Eyelid/Orbital Tumors PDF

Eyelid/Orbit Tumors Benign Lesions Eyelids Sebaceous cysts Infundibulum of Hair Follicle/ Gland of Zeis Sebaceous cysts Superficial Sebaceous cysts Deep AKA Epidermal Inclusion Cyst Sebaceous cysts Treatment: (If patients desires) Cosmetic Superficial: Clean area w/ alcohol. Incise cyst surface w/ n...

Eyelid/Orbit Tumors Benign Lesions Eyelids Sebaceous cysts Infundibulum of Hair Follicle/ Gland of Zeis Sebaceous cysts Superficial Sebaceous cysts Deep AKA Epidermal Inclusion Cyst Sebaceous cysts Treatment: (If patients desires) Cosmetic Superficial: Clean area w/ alcohol. Incise cyst surface w/ needle or jeweler forceps, express w/ 2 Q-tips. Then apply polyosporin 1x. F/U 1 week Deep: refer for excision; if not reassurance Sudoriferous cyst On lid margins, it is a fluid cyst of gland of Moll, so it is transparent Asymptomatic Treatment: only for cosmetic Lance w/ needle (18-20 gauge), followed by polyosporin 1X. F/U 1 week Squamous Cell Papilloma Most common benign lesion of the eyelid Commonly AKA skin tag Viral origin suspected to be the cause (HPV); referred as verrucae Lesions may be single or multiple Commonly involves the eyelid margin Diagnosis is Primary Made by the Typical Clinical Appearance Characteristically are flesh-colored Epidermis demonstrates hyperkeratosis Sessile Pedunculated Treatment is simple excision at the base of the lesion. Xanthelasma Flat, yellowish, plaques most commonly in nasal/medial lid Middle age-older patients (greater in females) Usually bilateral Associated w/ hyperlipidemias Treatment Systemic cardiovascular work-up Education and reassurance If cosmetic concern: refer for excision F/U: PRN or accordingly Molluscum Contagiosum Pox virus Direct casual contact or fomites in children Sexual transmission route in adults Lesions may be single or multiple (up to 20) at the lid margin they may produce a pseudo-follicular conjunctivitis Diagnosis is mainly based on clinical appearance Biopsy is rarely required in an otherwise healthy individual Raised, Shiny, White-to-pink Nodule with A Central Umbilication Filled with Cheesy Material Differential - Keratoacanthoma AIDS and Molluscum Contagiosum Atypical presentation - may have 30-40 lesions on each eyelid, or a confluent mass - secondary folliculosis less frequently Treatment Usually self remission (within 1 year), but may be treated to reduce transmission and secondary symptomatology Simple incision or excision, incision and curettage, cryosurgery, and electrodesiccation More challenging in AIDS patients - extensive involvement and recurrences Keratoacanthoma Mainly presents as a solitary, rapidly growing nodule on sun-exposed areas of middle-aged and older individuals Believed to originate from the neck of the hair follicle Consider it to be a pre-cancerous lesion or variant squamous cell carcinoma About 6% of KA manifest itself as SCC if left untreated (involutional stage) Umbilicated lesion centrally filled with a keratin plug Progresses over a period of weeks Usually undergoes spontaneous involution within a period of 6 months May cause mechanical lid abnormalities Treatment Prompt action is warranted - speed recovery and limit scarring Complete excision is recommended - invasive variant exists Radiotherapy and intralesional fluorouracil Melanotic Nevus Melanotic cells overgrowth Patients with fair skin Common at the eyelid skin and eyelid margin Evolutional Histological Changes Epidermis Dermis Evolutional Histological Changes Junctional MN - Childhood - Small, flat, tan macules - Nest cells are found within the epidermis, at the dermalepidermal junction - Spread is gradual and radial Evolutional Histological Changes Compound MN - at older age - lesion ceases to increase in diameter and part nests of cells “drop off” into the dermis - lesion becomes slightly elevated and more pigmented Evolutional Histological Changes Intradermal MN - later in life - intradermal nevus - total of epidermal nests migrate into the dermis - Becomes dome-shaped, pedunculated, or papillomatous, and usually is less pigmented - In some case may transform to malignancy Diagnostic and Management Approach Based on the clinical appearance Removal is not required unless - cosmesis - mechanical irritation / lid function - suspicious malignancy or malignant transformation ✓ suspicious-looking lesions demonstrate irregular growth and loss of pigment ✓ malignant transformation rarely occur in junctional or compound stages ✓ ABCDE rule Actinic Keratosis Scaly, atrophic patch of skin – keratin overgrowth on the skin Flat, light gray-dark brown dry lesions Most commonly in middle age patients and older of fair skin Sun exposure Considered pre-cancerous lesions (10% of SSC in people with multiple lesions) Treatment Photodocument /measure/ draw F/U q 6 months Refer to dermatology for biopsy - if suspicious (erosion of borders, large or raised, infection or inflammation appearance): Refer for excision - cosmesis - cancerous Malignant Lesions Eyelids Basal cell carcinoma Most common malignancy of eyelid White, male Older patients Long Hx of sun exposure Hx or family Hx of cancer Rarely metastizes Dx by clinical appearances and histopathological examination Histology Early: Umbilication is Absent Vascularized nodule Umbilicated Center w/ Pearly Borders Loss of Lid Function Treatment Biopsy and excision w/ reconstruction Goal is complete removal of tumor cells with minimal collateral damage Mohs surgery - mainstay treatment of choice - micrographic surgery Other treatments – cryotherapy, electrodessication, laser ablation Treatment is almost always definitive, since these lesions rarely metastasize Squamous Cell Carcinoma Malignant tumor arising from the keratinized squamous cell layer of the epidermis and causing separation of the corneum stratum Second most common eyelid malignancy but far less common than BCC Has great potential for metastases Fair skin, elderly (70’) male UV exposure Actinic Keratosis can be a precursor Dx based on clinical appearance and biopsy Crusted, raised, plaques of keratin rapidly progressing and leading to destruction of tissue and impaired lid function Metastases to orbit and systemic More common on lower lid but it can be found anywhere along the eye lid Treatment Biopsy and excision w/ reconstruction Mohs surgery Radiation Sebaceous cell carcinoma A highly malignant neoplasm that arises from the meibomian glands, glands of Zeis, and from the sebaceous glands of the caruncle and eyebrow An aggressive tumor with a high recurrence rate, a significant metastatic potential, and a notable mortality rate Accounts for up to 5% of all eyelid cancers Occurs more often in women Usually occurs by the 6th decade of life Upper eyelid is the site of origin in about two thirds of all cases Dx by clinical apprearance/Hx and histological study Histology May have a variety of clinical appearances - it often presents as a firm, yellow nodule that resembles a chalazion (Remember: unilateral, recurrent and resistant: RED FLAG May have a variety of clinical appearances - a plaque-like thickening of the tarsal plate with destruction of meibomian gland orifices and tumor invasion of eyelash follicles leading to madarosis Treatment Wide surgical excision with microscopic monitoring of the margins is the procedure of choice Mohs' micrographic surgical excision may be used, but it may not be as successful as in BCC or SCC due to the possibility of spread Radiation therapy may be considered as an adjunct to local surgery; as recurrence is likely with primary treatment alone Malignant Melanoma Cutaneous malignant melanoma is an invasive proliferation of malignant melanocytes The nodular type is most common affecting the eyelids 1% of all eyelid malignancies Malignant melanoma causes about two thirds of all tumor-related deaths from cutaneous cancers Changing cutaneous moles, excessive sun exposure and sun sensitivity, family history, age, and Caucasian race Dx - clinical appearance examination / excisional biopsy and histopathological examination Malignant Melanoma A B C D E Histology Nodular lesion may present as a markedly pigmented or somewhat pigmented or amelanotic nodule that rapidly increases in size with associated ulceration and bleeding Treatment Wide surgical excision with 1cm of skin margins Kaposi’s Sarcoma Rare neoplasm that may affect the cutaneous or mucosal surfaces of the eyelids Prior to the advent of aids, this tumor was exceedingly rare, now occurs in 24-30% of aids pts Studies suggest that the endothelial cells characteristic of this tumor may be of viral origin Highly vascular, purple or red nodules on the cutaneous aspect of the eyelids and caruncle or on the conjunctiva (it also may involve the lacrimal sac and, rarely, the orbit Kaposi’s Sarcoma Diagnosis is made by clinical suspicion and confirmed by excisional biopsy and histopathologic examination Differential diagnosis – a number of lesions can resemble Karposi's sarcoma, but in the setting of an immunocompromised host the diagnosis is usually not difficult to make Treatment - cryotherapy, irradiation, surgical excision, and intralesional chemotherapy Choristomas Choristomas Common congenital lesions that possess little growth potential They contain both dermal and epithelial elements that are not normally found in the conjunctiva Solid limbal dermoid, dermolipoma, and the complex choristoma Solid Limbal Dermoids Compact, pale yellow growths that typically occur, unilaterally at the inferotemporal limbus Most are superficial and only minimally involve the cornea and sclera Histologic examination reveals a thick, collagenous lesion containing hair, sweat glands, fat, sebaceous glands, osseous tissue Complete excision may require reconstruction with penetrating keratoplasty Dermolipomas Less dense than SD / contain more adipose tissue These are true choristomas - fatty tissue usually is not found anterior to the orbital septum Superior temporal bulbar conjunctiva - between the SR and LR insertion to posteriorly to limbus or limbus Surgical Considerations Surgery is usually not required since the extension of the growth does not limit function Usually, surgery is restricted to partial resection of the anterior portion of the tumor Carefulness not to damage the extraocular muscles, levator, or lacrimal gland Goldenhar's syndrome Oculoauriculovertebral dysplasia Bilateral limbal dermoids or dermolipomas Unilateral colobomas of the upper lid and aniridia Preauricular skin tags Hypoplasia of the facial bones Vertebral anomalies Complex Choristomas Variable combinations of ectopic tissues Lesions resemble dermoids and lipodermoids but fleshy and vascularized Acinar elements form the majority of the tumor - (AKA ectopic lacrimal glands) Malignancy rare Tx - SE avoided (invade deeply into globe) , Complex Choristomas Orbital Tumors Jose M De Jesus, OD, MD, MA, FAAO Tumors ❑ Capillary Hemangiomas ❑ Cavernous Hemangiomas ❑ Optic Nerve Glioma ❑ Optic Nerve Sheet Meningioma ❑ Lymphoma Orbital Vascular Hamartomas ▪Vascular hamartomas – abnormal proportion of vascular elements present at a cutaneous site ▪Capillary Hemangioma ▪Cavernous Hemangioma Capillary Hemangioma ▪ High-flow hamartomatous proliferation of primitive vaso-formative tissues ▪ Most common orbital vascular tumor of childhood (1% to 2% of infants) ▪Female 3:2. ▪ One-third are noted at birth (virtually all are diagnosed by age 6 months) ▪ Largest size within 6 months and then typically involutes Capillary Hemangioma Histopathology Masses of plump proliferating vascular endothelial cells organized into a network of basement membrane Network has solid cellular zones and open zones with irregular lumina Macrophages are found in the interstitium and may be related to the clinical feature of partial spontaneous regression Cavernous Hemangioma ▪ Benign, noninfiltrative, low-flow hamartomas ▪ Vascular malformation that has relatively large blood-filled spaces ▪ They do not contain tissue of the organ in which they are situated ▪ Often cited as the most common primary orbital tumor of adults Skin Section Clinical Features ▪ Typically in the fourth and fifth decades (although the age range < 18 - > 78) ▪ 60% to 70% female preference ▪ Gradually increasing painless proptosis common ▪ Less common symptoms - orbital pain, eyelid swelling, diplopia, and gaze-induced amaurosis ▪ Hormonal changes during pregnancy may stimulate their growth Cavernous Hemangioma ▪ Usually isolated lesions ▪ Very rarely, associated with multiple hemangiomas - involving predominantly the arms and trunk (usually evident at birth) - visceral lesions commonly in the small intestine leading to gastrointestinal bleeding and iron deficiency anemia Cavernous Hemangioma Histopathology Large, endothelium-lined, blood-filled spaces separated by fibrous septa Created by multilaminar smooth muscle cells Creates larger lumens Optic Nerve Tumors ▪ONH Glioma (Juvenile) ▪ON Sheath Meningiomas ONH Glioma (juvenile) ▪ Benign tumor lesions of the ON glial tissue ▪ Within the orbit and adjacent to proximal optic nerve or intracranial ONH ▪ Can produce - ONH compressive swelling initially, then atrophy - Slowly progressive visual loss ▪ 1st two decades: 65% in 1st decade; 90% before age of 20 (adulthood gliomas are malignant) ▪ Intraorbital ON (47%); intracranial (26%) ▪ Associated w/ neurobfibromatosis 1 (30%) ▪ Proptosis as the main characteristic ONH Glioma (juvenile) Clinical characteristics ▪ Proptosis ▪ Decreased VA ▪ Decreased CV ▪ RAPD present ▪ VF: of ONH neuropathy types ONH Glioma (juvenile) ▪15% ONH swelling, 2/3 ONH is normal or pale (the most common), so more of a retrobulbar compression ▪ Optociliary shunts Optociliary Shunt Vessels ONH Glioma (juvenile) Management / Tx ▪ MRI/CT-MRI is better ▪ Investigate about Neurofibromatosis I ▪ Surgical resection ▪ Chemotherapy; radiation (>5y/o) ON Sheath Meningioma ▪Benign tumors from the intraorbital ON sheath (surrounding the ONH) ▪Arise from meningothelial cells of meninges (arachnoid "cap" cells of the arachnoid villi) in the meninges ▪ Women > 40y/o ( but can occur in children ▪Unilateral mainly ▪ Associated w/ Neurofibromatosis II specially if bilateral ON Sheath Meningiomas Clinical Characteristics ▪ Slowly progressive VA loss ▪ RAPD present ▪ Dyschromatopsia ▪ Proptosis later in the disease ▪ Diplopia from EOMS involvement ▪ Gazed evoked amaurosis ▪ ONH: 50% swelling; 50% pallor ▪ Optociliary shunts ▪ Central VF defects ON Sheath Meningioma Management / Tx ▪ Neuroimaging – MRI ▪Genetic testing for mutations in the NF2 gene ▪ If VA > 20/40:observation monthly ▪If VA worsens: radiation ▪Surgery may results in blindness Orbital Lymphoma Orbital Lymphoma ▪Lymphomas- solid malignant neoplasms that originate from mostly T and B lymphocytes and dendritic histiocytes ▪ MC in lymph nodes and primary lymphoid tissue (bone marrow, bursa, spleen, thymus) but also in extranodal sites, including the orbit ▪Two general classifications ▪ Hodgkin’s Lymphoma (HL) ▪ Non-Hodgkin’s Lymphoma (NHL) ▪Hodgkin's lymphoma - histopathologically characterized by multinucleated lymphocytes (Reed-Sternberg or RS) cells - has been associated to the EBV Orbital Lymphoma ▪NHL - a diverse group lymphatic cancer - studies have linked the disease to the polychlorinated biphenyl (organic pollutant) ▪Most systemic lymphomas and orbital malignant lymphoid tumors (lymphomas) are of the non-Hodgkin's B- cell variety ▪Hodgkin's disease in the orbit is extremely rare ▪There are no lymphocytes or lymph nodes present in the deep orbit normally, although there are lymphocytes native to the stroma of the conjunctiva and the acini of the lacrimal gland Orbital Lymphoma Systemic NHL ▪Painless lymph node enlargement in one or more nodes ▪Usually fifth or sixth decade, (presentation in childhood is extremely rare) ▪Fever, night sweats, and weight loss disease (characteristic of HL) generally are absent ▪ Splenomegaly may develop (20% of patients) ▪Patients usually have normal blood counts, although they may develop lymphocytosis, pancytopenia with anemia, ▪Patients with B-cell lymphomas tend to develop difficulty with bacterial infection ▪T-cell lymphoma may develop difficulty with viral infections Orbital Lymphoma ▪Lymphoid lesions are the third most common cause of proptosis in the adult after inflammation, caused either by Graves' disease or orbital pseudotumor, and cavernous hemangioma (despite being an extra nodal site) ▪50% have been shown to be reactive or atypical hyperplasia and 50%, malignant lymphoma (diverse histological manifestations) ▪Clinical Presentation - Primarily NHL and tend to present in the sixth and seventh decades (earlier than age 20 is rare) - lesions usually are located superiorly and anteriorly in the orbit (mold around orbital structures maily)) and present over several months with gradual painless proptosis of 5 mm or less, without conjunctival chemosis or injection - Usually only mild dysmotility (superior rectus-levator complex) or displacement of the globe, since, with the exception of orbital fat, lymphoid lesions tend to mold around rather than infiltrate preexisting structures Orbital Lymphoma ▪Clinical Presentation - Visual loss is uncommon, and bilateral blindness is rare - Lacrimal gland is involved in 30% of lesions (since there are native lymphocyte populations in the lobes) - Epibulbar lesions are referred to as “salmon-patch” lesions because of their pink fleshy color - are freely mobile and may be isolated or extend posteriorly into the orbit - Lymphadenopathy may be palpable and may indicate the presence of systemic disease Orbital Lymphoma Bilateral orbital lymphoma and the associated anterior, superior soft tissue swelling, ptosis, orbital proptosis, and globe displacement. Orbital Lymphoma Epibulbar “salmon-patch” lesion, which can be the presenting sign of reactive hyperplasia or malignant lymphomas Orbital Lymphoma Treatment ▪Disease localized to the orbit is treated with radiation - rapid response ▪Regression in the form of lymphoid hyperplasia is possible ▪ Steroids play little role in treating lymphoid lesions ▪ Surgical cure is not possible because lymphoid lesions are diffuse and infiltrated into orbital fat tissue ▪Commonly used doses of radiation are 2000 to 3000 rad for benign disease (lymphoid infiltration)and 3000 to 4500 rad for malignant disease ▪The patient diagnosed with orbital lymphoma must undergo extensive systemic work up to ensure that there is no systemic involvement before undergoing localized therapy - chest x-ray, CT scans of the thorax and abdominal viscera - bone scan, liver-spleen scan - complete blood count with peripheral blood smear - Coombs' test, serum protein electrophoresis, and bone marrow biopsy