Perineal Myxoid Liposarcomas: A Case Report and Literature Review
Journal name: The Malaysian Journal of Medical Sciences
Original article title: Perineal Myxoid Liposarcomas: A Case Report and Literature Review
The Malaysian Journal of Medical Sciences (MJMS) is a peer-reviewed, open-access journal published online at least six times a year. It covers all aspects of medical sciences and prioritizes high-quality research.
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Original source:
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Prabhu Ramasamy, Azmi Md Nor, Nil Amri M Kamil, Norra Harun, Mubarak M Yusof, Mohd Zailani Md Hassan
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The Malaysian Journal of Medical Sciences:
(A peer-reviewed, open-access journal)
Full text available for: Perineal Myxoid Liposarcomas: A Case Report and Literature Review
Year: 2010
Copyright (license): CC BY 4.0
Summary of article contents:
Introduction
Myxoid liposarcoma (MLS) is a significant subtype of liposarcoma, accounting for 30%–50% of all cases, and is typically located in the extremities and retroperitoneum. However, this report details a rare case of MLS in a 49-year-old male patient, where the tumor presented as a sizable perineal swelling affecting the para-rectal and para-anal regions. MLS, known for its potential malignant behavior, requires careful diagnosis, management, and prognosis evaluation to guide therapeutic approaches.
Importance of Surgical Intervention
Surgery remains the primary treatment for MLS, emphasizing the necessity of sufficient surgical margins to reduce the risk of local recurrence. In this case, the tumor was excised through a perineal approach, revealing a well-encapsulated, multi-loculated mass. The histological examination confirmed the diagnosis of MLS, characterized by a myxoid matrix and signet-ring lipoblasts. Post-operative radiation therapy was implemented due to close resection margins, highlighting that adequate pre-operative imaging and assessment are critical in surgical planning. Factors such as tumor size, location, and proximity to vital structures play a key role in determining treatment strategies and subsequent patient outcomes.
Conclusion
The management of myxoid liposarcoma requires a comprehensive understanding of its clinical presentation and the surgical techniques necessary for effective treatment. Proper pre-operative evaluation is essential for planning and executing successful surgical interventions, as the quality of surgical margins significantly impacts prognosis. With diligent follow-up and potential adjuvant therapy, the likelihood of local recurrence and metastasis can be effectively monitored and managed, ultimately improving patient survival outcomes.
FAQ section (important questions/answers):
What is myxoid liposarcoma and where does it commonly occur?
Myxoid liposarcoma (MLS) is a subtype of liposarcoma, accounting for 30-50% of these tumors. It primarily occurs in the extremities and retroperitoneum, though it can present unusually in locations like the perineum.
What are the typical symptoms of myxoid liposarcoma?
Patients with myxoid liposarcoma usually present with painless swellings that gradually increase in size. Symptoms may not appear until the tumor becomes quite large, and some patients might experience mild discomfort.
How is myxoid liposarcoma diagnosed and treated?
Diagnosis often requires imaging techniques such as CT or MRI. Surgery is the main treatment, where a wide excision of the tumor is performed. Adjuvant radiotherapy is provided if surgical margins are inadequate.
What is the prognosis for patients with myxoid liposarcoma?
The prognosis depends on surgical margins and tumor characteristics. Local recurrence occurs in 40%-50% of cases, while metastasis rates are about 20%-70%. Survival rates are approximately 80% at 5 years and 50% at 10 years.
Glossary definitions and references:
Scientific and Ayurvedic Glossary list for “Perineal Myxoid Liposarcomas: A Case Report and Literature Review”. This list explains important keywords that occur in this article and links it to the glossary for a better understanding of that concept in the context of Ayurveda and other topics.
1) Swelling:
Swelling refers to the enlargement or mass formation observed in the patient's perineal region, presenting as a significant clinical symptom. In the case report, the swelling progressed from a small size to a large mass that compressed nearby structures. Monitoring such symptoms aids in diagnosing soft tissue tumors like myxoid liposarcoma.
2) Arrow:
The term 'arrow' is used in the context of figures presented in the computed tomography images. An arrow indicates specific areas of interest, such as the tumor compressing anatomical structures like the rectum and anus. This visual indication is crucial for understanding the tumor's extent and the implications for surgical intervention.
3) Disease:
Disease refers to abnormal conditions affecting tissues and organs, manifesting through symptoms and clinical signs. Myxoid liposarcoma is a rare form of soft tissue sarcoma, and understanding the disease dynamics, including presentation, behavior, and treatment, is vital for effective patient management and prognosis.
4) Cancer:
Cancer denotes a broad category of diseases involving uncontrolled cell growth. Myxoid liposarcoma is a subtype of sarcoma, which is itself a type of cancer affecting soft tissues. Recognizing the cancer nature of liposarcomas is essential for determining treatment protocols and potential outcomes for affected patients.
5) Chemotherapy:
Chemotherapy involves the use of chemicals to treat cancer, aiming to kill or inhibit the growth of cancer cells. In the case of myxoid liposarcoma, chemotherapy might be considered for metastatic disease, although its use in non-metastatic cases is often debated. The treatment strategy largely depends on specific patient conditions.
6) Radiotherapy:
Radiotherapy employs high-energy radiation for the treatment of cancer, particularly effective after surgical resection in cases where margins are inadequate. In this case of myxoid liposarcoma, adjuvant radiotherapy was utilized to minimize the risk of local recurrence following surgery, showcasing its importance in treatment planning.
7) Table:
Table in the text refers to organizing data systematically, particularly the American Joint Committee on Cancer (AJCC) staging system. Such tables clarify the classification of soft tissue sarcomas, helping clinicians assess prognosis based on tumor size, grade, and metastasis, thereby guiding treatment decisions.
8) Reliability:
Reliability pertains to the consistency and dependability of the staging and grading systems used for cancer, such as the AJCC system mentioned in the text. Reliable assessment tools are vital for determining a patient's treatment plan and forecast, influencing clinical outcomes significantly.
9) Incision:
Incision denotes the act of cutting into tissue to access underlying structures during surgery. In myxoid liposarcoma treatment, the surgical incision is strategically planned to ensure adequate tumor removal while preserving vital surrounding tissues, which is crucial for successful patient recovery and preventing complications.
10) Quality:
Quality refers to the standard of surgical margins achieved during tumor resection in the case of myxoid liposarcoma. High-quality margins correlate with reduced local recurrence rates and improved patient prognosis, emphasizing the importance of meticulous surgical technique in oncological procedures.
11) Account:
Account in this context refers to the need for comprehensive consideration of various clinical factors when diagnosing and treating liposarcoma. This includes evaluating imaging results, patient symptoms, and tumor characteristics to inform decision-making, ultimately improving treatment efficacy and enhancing patient outcomes.
12) Surface:
Surface refers to the anatomical boundaries and outer aspects of tissues involved in disease processes. In the context of the study, understanding the tumor's relationship with adjacent surfaces is crucial for determining the extent of disease, planning surgical approaches, and assessing potential complications.
13) Thomas:
Thomas is part of a citation referring to an author involved in research on myxoid liposarcoma. Including reputable references strengthens the study's credibility by aligning findings with established scientific evidence, enhancing the understanding of this rare subtype of liposarcoma and its management.
14) Water:
Water is metaphorically referenced to describe the imaging characteristics of myxoid liposarcoma on CT and MRI scans. The low fat content causes the tumor to mimic the appearance of water, complicating diagnostic imaging. This metaphor underscores the need for careful interpretation of imaging studies.
15) Field:
Field refers to the area of investigation or focus, particularly in histological examination. In the case report, it describes the cellular composition of the tumor within observed areas, giving insight into the tumor's characteristics and behavior, crucial for accurate diagnosis and treatment planning.
16) Raven (Ravena):
Raven is another citation reference, indicating authorship of a significant source on cancer staging and management discussions. The acknowledgment of credible textual references fosters confidence in the study's findings and situates them within the broader scientific dialogue on sarcomas and patient care.
17) Rich (Ṛch):
Rich describes the abundance or complexity of components within the myxoid stroma of the tumor. The presence of delicate, arborizing capillary vasculature contributes to the characteristic 'chicken wire' appearance of myxoid liposarcoma, which aids pathologists in accurate diagnosis.
18) Pain (Paiṇ):
Pain in this text refers to the symptom reported by the patient associated with the swelling. Although myxoid liposarcoma commonly presents as a painless mass, any pain must be assessed regarding tumor size and involvement with adjacent structures, influencing treatment considerations and patient information.
19) Post:
Post refers to the situation or treatment that occurs after a primary procedure. In the article, it pertains to adjuvant therapies like radiotherapy following surgical intervention for myxoid liposarcoma, emphasizing the importance of continued care in managing patient prognosis and preventing local recurrence.
20) Male (Mālē):
Male denotes the gender of the patient in the case report, which can be relevant when examining epidemiological data and understanding sarcoma distributions across different demographics. Gender may influence the incidence, presentation, and outcomes of cancers like myxoid liposarcoma.
Other Health Sciences Concepts:
Discover the significance of concepts within the article: ‘Perineal Myxoid Liposarcomas: A Case Report and Literature Review’. Further sources in the context of Health Sciences might help you critically compare this page with similair documents:
Perineum, Differential diagnosis, Clinical examination, Surgery, Clinical presentation, Long-term follow-up, Histological examination, Clinical Follow-Up, Adjuvant therapy, Mesenchymal cells, Magnetic resonance imaging, Digital rectal examination, Malignant behaviour, Metastasis, Cystic lesion, Computed tomography, Perineal swelling, Soft Tissue Tumour, Wide excision, Metastatic disease, Metastatic potential, Tumor characteristics, Local recurrence, Histological grade, Tumour volume, Histological typing, Pre-operative evaluation, Distant metastasis, Surgical planning, Department of Surgery, International Islamic University Malaysia, Adjuvant radiation therapy, Soft tissue sarcoma, Clinical behavior, Soft tissue tumor, Oncology, Myxoid stroma, Retroperitoneum, Radiological diagnosis, Tumour bed, American Joint Committee on Cancer, Neoadjuvant therapies, Surgical margin, Multimodality management, High grade tumour, Computed tomography images, Tumour characteristic, Pelvic organ, Solid masses.