Diagnosis and treatment of pancreatic somatostatinoma:

Ppancreatic somatostatinoma represents a rare group of neuroendocrine tumors, which was first reported by Ganda and Larsson in 19771 Less than 50 cases have been reported to the present. We treated a case of pancreatic somatostatinoma at our hospital.     

Diagnosis and treatment of pancreatic somatostatinoma:a case report

Pancreatic somatostatinoma represents a rare group of neuroendocrine tumors,which was first reported by Ganda and Larsson in 1977.Less than 50 cases have been reported to the present.We treated a case of pancreatic somatostatinoma at our hospital.     

A 65-year-old man with infratentorial diffuse encephalopathy

Tuberculous encephalopathy (TBE) is an important diagnosis in countries with a high prevalence of tuberculosis. TBE is a life-threatening condition but rarely reported in the modern literature. We reported a case of a man with extensive parenchymal lesions involving the brainstem and right cerebellar hemisphere that resolved after treatment. The clinical, laboratory and pathological features of this case are highlighted and the pathogenesis is discussed.

     

Ewing''s sarcoma of the maxilla

Primary Ewing's sarcoma of the maxilla is exceedingly rare. Only more than 30 cases have been reported in the English literature.1 As far as we know Ewing's sarcoma of the maxilla has not been reported in the Chinese literature. A case of Ewing's sarcoma of the maxilla is presented and the entity is discussed.     

MEDIASTINAL CAVERNOUS HEMANGIOMA： REPORT OF ONE CASE

MEDIASTINAL cavernous hemangioma is a kind of primary mediastinal mesenchymal tumor that occurs rarely in any age. It was first reported by Shennon1 in 1914. In this study we reported a case of mediastinal cavernous hemangioma with abnormal vessel originating from left brachiocephalic vein.     

Epithelioid angiosarcoma of esophagus

Epithelioid angiosarcomas (EAS) are very uncommon tumors accounting for less than 1％ of all sarcoma and most frequently occur in the skin and subcuits.Primary angiosarcomas in alimentary tract are very rare,especially in esophagus and only one case has been reported in the Chinese literature.1 Evidence-based advice regarding optimal management is lacking.Here,we reported a case of 54-year-old man who was found to have EAS in the esophagus.     

SEMYXOID ADRENOCORTICAL CARCINOMA PRESENTING AS CUSHING SYNDROME: ONE CASE REPORT

Myxoid change in adrenocortical carcinoma is a rare phenomenon,and no case has been reported in Chinese population to date.We report here a case of myxoid adrenocortical carcinoma presenting as Cushing syndrome with an immunohistochemical study of the tumor and discuss the diagnosis with reference to the current literature.     

A case of multiple system atrophy: onset with the cold hands sign

To the Editor: The cold hands sign (CHS) presents a distinct feature in some multiple system atrophy (MSA) patients, but it is receiving little consideration.To our knowledge, there are few reported cases of MSA with onset of CHS.We reported here a case of MSA in a patient with onset of a typical CHS.     

A MYCOLOGICAL STUDY OF A CASE OF OTOMYCOSIS

Since Schwartze(I) first reported a case of otitis externa as being caused by aspergillus, various kinds of fungus have been found to cause aural disease. Sporothrix was isolated from the external ear by Bahre and Hansen(2) and streptothrix by Martin (3) Cann and Hollis (4) demonstrated the presence of actinomyces in a case of external otitis and Chisolm (5) and Gill (6) found several strains of mucor, aspergillus and penicillium in a series of cases of ear infections. According t。 Hatch(7) and Fort (8) otomycosis is rather common in the tropics, espe- cially in India and southern Florida. On the European continent, re- ports of the disease are infrequently seen, and in China there has not been a single case reported.     

Myxoid adrenocortical adenoma: a case report

Myxoid adrenocortical adenomas are extremely rare .with only 24 cases reported, The first case wasreported by Tang et al in 1979 which was diagnosed as a myxoid adrenocortical carcinoma. Nearly all the reports were of single case except Brown et al who described a group of 14 cases. We report here an additional case of myxoid adrenocortical adenoma with an immunohistochemical study of the tumor and discuss the diagnosis with reference to the current literature.     

Intracranial squamous cell carcinoma developing in remnant of an epidermoid cyst:case report and literature review

Epidermoid tumors, sometimes called "pearly tumor", because of the appearance of their outer surface, represent about 0.2%-1.8% of all the primary intracranial tumors1 and are benign developmental lesions. However in 1912, Ernst et al2 found a case of primary intracranial squamous cell carcinoma arising in an epidermoid cyst in autopsy and reported it. Hereafter, case reports about primary intracranial squamous cell carcinoma appeared in succession, especially after computed tomography (CT) and magnetic resonance imaging (MRI) were widely applied in clinical medicine. To our knowledge, few cases have been reported in our country until now. We present a case of intracranial squamous cell carcinoma developing in remnant of an epidermoid cyst 11 years after the operation resection with a review of the literature.     

Acro-dermato-ungual-lacrimal-tooth syndrome: case report

Acro-dermato-ungual-lacrimal-tooth syndrome （ADULT syndrome, OMIM 103285） is a rarely seen ectodermal dysplasia disorder first described by Propping and Zerres in 1993. ADULT syndrome is known as an autosomal dominant disorder. Only a family constellation and four independent cases were reported worldwide up to now. Here, we report a case of ADULT syndrome, which is the first case reported from China.     

Anterior internal lenticonus accompanied by congenital nuclear cataract

Internal lenticonus is a very rare morphologic abnormality of crystalline lens which has been reported in only several cases in the literature. We herein reported the clinical characteristics and surgical findings of the anterior internal lenticonus accompanied by congenital nuclear cataract. Cataract extraction accompanied with intraocular lens implantation was uneventfully performed, and a good visual outcome was achieved in this case. Viral infection during embryonal and fetal period might account for the formation of the anterior internal lenticonus and congenital nuclear cataract in our case.

     

Late-onset primary hyperoxaluria type 1 in a Chinese individual with absent alanine: glyoxylate aminotransferase activity

Nephrolithiasis is a common clinical problem, and its cause is often classified as idiopathic. Primary hyperoxaluria, mostly type 1, constitutes one of the rare causes of recurrent nephrolithiasis, but its diagnosis is often missed or delayed. The exact prevalence of primary hyperoxaluria type 1 ( PH1 ), therefore, has been unclear. The reported prevalence varies in different countries. No Chinese PH1 has ever been reported in the literature. We report a rare case of late-onset primary hyperoxaluria, which was diagnosed only after the development of end-stage renal failure. To our knowledge, this case is the first confirmed Chinese PH1.     

Late-onset primary hyperoxaluria type 1 in a Chinese individual with absent alanine:glyoxylate aminotransferase activity

Nephrolithiasis is a common clinical problem, and its cause is often classified as idiopathic. Primary hyperoxaluria, mostly type 1, constitutes one of the rare causes of recurrent nephrolithiasis, but its diagnosis is often missed or delayed. The exact prevalence of primary hyperoxaluria type 1 (PH1), therefore, has been unclear. The reported prevalence varies in different countries. No Chinese PH1 has ever been reported in the literature. We report a rare case of late-onset primary hyperoxaluria, which was diagnosed only after the development of end-stage renal failure. To our knowledge, this case is the first confirmed Chinese PH1.     

A case report of posterior spinal artery aneurysm

Posterior spinal arterial aneurysm (PSAA) is a rare disease.1-3 In the present article, we reported a case of PSAA associated with arterio-venous malformations (AVMs), which was successfully removed by surgery. Up to now, it is the only case of PSAA we know to be completely cured by surgery.     

OBSTRUCTION OF RIGHT VENTRICULAR OUTFLOW TRACT CAUSED BY INTRACAVITARY METASTATIC DISEASE OF PANCREATIC TUMOR

THE pericardium (90%) is the must common location of cardiac involvement by secondary tumor,followed by myocardium and endocardium (10%).Cases of fight ventricular outflow tract obstruction caused by intraeavitary metastatic tumor growth were rarely reported, although clinical presentation were unique. We herein reported such a case with literature review in order to improve the understanding of malignant cardiac metastasis.     

THE FIRST CASE OF PROTOTHECOSIS ZOPFII IN CHINA

Objective. Report of first case of Protothecosis zopfii in China and causes the skin infection in the world.Method. By clinical and laboratory examinations to confirm the diagnosis and the response to treatment. By the review of literatures to confirm the first case of human skin infection in the world.Result.From the literatures and the clinical pictures, it is confirmed that this is the first case report of Protothecosis zopfii of skin in the world.Conclusion.The first case of Protothecosis zopfii in human being was reported and successfully treated with local infiltration of Diflucan (fluconazole) 3ml (2mg/ml)/week×4.     

Lymphoepithelial cyst of the pancreas： a case report

BACKGROUND: Lymphoepithelial cyst of the pancreas is a rare lesion of undetermined pathogenesis that had been documented almost exclusively in males. The literature on this entity is limited to reports of single or a small number of cases, METHODS: The case we described herein was compared with a total of 36 cases reported elsewhere. RESULTS: The 37 cases of lymphoepithelial cyst of the pancreas including our case were reviewed, Lymphoepithelial cysts have uniform and distinctive clinicopathologic features.     

Early detection of Berry syndrome in a newborn with differential cyanosis

Berry Syndrome is a rare combination of congenital cardiac abnormalities firstly reported in 1982.1 It consists of aortopulmonary window, anomalous origin of the right pulmonary artery （RPA） from ascending aorta, intact ventricular septum, interruption of the aortic arch with patent ductus arteriosus（PDA）. This is the 26th case reported in literature2 and the first report in Hong Kong. Delayed recognition can result in potential lethal condition.