Granular cell tumor (Abrikossoff tumor) of the thyroid gland

Only 4 cases of granular cell tumor of the thyroid have been previously reported in the English literature. We report herein the fifth case of this uncommon neoplasm and the first one affecting a male patient. The tumor was well circumscribed and located to the left thyroid lobe, near the isthmus. Histologically, the tumor was composed of large, rounded, granular eosinophilic cells that were immunohistochemically positive for S-100, calretinin, and PGP9.5 and negative for cytokeratin AE1-3, TTF-1, and thyroglobulin.     

Myeloperoxidase deficiency: An epidemiological study and flow-cytometric detection of other granular enzymes in myeloperoxidase-deficient subjects

Summary MPO deficiency, as first studied in the 1960s, has been recorded with increasing frequency, following the introduction of the automated cytochemical count into clinical routine. However, with regard to the diseases correlated to MPO deficiency, no exact data on the frequency of co-existance have been recorded. Moreover, the question remains whether or not a further deficiency of other granular enzymes co-exists, especially with regard to acquired MPO deficiency. In order to answer these questions, an epidemiological study of more than 70000 unselected patients was performed; the resulting prevalence of MPO deficiency was 0.15%. Within this patient group the intercellular content of elastase-like protease (ELP) and lactoferrin was measured semiquantitatively in a flow cytometer by means of indirect immunofluorescence staining. The frequency of coinciding diseases did not differ from the frequency of diaseases in the hospital patients in general. The flow-cytometric studies revealed a normal content of ELP and lactoferrin in one group and a reduced content in another, suggesting the inherited form in the former and acquired MPO deficiencies in the latter group and thus indicating that differing mechanisms characterize the two forms of MPO deficiency. Nevertheless, we do not suggest distinguishing between acquired and inherited deficiencies solely with this technique. Instead, molecular-biologic and/or genetic methods should be referred to.     

眶脂肪重置矫正上睑凹陷

目的 探讨上睑眶隔脂肪重置及眶脂肪包膜内脂肪颗粒填充矫正上睑凹陷的手术方法及疗效.方法 对48例上睑凹陷者,采用切开法重睑成形术常规切口,水平切开眼轮匝肌,剪开眶隔膜,显露眶脂肪,松解眶脂肪包膜与眶隔膜间的疏松连接,将移位的眶脂肪牵引、复位到凹陷区,并做定位缝合.对眶脂肪容量不足的患者,在凹陷区的眶脂肪包膜内注入适量脂肪颗粒,并向后上方部分推移,达眶上缘空虚的区域,复位眶隔膜以补不足,常规缝合切口.结果 48例(96只眼)中32例(64只眼)获6～42个月随访,平均随访11个月,上睑凹陷消失,重睑线流畅、两侧对称,眼部外观接近美学标准,无并发症.结论 对上睑凹陷患者,施行眶脂肪重新分配和(或)眶脂肪包膜内脂肪颗粒移植,使眶脂肪的位置和容量达到解剖结构上的恢复,有利于眼外形的美化,可取得良好的手术效果.     

Soft tissue tumors of the urinary bladder, Part I: myofibroblastic proliferations, benign neoplasms, and tumors of uncertain malignant potential

Most bladder tumors arise from the urothelium. However, there are several uncommon but significant bladder lesions that must be differentiated from urothelial carcinomas. These include both benign and malignant spindle cell lesions. The first half of this 2-part review will describe benign myofibroblastic proliferations including inflammatory myofibroblastic tumor and postoperative spindle cell nodule; benign neoplasms including leiomyoma, hemangioma, neurofibroma, and schwannoma; and tumors of uncertain malignant potential including paraganglioma, granular cell tumor, and perivascular epithelioid cell tumor. Common clinical presentations, morphological characteristics, and immunohistochemical features are described to aid the practicing pathologist in the identification of these entities. This review also describes current theories as to the pathogenesis of inflammatory myofibroblastic tumor and postoperative spindle cell nodule and details the current molecular markers identifying several of these lesions.     

Imaging of perianal granular cell tumor with lung metastasis: A case report and literature review

The granular cell tumor or Abrikossoff''s tumor is a rare tumor, most often benign in evolution. Malignant forms are exceptional. We report, here, a very rare case of granular cell tumor, localized in the perianal region, in a 54-year-old woman with lung metastases. CT and MRI with contrast showed a locally advanced tumor process in the right para-anal region associated with multiple "balloon release" lung lesions. The diagnosis was confirmed by immunostaining after surgical biopsy. Very few cases of malignant granular cell tumors with lung metastasis have been reported in the literature.     

纤维支气管镜下支气管结核冷冻疗法对肉芽增殖型支气管结核的疗效与安全性

目的 研究纤维支气管镜下支气管结核冷冻疗法对肉芽增殖型支气管结核的疗效与安全性.方法 选取扬州市第三人民医院2015年6月至2019年5月收治的肉芽增殖型支气管结核患者60例,根据治疗方案不同分为两组:对照组(n=30)给予常规规范抗结核治疗,观察组(n=30)在对照组基础上给予纤维支气管镜下冷冻治疗.观察两组治疗4周...     

长期糖尿病患者和高血压患者肾损伤随访分析

目的：跟踪分析病程10年以上的糖尿病患者和高血压患者肾脏损害情况。方法：选择2008年7月就诊的糖尿病患者40例,高血压患者30例,糖尿病高血压患者32例,病程均在10年以上,健康对照组10（3例,检测血肌酐、血尿素氮及首次晨尿微量白蛋白、颗粒管型和透明管型,并用干化学方法测定首次晨尿蛋白,之后每1年左右对患者随访检测一次,随访时间截止至2013年7月。结果：随访期间糖尿病组、高血压组及糖尿病高血压组尿微量白蛋白、颗粒管型及透明管型、千化学尿蛋白阳性率均显著高于健康体检组（P〈0．01）,并呈逐年上升趋势;颗粒管型检出率高于微量白蛋白、透明管型以及干化学尿蛋白;各组患者中干化学尿蛋白阴性的患者尿颗粒管型阳性率均显著高于健康体检组（P〈0．01）。各组患者肌酐、血尿素氮均值显著高于健康对照组（P〈0．05）,但多数仍在正常范围内,部分病例发展为肾病。结论：颗粒管型、尿微量白蛋白、尿透明管型比血肌酐、尿素氮和干化学尿蛋白更敏感,定期检查有助于糖尿病肾病和高血压肾病的早期诊断;尿蛋白阴性并不能认为没有肾脏损伤,仍需要重点观察尿管型。     

Phenotypic non-penetrance in granular corneal dystrophy type II

Purpose  To report a possible case of phenotypic non-penetrance in granular corneal dystrophy type II (GCD-II). Methods  DNA analysis was performed on 11 patients with white granular corneal opacities and 50 normal controls after informed consent was obtained. The TGFBI gene was analyzed by sequencing DNA from epidermal keratinocytes obtained using adhesive tape. Results  The heterozygous R124H mutation of TGFBI gene was found in all 11 patients. Although 49 normal controls had no mutation in the TGFBI gene, one normal control, a 26-year-old man, had the heterozygous R124H mutation of TGFBI gene. His 55-year-old father had the same mutation, but no corneal opacities. Conclusion  As not all mutations are expressed in the phenotype, GCD-II gene mutation may have non-penetrance. This report documents a possible case of phenotypic non-penetrance in GCD-II.     

CD3-negative lymphoproliferative disease of granular lymphocytes in a girl with an unusual pattern of anti-Epstein-Barr virus antibodies

We describe an 11-year-old girl who initially had mild hepatosplenomegaly and then presented with abnormal expansion of CD3-negative granular lymphocytes in peripheral blood and Epstein-Barr virus (EBV) genome in the DNA obtained from the peripheral blood mononuclear cells (PBMNC). After approximately 3 years, she developed oedema, ascites, marked hepatosplenomegaly, and pancytopenia, and showed both a profile of anti-EBV antibodies of reactivated infection and a high titre of anti-cytomegalovirus antibody. Although she was treated with antibiotics, ganciclovir, and prednisolone, she died of hepatic failure.Conclusion Careful clinical observation, periodic examination of anti-EBV antibodies, and the analysis of EBV genome from PBMNC are needed in young patients with CD3-negative lymphoproliferative disease of granular lymphocytes.     

Granular cell tumor of the penis: clinicopathologic evaluation of 9 cases

The occurrence of granular cell tumor (GCT) in penile tissue is very rare, with only 9 examples reported to date in the English-language literature. Herein, we describe the clinicopathologic and immunohistochemical findings in 9 additional cases. The patients ranged in age from 20 to 60 years (mean, 42 years; median, 40 years) at time of diagnosis. All penile tumors were solitary and arose in the dermis of the penile shaft (n=4), prepuce (n=3), and corona (n=2). A patient had a history of multiple cutaneous GCTs. Duration of symptoms before surgery ranged from 5 days to 2 years with the presence of an asymptomatic nodule representing the most common tumor-related complaint (n=8). The lesions ranged in size from 0.6 to 2.5 cm (mean, 1.5 cm; median, 1.5 cm). Microscopically, the tumors were moderate to highly cellular and were composed of oval to polygonal-shaped cells with abundant coarsely granular eosinophilic cytoplasm. Tumor cells grew in infiltrating nests, cords, and trabeculae and showed neural (n=2) and vessel wall (n=1) invasion or formed a relatively well-marginated solid nodule. Bland cytological features with only rare cells showing nucleomegaly (n=7) or spindling (n=3) were exhibited by 8 tumors. A tumor demonstrated diffuse nuclear atypia and was classified as "atypical." Mitotic activity ranged from 0 to 8 mitoses (mean, 1.4 mitoses) per 50 high-powered fields with no atypical division figures identified. All tumors tested showed moderate to strong immunohistochemical expression of S100 protein (n=6) and low-affinity nerve growth factor receptor (n=5), which was useful for detecting small deposits of tumor and helpful in evaluating surgical margins. Focal tumor cell immunoreactivity was observed for calretinin (4/6 cases) and glial fibrillary acidic protein (1/6 cases). All patients underwent simple (local) excision of their tumor. Complete follow-up data (mean, 21 years; interval range, 0.5-28 years) were available for 6 patients. No patient experienced recurrence or metastatic spread of tumor although surgical margins were microscopically involved by tumor in 5 cases. Benign GCT involving superficial soft tissue of the penis can be adequately managed by a simple excision. Patients with microscopically involved surgical margins can be clinically followed without immediate additional surgery.