[Giant and ulcerated juvenile xanthogranuloma: an atypical presentation in infants]. / Xantogranuloma juvenil gigante y ulcerado: una presentación atípica en lactantes.

Giant Juvenile Xanthogranuloma (GJXG) corresponds to an infrequent variant of Juvenile Xantho- granuloma (JXG) and is characterized by a lesion larger than 2 cm in diameter. It usually presents as a plaque but infrequently, presents as an ulcerated nodule. OBJECTIVE: To report two cases of atypical presentation of GJXG, highlighting the importance of considering them in the differential diagnosis of large, ulcerated tumors in infants. CLINICAL CASES: Case 1: A 4-month-old healthy male infant presented with a rapid and progressive growing left inguinal nodule, present since 2 months of age. At physical examination he presented with a 2.6 cm indurated erythematous nodule with central ulceration. Histological study of an incisional biopsy was compatible with JXG. Ophthalmologic involvement was ruled out. Because of functional impairment and parents worry complete surgical removal was performed. The patient had favorable evolution without local recurrence at 4 years of follow-up. Case 2: A 6-month-old healthy male infant presented with a 2.4 cm scapular crusted nodule of rapid and progressive growth, present since birth. Histological study of an incisional biopsy confirmed JXG. Ophthalmologic involvement was ruled out. After 18 months of periodic clinical follow-up, there was a progressive reduction in size of the lesion. CONCLUSIONS: The cases presented highlight the importance of considering JXG in the differential diagnosis of large, ulcerated tumors in infants. When encountered to atypical JXG presentations, histologic studies help to confirm the diagnosis. Given the favorable prognosis of this diagnosis, periodic clinical follow-up is advised; in exceptional cases, surgical or ablative treatments may be considered.

Juvenile xanthogranuloma as a differential diagnosis for sellar and suprasellar lesions in child: Case report and review of literature.

Juvenile xanthogranuloma (JXG) is a rare type of non-Langerhans cell histiocytosis. Its systemic form affects 4% of patients. Lesions in the Central Nervous System (CNS) occur in 2% of systemic cases. Sellar JXG should be one of the differential diagnoses for sellar lesions in young. This is a 15-year-old patient with non-specific headache, progressive visual loss and magnetic resonance imaging showing sellar lesion with suprasellar extension. The patient underwent microsurgery by pterional craniotomy with partial resection of the tumor. Pathology evidenced JXG. It progressively evolved with impairment of neuroendocrine functions, new lesions in different CNS locations and death two years after diagnosis. Sellar JXG without cutaneous manifestations is rare. There are no specific findings of the disease. Diagnosis requires additional tests, being defined by pathological analysis. Total resection presents a greater potential control comparing to partial resection. Even so, some patients may have progressive disease with poor clinical outcome.

Isolated Forefoot Juvenile Xanthogranuloma: Unique Case Study and Treatment in a Pediatric Patient.

Juvenile xanthogranuloma is a benign histiocytic cell proliferative disorder that occurs in early childhood. The most common presentation occurs within the first 2 years of life with papular or nodular changes to the skin on the head, neck or upper trunk. This case study documents the findings and treatment of a single solitary soft tissue mass in the forefoot of a 17-year-old patient. Unique to this case, the initial diagnosis of tuberous xanthoma was made and, with referral to an outside hospital, changed to a juvenile xanthogranuloma. In addition, unlike most juvenile xanthogranulomas in the literature, there was no superficial dermatological abnormality seen clinically. This change was not a dramatically different diagnosis, but further immunohistochemical staining was necessary for ultimate diagnosis. The soft tissue mass was self-contained to the deeper tissue layers and not the epidermis. The patient was followed for 12 months for possible recurrence and medical workup, without postoperative complications. The purpose of this study was to report on a unique finding and presentation of a xanthogranulomatous soft tissue mass in the forefoot of a pediatric patient.

Xantogranuloma juvenil gigante: acometimento palpebral difuso e simultâneo / Giant Juvenile Xanthogranuloma: diffuse and simultaneous palpebral involvement

O xantogranuloma juvenil (XGJ) é um tumor benigno e o mais comum do grupo das doenças histiocitárias proliferativas nãoLangerhans. Lesões; 2cm são consideradas XGJ gigantes, com relatos de lesões de até 18cm. Lesões oculopalpebrais podem necessitar de tratamento cirúrgico para controle de sintomas. Esse trabalho relata o caso de um menino de 8 anos que teve as 4 pálpebras acometidas por XGJ gigantes, além do terço médio. Ele foi submetido a 3 ressecções, sendo uma bastante profunda, necessitando enxerto de pele de espessura total diretamente sobre o músculo levantador da pálpebra superior. Posteriormente, 3 procedimentos de lipoenxertia foram realizados, atingindo resultado funcional e estético adequado, sem recorrência lesional.

Juvenile xanthogranuloma (JXG) is the most common benign tumor of the group of non-Langerhans histiocytic proliferative diseases. Lesions >2 cm are considered giant JXG, with reports of lesions of up to 18 cm. Oculopalpebral lesions may require surgical treatment to control symptoms. This study reports a case of an 8-year-old boy who had four eyelids and the middle third of the face affected by giant JXG. He underwent three resections, one of which was of great depth that required a full-thickness skin graft directly on the levator palpebrae superioris muscle. Subsequently, four fat-grafting procedures were performed and adequate functional and

Oral Juvenile Xanthogranuloma: Report of Two Cases.

Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually occurs in the skin of children. Extracutaneous involvement is rare, and few cases affecting the oral cavity have been reported. The purpose of the present study was to report two cases of oral juvenile xanthogranuloma affecting the lower lip of a 14-year-old girl and the soft palate of a second 14-month-old girl, both present as yellowish papules. The lesions were surgically excised, and histological and immunohistochemical analysis showed a proliferation of non-Langerhans cells histiocytes and foamy cells, fulfilling the morphologic features of juvenile xanthogranuloma. The patients have been followed up, respectively, for 36 and 49 months with no signs of recurrence. Based on these results, juvenile xanthogranuloma should be included in the differential diagnosis of oral yellowish soft-tissue swellings in children and adolescents and should be managed through conservative surgical excision.

Atypical Fibroxanthoma in a 13-Year-Old Guatemalan Girl with Xeroderma Pigmentosum.

Xeroderma pigmentosum (XP) is a rare, autosomal recessive disease involving a defect in DNA repair leading to the premature development of numerous aggressive cutaneous malignancies. Although atypical fibroxanthoma (AFX) is a neoplasm typically found in the setting of extensive sun exposure or therapeutic radiation, AFXs are rarely associated with children with XP. We report the case of a 13-year-old Guatemalan girl with the XP type C variant who developed one of the largest AFXs reported on a child's finger.

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.

Juvenile xanthogranuloma: forms of systemic disease and their clinical implications.

OBJECTIVE: Juvenile xanthogranuloma (JXG) with systemic (extracutaneous) involvement is a rare histiocytic disorder in which significant morbidity and occasional deaths may occur. The objective of this study was to characterize the spectrum of anatomic involvement, associated clinical problems, and management considerations in children with systemic JXG. STUDY DESIGN: Two current cases and literature reports of 34 children with various forms of systemic AG were analyzed with respect to age, clinical presentation, site(s) of involvement, therapy, and outcome. RESULTS: The median age of the 36 patients was 0.3 years (range, birth to 12 years). Symptoms were usually referable to bulky or infiltrative disease. Twenty patients had disease in two or more sites. Cutaneous lesions were present in fewer than half the patients. The most frequent extracutaneous sites of disease were the subcutaneous soft tissue (12); central nervous system (8); liver/spleen (8); lung (6); eye/orbit, oropharynx, and muscle (4 each); with three or fewer instances of disease in each of several other sites. Most patients were treated with excision or had spontaneous regression (some with organ involvement). However, 12 patients received treatment that included radiation or systemic chemotherapy. Survivors, some with long-term disabilities, included young children who had received radiation therapy to the brain, eye, skin, or heart. Two patients died of disease. CONCLUSIONS: Systemic AG may involve varying numbers and combinations of extracutaneous sites. The extent of disease should be determined in patients with AG who are suspected to have systemic involvement. In contrast to the cutaneous form, systemic AG may be associated with significant complications requiring aggressive medical care. When feasible, surgical excision of lesions may be curative. Optimal treatment for symptomatic, unresectable disease is currently undefined but should be selected to minimize toxic effects in these children who are typically younger than 1 year old at presentation.

[A xanthogranulomatous psoas abscess 4 years after nephrectomy for xanthogranulomatous pyelonephritis]. / Absceso xantogranulomatoso del psoas 4 años después de nefrectomía por pielonefritis xantogranulomatosa.

We report a case of xanthogranulomatous abscess of the left psoas muscle in a patient who underwent nephrectomy for xanthogranulomatous pyelonephritis four years earlier. Pathogens unlike those usually found in this type of inflammatory reaction were grown on culture. The possible relationship between the two infectious disease processes is analyzed.

Xantogranuloma juvenil de iris / Juvenile xanthogranuloma of iris

Se comunica el caso de un lactante de 6 meses de edad, con lesión tumoral de color café amarillento, de rápido crecimiento, localizada a nivel de la raíz iridiana nasal de OD. Se realizó escisión sub-total de la lesión seguida por tratamiento esteroidal tópico. Un año más tarde, habiendo remitido completamente la lesión, la excentricidad de la pupila y la presencia de membrana pre-cristaliniana hicieron necesaria uba pupiloplastía. Durante esta cirugía se resecó pequeña pápula café amarillenta infraumbilical. El estudio histopatológico óptico y electrónico de las lesiones iridiana y cutánea concluyeron en xantogranuloma juvenil. Sus características clínicas y morfológicas se analizan y discuten