16层螺旋CT对左房黏液瘤的诊断价值

目的探讨16层螺旋CT对左房黏液瘤的诊断价值。方法使用16层螺旋CT对7例临床诊断为左房黏液瘤的患者进行CT检查,所有数据传至工作站进行重组和后处理。结果 7例患者平扫左房黏液瘤密度低于正常心肌密度;增强扫描病灶呈不均匀强化,三维重建清晰显示病灶有蒂与房间隔相连,多期相重建能显示肿块跨二尖瓣运动。结论 16层螺旋CT能较好地显示左房黏液瘤的位置、形态、大小、与房间隔关系,对左房黏液瘤的定性有较高价值。     

Familial cardiac myxoma with multifocal recurrences:a case report and review of the literature

We report a case of myxoma with multiple recurrences in both the atrium and ventricle in a 26-year-old woman five years after the surgical removal of left atrial myxoma.Her 52-year-old mother had a similar medical history.To our knowledge,this was the first familial case who suffered multifocal cardiac myxoma recurrences without any sign of the myxoma complex.Based on our understanding of the mechanism of recurrence,the approaches to prevent the recurrence,and markers to predict recurrence,we propose that m...     

Ockham's razor or Hickam's dictum: a right atrial mass following excision of left atrial myxoma

We report a case of a 54-year-old man presenting with a rightatrial mass 7 months after undergoing a left atrial myxoma excisionsurgery. The differential diagnosis included recurrent myxomaor thrombus. The patient underwent repeat open sternotomy oncardiopulmonary bypass. Histopathological evaluation of themass revealed an organizing thrombus. This report is, to ourknowledge, the first that demonstrates right atrial thrombusshortly following excision of left atrial myxoma.     

Risk factors for postoperative recurrence of cardiac myxoma and the clinical managements: a report of 5 cases in one center and review of literature

Background  Recurrence or metastasis of myxomas is not rare and can lead to malignancy. We aimed to analyze the risk factors for postoperative cardiac myxoma recurrence and to summarize its clinical characteristics, treatments and classification.

Methods  The clinical data of 5 patients with recurrent cardiac myxoma were retrospectively analyzed and our clinical experience was summarized. Moreover, the relevant literatures were reviewed.

Results  All the five cases of primary myxomas were derived from atypical positions. One patient had early distant metastasis, one had family history, and two suffered malignant recurrence. The recurrence interval was (2.30±2.16) years and the recurrent tumors were all found in different chambers from those of the corresponding primary tumors. Re-operation was performed after recurrence. One patient died of heart failure after malignant recurrence, and the other 4 cases had satisfactory therapeutic outcomes after re-operations. Our experience advocated a clinical classification of “typical” and “atypical” cardiac myxoma, the typical myxomas referred to the tumors locating at the left atria, with single pedicle, rooted at or around the fossa ovalis, involving no genetic causes, and the atypical myxomas included the familial tumors, tumors stemming from multiple chambers, rooted in abnormal positions of the left atrium, with evident genetic mutation, or with malignant tendency.

Conclusions  Postoperative follow-up is of vital importance for patients with myxomas characterized by multi-chamber distribution, early distant metastasis, atypical origin, and family history. Once recurs, re-operation is necessary and should be performed immediately.

     

卡尼复合征心脏黏液瘤的临床特征及外科治疗结果

目的  比较卡尼复合征（Carney complex）心脏黏液瘤与散发心脏黏液瘤的临床特征及外科治疗结果,为Carney complex心脏黏液瘤提供诊疗经验。 方法 回顾性分析阜外医院2009年1月至2013年12月共372例接受心脏黏液瘤切除手术患者的临床资料,其中7例确诊为Carney complex心脏黏液瘤。7例中男2例,女5例;年龄7～50岁,平均（2857±1326）岁;均接受外科手术切除。术后随访54～94个月,平均（7686±1549）个月。比较Carney complex心脏黏液瘤和散发心脏黏液瘤在年龄、性别、术前动脉栓塞率、心脏黏液瘤多发性和心脏黏液瘤切除术后的复发率。 结果 手术切除Carney complex心脏黏液瘤在总体心脏黏液瘤中的发病率为188％,最常见的发生部位是左房。所有7例Carney complex心脏黏液瘤术后没有早期死亡,随访期间无死亡。Carney complex心脏黏液瘤与散发性心脏黏液瘤在年龄、术前动脉栓塞率、心脏黏液瘤多发率及心脏黏液瘤术后复发率的差异均有统计学意义（P＜005）,而在性别上差异无统计学意义（P＞005）。 结论  Carney complex心脏黏液瘤可取得良好的外科疗效。与散发心脏黏液瘤相比,Carney complex心脏黏液瘤发病年龄更早,术前动脉栓塞率更高,常见多发肿瘤,术后复发率更高。对于Carney complex心脏黏液瘤,外科手术应更积极,术后应更密切随访。     

Atrial myxoma demonstrated by cine gradient refocused echo magnetic resonance imaging

A case of atrial myxoma presenting with syncope evaluated by echocardiography and MRI is described. Cine gradient‐echo MRI demonstrated atrial myxoma as a very low signal intensity mass indicating the presence of haemosiderin.     

Cellular ‘neurothekeoma’: an epithelioid variant of pilar eiomyoma? Morphological and immunohistochemical analysis of a series

Cellular neurothekeoma is a recently recognized benign cutaneous neoplasm, which is currently regarded as being of nerve sheath origin and is thought to represent a variant of conventional neurothekeoma (dermal nerve sheath myxoma). Nine new cases presenting predominantly in adolescents or young adults are described. Morphologically they were characterized by short fascicles or small nests of palely eosinophilic epithelioid or spindle-shaped cells which ramified in an ill-defined manner between dermal collagen bundles. Myxoid matrix was absent or sparse. Scattered normal mitoses and multinucleate giant cells were often present. Immunohistochemically all nine cases were strongly NK1/C3 positive, seven were weakly NSE positive and three were smooth muscle actin positive. Staining for S-100 protein, PGP 9.5, epithelial membrane antigen and desmin was negative in all cases. In view of its distinctive architecture and immunophenotype, both of which are totally different from conventional neurothekeoma, it is proposed that cellular 'neurothekeoma' is a separate discrete entity which may represent an epithelioid variant of pilar leiomyoma.     

Erythematous Papules in a Patient with Cardiac Myxoma: A Case Report and Review of the Literature

A 35-year-old woman was followed up by a physician for one and a half years as a case of transient ischemic attack (TIA) due to thrombosis. She had a few erythematous papules on the dorsal side of her right forearm and on the right side of her neck. From the history of TIA and the transient erythematous papules, we suggested the possibility of cardiac myxoma. Echocardiogram demonstrated a large mass in her left atrium. An immediate operation was carried out, and the cardiac myxoma was excised surgically. Histological examination of the erythematous papule revealed myxoma emboli.     

The problems of surgical treatment for cardiac myxoma and associated lesions

Twenty-four patients with cardiac myxomas consisting of 22 left and 2 right atrial myxomas were operated on. All myxomas were removed with an excision of the attachment walls using a cardiopulmonary bypass. Two myxomas required a partial cardiopulmonary bypass from the femoral vein to the artery prior to operation because they were on the verge of becoming stuck in the atrioventricular valves and potentially causing shock. For embolic complications of myxoma, the embolus of the external carotid artery was extirpated before undergoing cardiac surgery. In a patient with pulmonary infarction, the infarcted lung was resected simultaneously. Another patient with a cerebral infarction received a clipping of an aneurysm which later appeared in the infarcted area. For associated cardiac lesions, two patients underwent a coronary artery bypass graft and one mitral valve replacement with tricuspid annuloplasty. In the former two cases, the myxoma was removed prior to coronary artery bypass grafting because the use of retrograde coronary perfusion was considered to be sufficient to protect the heart. In the latter case, the removal of the myxoma first disclosed a significant mitral lesion which had been masked by the huge myxoma. All patients but one, who died of pneumonia, showed a good recovery. In this series, the problems of surgical treatment for cardiac myxoma and associated lesions are also discussed.