当前位置: 首页 > 期刊 > 《中华中西医杂志》 > 2003年第7期
编号:10395094
74例心脏肿瘤的临床病理及预后研究
http://www.100md.com 《中华中西医杂志》 2003年第7期
     【摘要】 目的 通过74例心脏肿瘤的病理学研究,分析其病理学改变与临床表现及预后的关系。方法 选自我科确诊的心脏肿瘤74例,其中收住院手术切除标本73例,尸检1例。用组织化学及特殊组织化学染色和免疫组化观察。结果 74例心脏肿瘤中,原发性72例、继发性即转移的肝癌2例。在原发性肿瘤中,良性瘤68例(94.4%),包括粘液瘤、纤维瘤、横纹肌瘤、心包囊肿;恶性瘤4例(5.6%),包括胚胎性横纹肌肉瘤、神经鞘纤维肉瘤、恶性间皮瘤。在粘液瘤中,73.8%为女性患者,90.2%发生在左心房内,均有一蒂并附着在房间隔卵圆窝处或附近。结论 心脏肿瘤与其他部位的肿瘤不同,即使是良性瘤由于引起血液动力学改变也可致命,需早期诊断、早期手术治疗,绝大多数预后良好。恶性瘤预后极差。

    关键词 心脏肿瘤 病理学 手术 免疫组化 预后

    【文献标识码】 A 【文章编号】 1606-8106(2003)07-0969-04

, 百拇医药     The clinicopathological and prognostic study

    on74cases of cardiac tumors

    Wang Xuzhou

    Dept.of Pathology,the Qilu Hospital of Shandong University,Jinan250012.

    【Abstract】 Objective To analyze the relation between the pathological changes and the clinical manifestaˉtions and the prognosis of cardiac tumors through the pathological study on74cases of cardiac tumors.Methods 74confirmed cardiac tumors of our department were selected,among which73was surgical specimen and1 was autopsy.The study was performed by using the common and special histochemical stainings and immunohistochemical methods.Results 72were primary and2were secondary or metastazed hepatocellular carcinoma.In the primary ones,68(94.4%)were benign,including myxoma,fibroma,rhabdomyoma and pericardial cyst,while4(5.6%)were malignant,inˉcluding neurolemmafibrosarcoma,embryonal rhabdomyosarcoma and malignant mesothelioma.In the myxomas,female patients account for73.8%,90.2%exists in the left atrium and every one has got a peduncle adhesive to the fossa oˉvalis or adjacency of the atrial septum.Conclusion The results of the pathological and prognostic study showes carˉdiac tumors are quite different from the tumors in other sites,i.e.even benign ones could cause fatal hemodynamic disˉturbance.Hence early diagnosis and early operation are necessary and if in such instance,the prognosis of most of the benign ones would be good.But the prognosis of the malignant tumors is worst.
, 百拇医药
    Key words cardiac tumor pathology surgical immunohistochemistry prognosis

    Cardiac tumor is a kind of ones rarely found.Missed and misdiagnosis are easily occured just because of no prominent differences of clinical symptoms with other diseases of heart so that some patients would lose the chance of treatment,especially in the areas with worse medical condition.We carried the clinicopatholoˉg

    ical analysis of74cardiac tumors,diagnosed by surgical pathology and autopsy,in order to improve the knowlˉedge of cardiac tumors and raise the ratio of early diagˉnosis and cure.
, 百拇医药
    1 Materials and methods

    Seventy-four cases of cardiac tumors were collectˉed from the Department of Pathology,the Qilu Hospital of Shandong University from1971to2002,which conˉsisted of73cases of operation specimen and1 autopsy.The specimens were formalin-fixed(10%conˉcentration),paraffin-embedded and stained with hemaˉtoxylin and eosin.Particular histochemical stainings as PAS,Alcian Blue,Masson’s,VG,modified Gordon-Sweets and immunohistochemical method were done in some cases.
, http://www.100md.com
    2 Results

    2.1 Myxoma 61cases totally,55of which were found in the left atrium while only6in the right.All of the tumors was singular with a peduncle in the fossa oˉvalis or adjacency of the atrial septum.45cases were feˉmale patients.Gross:the peduncle was0.2~2cm in length with abundant collagen fibers.The largest was11.

    5cm×4cm×2.4cm while the smallest was3cm×2.2cm×1cm.Their appearances showed spherical or polypoid or botryoid,slightly greyish red,smooth,semiˉtransparent,tremellose,soft and brittle.Microscopically:scanty cells were polygonal or spindle-shaped and singly or focally distributed in loose stroma rich in muˉcus and capillary(Fig.1).Strongly positive in alcian blue and weakly positive in PAS.
, 百拇医药
    2.2 Fibroma 1case only in the posterior wall and aˉtrial septum of the left atrium.Gross:88.5g in weight and12.5cm×11cm×9cm in dimension.The cutting surface was greyish red and tenacious.The surface was covered with numerous papillaries in variable size(Fig.2).Microscopically:the tumor was composed of fibroˉcytes and collagen fibers in interlaced arrangement,most of which had gone hyalinization.VG staining showed that abundant collagen fibers were red in interlaced and whorling arrangement.Immunostaining of vimentin was expressed.
, http://www.100md.com
    2.3 Cardiac rhabdomyoma 2cases in the left or right ventricle wall.Gross:4.5cm×2.6cm×1.6cm and3.5cm×3cm×2cm in dimension.nodular and clear deˉmarcation.The cutting surface was greyish red and tenaˉcious.Microscopically:in its spongy structure,the ramose or polygonal cells have got cross striation in the cytoplasm and clear cell membrane while some have got clear cytoplasm with prominent nucleoli and nuclei in the center,around which cytoplasm was in loose network forming the characteristic and diagnostic spider-like cell(Fig.3).PAS staining showed positive granules.Imˉmunostaining of myoglobin and desmin were expressed.
, http://www.100md.com
    2.4 Pericardial cyst 4case in the pericardium.Gross:6cm×6cm×5cm~30cm×25cm×12cm in diˉmension,smooth and cystic.The cyst wall was soft and0.1~0.3cm in thickness,the inner side of which was smooth,too.The cyst contained clear fluid.Microscopiˉcally:the wall was composed of loose connective tissue and lined with a layer of mesothelial cells.

    2.5 Embryonal rhabdomyosarcoma 1case in the right pericardial cavity.Gross:17cm×11cm×9cm in dimension,smooth,slightly greyish red,soft.The cutting surface was fish-flesh appearance.Microscopically:the characteristic cells with strong acidophilic cytoplasm and cross striations.The densely packed cells were ribb
, http://www.100md.com
    on or tadpole-shaped with marked atypia.The tumor cells were discrete distributed.Immunostaining of myoglobin and desmin were expressed.

    2.6 Neurolemmafibrosarcoma 1case in the right pericardium.Gross:Soft and nodular with the size of18cm×12cm×10cm.The cutting surface was fish-flesh appearance,accompanied with focal hemorrhages, necrosis and cyst-forming.Microscopically:the densely packed cells were oval or short spindle with marked atypia,arranged in fasciculi,whorls with slightly palisadˉing of nuclei.Mitosis was numerous(Fig.4).Immunosˉtaining of S-100and synaptophysin(Sy)were exˉpressed.
, 百拇医药
    2.7 Malignant Mesothelioma 2cases in the periˉcardium.Gross:The tumor surrounded the heart with difˉfuse thickness,nodular and dimension of13cm×7cm×1.5cm and12cm×7cm×3cm.The cutting surface was greyish red and hard.Microscopically:the marked atypiˉcal cells were arranged in solid nest or incomplete glands.Between the nests much short spindle cells with marked atypia were densely packed,representing the structure of fibrosarcoma(Fig.5).Modified Gordon-Sweets staining showed that reticular fibers were disˉtributed between the nests in some areas or between the tumor cells in other areas.Immunostaining of Keratin,EMA and Mesothelial cells(MC)were expressed.
, http://www.100md.com
    2.8 Metastatic hepatocellular carcinoma of heart 2cases in the right atrium,one of which was autopsy,which showed that a round,hard and greyish white massexisted in the right atriumwith a size of5cm×3.5cm×2.5cm(Fig.6).In the middle part of the right lobe of liver a mass,12cm in diameter,was found accompanied with hepatocirrhosis.Another one was operation speciˉmen with a size10cm×8cm×6cm.Microscopically:the tumor cells were polygonal,markedly atypical and arˉranged in cords with formation of blood sinusoids.Imˉmunostaining of AFP was expressed.
, http://www.100md.com
    3 Discussion

    3.1 Classification and incidence of cardiac tumors (1)Primary cardiac tumors:It is very seldom found,only amounting to0.09%of all the cardiac operations as reˉported [1] .Of these72primary ones,65(90.3%)was found in the heart proper and7(9.7%)in the periˉcardium while68(94.4%)was benign,including myxoˉma,fibroma,rhabdomyoma and pericardial cyst and4(5.6%)malignant,including embryonal rhabdomyosarcoˉma,neurolemmafibrosarcoma and malignant mesotheˉlioma.In the cardiac tumors,the benign ones are the commonest.61(84.7%)of the cases was myxoma which was the commonest of the primaries,amounting to89.7%of the benign and occured mostly in the females(73.8%),and55(90.2%)of which was found mostly in the left atrium and only6(9.8%)in the right,atˉtached to the fossa ovalis of the atrial septum.It is a true tumor originated from the undifferentiated mesenchymal cells of the endocardium [2,3] .The incidence of it and the one of it in the left atrium only had a very little variation between this study and the statistical results of China [4] ,which is very high,88.9%and90.4%respectively.A more seldom found tumor is cardiac fibroma,which is mostly i
, 百拇医药
    n children and probably originated from the fiˉbroblasts of endocardium or myocardium [5,6] .The diamˉeter as reported is usually3~7cm [1,7] .The one case in this study was a47-year-old male,who had got a rarely giant fibroma,88.5g in weight and12.5cm×11cm×9cm in dimension.Another rare tumor is cardiac rhabdomyoma,which is also mainly in children and half the cases accompanied with nodular cirrhosis [8] .(2)Secondary or metastatic cardiac tumors:It is much more frequently found,16~40times of the primaries as reˉported [1] .There’s only two secondary tumor in the study because surgeons didn’t carry out operations on the paˉtients with metastasis and autopsy couldn’t be done once the patients with metastasis died so that the exact inciˉdence of secondary tumors pathologicallywas too difficult to be known.The secondary tumors of heart usua
, 百拇医药
    lly come from the adjacent organs or tissues,which is commonly pulmonary carcinoma,esophageal carcinoma and maligˉnant tumors of mediastinum.The2cases in the study was hepatocellular carcinoma which had invaded into the inferior vena cava and right atrium,accopmaning hemorˉrhagic hydropericardium confirmed by operation or auˉtopsy.

    3.2 Diagnosis and differential diagnosis of cardiac tuˉmors Misdiagnosis often occured because the incidence of cardiac tumors is very low and the early symptoms are not obvious and in late stage the symptoms and signs were indiscernible with those of other organic heart disˉeases.Of the cases in the study,9was misdiagnosed as mitral stenosis,5as common cold and rheumatic heart diseases respectively,3as anaemic heart diseases and constrictive pericarditis respectively,2as pneumonia,cor pulmonale,pulmonary tumor,cirrhosis of liver,psyˉchoneurosis,hyperthyroidism,pleuritis,myocarditis,coroˉnary heart disease,tuberculous pericarditis,pericardial cyst and fibrosarcoma respectively,and6as heart disˉeases in suspicion at first visit.The case of fibroma was misdiagnosed as mitral stenosis,and carried out mitral commissurotomy and later was rediagnosed a
, http://www.100md.com
    s myxoma by echocardiogram.During the intracardiac surgery under extracorporeal circulation a year later,the tumor was found to increase a great deal and having involved the mitral valves,which was corrected as fibroma by patholoˉ gist.After operation the patient died of low cardiac outˉput syndrome.The case of cyst-forming n

    eurolemmafiˉbrosarcoma was misdiagnosed as pericardial cyst and the2cases of pericardial malignant mesothelioma as tuberˉculous pericarditis.Hence they missed their therapeutic chances because the tumors were too large to be excised when operated.
, http://www.100md.com
    The differentiation of myxomas with organized thrombi of heart should be emphasized.The clinicoˉpathological study of the61cases of myxoma showed the following differences:(1)Myxoma is commonly in the fossa ovalis of left atrium(90.2%)while the organized thrombus is commonly in the left auricle or other sites,usually accompanied with endocarditis,valvulopathy or myocardiosis,etc.(2)Myxoma is plemorphic,smooth,light greyish red,semitransparent,tremelloid and brittle with a peduncle while the organized thrombus is usually oval,rough,darkred or greyish white,solid and maybe laminated without peduncle.(3)The cells
, 百拇医药
    of myxoma are distributed in the mucus-rich ground substance while the organized thrombus may develop granulation tissues with mucus-free stroma.

    3.3 Treatment and prognosis of cardiac tumor Carˉdiac tumors are different from others because even beˉnign ones could cause fatal hemodynamic disturbance so that early diagnosis and operation are more important.The prognosis of the benign is good on the condition of early operation,but that of large and sessile ones is sometimes not good because of its extensive linking with heart [6] .The case of fibroma couldn’t be cured just beˉcause of its largeness even though the surgeons did their best to excise the tumor.The benign tumors were all cured although two cases of myoxma were done a second excision after recurrence except that8cases of the beˉnign in the study died of low cardiac output syndrome also because of their size,longer than6cm in diameter.The characteristics of the malignant tumors in the study is rapid growth,high invasiveness,marked atypia,all of which could make the prognosis worse,besides heart as a very special organ.Two of the malignant tumors is periˉcardial malignant mesothelioma,13cm×7cm×1.5cm or10cm×9cm×7cm in size,which surrounded the heart and adhered to the pericardium extensively.Another is neurolemmafibrosarcoma which grew rapidly to18cm×12cm×10cm only3months later after the emergence of the first symptom and was extensively adhered with periˉcardium,middle and inferior lobes of lungs and the veˉnae cava.These three cases all missed the possibility of operation and biopsy is what could be done in order to make a confirm diagnosis by pathologist.Another two are hepatocellular carcinoma metastasized to the right atriˉum,confirmed by operation or autopsy.The5cases of malignant tumors in the study died.Hence the prognosis of malignant cardiac tumors is worst. (本文图片略)
, 百拇医药
    References

    1 刘万车.心脏肿瘤.白求恩医科大学学报,1983,9(6):98.

    2 Fine G,Morales A,Horn RC.Cardiac Myxoma:AMorphologic and Hisˉtogenetic Appraisal.Cancer,1968,22:1156.

    3 Feldman PS,Horvath E,Kovacs K.An Ultrastructural Study of Seven Cardiac Myxomas.Cancer,1977,40(5):2216.

    4 中华医学杂志编辑委员会.心脏肿瘤专题座谈会纪要.中华医学杂志,1988,68(12):661.

    5 Geha AS,Weidman WH,Soule EH,et al.Intramural Ventricular Carˉdiac Fibroma:Successful Removal in Two Cases and Review of the Litˉerature.Circulation,1967,36:427.
, 百拇医药
    6 Van Der Hauwaert LG,Corbeel L,Maldague P.Fibroma of the Right Ventricle Producing Severe Tricuspid Stenosis.Circulation,1965,32:451.

    7 王绪洲.左心房巨大纤维瘤1例.山东医药,1992,32(6):62.

    8 Bruni C,Prioleau PG,Hivey T,et al.New Fine Structural Features of Cardiac Rhabdomyoma:Report of a case.Cancer,1980,46(9):2068.

    作者单位:250012山东大学齐鲁医院病理科

    (编辑 李年令), http://www.100md.com