MOLES,MELANOMAS AND EPITHELIOMAS IN CHILDREN

The management of moles which occur in childhood is important from the standpoint of clinical diagnosis and treatment. Melanomas of both the malignant and juvenile types are sometimes mistaken for ordinary moles. Malignant epithelial tumors are rare in childhood as are congenital tumors. There is a possible relationship between congenital defects of various types and childhood neoplasms.     

Moles,melanomas and epitheliomas in children

The management of moles which occur in childhood is important from the standpoint of clinical diagnosis and treatment. Melanomas of both the malignant and juvenile types are sometimes mistaken for ordinary moles. Malignant epithelial tumors are rare in childhood as are congenital tumors. There is a possible relationship between congenital defects of various types and childhood neoplasms.     

Neuroendocrine tumors of the colon - management guidelines (recommended by The Polish Network of Neuroendocrine Tumors)

The neuroendocrine tumors (NET) of the colon are rare with a rising incidence due to an increased number of diagnostic examination including screening colonoscopy. According to distinct prognosis and treatment these tumors are classified as colonic or rectal NET. This paper provides the consensus guidelines for management of patients with these neoplasms recommended by the Polish Neuroendocrine Tumor Network Group. Furthermore the epidemiology and clinical presentation are described.     

Malignant gastrointestinal stromal tumor presenting with hemoperitoneum in puerperium: report of a case with review of the literature

Background

Leiomysarcoma of intravascular origin is an exceedingly rare entity of malignant soft tissue tumors. They are most frequently encountered in the retroperitoneum arising from the inferior vena cava and are scarcely found to arise from vessels of the extremities. These tumors were analysed with particular reference to treatment outcome and prognosis. The aim of this article is to broaden the knowledge of the clinical course of this rare malignancy.

Method

During 2000 and 2009 twelve patients were identified with an intravascular origin of a leiomyosarcoma. Details regarding the clinical course, follow-up and outcome were assessed with focus on patient survival, tumor relapse and metastases and treatment outcome. 3 year survival probability was calculated using Kaplan-Meier method.

Results

Vascular leiomyosarcomas accounted for 0.7% of all malignant soft tissue tumors treated at our soft tissue sarcoma reference center. The mean follow up period was 38 months. Tumor relapse was encountered in six patients. 6 patients developed metastatic disease. The three year survival was 57%.

Conclusion

Vascular leiomysarcoma is a rare but aggressive tumor entity with a high rate of local recurrence and metastasis.     

Hidrocystomas--a brief review

Hidrocystomas, eccrine and apocrine, are rare cystic lesions that form benign tumors of the sweat glands. In this clinical review, we present a "classic" case of hidrocystoma and review the main epidemiologic, histologic, and clinical features. We also review the effectiveness of experimental treatment methods and present information about associated syndromes and differential diagnosis, focusing especially on hidrocystomas' resemblance to basal cell carcinoma.     

Radiotherapy for granulocytic sarcoma of the breast—Case report and review of the literature

Granulocytic sarcomas are rare tumors that can present in innumerous locations; thus there is very little clinical experience with these cases. Therefore there is no consensus on which is the best treatment for patients with this malignancy.The authors present a case of a female with a granulocytic sarcoma of the breast and review the literature for the role of radiotherapy in the management of this clinical entity.     

儿童胃肠间质瘤治疗进展

胃肠道间质瘤(Gastrointestinal stromal tumors, GIST)是最常见的间叶源性肿瘤。最新统计结果显示,其全球发病率约5~19/106。成人患者临床表现常无特异性,患者多有腹痛、血便、腹胀等症状。GIST主要发生在中老年群体,在儿科极为罕见,因此儿童患者经常被漏诊或误诊。随着近年来基因检测的广泛应用,以及有关儿童GIST研究的深入,发现它代表着一个不同于成人GIST的临床疾病。成人GIST当前主要治疗方式以手术为主,同时根据患者病情应用靶向药物辅助治疗。然而对于儿科患者,一些用于成人肿瘤的治疗方案难以达到理想的治疗效果,甚至会造成不良后果。儿童胃肠道间质瘤的独特生物学特性,决定了它需要独特的治疗方案,以达到最佳的临床疗效。本综述旨在研讨最新文献,提供不同的儿童胃肠道间质瘤的治疗方式。     

放射性药物在肿瘤靶向治疗中的应用

放射性药物指供临床诊断或治疗用的放射性核素制剂或其标记化合物。放射性核素靶向治疗是利用对肿瘤细胞具有特异高亲和力的分子载体将核素定向导入特定的肿瘤组织,对肿瘤进行治疗。与传统的放疗和化疗相比,其具有选择性杀伤肿瘤细胞的特点。随着核医学的发展,SPECT/CT、PET/CT的普及,新靶点的发现和新型放射性药物的研发,利用放射性药物进行靶向治疗在肿瘤临床治疗中占据的地位越来越重要。本文简述了放射性药物的分类、组成及特点;综述了针对肿瘤相关抗原的放射免疫药物在非霍奇金淋巴瘤、结直肠癌和前列腺癌中的应用;受体介导的放射性核素药物在治疗神经内分泌肿瘤、前列腺癌和乳腺癌中的临床应用以及基于基因修饰的放射性药物在肿瘤靶向治疗中的实验研究进展。最后总结了放射性药物在肿瘤靶向治疗中的应用前景与面临的挑战,以期为靶向治疗肿瘤的放射性药物的开发和临床应用提供一些参考。     

一例少见青少年T幼淋巴细胞白血病的病例报道及文献回顾

目的:报道一例少见幼年幼淋巴细胞白血病(T-PLL)患者的病例资料及诊疗过程,并通过文献复习总结了T-PLL的临床特点和诊疗措施。方法:对病例资料进行对比分析,同时通过文献回顾研究T-PLL的特点及发生、发展及诊疗情况。结果:本病例为少见青少年型幼淋巴细胞白血病,细胞以成熟小淋巴细胞为主,特征免疫表型为CD7~+CD5~+CD4~+CD8~+CD3~+,无染色体异常,有TCR基因重排。结论:T-PLL病例具有多态性及复杂性,在临床诊断中需要密切联系临床特点及实验室诊断,综合判断疾病情况,做出最优治疗方案。     

Atypical lipomatous tumour/well-differentiated liposarcoma found in the buccal mucosa: A rare case report

Oral liposarcomas are uncommon diseases, the most predominant histopathological subtype being atypical lipomatous tumour/well-differentiated liposarcoma. In regard to its clinical aspects in the oral cavity, it is challenging to confirm a diagnosis and develop a treatment plan. In this case report, we present a rare case of atypical lipomatous tumour/well-differentiated liposarcoma in the right cheek of a 77-year-old male patient. Conservative surgery was performed considering the histopathological subtype of the neoplasm. Knowledge of the clinical and histopathological characteristics of this rare disease is essential to maintaining function and aesthetics through conservative treatment in older patients.     

Aromatase inhibitors in gynecologic cancers

The female genital tract is hormonally responsive, and consequently some tumors, which arise within in it, may be treated at least in part, with hormonal manipulation. The range of responses in clinical trials and case reports will be reviewed. Many of these diseases are too rare for clinical trial testing, and in some cases evidence is anecdotal at best. Recurrences of ovarian cancer have been treated with tamoxifen and megesterol acetate with variable response rates from 0 to 56%. The favorable toxicity profile of aromatase inhibitors led to trials of these agents for the treatment of relapsed epithelial ovarian cancer. These agents have proved tolerable with minor response rates but a significant disease stabilization rate, which may be prolonged in a minority of cases. It is unclear if these responses may be predicted by estrogen receptor expression or aromatase expression. Anastrazole has also been tried in combination with an EGFR receptor-inhibitor, again showing minor responses but possibly an increase in TTT in some patients. Granulosa cell tumors of the ovary are rare, hormonally sensitive tumors, with reported responses to a variety of hormonal manipulations, including aromatase inhibition. In addition, combined endocrine blockade, including aromatase inhibition, has been tried with reports of success. Endometrial cancers, particularly type I lesions, are often treated with hormonal manipulation, most commonly with progestins, but also with antiestrogens such as tamoxifen. A trial of aromatase inhibition in the treatment of recurrent endometrial cancer showed minimal responses. Endometrial stromal sarcoma, an uncommon uterine malignancy, has shown response to hormonal treatments, with multiple case reports of efficacy of aromatase inhibition. Despite the rarity of some of these tumor types, rare tumor study groups, such as within the Gynecologic Oncology Group, should make an effort to prospectively define the utility of these treatments.     

Current trends in photodynamic therapy of neoplasms and non-neoplastic diseases

The photodynamic therapy of tumors is a modern therapeutic modality for organ-preserving treatment of oncological diseases. The method is based on selective laser irradiation of tumor tissues previously sensitized by tumorotropic dyes. During the last decades, photodynamic therapy has become worldwide known as a proper approach to the treatment of the patients with malignant tumors of various locations and a number of nontumoral diseases. The characteristics of modern photosensitizers and light sources for photodynamic therapy and their clinical applications are reviewed.     

Conjunctival amelanotic melanoma--a case report

Conjunctival melanoma is a relatively rare malignancy. It is presented as pigmented nodule in any area of conjunctiva, amelanotic tumors are pink with smooth appearance. The authors describe an amelanotic melanoma of the conjunctiva in an 82-year-old female patient. Cytological, histopathological and immunohistochemical studies revealed an invasive amelanotic melanoma exhibiting S-100 and MART-1 positivity. The patient undervent surgical and chemotherapy treatment and three years after the initial treatment is in the terminal stage of metastatic disease. Absence of pigmentation delayed early clinical detection and treatment. Awareness of this nonpigmented melanoma is crucial for early recognition and appropriate management.     

原发性肾上腺非霍奇金淋巴瘤（附9 例报告）

目的:探讨原发性肾上腺淋巴瘤(Primary Adrenal Lymphoma,PAL)的临床特点、提高对PAL的认识。方法:回顾分析解放军总医院1995年12月至2007年6月收治的9例PAL的临床表现、实验室检查、影像学特点、组织病理类型以及治疗方法等临床资料,并结合国内外文献进行分析。结果:9例患者中,1例因常规体检发现,8例因腹痛、腹胀或腰痛就诊发现;其中单侧3例,双侧6例,实验室检查无明显异常,影像学检查仅发现肾脏肿瘤,但术后病理组织学诊断为非霍奇金淋巴瘤(non-Hodgkin’s lymphoma,NHL),其中8例弥漫大B细胞淋巴瘤,1例T细胞淋巴瘤;7例患者术后均接受了CHOP或RCHOP方案化疗为主的综合治疗,2例常规治疗;随访至2010年2月,1例弥漫性大B细胞淋巴瘤患者存活4年,1例在术后3年2个月死亡,余7均在2年内死亡。结论:PAL是一种罕见的、恶性程度较高的肿瘤,临床表现和影像学检查缺乏特异性,组织病理学及免疫组织化学是明确诊断的好方法。术前确诊肾上腺原发性非霍奇金淋巴瘤可避免手术,联合化疗应为治疗首选。     

Enhancing immune checkpoint blockade therapy of genitourinary malignancies by co-targeting PMN-MDSCs

Immune checkpoint blockade (ICB) as a powerful immunotherapy has transformed cancer treatment. The application of ICB to genitourinary malignancies has generated substantial clinical benefits for patients with advanced kidney cancer or bladder cancer, yet very limited response to ICB therapy was observed from metastatic castration-resistant prostate cancer. The efficacy of ICB in rare genitourinary tumors (e.g. penile cancer) awaits results from ongoing clinical trials. A potential barrier for ICB is tumor-infiltrating polymorphonuclear myeloid-derived suppressor cells (PMN-MDSCs) with their functions and mechanisms recently revealed. Preclinical studies suggest that successful therapeutic inhibition of PMN-MDSCs synergizes effectively with ICB to eradicate ICB-refractory genitourinary malignancies.     

Relationships between stem cells and cancer stem cells

Stem cells have been shown to exist in a variety of tissues. Recent studies have characterized stem cell gene expression patterns, phenotypes, and potential therapeutic uses. One of the most important properties of stem cells is that of self renewal. This raises the possibility that some of the clinical properties of human tumors may be due to transformed stem cells. Similar signaling pathways may regulate self renewal in normal and transformed stem cells. These rare transformed stem cells may drive the process of tumorigenesis due to their potential for self renewal. There are important ramifications for clinical cancer treatment if the growth of solid tumors is at least partially dependent on a cancer stem cell population. In the cancer stem cell model, tumor recurrence may be due to the non-targeted stem cell compartment repopulating the tumor. If cancer stem cells can be prospectively identified and isolated, it should be possible to identify therapies that will selectively target these cells.     

Neuroectodermal ovarian tumors: a brief overview

Primary neuroectodermal tumors of the ovary are rare monophasic teratomas composed exclusively or almost exclusively of neuroectodemal tissue. Approximately 60 neuroectodermal tumors of the ovary have been reported in the literature. These tumors were classified as ependymoma, astrocytoma, glioblastoma multiforme, ependymoblastoma or as primitive neuroepithelial tumors such as medullo-blastoma, medulloepithelioma and neuroblastoma. Most tumors were diagnosed in the third and fourth decades of life, but occasionally they were first discovered in children, adolescents or older women. Microscopically, they are identical to equivalent neuroectodermal tumors of the central nervous system. The review of the literature shows that most patients with clinical stage I and II were treated surgically, whereas those with stage III or IV tumors received additional radiation or chemotherapy, or both. The clinical stage at the time of diagnosis is the most important prognostic parameter of these tumors.     

Genetic and Clinical Characteristics of Phyllodes Tumors of the Breast

PURPOSE: Phyllodes tumors (PTs) of the breast are rare, accounting for less than 1% of all breast tumors. Among PTs, malignant PTs (MPTs) have malignant characteristics and distant metastases occur in about 20% to 30% of MPTs. However, there is no effective treatment for MPTs with distant metastasis, resulting in an abject prognosis. We performed targeted deep sequencing on PTs to identify the associations between genetic alterations and clinical prognosis. METHODS: We performed targeted deep sequencing to evaluate the genetic characteristics of PTs and analyzed the relationships between clinical and genetic characteristics. RESULTS: A total of 17 PTs were collected between 2001 and 2012. Histologic review was performed by pathologists. The samples included three benign PTs, one borderline PT, and 13 MPTs. The most frequently detected genetic alteration occurred in the TERT promoter region (70.6%), followed by MED12 (64.7%). EGFR amplification and TP53 alteration were detected in four MPTs without genetic alterations in MED12 and TERT promoter regions. Genetic alterations of RARA and ZNF703 were repeatedly found in PTs with local recurrence, and genetic alterations of SETD2, BRCA2, and TSC1 were detected in PTs with distant metastasis. Especially, MPT harboring PTEN and RB1 copy number deletion showed rapid disease progression. CONCLUSIONS: In this study, we provide genetic characterization and potential therapeutic target for this rare, potentially lethal disease. Further large-scale comprehensive genetic study and functional validation are warranted.     

Histochemical and immunohistochemical similarities between hepatic tumors in two chimpanzees and man

A well-differentiated trabecular hepatocellular carcinoma (HCC) and a well-differentiated tumor resembling HCC from each of two chimpanzees were found to have histochemical and immunohistochemical staining characteristics similar to those in human HCCs. Transforming growth factor α was overexpressed in both tumors. Oval cells, thought to be liver stem cell progeny with a possible role in hepatocarcinogenesis, were observed among nontumorous hepatocytes, particularly near the tumors. Hepatic tumors are rare in chimpanzees but their similarities to human HCC provides a useful research model.     

Cytologic diagnosis of small cell anaplastic hepatoblastoma: a case report

BACKGROUND: Small cell anaplastic hepatoblastoma (HB) is the least common subtype of HB. There are few articles in the literature describing the cytologic characteristics of this rare subtype. CASE: A 5-year-old girl present with a progressively enlarging abdominal mass. Ultrasound examination revealed the mass to be arising within the right lobe of the liver. Fine needle aspiration (FNA) of the mass revealed primitive cells with a high nuclear/cytoplasmic ratio and hyperchromatic, ovoid nuclei in poorly cohesive clusters as well as dispersed singly. These cells closely resembled those of small round cell tumors of childhood. CONCLUSION: The cytologic differential diagnosis of undifferentiated HB must include small round cell tumors of childhood. With knowledge of the typical cytomorphologic appearance of HB in association with clinical and radiologic information, one can offer a reliable preoperative diagnosis on FNA.