11/183. 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. ( info) |
12/183. Juvenile xanthogranuloma on the sole. Juvenile xanthogranulomas are benign, typically self-limiting tumors that most commonly occur on the head and trunk. We report one such tumor with an atypical clinical appearance, occurring at an unusual site, the sole of the foot. ( info) |
13/183. September 2000: 15 year old girl with intracranial lesion. The September COM: We reported the case of a 15 year old white girl with solitary brain cortical lesion clinically mimicking meningioma. Microscopic examination revealed histiocytic lesion with foamy and giant cells predominately of Touton type. Subsequent clinical examination did not reveal any similar lesion in the skin or in other location. Expression of S-100 protein in histiocytes is unusual finding, but has been reported recently in juvenile xanthogranulomas (JXG). The only specific ultra-structural finding differing JXG from histiocytic neoplasm of Langerhans' cells histiocytosis type seems to be Birbeck granules. ( info) |
14/183. Intracardiac juvenile xanthogranuloma in a newborn. Juvenile xanthogranuloma (JXG) presents a normolipemic non-langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions. ( info) |
15/183. Juvenile xanthogranuloma of peripheral nerve: a report of two cases. As a rule, juvenile xanthogranuloma (JXG) is a cutaneous lesion most often occurring in infancy. An inflammatory process of unknown etiology, it is self-limited and benign in nature. The spectrum of JXG has expanded to include adult examples, multifocal lesions, and ones arising at extracutaneous locations. Although a variety of extracutaneous sites may be affected, few reported lesions have involved cranial or peripheral nerves. Solitary examples have been reported in trigeminal nerve and spinal nerve root; affected individuals were children or adolescents. An optic nerve lesion has also been described. We describe two additional cases of JXG of nerve. One patient developed multiple dorsal nerve root lesions, as well as skin involvement. The other case featured isolated involvement of the left radial nerve. Both patients were adults with no known underlying systemic disorder. These cases further expand the spectrum of extracutaneous JXG, and underscore its consideration in the differential of nerve "tumors." ( info) |
16/183. Isolated juvenile xanthogranuloma of the subglottis: case report. BACKGROUND: Juvenile xanthogranulomatosis (JXG) is a relatively rare macrophage proliferative disorder. It usually presents as a localized cutaneous lesion but may affect other organs. Until now it has never been described in the subglottic region of the larynx. methods: We report the first case of juvenile xanthogranulomatosis (JXG) in the subglottis in a 3 year old child. RESULTS: The localization in the subglottis caused airway obstruction requiring tracheostomy to secure the airway. On the basis that most cutaneous lesions regress spontaneously the lesion was managed expectantly and regressed over a period of 28 months allowing decannulation of the child. CONCLUSION: JXG should be considered in the differential diagnosis of subglottic lesions. Once the airway has been secured, JXG of the subglottis can be managed conservatively. Long-term follow-up is required because of the possibility of relapse at other sites. ( info) |
17/183. Histopathologic maturation of juvenile xanthogranuloma in a short period. We present a case of solitary juvenile xanthogranuloma (JXG) on the scalp of an 8-month-old girl. The initial biopsy specimen showed a dense collection of small histiocytes as evidenced by CD68 staining without either lipidization or giant cell formation, admixed with a small number of lymphocytes. On the other hand, sections from the excised specimen obtained 2 weeks after the initial biopsy from the same site showed a mixed proliferation of abundant foam cells together with Touton giant cells, some small histiocytes, and small numbers of lymphocytes and eosinophils. Mitotic figures were fewer in the excised nodule than in the initial biopsy specimen. Fascicles of spindle-shaped cells arranged in a vague storiform pattern were additionally found in the deep portion of the nodule. Our case findings suggest that xanthomatization of the JXG could have been accelerated by the inflammation associated with the biopsy, based on the histopathologic fact that the change from an early phase to a mature form occurred within the very short period of 2 weeks. ( info) |
18/183. Juvenile xanthogranuloma: case report with immunohistochemical identification of early and late cytomegalovirus antigens. Juvenile xanthogranuloma (JXG) is a dermatological condition of unknown etiology that rarely affects the oral mucosa. There are conflicting reports suggesting that it may represent a reactive virally-induced lesion associated with cytomegalovirus (CMV) infection. The present paper reports an additional case of oral JXG and discusses its possible association with CMV infection. The biotin-streptavidin system was used to detect early and late CMV antigens. Positive immunolabelling for both antigens was demonstrated in some histiocytes in the lesion. These findings suggest that JXG may be associated with CMV infection. ( info) |
19/183. Isolated cervical juvenile xanthogranuloma in childhood. STUDY DESIGN: This is a report of an exceptional case of isolated cervical juvenile xanthogranuloma in a child. OBJECTIVES: This case report draws attention to the fact that isolated xanthogranuloma of the central nervous system should be considered among possible diagnosis of subdural extramedullary spinal masses in children and young adults. SUMMARY AND BACKGROUND DATA: Isolated juvenile xanthogranuloma of the central nervous system is extremely rare. When located in the spinal canal it behaves like any extramedullary mass-occupying lesion. MRI depicts the tumor's association with adjacent structures. In cases in which a subtotal surgical removal was possible, radiotherapy has been indicated. methods: A three-year-old girl presented severe pain in the right shoulder and spastic tetraparesis. The MRI showed an intradural extramedullary mass homogeneously enhancing after DTPA-gadolinium infusion. Complete surgical removal of the tumor was performed through open-door laminoplasty. RESULTS: The child was pain free immediately after the surgical removal of the tumor. A gradual complete recovery of the neurologic deficits followed. Open-door laminoplasty provided sufficient operative space, and it minimized the impact on the growing spinal column. CONCLUSIONS: Isolated juvenile xanthogranuloma does not show any predilections of localization inside the central nervous system. Both intracranial and spinal juvenile xanthogranulomas appear isointense in MRI and enhance homogeneously with gadolinium. Whenever possible, total surgical removal alone seems to be curative. Otherwise, a subtotal removal of the tumor might be followed by radiotherapy. Immunohistochemical tests ensure the diagnosis. ( info) |
20/183. Spectroscopic increase in choline signal is a nonspecific marker for differentiation of infective/inflammatory from neoplastic lesions of the brain. We report in vivo proton magnetic resonance (MR) spectroscopic findings in three benign infective/inflammatory lesions (one case each of tuberculoma, fungal granuloma, and xanthogranuloma), which showed high choline along with the presence of lipid/lactate, a feature characteristically described in neoplastic lesions. Histopathology of the lesions showed inflammatory cellular infiltrates with areas of necrosis/caseation. The spectroscopic-visible increased choline resonance in these lesions is probably the result of cellularity. We conclude that increased choline, along with the presence of lipid/lactate is a nonspecific finding and may not be of much value in the differentiation of neoplastic from nonneoplastic infective/inflammatory intracranial mass lesions. ( info) |