Langerhans cell sarcoma: Response to radiotherapy

Lucas, Anna; Barca, Eva González; Servitje, Octavio; Abenoza, Lina; González, María Noel; Macià, Miquel; Lozano, A.

doi:10.1016/j.rpor.2010.05.001

Cited by 6 publications

(4 citation statements)

References 12 publications

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“…Efficacy was also reported for MAID (Mesna, doxorubicin, ifosfamide, dacarbazin) [153], or cladribin (2-CDA) followed by ESHAP (etoposide, carboplatinum, cytarabin, methylprednisolone) [107]. Radiotherapy has at least a palliative effect [125]. In case BRAF mutations are found, the use of BRAF inhibitors may become a potential strategy [201].…”

Section: Langerhans Cell Sarcoma (Lcs) Langerhans Cell Mhmentioning

confidence: 99%

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

2016

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Rare histiocytoses, also called non-Langerhans cell histiocytoses, include all proliferative disorders of histiocytes, macrophages and dendritic cells that are not classified as Langerhans cell histiocytosis (LCH) and do not belong to the hemophagocytic lymphohistiocytosis (HLH) group of diseases. Thus, the term includes numerous benign or malignant, localized or systemic, adult or pediatric diseases. The classification of the histiocytic disorders has been revised several times. Here, we follow the classification recently published by Jean Francois Emile and an international expert panel, defining subgroups of histiocytoses described as L-Group, C-Group, M-Group, R-Group, and H-Group, which stands for LCH-like, cutaneous or mucocutaneous, malignant, Rosai-Dorfman-Disease like and HLH like. Some of the diseases have an excellent prognosis after resection or even disappear spontanously, others progress rapidly, requiring intensive systemic therapies. The malignant non-Langerhans cell histiocytoses in general have a poor prognosis, here, complex chemotherapy protocols are usually applied, with inconsistant results. An interesting perspective in non-malignant rare histiocytoses might be small molecular inhibitors, in particular BRAF inhibitors, since BRAF mutations have been found in some subtypes of non-Langerhans cell histiocytoses. By prospective and retrospective collection of experiences in a new registry (the "International Rare Histiocytic Disorders Registry", IRHDR), knowledge about these rare diseases might hopefully be improved.

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Section: Langerhans Cell Sarcoma (Lcs) Langerhans Cell Mhmentioning

confidence: 99%

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

2016

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“…Lucas et al reported that radiation therapy also has the potential to shrink tumour lesions [35]. They found that LCS responds well to radiotherapy and believe that irradiation is a good option in selected cases with symptomatic or bulky lesions.…”

Section: Discussionmentioning

confidence: 99%

“…Inflammatory cell infiltration, consisting of lymphocytes and eosinophil gran- Table I. Summary of the reported cases of Langerhans cell sarcoma [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35] ulocytes, was also found, and isolated mitotic figures were identified ( Fig. 3A and 3B).…”

Section: Case Presentationmentioning

confidence: 95%

Langerhans cell sarcoma: a case report and review of the literature

Liu

Friesenbichler²,

Holzer³

et al. 2016

pjp

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We present the case of a 62-year-old male patient with a three-month history of pain in the left shoulder. Magnetic resonance imaging of the left scapula showed an osteo-destructive lesion. H&E stained sections revealed a Langerhans cell sarcoma, and immunohistochemistry was performed additionally; CD68, CD163, CD14, fascin, HLA-DR, lysozyme, S100 CD1a and langerin showed a positive reaction, while CD20, CD30, CD34, CD31, pan-cytokeratin, AE/1AE3, SMA, desmin, EMA, ERG, INI-1, CD21, CD4, PLAP, MPO and CD117c were negative. We suggested palliative treatment with chemotherapy and radiation. The patient refused any treatment and died 2 weeks later.

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“…The management of LCS has also differed widely ranging from single modality (generally in cases with localized involvement); to combination therapy including surgery and/or chemo-radiotherapy, albeit no consensus exists on the sequence of the choice of therapy. In one reported case, Lucas et al noted a relapse after 3 months of therapy with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) and thereafter successfully treated the cutaneous lesions and nodal masses with palliative radiotherapy [12]. Bone marrow transplant has also been attempted and correlates to good outcomes in carefully selected patients, generally in the relapsed setting [13].…”

Section: Discussionmentioning

confidence: 99%

Langerhans Cell Sarcoma: Treatment with Dose-adjusted EPOCH

Swaika¹,

Sood²,

Menke³

et al. 2015

Chemotherapy (Los Angel)

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Background: Langerhans cell sarcoma (LCS) is a rare malignancy with no specific defined treatment strategy. Chemotherapy is the preferred option in cases with systemic involvement, though no consensus exists on the preferred regimen. Case: A sixty-six year old male presented with advanced-stage LCS involving the bone marrow (BM) and spleen. Immunohistochemical analysis of the BM-sample confirmed the diagnosis of LCS. The patient was treated with chemotherapy utilizing dose-adjusted Etoposide, Prednisone, Vincristine, Cyclophosphamide and Doxorubicin (DA-EPOCH). The patient achieved complete remission and was able to tolerate the entire course (6 cycles) of chemotherapy with continued response at 10 months. Conclusion: Due to the rarity of the disease no standard treatment modality exists; most successful treatment options described are anecdotal reports. Due to the paucity of such data and inability to include enough patients on clinical trials, various combination chemotherapy options have been used for best clinical response. The unattractive response rate to CHOP (Cyclophosphamide, Doxorubicin, Vincristine and Prednisone) in previously described case series and the benefit of Etoposide in treating Langerhans cell histiocytosis and primitive sarcoma regimens along with an acceptable adverse effect profile, had us chose DA-EPOCH for our patient. We demonstrate that DA-EPOCH is a tolerable and an efficacious treatment regimen for LCS.

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Langerhans cell sarcoma: Response to radiotherapy

Cited by 6 publications

References 12 publications

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

Langerhans cell sarcoma: a case report and review of the literature

Langerhans Cell Sarcoma: Treatment with Dose-adjusted EPOCH

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