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                        Fibrous Hamartoma of Infancy

     Dr Sampurna Roy MD

 
 


                    

Primary cardiac lymphoma include those lymphomas originating first in the heart (excluding those involving heart as part of disseminating lymphoma).

Primary cardiac lymphoma is extremely rare in immunocompetent patients. It is usually seen in immunocompromised patients, particularly in association with the acquired immunodeficiency syndrome. They are all high-grade B-cell lymphomas.

Cardiac malignant lymphoma in acquired immune deficiency syndrome. Cancer 1992; 70:2203-2207.

Age: The mean age at presentation is approximately 60 years.

Clinical presentation: Patients may present with dyspnea, congestive heart failure, chest pain, epigastric pain, pericardial tamponade, conduction abnormalities, or arrhythmia. Acute myocardial infarction may be simulated by diffuse myocardial infiltration.

Site: Primary cardiac lymphoma typically involves the right atrium ,as a mass and is often associated with pericardial effusion. Involvement of the other chambers is less common. Most patients have pericardial involvement, but extension into the valves is rare. Gross Image (AFIP)

 REPORTING OF CARDIAC TUMOURS

Type of Primary cardiac lymphomas :  Non Hodgkin's Type : (mostly of B-cell nature) -  Low ;  Intermediate ; High grade

Microscopic diagnosis is based on the same criteria as applied in lymphomas in general.   Image Link1(NEJM) ; Image Link2(AFIP) .

Cardiac lymphomas demonstrate a wide range of histologic types, including well-differentiated B-cell lymphomas, follicular center cell lymphomas, diffuse large cell lymphomas, and undifferentiated Burkitt-like lymphomas. The tumour usually infiltrate the surrounding muscle and epicardial fat . In some cases it may infiltrate the adventitia of the coronary arteries.

REPORTING OF ENDOMYOCARDIAL BIOPSY

Diagnosis of lymphoma after cardiac transplantation should be done carefully, as there are varieties of bizarre lymphocytic proliferations, which may be due to viral infection.

ENDOMYOCARDIAL BIOPSY REPORTING-(ALLOGRAFT REJECTION)

Prognosis is poor and treatment should be as for other aggressive lymphomas.

                 

Abstracts:

Fatal secondary pulmonary hypertension due to cardiac involvement in AIDS-associated Burkitt's lymphoma.Indian J Med Sci. 2006 Sep;60(9):380-4

Paroxysmal atrial flutter caused by cardiac lymphoma. Pacing Clin Electrophysiol. 2006 Jun;29(6):682-4.

Intra-cardiac burkitt's lymphoma mimicking acute pulmonary embolism.
J Coll Physicians Surg Pak. 2006 Aug;16(8):536-7.

Primary cardiac lymphoma: diagnosis and treatment. Report of 6 cases and review of the literature.Arch Mal Coeur Vaiss. 2005 Sep;98(9):875-80

Primary lymphoma of the heart: case report and literature review.Pathol Int. 2004 Mar;54(3):187-95.

Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 31-2003. A 44-year-old man with HIV infection and a right atrial mass.N Engl J Med. 2003;349(14):1369-77.

Primary cardiac lymphoma in immunocompetent patients: a report of three cases and review of the literature.Virchows Arch. 2002 Nov;441(5):456-61. Epub 2002 Sep 27

Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management.Cancer. 1997 Oct 15;80(8):1497-506

Primary cardiac lymphoma.Am J Hematol. 1997 Jan;54(1):79-83

Malignant lymphoma of the heart in acquired immune deficiency syndrome. Chest 1986; 90:243-246.

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