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    Tugas Besar: Makalah Retinoblastoma Modul Hematologi Onkologi

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    This document is a paper on retinoblastoma discussing its definition, epidemiology, etiology, pathophysiology, clinical manifestations, diagnosis, management, prognosis, and references. It defines retinoblastoma as a rare intraocular tumor most commonly found in children. Key points include that it occurs in 1 in 15,000-20,000 births, can be hereditary or sporadic, displays a variety of clinical manifestations depending on stage, is usually diagnosed clinically aided by imaging, and has a high mortality if left untreated but good survival rates with proper treatment confined to the eye.

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    Tugas Besar: Makalah Retinoblastoma Modul Hematologi Onkologi

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    This document is a paper on retinoblastoma discussing its definition, epidemiology, etiology, pathophysiology, clinical manifestations, diagnosis, management, prognosis, and references. It defines retinoblastoma as a rare intraocular tumor most commonly found in children. Key points include that it occurs in 1 in 15,000-20,000 births, can be hereditary or sporadic, displays a variety of clinical manifestations depending on stage, is usually diagnosed clinically aided by imaging, and has a high mortality if left untreated but good survival rates with proper treatment confined to the eye.

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    This document is a paper on retinoblastoma discussing its definition, epidemiology, etiology, pathophysiology, clinical manifestations, diagnosis, management, prognosis, and references. It defines retinoblastoma as a rare intraocular tumor most commonly found in children. Key points include that it occurs in 1 in 15,000-20,000 births, can be hereditary or sporadic, displays a variety of clinical manifestations depending on stage, is usually diagnosed clinically aided by imaging, and has a high mortality if left untreated but good survival rates with proper treatment confined to the eye.

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    53 views7 pages

    Tugas Besar: Makalah Retinoblastoma Modul Hematologi Onkologi

    Uploaded by

    KartikaSari
    This document is a paper on retinoblastoma discussing its definition, epidemiology, etiology, pathophysiology, clinical manifestations, diagnosis, management, prognosis, and references. It defines retinoblastoma as a rare intraocular tumor most commonly found in children. Key points include that it occurs in 1 in 15,000-20,000 births, can be hereditary or sporadic, displays a variety of clinical manifestations depending on stage, is usually diagnosed clinically aided by imaging, and has a high mortality if left untreated but good survival rates with proper treatment confined to the eye.

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    TUGAS BESAR

    MAKALAH RETINOBLASTOMA
    MODUL HEMATOLOGI ONKOLOGI

    Fasilitator:
    dr. Dian Mutiasari

    Disusun oleh:
    KARTIKA SARI
    FAA 113 056

    PROGRAM STUDI PENDIDIKAN DOKTER


    FAKULTAS KEDOKTERAN
    UNIVERSITAS PALANGKA RAYA
    2016
    1

    BAB I

    I.1 Definisi
    Retinoblastoma merupakan suatu keganasan pada intraokular yang paling sering
    ditemukan pada kasus anak dan menunjukkan persentase sebesar kurang lebih 4% dari semua
    kasus keganasan yang terjadi pada anak-anak.
    I.2 Epidemiologi
    Setiap tahunnya persentase terjadi kasus retinoblastoma di wilayah Amerika Serikat
    diperkirakan sekitar 1 dari 15.000 sampai 20.000 kelahiran. The average age at diagnosis is 18
    months, with unilateral cases being diagnosed at around 24 months and bilateral cases before 12
    months. Out of the newly diagnosed cases of retinoblastoma only 6% are familial while 94% are
    sporadic. It is bilateral in about 25-35% of cases. Bilateral retinoblastomas involve germinal
    mutations in all cases. Out of the unilateral cases, approximately 15% are caused by germinal
    mutations while the 85% are sporadic.
    I.3 Etiologi
    Sekarang ini diketahui bahwa retinoblastoma can be inherited as a familial tumor in
    which the affected child has a positive family history of retinoblastoma or as a nonfamilial
    (sporadic) tumor in which the family history is negative for retinoblastoma. Approximately 6%
    of newly diagnosed retinoblastoma cases are familial and 94% are sporadic. All patients with
    familial retinoblastoma are at risk to pass the predisposition for the development of the tumor to
    their offspring, but the manifestations are only 80% penetrant.
    I.4 Patofisiologi
    I.5 Manifestasi Klinik
    Manifestasi klinik dari retinoblastoma vary with the stage of the disease at the time of
    recognition. In its earliest clinical stage,a small retinoblastoma,ie,less than 2 mm in basal
    dimension,appears ophthalmoscopically as a subtle, transparent or slightly translucent lesion in
    the sensory retina. Slightly larger tumors lead to dilated retinal blood vessels that feed and drain
    the tumor. Some larger tumors show foci of chalk-like calcification that resemble cottage cheese.
    Any retinoblastoma of any size can produce leukocoria.The larger tumors more often present
    with this leukocoria (Fig 2). This white pupillary reflex is a result of reflection of light from the
    white mass in the retrolental area. Retinoblastoma growth patterns are subdivided into

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    intraretinal,endophytic,and exophytic (Fig 3). Intraretinal tumors are those listed above,limited to
    the substance of the retina. Endophytic retinoblastoma is one that grows from the retina inward
    toward the vitreous cavity. Hence, it is characterized by a white,hazy mass with obscuration of
    the retinal blood vessels. Because of its friable nature, an endophytic tumor can seed the vitreous
    cavity and anterior chamber and simulate endophthalmitis,especially toxocariasis,a parasitic
    disease found in young children.
    An exophytic retinoblastoma is one that grows from the retina outward into the subretinal
    space. Such tumors produce a progressive retinal detachment, with the retina often displaced
    anteriorly behind a clear lens (Fig 4). An exophytic retinoblastoma can clinically resemble
    Coats disease or other forms of exudative retinal detachment. Occasionally, a retinoblastoma
    can assume a diffuse infiltrating pattern,characterized by a relatively flat infiltration of the retina
    by tumor cells without an obvious mass. In such cases, the diagnosis may be more difficult, and
    this pattern can simulate uveitis or endophthalmitis. Less frequently, the presenting feature can
    be pseudohypopyon due to tumor seeding in the anterior chamber, hyphema secondary to iris
    neovascularization,vitreous hemorrhage, or signs of orbital cellulitis.

    BAB II

    II.1 Anamnesis
    II.2 Pemeriksaan Fisik
    II.3 Pemeriksaan Penunjang
    II.4 Diagnosis
    Diagnosis is usually clinical, aided by USG B-scan of the eye revealing tumor extension
    and intralesional calcification.4 Computed tomogrophy (CT) scan and magnetic resonance
    imaging (MRI) are reserved in cases with atypical presentation, extraocular extension or those

    2
    3

    having diagnostic dilemma. CT scan helps to delineate tumor extension and co-existent
    pinealoblastoma can be detected (trilateral tumor). MRI is useful when optic nerve or intracranial
    extension is suspected. Intralesional calcification on CT scan or USG B-scan is highly suggestive
    finding for retinoblastoma. On histopathology, poorly differentiated tumor consists of small to
    round cells having scanty cytoplasm and hyperchromatic nuclei. Well-differentiated tumors
    show rosettes and flueretes formation, which is highly suggestive of photoreceptor differentiation
    of tumor cells. Flexner-Wintersteiner rosettes consist of columnar cells arranged around a lumen
    and are highly characteristic of retinoblastoma.
    II.5 Penatalaksanaan
    The most important objective in the management of a child with retinoblastoma is
    survival of the patient, and the second most important goal is preservation of the globe. The
    currently available treatment methods for retinoblastoma include intravenous chemoreduction
    (sometimes combined with subconjunctival chemoreduction),thermotherapy,cryotherapy,laser
    photocoagulation, plaque radiotherapy, external beam radiotherapy, enucleation,orbital
    exenteration,and systemic chemotherapy for metastatic disease.
    a. Chemoreduction
    b. Subconjunctival Chemoreduction for Retinoblastoma
    c. Focal Therapies
    d. External Beam Radiotherapy
    e. Enucleation
    II.6 Prognosis
    If left untreated, the mortality rate of retinoblastoma is about 99%. The major factor in
    mortality rates for patients with retinoblastoma is whether or not the tumor is confined to the eye.
    Extraocular spread increases mortality rates markedly. If there are tumor cells at the cut end of
    the optic nerve (with an enucleation), the mortality rate is much higher. Even if tumor is in the
    lamina cribrosa but the cut end of the optic nerve is free of tumor, mortality rates are elevated.
    However, when tumor is confined to the globe when enucleated, survival rates are greater than
    92%.
    II.7 Edukasi

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    BAB III

    III.1 Kesimpulan

    4
    5

    DAFTAR PUSTAKA

    Desjardins L, Chefchaouni MC, Lumbroso L et al. Functional Results After Treatment of


    Retinoblastoma. J AAPOS 2002; 6:108-11.
    Isabelle A, Livia L, Marion G, Herv B, Franois D, Laurence D. Retinoblastom. Orphanet J
    Rare Dis. 2006; 1: 31. Published online 2006 August 25. doi: 10.1186/1750-1172-1-31
    Essuman V, Ntim-Amponsah CT, Renner L, Akafor S, Edusei L. Presentation of retinoblastoma
    at a paediatric eye clinic in Ghana. Ghana med J 2010; 14(1): 10- 14
    Provenzale J M, Gururangan S and Klintworth G Trilateral Retinoblastoma: Clinical and
    Radiologic Progression. AJR 2004; 183:505-511

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    6

    Gndz K, Mftoglu O, Gnal I, nal E, Tacyildiz N . .Metastatic retinoblastoma : Clinical


    features, treatment, and prognosis .Ophthalmology. September 2006; 113(9): 1558-1566.
    Abramson DH, Schefler AC. Update on retinoblastoma. Retina. 2004; 24(6):828848. doi:
    10.1097/00006982-200412000-00002.

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