BACKGROUND & AIMS: :Malignant transformation of synovial chondromatosis into chondrosarcoma is unusual. Thirteen cases and one series have been reported; only four of them developed in the hip. The overall survival is about 50%, possibly because of the difficulty of arriving at a correct early diagnosis (radiographically and histologically) and subsequent adequate surgical therapy. We report two patients (ages 30 and 50 years) in whom synovial chondrosarcoma developed in previously excised synovial chondromatosis of the hip. The diagnosis was made with modern imaging techniques (computed tomography and magnetic resonance imaging) and verified by open biopsy. The early recognition allowed a wide limb-saving resection; both patients are disease free 3 and 2 years after surgery.

背景与目标： : 滑膜软骨瘤病向软骨肉瘤的恶性转化是不常见的。已报告了13例和一系列; 其中只有四例在臀部发育。总生存率约为50%，可能是因为难以获得正确的早期诊断 (放射学和组织学) 以及随后的适当手术治疗。我们报告了两名患者 (年龄分别为30岁和50岁)，其中滑膜软骨肉瘤是在先前切除的髋关节滑膜软骨瘤中发展的。诊断是通过现代成像技术 (计算机断层扫描和磁共振成像) 进行的，并通过开放活检进行了验证。早期识别允许广泛的肢体切除; 两名患者在手术后3年和2年均无病。

BACKGROUND & AIMS: :Immune therapy have shown new and exciting perspectives for cancer treatment. Aim of our study was to evaluate toxicity and possible adverse effects from vaccination of patients with advanced colorectal cancer with autologous dendritic cells (DC) pulsed with lysate from a newly developed melanoma cell line, DDM-1.13. Six patients were enrolled in the phase I trial. Autologous DCs were generated in vitro from peripheral blood monocytes in the presence of granulocyte-macrophage colony-stimulating factor (GM-CSF) and interleukin-4 (IL-4). DCs were pulsed with melanoma cell lysate from a cloned and selected melanoma cell line enriched in expression of MAGE-A antigens and deficient in expression of melanoma differentiation antigens: tyrosinase, MART-1 and gp100. Vaccinations were administered intradermally on the proximal thigh with a total of five given vaccines at 2 weeks intervals. Each vaccine contained 3-5 x 10(6) DCs. Five of the six patients received all five vaccines. The treatment was well tolerated in all patients without any observed vaccine-correlated adverse effects. Treatment with this DC-based cancer vaccine proved safe and non-toxic.

背景与目标： : 免疫疗法为癌症治疗展示了新的令人兴奋的观点。我们研究的目的是评估用新开发的黑色素瘤细胞系DDM-1.13的裂解液对自体树突状细胞 (DC) 进行疫苗接种的毒性和可能的不良反应。6名患者参加了I期试验。在存在粒细胞-巨噬细胞集落刺激因子 (gm-csf) 和interleukin-4 (IL-4) 的情况下，从外周血单核细胞体外产生自体dc。用来自克隆和选择的黑色素瘤细胞系的黑色素瘤细胞裂解液对dc进行脉冲，该细胞系富含MAGE-a抗原的表达，并且缺乏黑色素瘤分化抗原的表达: 酪氨酸酶，MART-1和gp100。在大腿近端皮内接种疫苗，每隔2周共接种5种疫苗。每种疫苗含有3-5x10(6) dc。六名患者中有五名接受了全部五种疫苗。所有患者的治疗耐受性良好，没有观察到任何与疫苗相关的不良反应。使用这种基于DC的癌症疫苗进行治疗被证明是安全且无毒的。

BACKGROUND & AIMS: BACKGROUND:To determine guidelines for the management of perineal small bowel fistula (PSF) after total or posterior pelvic exenteration. METHODS:During 15 years, 315 curative pelvic exenterations were performed. PSF occurred in 15 patients (3.5%). We retrieved the precise modality of radiotherapy (fields and doses) and management of all patients (type of surgery, number of surgery and mortality). Delay of occurrence was divided in early (within 30 days or before hospital discharge) and delayed. RESULTS:All patients underwent surgery. Mortality rate was 13%. Fourteen patients (93%) had history of radiotherapy. No PSF was noted after anterior pelvic exenteration. Higher frequency of PSF was noted after total pelvic exenteration versus posterior pelvic exenteration (P = 0.04). Early PSF occurred in four patients (27%) with higher frequency of small bowel intraoperative injury. Late PSF occurred in 11 patients (73%) divided in small bowel injury in contact with pelvic staples (n = 4) and disease recurrence (n = 6, local recurrence or carcinomatosis). One patient had delayed PSF by ulceration of small bowel in contact with pelvic drain. CONCLUSION:PSF was a life-threatening complication of pelvic exenteration. Radiotherapy leads to weaken small bowel with difficulty of cicatrisation. During pelvic exenteration: (a) extreme careful dissection and interposition of great omentum could avoid small bowel injury, (b) control of pelvic vessels and closure of rectum remnant should not used staplers. Intraoperative management of PSF used successful simple repair in case of early PSF or segmentary resection indeed enlarged to right colon in case of delayed PSF. Postoperative courses had to use intravenous hyperalimentation and digestive tract discharge.

背景与目标：

BACKGROUND & AIMS: BACKGROUND:Cholecystocolocutaneous fistula (CCCF) is a rare complication of gallstone disease resulting from spillage of gallstones from perforation of an empyema of the gallbladder, which can pose diagnostic dilemmas. We describe a patient, who presented initially with a swelling followed by discharging sinuses on her right flank where a diagnosis of CCCF was made and was treated surgically with satisfactory outcome. METHODS:A computed tomography (CT) scan showed an ill-defined soft tissue mass in the right subhepatic space and a fistulogram demonstrated passage of contrast into the gallbladder fossa and hepatic flexure of colon. At laparotomy, a cutaneous fistula containing two pigment stones led to the gallbladder fossa and hepatic flexure of colon. RESULTS:Debridement of infected granulation tissues which had replaced the gallbladder, closure of the cystic duct stump and colonic fistula followed by excision of the fistula tract led to complete resolution. CONCLUSIONS:CCCF is a rare complication of perforated gallbladder with spillage of calculi, and a fistulogram is helpful in establishing the diagnosis. This case highlights the importance of retrieving spilled stones following interventions in the gallbladder to prevent the complication.

背景与目标：

BACKGROUND & AIMS: BACKGROUND:Rhinosporidiosis, a fungal infection due to Rhinosporidium seeberi, frequently produces polypoidal lesions in the nose. Sites like the conjunctiva, larynx, trachea, nasopharynx, skin and genitourinary tract are less frequently involved. Generalized rhinosporidiosis with skin and visceral involvement is extremely rare. This report describes the fine needle aspiration cytology (FNAC) of rhinosporidiosis occurring as a solitary lesion with erosion of cortical bone.

CASE REPORT:FNAC of a soft tissue swelling overlying a lytic lesion on the anterior aspect of the tibia was performed in a 40-year-old male. Smears revealed numerous sporangia and spores of R seeberi. There were no mucocutaneous lesions. Histologic examination confirmed the bony involvement.

CONCLUSION:The FNAC diagnosis of rhinosporidiosis is specific. Preoperative diagnosis is possible even in cases with unusual clinical presentations.

背景与目标： 背景 : 鼻孢子虫病是由seeberi鼻孢子虫引起的真菌感染，经常在鼻子中产生息肉样病变。结膜，喉，气管，鼻咽部，皮肤和泌尿生殖道等部位较少受累。皮肤和内脏受累的全身性鼻孢子虫病极为罕见。本报告描述了鼻孢子虫病的细针穿刺细胞学检查 (FNAC)，该检查是孤立的皮质骨侵蚀病变。
病例报告 : 在40岁的男性中，对胫骨前部的溶解性病变进行了软组织肿胀的FNAC。涂片显示出许多孢子囊和R seeberi的孢子。没有皮肤粘膜病变。组织学检查证实了骨受累。
结论 : 鼻孢子虫病的FNAC诊断是特异性的。即使在临床表现异常的情况下，也可以进行术前诊断。

BACKGROUND & AIMS: BACKGROUND:The effects of antipsychotic drug withdrawal have been inadequately studied. Case reports have described dyskinesia occurring in patients with several antipsychotics withdrawn, but studies on amisulpride withdrawal dyskinesia are lacking. CASE PRESENTATION:A 63-year-old man, who was diagnosed with schizophrenia at age 49, received amisulpride treatment since age 62. The dosage of amisulpride was reduced from 200 to 50 mg/day because of occurrence of akathisia during one admission. Severe withdrawal dyskinesia, mixed with dystonia and akathisia, was noted immediately after the dosage reduction. The abnormal involuntary movement showed improvement 2 weeks later when the dosage was increased to 100 mg/day. CONCLUSIONS:Withdrawal dyskinesia and other abnormal involuntary movements could be noted in a patient with reduction of amisulpride dosage or sudden termination. Withdrawal dyskinesia may subsequently lead to persistent tardive dyskinesia. Therefore, withdrawal-emergent dyskinesia should be promptly identified, and appropriate medical interventions should be given early.

背景与目标：

BACKGROUND & AIMS: :Proliferating trichilemmal tumor (PTT) is a rare but morphologically distinct tumor that usually arises on the scalp of elderly women. It is composed of multiple cysts consisting of squamous epithelium with trichilemmal keratinization without granular layer interposition. Vulvar proliferating trichilemmal cyst is very rare, with, to the best of our knowledge, only 3 cases previously reported in the literature. We describe a 39-year-old woman with recurrent PTT on the left labium majus of the vulva, which had been excised from the same side 5 years before. She had a palpable nodule, approximately 2 cm in size, which was firm, mobile, and nontender; without erythema and ulceration; and covered by normal skin on the vulva. There was no inguinal lymphadenopathy. The lesion was removed by wide surgical excision; because of the tissue elasticity, primary closure was possible. The pathology result was reported as proliferating trichilemmal carcinoma with tumor-free margins. Although local recurrence after wide excision is rare, we recommend complete excision for treatment of PTT and long-term follow-up because of the possibility of recurrence.

背景与目标： 增生性毛囊瘤 (PTT) 是一种罕见但形态上不同的肿瘤，通常发生在老年妇女的头皮上。它由多个囊肿组成，由鳞状上皮组成，具有三鞘角化，没有颗粒层插入。外阴增生的毛囊囊肿非常罕见，据我们所知，以前文献中仅报道了3例。我们描述了一名39岁的女性，在外阴的左阴唇上复发性PTT，该女性在5年前从同一侧切除。她有一个可触及的结节，大小约2厘米，坚硬，可移动且不触痛; 没有红斑和溃疡; 外阴被正常皮肤覆盖。无腹股沟淋巴结肿大。通过广泛的手术切除切除病变; 由于组织弹性，可以进行一次闭合。病理结果报告为增殖性毛囊癌，边缘无瘤。尽管广泛切除后的局部复发很少见，但由于复发的可能性，我们建议完全切除治疗PTT并进行长期随访。

BACKGROUND & AIMS: :The aim of this study was to explore how the level of shiftwork exposure during an individual's working life might be related to subjectively reported sleep quality and timing during retirement. Telephone interviews regarding past employment and sleep timing and quality (among other variables) were conducted using a pseudo-random age-targeted sampling process. Subjective sleep quality was assessed using a telephone version of the Pittsburgh Sleep Quality Index. Timing of reported habitual bedtimes and rise-times were assessed using the Sleep Timing Questionnaire. Questions measuring morningness and subjective health were also given. Retired seniors (aged >65 years, n = 1113) were studied. Analysis was by analysis of variance, with shiftwork exposure in three bins [0 (n = 387), 1-15 (n = 371) and >15 years (n = 355)], gender (n = 634 male, 479 female) and former occupation [in two broad categories, 'managerial' (n = 437) versus 'other' (n = 676)] as factors. In retired shiftworkers, relative to retired day workers, past exposure to shiftwork was associated with higher (worse) PSQI scores by 1.0 units (1-15 years) and 0.6 units (>15 years) (main effect P = 0.005). There were also main effects of gender and former occupation (males and managerials reporting better sleep), but neither variable interacted with shiftwork exposure. The timing of current mean habitual bedtimes and rise-times (and also the variance around them) were very similar for the three shiftwork exposure groups. The shiftwork exposure effect did not appear to be mediated by either morningness or current health. Prior exposure to shiftwork would appear to be related to currently reported sleep problems during retirement.

背景与目标： : 这项研究的目的是探讨个人工作期间的轮班工作暴露水平可能与主观报告的睡眠质量和退休时间有关。使用伪随机年龄目标抽样过程进行了有关过去工作，睡眠时间和质量 (以及其他变量) 的电话采访。使用电话版本的匹兹堡睡眠质量指数评估主观睡眠质量。使用睡眠时间问卷评估报告的习惯性就寝时间和上升时间的时间。还给出了衡量早晨和主观健康的问题。研究了退休老年人 (年龄> 65岁，n = 1113)。通过方差分析进行分析，在三个区间 [0 (n = 387)，1-15 (n = 371) 和> 15岁 (n = 355)]，性别 (n = 634男性，479女性) 和以前的职业 [在两个大类中，作为因素的 “管理” (n = 437) 与 “其他” (n = 676)]。在退休的轮班工人中，相对于退休的日间工人，过去暴露于轮班工作与1.0单位 (1-15岁) 和0.6单位 (>15岁) 的PSQI得分较高 (较差) 相关 (主要影响P = 0.005)。性别和以前的职业也有主要影响 (男性和管理人员报告睡眠更好)，但这两个变量都与轮班暴露相互作用。对于三个轮班暴露组，当前平均习惯性就寝时间和上升时间 (以及它们周围的变化) 的时间非常相似。轮班工作暴露效应似乎不是由早上或当前的健康状况介导的。先前接触轮班工作似乎与退休期间目前报告的睡眠问题有关。

BACKGROUND & AIMS: :Leiomyosarcoma of the innominate vein is a rare but usually lethal disease. We report the case of a 50-year-old woman, undergoing a curative resection of the tumor. She is alive and free of disease at 88-month follow-up. Surgical excision remains the current optimal treatment able to provide a chance of cure. KEY WORDS: Late survival, Venous leiomyosarcoma.

背景与目标： : 无名静脉平滑肌肉瘤是一种罕见但通常致命的疾病。我们报告了一名50岁的女性，正在接受肿瘤的根治性切除。在88个月的随访中，她还活着并且没有疾病。手术切除仍然是目前能够提供治愈机会的最佳治疗方法。关键词: 晚期生存，静脉平滑肌肉瘤。

BACKGROUND & AIMS: :Transabdominal fine needle aspiration biopsy of a solitary space-occupying lesion in the liver produced smears containing irregular bundles of smooth muscle cells with granular or fibrillary cytoplasm and slightly pleomorphic nuclei. In a few bundles, aggregates of mature fat cells were present, which is characteristic for an angiomyolipoma. Histologic examination of the resected mass showed it to be a solitary angiomyolipoma of the liver. The diagnosis was further confirmed by immunohistochemical and electron microscopic studies.

背景与目标： : 经腹细针穿刺活检肝脏孤立性占位病变产生的涂片，其中包含不规则的平滑肌细胞束，具有颗粒状或原纤维的细胞质和轻度多形性核。在几束中，存在成熟脂肪细胞的聚集体，这是血管平滑肌脂肪瘤的特征。切除的肿块的组织学检查显示它是肝脏的孤立性血管平滑肌脂肪瘤。免疫组织化学和电子显微镜研究进一步证实了诊断。

BACKGROUND & AIMS: BACKGROUND:Expanded polytetrafluoroethylene sheets have been successfully used as a biologic barrier in the treatment of pacemaker contact dermatitis. TECHNIQUE:In this report, we describe two patients with contact dermatitis related to the implantable pulse generator of a spinal cord stimulator who were treated with expanded polytetrafluoroethylene as a barrier. RESULTS:Resolution of the dermatitis was achieved in both cases. CONCLUSIONS:This technique is an effective way of treating contact dermatitis related to implantable pulse generators, without the need for explantation of the neuromodulation system.

背景与目标：

BACKGROUND & AIMS: :Serous carcinomas most commonly arise within the uterine corpus or ovary/fallopian tube, but there are 2 prior case reports of primary vaginal serous carcinoma. We report 2 examples of high-grade serous carcinoma arising within the urethra or a urethral diverticulum (1 case each). Both neoplasms exhibited the classic morphologic features of high-grade serous carcinoma, and a combination of clinical, radiologic, and pathologic examination excluded other possible sites of primary neoplasm.

背景与目标： : 浆液性癌最常见于子宫体或卵巢/输卵管，但有2例原发性阴道浆液性癌。我们报告了2例尿道或尿道憩室内发生的高级别浆液性癌 (各1例)。两种肿瘤均表现出高级别浆液性癌的经典形态特征，并且临床，放射学和病理学检查的组合排除了原发肿瘤的其他可能部位。

BACKGROUND & AIMS: :Spindle cell rhabdomyosarcoma is an uncommon subtype of embryonal rhabdomyosarcoma. Found almost exclusively in children, these tumors are classically located in the paratesticular and head and neck regions. Morphologically these lesions can resemble several other benign or malignant soft-tissue spindle cell lesions, especially smooth muscle or myofibroblastic tumors, and thus immunohistochemical staining is often needed to prove skeletal muscle differentiation. Although there is extensive literature reporting the genetics of embryonal rhabdomyosarcoma, little is reported specific to the spindle cell subtype. Below we present the case of a 7-month-old male presenting with a large posterior neck mass that was diagnosed as spindle cell rhabdomyosarcoma. Karyotype evaluation revealed a t(6;8) (p12;q11.2) chromosomal translocation within the lesion. We review the histologic and immunohistochemical diagnosis of these tumors and discuss the genetics of rhabdomyoscarcomas.

背景与目标： : 梭形细胞横纹肌肉瘤是胚胎性横纹肌肉瘤的一种罕见亚型。这些肿瘤几乎只在儿童中发现，通常位于睾丸旁和头颈部区域。从形态上讲，这些病变可能类似于其他几种良性或恶性软组织梭形细胞病变，尤其是平滑肌或肌纤维母细胞瘤，因此通常需要免疫组织化学染色来证明骨骼肌的分化。尽管有大量文献报道了胚胎横纹肌肉瘤的遗传学，但很少有针对梭形细胞亚型的报道。下面我们介绍一名7个月大的男性，其后颈部肿块较大，被诊断为梭形细胞横纹肌肉瘤。核型评估显示病变内有t(6;8) (p12;q11.2) 染色体易位。我们回顾了这些肿瘤的组织学和免疫组织化学诊断，并讨论了横纹肌肉瘤的遗传学。

BACKGROUND & AIMS: OBJECTIVES:There is paucity of data on Primary immunodeficiency disorders (PID) from India. Here we describe the frequency of different primary immunodeficiency disorders, their clinical features and disease complications of 159 patients with PID diagnosed in a tertiary care center from India over the last 3 years. METHODS:We retrospectively reviewed the records of all the patients identified to have specific PID from 2008 to 2011. The diagnosed patients were classified according to guidelines of International Union of Immunological Society (IUIS) into eight different sub groups. RESULTS:The distribution pattern was as follows: diseases of immune dysregulation (29 %), phagocytic defects (29 %), predominant antibody deficiency (13 %), combined T and B cell deficiency (19 %) and other well defined diseases (10 %). CONCLUSION:The distribution pattern of PID varied significantly from those reported by western studies. This study highlights the need for development of more advanced facilities for diagnosis and management of PID in India and also the need for establishing population and hospital based registries.

背景与目标：

BACKGROUND & AIMS: :Chondrolipoma is an extremely rare variant of lipoma with cartilaginous metaplasia. The presence of nonlipomatous components can lead to a variety of entities in the differential diagnosis from the radiologic findings. We describe an unusual case of a chondrolipoma occurring in the right ankle of a 9-year-old female. Physical examination showed a 3.5-cm, elastic-hard, poorly mobile, nontender mass adherent to the Achilles tendon. Plain radiographs revealed a faintly calcified soft tissue mass without bone involvement. Magnetic resonance imaging showed a well-defined mass with 2 components with heterogeneous signal intensity, suggesting the coexistence of a fatty area and another nonlipomatous component. Marginal excision of the tumor was performed. Histologically, the tumor was composed of mature adipose tissue studded with islands of mature hyaline cartilage. Based on these findings, the tumor was diagnosed as a chondrolipoma. The patient had no evidence of local recurrence within 9 months of follow-up. To the best of our knowledge, this is the first case of chondrolipoma originating from the ankle in a child.

背景与目标： : 软骨脂肪瘤是脂肪瘤的一种极其罕见的变异型软骨化生。非脂肪瘤性成分的存在可导致放射学发现的鉴别诊断疾病中的各种实体。我们描述了一个9岁女性右脚踝发生软骨脂肪瘤的不寻常病例。体格检查显示跟腱附着有3.5厘米，弹性坚硬，移动不良，不柔软的肿块。X线平片显示微弱的钙化软组织肿块，没有骨受累。磁共振成像显示出一个明确的质量，其中有2个成分，信号强度不均匀，表明脂肪区域和另一种非脂肪瘤成分并存。进行了肿瘤的边缘切除术。从组织学上讲，肿瘤由成熟的脂肪组织组成，这些脂肪组织散布着成熟的透明软骨岛。根据这些发现，该肿瘤被诊断为软骨脂肪瘤。在随访的9个月内，患者没有局部复发的证据。据我们所知，这是第一例起源于儿童脚踝的软骨脂肪瘤。