|Year : 2021 | Volume
| Issue : 3 | Page : 129-131
Are common things always common?
Vinod Kumar Viswanathan
Department of Pulmonary Medicine, Government Stanley Medical College, Chennai, Tamil Nadu, India
|Date of Submission||19-Feb-2022|
|Date of Acceptance||20-Mar-2022|
|Date of Web Publication||12-May-2022|
Vinod Kumar Viswanathan
Government Stanley Medical College, Chennai - 600 001, Tamil Nadu
Source of Support: None, Conflict of Interest: None
One axiom that we teach medical students is that “common things are always common” and physicians who make common diagnosis are almost always correct. However, it is also important to keep a watchful eye to identify rare diseases so that a timely diagnosis is made for the patient's benefit and that a disease is identified before it becomes too late. Presented here is a rare case of pleural angiosarcoma in a young male presenting as pleural effusion who was initially thought to have tuberculosis pleural effusion.
Keywords: Angiosarcoma, malignancy, pleural effusion, tuberculosis
|How to cite this article:|
Viswanathan VK. Are common things always common?. J Assoc Pulmonologist Tamilnadu 2021;4:129-31
| Introduction|| |
One basic dictum in medicine is that common diseases are always common. Occam's razor, also known as the principle of parsimony or the law of parsimony, is the problem-solving principle that “the simplest explanation is usually the best one.” This holds true in most situations in clinical practice but one must remember that there will be exceptions to this rule.
To illustrate this point is presented herewith a story of a young male who presented with pleural effusion and assumed to be Koch's pleural effusion due to his age of presentation but the final diagnosis revealed pleural angiosarcoma (AS), a rare disease and all the more rarer due to the patient's age.
| Case Report|| |
This 22-year-old male presented with left-sided chest pain of 4-day duration to a cardiologist and since a pleural effusion was detected during echo, he was referred to the author, a pulmonologist for management. He had no constitutional symptoms of fever or weight loss or loss of appetite nor any past history of any significant illnesses. A plain computed tomography (CT) chest had been done at the time of referral (no X-ray was available with the patient) and was reported as multiloculated pleural effusion of Koch's etiology [Figure 1].
|Figure 1: Noncontrast computed tomography suggestive of multiloculated pleural effusion|
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The patient was admitted for pleural tapping and initiation of anti-tuberculosis treatment at the insistence of the apprehensive parents. Pleural tapping done revealed hemorrhagic pleural effusion. The worried pulmonologist requested the radiologist for a contrast CT study which revealed a pleural based mass in the anterior mediastinum [Figure 2] highlighting the importance of doing contrast study in pleural effusion cases.
Cytological examination of pleural fluid revealed malignant cells and CT-guided biopsy of the pleural mass was reported as vascular neoplasm by histopathology and AS by immunohistochemistry. The patient has been referred to a medical oncologist for the favor of chemotherapy and further management.
| Discussion|| |
Pleural effusion in previously healthy, young adults has always posed a diagnostic problem to the medical profession. The general opinion is that 70%–80% of such effusions represent pleural manifestations of tuberculosis (TB); however, a multiplicity of etiological factors account for the other 20%–30% of pleural effusions. Among them may be listed primary or secondary carcinoma of the lungs, bacterial or viral pneumonia, lymphoblastoma, acute rheumatic fever, nephrosis, panserositis, cirrhosis, lung abscess, congestive heart failure, pulmonary embolism, acute polyarthritis, ovarian neoplasm, blood dyscrasia, infectious mononucleosis, and cholesterol effusion.
AS is a rare and highly aggressive malignant tumor, originating from lymphatic or vascular endothelial cell. It makes up <2% of all soft tissue sarcomas in humans and principally affects adult and elderly patients. As a clinically and genetically heterogeneous subgroup of sarcomas, AS can occur in any location of body. The most common sites of ASs are cutaneous lesions (about 60% of cases), particularly the head and neck, and can also present within the soft tissues, visceral organs, bone, and retroperitoneum.
AS is an easily infiltrative tumor with high rate of local recurrence and metastasis. The reported rates of advanced/metastatic disease at presentation vary from 16% to 44%, and the overall survival ranging from 6 to 16 months.
The pathogenesis of AS has not been fully understood, definite risk factors include chronic lymphoedema, history of radiation, environmental carcinogens (vinyl chloride, thorium dioxide, and arsenic), and several genetic syndromes.
Most diagnostic imaging in AS are non specific. Hence histopathology and immunohistochemistry are the cornerstones of diagnosis in AS.
Immunohistochemical stains have an important role in differential diagnosis. Expression of at least one of the endothelial markers including CD31, CD34, factor VIII, and FLI-1 is required to confirm the diagnosis of AS. Among them, CD31 is considered to be the most sensitive and specific.
Delayed diagnosis and the rarity of these tumors contribute to the difficulties in regarding best treatment and prognostic factors, radical surgery followed by adjuvant radiotherapy is thought the current optimal modality.
Primary pleural angiosarcoma (PPA) is a rare occurrence. Since its first description in 1943, only 39 reported cases of PPA have been published. Patients almost always die of the disease within months. Definite diagnosis is usually not possible using the examination of cytology or small biopsy specimens but often requires that of decortication or resection specimens. The histologic picture of biphasic spindle and epithelioid tumor cells along with immunoreactivity to epithelial markers, such as cytokeratin and CK7, may lead to an erroneous diagnosis of mesothelioma or metastatic sarcomatoid carcinoma. Identifying areas showing vasoformative tendency and immunostains with endothelial markers, such as CD31, CD34, factor VIII, and FLI-1, are diagnostically important.
The treatments of AS include surgical excision, radiotherapy, and/or chemotherapy. However, the clinical course is usually rapidly fatal, regardless of treatment modalities.
This case is presented here to highlight several points including that all pleural effusions in young are not always TB, importance of having a knowledge of rare diseases and rare presentations in medicine, rarity of this case both in terms of etiology and age of presentation of this case.
| Conclusion|| |
Although the axiom common diseases are always common makes sense and is usually correct, the art of medicine and experience teaches one that exceptions to this rule exist and must not be missed. All pleural effusions and all pleural effusions in the young are not always Koch's and malignancy should not be missed out.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]