A case of malignant eccrine spiradenoma metastatic to intramammary lymph node

Eccrine spiradenoma (ES) is a fairly common, benign, cutaneous tumor originating from the sweat glands. In contrast, the malignant counterpart of ES, malignant eccrine spiradenoma (MES), is extremely rare. A long-standing lesion rarely begins to enlarge rapidly. A growth that results in ulceration or discoloration may be associated with malignant transformation. We present the first reported case of this tumor metastasizing to an intramammary lymph node (IMLN). The uncommon metastasizing focus of the periumbilical MES and its histopathological similarity with a primary breast carcinoma made the diagnosis difficult.     

右下肢小汗腺螺旋腺癌1例报道并文献复习

目的:探讨小汗腺螺旋腺癌的诊断和治疗方法.方法:报道1例右下肢小汗腺螺旋腺癌,探讨小汗腺螺旋腺癌的诊治.结果:患者为67岁老年女性,因发现右下肢肿块进行性增大就诊,2015年6月4日手术切除病灶,术后病理确诊为小汗腺螺旋腺癌.患者术后全身PET/CT提示右髂血管旁及右侧腹股沟淋巴结转移,术后尝试“mFOLFOX”方案化疗.但患者分别于2015年8月31日及2015年12月23日因发现肿瘤局部复发而行减瘤手术.术后患者因体质弱,未再继续行抗肿瘤治疗,行最佳对症支持治疗,于2016年7月21日死亡.结论:小汗腺螺旋腺癌临床表现常缺乏特异性,需由组织病理确诊,大范围手术切除是其首选治疗方式,目前仍无公认的化放疗方法,预后极差,生存期短.     

Malignant eccrine spiradenoma of the hand

A malignant eccrine spiradenoma (MES) of the hand is described in a patient who developed bilateral pulmonary metastasis more than 10 years later. A review of the literature including observations on clinical behavior and recommendations for treatment of this rare adnexal tumor is presented.     

Bronchopneumonia disguising lung metastases of a painless central chondrosarcoma of pubis

Chondrosarcoma is a generally locally malignant chondroid-forming bone tumor with a low potential for distant metastases. A small and completely painless central chondrosarcoma of pubis metastasizing to the lungs in a 63-year-old woman with bronchopneumonia is reported. Here we emphasize the mimicry and low growth of the chondrosarcoma and the easiness with which the diagnosis in completely asymptomatic patients can be missed. Although painless chondrosarcoma metastasizing to lung is rather rare, this tumor should be always included in the differential diagnosis of malignancies in this age category.     

Pleomorphic liposarcoma of a young woman following radiotherapy for epithelioid sarcoma

A case of a metachronous epithelioid sarcoma and pleomorphic liposarcoma in a young woman is described. The first tumor was an epithelioid sarcoma (ES) with focal rhabdoid features localised in the left calf while the second lesion developed seven years later in the same region was diagnosed as pleomorphic liposarcoma resembling myxofibrosarcoma ( myxoid variant of malignant fibrous histiocytoma ) predominantly composed of moderately differentiated spindle cells. Multiple foci of uni- and plurivacuolated lipoblasts were seen. Following the resection of ES the patient received 57 Gy radiation to the region, therefore we regarded the second tumor as a radiation induced liposarcoma. A further interesting feature of this case is that the development of pleomorphic liposarcoma preceded by 6 months the solitary right parabronchial metastasis of ES and after 4 months of metastasectomy a third tumor developed at the site of the first lesion. This tumor showed dedifferentiation toward pleomorphic malignant fibrous histiocytoma. Our case represents a unique case of postirradiation liposarcoma developed on the base of ES.     

Metastasis of human tumors in athymic nude mice

The incidence of metastasis of xenogeneic tumors transplanted to nude mice is controversial. We studied 106 malignant human tumor lines in a total of 1,045 nude mice, and observed metastasis in only 14 instances (1.3%), involving 11 different tumor lines. Three of the lines showed repeated metastasis. Breast tumor lines metastasized with significantly greater frequency than other tumor types. None of the sarcoma lines metastasized. Tumors derived from human metastases were no more prone to metastasizing in nude mice than were tumors derived from primary sites. However, deep penetration of the body wall during growth of the tumor transplant was highly correlated with metastasis (p less than 0.001). Such factors as nude mouse health, tumor size and growth rate, and age and sex of the host mouse were not correlated with metastasis. Serial passage in nude mice did not select for a more malignant tumor line, since the incidence of metastasis did not differ at various passage levels. Thus, metastasis of human malignant tumors in nude mice would appear to depend primarily upon the site of tumor growth in the nude mouse, and upon the intrinsic metastasizing capability of the tumor line employed.     

小汗腺螺旋腺瘤1例报告

报告1例小汗腺螺旋腺瘤。患者,女,60岁。因发现右上臂肿块一年余就诊。皮肤科检查：肿块位于皮下,直径0.5cm,质地中等,活动度可。组织病理检查：肿瘤呈分叶状,与周围组织界限清楚。瘤细胞内毛细血管丰富,瘤细胞围绕毛细血管排列呈片状、实性条索状、花瓣样及腺管样,部分区域似玫瑰花样排列。肿瘤主要由两种瘤细胞组成：一种瘤细胞体积稍大,呈卵圆形及立方形,细胞核呈类圆形,淡染;另一种瘤细胞形态似基底细胞样,体积较小,卵圆形,细胞核深染。免疫组化：肿瘤细胞SMA（＋）,CK5/6（＋）,CK（＋）,EMA（＋）。结合临床和组织病理表现,诊断为小汗腺螺旋腺瘤。     

CXCR4 expression is associated with proneural-to-mesenchymal transition in glioblastoma

Glioblastoma (GBM) is the most common primary intracranial malignant tumor and consists of three molecular subtypes: proneural (PN), mesenchymal (MES) and classical (CL). Transition between PN to MES subtypes (PMT) is the glioma analog of the epithelial-mesenchymal transition (EMT) in carcinomas and is associated with resistance to therapy. CXCR4 signaling increases the expression of MES genes in glioma cell lines and promotes EMT in other cancers. RNA sequencing (RNAseq) data of PN GBMs in The Cancer Genome Atlas (TCGA) and secondary high-grade gliomas (HGGs) from an internal cohort were examined for correlation between CXCR4 expression and survival as well as expression of MES markers. Publicly available single-cell RNA sequencing (scRNAseq) data was analyzed for cell type specific CXCR4 expression. These results were validated in a genetic mouse model of PN GBM. Higher CXCR4 expression was associated with significantly reduced survival and increased expression of MES markers in TCGA and internal cohorts. CXCR4 was expressed in immune and tumor cells based on scRNAseq analysis. Higher CXCR4 expression within tumor cells on scRNAseq was associated with increased MES phenotype, suggesting a cell-autonomous effect. In a genetically engineered mouse model, tumors induced with CXCR4 exhibited a mesenchymal phenotype and shortened survival. These results suggest that CXCR4 signaling promotes PMT and shortens survival in GBM and highlights its inhibition as a potential therapeutic strategy.     

Malignant meningioma with extracranial metastasis

We report case of malignant meningioma involving the lumbar vertebra (3rd and 4th). A 53-year-old man who developed occipitalgia in April 1977 was operated under the diagnosis of posterior fossa tumor in August 1977. The tumor was totally removed macroscopically Simpson grade 2. The histological diagnosis was malignant meningioma, He complained of lumbago and right leg pain since June 1981. Destruction and tumor stain of the 3rd and 4th lumbar vertebrae were recognized on CT scans. In May 1982, surgical decompression and spinal fixation were attempted. The surgical specimen was verified as a malignant meningioma. Therefore, he underwent irradiation therapy to the lumbar spine (total dose 50 Gray). He presented with occipitalgia in March 1983. CT scan detected tumor recurrence in the suboccipital region. The recurrent tumor was totally removed immediately. The histological feature was malignant meningioma. The mode and site of metastasizing meningioma are discussed with a review of the literature.     

Epithelioid sarcomas with elevated serum CA125: report of two cases

Epithelioid sarcoma (ES) is a musculoskeletal malignant tumor with epithelioid appearance. It is a relatively rare soft tissue neoplasm, the histological diagnosis of which is difficult and the histogenesis is still obscure. Like many other musculoskeletal tumors, according to the literature, ES has no useful tumor marker to be monitored in the serum. We experienced two ES patients with elevated serum CA125 level. One patient was a 29-year-old woman with ES in her pubic area and the other a 26-year-old man with a tumor in his right calf. Laboratory examination revealed that the CA125 values of both patients were high and changed in accordance with the progress of the tumor growth. An immunohistochemical study demonstrated that the tumor cells in both cases expressed CA125. These results suggest that CA125 can be used as a useful tumor marker for diagnosing and monitoring tumor progress.     

Three cases of primary lung and mediastinal malignant germ cell tumors --clinical and pathological studies

This is a clinical and pathological study of malignant germ tumors in the lung or mediastinum. Germ cell tumors may be considered to originate from primitive germ cells with totipotency. One of 3 cases showed clinical and pathological findings suggesting that a part of the tumor differentiated into many kinds of tissue, such as growing and metastasizing. Initial diagnosis was performed radiographically and pathologically with difficulty. But finally, the first patient was diagnosed as malignant teratoma, the second as yolk sac tumor and the third as choriocarcinoma, in consideration of the tumor markers and clinical process.     

Pancreatic islet cell carcinoid: a highly malignant form of carcinoid tumor

A 23-year-old man died four months after being admitted to the hospital because of back pain, 9.9 kg weight loss in 2 months, 8 to 10 loose stools per day, and periodic sweating. The carcinoid syndrome was present but no hypoglycemia. At autopsy, malignant carcinoid tumor was found in the pancreas. No other tumor was found in the usual primary sites for carcinoids. Biochemical findings and clinical data in retrospect support the morphologic diagnosis of pancreatic islet cell carcinoid tumor. Our case and a review of the literature emphasize that metastasizing tumors of the pancreas associated with excessive production of indolic compounds are frequently aggressive and the survival time is short (range 3 months to 4 years; mean 15 months).     

Clinically tolerable concentrations of arsenic trioxide induce p53-independent cell death and repress NF-kappa B activation in Ewing sarcoma cells

Ewing sarcoma (ES), a highly malignant pediatric tumor, is consistently associated with translocations that fuse the EWS gene with a member of the ETS family gene, most commonly FLI-1. Despite significant advances with multiagent chemotherapy, surgery and radiotherapy, about 40% of ES patients still die from the disease. It is therefore necessary to explore novel agents for possible treatment of this tumor. Here the authors investigated the sensitivity of ES cells to clinically tolerable concentrations of arsenic trioxide (As2O3), a compound known to induce differentiation and apoptosis of other types of malignant cells. The authors report that As2O3 uniformly induced death of 6 ES-derived cell lines irrespective of their p53 status. As2O3 resulted in an apoptotic phenotype which was inhibited by the broad-spectrum caspase inhibitor ZVAD-fmk. These effects correlated with prolonged c-jun N-terminal kinase activation, which is a signal for apoptosis in ES cells. As2O3 also decreased basal and cytokine-induced NF-kappa B activity. Since the authors previously demonstrated that NF-kappa B exerts an antiapoptotic action in ES cells, As2O3 treatment may also result in a sensitization of these cells to other drugs used in combination therapy. These effects, combined with its antiangiogenic action, define As2O3 as a good candidate for future protocols to improve treatments of Ewing sarcomas, irrespective of the p53 status of the tumor.     

Highlighting the immunohistochemical profile of melanocytomas: review

The histological assessment of atypical melanocytic neoplasms is mandatory to ensure proper diagnosis and treatment. However, for some atypical lesions, expert pathologists report only moderate concordance in the diagnosis. In addition, certain atypical neoplasms have been coined differently in the literature. These designations include among others atypical and metastasizing Spitz tumor, malignant Spitz naevus, borderline and intermediate melanocytic tumor, and melanocytic tumor of uncertain malignant potential (MELTUMP) or Spitzoid melanocytic tumor of uncertain malignant potential (STUMP). These neoplasms are grouped here under the heading melanocytoma. Such melanocytic lesions have a benign outcome but exhibit an atypical and worrisome aspect. Rare individual cases of melanocytomas can progress to locoregional disease (agminate melanocytomas), and even beyond. At times, the distinction between melanocytoma and melanoma is difficult and may even be impossible. However, multipronged immunohistochemistry can help define malignancy risk stratification and therapeutic guidelines.     

Prediction of high risk Ewing's sarcoma by gene expression profiling

Ewing's sarcoma (ES) is the second most common primary malignant bone tumor in children and adolescents. Currently accepted clinical prognostic factors fail to classify ES patients' risk to relapse at diagnosis. We aimed to find a new strategy to distinguish between poor and good prognosis ES patients already at diagnosis. We analysed the gene expression profiles of 14 primary tumor specimens and six metastases from ES patients, using oligonucleotide microarray analysis. The over-expression of two genes was validated by quantitative PCR using the LightCycler system. We identified two distinct gene expression signatures distinguishing high-risk ES patients that are likely to progress from low-risk ES patients with a favorable prognosis of long-term progression-free survival. The microarray-based classification was superior to currently used prognostic parameters. Over-expressed genes in the poor prognosis patients included genes regulating the cell cycle and genes associated with invasion and metastasis, while among the downregulated genes were tumor suppressor genes and inducers of apoptosis. Our results indicate the existence of a specific gene expression signature of outcome in ES already at diagnosis, and provide a strategy to select patients who would benefit from risk-adapted improved therapy.     

Primary metastasizing aortic endothelioma

An instance of malignant endothelioma, primary in the aorta, metastasizing to intestine and bone, is reported. The aortic tumor was successfully resected. The unexpected finding of a large hepatic growth at autopsy raised the possibility that the aortic neoplasm was a metastasis. Histochemical, immunologic, and ultrastructural studies supported the diagnosis of an endothelial neoplasm. Although a number of malignant aortic and large vessel tumors have been reported, only four previous instances appear to be endotheliomatous.     

Canine classical seminoma: a specific malignant type with human classifications is highly correlated with tumor angiogenesis

Background  

Human seminoma is classified as classical seminoma (SE) and spermatocytic seminoma (SS). Human SE is known to be more malignant and metastasizing more frequently than SS. Tumor angiogenesis is highly related with tumor progression and metastasis, with microvessel density (MVD) being an important parameter of metastatic potential. Canine seminoma is not yet well-established as SE or SS type including correlation with angiogenesis. We classified canine SE and SS, and then compared them to tumor associated vessels.     

Epithelioid sarcoma in children and adolescents: a report from the Italian Soft Tissue Sarcoma Committee

BACKGROUND: Epithelioid sarcoma (ES) is an uncommon malignant soft tissue tumor. To the authors' knowledge, little information is available to date concerning its clinical features and management in children and adolescents, particularly with regard to the recently described proximal-type variant. The current study concerns 30 patients age<18 years who were enrolled in the Italian Soft Tissue Sarcoma Committee protocols. METHODS: Histopathologic specimens, clinical data, and treatment modalities were reviewed for the current analysis. Nineteen patients had classic ES and 11 had proximal-type ES. Surgery was the mainstay of treatment; radiotherapy was given to six patients considered to be at risk of local disease recurrence due to incomplete resection, and chemotherapy was administered to eight patients. RESULTS: With a median follow-up of 66 months, the 5-year event-free survival (EFS) and overall survival (OS) rates were 61.7% and 92.4%, respectively, but the OS rate dropped to 86.9% and 72.4%, respectively, at 10 years and 15 years. Local disease recurrence was the major cause of treatment failure. The most significant finding influencing both EFS and OS was tumor site, with a tumor location in the extremities predicting a favorable outcome. Initially unfavorable clinical findings and a worse outcome were associated with the proximal-type variant of ES. A response to chemotherapy was noted in three of the seven patients with measurable disease. CONCLUSIONS: The current study confirms some typical features of ES (i.e., the peculiar superficial distal location [i.e., the hand, fingers], indolent growth, and a tendency to recur locally). The current study data do not clearly confirm the strong tendency for the lymph node involvement described in adult ES patients. Further studies are needed to better define the clinical behavior and biology of the proximal-type variant of ES.