Cases reported "Xanthogranuloma, Juvenile"

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1/183. Verruciform xanthoma associated with squamous cell carcinoma.

    Verruciform xanthoma (VX) is a rare lesion of unknown etiology that is typically solitary and predominantly located within the oral cavity. Less commonly, they arise on the skin, with the majority of cases occurring in anogenital sites. They can be confused clinically with verruca vulgaris, condyloma, leukoplakia, verrucous carcinoma, and squamous cell carcinoma. Histologic features include acanthosis with uniform elongation of the rete ridges and xanthomatous cells that lie in and are typically confined to the papillary dermis. Although epidermal atypia is not a characteristic finding, we describe an unusual case of VX that has features of both VX and squamous cell carcinoma. In addition, there was a VX with typical histologic characteristics located at a separate site in the same patient. This case is also the first to our knowledge to be reported on the neck and axilla and is the third case associated with cutaneous graft versus host disease secondary to bone marrow transplant for acute lymphoblastic leukemia. ( info)

2/183. 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. ( info)

3/183. Giant juvenile xanthogranuloma of the tongue.

    Juvenile xanthogranulomas (JXGs) are rare, benign, fibrohistiocytic lesions. They usually appear as one or more cutaneous papules on the head, neck, or trunk in infants. Twelve cases of oral JXGs have been reported, four of which involved the tongue. We present a 6-year-old girl with a large tongue mass diagnosed as JXG after an excisional biopsy. Histological and immunohistochemical staining results are presented. This is the first reported case of a giant oral JXG. A review of the literature on these unusual lesions is presented, along with discussion of their differential diagnosis and key aspects of the patient's evaluation, management, and pathological diagnosis. ( info)

4/183. 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. ( info)

5/183. Radiological and clinicopathological features of orbital xanthogranuloma.

    BACKGROUND: Orbital xanthogranuloma, a diagnosis confirmed histologically, occurs rarely in adults and children. With its characteristic macroscopic appearance the adult form may be associated with a spectrum of biochemical and haematological abnormalities including lymphoproliferative malignancies. METHOD: The clinicopathological features and imaging appearances on computed tomography and magnetic resonance imaging of this condition are described in eight adults and a child. RESULTS: Radiological evidence of proptosis was present in seven patients. In all nine patients an abnormal infiltrative soft tissue mass was seen, with increased fat in six cases. All patients had associated enlargement of extraocular muscles suggestive of infiltration and five had lacrimal gland involvement. Encasement of the optic nerve, bone destruction, and intracranial extension was present only in the child with juvenile xanthogranuloma. Haematological and/or biochemical abnormalities were detected in seven patients and seven patients had other systemic diseases which were considered to have an immune basis. One patient subsequently developed non-Hodgkin's lymphoma. CONCLUSION: The investigation and management of orbital xanthogranulomas requires a multidisciplinary approach even though the diagnosis may be suspected clinically. Imaging delineates the extent of disease and involvement of local structures and may influence the differential diagnosis. The juvenile form may be more locally aggressive, causing bone destruction with consequent intracranial extension. ( info)

6/183. Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus?

    Benign cephalic histiocytosis (BCH) is best understood as a form of non-Langerhans cell histiocytosis, specifically as an early mononuclear variant of juvenile xanthogranuloma (JXG). However, the progression of BCH into JXG in the same patient has only been reported once before. We describe the case of a 2-year-old girl with asymptomatic, large, ill-defined infiltrated flat plaques over both cheeks, in addition to isolated papules. A punch biopsy of a plaque revealed dermal infiltration by vacuolated and scalloped histiocytes positive for CD68 KP-1, and that lacked expression of CD1a and S-100 protein, favoring macrophages over langerhans cells. Electron microscopy study showed comma-shaped intracytoplasmic bodies in the histiocytic cells leading to the diagnosis of BCH. One year later, after an episode of varicella-zoster infection, the flat plaques over the cheeks became large reddish-yellow nodules, and in a second biopsy appeared to progress to JXG. Virus-related mechanisms of progression are discussed. ( info)

7/183. 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. ( info)

8/183. Symptomatic bilateral xanthogranulomas of choroid plexus in a child.

    choroid plexus xanthogranulomas are uncommon lesions that occur almost exclusively in adults; most of them constitute incidental autopsy findings. A case of symptomatic bilateral xanthogranulomas of choroid plexus in a 6 year-old girl with progressive visual loss is reported. Theories on the pathogenesis of this entity are reviewed. ( info)

9/183. Multiple intracranial juvenile xanthogranulomas. Case report.

    The authors report on an 11-year-old boy in whom proptosis of the eye caused by a benign intraosseous xanthofibroma of the left orbital wall became clinically apparent at the age of 4 years. Two years later he developed bilateral papilledema, at which time computerized tomography and magnetic resonance studies revealed multiple enhancing intracranial lesions. The largest mass was located in the left middle fossa; other lesions were located at the tentorium cerebelli, in both lateral ventricles, near the superior sagittal sinus, and extracranially near the left jugular vein. The mass in the left middle fossa was resected and diagnosed as juvenile xanthogranuloma (JXG). Thirty months later, the patient again became symptomatic, exhibiting behavioral abnormalities and a decrease in mental powers. At that time, the two remaining lesions in both lateral ventricles had grown enough to cause trapping of the temporal horns and raised intracranial pressure. These lesions were successively resected and histopathologically confirmed to be JXGs. However, resection of the second intraventricular lesion was complicated by postoperative bilateral amaurosis, presumably caused by postdecompression optic neuropathy. According to a review of the literature, fewer than 20 patients with JXG involving the central nervous system have been reported. The patient described in this report is the first in whom multiple intracranial JXGs developed in the absence of cutaneous manifestations. Although JXGs are biologically benign lesions, the treatment of patients with multifocal and/or progressive intracranial manifestations is problematic. ( info)

10/183. Juvenile xanthogranuloma of the nasal cavity.

    Juvenile xanthogranuloma is a benign, normolipaemic, self-healing condition and a type of histiocytosis that occurs most frequently in infants and children. This condition usually presents with solitary or multiple cutaneous lesions and occasionally with visceral lesions. We report a case of juvenile xanthogranuloma occurring in the nasal cavity. We believe this is the first report, in the English literature, of juvenile xanthogranuloma occurring in this site. ( info)
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