Presentación clínica de patologías no malignas que simulan leucemia cutis: a propósito de dos casos / Clinical presentation of non-malignant diseases mimicking leukemia cutis: A report of two cases

La infiltración cutánea por células leucémicas conocida como leucemia cutis es una presentación infrecuente de esta patología y constituye un desafío diagnóstico. Los diagnósticos como infecciones, otras patologías neoplásicas con afectación cutánea y los trastornos histiocíticos, entre otros, constituyen los principales diagnósticos diferenciales, ya que configuran un escenario pronóstico y terapéutico diferente. Se presentan dos pacientes que fueron diagnosticados inicialmente como leucemia cutis, cuyo diagnóstico final fue de patologías no malignas.

The infiltration of leukemia cells into the skin, known as leukemia cutis, is a rare presentation of this disease and accounts for a diagnostic challenge. The main differential diagnoses include infections, other neoplastic diseases with skin involvement and histiocytic disorders, among others, as they entail different prognostic and therapeutic approaches. Here we describe two patients who were initially diagnosed with leukemia cutis, whose final diagnosis was of non-malignant diseases.

Clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous disease / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To examine the clinicopathological characteristics of adult orbital xanthogranulomatous disease(AOXGD).<p> METHODS: From January 2015 to January 2021, the researchers collected postoperative pathological diagnoses cases of AOXGD from Xi'an People's Hospital(Xi'an Fourth Hospital), retrospectively analyzed clinical pathological data, and reviewed related literature. <p>RESULTS: A total of five AOXGD cases were collected, including three cases of adult-onset xanthogranuloma(AOX), one case of necrobiotic xanthogranuloma(NBX), and one case of Erdheim-Chester disease(ECD). The five patients were composed of middle-aged and elderly patients with local orbital lesions were treated. Microscopic examination revealed that the orbital skin and subcutaneous tissue had foam-like non-Langerhans histiocytes that exhibited diffused or nested infiltration. Immunohistochemistry revealed that the cells were positive for CD68, CD163, FXⅢa, lysozyme, negative for S100, CD1a, Langerin, HMB-45, MDM2. One ECD patient had orbital lesions as the first symptom and exhibited a combination of retroperitoneal lesions, right atrium pseudotumor, and long bones, heart, kidney lesions. The diagnosis of AOXGD depends on clinical manifestations and pathological features. The treatment was based on subtypes and clinical manifestations to select corresponding strategies, which mainly include the administration of glucocorticoids, immunosuppressants, and surgical treatment. <p>CONCLUSION: AOXGD is relatively rare in clinical practice. AOXGD can manifest as a local orbital disease or an orbital disease combined with a systemic disease. In the clinical and pathological work of ophthalmology, the understanding and identification of this group of diseases should be strengthened, and correct diagnosis and standard treatment should be promoted.

Xantogranuloma juvenil del adulto con presentación única en el conducto auditivo externo, una entidad poco común y una localización infrecuente: reporte de caso / Juvenile xanthogranuloma in an adult with a single manifestation in the external auditory canal, an uncommon entity and unusual localization: Case report

La xantogranulomatosis juvenil es una patología infrecuente que se presenta predominantemente en la primera infancia, ya que los adultos pueden verse afectados con poca frecuencia. La manifestación cutánea se da en la mayoría de los casos como un nódulo rojo-amarillo indurado y solitario, que con frecuencia se presenta a nivel de cabeza y cuello, seguido del tronco, extremidades inferiores y superiores. Aunque infrecuentes, las manifestaciones extracutáneas pueden presentarse con principal compromiso oftalmológico (1). En el artículo se presenta el caso de un hombre de 42 años que consultó por la aparición de una lesión en el conducto auditivo externo derecho, con un aumento progresivo del tamaño asociado con otorrea serohemática intermitente e hipoacusia. El diagnóstico se realizó por medio de hallazgos clínicos, histopatológicos e inmunohistoquímicos. Se realizó escisión total de la lesión; posteriormente, el paciente presentó una evolución adecuada y mejoría de la sintomatología. Se presenta este caso por lo infrecuente de la entidad y por lo inusual de su localización.

Juvenile xanthogranulomatosis, an unusual pathology that occurs predominantly in early childhood, adults can be affected infrequently, the skin manifestation occurs in most cases, as a solitary, indurated red-yellow papule or nodule, with a highest frequency occurs at head and neck level, followed by the trunk and the lower and upper extremities. Extracutaneous manifestations are uncommon, however they can be present with principal ocular level involvement (1). We present the case of a 42-year-old man who consulted due to an appearance of a lesion in the right external auditory canal with a progressive increase in size associated with intermittent otorrhea and hearing loss. The diagnosis was made by clinical, histopathological and immunohistochemical findings. Excision of the entire lesion was performed, after which the patient presented adequate evolution and improvement of symptoms. This case is presented due to the infrequency of the entity and the unusual localization

Upper eyelid juvenile xanthogranuloma: a case report / Xantogranuloma juvenil em pálpebra superior: relato de caso

ABSTRACT Juvenile xanthogranuloma is a rare benign non-Langerhans cell histiocytosis. Clinical manifestation usually occurs up to the age of 2 years, with yellowish papules and variable clinical progression. Approximately 0.75% of patients had systemic involvement and 0.25%, ocular alterations. The purpose of this report is to describe a case of a preschool 2-year-old female patient, with nodules in the upper right eyelid, 0.5-cm wide, with well-defined edges, an uncertain date of onset, a stable growth for 6 months, with no inflammatory signs, pruritus, pain, bleeding, or other similar lesions in the body. No further changes were observed in the physical examination. Histopathological examination of the specimen showed a skin lesion with histiocytoid, spindle-shaped cells and xanthomized cells, inflammatory infiltrate and numerous Touton giant cells. The result was compatible with diagnosis of juvenile xanthogranuloma. Therefore, the importance of including juvenile xanthogranuloma in the differential diagnosis of eyelid lesions is emphasized, especially in children.

RESUMO O xantogranuloma juvenil é uma patologia histiocítica benigna rara. A manifestação clínica ocorre geralmente até os 2 anos de idade com pápulas amareladas e evolução clínica variável. Cerca de 0,75% dos pacientes apresentaram comprometimento sistêmico e 0,25%, comprometimento ocular. O objetivo deste relato é descrever o caso de uma pré-escolar de 2 anos do sexo feminino, com nodulação em pálpebra superior direita, 0,5cm de base e bordos bem definidos, data de início não estimada, mas crescimento estável há 6 meses, sem sinais flogísticos, prurido, dor, sangramentos ou outras lesões similares no corpo. Sem mais alterações ao exame físico. A análise histopatológica da peça evidenciou lesão cutânea com células histiocitoides, fusiformes e outras xantomizadas; infiltrado inflamatório de permeio e numerosas células gigantes do tipo Touton, resultado compatível com o diagnóstico de xantogranuloma juvenil. Assim, ressalta-se a importância da inclusão do xantogranuloma juvenil no diagnóstico diferencial de lesões palpebrais, especialmente em crianças.

Xantogranuloma múltiplo do adulto: relato de caso de tratamento exitoso com laser CO2 / Multiple adult xanthogranuloma: case report of successful treatment with CO2 Laser

O xantogranuloma múltiplo do adulto é uma apresentação mais rara e tardia do xantogranuloma juvenil, uma histiocitose de células não Langerhans. No adulto, normalmente, é uma lesão única, sendo a manifestação por múltiplas lesões infrequente e pouco descrita na literatura. Relatamos um caso de xantogranuloma múltiplo do adulto, com falha terapêutica à isotretinoína e ótima resposta ao tratamento com laser CO2 no modo cirúrgico

Multiple adult xanthogranuloma is a rare and late variant of Juvenile xanthogranuloma, a non-Langerhans cell histiocytosis. It usually corresponds to a single lesion in adults, and the manifestation of multiples lesions is uncommon. We report a case of multiple adult xanthogranuloma, with Isotretinoin therapy failure and optimal response to CO2 Laser treatment in the surgical mode.

Gammapatías monoclonales de significado clínico en reumatología / Monoclonal gammopathies with clinical significance in rheumatology

Las gammapatías monoclonales son un grupo amplio de enfermedades de células hematológicas con expresión clínica variable, con afectación sistémica o localizada. Muchos de estos trastornos simulan enfermedades reumáticas, y pueden presentarse previa- o posteriormente a la enfermedad de base, por lo cual dificultan su diagnóstico. El propósito de este estudio es comunicar los casos de cinco pacientes con manifestaciones clínicas de enfermedades reumáticas y diagnóstico final de enfermedades oncohematológicas. Se realizó un estudio descriptivo transversal mediante el análisis de historias clínicas de pacientes evaluados en el Servicio de Reumatología del Hospital José María Cullen de Santa Fe entre marzo del 2010 y junio del 2019. Se incluyó a cinco pacientes que fueron estudiados por sospecha de enfermedad reumatológica hasta llegar al diagnóstico final de gammapatía monoclonal. Cuatro pacientes presentaron mieloma múltiple manifestado como síndrome de Schnitzler; xantogranuloma del adulto y amiloidosis; aplastamientos vertebrales múltiples; falla renal aguda, respectivamente. El quinto paciente se presentó simulando una vasculitis sistémica con afectación multiorgánica y diagnóstico final de linfoma intravascular. Los pacientes fueron derivados al Servicio de Oncología y Hematología para su atención. A partir de la serie de casos analizados, se concluye que las manifestaciones reumáticas de las enfermedades oncohematológicas se deben tener presentes en el accionar diario para evitar la demora diagnóstica y los tratamientos innecesarios(AU)

Monoclonal gammapathies are a broad group of diseases from hematopoietic cells with variable clinical features and systemic or limited involvement. These entities could begin as a rheumatic disease, even previously to the diagnosis of MG. To describe five patients with rheumatic manifestations that lately were diagnosed as monoclonal gammapathies. We describe the more relevant features of five patients assisted in our rheumatology center. Four patients were diagnosed with multiple myeloma that begins as: 1) Schnitzler's syndrome, 2) Adult-onset xanthogranuloma and amyloidosis, 3) multiple vertebral fracture, 4) acute kidney failure. The 5th patient has a vasculitis-like syndrome due to an intravascular lymphoma. The rheumatic-like syndromes are infrequent but we should take into account this diagnosis in our clinical practice for rapid diagnostic and correct treatment(AU)

Necrobiotic Xanthogranuloma Coexists with Diffuse Normolipidemic Plane Xanthoma and Multiple Myeloma

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

Xantogranuloma juvenil múltiple, a propósito de un caso / Juvenile Multiple Xanthogranuloma, a case report

RESUMEN El xantogranuloma juvenil es la histiocitosis no Langenhans más frecuente en lactantes y niños menores de dos años, generalmente de carácter benigno y autolimitado. Clínicamente se presenta como neoformaciones únicas o múltiples papulonodulares, asintomáticas. Puede asociarse a neurofibromatosis tipo 1 y a leucemia mieloide crónica juvenil. Si bien el compromiso extracutáneo es infrecuente, siempre debe descartarse en lesiones múltiples. Presentamos el caso de un lactante de sexo masculino de 3 meses de edad con diagnóstico de xantogranuloma juvenil con lesiones múltiples y en progresión, por lo que se inicia tratamiento con corticoides tópicos de mediana potencia al que presenta buena respuesta.

ABSTRACT Juvenil xanthogranuloma is the most common non-Langenhans histiocytosis in children under two yeas old, usually benign and self-limited. Clinically presented as single or multiple neoformations like papules and nodules, asymptomatic. Can be associated with neurofibromatosis 1 and juvenile chronic myelogenous leukemia. Although the extracutaneous involvement is infrequent, it should always be discarded in multiple lesions. We present the case of a 3-month old infant with diagnosis of juvenile xanthogranuloma with multiple and increasing lesions, so topical corticoid treatment is initiated showing succesfull clinical response.

Necrobiotic xanthogranuloma associated with smoldering multiple myeloma: satisfactory response to cyclophosphamide, dexamethasone, and thalidomide

Abstract: Necrobiotic xanthogranuloma is a rare chronic condition, belonging to the group C non-Langerhans cell histiocytoses, which is relevant due to the possibility of extracutaneous involvement and association with systemic diseases, particularly hematologic malignancies. The case reported here was only diagnosed after nine years of evolution and was associated with plasma cell dyscrasia. After treatment with cyclophosphamide, dexamethasone, and thalidomide, there was a reduction of cutaneous lesions and serum levels of monoclonal protein.

Juvenile xanthogranuloma: a case report / Xantogranuloma juvenil: um caso clínico

ABSTRACT Objective: To report a rate case of Juvenile xanthogranuloma in a newborn infant. Case description: We present the case of a 31-week preterm newborn with multiple skin lesions whose clinical, histological and immunohistochemical findings allowed the diagnosis of juvenile xanthogranuloma. Currently, the patient has nine months-old, and there is no aggravation of the skin lesions or evidence of extra-cutaneous involvement, particularly ophthalmic. Comments: Juvenile xanthogranuloma is a rare and benign condition, included in the vast group of non-Langerhans histiocytosis. It typically occurs in the pediatric age and may have a neonatal presentation. It affects predominantly the skin, in the form of papules or yellow and/or erythematous nodules and could be asymptomatic, multiple or solitary. Extra-cutaneous involvement, is more common in toddlers and when multiple lesions are present. The eye is the most affected site. We highlight this clinical case by its presentation in the neonatal period and in the form of multiple lesions, which bestows an increased risk of extra-cutaneous involvement, although this has not yet been verified.

RESUMO Objetivo: Descrever um caso raro de xantogranuloma juvenil em recém-nascido. Descrição do caso: Apresentamos o caso de um recém-nascido pré-termo de 31 semanas com múltiplas lesões cutâneas cuja clínica, histologia e imuno-histoquímica permitiram o diagnóstico de xantogranuloma juvenil. Atualmente, com nove meses de idade, não apresenta agravamento das lesões nem evidência de envolvimento extracutâneo, nomeadamente oftálmico. Comentários: O xantogranuloma juvenil é uma patologia rara e benigna, pertencente ao vasto grupo das histiocitoses não Langerhans. Surge tipicamente em idade pediátrica, podendo ter apresentação neonatal. O envolvimento é predominantemente cutâneo sob a forma de pápulas ou nódulos de coloração amarela e/ou eritematosos, assintomáticos, solitários ou múltiplos. O envolvimento extracutâneo é mais frequente em crianças com menos de dois anos e com múltiplas lesões, sendo o olho o local mais afetado. Destacamos este caso clínico pela apresentação no período neonatal e sob a forma de múltiplas lesões, o que lhe confere risco acrescido de envolvimento extracutâneo, sem que, no entanto, tal se tenha verificado.

Infantile systemic juvenile xanthogranuloma case with massive liver infiltration

Infantile systemic juvenile xanthogranuloma (ISJXG) is an uncommon form of juvenile xanthogranuloma, a non-Langerhans cell proliferation of infancy and early childhood. In a small percentage of patients, the visceral involvement­most commonly to the central nervous system, liver, spleen, or lungs­may be associated with severe morbidity, and eventually fatal outcome. Here we describe the clinical and pathological findings of a 28-day-old girl with ISJXG who died with respiratory distress syndrome. She had few cutaneous lesions but massive liver and spleen infiltration; other affected organs were multiple lymph nodes, thoracic parasympathetic nodule, pleura, pancreas, and kidneys. Additional findings were mild pulmonary hypoplasia and bacteremia. Immunohistochemistry on fixed tissues is the standard for diagnosis. Immunophenotype cells express CD14, CD68, CD163, Factor XIIIa, Stabilin-1, and fascin; S100 was positive in less than 20% of the cases; CD1a and langerin were negative. No consistent cytogenetic or molecular genetic defect has been identified. This case demonstrates that the autopsy is a handy tool, because hepatic infiltration, which was not considered clinically, determined a restrictive respiratory impairment. In our opinion, this was the direct cause of death.

Primary Necrobiotic Xanthogranulomatous Sialadenitis with Submandibular Gland Localization without Skin Involvement

Necrobiotic xanthogranulomatous reaction is a multiorgan, non-Langerhans cell histiocytosis with an unknown etiology. Occurrence in the salivary gland is extremely rare. We recently identified a case of necrobiotic xanthogranulomatous sialadenitis in a 73-year-old Korean woman who presented with a painless palpable lesion in the chin. There was no accompanying cutaneous lesion. Partial resection and subsequent wide excision with neck dissection were performed. Pathological examination showed a severe inflammatory lesion that included foamy macrophages centrally admixed with neutrophils, eosinophils, lymphocytes, plasma cells, and scattered giant cells, as well as necrobiosis. During the 12-month postoperative period, no grossly remarkable change in size was noted. Necrobiotic xanthogranulomatous inflammation may be preceded by or combined with hematologic malignancy. Although rare, clinicians and radiologists should be aware that an adhesive necrobiotic xanthogranuloma in the salivary gland may present with a mass-like lesion. Further evaluation for hematologic disease and close follow-up are needed when a pathologic diagnosis is made.

Adult Xanthogranuloma: A Clinical, Histopathological, and Immunohistochemical Study of 19 Korean Cases / 대한피부과학회지

BACKGROUND: Juvenile xanthogranuloma is a benign, self-limited disorder that usually occurs in infants and young children. Xanthogranuloma is rare in adults, and therefore studies reporting adult xanthogranuloma are limited. OBJECTIVE: We investigated the clinical, histopathological, and immunohistochemical characteristics of adult xanthogranuloma. METHODS: In this study, we evaluated 20 lesions in 19 patients with adult xanthogranuloma. RESULTS: A male predominance was observed (male : female ratio 1.4 : 1), and the mean age of patients was 35.1±16.3 years (range 15∼66 years), with the peak incidence observed in patients in their 20s. Notably, 65.0% of the lesions developed on the head and neck. The nodular form was more common than the papular form of this condition. Histopathological examination revealed dense monomorphic histiocytic infiltration without lipidization and scattered eosinophils without multinuclear giant cells in 5 lesions (25.0%), foamy histiocytic infiltration with variations of completely developed Touton giant cells in 10 lesions (50.0%), and fibrohistiocytic proliferation in 3 lesions (15.0%). On immunohistochemical examination, histiocytes including giant cells showed positive test results with Factor XIIIa (90.9%), vimentin (100%), and CD68 (100%) and negative test results with CD1a, smooth muscle actin, and S-100 protein stains. Tumor excision was the treatment for choice. CONCLUSION: Adult xanthogranuloma most commonly manifested as the nodular form of the disease on the head and neck of men in their late 20s. Histopathologically, the classic Touton cell-rich stage was most commonly observed, followed by the stage of early predominantly mononuclear infiltration. This was a single-center, small-sized retrospective study; however, we expect the results of this study to contribute to a better understanding of adult xanthogranuloma.

Juvenile Xanthogranuloma: A Case Report and Review of the Literature / Xantogranuloma Juvenil: Informe de un Caso y Revisión de la Literatura

SUMMARY: Juvenile xanthogranuloma (JXG), is a benign histiocytic proliferation of uncertain histiogenesis which was first described by Adamson in 1905. It is a regressing disorder which occurs in children usually within first year of life. A child of ten months age reported to the Azeezia College of Dental Sciences and Research with a nodular swelling on the right side of the cheek and gave a history of swelling since the age of 5 months with gradual increase in size which was not associated with pain or itching. A provisional diagnosis of Haemangioma was made and excision biopsy of the lesion was done under general anaesthetia. Depending on the histopathologic and immunohistochemical findings a diagnosis of Juvenile Xanthogranuloma was made. The excisional biopsy site healed uneventfully with minimal scar formation. JXG is a benign fibrohistiocytic lesion and a type of granulomatous process. Pathogenesis of the lesion is unknown. It is generally considered to be a reactive lesion. Most common presentation is as solitary cutaneous lesion. Children are affected at a median age of 2 years with a male female ratio of 1.5:1. Classic histopathologic findings include Nodular to diffuse collection of histiocytes with finely vacuolated foamy cytoplasm and round to oval nuclei, Touton giant cells which are the cells with a central wreath of nuclei and peripheral rim of eosinophilic to vacuolated cytoplasm loaded with fat and Inflammatory infiltrate such as lymphocytes and eosinophils. JXG has to be clinically differentiated from Xanthoma, Molluscum contagiosum, Haemangioma and Neurofibroma. Mostly a self-limiting disease which spontaneously resolves. Conservative management is the treatment of choice. Excision may be done due to esthetic and diagnostic reasons. Recurrence is uncommon. JXG is a disease predominantly of early childhood, benign and self-healing.

RESUMEN: El xantogranuloma juvenil (JXG) es una proliferación histiocítica benigna de histiogénesis incierta que fue descrita por primera vez por Adamson en 1905. Es un trastorno regresivo que ocurre en los niños generalmente durante el primer año de vida. Un niño de diez meses de edad consultó al Colegio de Ciencias e Investigación Dental Azeezia por la presencia de hinchazón nodular en el lado derecho de la mejilla y un historial de hinchazón desde la edad de 5 meses con un aumento gradual en el tamaño que no estaba asociado con dolor o comezón. Se realizó un diagnóstico provisional de hemangioma y se realizó una biopsia de escisión de la lesión con GA. A partir de los hallazgos histopatológicos e inmunohistoquímicos, se realizó un diagnóstico de Xantogranuloma Juvenil. El sitio de la biopsia por escisión se curó sin incidentes con una formación de cicatriz mínima. JXG es una lesión fibrohistiocítica benigna y un tipo de proceso granulomatoso. La patogenia de la lesión es desconocida. Generalmente se considera que es una lesión reactiva. La presentación más común es como una lesión cutánea solitaria. Los niños se ven afectados a una edad media de 2 años con una proporción de hombres y mujeres de 1,5:1. Los hallazgos histopatológicos clásicos incluyen colección nodular a difusa de histiocitos con citoplasma espumoso finamente vacuolado y núcleos redondos a ovalados, células gigantes de Touton que son las células con una corona central de núcleos y margen periférico de citoplasma eosinófilo a vacuolado cargado con grasa e infiltrado inflamatorio como linfocitos y eosinófilos. JXG tiene que ser clínicamente diferenciado de Xanthoma, Molluscum contagiosum, Hemangioma y Neurofibroma. Es una enfermedad principalmente autolimitante que se resuelve espontáneamente. El tratamiento conservador es el tratamiento de elección. La escisión puede realizarse por razones estéticas y diagnósticas. La recurrencia es poco común. JXG es una enfermedad predominantemente de la primera infancia, benigna y autocurable.

Dermoscopy in the diagnosis of juvenile xanthogranuloma

Abstract: Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis. It manifests clinically as a red-yellow papule, usually showing spontaneous remission. The diagnosis is based on clinical and histological findings. We report the use of dermoscopy ("setting sun" pattern) as an adjuvant tool in the diagnosis of juvenile xanthogranuloma in a female patient presenting with a 2-month history of a pre-auricular papule.

Relevancia del xantogranuloma juvenil en pacientes con neurofibromatosis tipo 1: Revisión de un caso clínico / The relevance ofjuvenile xanthogranuloma in neurofibromatosis type i patients. Aclinical case

El xantogranuloma juvenil es una forma de histiocitosis de células no Langerhans que suele afectar a los niños y niñas dentro de los primeros años de vida. Su principal relevancia radica en la posible asociación a neurofibromatosis tipo 1, ya que su presentación conjunta conlleva un mayor riesgo de desarrollo de leucemia mielomonocítica crónica juvenil. Se presenta el caso de un lactante diagnosticado con neurofibromatosis tipo 1 en que se detectan lesiones múltiples compatibles con xantogranuloma juvenil.

Juvenile xanthogranuloma is a form of non-Langerhans cell histiocytosis which usually affects boys and girls in their early years. Its importance stemsfrom its possible association with neurofibromatosistype 1 asthe combined presentation brings a heightened risk of developing chronic juvenile myelomonocytic leukemia. We present the case of an infant diagnosed with neurofibromatosistype I who was found to have multiple lesions compatible with the diagnosis of juvenile xanthogranuloma.

A case of cutaneous tuberculosis presenting with generalized xanthogranulomas

@#<p>A 33-year-old male had a 22-year history of generalized xanthogranulomas but had a normal lipid profile. He also developed ulcerating plaques on his arm and back that were consistent with cutaneous tuberculosis on histopathologic and polymerase chain reaction PCR studies. In a normolipemic patient with generalized xanthogranulomas, a reactive granulomatous response of histiocytes to infection is explored since these share a common CD 14+ precursor with the macrophages that are vital in tuberculosis.</p>

Giant Sellar Xanthogranuloma after Surgical Treatment of Symptomatic Rathke's Cleft Cyst

Extremely massive sellar xanthogranuloma (XG) are rare, and the surgical outcome and prognosis are not well known. XG remain unknown whether they are derived from Rathke's cleft cysts (RCCs) or craniopharyngiomas (CPs) following extensive inflammation and metaplasia, to the point that no epithelium is readily identifiable. These lesions usually tend to occur in younger patients (mean 28.3 years), have a smaller diameter, and remain primarily intrasellar region with infrequent calcification. This 36-year-old man presented our hospital with visual deterioration. At the time of visit, there were no neurological problems other than visual field defect and hormonal disorder. He visited our hospital in 2007 due to headache and decreased vision, and underwent transphenoid surgery for pituitary RCC. Since then, he has received treatment at our hospital for postoperative hormonal disorders. Through preoperative imaging study, the author suspected CP and underwent surgery. During the operation, the adhesion of the tumor to the surrounding major neurovascular structures was severe in the naked eyes, but the tumor could be removed more easily than expected. The postoperative histological findings were confirmed as XG. The postoperative course was uneventful. Compared to the previous literature, this case is a case where the size of XG is very large in a sellar region and it can be proved that it originated from the RCC. And regular follow-up is necessary to confirm the prognosis after surgery.

Recurrent Abdominal Wall Inflammation After Resection of Urachal Xanthogranuloma

We report a very rare case of urachal xanthogranuloma. In this case, the inflammation recurred after 9 months even though it was removed by surgical resection. A 43-year-old woman presented with low abdominal walnut sized round mass, mild abdominal pain, and no specific voiding symptoms. Through radiologic evaluation with computed tomography (CT), we observed an infiltration in surrounding tissues of urachal mass from anteriosuperior aspect of bladder until umbilicus, so urachus inflammation as well as malignant tumor of urachus origin was suspected. Urachal mass excision and partial cystectomy were conducted with low midline incision and it was reported pathologically as xanthogranulomatous inflammation. Afterwards the patient lived without inconvenience but after 9 months she returned because of pain and palpable nodule in the right lower abdominal area. Despite taking antibiotics, there was no improvement and abscess formation was observed in CT. Finally, its symptoms improved after abscess incision and drainage.