Carney's triad: a new case

Carney's triad is a very unusual syndrome, associating three different tumours on the same patient, a young woman generally: a gastric leiomyoblastoma, a pulmonary chondroma and an extra-adrenal paraganglioma. The authors report through the literature review and a new case of Carney's syndrome associating gastric leiomyoblastoma and extra-adrenal paragangliomas, the different diagnostic and therapeutic criterias.     

Carney's triad with recurrent gastric leiomyoblastoma

The triad of gastric leiomyoblastoma, extra-adrenal paraganglioma, and pulmonary chondroma was first described by Carney in 1977. Fewer than 30 patients with this syndrome have since been reported. In most patients the initial lesion has been a gastric leiomyoblastoma. The patient we describe had recurrences of a gastric leiomyoblastoma 15 and 17 years, respectively, after the initial gastric resection. Subsequently, multiple pulmonary lesions were excised and confirmed to be chondromas, but no evidence of a paraganglioma has yet been identified. Gastric leiomyoblastomas in patients with Carney's triad frequently recur; however, the prognosis is quite favorable in contrast to that of sporadic cases. Whenever a patient with one of the classical lesions of Carney's triad is encountered, the possibility of this unusual syndrome should be considered. The evaluation and follow-up of these patients should include a search for the other two components.     

Carney's triad: a 16-year follow-up

We report a female teenager who presented with a gastrointestinal stromal tumor of the stomach and a paraganglioneuroma. She later developed a pulmonary chondroma, fulfilling the requirements of Carney's triad. This patient demonstrates the course of the disease, which included severe emotional symptoms. She died, at 30 years of age, 16 years after the onset of disease, riddled with metastases. Many patients with Carney's triad survive for many years, but we can not predict the prognosis in any patient. During her lifetime, the patient had considerable emotional suffering, perhaps because of her disease.     

An unusual case of complete Carney's triad in a 14-year-old boy

Carney's triad represents the association of gastric gastrointestinal stromal tumor, pulmonary chondroma, and extraadrenal paraganglioma. Only 79 cases of this rare condition have been described. Here, the authors describe the unusual case of a 14-year-old boy who presented with a complete Carney's triad. This is only the second reported case in the world literature of a patient manifesting a complete Carney's triad at presentation. The management of each tumor is discussed. J Pediatr Surg 37:1228-1231.     

Pulmonary chondroma, extra-adrenal paraganglioma, and gastric leiomyosarcoma: Carney's triad

The triad of pulmonary chondroma, extra-adrenal paraganglioma, and gastric leiomyosarcoma has been reported in five patients, all young women. The association of tumors apparently constitutes a syndrome. This paper describes the sixth affected patient, also a young woman, draws attention to the frequency of thoracic paraganglioma in the syndrome, and notes that the pulmonary cartilaginous lesion has frequently been misinterpreted as pulmonary hamartoma. Early exploration of the chest is indicated not only to rule out the possibility of metastatic gastric tumor but also to search for paragangliomas.     

Experience of complete Carney's triad showing pulmonary hamartoma associated with gastrointestinal stromal tumor and para-arotic paraganglioma

A 34-year-old man, who had undergone the gastrectomy for gastrointestinal stromal tumor (GIST) and para-aortic paraganglioma 3 years before, was found to have a left lung tumor on a computed tomography. The tumor was revealed to be a pulmonary hamartoma, and diagnosed as Carney's triad. This is a rare case of complete type Carney's triad of an adult male.     

Testicular tumors in Carney's complex

PURPOSE: Bilateral sex cord stromal testicular tumors are common in the syndrome of myxoma, spotty pigmentation and endocrine overactivity (Carney's complex). Large cell calcifying Sertoli cell tumor is the particular testicular tumor found in Carney's complex. A clinicopathological review of 26 patients is presented. MATERIALS AND METHODS: We report 2 cases of Carney's complex with testicular tumors. An additional 24 patients with Carney's complex and testicular tumors were identified by MEDLINE search and review of the literature. RESULTS: Bilateral testicular tumors were found in 16 patients (61%) with a familial occurrence in 10 (38%). A testicular mass was the most common presentation. The associated findings of Carney's complex included cardiac myxoma in 16 patients, skin myxoma in 16, skin pigmentation in 15, Cushing's syndrome in 8, acromegaly in 3 and schwannoma in 3. Excisional biopsy, surveillance, bilateral orchiectomy and unilateral orchiectomy were performed in 7, 4, 7 and 8 patients, respectively. CONCLUSIONS: No local tumor recurrence or metastasis has developed in patients with bilateral and/or multifocal testicular tumors. Excisional biopsy or surveillance only are treatment options for bilateral testicular tumors in Carney's complex.     

Carney's syndrome: a case report

The peculiar aspects of the clinico-pathological picture and surgical treatment of Carney's syndrome, a rare morbid association of multiple non-epithelial neoplasms of the stomach, lung and chromaffin tissue, are focused on and discussed on the basis of the observation of a clinical case. The patient was a 22-year-old male with a multiple stromal tumour of the stomach, oesophageal leiomyoma, a left thoracic terato-chondroid neoplasm and gallbladder adenomyoma, surgically treated in two stages: laparotomy for gastric resection and cholecystectomy followed by left thoracotomy for resection of the intrathoracic neoplasm and excision of the oesophageal leiomyoma. The postoperative course was characterised by low morbidity and healing was monitored by means of thorough follow-up examinations. The case reported here is classed among the variants of Carney's classical triad. A knowledge of the pathological associations of these uncommon tumours and their natural history is of great importance for implementing appropriate diagnostic and therapeutic protocols.     

Left ventricular mass in a patient with Carney's complex

IMPLICATIONS: Carney's complex is characterized by cardiac myxomas, adrenocortical disease, growth hormone-secreting adenomas, and other types of tumors. Its prevalence and incidence are unknown. The anesthesiologist must examine the patient or order tests to exclude cardiac tumors, signs of excess cortisol secretion, acromegaly, and possible peripheral nerve root involvement.     

Injuries to the portal triad

We reviewed the management and clinical course of 21 patients with extrahepatic injuries to the portal triad seen over the past 11 years at a Level I trauma center. These represented only 0.21% of patients with multiple trauma admitted during this time. Portal triad injury was never specifically diagnosed preoperatively. Extrahepatic bile duct injury occurred in 4 patients, portal vein injury in 14, and hepatic artery injury in 7; 3 patients had combined injuries. Eleven patients (52%) died, all due to uncontrolled hemorrhage from either an injured portal vein or associated intra-abdominal injuries. Management of the bile duct injuries included drainage alone, bile duct ligation, and Roux-Y hepaticojejunostomy. Survivors of portal vein injury were managed with lateral venorrhaphy. Ligation of the hepatic artery appeared to be optimal for injuries incurred by this vessel. Complications necessitating reoperation or percutaneous drainage procedures were encountered in 8 of 10 surviving patients (80%). Injuries to the portal triad are uncommon, difficult to diagnose, and technically challenging. Mortality is most directly related to uncontrolled intraabdominal hemorrhage, and salvage requires rapid control of bleeding as the first treatment priority.     

Lethal triad in severe burns

IntroductionHypothermia, acidaemia and coagulopathy in trauma is associated with significant mortality. This study aimed to identify the incidence of the lethal triad in major burns, and describe demographics and outcomes.MethodsPatients admitted during a 71 month period with a total body surface area burn (TBSA) ≥ 30% were identified. A structured review of a prospective database was conducted. The lethal triad was defined as a combination of coagulopathy (International normalised ratio > 1.2), hypothermia (temperature ≤ 35.5 °C) and acidaemia (pH ≤ 7.25).ResultsFifteen of 117 patients fulfilled the criteria for the lethal triad on admission. Lethal triad patients had a higher median (IQR) abbreviated burn severity index (ABSI) (12 (9–13) vs. 8.5 (6–10), p = 0.001), mean (SD) TBSA burn (59.2% (18.7) vs. 47.9% (18.1), p = 0.027), mean (SD) age (46 (22.6) vs. 33 (28.3) years, p = 0.033), and had a higher incidence of inhalational injury (p < 0.0001) and full-thickness burns (p = 0.021). Both groups received similar volumes of fluid (p > 0.05).The lethal triad was associated with increased mortality (66.7% vs. 13.7%, p < 0.0001). With logistic regression analysis and adjustment for ABSI, the lethal triad was not shown to be a predictor of mortality (p > 0.05).ConclusionBurn patients with the lethal triad have a high mortality rate which reflects the severity of the injury sustained.     

肘关节恐怖三联征

[目的]介绍"肘关节恐怖三联征"的慨念、发生机制、损伤后肘关节稳定性变化以及诊疗中的注意事项.[方法]2008年2月～2009年7月共收治肘关节后脱位合并桡骨头和冠状突骨折患者13例.男8例,女5例,平均年龄35.7岁(17～54岁).桡骨小头骨折按照Mason分型:I型3例,Ⅱ型6例,Ⅲ型4例;尺骨冠状突骨折按照Regan-Morrey分型:I型5例,Ⅱ型6例,Ⅲ型2例.均行手术治疗,平均手术时间为伤后7.5 d(1～12 d).术后测量肘关节活动度,并对肘关节功能采用Mayo肘关节功能评分(MEPS)进行评分.[结果]本组13例患者均得到随访,平均随访19个月(14～31个月). 次随访肘关节平均屈伸范围117°;平均前臂旋转140°骨折均达到骨性愈合,2例出现异位骨化.5例患者术后1年肘关节活动时仍感疼痛.随访结束时平均MEPS评分81分(78～96分),其中优4例,良6例,可2例,差1例.[结论]上肢外展、前臂外翻及向后外侧旋转时的高能量损伤是发生肘关节在联损伤的主要原因,这种损伤导致了肘关节的严重小稳.早期手术恢复肘关节稳定、术后早期功能锻炼足预防肘关节三联损伤并发症的关键.     

Terrible triad of the shoulder

The purpose of this study was to assess the functional outcome of the terrible triad of the shoulder, a rare combination of anterior shoulder dislocation, massive rotator cuff tear, and neurologic injury. Six patients with this condition have been treated at our institution since 1990. The mean age was 57 years. All patients underwent rotator cuff repair (RCR). The mean time from injury to surgery was 5 months. Follow-up averaged 5.6 years. Functional outcome was recorded by use of the Shoulder Pain and Disability Index. Preoperative mean active forward elevation was 24 degrees, as compared with 98 degrees postoperatively. Strength improved from 3 lb to a mean of 12 lb in forward elevation and from 2 lb to 16 lb in external rotation. Clinically, 5 of 6 patients achieved recovery of their nerve injury. Total Shoulder Pain and Disability Index postoperative scores revealed good or excellent results in 4 of 6 patients. For this injury pattern, performing an RCR offers the patient the best chance for a favorable outcome. Waiting for neurologic recovery before performing RCR is not recommended.     

Testicular histology in triad syndrome

A twenty-year-old patient with the triad syndrome had a Sertoli-cell-only histologic appearance in his intra-abdominal testes. Testicular histology was then reviewed in 6 other boys with the triad syndrome, and no spermatogonia were seen. While fertility is doubtful in patients with this syndrome, they additionally may not be at risk for germ cell testicular tumors.