1/12. Juvenile xanthogranuloma presenting as obstructive jaundice.The authors report the case of a 9-month-old infant presenting with obstructive jaundice. Preoperative investigation findings showed distension of the extrahepatic biliary tract. urine analysis result was positive for cytomegalovirus. At laparotomy, a 1-cm round mass was found within the head of the pancreas, near its upper border and in the course of the common bile duct. Findings on frozen sections could not rule out a malignant process and an hepaticoduodenostomy was constructed. Final diagnosis was juvenile xanthogranuloma. An association between cytomegalovirus and juvenile xanthogranuloma has been reported previously in the literature.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
2/12. Juvenile xanthogranuloma variant: a clinicopathological case report and review of the literature.Juvenile xanthogranuloma is a relatively rare cutaneous lesion. In order to make an early diagnosis and be alert to the possibility of visceral complications and associated medical conditions, plastic surgeons should be aware of the entity. The classic presentation is that of successive eruptions in the head, neck and upper trunk of initially red papules or nodules which later become yellow and finally brown flattened plaques or macules. This report is of an unusual variant with atypical histology including frequent mitoses and a lack of Touton giant cells.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
3/12. Neonatal juvenile xanthogranuloma. Report of a case with fine needle aspiration cytologic findings in a soft tissue mass.BACKGROUND: Juvenile xanthogranuloma is an infrequent, benign histiocytic lesion, the recognition and diagnosis of which by fine needle aspiration biopsy are important for ascertaining whether a case will have a benign course or spontaneous regression. CASE: A case of juvenile xanthogranuloma was located in the upper lip of a newborn male. CONCLUSION: Juvenile xanthogranuloma has characteristic cytologic features that may allow recognition in fine needle aspiration cytology smears.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
4/12. Juvenile xanthogranuloma of the orbit in an adult.We present a case of juvenile xanthogranuloma (JXG) with unilateral involvement of the orbit and eyelid and proptosis, histologically confirmed in a 32-year-old man with a 1-year history of a pansinusitis and dacryoadenitis with rhinitis. Nine months later an infiltration of the anterior upper part of the right orbit and right eyelid appeared. Computed tomography scan and magnetic resonance imaging studies confirmed the presence of pansinusitis and infiltration. The patient underwent a blepharoplasty and excision of the infiltrated tissues of the orbit, eyelid, and levator muscle. hematoxylin-eosin and immunohistochemical studies revealed features consistent with a diagnosis of JXG (Touton giant cells). JXG, a non-Langerhans'-type benign proliferation, is a rare condition in adulthood.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
5/12. Solitary spindle-cell xanthogranuloma of the eyelid.PURPOSE: To describe a rare presentation of a solitary spindle-cell xanthogranuloma in a 3-year-old boy with a 6-month history of a right upper lid soft tissue lesion. DESIGN: Interventional case report. methods: An excisional full-thickness biopsy of the right upper lid was performed. Histologic examination and immunohistochemistry of the specimen were performed. RESULTS: Histopathologic evaluation revealed a well-demarcated nodule arising from the tarsus that contained a storiform array of spindle cells admixed with numerous multinucleated giant cells (some Touton type) and lymphocytes. The spindle cells labeled with antibodies to factor xiiia, CD68, lysozyme, Mac 387, and vimentin. The multinucleate giant cells labeled with CD68, lysozyme, and vimentin. Findings were consistent with the diagnosis of solitary spindle-cell xanthogranuloma. CONCLUSIONS: Spindle-cell xanthogranuloma resembles juvenile xanthogranuloma and fibrous histiocytoma (dermatofibroma) and should be considered in the differential diagnosis of spindle-cell tumors of the eyelid and periocular region. If similar lesions are noted elsewhere, the diagnosis of progressive nodular histiocytoma should be considered.- - - - - - - - - - ranking = 2keywords = upper (Clic here for more details about this article) |
6/12. Ulcerated juvenile xanthogranuloma of the scalp.A 7-month-old girl presented with a single rapidly enlarging thickly crusted lesion on her frontal scalp. histology was consistent with a juvenile xanthogranuloma (JXG) with ulceration and surface crust formation. Reports of ulcerating, crusted lesions of JXG are rare and the few reports have been confined to giant forms or those involving mucosal sites. This unusual presentation delayed diagnosis. Our report highlights the great variability in clinical presentation of JXG and is a reminder to consider this diagnosis in the differential of any lesion developing in an infant, particularly on the head, neck and upper trunk.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
7/12. Clinical, pathologic, and biochemical features of a cholesterol lipidosis accompanied by hyperlipidemia and xanthomas.We describe the unique clinical and histopathologic features of a child with biochemical and immunocytochemical features of Niemann-Pick disease type C (NPC). Clinically, she was found to have multiple xanthomas of the upper aerodigestive tract with dysphagia and expressive language delay, splenomegaly, bony infarcts, and type IIb hyperlipidemia. neurologic examination was otherwise normal. microscopy revealed foam cells in her bone marrow, liver, tongue, tonsils, glottis, and in normal-appearing peritonsillar mucosa. Lipid analysis of a liver biopsy specimen showed a small increase in phospholipids, a twofold increase in sphingomyelin, a fivefold increase in cholesterol, and a marked (25-fold) increase in bis(monoacylglycerol) phosphate. Lysosomal acid hydrolase activities in cultured skin fibroblasts were nondiagnostic. Biochemical and immunocytochemical studies of cultured fibroblasts demonstrated lysosomal accumulation of unesterified LDL-derived cholesterol as well as delayed induction of homeostatic responses to endogenous cholesterol consistent with a diagnosis of NPC. Based upon these observations, we speculate that this patient could have a new phenotypic expression of NPC or represents a new cholesterol lipidosis biochemically resembling NPC. The chance occurrence of two separate lipid disorders seems less likely.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
8/12. A boy with autosomal recessive hypercholesterolaemia.We describe a 9-year-old Iranian boy with tuberous xanthomas, elevated LDL-cholesterol levels of 15.5 mmol/l, and vague complaints of chest pain while playing soccer. The consanguineous parents of the boy had normal cholesterol concentrations, which indicated an autosomal recessive disorder rather than autosomal dominant familial hypercholesterolaemia. The diagnosis of autosomal recessive hypercholesterolaemia (ARH) was confirmed by the presence of a mutation in the phosphotyrosine binding domain of a putative adaptor protein, which prevents normal internalisation of the LDL receptor (LDLR) in the liver. The clinical phenotype of ARH is similar to that of classical homozygous familial hypercholesterolaemia caused by defects in the LDLR gene, but it is more variable, generally less severe, and more responsive to lipid-lowering therapy. The patient's complaints of chest pain were not caused by ischaemia as was tested by an exercise and 24-hour electrocardiogram and by a myocardial perfusion scan. His LDL-C dropped by about 6o% after being treated with a combination of 40 mg atorvastatin and 10 mg ezetimibe.- - - - - - - - - - ranking = 1.3301913469696keywords = chest (Clic here for more details about this article) |
9/12. Juvenile xanthogranuloma presenting with unilateral prominent nodule of the eyelid: report of a case and clinicopathological findings.BACKGROUND: The purpose of this study is to report and describe the clinical and histopathologic findings in a patient with juvenile xanthogranuloma presenting as a relatively large, solitary, pedunculated mass on the left upper eyelid. CASE: A 31-month-old Japanese girl presented with a pedunculated nodular lesion that had been developing since 4 months earlier. Otherwise, no abnormal findings were identified in either eye. In addition, no other abnormalities, including skin eruptions or nodules, were detected anywhere. The lesion was completely excised and examined histopathologically. OBSERVATIONS: Microscopic study revealed a histiocytic proliferation accompanied by lymphocytes and occasional plasma cells and eosinophils within the dermis. A moderate number of giant cells of the foreign-body and Touton types were present. In the immunohistochemical analysis, the proliferative histiocytes and giant cells showed positive immunoreactivity for vimentin and macrophage markers and negativity for S-100 protein. The diagnosis of juvenile xanthogranuloma was made. The findings of further detailed ophthalmologic and systemic examinations and evaluations were unremarkable. Therefore, the diagnosis was made of juvenile xanthogranuloma, solitary type, occurring on the eyelid. CONCLUSIONS: Although juvenile xanthogranuloma of the eyelid is uncommon and its occurrence anywhere in the eye is rare, ophthalmologists should be familiar with this entity. Juvenile xanthogranuloma should be included in the differential diagnosis of eyelid mass lesions.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
10/12. Pediatric horner syndrome: etiologies and roles of imaging and urine studies to detect neuroblastoma and other responsible mass lesions.PURPOSE: To evaluate the frequency of etiologies of horner syndrome in children and suggest an imaging and laboratory diagnostic protocol to evaluate for neuroblastoma and other lesions in a child presenting with horner syndrome and no known cause. DESIGN: Retrospective chart and data review. methods: A retrospective review of all children seen at a large pediatric neuro-ophthalmology referral center with a diagnosis of horner syndrome between 1993 and 2005 with particular attention to underlying etiologies and the results of imaging and urine catecholamine studies. RESULTS: Fifty-six children met criteria for horner syndrome and further review. Twenty-eight children (50%) had no previously identified cause for horner syndrome. Of these children, 24 (85.7%) had urine catecholamine metabolite studies, and all had negative results. Twenty (71.4%) had complete modern imaging of the brain, neck, and chest. Of the 18 children who had complete imaging and urine studies, responsible mass lesions were found in six (33%). Four had neuroblastoma, one had Ewing sarcoma, and the other had juvenile xanthogranuloma. Of all patients (diagnosis known and unknown), neoplasm was the etiology in 13 of 56 (23%) of patients. CONCLUSIONS: We confirm that horner syndrome in a child of any age without a surgical history requires a complete examination to exclude a mass lesion. In such patients, we recommend brain, neck, and chest magnetic resonance imaging (MRI) with and without contrast as well as urinary catecholamine metabolite testing. However, imaging is more sensitive than urine testing in this setting.- - - - - - - - - - ranking = 1.3301913469696keywords = chest (Clic here for more details about this article) |
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