Breast cancer with an unusual leukemia-like presentation: case report and literature review

Breast cancer is the most common cancer in women in the world and the second-most common cancer in women in Taiwan. However, breast cancer with plentiful leukemia blast-like cells circulating in the peripheral blood is very rare. A 48-year-old woman with breast cancer and leukemia-like manifestations was admitted in August, 2006. Special staining and immunophenotyping studies to determine the origin of her peripheral blast cells confirmed that they were from her breast carcinoma. Carcinocythemia (carcinoma cell leukemia) was diagnosed and then systemic chemotherapy and hormone therapy were given. Nevertheless, she expired 9 months after carcinocythemia presentation. The differential diagnosis of this condition, which is complicated by chemotherapy or radiotherapy for solid tumors, is very important. Patients with carcinocythemia have poor prognosis and survival.     

Medulloblastoma with melanotic differentiation: case report and review of the literature

Medulloblastoma with melanotic differentiation, a rare variant of medulloblastoma, often carries a poor prognosis. We present such a case of a 4 year male with this rare, aggressive tumor. Additionally, we have reviewed the literature and report on the features important in the pathologic and radiologic diagnosis in this type of tumor, as well as review clinical outcomes. This subtype of medulloblastoma occurs more frequently in males, at a younger median age than the other subtypes of medulloblastoma. The prognosis is generally very poor. However, it is important to note, that a subset of patients with M0 disease who can achieve a gross total resection followed by radiation and platinum based chemotherapy can become long term survivors of this aggressive subtype of medulloblastoma.     

Subcutaneous granuloma of the cheek an unusual case report and review of literature

With the increase in prevalence of extra pulmonary tuberculosis, it has become imperative for clinicians to review their knowledge of unusual presentations of mycobacterial infections. Tuberculous involvement of the subcutaneous tissue and skeletal muscle is rare. Apart from tuberculous lymphadenitis, diagnosis of extrapulmonary tuberculosis may be difficult. We present here a case of tuberculous granuloma of the cheek — an unusual presentation in ENT practice     

Acute lymphoblastic leukemia with chromosomal 5;14 translocation and hypereosinophilia: case report and literature review

A 19-year-old man with acute lymphoblastic leukemia (ALL) presented with 82,000 WBC/microL, 57% eosinophils, and cardiorespiratory symptoms. Lymphoblast infiltration of the meninges and testes developed without eosinophil infiltration at these sites and peripheral blood and marrow lymphoblast counts progressively increased, while blood eosinophilia disappeared. The patient's bone marrow cells had a clonal cytogenetic abnormality--t(5;14), (q?,q32)--which disappeared during remission and reappeared during disease relapse. Including this case, three patients with ALL and hypereosinophilia have had cytogenetic studies with G-banding. All three had 14q + chromosomal abnormalities and two had a similar translocation t(5,14), (q?,q32). Survival of the 26 ALL patients with hypereosinophilia reported since 1973 was similar to that of 52 age- and sex-matched historical-control patients without hypereosinophilia treated during the same time interval.     

Gliosarcoma with primitive neuroectodermal differentiation: case report and review of the literature

The clinical and pathological features of a gliosarcoma with a primitive neuroectodermal component in a 52-year-old male are presented. To our knowledge, only three other cases of such an entity have been reported in the English literature.     

Low-grade spindle-cell ameloblastic carcinoma: report of an unusual case with immunohistochemical findings and review of the literature

Spindle-cell differentiation in ameloblastic carcinoma is a rare event. Although reported by many authors, it was first described as a separate entity in 1999 by Slater under the heading "low-grade spindle-cell ameloblastic carcinoma." Here, we report a case of low-grade spindle-cell ameloblastic carcinoma arising in pre-existing unicystic ameloblastoma.The patient, a 60-year-old Indian woman, had a large irregular swelling in the left mandibular region. Histologically, the lesion was composed of a large cystic cavity with an ameloblastomatous lining and areas showing spindle-cell proliferation. The spindle cells showed hyperchromatism, nuclear pleomorphism, and scattered mitotic figures. To our knowledge, 6 cases of spindle-cell ameloblastic carcinoma have been published to date, and this case appears to be the first reporting malignant transformation with spindle-cell differentiation in unicystic ameloblastoma.     

腹膜后纤维瘤病1例报告并文献复习

目的:探讨腹膜后纤维瘤病临床特点。方法:报道1例腹膜后纤维瘤病侵袭输尿管致严重血尿的诊治,并复习有关文献。结果:术中发现腹膜后右侧输尿管末端有一直径6cm实性肿瘤,固定于盆壁,与髂外动静脉致密粘连,包裹右侧输尿管末端,上段输尿管扩张。行肿瘤大部切除术+输尿管再植术,切除肿瘤大部分为肌瘤样组织,中间坏死形成囊腔,与输尿管相通;术中快速冰冻片报告梭形细胞肿瘤,良恶性待定;术后病理报告为腹膜后纤维瘤病,免疫组化:CD34（-）、CD117（-）、Dog-1（-）、SMAS-100（-）、Vimentin（+）、Desmin（-）、NSE（-）。结论:腹膜后纤维瘤病是一种十分罕见的软组织肿瘤,可以因发生位置不同而以不同临床症状就诊,提高对这一肿瘤的认识可以减少误诊。     

急性全髓增殖症伴骨髓纤维化一例并文献复习

 【摘要】目的：认识“急性全骨髓增殖性骨髓纤维化”这一新的疾病。方法：对1例急性全骨髓增殖性骨髓纤维化病例进行诊断回顾并复习文献。结果：患者,男性,52岁,临床表现为全血细胞减少,外周血和骨髓少量幼稚细胞,骨髓干抽,骨髓纤维化,脾脏不肿大,临床过程呈现进展状态,最后表现为急性粒细胞性白血病,患者在一年内死亡。结论：急性全骨髓增殖性骨髓纤维化是一个独立的疾病,需注意与急性骨髓纤维化、急性白血病伴骨髓纤维化、急性髓细胞性白血病伴多系发育异常等疾病鉴别。     

Glioblastoma multiforme with an abscess: case report and literature review

An intratumoral or peritumoral microbial intracranial abscess is an infrequent diagnosis. The development of this complication may not be preceded by apparent local or general infection in all cases. To identify this diagnosis by radiological (MRI) or laboratory investigations is very intricate. Nevertheless, the recommended life-saving strategy is early surgery with resolution of both the tumor and infection. If subsequent oncological treatment is required, it has to be adjusted for prevention of re-inflammation. The described patient suffered from an intracranial abscess superimposed on a Glioblastoma Multiforme. The confirmed etiological agent was Staphylococcus aureus. The suspected route of microbial migration and colonization in this tumor was bacteremia via agents from thrombophlebitis. The patient is in a good condition following surgery, antimicrobial treatment, and radiotherapy.     

Recurrent intracranial hemangiopericytoma with extracranial and unusual multiple metastases: case report and review of the literature

Hemangiopericytoma is a rare tumor with uncommon location in the central nervous system. It has only recently been included (WHO classification 1993) in a specific group of CNS tumors and subsequently (WHO classification 1997 and 2000) as a group by itself, while before it was confused with meningeal tumors. We report on a case of a 48-year-old woman affected by this tumor. The neoplasm was located in the posterior fossa. The patient underwent primary surgery in 1990, not followed by any adjuvant therapy because of the histopathological diagnosis of meningioma. After being free from disease for eight years she developed a local recurrence in 1998. Subtotal excision of the tumor, which was finally identified as a hemangiopericytoma, was carried out, followed by adjuvant radiotherapy (64 Gy). After six months multiple metastases were found in the liver and right kidney. A radical metastasectomy was performed, followed by systemic chemotherapy. One year later (2001) the tumor recurred again intracranially and a metastases was detected in the right breast, so the patient again underwent cranial irradiation (40 Gy) and second-line chemotherapy. She died in September 2002, 12 years after the diagnosis. We may conclude that, despite the tumor's natural tendency to recur several times and the ability of intracranial hemangiopericytoma to spread outside the CNS, it is possible to ensure a long survival time.     

伴高白细胞综合征的CD5阴性白血病样套细胞淋巴瘤一例并文献复习

目的 提高对不典型表型特征套细胞淋巴瘤(MCL)临床特征和治疗转归的认识.方法 对江苏省人民医院血液科收治的1例高龄伴高白细胞综合征白血病样MCL患者进行细胞形态学、免疫学、细胞遗传学分析,明确诊断后根据患者临床特点制定个体化治疗方案并随访病情变化.结果 该例患者骨髓及外周血细胞形态学分析均提示幼稚淋巴细胞比例异常升高(骨髓0.452,外周血0.580),流式细胞术(FCM)提示外周血淋巴细胞占93％,其中CD5+CD19+<20％,CD23+CD19+<20％,间期荧光原位杂交(FISH):IGH/CCND1融合基因提示t(11;14)易位.确诊CD5-MCL,采用小剂量CP方案(环磷酰胺、糖皮质激素)预处理降低肿瘤负荷后对该病行来那度胺维持治疗,控制良好.结论CD5-MCL一般表现类似于惰性MCL,出现白血病样表现和复杂核型改变时预后差,临床表现更凶险.此例老年患者伴基础疾病,不适宜采用常规一线方案治疗,如何选择最佳治疗方案需要进一步临床探讨.     

Blastic natural killer (NK)-cell lymphoma: report of an unusual CD4 negative case and review of the CD4 negative neoplasms with blastic features in the literature

Blastic Natural Killer (NK)-cell lymphoma is a relatively new entity which has been recently included in the WHO classification. CD4 expression is observed in most cases of blastic NK-cell lymphomas and has been related with skin tropism. We report an unusual CD4 negative blastic NK-cell lymphoma with primary presentation in the skin, subsequent infiltration of the bone marrow and aggressive behavior. It is emphasized that extensive immunophenotyping and EBER RNA in situ hybridization are required in order to establish the diagnosis of blastic NK-cell lymphoma. We also present a review of the literature with respect to the CD4 negative NK-cell lymphomas with blastic morphological features.     

甲状腺伴胸腺样分化的梭形细胞肿瘤临床病理观察

目的:探讨甲状腺伴胸腺样分化的梭形细胞肿瘤的临床病理学特征.方法:报道1例极少见的甲状腺伴胸腺样分化的梭形细胞肿瘤,并结合文献对其临床特征、组织形态、免疫组化特点及鉴别诊断等进行分析.结果:大体见4.5cm×3.5cm×2cm大小的灰白色结节,与周围界限较清.镜下见肿瘤主要为梭形细胞成分与腺样成分互相融合的双相分化表现.免疫表型Keratin、EMA和Vim阳性.结论:甲状腺伴胸腺样分化的梭形细胞肿瘤是一种非常少见的肿瘤,临床表现为无痛性肿块,无功能改变,确诊需结合组织形态及免疫组化.鉴别诊断包括甲状腺未分化癌、滑膜肉瘤、间质高度增生呈结节状筋膜炎样型甲状腺乳头状癌等.手术切除预后较好.     

Primary bone lymphoma: Report of an unusual case with a review of the literature

Primary bone lymphoma is uncommon and usually involves the long bones. We report a patient with involvement of a metacarpal bone, and review the literature.     

胸腺类癌1例及文献复习

目的:探讨胸腺类癌的病理学特点及临床表现.方法:对1例胸腺类癌进行组织学表现、电镜和免疫组化染色观察及文献复习.结果:组织学特点:瘤细胞多排列为不规则巢状,条索状,伴有大量坏死,瘤细胞大小较一致,胞质弱嗜酸,颗粒状,边界不清,胞核呈圆形或椭圆.免疫组化特点:肿瘤细胞表达Syn(+)、CgA(+)、NSE(+).电镜:部分瘤细胞及突起内见有神经内分泌颗粒.结论:胸腺类癌是前纵隔一种少见的恶性肿瘤,主要与胸腺瘤相鉴别,治疗以手术切除的疗效为最好.     

腹膜后具有血管周上皮样细胞分化的肿瘤－例报道及文献复习(英文)

The retroperitoneal neoplasm with perivascular epithelioid cell differentiation (PEComa) is an extremely rare pathological entity. In this article, we reported one case of a 45-year-old woman who was admitted to our hospital (The Second People’s Hospital of Hefei, China) for retroperitoneal neoplasm with perivascular epithelioid cell differentiation. The B ultrasonic examination showed echopoor in the region of cavitas pelvis. The histologic characteristics and immunohistochemical phenotype both revealed the neoplasm with perivascular epithelioid cell differentiation.