Intramedullary epidermoid cyst. A case report

The authors add to the literature a case report of a 32-year-old man with an intramedullary epidermoid cyst at the level of D 3/4, that was successfully operated on. There are several previous reports in the literature, but only five of these include MRI studies.     

超声诊断骶尾部表皮囊肿1例

患者女,40岁,因直肠憋胀感6个月、外院发现盆腔包块4天来我院就诊。经腹超声：膀胱充盈好,子宫及双侧卵巢结构未见明显异常,盆腔未见明显包块和积液。经阴道超声：直肠后方见约7.3 cm×3.5 cm囊实性包块,形态不规则,似＂哑铃＂型,边界清,似有包膜,内部回声偏低,不均匀,     

A large epidermoid cyst developing in the palm: A case report

INTRODUCTIONEpidermoid cysts, also called atheromas, are frequently observed. These cysts originate mostly in the pilosebaceous apparatus but rarely in areas without hair, such as the palm and sole. The mechanism of their development has been reported to be epidermal inclusion secondary to trauma or human papillomavirus (HPV)-60 infection. We report the patient who developed an epidermoid cyst in the palm without any known cause.PRESENTATION OF CASEA 93-year-old male noticed a mass in the left palm 3 years earlier, was diagnosed with an epidermoid cyst based on incisional biopsy, and underwent incision drainage several times during the 3-year period. However, since the cyst spontaneously ruptured and was infected, debridement and resection of the cyst were performed. The postoperative course was favorable, without signs of infection. The pathological diagnosis of the resected specimen was an epidermoid cyst.DISCUSSIONThis case was a rare epidermoid cyst originating in an area without hair, and there was neither a clear history of injury nor findings strongly suggesting HPV-60 infection. Causes other than repeated loading on the palm could not be considered for epidermal inclusion. To our knowledge, this case is the largest reported epidermoid cyst.CONCLUSIONEpidermoid cysts are frequently observed, but their development in areas without hair is rare, and its mechanism has not been clarified. The association between this cyst and trauma or HPV is clear. However, repeated loading on areas such as the palm that tend to undergo chronic loading can also be a cause, as in this patient.     

Epstein-Barr virus-associated primary central nervous system lymphoma in a patient with diffuse cutaneous systemic sclerosis on long-term mycophenolate mofetil

BackgroundEpstein Barr virus (EBV)-associated primary central nervous system lymphoma (ePCNSL) is increasingly recognized in immunocompromised subjects, including patients receiving systemic immunosuppressive therapy. Here, we report the first case of primary CNS lymphoma associated with EBV in a patient with diffuse cutaneous systemic sclerosis (dcSSc) receiving long-term mycophenolate mofetil (MMF).Case reportA 51-year-old female with dcSSc had been on MMF 2 grams daily, which was initiated for a rapidly rising modified Rodnan skin score (mRSS), severe pruritus, and progressive joint contractures. She had an impressive response to this therapy with a significant decrease in her mRSS. Her condition remained stable for the next five years, after which she developed worsening headaches for 2–3 weeks, associated with dizziness, gait instability, and left homonymous hemianopia. MRI scan of the brain revealed a solitary 2.4 cm peripherally enhancing right parietal lobe mass. Excised tissue from the right parietal lobe mass showed EBV-associated diffuse large B cell lymphoma. She received four cycles of chemotherapy (high dose methotrexate and rituximab). Currently, her condition is being monitored. Her left homonymous hemianopia persists.ConclusionBecause of a favorable toxicity profile, MMF is increasingly being used as long-term immunomodulatory therapy for a wide variety of autoimmune disorders. Nevertheless, patients on long-term MMF should still undergo regular CNS surveillance, not only for opportunistic infections but also for opportunistic malignancies such as PCNSL. Progressive focal or non-focal neurological deficits should always raise the alarm. Prompt evaluation and management can prevent irreversible neurological sequelae.     

Isolated primary central nervous system lymphoma arising from the optic chiasm

A 58-year-old previously healthy woman rapidly developed progressive bilateral visual loss. Magnetic resonance imaging revealed a bulging appearance of the optic chiasm, with homogeneous enhancement after gadolinium administration, which suggested an optic glioma or inflammatory disease. In the absence of (para)clinical clues for a specific diagnosis despite extensive investigation, a biopsy of one optic nerve was performed, resulting in a diagnosis of non-Hodgkin B-cell lymphoma. There was no evidence of any other ocular or systemic involvement, therefore the conclusion was that this immunocompetent patient had a primary central nervous system lymphoma isolated in the anterior visual pathway. Treatment included two cycles of polychemotherapy (rituximab, methotrexate, carmustine, etoposide, methylprednisolone), followed by autologous peripheral blood stem cell transplantation and rituximab plus cytarabine consolidation therapy. Subsequently, the patient exhibited significant improvement in vision, and was still disease-free at the 1-year follow-up examination. The aim of the present paper was to provide well-documented clinical, radiological, and intraoperative features of isolated primary malignant lymphoma arising from the anterior visual pathway. A better recognition of this rare pathological entity is necessary for clinicians who may encounter similar presentations, as prompt management is crucial for both a visual and vital prognosis.     

A large epidermoid cyst of breast mimicking carcinoma: A case report and review of literature

INTRODUCTIONTriple assessment of a suspicious breast lesion may not always provide a definite diagnosis. We report a case of epidermoid cyst of breast, which caused diagnostic dilemma in spite of a thorough triple assessment and entailed mastectomy.PRESENTATION OF CASEA 69-year-old woman presented with a large painful retroareolar left breast mass. Clinical examination, ultrasound and mammography were highly suspicious of malignancy. However, core biopsy suggested a benign lesion. Due to size of the lesion and diagnostic uncertainty, various options were discussed with the patient. She opted for a simple mastectomy. The histology confirmed a large epidermoid cyst.DISCUSSIONIt is rare for an epidermoid cyst to present as such an advanced lesion, mimicking carcinoma. Excision of such a large retroareolar ‘benign’ lesion, however, may sometime entail mastectomy. This is the first reported case of an epidermoid cyst of breast necessitating mastectomy.CONCLUSIONDiagnostic dilemma while dealing with a suspected breast cancer is not rare. Involvement of multidisciplinary team as well as patient is important in the decision-making. The report illustrates a rare presentation of a deep seated large epidermoid cyst of breast, which mimicked carcinoma, caused diagnostic confusion and entailed mastectomy. We strongly advocate the option of breast reconstruction in such cases.     

指骨表皮样囊肿一例

患者男 ,38岁 ,左拇指外伤 2 0年 ,局部肿痛 2 0天入院。查体 :左拇指指甲弯曲畸形 ,末节手指尺侧缘肿胀 ,轻压痛 ,似有波动感。Ｘ线片显示 :左拇末节指骨远端尺侧缘可见半圆形骨质缺损区 ,边缘光滑硬化 ,无骨膜反应。ＣＴ检查 :左拇末节指骨内缘被软组织肿块挤压呈弧形凹陷 ,软组织影呈抱球状 ,0 6ｃｍ× 0 6ｃｍ× 1 2ｃｍ大小 ,ＣＴ值 4 8Ｈｕ ,受压骨边缘光滑整齐有硬化缘。化验 :碱性磷酸酶、血沉、血常规均正常。初步诊断 :左拇指末节指骨表皮样囊肿。术中所见 :拇指末节手指尺侧缘皮下有一0 5ｃｍ× 0 5ｃｍ× 1 5ｃｍ包块 ,质软…     

Intraparenchymal schwannomas of the central nervous system: An additional case report and review

Intraparenchymal location of schwannomas in the central nervous system (CNS) is rare. Occasional cases involving the cerebrum, cerebellum, brain stem, and spinal cord have been reported. We report here an additional case of thoracic intramedullary schwannoma in a 42 year old woman. The literature concerning intraparenchymal schwannomas in the CNS is reviewed and discussed.     

Primary malignant lymphoma of the central nervous system

The occurrence of primary malignant lymphomas of the central nervous system in a population of approximately 1 million people has been analyzed retrospectively. Over a 12-year period, about 1000 tumors of the central nervous system were registered. Among these, 22 were primary malignant lymphomas; incidence, 1.83 per million per year. Thirteen of these tumors were of high-grade malignancy and nine were of low-grade malignancy (Kiel classification of non-Hodgkin lymphomas). The majority of the tumors were B-cell lymphomas, and there were no Hodgkin lymphomas. Neither spinal nor meningeal lymphomas occurred. Nine patients were treated only surgically, whereas 11 received postoperative irradiation or chemotherapy, or both. Of the latter patients, six are alive and well at the time of writing. The importance of making a correct diagnosis preoperatively is stressed, inasmuch as radical operation is unfavorable for these patients. A better prognosis is obtained with combinations of irradiation and chemotherapy.     

原发性中枢神经系统淋巴瘤MRI表现

目的探讨原发性中枢神经系统淋巴瘤(PCNSL)的MRI表现。方法回顾性分析29例接受MR平扫+增强检查,且经手术病理证实,具有完整临床资料的PCNSL病变的MRI表现,观察病灶的部位、大小、形态、边缘轮廓、瘤周水肿、占位效应、MRI平扫信号特点,重点观察增强后病灶的典型及非典型强化特点。结果 29例中单发者16例,多发者12例(30个病灶),共46个病灶,弥漫浸润型1例。肿瘤好发于胼胝体、基底节区等深部脑组织(35/46,76.09%)。瘤周水肿多为中度(15/46,32.61%)或重度(27/46,58.70%),未见出血及钙化。肿瘤T1WI多呈等低信号(30/46,65.22%)或等信号(14/46,30.43%),T2WI多呈等信号(14/46,30.43%)或等高信号(29/46,63.04%),DWI多为高信号(42/46,91.30%)。增强扫描表现出多种强化方式:典型强化方式包括均匀一致团块状或结节状强化、"裂隙征"、"蝶翼征"、"皮层下刻痕征"、以及"卫星灶征";不典型强化方式包括环形强化、"开环样"强化、4脑室匍匐贴壁生长病灶,沿软脑膜弥漫分布粟粒结节样病灶。结论 PCNSL的MRI表现具有一定的特征性,对PCNSL的诊断及鉴别诊断具有重要的价值,但确诊仍有赖于病理。     

Retrorectal epidermoid cyst in an elderly woman: Report of a case

An epidermoid cyst is a common occurrence in the skin. Such a cyst occurring in the retrorectal space, however, is extremely rare. We herein present a case of retrorectal epidermal cyst in a 67-year-old Japanese woman. She reported no symptoms and a perirectal mass behind the lower rectum was accidentally detected by computed tomography (CT). Contrast CT and magnetic resonance imaging revealed a cystic mass (76 x 70 x 63 mm) with a slightly enhanced thin wall which was attached to the lower rectum. The patient underwent surgery via a posterior approach. The mass was exposed after an excision of subcutaneous fat and was firmly adhered to the anal sphincter muscle. The cystic mass was completely removed without a proctectomy. Histologically, the cyst was diagnosed to be an epidermoid cyst. The postoperative course was uneventful, and the patient was discharged on postoperative day 9. A diagnosis of retrorectal cystic tumor is difficult, and a complete resection is necessary for the treatment of such tumors.     

Epidermoid cyst in an intrapancreatic accessory spleen: a case report

Ectopic splenic tissue in the abdominal cavity is a common entity, with a reported incidence of 10% in the general population. However, an intrapancreatic accessory spleen is a rare disease, and moreover cyst formation in it is exceedingly rare. A 58-year-old woman with a 25-mm multilocular cyst in the tail of the pancreas detected incidentally by ultrasonography was admitted for further evaluation. Because malignancy could not be ruled out, a spleen-preserving distal pancreatectomy was performed. The cut surface of the surgical specimen showed a multilocular cyst surrounded by brown solid tissue resembling normal spleen. Pathological examination revealed it was stratified squamous epithelium and was surrounded by splenic tissue. The final pathological diagnosis was epidermoid cyst in an accessory spleen in the pancreas. This cyst has no characteristic features on diagnostic imaging. Consequently, it is not possible to make a definite preoperative diagnosis in most cases. Epidermoid cyst in intrapancreatic splenic tissue is another lesion to be considered in the differential diagnosis of pancreatic tail tumors.     

原发性中枢神经系统淋巴瘤的诊断和治疗

目的探讨原发性中枢神经系统淋巴瘤（PCNSL）的临床特点,提高对PCNSL的认识。方法回顾性分析43例PCNSL患者的临床表现、实验室检查、影像学特点、组织病理类型以及治疗方法。结果单发病灶36例,多发病灶7例,共手术治疗47次。首发症状为颅内压增高22例（51．2％）,肢体偏瘫、抽搐、语言障碍16例（37．2％）,记忆力下降、意识情感障碍10例（23．3％）。脑脊液蛋白含量高于正常者66．7％（8／12）。B细胞型淋巴瘤40例（97．6％）,T细胞型1例（2．4％）。Ki-67阳性表达指数42．8％±23．3％。结论CT、MRI及脑脊液检查是术前诊断PCNSL的重要方法,外周血淋巴细胞增高不能作为诊断PCNSL的依据。手术在于缓解症状和确定病变的病理类型,术后化疗加全脑放疗是治疗PCNSL的有效方法。     

Secondary central nervous system involvement by follicular lymphoma: case report and review of the literature

BACKGROUND: We report a patient with indolent stage IV follicular lymphoma, grade 1, initially successfully treated with chemotherapy, who later developed aggressive diffuse large B-cell lymphoma in the parieto-occipital lobe 8 years after initial presentation. The differing patterns of lymphomatous involvement of the central nervous system (CNS) are briefly reviewed, with a focus on the patterns seen in secondary CNS spread by low-grade lymphomas. CASE DESCRIPTION: A 53-year-old man was diagnosed with stage IV follicular lymphoma, grade 1, in 1996. Although initial chemotherapy was successful, he developed several recurrences of lymphoma over the following years. In May 2004, he presented with a discrete, single, massive parieto-occipital lobe brain lesion. The mass failed to regress with empiric cranial external beam radiotherapy. Because of suspicion of an unusual infection, the lesion was surgically excised in its entirety. The mass proved to be an aggressive diffuse large B-cell lymphoma, transformed from his previous follicular cell lymphoma, with retention of strong Bcl-2 and Bcl-6 immunoreactivity. CONCLUSIONS: Parenchymal brain involvement, as opposed to dural or leptomeningeal, is a relatively uncommon pattern of spread to the CNS for systemic lymphomas. More significantly, follicular lymphomas are one of the least frequent types of indolent lymphomas to develop clinically apparent, secondary CNS spread. The presentation of an indolent follicular lymphoma with transformation to an aggressive diffuse large B-cell lymphoma within the brain parenchyma is rare. Its manifestation as a massive, singular lesion is unique and prompted diagnostic confusion.     

原发性中枢神经系统淋巴瘤Toll样受体4表达水平与弥散加权成像表观弥散系数值的相关性

目的 观察原发性中枢神经系统淋巴瘤(PCNSL)Toll样受体4(TLR4)表达水平与MR弥散加权成像(DWI)表观弥散系数(ADC)值的相关性。方法 回顾性分析17例经病理确诊的PCNSL患者,观察病灶ADC值、TLR4及NF-κB表达水平之间的相关性。结果 PCNSL病灶ADC值为460×10^－6~1 034×10^－6 mm²/s,平均(756±147)×10^－6 mm²/s;TLR4表达水平为0.02~0.11,平均0.06±0.03,NF-κB表达水平为0.04~0.15,平均0.08±0.03。TLR4表达水平与ADC值呈负相关(r=－0.76,P<0.01);NF-κB表达水平与TLR4及ADC值之间无明显相关性(P均>0.05)。结论 PCNSL的TLR4表达水平与其ADC值呈负相关。     

Spontaneous regression of primary central nervous system lymphoma: a case report

A rare case of primary central nervous system lymphoma that regressed spontaneously as shown on serial cranial magnetic resonance imagings (MRI) is described. A 60-year-old woman was admitted to our hospital with a well-enhanced mass lesion in the cerebellum although MRI had demonstrated no abnormal findings 4 months before admission. On admission, She complained of headache, but no neurological deficits were observed. The patient underwent exploratory craniotomy and the tumor was partially removed on February 14. Pathologic examination suspected B-cell type of malignant lymphoma. Postoperative MRI performed on February 23, 2001 showed disappearance of the mass lesion without further treatment. Different polymerase chain reaction (PCR) assays detected clonal immunoglobulin heavy chain gene rearrangements in paraffin-embedded tissues diagnosed as B-cell lymphoma. The patient was discharged without any neurological deficits for two weeks and was followed up on MRI. One year after operation, MRI revealed an abnormal finding in the cerebellum without apparent neurological deterioration. Radiation therapy of the whole brain and the local site was carried out with a total dose of 50Gy. MRI demonstrated complete remission of the tumor following radiation therapy. She was admitted again to our hospital with right hemiparesis on May, 2003. Subsequently, systemic chemotherapy was carried out. The patient died 2 years and 10 months after her initial presentation.     

Methotrexate-associated primary hepatic malignant lymphoma following hepatectomy: A case report

IntroductionRecently, immunosuppressant-associated malignant lymphoma (ML) cases have been increasing along with the development of several effective immunosuppressant drugs for rheumatoid arthritis (RA). Among methotrexate (MTX)-associated lymphoproliferative disorders, primary hepatic lymphoma (PHL) in patients with RA following surgical resection has not been reported previously.Presentation of caseA 65-year-old woman who is a hepatitis B virus carrier with a history of RA was admitted. MTX was introduced seven years prior as an RA treatment. Her laboratory data showed no elevation of several tumor markers, and liver function test results were normal. On contrasted computed tomography (CT) scanning, a slightly enhanced tumor was detected at the early phase, and tumor staining was sustained at the delayed phase. Further, subsegmentectomy of the S6 was performed. The pathological diagnosis was diffuse large B-cell lymphoma. However, positron emission tomography-CT and bone marrow aspiration sample showed no resident sign of ML.DiscussionDiagnosis of PHL before surgery is difficult. If the mass lesion was solitary and had a certain degree of size, then resection could be performed for its treatment and diagnosis. The treatment for ML requires a diagnosis of the subtypes to select a therapeutic agent and determine the prognosis. Once a precise preoperative diagnosis was made, withdrawing MTX could be the first treatment in case of MTX-related ML.ConclusionLong-term usage of immunosuppressant drugs could cause proliferative ML. Considering the increasing occurrence of MTX-related ML, withdrawing MTX should be considered, especially in patients with long-term immunosuppressant usage for RA.     

Glandular odontogenic cyst: A case report and clinicopathologic analysis of the relationship to central mucoepidermoid carcinoma

Glandular odontogenic cyst (GOC), an unusual multilocular cystic lesion of jaws exhibiting the features of both botryoid odontogenic cyst and mucoepidermoid tumor has been reported recently as a new entity with uncertain histogenesis. The World Health Organization named GOC as an independent pathologic entity and classified it as a developmental odontogenic epithelial cyst. We report an additional case, describe its clinicopathologic features, and discuss the similarity to the central mucoepidermoid carcinoma. © 1997 John Wiley & Sons, Inc. Head Neck 19: 227–231, 1997.     

基于SEER数据库的原发性中枢神经系统淋巴瘤不同治疗方法的疗效分析

目的探讨原发性中枢神经系统淋巴瘤(PCNSL)患者的预后因素及不同治疗方法的临床效果。方法回顾性分析1975年1月至2016年12月美国SEER数据库中4812例PCNSL患者的临床资料。其中男性2831例,女性1981例,男女比例为1.4∶1.0;发病年龄<60岁者2236例(46.47%),60~<75岁者1718例(35.70%),≥75岁者858例(17.83%);幕上肿瘤2417例(50.23%),幕下肿瘤299例(6.21%),脑内多发肿瘤554例(11.51%),其他或未指明部位的脑部肿瘤1542例(32.04%);弥漫大B细胞淋巴瘤(DLBCL)3513例(73.00%),非DLBCL 234例(4.86%),其他或未指明类型的淋巴瘤1065例(22.13%);治疗方式为单独活检的2010例(41.77%),单独部分切除61例(1.27%),单独完全切除54例(1.12%),活检+化疗2384例(49.54%),部分切除+化疗159例(3.30%),完全切除+化疗144例(2.99%)。采用单因素及多因素Cox回归模型分析影响患者总体生存的预后因素;采用Fine-Gray检验与竞争风险模型研究影响患者肿瘤特异性生存的预后因素;生存分析采用Kaplan-Meier法,并通过Log-rank进行比较。结果单因素及多因素Cox风险回归模型分析结果显示,影响PCNSL患者总体生存的独立预后因素包括年龄、种族、婚姻状态、肿瘤部位、病理学亚型、手术、化疗、合并其他恶性肿瘤、合并HIV感染等。Fine-Gray检验与竞争风险模型分析结果显示,影响患者肿瘤特异生存的独立预后因素包括年龄、种族、婚姻状态、肿瘤部位、病理学亚型、手术方式、化疗、合并其他恶性肿瘤、合并HIV感染,而性别与放疗均与肿瘤特异生存无明显相关性。与活检相比,PCNSL患者可能从手术切除中获益(部分切除:HR=0.805,95%CI:0.656~0.989,P=0.04;完全切除:HR=0.521,95%CI:0.414~0.656,P<0.01)。Kaplan-Meier生存分析结果显示,活检+化疗组中位生存时间为28个月(95%CI:24.497~31.503),单独活检组为2个月(95%CI:1.756~2.244),单独部分切除组为2个月(95%CI:1.410~2.590),单独完全切除组为19个月(95%CI:0~39.311),部分切除+化疗组为67个月(95%CI:46.187~87.813),完全切除+化疗组为84个月(95%CI:57.448~110.552),使用不同治疗方法的患者的中位生存时间的差异有统计学意义(P<0.01)。结论手术切除可能改善部分PCNSL患者的预后。化疗可能使肿瘤完全切除或部分切除患者的肿瘤特异生存时间延长。     

原发性中枢神经系统淋巴瘤常规及功能磁共振成像特点

目的探讨原发性中枢神经系统淋巴瘤(PCNSL)常规MRI及fMRI特点。方法回顾性分析21例经病理证实的PCNSL的常规MRI及DWI、1 H-MRS、PWI表现。结果 21例患者共39个PCNSL病灶的ADC值为(0.78±0.14)×10-3 mm2/s,对侧半球正常脑白质ADC值为(0.89±0.21)×10-3 mm2/s,二者差异有统计学意义(t=1.24,P0.05)。相对于正常脑白质,PCNSL病灶1 H-MRS波谱表现为Cho/Cr值增高(4.62±2.05vs 0.83±0.25)、Cho/NAA值增高(6.86±2.36vs 0.63±0.34)、NAA/Cr值减低(0.67±1.73vs 0.63±.034),差异均有统计学意义(P均0.05)。PCNSL病灶PWI表现为相对低灌注,脑血容量低于周围正常脑实质。结论采用MR DWI、1 H-MRS、PWI可从不同方面反映PCNSL的特征,结合常规MRI征象可进一步提高诊断准确率。