1/30. 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.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/30. 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 = life (Clic here for more details about this article) |
3/30. 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.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
4/30. 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.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/30. 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.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/30. association of juvenile xanthogranuloma with cafe-au-lait macules.A 2-year-old boy was referred to the dermatology services for the evaluation of yellowish-brown raised lesions over the face of 3 months' duration. In addition, he had multiple asymptomatic hyperpigmented spots over the trunk, which his parents had noticed at the time of birth. His physical and mental milestones of development were normal. Apart from the cutaneous lesions, the child was otherwise well and there was no history of seizures. He was the only child born to nonconsanguineous parents. No family history of similar lesions was available. The boy weighed 14 kg, had a height of 88 cm, and a head circumference of 48 cm. General physical and systemic examination was normal. Cutaneous examination revealed multiple, yellowish-brown papules over the forehead, sides of the face, and shoulders. The papules measured 0.5-0.8 cm in diameter, were discrete, well defined, oval and flat topped without any surface changes (Fig. 1). Multiple cafe-au-lait macules varying from 0.5 to 3.0 cm in diameter were present over the trunk (Fig. 2). There was no freckling in the axillary or the inguinal regions and no neurofibromas were present. A diagnosis of juvenile xanthogranuloma was considered. Complete blood counts, urine analysis, hepatic and renal function tests, and serum lipid levels were normal. A skin biopsy from a papule over the shoulder revealed a dense infiltrate in the dermis underlying a normal epidermis. The infiltrate was well demarcated and comprised lymphocytes, eosinophils, and foamy histiocytes along with Touton giant cells and proliferating fibroblasts. This confirmed the diagnosis of juvenile xanthogranuloma. The parents were informed about the benign and self-limiting nature of the disorder and were advised to bring the child for regular follow-up.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/30. A case of juvenile xanthogranuloma arising on a nevus sebaceus.Various epithelial and mesenchymal neoplasms can arise in nevus sebaceus (NS). Juvenile xanthogranuloma arising on an NS has not been reported previously. Juvenile xanthogranuloma, a disease characterized by reactive histiocytic proliferation, could be included in the list of secondary alterations arising on an NS.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/30. Mitotically active juvenile xanthogranuloma.Juvenile xanthogranuloma is a histiocytic inflammatory disorder capable of presenting different histologic patterns. The classic form consists of sheets of foamy histiocytes and numerous multinucleated Touton-type giant cells containing no to very few mitotic figures. Nonlipidized juvenile xanthogranuloma consists of a diffuse monomorphic histiocytic infiltrate and usually shows a slightly higher mitotic index. Histologic hematoxylin-eosin staining techniques were used after excision of a pink ulcerated nodule in a 2-year-old girl. Histopathologic examination showed a nonencapsulated cellular proliferation mainly occupying dermis, but extending into the subcutaneous tissue as well. Most of the cells were epithelioid histiocytes, but foamy and Touton giant cells were also evident. The most striking feature was the presence of a high number of mitotic figures, as many as 23 per 10 high-power fields. We present an unusual case of classic juvenile xanthogranuloma with areas similar to those found in the nonlipidized type and with a high mitotic index.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/30. Juvenile xanthogranuloma invading the muscles in the head and neck: clinicopathological case report.Juvenile xanthogranuloma (JXG) is a member of the non-Langerhans cell group of histiocytic proliferative disorders. It typically presents as a solitary, benign, rapidly growing cutaneous tumor that may regress spontaneously. Most cutaneous lesions of JXG occur in the head and neck region of infants and young children. JXG has been documented in many visceral locations including the lung, bone, testis, gastrointestinal tract, kidney, heart, eye, and oral cavity. Intramuscular location is extremely rare. The few intramuscular lesions that have been described involve the trunk, with the musculature of the back involved most frequently. The authors present a patient with juvenile xanthogranuloma on her chin deeply invading the underlying facial muscles. Histopathological analysis confirmed the intramuscular extension. To the authors' knowledge, this is the first detailed clinicopathological report of juvenile xanthogranuloma infiltrating the muscles in the head and neck.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/30. Solitary intramuscular nasal Juvenile Xanthogranuloma: a case report with review of literature.Juvenile Xanthogranuloma is a non-langerhans cell histiocytosis characterized by yellowish cutaneous nodules that usually appear in early infancy and childhood. Intramuscular variant is a rare form, with only eight reported cases, and none reported in ala of nose. Sheets of histiocytes, few touton giant cells and infiltrative borders makes it susceptible to misdiagnosis as childhood sarcomas or lymphoproliferative disorders. awareness of the lesion aided by immunohistochemistry helps in reaching the proper diagnosis.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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