Osteoclastic and pleomorphic giant cell tumors of the pancreas:A review of clinical,endoscopic,and pathologic features

Giant cell tumors of the pancreas come in three varieties-osteoclastic,pleomorphic,and mixed histology.These tumors have distinctive endoscopic,clinical,and cytological features.Giant cell tumors have a controversial histogenesis,with some authors favoring an epithelial origin and others favoring a mesenchymal origin.The true origin of these lesions remains unclear at this time.These are also very rare tumors but proper identification and differentiation from more common pancreatic adenocarcinoma is important.The risk factors of these tumors and the prognosis may be different from those associated with standard pancreatic adenocarcinoma.Recognition of these differences can significantly affect patient care.These lesions have a unique appearance when imaged with endoscopic ultrasound(EUS),and these lesions can be diagnosed via EUS guided Fine Needle Aspiration(FNA).This manuscript will review the endoscopic,clinical,and pathologic features of these tumors.

Effects of Low Concentrations of Di-(2-ethylhexyl) and Mono-(2-ethylhexyl) Phthalate on Steroidogenesis Pathways and Apoptosis in the Murine Leydig Tumor Cell Line MLTC-1

The aim of this study was to evaluate the effects of low concentrations of DEHP and MEHP on steroidogenesis in a murine Leydig tumor cell line （MLTC-1） in vitro. The result of flow cytometry analysis revealed that the proportion of apoptotic cells was significantly increased after the exposure to DEHP. All three genes （P450scc, P450c17, and 38HSD） under study showed an increased expression following exposure to DEHP or MEHP, although some insignificant inhibitory effects appeared in the 10μmol/L treatment group as compared with the controls. It was also found that DEHP or MEHP stimulated INSL3 mRNA and protein especially in the 0.001 μmol/L treatment group. Testosterone secretions were stimulated after the exposure to DEHP or MEHP. Alterations of steroidogenic enzymes and INSL3 in MLTC-1 cells might be involved in the biphasic effects of DEHP/MEHP on androgen production.

The surgical Treatment and Outcome of Pathological Fracture in Patients with Giant Cell Tumor of Bone

Between 1992 and 2008, we treated 35 patients with giant cell tumor (GCT) of bone, seven of the 35 pre-sented with a pathological fracture. The fractures were located in the femur in five, and in the humerus and radius in one patient each. The surgical treatments were curettage in six cases and wide resection in the distal radius case. Two of the seven patients developed local recurrence, giving a local recurrence rate of 29%. The local recurrence rate in GCT patients without a pathological fracture was 21%. There was a tendency for there to be a higher recurrence rate associated with fractured GCT, but no statistically significant difference was observed between the two groups. Therefore, it was considered that a pathological fracture was not a risk factor for local recurrence in GCT.

Detecting circulating tumor material and digital pathology imaging during pancreatic cancer progression

Pancreatic cancer(PC) is a leading cause of cancerrelated death worldwide. Clinical symptoms typically present late when treatment options are limited and survival expectancy is very short. Metastatic mutations are heterogeneous and can accumulate up to twenty years before PC diagnosis. Given such genetic diversity, detecting and managing the complex states of disease progression may be limited to imaging modalities and markers present in circulation. Recent developments in digital pathology imaging show potential for early PC detection, making a differential diagnosis, and predicting treatment sensitivity leading to long-term survival in advanced stage patients. Despite large research efforts, the only serum marker currently approved for clinical use is CA 19-9. Utility of CA 19-9 has been shown to improve when it is used in combination with PC-specific markers. Efforts are being made to develop early-screening assays that can detect tumor-derived material, present in circulation, before metastasis takes a significant course. Detection of markers that identify circulating tumor cells and tumor-derived extracellular vesicles(EVs) in biofluid samples offers a promising non-invasive method for this purpose. Circulating tumor cells exhibit varying expression of epithelial and mesenchymal markers depending on the state of tumor differentiation. This offers a possibility for monitoring disease progression using minimally invasive procedures. EVs also offer the benefit of detecting molecular cargo of tumor origin and add the potential to detect circulating vesicle markers from tumors that lack invasive properties. This review integrates recent genetic insights of PC progression with developments in digitalpathology and early detection of tumor-derived circulating material.

Diagnosis and Treatment of a Case of Leydig Cell Tumor in Dogs

Based on such diagnostic measures as clinical diagnosis and lab etiological examination,the disease was diagnosed as Leydig cell tumor in dogs. Combined with the clinical examination results of the dog,testicular tumor removal operation was conducted,and the prognosis was favorable.

卵巢Sertoli-Leydig细胞瘤临床病理特征分析(附5例)

目的:分析卵巢Sertoli-Leydig细胞瘤(Sertoli-Leydig cell tumor, SLCT)临床病理特征。方法:报道5例卵巢sertoli-Leydig细胞瘤,总结其镜下表现,免疫组化特点,并复习文献。结果:5例卵巢Sertoli-Leydig细胞瘤,2例低分化,其余3例为高分化,中分化和网状型。Sertoli细胞,表达α-inhibin、Calretinin、WT-1,Leydig细胞α-inhibin、Claretinin表达比Sertoli细胞强,还可表达Melan-A。结论:卵巢Sertoli-Leydig细胞瘤是一种罕见的卵巢肿瘤,其形态丰富要注意和许多疾病鉴别诊断,病理医生应充分了解其病理特征避免出现误诊和漏诊。

Virilizing Ovarian Leydig Cell Tumor with Multiple Non-Functional Endocrine Neoplasias: A Case Report

Ovarian Leydig cell tumor, a sub-type of ovarian steroid cell tumor, accounts for less than 0.1% of all ovarian tumors. It can affect women of any age group but is most common in postmenopausal women. We here report a case of virilizing ovarian Leydig cell tumor with multiple non-functional endocrine neoplasias (pituitary and adrenal adenomas) in a 48-year-old woman. She first presented with sub-abdominal pain and hirsutism since menopause three years ago. Subsequently, she had slight facial acne, voice deepening, breast atrophy, and a prominent Adam’s apple. Her hormone profile showed an elevated level of testosterone, high free androgen index, low levels of luteinizing hormone and follicle stimulating hormone, and normal levels of random cortisol, androstenedione, 17-hydroxyprogesterone and dehydroepiandrosterone sulfate. A pelvic enhanced magnetic resonance imaging (MRI) scan showed nodules in the right ovary, and a pituitary enhanced MRI revealed a microadenoma. An enhanced computerized tomography scan of the adrenal gland revealed left adrenal nodules, possibly adenomas. After a right cystectomy and right fallopian tube resection, her testosterone level declined to 0.38 nmol/L and the symptoms associated with hyperandrogenism improved. This is a rare case of virilizing ovarian Leydig cell tumor with multiple non-functional endocrine neoplasias. We believe our findings will be helpful in the clinical diagnosis and treatment of hyperandrogenism.

Sertoli-Leydig Cell Tumor from Latifa Hospital, Dubai UAE: Case Report

Sertoli-Leydig cell tumors (SLCTs) are rare tumors. Mass and pain are the presenting feature. The varying histopathology and differentiation of this tumor present difficulties with proper diagnosis and development of optimal treatment regimens. The prognosis depends on tumors grading and staging. Surgery is main stay management option. Chemotherapy and radiation options are still of choice.?We aimed?to?present Sertoli-Leydig cell tumor managed surgical at Latifa Hospital in Dubai, UAE with acceptable outcome and good patient satisfaction.

Retiform Sertoli-Leydig Cell Tumor of the Ovary

Sertoli-Leydig cell tumor of the ovary is a kind of sex cord-stromal tumor, which occurs between teens and twenties with symptoms including abdominal pain and swelling. The incidence rate is infinitely rare comprising less than 0.5% of all ovarian tumor. The average age of “retiform Sertoli-Leydig cell tumor” is 17 years as compared to 25 years for Sertoli-Leydig cell tumors as a group. We have experienced this rare case of retiform Sertoli-Leydig cell tumor in a 25-year-old foreign patient with the complaint of palpable mass on the right lower quadrant and an irregular menstrual period. The patient underwent right salpingo-oophorectomy and tumor stage was FIGO stage 1A. We report with a brief review of literature.

Pregnancy and Childbirth After Sertoli-Leydig Cell Tumor Resection:A Case Study and Literature Review

Objectives:To explore the clinical manifestations and pathological features in the biopsy of ovarian Sertoli-Leydig cell tumor,as well as to improve the clinical understanding of the disease.Methods:A case of pregnancy and childbirth after Sertoli-Leydig cell tumor resection was retrospectively analyzed.The patients’clinical data were collected,including the clinical manifestations,postoperative biopsy results,auxiliary examination results,immunohistochemical results,treatment,and prognosis of the patient.Results:(1)SLCT occurred unilaterally;(2)according to the International Federation of Obstetrics and Gynecology(FIGO),the clinical staging was stage IA;according to the pathological classification of malignant tumors,it was gradeⅡ(moderately differentiated);(3)a healthy female live baby was delivered.Conclusion:Such tumors are rare low-grade malignancies and are even rarer in pregnancy.An increase in preoperative testosterone levels with positive ultrasonography results can be used to assist diagnosis;however,postoperative biopsy pathology remains the“gold standard”for the diagnosis of SLCTs.The definite diagnosis of SLCTs is of great significance for surgical planning and prognostic evaluation.

Classification of gastric neuroendocrine tumors and its clinicopathologic significance

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Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor

To investigate the clinical and computed tomography(CT)features of desmoplastic small round cell tumor(DSRCT),we retrospectively analyzed the clinical presentations,treatment and outcome,as well as CT manifestations of four cases of DSRCT confirmed by surgery and pathology.The CT manifestations of DSRCT were as follows:(1)multiple soft-tissue masses or diffuse peritoneal thickening in the abdomen and pelvis,with the dominant mass usually located in the pelvic cavity;(2)masses without an apparent organbased primary site;(3)mild to moderate homogeneous or heterogeneous enhancement in solid area on enhanced CT;and(4)secondary manifestations,such as ascites,hepatic metastases,lymphadenopathy,hydronephrosis and hydroureter.The prognosis and overall survival rates were generally poor.Commonly used treatment strategies including aggressive tumor resection,polychemotherapy,and radiotherapy,showed various therapeutic effects.CT of DSRCT shows characteristic features that are helpful in diagnosis.Early discovery and complete resection,coupled with postoperative adjuvant chemotherapy,are important for prognosis of DSRCT.Whole abdominopelvic rather than locoregional radiotherapy is more effective for unresectable DSRCT.

Very high alpha-fetoprotein in a young man due to concomitant presentation of hepatocellular carcinoma and Sertoli cell testis tumor

Studies reported that there is a close relationship between hepatocellular carcinoma (HCC) and testis carcinoma. Both tumors can be presented as synchronal tumors, or as testicular metastases of HCC or as hepatic metastases of testicular tumor[7]. HCC is one of the most common malignancies worldwide and the incidence of HCC increases with age[8]. The relationship between hepatitis B incidence and HCC rates is also well recognized. Alpha fetoprotein (AFP) is produced by 70% of HCC. Though a level of AFP ＞400 ng/mL is diagnostic for HCC, in the presence of active hepatitis B infection, the cut-off level should be considered to be at least 1000-4 000 ng/mL. Like HCC, germ cell tumors of the testis also release AFP; but it is shown that some of Sertoli cell tumors of testis can also release AFP[10].Herein we have reported about the first case of HCC in the literature which is presented concomitantly with Sertoli-Leydig tumor of testis, leading to extremely high level of AFP in a 21-year-old man.

Reduction of tumorigenicity of SMMC-7721 hepatoma cells by vascular endothelial growth factor antisense gene therapy

AIM To test the hypothesis to block VEGFexpression of SMMC-7721 hepatoma cells mayinhibit tumor growth using the rat hepatomamodel.METHODS Amplifiy the 200 VEGF cDNAfragment and insert it into human U6 genecassette in the reverse orientation transcribingsmall antisense RNA which could specificallyinteract with VEGF165, and VEGF121 mRNA.Construct the retroviral vector containing thisantisense VEGF U6 cassette and package thereplication-deficient recombinant retrovirus.SMMC-7721 cells were transduced with thesevirus and positive clones were selected withG418. PCR and Southern blot analysis wereperformed to determine if U6 cassette integratedinto the genomic DNA of positive clone.Transfected tumor cells were evaluated for RNAexpression by ribonuclease protection assays.The VEGF protein in the supernatant of parentaltumor cells and genetically modified tumor cellswas determined with ELISA. In vitro and in vivogrowth properties of antisense VEGF cell clonein nude mice were analyzed.RESULTS Restriction enzyme digestion andPCR sequencing verified that the antisense VEGFRNA retroviral vector was successfullyconstructed. After G418 selection, resistantSMMC-7721 cell clone was picked up. PCR andSouthern blot analysis suggested that U6cassette was integrated into the cell genomicDNA. Stable SMMC-7721 cell clone transducedwith U6 antisense RNA cassette could express200bp small antisense VEGF RNA and secretereduced levels of VEGF in culture condition.Production of VEGF by antisense transgeneexpressing cells was 65 ± 10 ng / L per 106 cells,420 ± 45 ng/L per 106 cells in sense group and 485± 30 ng/L per 106 cells in the negative control group, (P＜0.05). The antisense-VEGF cell clone appeared phenotypically indistinguishable from SMMC-7721 cells and SMMC-7721 cells transfected sense VEGF. The growth rate of the antisense-VEGF cell clone was the same as the control cells. When S. C. was implanted into nude mice, growth of antisense-VEGF cell lines was greatly inhibited compared with control cells.CONCLUSION Expression of antisense VEGFRNA in SMMC-7721 cells could decrease thetumorigenicity, and antisense-VEGF genetherapy may be an adjuvant treatment forhepatoma.

卵巢Sertoli-Leydig细胞瘤8例临床病理分析

目的 探讨卵巢Sertoli-Leydig细胞瘤(Sertoli-Leydig cell tumour, SLCT)的临床病理学特征、诊断、鉴别诊断、治疗及预后。方法 收集8例SLCT标本,观察大体及镜下特征,分析临床病理特点,行免疫组化方法检查,并复习相关文献。结果 8例患者平均发病年龄42.8岁,肿瘤均为单侧发生,右侧多见,多为实性或囊实性,可分泌雄激素,中-低分化者多见,病变局限于卵巢。免疫组化α-抑制素(α-inhibin)、广谱细胞角蛋白(CK-pan)、波形蛋白(Vimentin)、钙结合蛋白(Calretinin)、细胞表面糖蛋白99(CD99)、细胞表面金属肽链内切酶(CD10)、翼状螺旋/叉头转录因子2(FOXL2)、雌激素受体(ER)、雄激素受体(AR)均有较高阳性表达,细胞增殖标记(Ki-67)一般呈低表达,上皮细胞膜抗原(EMA)、甲胎蛋白(AFP)、嗜铬素A(CgA)、突触素(SYN)均为阴性,网染可见网状纤维围绕Sertoli细胞巢周围分布,AR在所有肿瘤中呈阳性表达,Sertoli细胞强于Leydig细胞。结论 SLCT发病率低,形态复杂,临床易误诊,确诊有赖于病理诊断和免疫组织化学染色,血清学雄激素的升高及免疫组化雄激素的表达对于诊断该肿瘤有一定指导意义,肿瘤的临床分期是评判肿瘤预后最有意义的指标,治疗采用以手术为主的综合措施。

Facial basal cell carcinoma:A retrospective study of 67 cases

BACKGROUND Basal cell carcinoma(BCC)is a slow-growing malignant tumor characterized by local invasiveness but an exceptionally rare metastatic potential.It ideally affects sun-exposed skin of older patients with more propensity for the facial region.AIM To evaluate the different clinicopathological characteristics of the facial BCC and the efficacy and safety of diode laser for the treatment of these lesions.METHODS We retrospectively reviewed facial BCC lesions of<1.5 cm in diameter and subjected them to diode laser ablation during the period from September 2016 to August 2021 at Al-Ramadi Teaching Hospital,Ramadi City,Iraq.Data matching the age,gender,duration,site,and clinical and histological types were registered for every subject.The functional and aesthetic outcomes and complications following diode laser ablation for each patient were also recorded.RESULTS Of 67 patients with facial BCC,there was 65.67%from the age group≥60 years and 58.21%males.The mean duration of the lesions was 5.15±1.836 mo.The most involved location was the nose(29.85%).About half of the cases belong to the noduloulcerative type.Solid histological type comprises 40.3%of the cases,while the least was keratotic(13.4%).Moreover,65.2%of the solid cases were from the age group≤60 years and 38.6%of the adenoid type from the age group>60 years(P value=0.007).Excellent aesthetic and functional outcomes were reported in all cases after 6 mo of follow-up.Few complications were reported after diode laser ablation.CONCLUSION Facial BCC was mostly seen in the elderly and men.The mean duration was 5.15 mo.The nose was the commonest involved site.Noduloulcerative lesions were seen in approximately half of the lesions.The age of the patients determined the histological type of the lesion(solid type was mostly seen in the age group≤60 years,while,adenoid in the age group>60 years).Diode laser ablation showed excellent functional and aesthetic outcomes following a 6-mo follow-up.

卵巢支持-间质细胞瘤的影像表现及临床特征

目的:总结卵巢支持-间质细胞瘤(SLCT)的影像表现及临床特征,提高对本病的认识。方法:回顾性分析本院经手术及病理证实的15例SLCT患者的临床及影像学资料,其中15例均行MR扫描,5例同时行CT扫描。术前均行卵巢肿瘤标记物检测,10例行内分泌激素水平检测。结果:15例均为单侧单发病变,左侧8例,右侧7例,其中实性4例,囊实性11例。MRI表现:平扫实性成分呈等T1稍长T2信号为主,DWI高信号,ADC图信号减低,囊性成分呈长T1长T2信号;增强扫描4例实性肿瘤明显强化,11例囊实性中5例实性成分呈结节样、不规则分隔样或囊壁样明显强化,另6例呈混合强化,其中1例病理为中分化,余5例为中低及低分化。CT表现:实性部分呈软组织密度,液性部分呈水样密度。3例实性肿瘤增强扫描明显强化,2例囊实性肿瘤中1例实性成分明显强化,1例混合强化。激素水平检测结果9例睾酮升高,肿瘤标记物检测结果3例AFP水平升高,3例CA125升高,1例CA199升高。10例患者表现为雄激素刺激相关表现,2例雌激素刺激相关表现,2例为雌雄激素共同作用表现,1例无症状。病理结果2例为高分化SLCT,7例中分化,5例中低分化,1例低分化。结论:卵巢SLCT具有一定的影像及临床特征,呈实性或囊实性肿块,增强扫描实性成分呈明显强化或混合强化,中低或低分化者多呈混合强化;常伴有激素刺激相关表现,以雄激素刺激相关表现多见。

卵巢支持-间质细胞瘤临床病理学及分子遗传学特征分析

目的 探讨卵巢支持-间质细胞瘤(SLCT)的临床病理学特征及分子遗传学特征。方法 回顾郑州大学第一附属医院收治的13例SLCT患者的临床病理资料,Sanger测序检测DICER1热点区域体细胞突变(第24、25外显子相关位点),完善随访资料,分析DICER1突变与SLCT临床病理特征的相关性,并检索相关文献。结果 SLCT中位数年龄24岁,首发临床症状为月经失调、盆腔包块或下腹胀痛;镜下见管状、条索状及肉瘤样等区域,免疫组化表达CR、WT-1、Inhibin-α、SF-1等。预后随访中30.8%(4/13)患者复发,76.9%(10/13)检测到DICER1基因突变,且DICER1基因突变在年龄上差异有统计学意义(P<0.05),在组织学分化程度、肿瘤是否复发上差异无统计学意义(P>0.05),有异源性分化者DICER1突变率可能高。结论 SLCT形态多样,诊断需要结合临床特点、免疫组化及DICER1基因检测。DICER1突变多发生于年轻患者,与组织学分化、肿瘤是否复发的关系需进一步研究。

卵巢支持-间质细胞肿瘤的临床及影像学特征

目的探讨卵巢支持-间质细胞肿瘤(SLCT)的临床及影像学特征,以提高对本病的诊断准确率。方法选取在本院经手术和病理证实的11例SLCT患者资料,分析其临床和影像学(MRI/CT)特征。结果11例患者中3例表现为男性化特征,2例为去女性化特征,5例为雌激素过多的女性化特征,1例无症状。5例血清睾酮升高。所有肿瘤均为单侧,边界清楚。9例为实性或实性为主肿瘤,1例为囊实性肿瘤,1例为囊性肿瘤。MRI检查显示肿瘤实性部分T_(1)WI呈稍不均匀等或稍低信号,T_(2)WI呈稍高信号,DWI呈高信号,ADC值减低,增强扫描实性部分明显强化呈高信号,部分显示肿瘤边缘丰富血管影。CT平扫显示实性部分呈软组织密度影,增强扫描显示富血供明显强化。结论卵巢SLCT的MRI/CT表现具有一定特征性,结合临床表现和血清睾酮升高的特点,有助于术前诊断和合理治疗。

透明细胞乳头状肾细胞肿瘤临床病理研究

目的:透明细胞乳头状肾细胞肿瘤(clear cell papillary renal cell tumor,CCPRCT)是一种少见但重要的肾肿瘤类型,与其他肾细胞肿瘤具有类似的形态学特征,易导致误诊。本研究旨在探讨CCPRCT的临床病理特征、诊断及鉴别诊断要点,以提高其病理诊断的准确性。方法:收集15例CCPRCT患者,观察其临床及影像学特点,分析其镜下形态、免疫表型,并复习相关文献。结果:15例患者中,男10例,女5例,年龄为(54±17)岁,10例患者肿瘤位于左肾,5例患者肿瘤位于右肾。组织学上肿瘤均由增厚的纤维囊包裹,并局限于肾实质内;肿瘤细胞排列成乳头状、管状、囊状、腺泡及实性等结构;乳头由小到中等大小、透明细胞质的单层细胞组成,肿瘤均为世界卫生组织/国际泌尿病理学会(World Health Organization/International Society of Urological Pathology,WHO/ISUP)1级或2级;细胞核反极性分布,即核上移,靠近腔面,远离基底,类似鲨鱼牙齿排列。免疫组织化学染色显示:细胞角蛋白7、碳酸酐酶9(carbonic anhydrase 9,CA9)和高分子量细胞角蛋白(34βE12)均为阳性表达;α甲基酰基辅酶A消旋酶、CD117、转录因子E3均为阴性表达;CCPRCT中典型的CA9表现方式是“U型”着色,即腔缘不表达,基底和侧面表达。患者术后随访均未见复发或转移。结论:CCPRCT是一种惰性肾细胞肿瘤,预后好,临床可能存在过诊断,其形态学为特征性的核朝向腔缘的线性排列,特殊的免疫表型CA9呈“U型”阳性,可以与其他肾细胞肿瘤区分开来,但仍然需要积累更多患者来阐释其预后。