The patient was treated with a modified E-CHOP regimen consisting of etoposide (0.1 g/m2, days 1–3), cyclophosphamide (750 mg/m2, day 1), vindesine (4 mg/m2, day 1), and dexamethasone (10 mg/kg/day, days 1–5). A month later, abdominal computed tomography revealed that the hypogastric lymph nodes and nodules had significantly decreased in size. Following these results, the patient was given a total of 6 E-CHOP treatment cycles and is currently receiving follow-up care.
Langerhans cells are dendritic cells that localize to the skin and mucosa and were first described by Paul Langerhans in 1868. LCS is extremely rare and has a dismal overcome even with intensive chemotherapy. It is generally considered to be a malignant variant of histiocytosis that can develop de novo or from antecedent LCH. According to the 2008 WHO tumor classification, dendritic cell-derived tumors can be classified as LCH, LCS, finger-like dendritic cell sarcoma, follicular dendritic cell sarcoma, or declassified dendritic cell sarcoma .
The diagnostic criteria for LCS include malignant cytological features—such as atypia, hyperchromatic nuclei, prominent nucleoli, and frequent mitotic figures—and the expression of typical immunophenotype markers, including CD1a, S100, and langerin (CD207). They can also exhibit CD68 and weak lysozyme expression. The appearance of Birbeck granules characteristic of Langerhans cells can also aid differential diagnosis; however, these granules can be damaged during tissue processing prior to microscopic analysis. It should also be noted that the genetic mutations in the Langerhans cell-specific marker langerin are associated with a lack of Birbeck granules and can also complicate diagnosis.[6,7] Moreover, CD31 expression has been attributed to an enhanced migratory capacity. Despite a similar cellular phenotype, LCH cells exhibit lower mitotic rates and cytologically benign nuclei that allow for its discrimination from malignant LCS.
LCS is typically associated with multiorgan involvement, including the skin, lymph nodes, lungs, bone, liver, spleen, and other soft tissues. The disease shows no gender preference and patients vary greatly in age—from 11 months to 81 years based on the reviewed literature.[5,9] Because of its rarity, an optimal treatment strategy for LCS has not been established, owing to its rarity; however, surgical resection appears to be a good option for localized lesions or confined nodal disease. For instance, Çalli et al reported a case with axillary lymph node involvement treated by curative mass resection. The patient did not receive any other adjuvant therapy and had no evidence of recurrence at annual follow-up. In addition, radiotherapy may be effective in treating minimally invasive LCS lesions, as Nakayama et al reported on a patient with a localized tumor in the cervical lymph node treated with radiotherapy alone that displayed no signs of recurrence for 45 months.
The successful treatment of advanced LCS with multiple-organ involvement is feasible with a variety of chemotherapeutic regimens. Systemic combination chemotherapy, such as the CHOP or CHOP-like regimens, may be helpful in some cases.[12–14] Yoshimi et al used the ESHAP (etoposide, carboplatin, cytarabine, methylprednisolone) regimen to treat a case refractory to the CHOP regimen, and observed a remarkable improvement. Thus, they supported use of the ESHAP regimen as a treatment for refractory disease. Comparatively, Keklik et al treated a patient with the 2-chlorodeoxyadenosine (2-CDA) regimen but, despite an initial improvement, the disease progressed after the fourth cycle of therapy. They subsequently administered 2 cycles of ESHAP as a second-line therapy; however, the patient developed advanced disease and died. Nevertheless, current data indicate that the ESHAP regimen may be partially effective in treating relapsed patients.
In conclusion, our case supports E-CHOP as an effective first-line therapy to treat LCS cases that develop from antecedent LCH and display a typical immunophenotype with S100, CD68, CD1a, and langerin expression; but the mechanism is unclear. Further data on clinical outcomes are necessary to establish the optimal treatment strategy for LCS.
WY drafted the manuscript. W-YC made the clinical diagnosis. T-XY made the clinical diagnosis, LWN and J-PL collected the data and helped draft the manuscript.
Formal analysis: Wu Yi.
Methodology: Wan-yuan Chen, Xin tian Yang.
Supervision: Liang wen Na.
Writing – original draft: Wu Yi.
Writing – review & editing: Ping Jian Lan.
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Keywords:Copyright © 2019 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.
chemotherapy; langerhans cell sarcoma; surgical resection