Rare Presentation of Pseudomyxoma Retroperitonei: Stretching the Limits

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Journal name: The Malaysian Journal of Medical Sciences
Original article title: Rare Presentation of Pseudomyxoma Retroperitonei: Stretching the Limits
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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Author:

Roumina Hasan, Sandeep Kumar, Anuradha ck Rao, Rajagopal Kadavigere


The Malaysian Journal of Medical Sciences:

(A peer-reviewed, open-access journal)

Full text available for: Rare Presentation of Pseudomyxoma Retroperitonei: Stretching the Limits

Year: 2016 | Doi: 10.21315/mjms2016.23.4.11

Copyright (license): CC BY 4.0


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Summary of article contents:

Introduction

Pseudomyxoma retroperitonei (PMR) is an infrequent condition characterized by a mucinous collection within the retroperitoneal space, which has only been documented in 45 reported cases in the English literature. The most common clinical presentation of PMR includes an abdominal mass accompanied by pain, although some instances have manifested as spontaneous cutaneous fistulae. In this case study, we present a unique presentation of PMR, where a patient developed a non-healing cutaneous sinus in the right supragluteal region, initially misdiagnosed as a gluteal abscess. Imaging studies revealed an extensive retroperitoneal cystic mass, and treatment involved surgical intervention.

The Importance of Imaging in Diagnosis

A significant aspect of managing PMR is the utilization of imaging techniques, such as MRI and CT scans, which play a crucial role in diagnosis and surgical planning. In this case, MRI revealed a multiloculated retroperitoneal cystic lesion demonstrating distinct signal characteristics — hypointense on T1-weighted images and hyperintense on T2-weighted images, along with the observation of a fistulous tract leading to the skin. These imaging findings were integral in distinguishing PMR from other conditions, as the structural features help to identify the primary pathology, allowing for accurate preoperative assessments. Ultimately, imaging guided the decision for an explorative laparotomy, leading to an appendectomy and successful management of the disease.

Conclusion

This case underscores the rare occurrence of pseudomyxoma retroperitonei presenting as a non-healing sinus, emphasizing the importance of vigilance among clinicians in evaluating atypical presentations. Awareness of PMR's possible manifestations is essential, especially when dealing with chronic sinus formations that may seem innocuous at first. Furthermore, thorough imaging studies are crucial for accurate diagnoses and management decisions in cases where PMR is suspected, as they provide clarity regarding the nature of the lesions and the involvement of adjacent structures. In this instance, effective surgical intervention resulted in the patient being disease-free at the six-month follow-up, highlighting the potential for favorable outcomes when PMR is adequately diagnosed and treated.

FAQ section (important questions/answers):

What symptoms did the 55-year-old woman present with?

The patient presented with a mucopurulent discharge from the right supragluteal region and symptoms that had persisted for five months, eventually leading to the diagnosis of a gluteal abscess.

What imaging studies were performed for diagnosis?

A CT scan and an MRI of the abdomen were performed, revealing a large heterogeneous retroperitoneal cystic mass, a tubular projection affecting the ileocaecal junction, and a fluid-filled cutaneous fistulous tract.

What was the final diagnosis after treatment?

The final diagnosis confirmed via histopathology was appendiceal mucinous cystadenoma with pseudomyxoma retroperitonei after an exploratory laparotomy and appendectomy.

What is pseudomyxoma retroperitonei and its common origin?

Pseudomyxoma retroperitonei is a rare condition characterized by mucinous collections in the retroperitoneum, primarily of appendiceal origin, although other sites like ovaries and colon can also be involved.

Glossary definitions and references:

Scientific and Ayurvedic Glossary list for “Rare Presentation of Pseudomyxoma Retroperitonei: Stretching the Limits”. 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) Arrow:
An arrow represents direction, often used in imaging studies to indicate the location or extension of anatomical structures or lesions. In medical imaging, arrows help the clinician and radiologist pinpoint areas of interest, making it easier to identify abnormalities such as cysts or tumors, guiding treatment and diagnosis.

2) Disease:
A disease is a pathological condition characterized by various signs and symptoms negatively impacting bodily functions. In the context of this study, diseases like pseudomyxoma retroperitonei exemplify rare manifestations that complicate diagnosis and treatment, highlighting the importance of accurate identification and timely intervention.

3) Table:
Tables in medical literature often summarize data or findings, providing readers with organized and concise information. They facilitate comparisons and highlight key points regarding demographics, symptoms, or laboratory results, making it easier for clinicians and researchers to interpret the relevance of the study's findings.

4) Post:
In medical discussions, 'post' often refers to events that occur after a certain procedure or intervention. For instance, 'post-operative' indicates observations or complications following surgery, which are crucial for assessing patient recovery, outcomes, and potential future interventions or follow-ups in medical studies.

5) Swelling:
Swelling is a common clinical sign characterized by an increase in size due to fluid accumulation or inflammation. It can signify various pathologies, including infections or abscesses, thereby serving as an important diagnostic criterion for clinicians evaluating patients and determining further diagnostics or treatments.

6) Pain:
Pain is a subjective experience that indicates tissue damage or underlying pathology, often prompting medical evaluation. In this context, pain associated with pseudomyxoma retroperitonei helps clinicians understand the severity of a patient's condition, guiding the necessity for intervention and treatment options based on reported symptoms.

7) Chemotherapy:
Chemotherapy refers to the use of drugs to treat cancer by killing or inhibiting growth in cancer cells. It becomes relevant in discussions of tumor types like cystadenocarcinoma, where the patient's potential need for chemotherapy post-surgery can determine their treatment plan and prognosis in a study.

8) Study (Studying):
In medical literature, a 'study' typically refers to a systematic investigation aimed at discovering new information, evaluating a hypothesis, or understanding the implications of a health condition. This aspect is vital for advancing medical knowledge, informing practice, and guiding patient care, particularly in rare diseases.

9) Wall:
In medical imaging, 'wall' often pertains to the structural lining of organs or lesions observed via scans like MRI or CT. Assessment of the thickness, integrity, and enhancement characteristics of the wall is crucial for distinguishing between benign and malignant pathologies, impacting diagnostic conclusions.

10) Activity:
Activity in this medical context may refer to cellular or metabolic functions that can indicate disease presence or progression. In the study of pseudomyxoma retroperitonei, assessing the pathological activity in tissues can reveal important information regarding the aggressiveness of tumors and intervention strategies.

11) Male:
The term 'male' is relevant in medical diagnostics and epidemiology, particularly when discussing disease prevalence, risk factors, and manifestations. In the context of pseudomyxoma retroperitonei, understanding gender differences helps the clinician tailor patient assessments and consider varying presentations of this often-theorized rare condition.

12) Radiotherapy:
Radiotherapy uses high-energy radiation to kill cancer cells, often discussed alongside chemotherapy as a treatment modality. In managing conditions like malignant appendiceal tumors, understanding the role and timing of radiotherapy is crucial for optimizing care post-surgery, impacting overall survival and quality of life.

13) Discussion:
In scientific literature, the discussion section interprets study findings, exploring their significance, limitations, and broader implications. This segment is vital for contextualizing results, guiding future research trajectories, and providing actionable insights for clinical practice, especially for complex conditions like pseudomyxoma retroperitonei.

14) Antibiotic (Antibacterial):
Antibiotics are medications used to treat bacterial infections. Their role is significant in managing secondary infections or complications, as illustrated in this study where the patient's prior antibiotic treatments for the suspected gluteal abscess might have influenced the underlying condition's progression and diagnosis.

15) Dressing:
Dressings are materials applied to a wound to promote healing and prevent infection. In post-operative care, as noted in the study, the choice and management of dressings can significantly affect recovery, particularly in cases involving chronic wounds or surgical sites influenced by underlying pathologies.

16) Account:
In a medical context, 'account' can refer to a description or summary of a patient's symptoms, diagnosis, and treatment plan. It is essential for comprehensive care and documentation, ensuring all healthcare professionals involved have a clear understanding of the case's context and clinical progression.

17) Filling (Filled):
Filled typically indicates an area occupied by a substance, relevant in diagnostics where lesions or cysts are assessed based on their contents. In pseudomyxoma retroperitonei, the filling with mucinous material is a critical characteristic for radiologists to identify the condition’s nature and severity.

18) Cancer:
Cancer encompasses a range of diseases marked by aberrant cell growth. Its recognition as a potential outcome of conditions like pseudomyxoma retroperitonei underscores the importance of diagnostic vigilance, particularly in determining treatment options and comprehensively managing patient care and expectations regarding their health.

19) Water:
In medical imaging, water density often serves as a reference for evaluating the attenuation of lesions or cystic structures. Understanding the fluid composition in various lesions helps distinguish between benign and malignant processes, crucial in forming accurate diagnoses and management plans.

20) Fever:
Fever is a common systemic response indicating infection or inflammation, serving as a vital sign in clinical assessments. In this study, the presence of fever informs the underlying disease process and assists clinicians in evaluating the severity of the patient's condition and necessary interventions.

21) Bile:
Bile is a digestive fluid that can play a role in certain abdominal pathologies. While not directly implicated in pseudomyxoma retroperitonei, understanding bile's function may assist in evaluating related conditions affecting the biliary tract, adding context to a patient's overall digestive health.

22) Sign:
In the clinical context, a 'sign' refers to an objective indication of a medical condition observable during examination. Recognizing signs in pseudomyxoma retroperitonei can guide diagnostic processes, treatment plans, and overall patient management, serving as crucial components in clinical decision-making.

Other Science Concepts:

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Discover the significance of concepts within the article: ‘Rare Presentation of Pseudomyxoma Retroperitonei: Stretching the Limits’. Further sources in the context of Science might help you critically compare this page with similair documents:

Mri, Intermittent fever, Antibiotic treatment, CT scan, Systemic examination, Laboratory investigation, C-Reactive Protein, Histopathological examination, Histopathology, Magnetic resonance imaging, WBC count, Spinal anaesthesia, Fistulous tract, Appendectomy, Gluteal abscess, Computed tomography, T1 weighted image, T2 weighted image, Prognostic classification, Asymptomatic, Purulent discharge, CA-125 levels, Ileocaecal junction, Cystic degeneration, Abdominal mass, Cystic mass, Explorative laparotomy, Surgical evacuation, Preoperative Diagnosis, Leucocytosis, Low grade dysplasia, Neutrophilia, Soft tissue swelling, Survival outcomes.

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