Giant cell arteritis of the breast: case report and literature review

The clinical and pathologic features of a case of giant cell arteritis presenting in the breast and those of seven previously reported similar cases are described. A unique finding in the present case was a coincidental in situ and infiltrating ductal carcinoma in the same biopsy specimen. All the patients were postmenopausal women who presented with a firm mass in one or both breasts that mimicked a carcinoma on physical examination. There was no definite clinical evidence of temporal artery involvement in any patient, and two patients had normal temporal arteries on histologic examination. Five patients, however, had systemic manifestations, arteritis in another site, or both. Giant cell arteritis presenting in the breast may occur as an isolated finding or represent a manifestation of more widespread disease.     

Primary angiitis of the central nervous system

Despite the multiple areas of progress that have been made in our understanding of primary angiitis of the central nervous system (PACNS ), many challenges exist when facing this diagnosis. This review will address the clinical presentation, diagnosis and differential diagnosis of PACNS, with emphasis on the recognized advancement in the field. Appreciation of different mimics especially the recognition of reversible cerebral vasoconstriction syndromes (RCVS) as a major mimic will be addressed. Finally, approach to treatment will be summarized.     

Primary central nervous system histiocytic sarcoma with relapse to mediastinum: a case report and review of the literature

Histiocytic sarcoma is a rare, malignant neoplasm of the lymphohematopoietic system that usually occurs in the skin, lymph node, and intestinal tract. Here we describe a unique case of primary central nervous system histiocytic sarcoma that initially showed an indolent clinical course following local resection and radiotherapy. However, relapse of disease within the mediastinum was noted 3 1/2 years later. Biopsies of the initial brain lesion and subsequent mediastinal recurrence each revealed an identical, diffuse proliferation of histiocytes with expression of CD45, CD68, and CD163 but not pan-cytokeratin, epithelial membrane antigen, CD3, CD15, CD20, CD30, CD43, CD79a, CD138, myeloperoxidase, ALK-1, PAX-5, CAM 5.2, S100, CD1a, or glial fibrillary acidic protein. In the literature, central nervous system histiocytic sarcoma portends a poor prognosis with median survival of 4.5 months. To our knowledge, this case represents the first case of "low-grade" primary central nervous system histiocytic sarcoma with relatively indolent clinical course. A thorough discussion of the differential diagnosis of histiocytic sarcoma and a review of primary central nervous system histiocytic sarcoma are also presented.     

Giant blastoconidia of Candida albicans. A case report and review of the literature

We describe a patient with extranodal non-Hodgkin lymphoma who developed systemic candidiasis after treatment with a cyclophosphamide-based chemotherapy regimen. Histologically, the fungal organisms demonstrated markedly enlarged blastoconidia with a variety of morphologic forms, mimicking other mycotic organisms, such as Cryptococcus neoformans, Blastomyces dermatitidis, and Paracoccidioides brasiliensis. The in vivo occurrence of such giant forms is rare, and when observed histologically may result in an erroneous diagnosis or a diagnosis of multiple mycotic organisms.     

Primary renal diffuse large B-cell lymphoma with central nervous system involvement: a rare case report and literature review

Primary renal lymphoma is a rare entity. Of these, diffuse large B-cell lymphoma is the most common pathological type and, R-CHOP regimen was the preferred chemotherapy for it. Here we present an adult case of primary renal diffuse large B-cell lymphoma.     

Giant adrenal myelolipoma: case report and review of the literature

Adrenal myelolipomas are rare tumors that consist of mature fat and bone-marrow elements. The majority that have been reported are small, asymptomatic lesions incidentally observed at the time of autopsy. In recent years, larger, symptomatic myelolipomas have been successfully resected. We studied the case of a giant adrenal myelolipoma in a 70-year-old woman. She was obese, hypertensive, and had abdominal pain, findings frequently associated with these lesions. Unusual features included formation of prominent bony spicules, a 52-year history of an abdominal mass, and massive size (5,500 g). To our knowledge, it is the largest myelolipoma yet reported.     

Giant adrenal myelolipoma: case report and review of the literature

Myelolipoma is a tumor-like growth composed of mature fat tissue and bone marrow elements. It occurs in the adrenal gland or as an isolated soft tissue mass. It may be associated with endocrine disorders such as hermaphroditism, Cushing's disease, Addison's disease and obesity of unknown cause. These lesions rarely measure more than 5 cm in diameter, although giant tumors have been reported in the literature. The fifth largest surgically resected adrenal myelolipoma in the literature is reported and its clinical associations and, macroscopic and microscopic features are discussed.     

Giant cell fibroblastoma. A case report

A case of giant cell fibroblastoma occurring in the knee of a 16-month-old girl is reported. The ill-defined subcutaneous tumor measuring 2 x 2 cm was composed of diffusely proliferating spindle-shaped tumor cells with scattered, atypical multinucleated giant cells in a myxoid or collagenous background and irregularly branching sinusoid-like tissue spaces. In addition to floret-type giant cells, a few osteoclast-like giant cells were present in a cellular area where tumor cells were focally arranged in a storiform pattern. Immunohistochemically, the tumor cells gave positive reactions for only vimentin and actin. In spite of the high recurrence rate of this type of tumor, the course of the patient after excision of the tumor has been uneventful. It is important to distinguish this rare, peculiar fibrous tumor from other soft tissue tumors including some sarcomas.     

Desmoplastic small round cell tumor of the central nervous system: report of two cases and review of the literature

Desmoplastic small round cell tumor (DSRCT) is a malignant tumor often involving the abdominal and/or pelvic peritoneum. Only one fully documented example has arisen in the central nervous system (CNS). Herein, we describe two additional examples, fulfilling the morphologic, immunohistochemical, and molecular criteria (EWS/WT1 translocation) of DSRCT. Both arose in the cerebellopontine angle (CPA) and underwent spinal dissemination. Patient 1, a 37-year-old male, underwent a subtotal resection, and 2 years later died of recurrent disease with spinal dissemination. Patient 2, a 39-year-old man, presented with cerebellar and CPA lesions as well as spinal leptomeningeal deposits. After 27 months of adjuvant therapy, he is alive with progressive disease. In conclusion, CNS DSRCT follows a similar aggressive course as do peritoneal examples. Although rare, DSRCT warrants consideration in the differential diagnosis of “malignant small blue cell tumors” of the CNS. For consideration of publication in Virchows Archiv.     

Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review

Inflammatory pseudotumors (IPs), mostly benign lesions characterized by fibrotic ground tissue and polyclonal mononuclear infiltrate, may affect all organ systems. IPs originating in the central nervous system (IP-CNS) are very rare, and their distinct histopathologic features are poorly characterized. Three otherwise healthy patients (age 8, 15, and 17 years) presented with focal neurologic symptoms (seizures, n = 2; headaches, n = 1), corresponding to a left temporal, left occipital, and left frontal IP, respectively, extending from meningeal structures into brain tissue. After resection, no recurrence was observed in patient 1 during 5 years of follow-up, whereas patient 2 developed a rapidly progressive local recurrence and a second intracerebral lesion despite antiviral, immunosuppressive, antibiotic, and radiation therapy. In patient 3, who also showed local recurrences, sequential histopathologic investigations revealed transformation to a semimalignant fibrohistiocytic tumor. In this patient, anaplastic lymphoma kinase (ALK) expression was also positive, whereas it was negative in patient 1. A detailed literature analysis confirmed that most IP-CNS arise from dural/meningeal structures (n = 34). Intraparenchymatous (n = 7), mixed intraparenchymatous/meningeal (n = 4), and intraventricular lesions (n = 7) or IP extending per continuitatem from intracerebral to extracerebral sites (n = 5) were rare. The recurrence rate was 40% within 2 years in general. It was increased after incomplete resection and in female patients (multivariate Cox regression model, P < 0.02). Although rare, IP-CNS are important differential diagnoses among tumor-like intracranial lesions. Their potential risk of malignant transformation and high risk of recurrence necessitate close follow-up, especially when resection is incomplete. Prospective multicenter trials are needed to optimize classification and treatment of this rare inflammatory lesion.     

婴儿中枢神经系统白色念珠菌病5例并文献复习

目的总结婴儿中枢神经系统白色念珠菌病的临床特点和诊断治疗经验。方法收集首都医科大学附属北京儿童医院2009至2011年收治的婴儿中枢神经系统白色念珠菌病连续病例（5例）的临床资料,总结其临床表现、实验室检查、影像学特征、治疗和随访情况,并文献复习。结果男4例,女1例,年龄3～5月龄（平均4月龄）,均无明显免疫缺陷证据及基础疾病。发病至诊断时间为2d至2个月。1例以脑梗死（局限性抽搐、面瘫）起病,4例以反复发热起病,精神反应相对较好,感染中毒症状不严重,无明显颅内压增高症状;4例合并抽搐发作。5例患儿均经CSF培养发现白色念珠菌生长。1例头颅MRI检查未见异常;4例病程中头颅MRI检查先后出现颅内血管受累,包括脑梗死和静脉窦栓塞,其中1例后期出现梗死部位脑软化,1例CT示基底节梗死。CSF常规改变均为WBC总数正常或轻度升高,分类以多核为主,CSF生化示糖明显降低,蛋白显著升高。1例应用氟康唑治疗2.5个月痊愈,随访1年无复发;2例先后应用氟康唑、两性霉素B和氟胞嘧啶联合治疗2周至1个月,临床好转出院,出院后均随访1年,其中1例出院口服伏立康唑2个月痊愈,另1例间断口服氟康唑2个月,病情好转,遗留智力和语言发育障碍;2例放弃治疗并失访。结论婴儿中枢神经系统白色念珠菌病临床表现不典型,感染中毒症状常不严重,脑脊液改变与化脓性脑膜炎类似,糖降低明显,较易出现颅内血管病变,应早期进行病原学检查明确诊断和指导治疗。     

Primary (granulomatous) angiitis of the central nervous system: a clinicopathologic analysis of 15 new cases and a review of the literature.

The clinical and pathologic features of 15 new cases of the uncommon primary or granulomatous angiitis of the central nervous system (PACNS) are described. To date, only 108 such cases have been reported in the English literature. Clinically, most PACNS patients have been young or middle-aged (mean age, 45 years; range, 3 to 96 years), with men outnumbering women slightly by a ratio of 4 to 3. The most frequent presenting complaints are headache, weakness, and confusion; less common complaints are aphasia, dysphasia, nausea or vomiting, loss of memory, and seizure disorder. There is usually no evidence of a systemic disease; the erythrocyte sedimentation rate is almost invariably normal, and there are no diagnostic laboratory tests. The cerebral angiogram usually shows multifocal, segmental stenosis or irregularity of small and medium-sized leptomeningeal and intracranial blood vessels, often with a beading or aneurysmal appearance, and alterations in blood flow in the affected regions. Anatomically, the angiitis is focal and segmental in distribution. An isolated negative biopsy, therefore, does not rule out the disease. Histologically, PACNS may be granulomatous, necrotizing, or lymphocytic in character, and mixed morphologic types often occur. Large- and small-vessel thrombosis is common. Acute lesions frequently coexist with healing or healed lesions. Involvement of extracranial blood vessels occurs only rarely. Past or current herpes zoster infection and Hodgkin's lymphoma are the most noteworthy clinical associations of PACNS, but whether they are causally related remains uncertain.     

原发性中枢神经系统淋巴瘤2例临床病理分析并文献复习

目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的临床特点、病理诊断、治疗及预后。方法对2例PCNSL进行免疫组化染色并复习相关国内外文献。结果 2例PCNSL,1例为间变性大细胞型-T细胞来源,另1例为弥漫性大B细胞型,HIV均阴性。PCNSL临床表现无特异性,颅内压增高、精神失常为常见症状,影像检查缺乏特征性改变,免疫表型以B细胞为主(87.5%～98%),大剂量氨甲蝶呤联合全脑放疗缓解率高。结论 PCNSL术前难以诊断,需依赖病理检查确诊,治疗困难,预后差。     

Primary central nervous system lymphoma: a report of 2 cases with clinicopathologic analysis and literature review

目的 探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的临床特点、病理诊断、治疗及预后.方法 对2例PCNSL进行免疫组化染色并复习相关国内外文献.结果 2例PCNSL,1例为间变性大细胞型-T细胞来源,另1例为弥漫性大B细胞型,HIV均阴性.PCNSL临床表现无特异性,颅内压增高、精神失常为常见症状,影像检查缺乏特征性改变,免疫表型以B细胞为主(87.5%～98%),大剂量氨甲蝶呤联合全脑放疗缓解率高.结论 PCNSL术前难以诊断,需依赖病理检查确诊,治疗困难,预后差.     

Localized amyloid of the ureter—case report and review of the literature

An unusual case of localized amyloidosis of the ureter is described and a review of the 12 previously reported cases is presented. No case had been diagnosed prior to surgery. Loin pain, with or without haematuria, associated with a sterile pyuria and an abnormal pyelogram were the commonest clinical signs. In the case reported an incidental small renal cell carcinoma was also present and the association of amyloidosis and neoplasia is discussed.     

Cytogenetics of tumors of the central nervous system: a case study and review of the literature

The introduction of cytogenetic techniques, especially chromosome banding techniques, has facilitated a more detailed study of the chromosomal basis of hematological malignancies and solid tumours. With the advent of molecular cell biology many new insights have been gained into the pathogenetic mechanisms of cancer. With G banding technique we have studied chromosomal aberrations of 174 tumours of the Central Nervous System in adults and the results obtained have been compared with the literature data.     

Spindle cell rhabdomyosarcoma in adults. A case report and literature review

Spindle cell rhabdomyosarcoma, a recently described variant of embryonal rhabdomyosarcoma in children, carries a favorable prognosis when compared with other types of rhabdomyosarcoma. This tumor is rare in adults, and only four cases have been documented previously. The clinicopathological study of such a case occurring in the retroperitoneal space of a 53-year-old man is herein reported. The patient died of uncontrolled local recurrence and hepatic metastases seven months after diagnosis. Based on the analysis of the data of the five cases reported, including the present one, it can be stated that spindle cell rhabdomyosarcoma in adults is not associated with the favorable outcome observed in the pediatric population.     

Giant osteochondroma of ilium: a case report and literature review

Osteochondroma is one of the most common benign bone tumors. It usually grows on the metaphysis of long bones and rarely develops in bones of scapula, feet, hands, and pelvis. The management of this disease is en-bloc excision of the tumor. We present a 45-year-old female subject, who complained of having found a mass on the right hip for more than 20 years which was diagnosed to be osteochondroma on X-ray, computed tomography (CT) and 3-dimensional (3-D) reconstruction. We performed en-bloc excision for the patient. Pathologic examination of surgical specimen confirmed the diagnosis of osteochondroma. The patient made a complete recovery and there has been no recurrence after one year of follow-up. Osteochondroma usually represents an osteo-cartilaginous aberrant overgrowth of normal epiphyseal growth plates. The disease has a slow onset and a long history. X-rays and CT scans are sufficient for diagnosis before surgery and the final diagnosis should based on pathology. Differential diagnosis includes chondrosarcoma or other neoplasms. When osteochondroma causes pain, compression of peripheral nerves, or continuous growth and other clinical symptoms, en-bloc excision of the tumor is needed. Better recognition and more comprehensive evaluation of these rare cases should be highlighted to avoid misdiagnosis during our clinical practice.