Rhabdomyosarcoma is an aggressive mesenchymal malignancy and is established as one of the most important pediatric soft-tissue tumors in the head and neck area, of which 10% develop in the orbit. The clinical presentation of orbital rhabdomyosarcoma somewhat depends on the location and the extent of tissue invasion, but often includes symptoms such as proptosis and displacement of the globe of the eye. The diagnosis rests on clinical criteria, imaging studies, and a biopsy of the observed lesion.
Orbital rhabdomyosarcomas (ORM) are tumors of mesenchymal tissue and are primarily detected in the pediatric population  . Namely, rhabdomyosarcomas encompass up to 4% of all tumors in children and orbital localization is detected in approximately 10% of affected patients . Furthermore, ORM was identified as the second-most common orbital neoplasm (after lymphoma) in large isolated studies . Together with the fact that 5-year survival rates of approximately 60% illustrate the highly aggressive nature of this lesion , its recognition must be made early on. The clinical presentation is described either as insidious, with only minor signs and symptoms causing a diagnostic delay, or rapidly progressive . The most frequent complaints are the onset of proptosis within a period of weeks (protrusion of the eye) and displacement of the globe . Because the malignancy is predominantly located in the superonasal area, the globe is displaced anteroinferiorly in the majority of cases . If nasal spread or nasopharyngeal localization occurs, chronic otitis or nasal obstruction can be observed . On the other hand, headaches and cranial nerve palsies indicated cranial and parameningeal extension, respectively . Without early treatment, hematogenous spread of metastasis to distant organs (the bone marrow and the skeletal system, as well as the lungs) might be noticed .
Entire Body System
We advocate active coopera-tion of pathologist, pediatrician, brain surgeon, otolaryngologist and ophthalmologist in the diag-nosis and treatment of orbital rhabdomyosarcoma in children. Copyright © 1990, Igaku-Shoin Ltd. All rights reserved. [webview.isho.jp]
Biopsy : The only way to determine the type of tumor is to obtain a piece of it for examination by a pathologist. This is known as a biopsy. In some cases, the primary tumor can be completely removed at the same time. [childrensoncologygroup.org]
[…] a a a « » Determining the Type of Orbital Tumor Only after a pathologist analyzes some cells or actual pieces of tissue from the lesion will your doctor be able to tell you if you have cancer. [headandneckcancerguide.org]
If the surgeon or pathologist are concerned that microscopic cells may have been left behind after surgery, either at the tumor margin or in the lymph nodes, post-operative radiation will likely be recommended. [oncolink.org]
The early diagnosis was very important in this case, due to the imaging and histopathological exams in question with the association of experienced pathologists. [wjgnet.com]
Voute Springer Science & Business Media, 06.12.2012 - 317 Seiten With the fall in mortality from infectious diseases, the impact of childhood cancer in the Western World has increased to become the second commonest cause of death in the age group 1-14 [books.google.de]
Low-risk group About 1 in 3 children with RMS falls into the low-risk group. [cancer.org]
Zimbabwe : Bulawayo, Harare, Mutare, Victoria Falls, etc. [maria-online.com]
Habermann W, Kiesler K, Eherer A, Luxenberger W, Beham A, Friedrich G (1999) Ein Gichttophus am Krikoarytenoidalgelenk: Der seltene Fall einer laryngealen Gichtmanifestation. 43. Österreichischer HNO-Kongreß 1999, Rust 2. [hno-habermann.at]
[…] prior-to-admission medications Family History: Father—Chronic myelogenous leukemia s/p bone marrow transplant Mother—Hypertension Maternal Grandmother—Colon cancer Social History: Lives with both parents and his 15-year-old sister Beginning 6th grade this fall [webeye.ophth.uiowa.edu]
The notable absence of erythema, calor and fever separates rhabdomyosarcoma from cellulitis due to an adjacent sinus infection. Radiologic Studies: The tumor often appears deceptively well circumscribed on scan. [missionforvisionusa.org]
Eyelid and conjunctival RMS often present as a gradually enlarging, well circumscribed mass, that may have associated eyelid edema and erythema as well as chemosis. [eyewiki.aao.org]
- Unilateral Proptosis
Orbital rhabdomyosarcoma is the most common orbital malignancy of childhood with the common presentation of rapidly evolving unilateral proptosis. [ncbi.nlm.nih.gov]
Material and methods A total of 148 cases of unilateral proptosis of neoplastic origin were examined. [ijo.in]
Definition / general Most common orbital sarcoma in childhood Usually embryonal and alveolar subtypes (alveolar more aggressive) Often rapid onset of unilateral proptosis May occur after radiation therapy for retinoblastoma, close to previously irradiated [pathologyoutlines.com]
- Excessive Tearing
The patient had been well until the age of six months, when excessive tearing developed. Over the next two days, his right eye was noted to be puffy and red. His pediatrician made a diagnosis of conjunctivitis. [nejm.org]
The life-threatening nature of the ORM mandates a thorough diagnostic approach for undisclosed ocular and nasopharyngeal symptoms in the pediatric population. The first step is to obtain a detailed patient history from the child (or from the parents if necessary), during which the onset of symptoms, their features, and course should be carefully assessed, as the diagnosis can often be misinterpreted as cellulitis, other inflammatory conditions or benign tumors  . After a detailed inspection of the orbit, imaging studies need to be employed. Ultrasonography is of little benefit in the assessment, which is why computed tomography (CT), but more importantly magnetic resonance imaging (MRI), particularly with gadolinium as a contrast agent, are recommended   . Orbital rhabdomyosarcoma can be readily detected using MRI, exhibiting an isointense or hypointense signal on T1-weighted studies and a hyperintense signal on T2-weighted images   . Finally, a biopsy of the lesion is advised to determine the exact subtype and depending on the location and severity of tumor invasion, both excisional and incisional approaches might be performed, whereas fine-needle aspiration provides limited efficacy in obtaining a viable sample for histopathological testing .
Seven patients relapsed (no local treatment, N = 2; surgery and brachytherapy, N = 2; external beam radiotherapy, N = 2; surgery only, N = 1). The authors found no patient, tumor, or treatment characteristics that predisposed for treatment failure. [ncbi.nlm.nih.gov]
The main outcome measures are a rare cause ofproptosis in an adult, discussion on treatment options and prognosis ofrhabdomyosarcoma. The patient underwent total orbital exenteration and was referred for radiotherapy and chemotherapy. [ncbi.nlm.nih.gov]
The objective of our retrospective analysis was to define the prognosis in correlation to changes of treatment in an unselected patient group of a single institution. [link.springer.com]
Prognosis: Orbital rhabdomyosarcomas are classified slightly differently and have a better prognosis (overall five-year survival of 92%) than do their non-orbital tumors. [missionforvisionusa.org]
Rhabdomyosarcoma of the Orbit Definition: malignant neoplasm with skeletal muscle differentiation Incidence/Prevalence: the most common primary malignant orbital tumor of childhood Etiology: The alveolar subtype of rhabdomyosarcoma has a consistent chromosomal [missionforvisionusa.org]
In a retrospective review by Shields et al, the primary site of ophthalmic RMS was the orbit in 76%, eyelid in 3%, conjunctiva in 12%, and the uveal tract in 9%.  Etiology RMS initially was believed to originate directly from striated muscle. [eyewiki.aao.org]
Journal Journal ID (nlm-ta): Pan Afr Med J Journal ID (iso-abbrev): Pan Afr Med J Journal ID (publisher-id): PAMJ Title: The Pan African Medical Journal Publisher: The African Field Epidemiology Network ISSN (Electronic): 1937-8688 Publication date (Electronic [scienceopen.com]
National Cancer Institute; Surveillance, Epidemiology, and End Results (SEER) Program [homepage on the Internet]. [dovepress.com]
Cancer 61:209–220 PubMed CrossRef Google Scholar Miller RW (1968) Relation between cancer and congenital defects: an epidemiologic evaluation. [link.springer.com]
Paediatric and Perinatal Epidemiology. 8 (1): 107–119. doi : 10.1111/j.1365-3016.1994.tb00439.x. ISSN 1365-3016. ^ Yang, P.; Grufferman, S.; Khoury, M. J.; Schwartz, A. G.; Kowalski, J.; Ruymann, F. B.; Maurer, H. M. (1995-01-01). [en.wikipedia.org]
[…] ear ( J Clin Oncol 1995;13:610 ) Also common in the paratesticular soft tissues and the genitourinary tract Extremity involvement of the somatic soft tissues is less common (< 9%) Metastasizes to soft tissue, serosa, lung, lymph nodes and bone marrow Pathophysiology [pathologyoutlines.com]
Pathophysiology The tumor is believed to arise from primitive muscle cells, but tumors can occur anywhere in the body; however, a primary bone rhabdomyosarcoma has not been reported. [emedicine.medscape.com]
It is important to diagnose orbital rhabdomyosarcoma on time to prevent problems with the eyesight and prevent from spreading in the brain where it will be more difficult to eradicate. [bookinghealth.com]
Earlier recognition of orbital metastasis through radiographic and biopsy findings, along with prompt and aggressive treatment, may prevent fulminant spread of rhabdomyosarcoma. [ncbi.nlm.nih.gov]
They can be used to evaluate medications or treatments to prevent cancer, detect it earlier, or help manage side effects. Clinical trials are extremely important in furthering our knowledge of this disease. [oncolink.org]
Radiotherapy [ edit ] General: RMS infiiltrates along tissues planes, and may extend beyond obvious visible margins Treat pre-chemo, pre-surgery tumor volume. 2 cm margin is sufficient (per IRS-IV) For parameningeal sites, treatment of adjacent meninges can prevent [en.wikibooks.org]
The use of RT in most patients as part of the initial management in the North American approach aims to minimize disease recurrence, whereas the European approach attempts to prevent radiation-induced effects by avoiding the use of upfront RT. 11 In this [dovepress.com]
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- Boparai MS, Dash RG Clinical, ultrasonographic and CT evaluation of orbital rhabdomyosarcomas with management. Indian J Ophthalmol. 1991;39:129–131.
- Mu X, Wang H, Li Y, Hao Y, Wu C, Ma L. Magnetic resonance imaging and DWI features of orbital rhabdomyosarcoma. Eye Sci. 2014;29(1):6-11.