SevereRhodococcus equi pneumonia: Case report and literature review

Rhodococcus equi is an aerobic, gram-positive, non-motile pleomorphic bacillus infecting immunocompromized patients. Forty-nine cases ofRhodococcus equi infection have been reported, mainly in patients infected with the human immunodeficiency virns (HIV). A case in whichRhodococcus equi eaused severe pulmonary infection, the most common presentation, is described. Clinically, patients have symptoms of pneumonia with hemoptysis as a prominent feature. X-ray will often show a cavitating upperlobe infiltrate, resembling infection with mycobacteria.Rhodococcus equi is easily cultured from blood or sputum on standard media, but is frequently regarded as a contaminant. Mortality fromRhodococcus equi pneumonia is high (25 %) and early surgical intervention has been recommended. Based on this review, the benefit of surgery seems dubious, whereas good results have been obtained using long-term antibiotic treatment with erythromycin plus rifampicin, or vancomycin in combination with either of these antibiotics.     

Malarial pancreatitis: Case report and systematic review of the literature

Malaria can cause a wide spectrum of clinical manifestations ranging from uncomplicated febrile illness to multiorgan failure. Pancreatitis is a rare complication of malaria with only a few reported cases. Herein, we describe a case of acute pancreatitis with multiorgan failure due to Plasmodium falciparum managed successfully with antimalarials and conservative treatment. We also perform a systematic review of literature for reports of acute pancreatitis due to Plasmodium infection.     

Amyloidosis secondary to chronic pulmonary aspergillosis: Case report and a systematic review of literature

Secondary amyloidosis results from the deposition of abnormally folded proteins in body organs due to chronic inflammatory disorders. Kidneys are the most commonly affected organ and manifest as nephrotic syndrome with or without renal failure. Chronic pulmonary aspergillosis (CPA) is a chronic infection of lung parenchyma affecting those with an underlying structural lung disease. Herein, we present a case of CPA where the initial manifestation was that of nephrotic syndrome due to renal amyloidosis. We also perform a systematic review for studies describing secondary amyloidosis due to CPA.     

Pasteurella pneumonia: report of a case and review of the literature.

A case of pneumonia due to Pasturella ureae was encountered in a 57-year-old man who developed bilateral pulmonary infiltrates and respiratory insufficiency while convalescing in the hospital from a hip fracture and multiple rib fractures. Cultures of his sputum grew an essentially pure growth of Pasturella ureae. This organism, a small gram-negative rod, could be differentiated from the other Pasteurella species by its ability to hydrolyze urea and failure to produce indole. The literature on Pasteurella infections is briefly reviewed, and the recent taxonomic revisions of the genus Pasteurella are discussed.     

Corticotroph pituitary carcinoma: Case report and literature review

Pituitary carcinomas, currently defined as primary adenohypophyseal neoplasms with evidence of either brain invasion or metastatic spread, are exceptionally rare. A case of corticotroph pituitary carcinoma is reported. A 17-yr-old female first presented with an invasive and clinically nonfunctioning pituitary macroadenoma. The primary pituitary lesion lacked atypical histological features and retrospective immunohistochemical studies confirmed its corticotrophic nature. Repeated episodes of local recurrence followed together with the acquisition of severe Cushing’s disease. Local disease control was not obtained despite repeated surgical decompression and courses of radiation therapy. Systemic dissemination with multiple bone metastases became manifest eleven years after the first presentation. Atypical histological features and Crooke’s hyaline change were evident in both the recurrent and metastatic disease. She remains alive at last follow-up with severe complications relating to persisting sellar and metastatic disease and ongoing endocrine dysfunction. A further 32 cases of pituitary corticotroph carcinoma reported in English are reviewed to highlight the clinicopathological features of this rare form of pituitary neoplasm. Difficulties associated with the diagnosis and management of pituitary carcinoma are also discussed.     

Retroperitoneal ectopic brain: Case report and literature review

Heterotopic neural tissue (HNT) has been identified in many sites. In our literature review, we have found only two cases of HNT located in the retroperitoneum. As far as we know, cytological features of HNT have not been described. We here report a case of retroperitoneal HNT diagnosed by fine‐needle aspiration (FNA) cytology and subsequent resection. Our patient was a male infant born at 26 weeks’ gestation. A retroperitoneal solid‐cystic lesion measuring 3.8 cm was identified by abdominal ultrasound. FNA was performed and cytology showed highly cellular smears with single cells and masses of fibrillary material. Cells were small with rounded irregular nuclei and variable cytoplasm. Multinucleated cells, cells with neuronal morphology, calcifications, and hemosiderophages could also be seen. The lesion was excised and histology showed fibrillary areas, glial cells, neurons, and ependymal cells. Synaptophysin, GFAP, and EMA were expressed in distinct areas and cell types. No recurrences were observed.     

Rhupus: a systematic literature review

“Rhupus” or “rhupus syndrome” is a poorly described and underdiagnosed disease in which features of both rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) appear in the same patient, most often sequentially. The SLE-related involvement is usually mild, dominated by hematological abnormalities and skin, serosal and renal involvement. The natural history of rhupus arthritis follows an RA-like pattern and can progress towards typical inflammatory erosions, deformations and disability. Despite the lack of consensus on the definition of rhupus and on its place in the spectrum of autoimmunity, a growing number of studies are pointing towards a true overlap between RA and SLE. However, the inclusion criteria employed in the literature during the last 4 decades are heterogeneous, making the already rare cohorts and case reports difficult to analyze. Because of this heterogeneity and due to the rarity of the disease, the prevalence, pathophysiology and natural history as well as the radiological and immunological profiles of rhupus are poorly described. Moreover, since there is no validated therapeutic strategy, treatment is based on clinicians’ experience and on the results of a few studies.We herein present a systematic literature review to analyze the clinical and laboratory data of all reported rhupus patients and to provide up-to-date information about recent advances in the understanding of the pathophysiological mechanisms, diagnostic tools and treatment options.     

Multiple accessory spleens: Case report and literature review

Accessory spleens may be overlooked during splenectomy. Consequently, a second splenectomy may be necessary. This report concerns the occurrence of multiple accessory spleens discovered during routine dissection of a male cadaver. Their size ranged from 0.5 to 6 cm, and they were located in the hilus and ligaments of a spleen with a normal appearance. Their splenic structure was confirmed histologically. The embryological basis of accessory spleens is re-examined, and the frequency of their occurrence and their clinical significance are reviewed. The purpose of this paper is to alert the practicing surgeon again to the existence and significance of accessory spleens. © 1993 Wiley-Liss, Inc.     

Case report and literature review: primary cutaneous carcinosarcoma

Carcinosarcomas are rare but aggressive neoplasms commonly described in organs such as the breast, urinary bladder, uterus, liver, and lungs. Histopathologically, they are characterized by the presence of malignant epithelial and mesenchymal components. The exact histogenesis of carcinosarcomas remains unknown and is debated in the literature. Primary carcinosarcomas of the skin are uncommon. To our knowledge, 20 cases of primary cutaneous carcinosarcoma have been described in the world literature. Most of these tumors were seen on the head and neck region of older individuals, both male and female. Microscopically, the more common carcinoma component is a squamous cell carcinoma followed by basal cell carcinoma, whereas the most common sarcoma component is an osteosarcoma. We report an example of this rare entity and speculate on its histogenesis in the skin.     

Yersinia enterocolitica endocarditis: Case report and literature review

Endocarditis is a rare manifestation ofYersinia enterocolitica infection. The case of a 45-year-old man who presented with high fever and in whom prosthetic valveYersinia enterocolitica endocarditis was diagnosed is described. The patient was successfully treated with ceftriaxone plus tobramycin, as proved by negative cultures of the prosthesis removed at the end of therapy. Including the patient reported, only 12 cases ofYersinia enterocolitica endocarditis have been published to date, two of which describe prosthetic cardiac valve endocarditis. The clinical characteristics do not distinguish septicemia from involvement limited to the cardiac valves. Diagnosis, however, has been improved by progress in echocardiography. Prognosis is grave but can be ameliorated if appropriate antimicrobial agents are administered, i.e. the combination of a third-generation cephalosporin plus an aminoglycoside. Fluoroquinolones may also constitute an attractive therapeutic alternative.     

Metaplastic breast carcinoma: a case report and systematic review of the literature

A 78‐year‐old retired woman was diagnosed with metaplastic breast carcinoma (MBC), a rare tumor, in our hospital. We reviewed 15 articles with a total of 1328 patients to determine the epidemiology, clinical features, biomarkers, histology, management and outcome of patients with this tumor. The mean age at presentation is 58.5 years (range 32–83). Eighty‐one percent of patients presented either with a breast mass or abnormal mammographic finding. Twenty‐three percent of patients had a family history of breast cancer. Estrogen receptors were only found in 12%, progesterone receptors in 10% and HER2 in 6% of patients. The main method of treatment was mastectomy (66.9%) in combination with chemotherapy (57%) and radiotherapy (47%). Five‐year disease‐free survival ranged between 40% and 84% and 5‐year overall survival ranged between 64 and 83%. We have further reviewed the nature of this disease in the light of advancement in genetics, such as microarray gene expression profiling. The relationship of MBC with triple‐negative tumor and basal‐like tumor is discussed. It is hoped that advances in genetics and biomarkers will bring forward the era of personalized medicine in the treatment of breast carcinoma.     

Meningoencephalomyelitis with vasculitis due to varicella zoster virus: a case report and review of the literature

Varicella zoster virus (VZV) encephalitis is associated with large or small vessel vasculopathy. We report the case of a 67-year-old woman with a history of non-Hodgkin's lymphoma and cancers of the breast and colon, who presented with a zosteriform rash and Brown-Sequard syndrome. Despite 10 days therapy with intravenous acyclovir, meningoencephalitis developed and the patient died 15 days after onset of neurological symptoms. Autopsy showed meningoencephalomyelitis with necrotising vasculitis of leptomeningeal vessels, which is a rare complication of VZV, and we review the literature of the nine similar published cases. Polymerase chain reaction of cerebrospinal fluid for VZV was negative 6 days after onset of neurological symptoms, but became positive by day 10. Only one multinucleated giant cell with intranuclear Cowdry type A inclusions was seen within an endothelial cell in a leptomeningeal vessel involved by vasculitis.     

Prostatic signet-ring cell carcinoma: Case report and literature review

Signet-ring cell carcinoma (SRCC) of the prostate is a very rare neoplasm and there have been only 38 cases reported to date. Here the 39th case of prostatic SRCC containing a small amount of neutral mucin, prostatic specific antigen (PSA) and prostatic specific acid phosphatase (PSAP) in the signet-ring cells is reported. It was also found that some intracytoplasmic lumina were derived from the shallow or deep invagination of luminal membranes of cancer cells that formed the neoplastic glands. Using immunohistochemistry, a combination of monoclonal antibodies against cytokeratins 7 and 20 as well as PSA and PSAP may be useful in differentiating prostatic primary SRCC from metastatic SRCC originating in the gastrointestinal tract.     

Adenovirus related lymphohistiocytic hemophagocytosis: Case report and literature review

IntroductionAdenoviral infection is a classic cause of lymphohistiocytic hemophagocytosis (LH) in bone marrow transplantation but is rare outside this setting.Case reportA 31-year-old female, with a history of treated mesencephalic astrocytoma, was hospitalized for fever, pancytopenia, elevated liver enzymes, hyperferritinemia and hypertriglyceridemia. Adenovirus viral load in blood was 7.3 × 10⁹ copies/mL. Bone marrow aspirate examination confirmed LH. The patient recovered without specific LH or adenovirus-directed treatment.ConclusionAdenovirus-related LH, common in bone marrow transplant recipients, should also be considered in patients with chemotherapy in solid tumors.     

Antley-Bixler syndrome from a prognostic perspective: report of a case and review of the literature

The Antley-Bixler syndrome (ABS) is characterized by craniosynostosis, radiohumeral synostosis, and femoral bowing. Other findings include a trapezoid-shaped head, deformed ears, severe midface hypoplasia, choanal atresia or stenosis, and long bone fractures. Most ABS cases have died in the first months of life from respiratory complications. The poor prognosis in this condition makes counseling difficult and early termination of pregnancy a consideration. The medical and surgical management information presented here can be used as a guide for counseling parents in the future. We report on a new patient with ABS who now at age 3 yr, has been followed by the medical staff of Riley Children's Hospital since birth. She has had successful medical and surgical management. Although the multisynostoses seen in this disorder is undoubtedly related to the soft tissue malformations such as choanal stenosis and midface hypoplasia, the cause remains unknown. The literature is also reviewed in this condition.     

Kartagener综合征一例并文献复习

目的探讨Kartagener综合征的临床特点、诊断标准,以提高对Kartagener综合征认识。方法 Kartagener综合征1例。患儿,女,10岁余。因反复咳嗽1年,咯血4次入院。回顾性分析该患儿临床资料,检索国内外文献,进行总结分析。结果患儿隐匿起病,主要表现为反复咳嗽,咳脓痰,伴咯血。CT示双肺支扩伴感染,全内脏反位,鼻窦炎。经抗感染对症治疗1周后,临床症状消失出院。检索国内外文献发现,Kartagener综合征以全内脏反位,支气管扩张,副鼻窦炎三联征为诊断标准。治疗以对症治疗为主。结论 Kartagener综合征是一种罕见的常染色体隐性遗传病,加强对该病的认识,有利于提高对该病的诊断率,减少误诊率。对该病的致病基因、机制的进一步研究,可能提供更好的诊断和治疗方法。