Spindle cellEmbryonal rhabdomyosarcomaTumorsHead and neck rhabdomyosarcomaAlveolarOrbitAnaplastic rhabdomyosarcomaMetastaticTumorCarcinomaMesenchymalBenignLymphomaCongenitalArisesMetastasesPrognosisEyelidsImmunohistochemicalMetastasisBiopsySurvival ratesExtentPrimarySignsTissueInfectiousPatientTreatmentLesionPatientsCasesSitesResultsLargePain
Spindle cell1
- The patient was diagnosed with orbital spindle cell rhabdomyosarcoma and was referred to the Wills Eye Hospital Ocular Oncology service as well as the Thomas Jefferson University Hospital Otolaryngology and Hematology/Oncology departments. (reviewofophthalmology.com)
Embryonal rhabdomyosarcoma3
- Embryonal rhabdomyosarcoma is evidenced by a variable cell population consisting of small, round tumor cells with hyperchromatic nuclei and of large, polygonal-shaped tumor cells with abundant eosinophilic cytoplasm, which often contains diagnostic cross striations (arrow). (medscape.com)
- Embryonal rhabdomyosarcoma is the most common subtype observed in children, accounting for approximately 60% of all cases in this age group. (medscape.com)
- Embryonal rhabdomyosarcoma usually arises in the superonasal orbit and thus produces inferolateral globe displacement. (reviewofophthalmology.com)
Tumors11
- Tumors in the eye and orbit can be benign like dermoid cysts, or malignant like rhabdomyosarcoma and retinoblastoma. (wikipedia.org)
- The indications for orbital exenteration can be divided into four general categories: tumors of the eyelid or conjunctiva for which salvage of the globe is not possible, intraocular tumors with extension to the orbital soft tissue, orbital extension of tumors of the paranasal sinuses or nasal cavity, and orbital extension from intracranial processes. (entokey.com)
- Orbital exenteration may not be appropriate in patients with widespread metastatic disease, patients with tumors expected to have a high likelihood of local-regional recurrence despite orbital exenteration, elderly patients with multiple medical comorbidities, or patients with a short life expectancy. (entokey.com)
- Heart tumors (HT) are uncommon in companion animals, accounting for roughly 0.2% of all neoplasms. (biomedcentral.com)
- Tumors affecting the orbit include benign and malignant neoplasms arising from the various structures in the orbit including the blood vessels, nerves, bones, orbital fat and other soft tissues. (cybersight.org)
- The first step in the management of orbital tumors is to differentiate the malignant tumors from the benign ones. (cybersight.org)
- Complete surgical excision remains the primary treatment of choice for well-defined, clinically benign orbital tumors. (cybersight.org)
- And I'll be speaking to you today about logical approach to orbital tumors. (cybersight.org)
- Orbit is supposed to be Pandora's Box, full of evil because 50% of orbital tumors are supposed to be malignant, and hence potentially life threatening. (cybersight.org)
- So, when we deal with orbital tumors, the need is to have a logical approach where oncological principles are respected. (cybersight.org)
- Before I go into it, orbital tumors are primarily classified as benign and malignant primary tumors or secondary tumors. (cybersight.org)
Head and neck rhabdomyosarcoma3
- In a retrospective study of 28 pediatric patients with head and neck rhabdomyosarcoma published in 2018, Häußler et al found the 5-year overall survival rate to be 91.3%, with the median period of progression-free survival reported to be 46 months. (medscape.com)
- Pediatric head and neck rhabdomyosarcoma: An analysis of treatment and survival in the United States (1975-2016). (nih.gov)
- Clinicopathological analysis of head and neck rhabdomyosarcoma: A series of 10 cases and literature review. (nih.gov)
Alveolar1
- UCP1 was variably expressed by tumour cells in a few non-adipose sarcomas including leiomyosarcoma, rhabdomyosarcoma, alveolar soft part sarcoma, synovial sarcoma and clear cell sarcoma. (biomedcentral.com)
Orbit5
- A malignant mesenchymal neoplasm that arises from the orbit. (nih.gov)
- Orbital exenteration is the surgical removal of the contents of the orbit and the periorbital soft tissue, including the eye, extraocular muscles, optic nerve, periorbita, orbital adipose tissue, eyelids, and periorbital skin. (entokey.com)
- The most common indication for orbital exenteration is a malignancy of the eye, orbit, ocular adnexa, or periocular region for which salvage of the globe is not possible. (entokey.com)
- During the physical examination in a patient being considered for orbital exenteration, the tumor should be carefully evaluated, and its exact location within the orbit, ocular adnexal structures, paranasal sinuses, or nasal cavity should be noted. (entokey.com)
- When an "eyelid-sparing" orbital exenteration may be possible, the skin of the upper and lower eyelids should be carefully examined, and the degree of laxity and redundant skin should be noted as this skin will be used to line the orbit. (entokey.com)
Anaplastic rhabdomyosarcoma1
- Finally, the classic pleomorphic variant is now a subset within the anaplastic rhabdomyosarcoma category used in modern classification systems. (reviewofophthalmology.com)
Metastatic2
- Prognosis of children and young adults with newly diagnosed rhabdomyosarcoma metastatic to bone marrow treated on Children's Oncology Group studies. (stanfordchildrens.org)
- The differential diagnosis should include rhabdomyosarcoma, metastatic neuroblastoma and lymphomas. (blogspot.com)
Tumor5
- Eye neoplasms can affect all parts of the eye, and can be a benign tumor or a malignant tumor (cancer). (wikipedia.org)
- Rhabdomyosarcoma is a malignant tumor of striated muscle origin. (medscape.com)
- They may range from highly differentiated neoplasms containing rhabdomyoblasts with large amounts of eosinophilic cytoplasm and cross striations similar to that of poorly differentiated tumor cells (see the image below). (medscape.com)
- Rhabdomyosarcoma (RMS) is a soft tissue tumor derived from mesenchymal tissue with myogenic differentiation and associated with the embryogenesis of striated muscle. (biomedcentral.com)
- When you have a patient with orbital tumor, we have to ask these questions and answer ourselves. (cybersight.org)
Carcinoma1
- Orbital exenteration can be required for treatment of squamous cell carcinoma, basal cell carcinoma, sebaceous carcinoma, conjunctival melanoma, uveal melanoma with extrascleral or orbital extension, epithelial cancers such as adenoid cystic carcinoma of the lacrimal gland, rhabdomyosarcoma, and other rare sarcomas. (entokey.com)
Mesenchymal1
- Rhabdomyosarcoma is a neoplasm that develops from undifferentiated mesenchymal cells that have the capacity to differentiate into striated muscle. (reviewofophthalmology.com)
Benign3
- Orbital dermoid cysts are benign choristomas which are typically found at the junction of sutures, most commonly at the fronto-zygomatic suture. (wikipedia.org)
- The differential diagnosis also included infectious and neoplastic disorders such as cellulitis, primary bone lesions (benign or malignant), lymphoproliferative or vascular disorders, and secondary neoplasms including sinus-related lesions or distant metastases. (reviewofophthalmology.com)
- Malignant neoplasms show a greater degree of anaplasia and have the properties of invasion and metastasis, compared to benign neoplasms . (lookformedical.com)
Lymphoma2
- The most common orbital malignancy is orbital lymphoma. (wikipedia.org)
- Most patients with orbital lymphoma can be offered chemotherapy or radiation therapy. (wikipedia.org)
Congenital1
- A case of congenital rhabdomyosarcoma. (nih.gov)
Arises1
- The second neoplasm may have the same or different histological type and can occur in the same or different organs as the previous neoplasm but in all cases arises from an independent oncogenic event. (lookformedical.com)
Metastases1
- Abnormal growths of tissue that follow a previous neoplasm but are not metastases of the latter. (lookformedical.com)
Prognosis1
- Treatment and prognosis of orbital non-Hodgkin's lymphomas. (uchicago.edu)
Eyelids2
- Exenteration - Removal of the eye, all orbital contents, which can involve the eyelids as well. (wikipedia.org)
- Erythema of the upper and lower eyelids did not extend beyond the orbital rim. (contemporarypediatrics.com)
Immunohistochemical1
- The immunohistochemical staining pattern confirmed rhabdomyosarcoma. (biomedcentral.com)
Metastasis1
- A careful examination for signs of local or regional disease spread is essential because orbital exenteration may not be appropriate in patients with regional lymph node or distantorgan metastasis. (entokey.com)
Biopsy1
- The patient underwent an orbital biopsy. (reviewofophthalmology.com)
Survival rates1
- As a result, 5-year survival rates increased from 25% in 1970 to 73%, as shown in the Intergroup Rhabdomyosarcoma Study (IRS)-IV reported in 2001. (medscape.com)
Extent3
- However, computed tomography (CT) scanning, magnetic resonance imaging (MRI), or both may be indicated to evaluate the surgical anatomy and to determine the presence and extent of rhinosinusitis, foreign bodies, and neoplasms. (medscape.com)
- Having a thorough knowledge on the orbital anatomy and detailing out the size and extent of the lesion using pre-operative imaging can help the surgeon decide on the right approach. (cybersight.org)
- Methods which attempt to express in replicable terms the extent of the neoplasm in the patient. (lookformedical.com)
Primary2
- This is the first description of the primary multiple heart rhabdomyosarcoma in a dog. (biomedcentral.com)
- Transfer of a neoplasm from its primary site to lymph nodes or to distant parts of the body by way of the lymphatic system. (lookformedical.com)
Signs1
- This neoplasm may present with renal signs and symptoms, such as hypertension, hematuria and abdominal pain. (aafp.org)
Tissue5
- [ 1 ] Rhabdomyosarcoma of the head and neck is primarily a disease of the first decade of life, and it is the most common soft tissue sarcoma in childhood. (medscape.com)
- In some cases, not only the orbital and periorbital soft tissue but also the bony orbital walls must be removed. (entokey.com)
- Side-by-side CT coronal images which demonstrate an expansile soft-tissue and osteolytic lesion involving the right zygoma and orbital space. (reviewofophthalmology.com)
- Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. (stanfordchildrens.org)
- Ability of neoplasms to infiltrate and actively destroy surrounding tissue. (lookformedical.com)
Infectious1
- Finally, inflammatory or infectious processes associated with refractory orbital pain (e.g., invasive fungal infections such as mucormycosis) may be indications for orbital exenteration. (entokey.com)
Patient2
- Given the dramatic changes in function and appearance caused by orbital exenteration, counseling of the patient prior to surgery is essential. (entokey.com)
- Although used in the original Intergroup Rhabdomyosarcoma Studies, in recent years this scheme has been modified and adapted by investigating organizations including the National Cancer Institute with the goal of creating a classification system that would better predict patient outcome. (reviewofophthalmology.com)
Treatment3
- Over several decades, great progress has been made in the treatment of rhabdomyosarcoma. (medscape.com)
- Frequency and Clinical Course of Residual Orbital Masses After Treatment of Orbital Rhabdomyosarcoma. (nih.gov)
- The development of the second neoplasm may or may not be related to the treatment for the previous neoplasm since genetic risk or predisposing factors may actually be the cause. (lookformedical.com)
Lesion1
- Each orbitotomy technique is designed to provide the best surgical exposure to the lesion of interest, while causing least damage to the orbital structures, with a goal to achieve optimal functional and cosmetic outcome. (cybersight.org)
Patients3
- Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. (nih.gov)
- In most instances, orbital exenteration is only appropriate for patients in whom the possibility of cure is likely enough to justify this radical procedure. (entokey.com)
- For such patients, palliative radiation therapy, other palliative medical treatments, or various degrees of debulking may offer a better quality of life than orbital exenteration. (entokey.com)
Cases4
- Approximately 90% of all cases of rhabdomyosarcoma are diagnosed in individuals younger than 25 years, and within this group, 60-70% are younger than 10 years. (medscape.com)
- Rhabdomyosarcoma represents 3.5% of all malignancies in children aged 0-14 years, with approximately 250 new cases diagnosed each year. (medscape.com)
- The annual incidence of rhabdomyosarcoma in the United States is 4.5 cases per 1 million children younger than 14 years. (medscape.com)
- In the head and neck, the most common sites of rhabdomyosarcoma are parameningeal and orbital locations, which account for 16% and 9% of all cases of the disease, respectively. (medscape.com)
Sites1
- The neoplasms may be histologically the same or different, and may be found in the same or different sites. (lookformedical.com)
Results1
- Orbital exenteration not only results in loss of the eye and its function but also causes significant facial disfigurement. (entokey.com)
Large1
- Large deep orbital dermoid cysts can cause pressure effects on the muscles and optic nerve, leading to diplopia and loss of vision. (wikipedia.org)
Pain1
- Orbital exenteration may also be indicated for certain nonmalignant neoplasms, such as neurofibromatosis causing severe orbital displacement, immobility, and blindness or extensive lymphangioma causing disfigurement or orbital pain. (entokey.com)