Orbital rhabdomyosarcomas (ORM) are tumors of mesenchymal tissue and are primarily detected in the pediatric population [1] [2]. Namely, rhabdomyosarcomas encompass up to 4% of all tumors in children and orbital localization is detected in approximately 10% of affected patients [1]. Furthermore, ORM was identified as the second-most common orbital neoplasm (after lymphoma) in large isolated studies [3]. Together with the fact that 5-year survival rates of approximately 60% illustrate the highly aggressive nature of this lesion [2], 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 [4]. The most frequent complaints are the onset of proptosis within a period of weeks (protrusion of the eye) and displacement of the globe [1]. Because the malignancy is predominantly located in the superonasal area, the globe is displaced anteroinferiorly in the majority of cases [1]. If nasal spread or nasopharyngeal localization occurs, chronic otitis or nasal obstruction can be observed [4]. On the other hand, headaches and cranial nerve palsies indicated cranial and parameningeal extension, respectively [4]. 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 [1].

• Burkina Faso

  Burkina Faso : Bobo-Dioulasso, Ouagadougou, etc. Burundi : Bujumbura, etc. Cambodia : Angkor, Battambang, Kampot, Kep, Phnom Penh, Siem Reap, Sihanoukville, etc. Cameroon : Bafoussam, Bamenda, Douala, Garoua, Kribi, Limbe, Maroua, Yaoundé, etc. [maria-online.com]

• Normal Stature

  Gonadal development was normal. Statural growth was retarded in 61% of the patients. All children were in school, with five having learning or behavioral problems. One child developed acute myeloblastic leukemia. [ncbi.nlm.nih.gov]

• Bony Swelling

  There was no lymphadenopathy or any other bony swellings. Hemogram, differential count, bone marrow examination and chest X-ray were normal. Bone scan revealed uptake in right orbit and left mandible. [cancerjournal.net]

• 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]

• Behavior Problem

  All children were in school, with five having learning or behavioral problems. One child developed acute myeloblastic leukemia. [ncbi.nlm.nih.gov]

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 [1] [4]. 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 [1] [5] [6]. 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 [1] [5] [6]. 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 [1].

• Increased Bone Density

  X-ray findings showed increased bone density in two cases (Cases 3 and 4) and bony erosion in one case (Case 1). [indianjcancer.com]

• Anisocytosis

  Laboratory investigations showed an erythrocyte sedimentation rate of 70 mm/h westergreen, white blood cells 5.6 × 10 9, neutrophils 44%, lymphocytes 51%, monocyte 0.2% and eosinophils 0.3%, presence of anisocytosis, hypochromatosis, and adequate platelets [jmedtropics.org]

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 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]

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 main outcome measures are a rare cause of proptosis in an adult, discussion on treatment options and prognosis of rhabdomyosarcoma. Result: The patient underwent total orbital exenteration and was referred for radiotherapy and chemotherapy. [nepjol.info]

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%. [5] Etiology RMS initially was believed to originate directly from striated muscle. [eyewiki.aao.org]

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]

1. Jurdy L, Merks JHM, Pieters BR, et al. Orbital rhabdomyosarcomas: A review. Saudi J Ophthalmol. 2013;27(3):167-175.
2. Turner JH, Richmon JD. Head and neck rhabdomyosarcoma: a critical analysis of population-based incidence and survival data. Otolaryngol Head Neck Surg. 2011;145:967–973.
3. Koopman JH, van der Heiden-van der Loo M, van Dijk MR, Bijlsma WR. Incidence of primary malignant orbital tumours in the Netherlands. Eye (Lond). 2011;25(4):461-465.
4. Freling NJM, Merks JHM, Saeed P, et al. Imaging findings in craniofacial childhood rhabdomyosarcoma. Pediatr Radiol. 2010;40(11):1723-1738.
5. Boparai MS, Dash RG Clinical, ultrasonographic and CT evaluation of orbital rhabdomyosarcomas with management. Indian J Ophthalmol. 1991;39:129–131.
6. 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.