoma
Introduction
Merkel cell carcinoma is a very rare type of cancer of the skin. The Merkel cell carcinoma is a neuroendocrine carcinoma arising from uncontrolled growth of Merkel cells in the skin. Predominantly seen in fair-skinned individuals over the age of 60 years. Seen in sun-exposed areas. The patient is seeking the doctors due to a fast growing skin tumor with red to blue colour (Figure 19).
Clinical Signs and Symptoms
A fast growing firm, painless, flesh-colored tumor on the neck, head, eyelid, arms.
Investigations
Biopsy of the tumor will give the diagnosis. A thorough investigation follows in the search for metastases as even small
Figure 19: Recurrence of Merkel cell carcinoma restricted eye movement due to orbital invasion
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Merkel cell carcinomas have the potential to metastasizing firstly to the regional lymph nodes later to liver, bone, lungs and brain.
Differential Diagnosis
Other neuroendocrine tumors and other infiltrative tumors as melanoma, basal cell cancer and squamous cell cancer are candidates.
Treatment
It is important to know the stage of the Merkel cell carcinoma.
Stage 1: The primary tumor has not spared to lymph nodes or other distance parts of the body.
Stage 2: The cancer has spread to nearby lymph nodes.
Stage 3: The cancer has spread to nearby lymph nodes and other distance parts of the body.
Recurrent stage: The cancer has recurred after treatment Wide surgical excision, radiation and chemotherapy are
recommended in stage 2. In stage 1, surgery followed by regional radiation to the lymph nodes. In stage 3, chemotherapy. In recurrent stage all three treatments might be tried.
The mortality is extremely high, increasing with age.
Inference
Merkel cell carcinoma is very uncommon, highly aggressive with an alarming mortality.
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CONCLUSION
The evaluation and treatment of neoplasms of the orbit is a multidisciplinary task involving otorhinolaryngologist, radiologist, oncologist, plastic surgeon, oromaxillofacial surgeon and ophthalmologist. In bigger hospitals orbital teams are already organized to cope with the task so no time is loosed.
An increase in incidence has been reported of the most common seen tumor, lymphoma in most countries. In some parts of the world HIV (human immunodeficiency viruses) has been the cause to the increase. In Denmark a study over the period 1974 to 1996 has shown a steady increase in the incidence of both benign and malignant orbital neoplasms. The more widely use of immunosuppressants is thought to be the course.
(Courtesy to my colleagues at the otorhinolaryngeal department University Hospital Örebro for providing information and pictures: Hans Gertze‘n,MD, Christer Nilsson,MD, Hans Gustafsson,MD, Svante Hugosson,MD.
Courtesy to Jan-Erik Axelsson,MD Dermatology Clinic Korsta. For providing pictures.
Literature: Orbital Tumors. Diagnosis and Treatment, Editor Zeynel A. Karcioglu).
INTRODUCTION
Orbital lesions in pediatric age group are of extreme concern. Most orbital tumors in children are benign and familiarity with the more common orbital lesions is required to aid the clinician in making a timely and accurate diagnosis.
CAPILLARY HEMANGIOMA
It is most common orbital tumor of childhood. It is diagnosed at birth or in the first six months of life (Figure 1A).
Symptoms and Signs
They are most commonly situated in the upper lid or orbit. Cutaneous lesions are known as strawberry nevus (Figure 1B). Depending upon their location these tumors can cause proptosis, ptosis, strabismus, astigmatism and amblyopia.
Investigations
CT scan shows homogenous soft tissue density (Figure 1C). Enhancement is variable according to the vascularity of the lesion.
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Figures 1A to C: (A) 8-month-old child presented with proptosis. (B)
Showing telangiectatic vessels on the scalp (C) CT scan showing diffusely infiltrating non-encapsulated mass in intraconal and extraconal spaces
Management
Most of them does not require treatment. Amblyopia is the most common indication for treatment. Intralesional corticosteroid, i.e a combination of triamcinolone and dexamethasone is a preferred treatment (Figure 2).
Prognosis
These lesions increase in size in the first year of life and spontaneously regresses over the years. Complete surgical resection may not be possible.
LYMPHANGIOMA
It is a benign vascular tumor that become apparent in the first decade of life. It may involve eyelid, conjunctiva and orbit
(Figure 3A and B).
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Figure 2: Inj. Triamcinolone being injected
Figures 3A and B: An 8-year-old boy with multiloculated lymphangioma
Symptoms and Signs
They may present as a slowly progressive proptosis or sudden presentation due to hemorrhage following a trivial trauma. It may be associated with ptosis, strabismus, amblyopia, glaucoma and optic nerve compression.
Investigations
Imaging studies show the multicompartmental nature of the venous-lymphatic malformations. MR imaging is preferred over CT scan as it delineates the internal structure of the various cysts of the lesion.