Tohoku J. Exp. Med., 2021 September, 255(1)

The Expression of Insulin-Like Growth Factor 2 Messenger RNA-Binding Protein 3 in Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma

Konomi Yashige,1,2 Tatsuki R Kataoka,1,3 Yosuke Yamada,1 Hirona Maeda,1 Marina Oji-Tsujimura,1 Tetsurou Yamamoto,2 Yusuke Takei,1,4 Koki Moriyoshi,1,5 Kazuo Ono,6 Yo Kaku,7,8 Tetsuya Tabata,9 Ichiro Murakami,10 Hirokazu Nakamine11 and Hironori Haga1

1Department of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Kyoto, Japan
2Department of Diagnostic Pathology, Hyogo Prefectural Amagasaki General Medical Center, Amagasaki, Hyogo, Japan
3Department of Pathology, Iwate Medical University, Shiwa-gun, Iwate, Japan
4Department of Diagnostic Pathology, Saiseikai Noe Hospital, Osaka, Osaka, Japan
5Department of Diagnostic Pathology, Kyoto Medical Center, Kyoto, Kyoto, Japan
6Department of Pathology, Japan Red Cross Society Wakayama Medical Center, Wakayama, Wakayama, Japan
7Deparment of Dermatology, Kyoto University Hospital, Kyoto, Kyoto, Japan
8Department of Dermatology, Faculty of Medicine, Kagawa University, Miki, Kagawa, Japan
9Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Okayama, Japan
10Department of Pathology, Kochi Medical School, Kochi University, Nangoku, Kochi, Japan
11Department of Pathology, The Japan Baptist Hospital, Kyoto, Kyoto, Japan

Langerhans cell neoplasms, which include Langerhans cell histiocytosis and Langerhans cell sarcoma, are tumors that originate from dendritic cells. Langerhans cell sarcoma is defined as a high-grade neoplasm with overtly malignant cytological features and the Langerhans cell-like phenotype, and generally has a poorer prognosis and more aggressive phenotype than Langerhans cell histiocytosis. Insulin-like growth factor 2 messenger RNA-binding protein 3 (IGF2BP3 or IMP3) is an oncofetal protein that is expressed in various cancer types; its expression is often associated with a poor prognosis and aggressive phenotype. Here, we used immunohistochemistry to evaluate IGF2BP3 expression in Langerhans cell neoplasms. IGF2BP3 expression was scored as negative (< 1%) or positive (≥ 1%) by immunohistochemistry. All 4 patients with Langerhans cell sarcoma (100%) and 6 of 22 pediatric (age < 18 years) patients with Langerhans cell histiocytosis (27.3%) had positive results for IGF2BP3; however, 16 of 22 pediatric patients with Langerhans cell histiocytosis (72.7%) and all 15 adult (age ≥ 18 years) patients with Langerhans cell histiocytosis (100%) had a negative result. Among patients with Langerhans cell histiocytosis, IGF2BP3 expression was independent of sex, location, prognosis, and BRAF V600E staining results. Taken together, these results indicate that IGF2BP3 expression may be a helpful marker for distinguishing Langerhans cell sarcoma from Langerhans cell histiocytosis in adult patients.

Key words —— IGF2BP3; immunohistochemistry; Langerhans cell histiocytosis; Langerhans cell neoplasm; Langerhans cell sarcoma

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Tohoku J. Exp. Med 2021, 255, 27-31.

Correspondence: Tatsuki R. Kataoka, M.D., Ph.D., Department of Pathology, Iwate Medical University, 1-1-1 Idaidori, Yahaba-cho, Shiwa-gun, Iwate 028-3694, Japan.

e-mail: trkata@iwate-med.ac.jp; trkataoka@yahoo.co.jp