BMS2 eyelid tumors.pdf

Infendulum Glandulad Superficial e y Eyelid/Orbit Tumors Presenta5on Benign Lesion Eyelids Sebaceous cysts o Infundibulum of Hair Follicle/Gland of Zeis Deep o Superficial o Deep AKA Epidermal Inclusion Cyst Treatment (If pa4ent desires) o Cosme@c o Superficial: Clean area w/ alcohol. Incise cyst surf...

Infendulum Glandulad Superficial e y Eyelid/Orbit Tumors Presenta5on Benign Lesion Eyelids Sebaceous cysts o Infundibulum of Hair Follicle/Gland of Zeis Deep o Superficial o Deep AKA Epidermal Inclusion Cyst Treatment (If pa4ent desires) o Cosme@c o Superficial: Clean area w/ alcohol. Incise cyst surface w/ needle or jeweler forceps, express w/ 2 Q-@ps. Then apply polyosporin 1x. F/U 1 week liquido o Deep: refer for excision; if not reassurance de Sudoriferous cyst o On lid margins, it is a fluid cyst of gland of Moll, so it is transparent o Asymptoma@c uleit remoll cretención ~ Treatment: only for cosme4c o Lance w/ needle (18-20 gauge), followed by polyosporin 1X. Squamous all papilloma o F/U 1 week Squamous Cell Papilloma o Most common benign lesion of the eyelid o Commonly AKA skin tag o Viral origin suspected to be the cause (HPV); referred as verrucae o Lesions may be single or mul@ple o Commonly involves the eyelid margin Diagnosis is Primary Made by the Typical Clinical Appearance o Characteris@cally are flesh-colored Epidermis demonstrates hyperkeratosis o Treatment: Simple excision at the base of the lesion Pedenculated sessile Xantholasma Xanthelasma o Flat, yellowish, plaques most commonly in nasal/medial lid o Middle age-older pa@ents (greater in females) & Quassterd 50 Luegodelos o Usually bilateral o Associated w/ hyperlipidemias pacientes ↳ jóvenes Treatment o Systemic cardiovascular work-up o Educa@on and reassurance Molluscum Contagiosure o If cosme@c concern: refer for excision o F/U: PRN or accordingly Molluscum Contagiosum o Pox virus o Direct casual contact or fomites in children o Sexual transmission route in adults o Lesions may be single or mul@ple (up to 20) at the lid margin they may produce a pseudo-follicular conjunc@vi@s o Diagnosis is mainly based on clinical appearance en o Biopsy is rarely required in an otherwise healthy individual o Raised, Shiny, White-to-pink Nodule with A Central Umbilica@on Filled with Cheesy Material o Differen@al – Keratoacanthoma AIDS and Molluscum Contagiosum o Atypical presenta@on - may have 30-40 lesions on each eyelid, or a confluent mass - secondary folliculosis less frequently Treatment o Usually self remission (within 1 year), but may be treated to reduce transmission and secondary symptomatology o Simple incision or excision, incision and curefage, cryosurgery, and electrodesicca@on o More challenging in AIDS pa@ents - extensive involvement and recurrences Keratoacanthoma o Mainly presents as a solitary, rapidly growing nodule on sun-exposed areas of middle-aged and older individuals o Believed to originate from the neck of the hair follicle o Consider it to be a pre-cancerous lesion or variant squamous cell carcinoma o About 6% of KA manifest itself as SCC if lei untreated (involu@onal stage) o Umbilicated lesion centrally filled with a kera@n plug o Progresses over a period of weeks o Usually undergoes spontaneous involu@on within a period of 6 months o May cause mechanical lid abnormali@es Treatment o Prompt ac@on is warranted - speed recovery and limit scarring o Complete excision is recommended - invasive variant exists o Radiotherapy and intralesional fluorouracil Melano4c Nevus o Melano@c cells overgrowth o Pa@ents with fair skin o Common at the eyelid skin and eyelid margin Evolu4onal Histological Changes Evolu4onal Histological Changes o Junc@onal MN - Childhood - Small, flat, tan macules - Nest cells are found within the epidermis, at the dermal-epidermal junc@on - Spread is gradual and radial o 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 o Intradermal MN - Later in life - Intradermal nevus – total of epidermal nests migrates into the dermis - Becomes dome-shaped, pedunculated, or papillomatous, and usually is less pigmented - In some case may transform to malignancy Diagnos4c and Management Approach o Based on the clinical appearance o Removal is not required unless - cosmesis - mechanical irrita@on / lid func@on - suspicious malignancy or malignant transforma@on § Suspicious-looking lesions demonstrate irregular growth and loss of pigment § Malignant transforma@on rarely occurs in junc@onal or compound stages § ABCDE Ac4nic Keratosis o Scaly,atrophicpatchofskin–kera@n overgrowth on the skin o Flat, light gray-dark brown dry lesions o Most commonly in middle age pa@ents and older of fair skin o Sun exposure o Considered pre-cancerous lesions (10% of SSC in people with mul@ple lesions) Treatment o Photodocument /measure/ draw o F/U q 6 months o Refer to dermatology for biopsy - if suspicious (erosion of borders, large or raised, infec@on or inflamma@on appearance): o Refer for excision - cosmesis - cancerous Malignant Lesions Eyelids Basal cell carcinoma o Most common malignancy of eyelid o White, male o Older pa@ents o Long Hx of sun exposure o Hx or family Hx of cancer o Rarely metas@zes o Dx by clinical appearances and histopathological examina@on Early: Umbilica@on is Absent Vascularized nodule Umbilicated Center with Pearly Borders Treatment o Biopsy and excision w/ reconstruc@on o Goal is complete removal of tumor cells with minimal collateral damage o Mohs surgery - mainstay treatment of choice - micrographic surgery o Other treatments – cryotherapy, electrodessica@on, laser abla@on Loss of Lid Func@on o Treatment is almost always defini@ve, since these lesions rarely metastasize Squamous Cell Carcinoma o Malignant tumor arising from the kera@nized squamous cell layer of the epidermis and causing separa@on of the corneum stratum o Second most common eyelid malignancy but far less common than BCC o Has great poten@al for metastases o Fair skin, elderly (70’) male o UV exposure o Ac@nic Keratosis can be a precursor o Dx based on clinical appearance and biopsy o Crusted, raised, plaques of kera@n rapidly progressing and leading to destruc@on of @ssue and impaired lid func@on o Metastases to orbit and systemic o More common on lower lid but it can be found anywhere along the eye lid Treatment o Biopsy and excision w/ reconstruc@on o Mohs surgery o Radia@on Sebaceous cell carcinoma o A highly malignant neoplasm that arises from the meibomian glands, glands of Zeis, and from the sebaceous glands of the caruncle and eyebrow o An aggressive tumor with a high recurrence rate, a significant metasta@c poten@al, and a notable mortality rate o Accounts for up to 5% of all eyelid cancers o Occurs more oien in women o Usually occurs by the 6th decade of life o Upper eyelid is the site of origin in about two thirds of all cases o Dx by clinical appearance/Hx and histological study o Clinical appearance: it oien presents as a firm, yellow nodule that resembles a chalazion (Remember: unilateral, recurrent and resistant: ⛳⛳ o Clinical appearance: a plaque-like thickening of the tarsal plate with destruc@on of meibomian gland orifices and tumor invasion of eyelash follicles leading to madarosis Treatment o Wide surgical excision with microscopic monitoring of the margins is the procedure of choice o Mohs’s micrographic surgical excision may be used, but it may not be as successful as in BCC or SCC due to the possibility of spread. o Radia@on therapy may be considered as an adjunct to local surgery; as recurrence is likely with primary treatment alone Malignant Melanoma o Cutaneous malignant melanoma is an invasive prolifera@on of malignant melanocytes o The nodular type is most common affec@ng the eyelids o 1% of all eyelid malignancies o Malignant melanoma causes about two thirds of all tumor-related deaths from cutaneous cancers o Changing cutaneous moles, excessive sun exposure and sun sensi@vity, family history, age, and Caucasian race o Dx - clinical appearance examina@on / excisional biopsy and histopathological examina@on o Nodular lesion may present as a markedly pigmented or somewhat pigmented or amelano@c nodule that rapidly increases in size with associated ulcera@on and bleeding Treatment: Wide surgical excision with 1cm of skin margins Kaposi’s Sarcoma o Rare neoplasm that may affect the cutaneous or mucosal surfaces of the eyelids o Prior to the advent of aids, this tumor was exceedingly rare, now occurs in 24-30% of aids pts o Studies suggest that the endothelial cells characteris@c of this tumor may be of viral origin o Highly vascular, purple or red nodules on the cutaneous aspect of the eyelids and caruncle or on the conjunc@va (it also may involve the lacrimal sac and, rarely, the orbit o Diagnosis is made by clinical suspicion and confirmed by excisional biopsy and histopathologic examina@on o Differen@al diagnosis – a number of lesions can resemble Karposi's sarcoma, but in the serng of an immunocompromised host the diagnosis is usually not difficult to make Treatment: Cryotherapy, irradia@on, surgical excision, and intralesional chemotherapy Choristomas o Common congenital lesions that possess lifle growth poten@al o They contain both dermal and epithelial elements that are not normally found in the conjunc@va o Solid limbal dermoid, dermolipoma, and the complex choristoma Solid Limbal Dermoids o Compact, pale yellow growths that typically occur, unilaterally at the inferotemporal limbus o Most are superficial and only minimally involve the cornea and sclera o Histologic examina@on reveals a thick, collagenous lesion containing hair, sweat glands, fat, sebaceous glands, osseous @ssue o Complete excision may require reconstruc@on with penetra@ng keratoplasty Dermolipomas o Less dense than SD / contain more adipose @ssue o These are true choristomas - fafy @ssue usually is not found anterior to the orbital septum o Superior temporal bulbar conjunc@va - between the SR and LR inser@on to posteriorly to limbus or limbus Surgical Considera4ons o Surgery is usually not required since the extension of the growth does not limit func@on o Usually, surgery is restricted to par@al resec@on of the anterior por@on of the tumor o Carefulness not to damage the extraocular muscles, levator, or lacrimal gland Goldenhar’s Syndrome o Oculoauriculovertebral dysplasia o Bilateral limbal dermoids or dermolipomas o Unilateral colobomas of the upper lid and aniridia o Preauricular skin tags o Hypoplasia of the facial bones o Vertebral anomalies Complex Choristomas o Variable combina@ons of ectopic @ssues o Lesions resemble dermoids and lipodermoids but fleshy and vascularized o Acinar elements form the majority of the tumor - (AKA ectopic lacrimal glands) o Malignancy rare o Tx - SE avoided (invade deeply into globe) Orbital Tumors Orbital Vascular Hamartomas o Vascular hamartomas: Abnormal propor@on of vascular elements present at a cutaneous sites. Types: Capillary Hemangioma & Cavernous Hemangioma Capillary Hemangioma o High-flow hamartomatous prolifera@on of primi@ve vaso-forma@ve @ssues o Most common orbital vascular tumor of childhood (1% to 2% of infants) o Female 3:2. o One-third are noted at birth (virtually all are diagnosed by age 6 months) o Largest size within 6 months and then typically involutes Capillary Hemangioma-Histopathology o Masses of plump prolifera@ng vascular endothelial cells organized into a network of basement membrane o Network has solid cellular zones and open zones with irregular lumina o Macrophages are found in the inters@@um and may be related to the clinical feature of par@al spontaneous regression Cavernous Hemangioma o Benign, noninfiltra@ve, low-flow hamartomas ▪ Vascular malforma@on that has rela@vely large blood-filled spaces o They do not contain @ssue of the organ in which they are situated o Oien cited as the most common primary orbital tumor of adults o Usually isolated lesions o Very rarely, associated with mul@ple hemangiomas - involving predominantly the arms and trunk (usually evident at birth) - visceral lesions commonly in the small intes@ne - leading to gastrointes@nal bleeding and iron deficiency anemia Clinical Features o Typically in the fourth and fiih decades (although the age range < 18 - > 78) o 60% to 70% female preference o Gradually increasing painless proptosis common o Less common symptoms - orbital pain, eyelid swelling, diplopia, and gaze-induced amaurosis o Hormonal changes during pregnancy may s@mulate their growth Histopathology o Large, endothelium-lined,blood-filled spaces separated by fibrous septa o Created by mul@laminar smooth muscle cells o Creates larger lumens Op5c Nerve Tumors ONH Glioma (juvenile) o Benign tumor lesions of the ON glial @ssue o Within the orbit and adjacent to proximal op@c nerve or intracranial ONH o Can produce ONH compressive swelling ini@ally, then atrophy - Slowly progressive visual loss o 1st two decades: 65% in 1st decade; 90% before age of 20 (adulthood gliomas are malignant) o Intraorbital ON (47%); intracranial (26%) o Associated w/ neurobfibromatosis 1 (30%) o Proptosis as the main characteris@c Clinical characteris4cs o Proptosis o Decreased VA o Decreased o RAPD present o VF: of ONH neuropathy types o 15% ONH swelling, 2/3 ONH is normal or pale (the most common), so more of a retrobulbar compression o Optociliary shunts Treatment: o MRI/CT-MRI is befer o Inves@gate about Neurofibromatosis I o Surgical resec@on o Chemotherapy; radia@on (>5y/o) ON Sheath Meningioma o Benign tumors from the intraorbital ON sheath (surrounding the ONH) o Arise from meningothelial cells of meninges (arachnoid "cap" cells of the arachnoid villi) in the meninges o Women > 40y/o ( but can occur in children o Unilateral mainly o Associated w/ Neurofibromatosis II specially if bilateral Clinical Characteris4cs o Slowly progressive VA loss o RAPD present o Dyschromatopsia o Proptosis later in the disease o Diplopia from EOMS involvement ▪ Gazed evoked amaurosis o ONH: 50% swelling; 50% pallor o Optociliary shunts o Central VF defects o Neuroimaging – MRI o Gene@c tes@ng for muta@ons in the NF2 gene ▪If VA > 20/40:observa@on monthly o If VA worsens: radia@on o Surgery may results in blindness Orbital Lymphoma o Lymphomas- solid malignant neoplasms that originate from mostly T and B lymphocytes and dendri@c his@ocytes MC in lymph nodes and primary lymphoid @ssue (bone marrow, bursa, spleen, thymus) but also in extranodal sites, including the orbit o Two general classifica@ons - Hodgkin’s Lymphoma (HL) - Non-Hodgkin’s Lymphoma (NHL) o Hodgkin's lymphoma - histopathologically characterized by mul@nucleated lymphocytes (Reed-Sternberg or RS) cells - has been associated to the EBV o NHL - a diverse group lympha@c cancer - studies have linked the disease to the polychlorinated biphenyl (organic pollutant) o Most systemic lymphomas and orbital malignant lymphoid tumors (lymphomas) are of the non-Hodgkin's B- cell variety o Hodgkin's disease in the orbit is extremely rare o There are no lymphocytes or lymph nodes present in the deep orbit normally, although there are lymphocytes na@ve to the stroma of the conjunc@va and the acini of the lacrimal gland Systemic NHL o Painless lymph node enlargement in one or more nodes o Usually fiih or sixth decade, (presenta@on in childhood is extremely rare) ▪Fever, night sweats, and weight loss disease (characteris@c of HL) generally are absent o Splenomegaly may develop (20% of pa@ents) o Pa@ents usually have normal blood counts, although they may develop lymphocytosis, pancytopenia with anemia, o Pa@ents with B-cell lymphomas tend to develop difficulty with bacterial infec@on o T-cell lymphoma may develop difficulty with viral infec@ons o Lymphoid lesions are the third most common cause of proptosis in the adult aier inflamma@on, caused either by Graves' disease or orbital pseudotumor, and cavernous hemangioma (despite being an extra nodal site) o 50% have been shown to be reac@ve or atypical hyperplasia and 50%, malignant lymphoma (diverse histological manifesta@ons) Clinical Presenta4on o Primarily NHL and tend to present in the sixth and seventh decades (earlier than age 20 is rare) o 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 conjunc@val chemosis or injec@on o Usually only mild dysmo@lity (superior rectus-levator complex) or displacement of the globe, since, with the excep@on of orbital fat, lymphoid lesions tend to mold around rather than infiltrate preexis@ng structures o Visual loss is uncommon, and bilateral blindness is rare o Lacrimal gland is involved in 30% of lesions (since there are na@ve lymphocyte popula@ons in the lobes) o 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 o Lymphadenopathy may be palpable and may indicate the presence of systemic disease Orbital Lymphoma: Bilateral orbital lymphoma and the associated anterior, superior soi @ssue swelling, ptosis, orbital proptosis, and globe displacement. o Orbital Lymphoma: Epibulbar “salmon-patch” lesion, which can be the presen@ng sign of reac@ve hyperplasia or malignant lymphomas Treatment o Disease localized to the orbit is treated with radia@on - rapid response o Regression in the form of lymphoid hyperplasia is possible o Steroids play lifle role in trea@ng lymphoid lesions o Surgical cure is not possible because lymphoid lesions are diffuse and infiltrated into orbital fat @ssue o Commonly used doses of radia@on are 2000 to 3000 rad for benign disease (lymphoid infiltra@on)and 3000 to 4500 rad for malignant disease o The pa@ent 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