Juvenile xanthogranuloma as a sequel to Langerhans cell histiocytosis: a report of three cases

Hoeger, Peter H.; Díaz, Carlos Guerrero; Malone, Marian; Pritchard, Jon; Harper, John

doi:10.1046/j.1365-2230.2001.00842.x

Cited by 46 publications

(28 citation statements)

References 14 publications

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“…Recently Hoeger et al [21]have reported 3 children with multisystem LCH who developed JXG 3–6 years after the initial manifestation of LCH, after many courses of chemotherapy. The authors hypothesized that the inflammatory reaction associated with LCH may precipitate the development of JXG.…”

Section: Commentmentioning

confidence: 99%

Langerhans Cell Histiocytosis and Juvenile Xanthogranuloma

Patrizi

Neri²,

Bianchi³

et al. 2004

Dermatology

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Background: Histiocytoses represent a large, puzzling group of diseases which may involve the skin and other organs. At present, juvenile xanthogranuloma is the disorder most often confused with Langerhans cell histiocytosis. A complex overlap exists between juvenile xanthogranuloma and Langerhans cell histiocytosis, with lesions showing clinical and/or pathological features of both disorders. Observations: We report 2 patients affected by Langerhans cell histiocytosis who, during chemotherapy, presented cutaneous lesions with clinical and histological features of juvenile xanthogranuloma. During the therapy, in both cases, histological examination of new biopsies revealed the presence of Touton giant cells in the dermis with a few histiocytic cells; immunohistochemical staining was negative for CD1a, and no Birbeck granules were seen by ultrastructural examination. Results and Conclusion: A possible explanation for the link between Langerhans cell histiocytosis and juvenile xanthogranuloma regards the lineage development and the relationships of histiocytes. We suggest that chemotherapy can modify the production of cytokines by influencing the conversion or ‘maturation’ of pathological cells into macrophages or xanthomatous cells and fusing them to form multinucleated giant Touton cells. In our opinion, the modification of the cutaneous lesions during chemotherapy in Langerhans cell histiocytosis patients, as observed in our cases, could be a favorable prognostic factor.

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Section: Commentmentioning

confidence: 99%

Langerhans Cell Histiocytosis and Juvenile Xanthogranuloma

Patrizi

Neri²,

Bianchi³

et al. 2004

Dermatology

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“…These include JXG lesions occurring after LCH [48,49] and concurrent LCH and Erdheim-Chester Disease (ECD) or ECD following LCH [50], However, there have been no previous reports, to our knowledge, of concurrent LCH and JXG-family of lesions occurring in the thymus. Our 2 cases are both from young patients.…”

Section: Discussionmentioning

confidence: 99%

Histologic Patterns of Thymic Involvement in Langerhans Cell Proliferations: A Clinicopathologic Study and Review of the Literature

et al. 2015

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Thymic involvement by Langerhans cell histiocytosis (LCH) has been described mainly in isolated case reports. A description of the histopathologic patterns of LCH proliferations in the thymus, together with therapeutic implications, has not, to our knowledge, been previously addressed. The pathology consultation files at Children's Hospital of Pittsburgh of the University of Pennsylvania Medical Center were reviewed for cases of thymic involvement by LCH. Relevant cases in the literature were also reviewed, and the histopathology and clinical course of those cases were collected. Nine consultation cases of thymic involvement were reviewed, together with 23 cases in the literature, which provided adequate pathologic description and ancillary confirmation (n = 32), revealing 4 distinct pathologic groups. Group 1 showed microscopic collection of hyperplastic LCH-like cells in incidental thymectomies of patients without LCH disease, requiring no further treatment (n = 7; 22%). Group 2 showed solitary and/or cystic LCH of the thymus with gland disruption, and at least 3 cases resolved without systemic therapy (n = 10; 31%). Group 3 showed more variable thymic involvement in multisystemic LCH disease, with either a medullary restricted pattern or more diffuse gland involvement, requiring adjuvant therapy and having a higher mortality rate (n = 13; 41%). Group 4 showed a mixed histiocytic lesion with a concurrent LCH and juvenile xanthogranuloma-like proliferation (n = 2; 6%). Thymic involvement in LCH is quite rare. Based on our cases and those in the literature, we propose 4 distinct pathologic groups of thymic involvement in Langerhans cell proliferations with relevance for diagnosis and treatment.

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“…1,2,5,6,9,11,12,14,16,19 Moreover, the segregation of LCH from non-LCH is called into question when case reports appear that exhibit overlapping features of both conditions. 8,10,13,15,26 Depending on the timing of the biopsy, LCH can cytomorphologically resemble non-LCH and vice versa. For example, early lesions of XG are often devoid of TGC and lipid-laden histiocytes, and can even contain a few LC, whereas LC in older lesions of LCH often accumulate lipids.…”

Section: Discussionmentioning

confidence: 99%

“…For example, early lesions of XG are often devoid of TGC and lipid-laden histiocytes, and can even contain a few LC, whereas LC in older lesions of LCH often accumulate lipids. 8,15 Moreover, Birbeck granules, pathognomonic organelles of LC, are only identified in approximately half of LC in LCH. 8 Lastly, the S-100 and CD1a immunohistochemical stains routinely expressed by LC are occasionally expressed in non-LCH histiocytoses.…”

Section: Discussionmentioning

confidence: 99%

“…[3][4][5][6][7][8][9][10] Furthermore, cases exist that exhibit concomitant lesions of LCH and non-LCH, and cases where LCH cells were converted into mature histiocytes with chemotherapy; thereby providing further evidence that an overlap exists both within and between the various histiocytoses. 5,6,[8][9][10][11][12][13][14][15][16] Herein we present a patient who does not easily fit into either the non-LCH or LCH category, but rather displayed clinical and histologic features that bridged both conditions. Through this novel case we review what is currently known about the various conditions classified under the non-LCH and LCH rubric.…”

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confidence: 99%

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