CT and MR demonstration of fat within an adrenal cortical carcinoma

Detection of fat within an adrenal primary mass is virually pathognomonic of myelolipoma, and in most cases such a diagnosis excludes any further diagnostic and therapeutic approaches. We report our findings, with computed tomography (CT) and magnetic resonance (MR), of the first, to our knowledge, pathologically proven case of fat tissue within an adrenal cortical carcinoma. We also discuss the possible origin of fat within such malignancies and the potential diagnostic dilemmas it might cause.     

肾上腺嗜酸细胞型皮质癌的临床病理特征及表皮生长因子受体基因状态

  目的  探讨肾上腺嗜酸细胞型皮质癌临床病理特征及其表皮生长因子受体(epidermal growth factor receptor, EGFR)蛋白表达、基因突变和基因拷贝数改变情况。  方法  回顾性分析2000年1月至2009年12月在北京协和医院手术切除的9例肾上腺嗜酸细胞型皮质癌的临床特点、组织形态学特征, 并以9例肾上腺嗜酸细胞型皮质腺瘤为对照, 应用免疫组织化学、Scorpion ARMS突变系统及荧光原位杂交(fluorescence in situ hybridization, FISH)的方法分析EGFR在二者中的蛋白表达、基因突变和基因拷贝数改变的情况。  结果  9例肾上腺嗜酸细胞型皮质癌中, Cushing综合征2例, 无功能性皮质癌7例。病例随访时间6~56个月, 皮质癌患者失访2例, 死亡6例。肾上腺嗜酸细胞型皮质癌中EGFR蛋白过表达率为77.8%(7/9), EGFR FISH阳性率为55.6%(5/9);仅有22.2%(2/9)皮质腺瘤存在EGFR蛋白的表达, 且EGFR FISH均为阴性。肾上腺嗜酸细胞型皮质腺瘤及皮质癌中均未检测出EGFR基因扩增及基因突变。  结论  肾上腺嗜酸细胞型皮质癌非常罕见, 其诊断依据病理学及免疫表型, EGFR蛋白过表达和7号染色体的高多体性较腺瘤更常见, 可能有助于与后者的鉴别诊断, 也有可能成为今后临床分子靶向治疗的潜在方向。     

Disseminated cryptococcosis in a patient with adrenocortical carcinoma and Cushing's syndrome

Corticosteroids have been used for the treatment of a great variety of diseases. Therefore, the relationship between high doses of cortisol and infections has been widely known and prophylactic therapy has been established. Adrenocortical carcinoma is a rare malignancy with poor prognosis, so little is known about how to deal with the complications resulting from hormone excess such as Cushing's syndrome. Here we report a case of an 82-year-old woman who presented with a 1-day history of dyspnea and was finally diagnosed as disseminated cryptococcosis at autopsy. The patient had received a diagnosis of metastatic adrenocortical carcinoma and Cushing's syndrome three months earlier. The findings of this study indicate the relation between severe hypercortisolemia due to adrenocortical carcinoma and opportunistic infections. In the setting of adrenocortical carcinoma with Cushing's syndrome, clinicians should maintain a high index of suspicion for opportunistic infections including cryptococcosis, which are potentially treatable by early detection and prompt intervention.     

肾上腺皮质嗜酸细胞瘤1例报告并文献复习

目的：探讨肾上腺皮质嗜酸细胞瘤临床、病理学特征及治疗方法。方法：报告1例肾上腺皮质嗜酸细胞瘤患者临床资料并复习文献。结果：患者临床表现库欣综合症,皮质醇节律异常,腹部CT示左肾上腺肿瘤,行后腹腔镜下肿瘤切除手术,术后病理证实为肾上腺皮质嗜酸细胞瘤。术后随访12个月,患者未见肿瘤复发和转移。结论：肾上腺皮质嗜酸细胞瘤临床罕见,大部分为偶发瘤。良性肿瘤多见,手术切除肿瘤为首选治疗,预后良好。     

以颈部囊性转移为首发症状的扁桃体鳞状细胞癌一例及文献复习

目的：探讨以颈部囊性转移为首发症状的扁桃体鳞状细胞癌的临床病理组织学特点、诊断及鉴别诊断。方法复习1例以颈部囊性转移为首发症状的扁桃体鳞状细胞癌的相关临床资料,对其进行免疫组织化学及HPV基因分型检测,并结合文献进行探讨。结果患者临床症状与体征酷似颈部淋巴结核,病理组织学与鳃裂癌相似。免疫组织化学染色 p16阳性,P53（＋）,Ki-67指数约20%,HPV 基因分型HPV16型阳性。结论扁桃体鳞状细胞癌以首发颈部囊性转移性包块症状者少见,其临床需要与颈部淋巴结核及鳃裂癌鉴别,避免误诊。     

肾上腺疾患的声学鉴别诊断

本文回顾性分析了64例肾上腺及其相关病变的超声波表现并进行了统计学检验，以探讨肾上腺病变的声学鉴别诊断规律。文章证明肾上腺皮质腺瘤为单侧低回声小结节；皮质增生为边界不清的“胖三角”，双侧发病更具特征；髓样脂肪瘤为不均匀强回声肿块；节细胞神经瘤和神经鞘瘤为低回声肿块，可有液化和钙化；嗜铬细胞瘤以瘤内液化囊变为特征；皮质腺癌为较大的混杂回声肿块，半数有钙化；转移瘤有明确的原发灶病史。     

脂肪对比剂灌肠螺旋CT在结直肠癌术前分期中的作用

目的探讨脂肪对比剂螺旋CT对结直肠癌术前分期的价值.方法回顾性分析29例经手术病理证实的结直肠癌患者术前脂肪对比剂灌肠螺旋CT资料,扫描前充分肠道准备、肌注低张剂后,经直肠导管注入脂肪对比剂(CT值-200～-240)1500 ml,行螺旋CT增强扫描,扫描范围从膈顶至耻骨联合.结果脂肪对比剂灌肠低张螺旋CT总的分期准确率为65.5%(19/29).B期分期的准确率为75%(9/12); C期分期的准确率63.6%(7/11).肿瘤浆膜外侵犯的敏感性和特异性分别为92%(23/25)和50% (2/4).淋巴结转移的敏感性和特异性分别为60%(12/20)和90%(9/10).结论超低密度脂肪对比剂螺旋CT对结直肠癌术前分期有重要意义,有助于判断肿瘤浆膜外侵犯及淋巴结和远处转移.     

原发性肾上腺淋巴瘤的诊断与临床处理

原发性肾上腺淋巴瘤(primary adrenal lymphoma,PAL)临床表现不典型,多数患者存在局部疼痛或全身发热、消瘦症状,在双侧受累患者中肾上腺皮质功能不全很常见,但仅少数患者进行全面系统的检查。正电子发射断层扫描/计算机体层成像是定性定位诊断这类肾上腺肿瘤的有效手段,磁共振成像用来区别肾上腺淋巴瘤与皮质癌尚有待更多研究证实。对于PAL的治疗尚需进一步研究,以对此类患者制定最佳的诊疗方案。目前,尚不清楚利妥昔单克隆抗体联合CHOP方案(环磷酰胺+阿霉素+长春新碱+强的松)治疗高危侵袭性PAL患者是否可提高患者完全反应率。病变早期,尤其是在肾上腺皮质功能不全出现之前诊断PAL,有助于减少患者的发病率和死亡率。     

A case of MEN IIA variant associated with ectopic ACTH production and mammary carcinoma

A 69-year-old house wife admitted to our hospital with complaints of fever, drowsiness, watery diarrhea and uncontrolled blood sugar level. She had been operated for the right mammary carcinoma 11 years ago. Left adrenal mass and nodules in the thyroid gland were discovered by CT and hypersecretion of adrenocortical hormone, catecholamine and PTH as well as ectopic ACTH production were indicated endocrinologically. Autopsy and histopathological examination revealed left adrenal pheochromecytoma associated with bilateral adrenocortical hypertrophy, adenomas in 2 of 5 parathyroid glands and papillary thyroid carcinoma with adenomatous goiter. Ectopic ACTH production was identified immunohistochemically in the pheochromecytoma. This case is a rare variant of non-familial MEN (multiple endocrine neoplasm) type IIA with ectopic ACTH production and metachronous mammary carcinoma.     

Adrenocortical carcinoma arising from a long-standing adrenal mass

Adrenocortical carcinoma is a rare tumor with a dismal prognosis. In stark contrast, benign incidental adrenal lesions are detected commonly on routine abdominal imaging. We report a case of a 74-year-old man with a history of germ cell testicular carcinoma who presented with a 4.8-cm left adrenal lesion. The lesion remained stable for 8 years, at which time the patient became symptomatic from an excess of cortisol hormone. Biopsy findings confirmed adrenocortical carcinoma. We describe the presentation, clinical findings, diagnostic work-up, and follow-up of this patient and review the literature.     

Molecular adrenocortical tumourigenesis

Adrenocortical neoplasms are the most frequent abnormality of the adrenal cortex. Most of these lesions are clinically silent and are detected incidentally by ultrasound or computed tomography. The prevalence of these so-called 'incidentalomas' in the general population is around 1%, increasing with age and reaching 6% among those in the age range 60–70 years. In contrast, primary adrenocortical carcinoma, a highly malignant tumour, is rare, having an incidence of one case per million per year. Recent progress has been achieved in the understanding of adrenocortical tumourigenesis by mapping and identification of genes responsible for hereditary tumours that involve the adrenal gland. Investigation of the clonal composition of adrenal tumours demonstrates that adrenal carcinomas are monoclonal, whereas adrenal adenoma may be polyclonal in approximately 25–40% of cases. Oncogenes and tumour-suppressor genes involved in adrenal carcinomas include mutations in the p53 tumour-suppressor gene and rearrangements of the chromosomal locus 11p15.5 associated with IGF II hyperexpression. Constitutive activation of the ACTH receptor-G protein-cAMP signal cascade does not play a role in adrenal tumour formation. Conversely, deletions of the ACTH receptor gene have recently been found in undifferentiated adenomas and in aggressive adrenocortical carcinomas, and, more recently, confirmed in a larger series of tumours. The available literature indicates that the signalling pathways of adrenocortical tumours are different from those of other endocrine neoplasms, such as pituitary and thyroid adenomas.     

肾上腺皮质腺瘤CT诊断（附31例分析）

本文报道31例经手术病理证实的肾上腺腺瘤。发现61％的腺瘤位于腺体的表面。认为CT扫描可以从结节的大小、增强前后密度及肿块与邻近腺体关系较明确地诊断肾上腺皮质腺瘤。     

Adrenal Insufficiency in Critically Ill Emergency Department Patients: A Taiwan Preliminary Study

OBJECTIVE: Unrecognized adrenal insufficiency can have serious consequences in critically ill emergency department (ED) patients. This prospective pilot study of adrenal function in patients with severe illness was undertaken to determine the prevalence of adrenal dysfunction and any relation to prior herbal drug use. METHODS: In a high-volume urban tertiary care ED, adult patients with sepsis or acute myocardial infarction (AMI) were eligible for the study. Over a two-month period, a convenience sample was enrolled by the authors on arrival to the ED. Inclusion criteria were systemic inflammatory response syndrome (SIRS) criteria plus evidence of at least one organ dysfunction or cardiac marker plus electrocardiogram-proven AMI. Exclusion criteria included known corticosteroid use. Serum cortisol was measured on arrival and for those patients with a level of <15 microg/dL (<414 nmol/L), an adrenocorticotropic hormone (ACTH) stimulation test was performed. RESULTS: Of the 30 enrolled patients, 23 (77%) were suffering from severe sepsis and the other seven (23%) had an AMI. Thirteen of the 30 patients (43%; 95% CI = 25% to 65%) had serum cortisol levels of <15 microg/dL, consistent with adrenal insufficiency, nine with severe sepsis and four with an AMI. Eight (62%; 95% CI = 32% to 86%) of the 13 patients with low cortisol levels reported using herbal medications, while only two (12%; 95% CI = 1% to 36%) of the 17 with normal cortisol levels reported taking herb drugs (p = 0.01). Only two (15%; 95% CI = 2% to 45%) of the patients with low cortisol levels failed their corticotropin stimulation test, suggestive of true adrenocortical insufficiency. Both reported using herbal preparations. CONCLUSIONS: These results indicate that adrenal dysfunction is common among a group of critically ill patients seen in this Taiwanese ED. Moreover, the use of herbal drugs was high in the patients with low serum cortisols. Further studies are required to both confirm these findings and clarify whether a number of herbal medications contain corticosteroids.     

ACTH-independant macronodular adrenal hyperplasia: imaging findings of a rare condition

Endogenous Cushing’s syndrome is a relatively rare disease. Most cases being ACTH-dependent, ACTH-independent Cushing’s syndrome (AICS) is an even rarer condition [15%–20%]. In more than 95% cases the cause of AICS is unilateral adrenal enlargement caused by adenoma or carcinoma. Bilateral adrenal disease is caused by primary pigmented nodular adrenal dysplasia (PPNAD) and ACTH-independent macro nodular hyperplasia (AIMAH). Only few case reports of the latter condition exist in the radiology literature, PPNAD being the commoner of two as the cause for AICS.     

Interventional ultrasound for adrenal masses

Purpose To determine the value of interventional ultrasound (US) for adrenal masses, especially incidentally discovered adrenal masses. Methods Demographic, clinical, and pathological data were reviewed for eight patients who underwent percutaneous US-guided puncture or biopsy for adrenal masses from September 1994 through March 2002 in our institute. Results US-guided intervention was successfully performed for seven patients: two with adrenal cysts, two with adrenocortical adenomas, and three with metastatic adrenal tumors (one from prostate cancer, one from lung cancer, and one from renal cell carcinoma). The remaining patient had bilateral adrenal masses, and a biopsy specimen could not be obtained because safe puncture was difficult. For all patients there was no postoperative hemorrhage or pain, and no major complications were observed during the procedure. Conclusions Interventional US using the color Doppler method for adrenal masses is a useful procedure for safe puncture to reveal the orientation of adjacent viscera and blood vessels at the puncture site and to avoid complications including hemorrhage and pneumothorax. US, including color Doppler US, is also useful for detection of complications and follow-up studies because it is noninvasive and can be used for real-time examinations. In addition, pathological examination of specimens obtained by percutaneous biopsy or fine needle aspiration is useful for avoiding unnecessary surgery in patients with metastatic adrenal masses.     

Rectal carcinoma: Preoperative staging and detection of postoperative local recurrence with transrectal and transvaginal ultrasound

Transrectal ultrasound (TRUS) was performed preoperatively in 35 patients with rectal carcinoma and the results were compared to histologic findings. In the same group, postoperative studies were performed in 22 patients; in women, transvaginal ultrasound (TVUS) was added to the transrectal study. According to Duke's classification modified by Astler-Coller, in relation to the T parameter, TRUS correctly staged 33 of 35 neoplasms (accuracy, 94.3%); one was overstaged and one was understaged. In detection of lymph node involvement, accuracy was 74% (sensitivity 69%, specificity 73.9%). Recurrent local tumors, histologically confirmed, developed in two of 22 postoperative patients who had undergone curative anterior resection. This study demonstrates that TRUS is an accurate method in preoperative staging of rectal carcinoma. In the prospective study, the role of follow-up TRUS and TVUS in detection of local recurrences is evaluated.     

Assessment of distant metastases with ultrasound-guided fine-needle aspiration biopsy and cytologic study in carcinoma of the esophagus and gastroesophageal junction

The use of ultrasound (US)-guided fine-needle aspiration biopsy (FNAB) for the assessment of distant metastases was prospectively studied in 135 consecutive patients with carcinoma of the esophagus and gastroesophageal junction. Patients with accessible lesions on US and computed tomographic (CT) studies of the supraclavicular regions and the abdomen underwent US-guided FNAB. In patients with multiple lesions biopsies were preferentially performed on enlarged supraclavicular nodes. Forty-nine patients underwent US-guided FNABs of 53 lesions. A cytologic diagnosis was established in 46 of 53 (87%) biopsies. Seven of 53 (13%) biopsies were nondiagnostic. Distant metastases were diagnosed by means of cytologic study in 33 of 135 (24%) patients. Supraclavicular metastases were diagnosed in 22 patients and abdominal metastases were diagnosed in 12 patients, including one patient who also had supraclavicular metastases. US-guided FNAB can improve the selection of patients for surgical and nonsurgical treatment by diagnosing distant metastases in an important number of patients.     

肾上腺髓性脂肪瘤的超声诊断

本文报告了由超声诊断并经手术及病理检查证实的３例肾上腺髓性脂肪瘤。以肾上腺区包膜清晰的偏强回声四块为声像图特征，对本病的诊断具有重要的临床意义。与ＣＴ结果对照，Ｂ超对本病的诊断不亚于ＣＴ。文中讨论和分析了定位诊断中与肾脏肿瘤、肝脏肿瘤、胰腺肿瘤及定性诊断中与肾上腺皮质腺瘤、肾上腺嗜铬细胞瘤、转移性肾上腺肿瘤等疾病的鉴别要点。     

Pericaval fat collection mimicking an intracaval lesion on CT in patients with chronic liver disease

Background: Localized fat collections, which mimic fat-containing lesions of the inferior vena cava (IVC) on computed tomographic (CT) images, have been reported as rare incidental findings. Our goal was to evaluate the association of this CT finding with chronic liver disease. Methods: Sixty-one patients with chronic liver disease were prospectively studied with contrast-enhanced abdominal CT. The prevalence, attenuation, location, shape, and size of the pericaval fat collections were assessed. Multidirectional reformatted CT images were obtained from helical CT data to identify origins of the pericaval fat collections. Sixty-one patients without chronic liver disease were studied as control subjects. Results: Pericaval fat collections were seen on abdominal CT in 16 (26.2%) of the 61 patients. On the reformatted images, the fat collections were contiguous to the fat tissue around the subdiaphragmatic esophagus in all 16 patients. The fat collections were located at the posterior aspect of the IVC in 12 patients. In the control group, pericaval fat collection mimicking an intracaval lesion was not seen. Conclusion: In patients with chronic liver disease, pericaval fat collections are not rare CT findings and their characteristic location is considered to be posterior to the IVC. It is important not to misinterpret such CT findings as abnormalities of the IVC, such as thrombus or tumors. Received: 18 October 2000/Accepted: 15 November 2000     

Inflammatory fibroid polyp of the ileum

We report a patient who presented with recurrent right upper quadrant colics after a previous cholecystectomy. Radiological examination revealed the presence of a polypoid mass in the ileum caused by an anatomopathologically proven inflammatory fibroid polyp (IFP) in the ileum. Clinical and pathological aspects related to this rare benign lesion are discussed.