ESHAP therapy effective in a patient with Langerhans cell sarcoma

Abstract

We describe the rare case of a 53-year-old woman with systemic involvement of Langerhans cell sarcoma (LCS) who had undergone living-related liver transplantation. We chose the CHOP regimen as first-line chemotherapy, and clinical improvement of LCS was obtained. Intensive care was necessary due to the systemic involvement of LCS and severe infectious diseases. After the third cycle of CHOP therapy, however, disease progression was observed, and we administrated a modified ESHAP regimen (etoposide, carboplatin, cytarabine, methylprednisolone) as second-line therapy. A marked response was obtained after four cycles of this combination chemotherapy. Modified ESHAP may be a very effective combination chemotherapy regimen for LCS.

Langerhans Cell Sarcoma in the Cervical Lymph Node: A Case Report and Literature Review

“…The etiology and pathogenesis of LCS, although not fully understood, is reported to be related to immune abnormalities [1]. In addition, LCS occurring after organ transplantation has been reported, such as following liver transplantation [5,6], likely related to long-term immunosuppression. Recently, LCS arising from other tumors, especially B cell leukemia, has been reported [7,8,9].…”

“…Anthracycline-based chemotherapy, such as CHOP, were effective in some cases while the same treatment failed in other cases. Yoshimi et al [6] proposed a modified ESHAP program as salvage therapy if CHOP regimen fails. Uchida et al [14] reported a case in which MAID regimen successfully treated LCS emanating from the upper arm cutaneous tissue.…”

Langerhans cell sarcoma: Response to radiotherapy

“…Interestingly, a second case of a LCS patient with a transplanted liver was published in 2008. 7 It appears that LCS occurs in a wide age range, though mainly in the third and fourth decades of life. Although the most commonly affected organs are the lymph nodes and skin, multiorgan involvement -which occurred in the case we present here -is also known to be characteristic.…”

“…Chemotherapy has been used for the treatment of metastatic disease. Classical hematological schemes, such as CHOP and ESHAP, have been administered, 7 and other drug combinations with Adriamycin plus Ifosfamide, such as MAID (mesna, doxorubicin, ifosfamide, dacarbazine) have also been tested. 9,11,12 Nevertheless, the best option for systemic treatment of LCS has not yet been established.…”

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

“…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).…”