1/14. Development of acute lymphoblastic leukemia in a child after treatment of Langerhans cell histiocytosis: report of one case.Langerhans cell histiocytosis (LCH) is a group of poorly understood disorders. To our knowledge, LCH is a non-malignant disorder. The association of LCH with a secondary neoplasm has not been well assessed, however, a few cases have been reported. We report a case of LCH, a localized osteolytic lesion over metaphysis of left femur, who was treated with local curettage and chemotherapy with vincristine, prednisone and 6-mercaptopurine (6-MP) for eight months from end of 1991 to August, 1992. Six years later, she had acute lymphoblastic leukemia (ALL) in 1998. In review of current literature, only 5 cases of LCH, including our case, have preceded ALL. The possible association, a reactive process or a therapy-related process, between LCH and acute leukemia is still unclear at present and needs to be explored by more studies in the future.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/14. Concurrent Langerhans cell histiocytosis and myelodysplasia in children.BACKGROUND: Langerhans cell histiocytosis (LCH) is characterized by the proliferation of abnormal histiocytes (langerhans cells), whose origin as a reactive process or a neoplastic disorder is still poorly understood. Although LCH has been recorded as being associated with malignant neoplasms, concurrence of LCH and myelodysplastic syndrome has not been reported so far. PROCEDURE: We report on four children aged 23, 25, 26, and 53 months with multisystem LCH with organ dysfunction (bone marrow and liver) whose bone marrow pictures, taken at diagnosis, revealed the presence of myelodysplastic abnormalities (RA, RAEB, RAEB-t). RESULTS: We suggest that the commonly used expression of "organ dysfunction," which refers to clinical and functional alterations, could be explained by a myelodysplastic-like disorder. CONCLUSIONS: The contemporary presence of both events may provide a better understanding of the pathogenesis of LCH, especially in young children with multisystem disease and organ dysfunction, who are known to have a very poor outcome.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/14. CD79a( ) T-cell lymphoblastic lymphoma with coexisting Langerhans cell histiocytosis.Although there is a close association between Langerhans cell histiocytosis and malignant neoplasms, simultaneous occurrence of lymphoblastic lymphoma and Langerhans cell histiocytosis in the same lymph node is an extremely rare finding. Herein, we describe such a case in a 26-year-old woman who presented with progressive cervical lymphadenopathy. The lymphoma cells have an immature T-cell phenotype (terminal deoxynucleotidyl transferase( ), HLA-DR( ), CD34( ), CD38( ), and CD7( )) with expression of both CD3 and CD79a on immunohistochemical stain. The langerhans cells are present focally with the characteristic morphologic features and immunophenotype (CD1a( ) and S100( )). The significance of CD79a coexpression in T-cell lymphoblastic lymphoma and the association between lymphoblastic lymphoma and Langerhans cell histiocytosis are discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/14. Pulmonary Langerhans' cell histiocytosis (histiocytosis X) following metastasizing malignant melanoma.BACKGROUND: Pulmonary Langerhans' cell histiocytosis (histiocytosis X) is an uncommon, diffuse interstitial lung disease of unknown cause, mostly presenting in young smokers. association of pulmonary Langerhans' cell histiocytosis with a malignant neoplasm is rare. CASE DESCRIPTION AND RESULTS: We present and discuss the case of a 48-year-old man (ex-smoker) with metastasising malignant melanoma. A few months after chemotherapy and a modified Whipple procedure for retroduodenal metastasis of a malignant melanoma, computer tomographic scans revealed intrapulmonary "ring-shaped structures". Endobronchial biopsies and bronchioalveolar lavage showed no evidence of neoplasm or inflammation. Open-lung biopsy was performed and revealed pulmonary Langerhans' cell histiocytosis. CONCLUSION: To our knowledge this is the first reported case of pulmonary Langerhans' cell histiocytosis in association with malignant melanoma. Chemotherapy for malignant melanoma may be related to the development of pulmonary Langerhans' cell histiocytosis.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
5/14. Coexistence of localized Langerhans cell histiocytosis and cutaneous Rosai-Dorfman disease.Rosai-Dorfman disease (RDD; sinus histiocytosis with massive lymphadenopathy) and Langerhans cell histiocytosis (LCH) are two different yet pathogenetically related histiocytic disorders. While systemic and localized forms have been identified in both diseases, each has its own characteristic histological, immunohistochemical and ultrastructural profile. Rarely, either RDD or LCH can also occur in the context of certain malignant neoplasms. However, the coexistence of RDD and LCH has never been described. We report a case of cutaneous RDD in which a focus of LCH was found. Clinical and laboratory examinations revealed no evidence of extracutaneous involvement of RDD or LCH. We believe that this is the first report of such a coexistence, and the possible pathogenesis is discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/14. Langerhans cell histiocytosis of bone--a case report.Langerhans cell histiocytosis of bone causes bone destruction and frequent bone reactions that mimic benign and malignant neoplasms as well as osteomyelitis. The bony lesion of Langerhans cell histiocytosis consists of histiocytes with variable numbers of eosinophils, lymphocytes, and neutrophils. We present a rare case of Langerhans cell histiocytosis of bone, including imaging studies and pathology. In addition, we discuss the classification and pathology of Langerhans cell histiocytosis.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/14. Solitary plasmacytoma in the setting of Langerhans cell histiocytosis.Langerhans cell histiocytosis (LCH) is an intriguing disorder characterized by the accumulation of specialized dendritic cells called langerhans cells in several diverse tissues and body sites. It has been cited in numerous case reports to be associated with a wide variety of malignant neoplasms. Although many hypotheses have been suggested, the basis for such associations remains essentially unknown. We describe another association here that to our knowledge has not been reported thus far: a solitary plasmacytoma occurring at a site of previous involvement by LCH. This constitutes a new addition to the now fairly lengthy list of malignant neoplasms that have been reported to occur in the setting of LCH. The possible reasons for such an association are discussed along with a brief review of LCH.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
8/14. Mondini dysplasia and recurrent bacterial meningitis in a girl with relapsing Langerhans cell histiocytosis.We report a case of a girl with Langerhans cell histiocytosis (LCH) of multifocal bone disease, who developed recurrent bacterial meningitis and unilateral sensorineural hearing loss during the relapsing course of the disease. Mondini dysplasia, a congenital inner ear anomaly, was suspected by high resolution computed tomographic scan and the dysplasia with cerebrospinal fluid leakage was confirmed by surgery in the ipsilateral ear showing hearing loss. Although rare, congenital inner ear anomalies such as Mondini dysplasia should be kept in mind in pediatric patients with hearing impairment and/or recurrent bacterial meningitis during chemotherapy for various types of neoplasms including LCH.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/14. CD56/NCAM-positive langerhans cell sarcoma: a clinicopathologic study of 4 cases.This report concerns the clinicopathologic features of 4 patients with CD56/neural cell adhesion molecule (NCAM)-positive langerhans cell sarcoma (LCS). Three of the patients were elderly, between 59 and 62 years of age at presentation, and the other was 35 years old. The presenting symptoms included fever, bone pain, and weakness. The patients shared some clinical findings, such as multiorgan involvement of lymph nodes, skin, lung, bone marrow, and spleen. LCS carries a poor prognosis, and 3 of the patients died of the disease within several years of presentation despite multiagent chemotherapy and radiotherapy. Of special interest is that all of the cases showed CD56 expression on the tumor cells in addition to expression of CD1a, S100beta, and langerin, the presence of which suggests derivation from langerhans cells. For control, CD56 was also examined in 8 cases of Langerhans cell histiocytosis (LCH), a single-system unifocal or multifocal disease, and the results of staining of the tumor cells were negative. Our findings indicated that CD56 may be a clinically relevant biologic marker for predicting an intractable course of Langerhans cell neoplasms, although it is often difficult to draw a definite morphologically-based distinction between LCS and LCH.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/14. Lesions resembling Langerhans cell histiocytosis in association with other lymphoproliferative disorders: a reactive or neoplastic phenomenon?Langerhans cell histiocytosis (LCH) has been described in association with a variety of neoplasms preceding, after, or synchronous with the other tumor. In some cases, a neoplasm may arise as a complication of therapy for LCH, and in others, the association may be coincidental. Synchronous occurrence has been reported most commonly in association with malignant lymphoma in which discrete proliferations of langerhans cells (LCs) histologically indistinguishable from LCH are seen. In most cases, these LCs are closely related to or intermingling with the primary pathology. The nature of LCs in this context remains elusive with debate as to whether they represent a true clonal neoplasm or an exaggerated reactive phenomenon. The lack of evidence for LCH progression or disease elsewhere strongly supports the latter. We have encountered 5 examples of LCH-like proliferations occurring in the context of other lymphoproliferative disorders. These include 2 cases of mycosis fungoides and 1 of cutaneous B-cell pseudolymphoma, associations that to our knowledge have not been described before. Two patients were female, and the clonality of the LC proliferation was assessed using laser capture microdissection and the human androgen receptor. The results showed that the LCs forming discrete nodules in a case of cutaneous B-cell pseudolymphoma and a case of Hodgkin's lymphoma were polyclonal. This suggests that, at least in a proportion of cases, the aggregates of LCs occasionally identified within other lymphoproliferative lesions represent a reactive proliferation rather than a potentially aggressive second neoplasm.- - - - - - - - - - ranking = 4keywords = neoplasm (Clic here for more details about this article) |
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