Heart

Protocol applies to all primary cardiac tumors.

Procedures

• Cytology

• Incisional Biopsy

• Excisional Biopsy

This protocol is intended to assist pathologists in providing clinically useful and relevant information as a result of the examination of surgical specimens. Use of this protocol is intended to be entirely voluntary. If equally valid protocols or similar documents are applicable, the pathologist is, of course, free to follow those authorities. Indeed, the ultimate judgment regarding the propriety of any specific procedure must be made by the physician in light of the individual circumstances presented by a specific patient or specimen.

It should be understood that adherence to this protocol will not guarantee a successful result. Nevertheless, pathologists are urged to familiarize themselves with the document. Where a physician chooses to deviate from the protocol based on the circumstances of a particular patient or specimen, the physician is advised to make a contemporaneous written notation of the reason for the procedure followed.

The College recognizes that this document may be used by hospitals, attorneys, managed care organizations, insurance carriers, and other payers. However, the document was developed solely as a tool to assist pathologists in the diagnostic process by providing information that reflects the state of relevant medical knowledge at the time the protocol was first published. It was not developed for credentialing, litigation, or reimbursement purposes. The College cautions that any uses of the protocol for these purposes involve considerations that are beyond the scope of this document.

 

I. Cytologic material (pericardial fluid)               back     Top     Main Page

A.  Clinical Information

            1.   Patient identification

                  a.   Name

                  b.   Identification number

                  c.   Age (birth date)

                  d.   Gender

            2.   Responsible physician(s)

            3.   Date of procedure

            4.   Other clinical information

                  a.   Relevant history

                        (1)  primary cardiovascular disease

                        (2)  myocarditis

                        (3)  congenital heart

                        (4)  history of tumor elsewhere

                        (5)  immunosuppression

                        (6)  tuberous sclerosis

                        (7)  previous irradiation

                  b.   Relevant findings (e.g. ECHO findings, evidence of tumor elsewhere in body)

                  c.   Clinical diagnosis

                  d.   Procedure (e.g. fine needle aspiration of pericardial fluid)

                  e.   Anatomic site(s) of specimen (e.g. anterior pericardial sac)

 

      B.   Macroscopic Examination

            1.   Specimen

                  a.   Description

                  b.   Unfixed/fixed (specify fixative)

                  c.   Number of slides received (if appropriate)

                  d.   Quantity, appearance of fluid specimen (if appropriate)

                  e.   Results of intraprocedural consultation

            2.   Material submitted for microscopic evaluation

            3.   Results of rapid smear review

            4.   Special studies (specify)

      C.  Microscopic Evaluation

            1.   Adequacy of specimen (if unsatisfactory for evaluation, specify reason)

            2.   Tumor (Note A)

                  a.   Histologic type (if possible)

                  b.   Histologic grade (if possible)

            3.   Additional pathologic findings, if present

                  a.   Therapy-related changes

                  b.   Degenerative changes

                  c.   Atypical cellular reaction

                  d.   Inflammation

                  e.   Other

            4.   Status/results of special studies (specify)

            5.   Comments 

                  a.   Correlation with intraprocedural consultation, as appropriate

                  b.   Correlation with other specimens, as appropriate

                  c.   Correlation with clinical information, as appropriate

 

II. Incisional or excisional biopsy                       back     Top     Main Page

      A.  Clinical Information

            1.   Patient identification

                  a.   Name

                  b.   Identification number

                  c.   Age (birth date)

                  d.   Gender

            2.   Responsible physician(s)

            3.   Date of procedure

            4.   Other clinical information

                  a.   Relevant history

                        (1)  primary cardiovascular disease

                        (2)  myocarditis

                        (3)  congenital heart

                        (4)  history of tumor elsewhere

                        (5)  immunosuppression

                        (6)  tuberous sclerosis

                        (7)  previous irradiation

                  b.   Relevant findings (e.g. ECHO findings, evidence of tumor elsewhere in body)

                  c.   Clinical diagnosis

                  d.   Procedure

                  e.   Operative findings

                  f.    Anatomic site(s) of specimen (e.g. pericardium, left/right ventricle, atrium)

 

      B.   Macroscopic Examination

            1.   Specimen

                  a.   Tissue(s) received

                  b.   Unfixed/fixed (specify fixative)

                  c.   Number of fragments

                  d.   Dimensions

                  e.   Descriptive features (color/consistency)

                  f.    Orientation (if designated by surgeon)

                  g.   Results of intraoperative consultation

            2.   Tumor

                  a.   Size (Note B)

                  b.   Descriptive features (e.g. consistency, color, hemorrhage, necrosis)

                  c.   Extension

            3.   Margins (if appropriate)

                  a.   Vascular

                  b.   Pericardial

                  c.   Other

            4.   Tissue submitted for microscopic evaluation

                  a.   Tumor (Note C)

                  b.   Designated areas including those marked adherent to other structures

                  c.   Margin(s)

                  d.   Frozen section tissue fragment(s) (unless saved for special studies)

                  e.   Other (specify)

            5.   Special studies (specify) (e.g. histochem-istry, immunohistochemistry, electron microscopy, morphometry, DNA analysis [specify type])

 

      C.  Microscopic Evaluation

            1.   Tissue(s) present

            2.   Tumor

                  a.   Histologic type(s) (Note D)

                  b.   Histologic grade (Note E)

                  c.   Status of designated areas

                  d.   Extent of invasion (adjacent tissues)

            3.   Margins (as appropriate)

            4.   Additional pathologic findings, if present

                  a.   Benign tumor

                  b.   Therapy-related changes

                  c.   Degenerative changes

                  d.   Atypical cellular reaction

                  e.   Inflammation

                  f.    Other

            5.   Results/status of special studies (specify) (Note F)

            6.   Comments

                  a.   Correlation with intraoperative consultation, as appropriate

                  b.   Correlation with other specimens, as appropriate

                  c.   Correlation with clinical information, as appropriate

 

EXPLANATORY NOTES

A. Cytologic Findings        back     Top     Main Page

Pericardial effusions are rarely caused by primary cardiac tumors.  The most common causes of malignant pericardial effusions are metastatic adenocarcinoma from lung or breast, malignant melanoma, or extension of malignant mesothelioma into the pericardium.  The pathologist should evaluate the nature and clinical significance of a malignant pericardial effusion by discussing the findings with the clinician and/or reviewing the patient’s medical record.  Cellular changes considered to be infective, reactive, or degenerative (e.g. viral infection, immunotherapy, chemotherapy, or radiation effect) should be clearly distinguished from malignant or atypical (potentially malignant) cytologic findings.  Additional patient history and pertinent clinical findings may be definitive.

 

B.   Staging       back     Top     Main Page

The greatest diameter of the tumor in centimeters should be recorded. There is no published staging system for primary cardiac tumors.

 

C.  Number of Sections            back     Top     Main Page

The number of sections varies with the size of the specimen and the nature of the neoplasm. The pathologist should sample areas with diverse gross appearances. In addition to tumor evaluation, routine sampling of the non-neoplastic components of the specimen should be performed.

 

D. Histologic Type            back     Top     Main Page

The classification of malignant cardiac tumors as recommended by the Armed Forces Institute of Pathology (AFIP) fascicle on tumors of the heart and great vessels is listed below.(1) This protocol, however, does not preclude the use of other histologic classifications.

 

AFIP Classification of Malignant Cardiac Tumors

•      Angiosarcoma

•      Malignant fibrous histiocytoma

•      Myxosarcoma

•      Fibrosarcoma

•      Leiomyosarcoma

•      Rhabdomyosarcoma

•      Osteosarcoma

•      Synovial sarcoma

•      Malignant schwannoma (malignant peripheral nerve sheath tumor)

•      Malignant mesenchymoma

•      Malignant mesothelioma

•     Other

As with sarcomas in other sites, a variety of histologic patterns may be found. Although not included in the classification, lymphomas are also found in the heart.

 

E.   Histologic Grade           back     Top     Main Page

Pathologists should grade the tumor and indicate the grading system used. Most malignant tumors of the heart are sarcomas. Necrosis of groups of cells and mitotic rates of >5 mitoses/ 10 high-power fields have been associated with reduced survival.(1)

 

F.   Special Studies       back     Top     Main Page

Immunohistochemistry can be used to ascertain the histogenesis of a sarcoma or substantiate the diagnosis of mesothelioma. Generally speaking, mesotheliomas can contain cytokeratins which are usually lacking from sarcomas. Transmission electron microscopy is also very helpful in the distinction of these tumor types.  Myxoma, the most common benign tumor, has no distinctive immunohistochemical features. 

 

REFERENCES            back     Top     Main Page

1.     Burke AP, Renu V. Atlas of Tumor Pathology, Tumors of the Heart and Great Vessels. 3rd series. Fascicle 16. Washington, DC: Armed Forces Institute of Pathology; 1996.

 

BIBLIOGRAPHY

•      Blondeau P. Primary cardiac tumors:  French studies of 533 cases. Thorac Cardiovasc Surg. 1990;38 (Suppl 2):192-195.

•      Burke AP, Rosado-de-Christenson M, Templeton PA, Virmani R. Cardiac fibroma: Clinico-pathologic correlates and surgical treatment. J Cardiovasc Surg. 1994;108:862-870.

•      Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer. 1992;69:387-395.

•      Burke AP, Virmani R. Osteosarcomas of the heart. Am J Surg Pathol. 1991;15:289-295.

•      Dein JR, Frist WH, Stinson EB, et al. Primary cardiac neoplasms:  Early and late studies of surgical treatment in 42 patients. J Thorac Cardiovasc Surg. 1987;93:502-511.

•      Melo J, Ahmad A, Chapman R, Wood J, Starr A. Primary tumors of the heart: A rewarding challenge. Am Surg. 1979;45:681-683.

•      Miralles A, Bracamonte L, Soncul H, et al. Cardiac tumors: Clinical experience and surgical results in 74 patients. Ann Thorac Surg. 1991;52:886-895.

•      Murphy MC, Sweeny MS, Putnam JB Jr, et al. Surgical treatment of cardiac tumors: A 25-year experience. Ann Thorac Surg. 1990;49:612-617.

•      Reece IJ, Cooley DA, Frazier OH, Hallman GL, Powers PL, Montero CG. Cardiac tumors: Clinical spectrum and prognosis of lesions other than classical benign myxoma in 20 patients. J Thorac Cardiovasc Surg. 1984;88:439-446.

•      Ryan RE Jr, Obeid AI, Parker FB Jr. Primary cardiac valve tumors. J Heart Valve Dis. 1995;4:222-226.

•      Tazelaar HD, Locke TJ, McGregor CG. Pathology of surgically excised primary cardiac tumors. Mayo Clin Proc. 1992;67:957-965.

•      Turner A, Batrick N. Primary cardiac sarcomas: A report of three cases and a review of the current literature. Int J Cardiol. 1993;40:115-119.

•      Verkkala K, Kupari M, Maamies T, et al. Primary cardiac tumors—Operative treatment of 20 patients.  J Thorac Cardiovasc Surg. 1989;37:361-364.

Author:

Elizabeth H. Hammond, MD

DRAFT ©1998. College of American Pathologists (CAP). All rights reserved. None of the contents of this publication may be reproduced, stored in a retrieval system or transmitted in any form or by any means (electronic, mechanical, photocopying, recording, or otherwise) without prior written permission of the publisher.

Expires as CAP policy in May 2001. A year prior, the protocol will be reviewed and updated.

Contributors:          back     Top     Main Page
CAP Cancer Committee; Robert L. Yowell, MD, PhD; Robert L. Flinner, MD; Donald B. Doty, MD