Ewing Sarcoma of Femur
Description
● Patient History & Profile
➣Age/Sex: boy, 10 years old
➣Chief complaint:
•The main complaint is soreness in the right thigh for a few weeks.
➣Medical history:
•began as a dull, sporadic discomfort and progressed to excruciating, ongoing agony.
•Nighttime pain is worse (7/10 morning → 9/10 evening).
•No alleviation from NSAIDs
•No past trauma
•Non-contributory family history
➣clinical examination:
•Vital signs observed is stable (T 36°C, HR 90, BP 110/70, RR 14)
➣Local examination:
•Right thigh swelling and severe soreness
•felt firm mass that is connected to bone
➣Other systems:
•Abdominal, pulmonary, and cardiac normal
● Investigations Using Radiology
➣X-ray femur:
•permeative pattern, cortical-based lesion
•broad transitional zone, richness of soft tissues
•No discernible periosteal response
➣Doppler ultrasonography:
•substantial heterogeneous solid mass in soft tissue
•There is internal vascularity.
➣MRI:
•Osteous lesion affecting the femur's diaphysis
•Cortical erosion, periosteal response
•Large soft tissue component that enhances
● Histopathology (Results of Biopsy)
➣Microscopy:
•Sheets of uniformly sized, tiny, spherical blue cells
•Lack of cytoplasm and dark nuclei
•Frequently occurring mitotic figures
➣Immunohistochemistry:
•FLI-1, Cytokeratin, Positive → CD99 (diffuse membrane), Negative → lymphoid markers, MyoD1
➣Molecular genetics:
RT-PCR reveals the EWSR1/FLI-1 fusion gene (t(11;22)) → diagnostic
➣Diagnosis:
Diaphyseal femoral lesion, or Ewing Sarcoma
●Pathological Features of Ewing Sarcoma
➣Gross:
•Haemorrhage, necrosis, soft gray/tan-white mass, and cortical penetration into soft tissue and the periosteum
•Under a microscope, tiny, spherical, blue cells with sheets or nests, little cytoplasm, and uniformly spaced chromatin
•Unique characteristics: Cytoplasm rich in glycogen (PAS positive), perhaps Rosettes after Homer-Wright
●Clinical Features (General)
•Localised pain and swelling (pain at night that NSAIDs can't cure)
•Tender, firm, and bone-attached palpable mass
•Joint motion limitation, erythema, and swelling
•Systemic symptoms include fever, exhaustion, anaemia, and weight loss (10–20% of cases).
•can resemble leukocytosis, fever, and discomfort associated with osteomyelitis.
● Factors at Risk and Epidemiology
• Age: peak adolescence, 80% under 20
•Sex: 1.4:1 Male > Female
•White ethnicity > African and Asian
•Incidence in the West: 1-3 million per year
•6–10% of initial malignant bone tumours are caused by it.
Protocol
●Management
• Multimodal therapy is essential due to early micrometastases.
•Neoadjuvant chemotherapy is used to treat micrometastases and reduce tumours.
•Typical routines:
Vincristine, Cyclophosphamide (VDC), Doxorubicin, and Ifosfamide alternate with Etoposide (IE).
•Surgery: Resection of the affected limb is preferable to amputation.
•Adjuvant chemotherapy:
to eliminate any remaining illness. If the margins are positive, the tumour cannot be removed, or there are spinal or pelvic abnormalities, radiotherapy is employed.
●Nursing and Management Interventions:
•Hold frequent training sessions to enhance knowledge of the nursing process and its documentation.
•To streamline and expedite the process, establish and implement standard documentation processes utilising EHR (Electronic Health Record) technology.
•To emphasise proper techniques, set up seminars with case studies and hands-on activities.
•Nurses find it challenging to provide individualised care and adhere to the nursing process due to heavy patient loads and time constraints.
•To increase nurse efficiency, implement shift planning software and time-management systems.
•To make the most use of nurses' time, assign duties or hire support workers.
•assigning support staff or nursing assistants to non-essential duties so that licensed nurses may focus on providing direct patient care.
●Physician Domination in Decision-Making
Nurses' autonomy in patient care planning is often restricted by management systems that prioritise physician-dominated decision-making.
➣ Nursing and Management Interventions:
• Encourage a cooperative workplace where nurses' opinions are respected during care planning sessions.
•Revise hospital regulations to enable nurses to take the initiative and, when necessary, lead autonomous interventions.
•To promote nursing responsibilities in multidisciplinary teams, train nurse leaders.
●Education versus Variable Clinical Practice
Confusion and a decline in the quality of treatment can result from the disconnect between what nurses learn in school and what they actually do in clinics.
➣Nursing and Management Interventions:
•Update clinical guidelines often to take into account the most recent evidence-based procedures.
•Plan seminars and bridging programs to bring clinical procedures into line with modern teaching methods.
•Assemble groups of academic and clinical specialists to offer guidance and ongoing professional growth.
●Specific Nursing Interventions
The following are some issues that are often encountered and associated nurse interventions:
➣ Pain management involves using instruments for measuring pain, giving prescription drugs, and teaching coping mechanisms such guided relaxation or meditation.
➣Chronic disease management involves keeping an eye on vital signs, making ensuring that medications are taken as prescribed, educating patients about lifestyle modifications, and offering dietary and exercise resources.
➣Postoperative Recovery: Support nutrition, assist with movement, give bedside care, and keep an eye on the patient's progress.
➣Education and Family Support: Educate families on patient care and provide them consolation as they heal.
Notes
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