Myxopapillary ependymoma: cytomorphologic characteristics and differential diagnosis

Myxopapillary ependymoma (ME) is a rare tumor with a predilection for sacrococcygeal area of adults. We present the cytomorphology of one such case in a 21-yr-old man, diagnosed by fine-needle aspiration (FNA). The tumor disclosed biphasic morphology with nests and aggregates of epithelioid malignant cells as well as branching cords of myxohyaline material. Most noticeable was the presence of distinct hyaline globules surrounded by neoplastic cells. The differential diagnosis of ME includes chordoma, adenoid cystic carcinoma, mucinous adenocarcinoma, and germ cell tumor. An accurate preoperative diagnosis of ME on FNA can be achieved based on its distinctive cytomorphologic features.     

Fine-needle aspiration diagnosis of metastatic anaplastic sacrococcygeal ependymoma to the lungs

Ectopic ependymomas are uncommon neoplasms, and most of them occur in the sacrococcygeal area. They usually present as subcutaneous sacral masses. The most common histological subtype is the myxopapillary. We describe a case of anaplastic sacrococcygeal ependymoma metastatic to the lungs diagnosed by fine-needle aspiration biopsy. Diagn Cytopathol 1996;15:228–230. © 1996 Wiley-Liss, Inc.     

Cytologic differential diagnosis of myxoid and mucinous neoplasms of the sacrum and parasacral soft tissues

A diagnostically important group of lesions involving the sacrum, spinal canal, and parasacral soft tissues is characterized by a myxoid or mucinous background in cytologic smears. This group of myxoid/mucoid neoplasms includes chordoma, myxopapillary ependymoma, metastatic mucinous adenocarcinoma, and extraskeletal myxoid chondrosarcoma. Despite the similarity of the background substance, each neoplasm within this differential diagnosis has a characteristic composite set of morphologic and immunophenotypic features. Because many of these masses are not easily surgically biopsied, fine-needle aspiration (FNA) is often used for their diagnosis. The private consultation records of the author and the cytology files of the University of Utah Department of Pathology were searched for all lesions arising in or around the sacrum. These cases were reviewed to determine which had a myxoid/mucinous background. Fourteen neoplasms were found and comprise the study set. Four of these cases had cell block material in addition to Diff-Quik-stained smears; a panel of antibodies, including cytokeratin, glial fibrillary acid protein (GFAP), S-100 protein, and carcinoembryonic antigen (CEA), was performed on the cell block material. The smears were evaluated for cytologic features, including the presence of rosette-like structures, physaliphorous cells, gland-like structures, chondroid fragments, "signet ring" and "goblet" cells, as well as the character of the myxoid/mucinous background substance.The cases included one myxopapillary ependymoma, 10 chordomas, two mucinous adenocarcinomas of colonic or gastric origin, and one myxoid chondrosarcoma. Physaliphorous cells were found to be highly specific for chordoma, while a fibrillary myxoid stroma containing cells with elongated cytoplasmic processes and cells lying in a rosette-like pattern around central cores of myxoid to fibrillary stroma were highly characteristic of myxopapillary ependymoma. Fragments of a myxoid/chondroid matrix with lacunar-like spaces strongly supported the diagnosis of myxoid chondrosarcoma. "goblet" or "signet ring" cells with a single distinct vacuole favored mucinous adenocarcinoma. There appear to be sufficient cytomorphologic features present within the FNA smears and cell block material to allow cytologic separation of the more common myxoid and mucinous neoplasms involving the sacral and parasacral tissues.     

Tanycytic ependymoma in a 76-year-old Puerto Rican male

Ependymoma is a slowly growing tumor in children and young adults originating from the wall of the ventricles or from the spinal canal that is composed of neoplastic ependymal cells. Tanycytic ependymoma is a rare variant of ependymoma usually arising in the intra medullary spine. The World Health Organization classifies the tanycytic ependymoma as a grade II tumor. The diagnosis of tanycytic ependymoma is challenging since the morphology of the lesions resemble those found in schwannoma and astrocytomas. In the present study, we show a case of a 76 years old male with a progressive paraparesis for 8 years, due to a spinal tumor. Radiological and histological studies were used to classify the tumor as tanycytic ependymoma. Therefore, it is important to be aware of tanycytic ependymoma and its immunohistochemistry profile in older patients, especially within the Caribbean Hispanic population. To our knowledge this is the oldest patient known to have this rare tumor and the first case reported in Puerto Rico.     

Squash cytology of tanycytic ependymoma: A report of two cases

Tanycytic ependymoma is a rare variant of ependymoma which has a predilection for the spinal cord. It is a WHO grade II tumour with favourable outcome. Although squash cytology of ependymoma is well described, there is sparse literature available on squash cytomorphology of tanycytic variant. Here we present two cases of squash cytology of tanycytic ependymoma. In the first case the diagnosis of tanycytic ependymoma was considered. However, in the second case a diagnosis of usual ependymoma was offered. Subsequently histopathology confirmed tanycytic nature in both the cases. In this article we discuss the squash cytological features of tanycytic ependymoma along with its differential diagnosis. Diagn. Cytopathol. 2017;45:270–273. © 2016 Wiley Periodicals, Inc.     

Multiple chromosomal monosomies are characteristic of giant cell ependymoma

Giant cell ependymoma, a rare ependymoma subtype, was recently recognized as a separate diagnostic entity with variations both in malignant potential and course of disease. We analyzed the first supratentorial giant cell ependymoma using G-band karyotyping, DNA ploidy analysis, and array comparative genomic hybridization. The tumor was hypodiploid, and the karyotype showed multiple monosomies. This novel cytogenetic pattern seems specific for giant cell ependymoma as the only previous cytogenetic analysis of a giant cell ependymoma found similar monosomies. We were also able to analyze cytogenetically the subsequent recurrent tumor, phenotypically an anaplastic ependymoma, allowing a first insight into the genetic events involved in disease progression.     

Cytogenetic t(11;17)(q13;q21) in a pediatric ependymoma. Is 11q13 a recurring breakpoint in ependymomas?

Cytogenetic studies on a supratentorial ependymoma from a 1-year-old boy showed a t(11;17)(q13;q21). This is the second ependymoma reported with a rearrangement at 11q13; to our knowledge the 11q13 is the first recurring breakpoint reported in ependymoma.     

伸展细胞型室管膜瘤的临床病理特点

目的：观察一少见的特殊类型室管膜瘤的临床病理特点,拟译名为伸展细胞型室管膜瘤。方法：复习3例此型的临床病理和免疫组化资料及有关文献。结果：3例肿瘤均位于脊髓内。它们的临床表现和放射影像学与室管膜瘤其他类型表现相同,病理形态学上,瘤细胞呈现双极的长梭形胶质细胞,形成不典型的血管周菊形团,GFAP、EMA弱阳性,而vimentin强阳性,而可与星形细胞瘤及神经鞘瘤相鉴别,其预后与其他类型室管膜瘤相类似。结论：由于肿瘤由双极、长梭形细胞组成。及tanycyte来源于tanyos有stretch伸出展开之意,“tanycytic ependymona ”以译成“伸展细胞型室管膜瘤”为妥。     

Giant cell ependymoma-report of three cases and review of the literature

Ependymomas constitute the most common type of primary spinal cord tumors, and are subclassified as myxopapillary ependymoma, classic ependymoma, and anaplastic ependymoma. Ependymomas can be further subclassified based on morphologic phenotype: cellular, papillary, tanycytic, clear cell, pigmented and epithelioid. Giant cell ependymoma (GCE), a rare variant, has recently been described. Reported cases have exhibited a wide anatomic distribution, including spinal cord, cerebrum and cerebellum. We report here three cases of GCE, arising from cerebrum in a 5-year-old girl, spinal cord in a 34-year-old female and cerebellum in an 86-year-old female respectively. Histologically those cases showed prominent pleomorphic giant cells with focal perivascular pseudorosettes in all cases. Tumor cells were immunopositive for GFAP and EMA. Only the first case was qualified for anaplastic ependymoma. No recurrence was noted in these three cases after 57, 46 and 6 months of follow-up respectively. By reviewing the literature, GCEs arising from spinal cord and cerebellum tended to have low-grade morphology while supratentorially located GCEs tended to have anaplastic features. GCEs were preferentially located in extraventricular regions. Anaplastic GCEs in adult population seemed to pursue a more aggressive behavior. Gross total resection should still be the main treatment for GCEs.     

Histological alterations following fine-needle aspiration for parathyroid adenoma: Incidence and diagnostic problems

This study aimed to clarify the histological alterations following fine-needle aspiration for parathyroid adenoma and discuss the occurrence of diagnostic problems. Among the 392 patients with parathyroid adenoma who underwent resection, fine-needle aspiration was performed for 21 (5.1%) parathyroid adenoma nodules. Histological findings that were significantly more frequent in cases that underwent fine-needle aspiration were considered histological alterations following fine-needle aspiration for parathyroid adenoma, including the following six findings: thick fibrous capsule (71.4%), multilayered fibrous capsules (14.3%), capsular pseudo-invasion (42.9%), fibrous bands (57.1%), hemosiderin deposition (14.3%), and tumor implantation (14.3%). Eighteen parathyroid adenoma nodules (85.7%) exhibited one or more of the six findings. Tumor cells and adipocytes entrapped within the thick fibrous capsule were occasionally observed. The fibrous bands were frequently connected to the thick fibrous capsule. The number of passes, duration between fine-needle aspiration and resection, tumor size, and purpose of fine-needle aspiration were not related to the incidence of histological findings. Because of the histological alterations following fine-needle aspiration for parathyroid adenoma that can be easily mistaken for signs of atypical adenoma or parathyroid carcinoma, we recommend that the six findings be excluded from pathological findings indicating atypical adenoma or parathyroid carcinoma in patients with preoperative fine-needle aspiration.     

17-year-old girl with headache and complex partial seizure

Supratentorial cortical ependymoma is a rare clinical entity where ependymoma occurring in the cortex without any connection to the ventricular system since ependymoma usually arises from the lining of the ventricular system or central canal of spinal cord. There have been 14 such cases reported in the literature.We report the first case of a supratentorial extraaxial cortical anaplastic ependymoma with minimal cortical attachment in a 17-years-old girl, presented with headache and complex partial seizure. Histological features of anaplasia including numerous mitoses, necrosis and high Ki-67 lead to postoperative adjuvant radiation therapy although gross total resection was achieved.     

A 40‐Year‐Old Male with An Intraventricular Tumor

Combined tumors showing histologic features of both ependymoma and subependymoma have been described. In this report we present a case of combined tanycytic ependymoma with foci of subependymoma (WHO grade II), occurring in a 40 year‐old male, which arose in the wall of the lateral ventricle. The tanycytic ependymoma component showed elongated fibrillary cells with a fascicular pattern of growth, while the subependymoma component showed clustered cell bodies surrounded by a fibrillary stroma with a microcystic appearance. We consider the present case to be an unusual example of tanycytic ependymoma; which to the best of our knowledge has not been associated with a subependymoma.     

Diagnosis of gastrointestinal stromal tumor by fine-needle aspiration biopsy: a cytological and immunocytochemical study

The diagnosis of gastrointestinal stromal tumors (GIST) is generally established on histopathologic examination of surgical specimens. The present study was undertaken to characterize the cytologic appearance of these tumors and to assess the utility of fine-needle aspirates, including immunocytochemistry for the demonstration of c-kit (CD117) and CD34, in the preoperative diagnosis. The study comprised preoperative fine-needle aspirates in three cases and aspirates performed on fresh surgical specimens in eight cases. The routinely stained smears revealed a mesenchymal cell population, predominated by spindle cells. In five of six cases a strong positivity for c-kit was demonstrated and in three of five cases a strong CD34-expression was found in the tumor cells. These results show that fine-needle aspiration appears to be a useful method for the recognition and diagnosis of GISTs or ICC (interstitial cell of Cajal) tumors.     

Diagnosis of tuberculosis of bone and soft tissue by fine-needle aspiration biopsy.

Fine-needle aspiration biopsy is a well-established procedure in the detection of various neoplastic processes. However, there are only limited reports on the efficacy of this technique in nonneoplastic conditions. In this study, fine-needle aspiration cytology findings of bone and soft tissue lesions in 11 patients with tuberculosis are reported. Spine, scapula, chest wall, flank areas, tibia, ring, and index fingers were the sites of fine-needle aspiration biopsies. The age of the patients ranged from 21 to 65 years. Granulomatous reaction with or without caseation necrosis was seen in 73%. The aspirated material was acellular or predominantly composed of necrotic material and inflammatory infiltrates in 27%. Acid-fast bacilli (AFB) could be demonstrated in 64%. Culture for Mycobacterium tuberculosis was positive in 83%. This study supports previous suggestions that fine-needle aspiration biopsy is a simple alternative to open biopsy for the diagnosis of TB of bone and soft tissue lesions.     

Ependymoma with Signet-Ring Cells

Two patients presented with unusual primary brain tumors having abundant signet-ring cells, for which electron microscopic study was necessary to establish the diagnosis of ependymoma. The light and electron microscopic features of these tumors are discussed, with brief comments on the differential diagnosis.     

伸长细胞型室管膜瘤2例报道及文献复习

目的探讨伸长细胞型室管膜瘤的病理形态学特征。方法复习2例伸长细胞型室管膜瘤的临床病理资料，对手术切除的肿瘤组织，常规石蜡切片，HE染色，光镜观察，同时进行免疫组织化学标记GFAP、EMA、S-100蛋白、CKpan、Syn、CD34。结果例1位于腰2～3椎管内终丝，临床上主要表现出脊髓压迫症状，如运动及感觉障碍；例2发生于侧脑室，主要表现为头痛、呕吐、视物模糊。病理组织学上肿瘤富于细胞，排列成束状、交织状，细胞胞突细长双极，核异型性小，少数肿瘤细胞围绕血管周围形成室管膜瘤的无核区结构。免疫组化显示肿瘤细胞表达GFAP、EMA、CD99，而S-100蛋白阴性。结论伸长细胞型室管膜瘤是室管膜瘤的一种罕见亚型，病理诊断时尤其应注意与椎管内的神经鞘瘤和星形细胞瘤鉴别。     

Detection of telomerase expression in fine-needle aspirations and fluids

The cytologic examination of fine-needle aspirates and fluid specimens is plagued by a persistent false negative rate. The rate of false negative results will be decreased if sensitive molecular assays can be developed to detect cytologically malignant cells. The current study investigated telomerase expression as a potential marker of malignancy, using the telomeric repeat amplification protocol (TRAP) in fine-needle aspirates and fluid specimens. TRAP was performed on 24 fine-needle aspirate and 24 fluid specimens from different body sites and of different histological diagnoses. We found that 6 of 12 fine-needle aspirate specimens that were cytologically positive for malignant cells expressed telomerase activity, while no specimens that were cytologically suspicious for malignancy, atypical, or negative tested positive for telomerase activity. Of the fluid specimens, 4 of 6 cytologically positive cases and 1 of 18 cytologically negative cases expressed telomerase. Seven of eight telomerase negative, cytologically positive specimens contained only rare malignant cells in a very bloody background. Peripheral blood contamination is a possible pitfall in the TRAP assay, as applied in the current study, because the assay is standardized to protein concentration that may be derived from lysed red blood cells. We conclude that with further technical refinement, the TRAP assay could become a useful adjunct in the cytologic examination of fine-needle aspirates and fluid samples. Diagn. Cytopathol. 1998;18:431–436. © 1998 Wiley-Liss, Inc.     

Cytologic criteria for the diagnosis of pancreatic carcinoma

Fine-needle aspiration and endoscopic aspiration of pancreatic cells permit the diagnosis of pancreatic carcinoma and avoid the complications and morbidity of pancreatic biopsy. In this study, the accuracy of fine-needle and endoscopic aspiration were compared, and cytologic criteria for pancreatic carcinoma were sought. Pancreatic cytologic preparations from 79 patients, including 39 fine-needle aspirates and 48 endoscopic aspirates, were retrospective reviewed. When compared with definitive tissue diagnosis or clinical course, fine-needle aspiration had a sensitivity for pancreatic carcinoma of 79%. Endoscopic aspiration of pancreatic secretions had a sensitivity of only 33%. There was a single falsely suspicious fine-needle aspirate, but there were no false positive diagnoses when using either collection technic. Seventeen cytologic features were examined to determine cytologic criteria of malignancy. The presence of disoriented or crowded cells in three-dimensional groups, and extreme nuclear enlargement combined with nuclear contour irregularity were the best criteria for pancreatic malignancy.     

Ki67 index in intracranial ependymoma: a promising histopathological candidate biomarker

Aims:  The Ki67 tumour cell proliferation index is an independent prognostic factor in ependymoma patients. Essential prerequisites for validation of the Ki67 index as a histopathological biomarker are the reproducibility of this factor and its prognostic influence by different observers (proof of objective clinical and analytical performance). To this end, the aim was to analyse systematically inter- and intraobserver agreement and reproducibility of the prognostic impact of the Ki67 index in intracranial ependymoma.
Methods and results:  The study cohort contained 78 cases of intracranial ependymoma. In all cases, the Ki67 index was assessed by four experienced observers (EOs) and by four inexperienced observers (IOs) using the manual hot-spot method. There was considerable agreement on Ki67 index assessment. There was higher observer agreement among EOs compared with IOs. For each observer, survival analysis showed significant association of low Ki67 index with favourable patient outcome.
Conclusions:  Our data show that the Ki67 index in intracranial ependymoma is a reproducible and robust prognostic factor and can be considered a promising histopathological candidate biomarker. Attainment of biomarker status requires further translational studies in the context of prospective therapeutic trials.     

MRI征象及ADC值鉴别成人颅内髓母细胞瘤与室管膜瘤的价值

目的:探索磁共振成像（MRI）征象及表观扩散系数（ADC）值在鉴别成人颅内髓母细胞瘤与室管膜瘤中的价值。方法:回顾性分析手术及病理证实的16例成人颅内髓母细胞瘤及24例室管膜瘤临床、MRI及病理资料,对比MRI征象及ADC值差异,使用受试者工作特征（ROC）曲线评估ADC值在两组肿瘤鉴别诊断中的效能。结果:成人髓母细胞瘤与室管膜瘤在年龄、肿瘤位置和强化程度比较差异有统计学意义（P<0.05）,两组患者性别、形态、肿瘤边界、囊变、瘤周水肿、肿瘤最大径、水肿最大径等比较差异无统计学意义（P>0.05）;成人室管膜瘤ADCmean、ADCmin及rADC值均大于成人髓母细胞瘤,差异有统计学意义（P<0.05）。ADCmean阈值为665.00 mm2/s时,鉴别诊断成人髓母细胞瘤与室管膜瘤的性能最佳,AUC值为0.966,敏感度和特异度分别为0.958、0.937。结论:通过MRI征象联合ADC值对术前鉴别成人髓母细胞瘤与室管膜瘤具有重要的临床价值,有助于辅助临床术前制定个性化诊疗方案。