Tocilizumab for the treatment of paraneoplastic inflammatory syndrome associated with angiomatoid fibrous histiocytoma

Eberst, Lauriane; Cassier, Philippe; Brahmi, Mehdi; Tirode, Franck; Blay, Jean‐Yves

doi:10.1136/esmoopen-2020-000756

Cited by 6 publications

(13 citation statements)

References 5 publications

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“…31 In addition, another case with refractory AFH showed high plasma levels of IL-6; this case was treated with tocilizumab, an anti-IL-6 receptor monoclonal antibody. 32,33 We investigated the serum concentrations of IL-6, IL-18, MIG, and IP-10 before and after the operation and found that elevated serum IL-6 levels returned to normal after resection, similarly to the previous report. 29 IL-6 expression was observed both on tumor cells and few perivascular lymphocytes.…”

Section: Discussionsupporting

confidence: 78%

Systemic inflammation caused by an intracranial mesenchymal tumor with a EWSR1::CREM fusion presenting associated with IL‐6/STAT3 signaling

et al. 2022

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Pediatric neoplastic diseases account for about 10% of cases of fever of unknown origin (FUO), and most neoplastic disease cases are leukemia, lymphoma, and neuroblastoma. Brain tumors are rarely reported as the cause of FUO, although craniopharyngioma, metastatic brain tumor, and Castleman's disease have been reported. We report a case of intracranial mesenchymal tumor (IMT) with a FET:CREB fusion gene, which had inflammatory phenotype without neurological signs. A 10‐year‐old girl was admitted with a 2‐month history of intermittent fever and headache, whereas her past history as well as her family history lacked special events. Sepsis work‐up showed no pathological organism, and empirical antibiotic therapy was not effective. Bone marrow examination showed a negative result. Cerebrospinal fluid examination showed elevated protein as well as cell counts, and head magnaetic resonance imaging showed a hypervascular mass lesion with contrast enhancement in the left cerebellar hemisphere. The patient underwent tumor excision, which made the intermittent fever disappear. Pathological examinations resembled those of classic angiomatoid fibrous histiocytoma (AFH), but the morphological features were distinct from the AFH myxoid variant; then we performed break‐apart fluorescence in situ hybridization and confirmed the tumor harbored the rare EWSR1::CREM fusion gene (Ewing sarcoma breakpoint region 1 gene (EWSR1) and cAMP response element binding (CREB) family gene). Consequently, we diagnosed the condition as IMT with EWSR1::CREM fusion. Elevated serum concentration of interleukin 6 (IL‐6) was normalized after tumor resection, which suggested the fever could be caused by tumor‐derived IL‐6. This is the first case of IMT with EWSR1::CREM fusion that showed paraneoplastic symptoms associated with the IL‐6/signal transducer and activator of transcription 3 (STAT3) signaling pathway. Although brain tumors are rarely diagnosed as a responsible disease for FUO, they should be considered as a cause of unknown fever even in the absence of abnormal neurological findings.

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Section: Discussionsupporting

confidence: 78%

Systemic inflammation caused by an intracranial mesenchymal tumor with a EWSR1::CREM fusion presenting associated with IL‐6/STAT3 signaling

et al. 2022

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“…Whether the EWSR1-ATF1 transcript detected in this tumor can activate the IL-6 gene similarly is unknown. According to a study, an excessive production of IL-6 is detected in AFH with the EWSR1-ATF1 fusion gene, similar to this case [9]. IL-6 triggers STAT3 activation related to cell growth, antiapoptosis, and inflammatory reactions and acts as an autocrine growth factor in a malignant tumor, such as renal cell or prostate cancer [16,17].…”

Section: Discussionsupporting

confidence: 67%

“…According to other studies, tocilizumab treatment for patients with AFH associated with EWSR1-ATF1 translocations did not suppress tumor progression [9]. A difference in tocilizumab's tumor suppression between EWSR1-ATF1 and EWSR1-CREB1 translocations possibly exists.…”

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confidence: 86%

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Tocilizumab Controls Paraneoplastic Inflammatory Syndrome but Does Not Suppress Tumor Growth of Angiomatoid Fibrous Histiocytoma

Sabe

Inoue

Nagata

et al. 2021

Case Reports in Oncological Medicine

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Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that rarely metastasizes but lacks effective systemic therapy once it propagates. In some reports, high interleukin-6 (IL-6) production promotes tumor growth by autocrine stimulation and tocilizumab, an IL-6 receptor antagonist, can control AFH growth. Here, we present a case report on a patient with local recurrence and distant lymph node metastasis of AFH treated with tocilizumab. As a result, the inhibition of the IL-6 signaling pathway controlled paraneoplastic inflammatory syndrome (PIS); however, the local recurrent tumor progressed. This case implied that IL-6 is not necessarily the cause of tumor growth in AFH. Therefore, physicians should bear in mind that watchful observation is needed whether tocilizumab can control tumor progression despite the amelioration of PIS associated with the attenuated effect of IL-6 on AFH.

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“…Tocilizumab, an anti-IL-6 monoclonal antibody, has been used previously alongside systemic anticancer therapy in the treatment of paraneoplastic syndrome associated with AFH. 8 A child with unresectable metastatic AFH and PIS had a sustained disease response to tocilizumab as a systemic anticancer therapy, although the disease recurred once tocilizumab was stopped with a reresponse when it was restarted. 20 An adult patient with an undiagnosed AFH (thought to be a Baker cyst) presented with PIS and symptoms resolved with tocilizumab.…”

Section: Discussionmentioning

confidence: 99%

Evidence of Chemoresponsiveness in Unresectable Metastatic Angiomatoid Fibrous Histiocytoma

Corley

Pace

Barnacle

et al. 2023

Journal of Pediatric Hematology/Oncology

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Angiomatoid fibrous histiocytoma (AFH) is a soft tissue neoplasm of intermediate biological potential. Typically a slowgrowing tumor, it can recur locally. Rarely, it manifests as a soft tissue sarcoma capable of metastasis. When metastases are nonamenable to local therapy, it is believed uniformly fatal. We present 3 patients with metastatic AFH who demonstrated a sustained response to chemotherapy; including one who achieved complete remission with cryoablation. These cases reinforce the potential value of chemotherapy in some patients with unresectable metastatic AFH and provide the first case in the literature of cryoablation in AFH.

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Tocilizumab for the treatment of paraneoplastic inflammatory syndrome associated with angiomatoid fibrous histiocytoma

Cited by 6 publications

References 5 publications

Systemic inflammation caused by an intracranial mesenchymal tumor with a EWSR1::CREM fusion presenting associated with IL‐6/STAT3 signaling

Systemic inflammation caused by an intracranial mesenchymal tumor with a EWSR1::CREM fusion presenting associated with IL‐6/STAT3 signaling

Tocilizumab Controls Paraneoplastic Inflammatory Syndrome but Does Not Suppress Tumor Growth of Angiomatoid Fibrous Histiocytoma

Evidence of Chemoresponsiveness in Unresectable Metastatic Angiomatoid Fibrous Histiocytoma

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