# Japanese clinical practice guidelines for vascular tumors, vascular malformations, lymphatic malformations, and Lymphangiomatosis 2022

**Authors:** Yoshiaki Kinoshita, Kosuke Ishikawa, Sadanori Akita, Katsuyoshi Koh, Satoru Sasaki, Masatoshi Jinnin, Hidefumi Mimura, Keigo Osuga, Michio Ozeki, Michiko Nagahama, Akihiro Fujino, Yoko Aoki, Akiko Asai, Noriko Aramaki‐Hattori, Ryohei Ishiura, Masanori Inoue, Yuki Iwashina, Takafumi Ohshiro, Keiko Ogawa, Mine Ozaki, Junko Ochi, Shiro Onozawa, Motoi Kato, Takahide Kaneko, Tamihiro Kawakami, Akira Kitagawa, Masakazu Kurita, Yoshihiro Kuwano, Taro Kono, Shien Seike, Shinsuke Takagi, Nobuyuki Takakura, Takao Tachibana, Shuichi Tanoue, Kumiko Chuman, Hiroki Nakaoka, Yasuhiro Nakamura, Fumio Nagai, Yasunari Niimi, Shunsuke Nosaka, Taiki Nozaki, Tadashi Nomura, Kazuki Hashimoto, Ayato Hayashi, Satoshi Hirakawa, Takeshi Hirabayashi, Taizo Furukawa, Hiroshi Furukawa, Yumiko Hori, Takanobu Maekawa, Kentaro Matsuoka, Hideki Mori, Eiichi Morii, Akira Morimoto, Yuta Moriwaki, Shunsuke Yuzuriha, Naoaki Rikihisa, Munezumi Fujita, Yasuyuki Yamahana, Kyoichi Deie, Asami Tozawa, Daisuke Hasegawa, Akira Higashiyama, Daisuke Maeda, Sachiko Asayama, Yuhki Arai, Yohei Iwata, Mayu Uka, Hidehito Usui, Mizuki Uchiyama, Saori Endo, Hideki Endo, Rintaro Ono, Naoya Oshima, Toshihiro Otsuka, Kuniaki Ohara, Shinji Kagami, Tomo Kakihara, Mototoshi Kato, Hiroki Kanamori, Masafumi Kamata, Ami Kawaguchi, Akiko Kishi, Hiroshi Kitagawa, Kiyokazu Kim, Tamotsu Kobayashi, Takeshi Saito, Yusuke Shikano, Shuichi Shimada, Keisuke Suzuki, Masataka Takahashi, Shohei Takami, Reiko Takeda, Aya Tanaka, Kaishu Tanaka, Satoru Tamura, Masashi Tamura, Kanako Danno, Kenji Tsuboi, Yuta Nakajima, Ryo Nakatani, Miho Noguchi, Akifumi Nozawa, Naoki Hashizume, Masashi Hayakawa, Daichi Hayashi, Takaya Fukumoto, Mamoru Honda, Norifumi Matsuda, Hayato Maruguchi, Naoki Murakami, Kiichiro Yaguchi, Shiho Yasue, Hiroki Yoshihara, Rika Yoshimatsu, Kiyohito Yamamoto, Shinji Wada

PMC · DOI: 10.1111/ped.70333 · Pediatrics International · 2026-03-25

## TL;DR

This paper presents updated clinical guidelines for treating vascular tumors and malformations in Japan, based on evidence-based medicine and systematic reviews.

## Contribution

The paper introduces new evidence-based guidelines for managing vascular anomalies in Japan, covering treatment efficacy and safety.

## Key findings

- The guidelines include 38 clinical questions addressing treatment efficacy and safety.
- Recommendations cover resection, sclerotherapy, drug therapy, and other treatments for vascular anomalies.
- The guidelines emphasize evidence-based approaches and systematic reviews for improved patient outcomes.

## Abstract

The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety, and systematizing treatment, employing evidence‐based medicine techniques and aimed at improvement of the outcomes. Clinical questions (CQs) were decided based on the important clinical issues. For document retrieval, key words for literature searches were set for each CQ and literature published from 1980 to the end of December 2020 was searched in PubMed and Japana Centra Revuo Medicina (JCRM). The strengths of evidence and recommendations acquired by systematic reviews were determined following the Medical Information Network Distribution Service (Minds) technique. A total of 38 CQs were used to compile recommendations and the subjects included efficacy of resection, sclerotherapy/embolization, drug therapy, laser therapy, radiotherapy, and other conservative treatment, differences in appropriate treatment due to the location of lesions and among symptoms, appropriate timing of treatment and tests, pathological diagnosis deciding the diagnosis, and causal genes of vascular anomalies. Thus, the Japanese Clinical Practice Guidelines for Vascular Tumors, Vascular Malformations, Lymphatic Malformations, and Lymphangiomatosis 2022 have been prepared as the evidence‐based guidelines for the management of vascular anomalies.

## Linked entities

- **Diseases:** vascular tumors (MONDO:0002095)

## Full-text entities

- **Genes:** VEGFA (vascular endothelial growth factor A) [NCBI Gene 7422] {aka L-VEGF, MVCD1, VEGF, VPF}, FGB (fibrinogen beta chain) [NCBI Gene 2244] {aka HEL-S-78p}, PIK3CB (phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit beta) [NCBI Gene 5291] {aka P110BETA, PI3K, PI3KBETA, PIK3C1}, MTOR (mechanistic target of rapamycin kinase) [NCBI Gene 2475] {aka FRAP, FRAP1, FRAP2, RAFT1, RAPT1, SKS}, MAPK1 (mitogen-activated protein kinase 1) [NCBI Gene 5594] {aka ERK, ERK-2, ERK2, ERT1, MAPK2, NS13}, VWF (von Willebrand factor) [NCBI Gene 7450] {aka F8VWF, VWD}, REN (renin) [NCBI Gene 5972] {aka ADTKD4, HNFJ2, RTD}, SST (somatostatin) [NCBI Gene 6750] {aka SMST, SST1}, PIK3CA (phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha) [NCBI Gene 5290] {aka CCM4, CLAPO, CLOVE, CWS5, HMH, MCAP}, AKT1 (AKT serine/threonine kinase 1) [NCBI Gene 207] {aka AKT, PKB, PKB-ALPHA, PRKBA, RAC, RAC-ALPHA}, SELP (selectin P) [NCBI Gene 6403] {aka CD62, CD62P, GMP140, GRMP, LECAM3, PADGEM}
- **Diseases:** torsion (MESH:D050723), hemangioendothelioma (MESH:D006390), thrombotic diathesis (MESH:D004198), PHACES syndrome (MESH:C537892), airway inflammation (MESH:D007249), blood clotting abnormalities (MESH:D013927), growth disorder (MESH:D006130), dysphagia (MESH:D003680), lymphangioma circumscriptum (MESH:D008202), abdominal lesions (MESH:D000008), hypoglycemia (MESH:D007003), thrombocytopenia (MESH:D013921), ocular muscle paralysis (MESH:D012133), patent foramen ovale (MESH:D054092), of III-IV (MESH:D006011), arrhythmia (MESH:D001145), gastroenteritis (MESH:D005759), vomiting (MESH:D014839), vascular lesion (MESH:D014652), erosion (MESH:D014077), skin (MESH:D012871), VM (MESH:C563977), lymphatic vessel dysplasia (MESH:D018190), type II lesions (MESH:D005776), Pulmonary fibrosis (MESH:D011658), edema (MESH:D004487), nutritional disturbance (MESH:D009748), streptococcal infection (MESH:D013290), Respiratory disturbances (MESH:D012131), anxiety (MESH:D001007), congenital heart disorder (MESH:D006330), Angiosarcoma (MESH:D006394), Tract (MESH:D014570), Bean syndrome (MESH:C536240), splenic lesions (MESH:D013158), pigmentation (MESH:D010859), mediastinal (MESH:D008480), malignant lymphoma (MESH:D008223), tumor lysis syndrome (MESH:D015275), liver disorder (MESH:D017093), embolism (MESH:D004617), anorexia (MESH:D000855), Proteus syndrome (MESH:D016715), parotid gland hemangiomas (MESH:D010309), acute pulmonary disorder (MESH:D012120), swelling of the lesion (MESH:D011654), lymphatic diseases (MESH:D008206), finger necrosis (MESH:D009336), Chylous pleural effusion (MESH:D010996), Respiratory Dysplastic/hypoplastic Disease (MESH:D012140), eczema (MESH:D004485), Yakes type IV lesions (MESH:C000631847), necrotizing enteritis (MESH:D004751), dental problems (MESH:D019973), asthma (MESH:D001249), impotence (MESH:D007172), pulmonary hypertension (MESH:D006976), facial (orbital) or oral lesions (MESH:D009916), stage (MESH:D062706), urticaria (MESH:D014581)
- **Chemicals:** Everolimus (MESH:D000068338), carteolol (MESH:D002354), sorafenib (MESH:D000077157), nitrogen (MESH:D009584), glucose (MESH:D005947), argon (MESH:D001128), sunitinib (MESH:D000077210), water (MESH:D014867), isobutyl cyanoacrylate (MESH:D002015), tetracycline (MESH:D013752), actinomycin D (MESH:D003609), CO2 (MESH:D002245), Steroids (MESH:D013256), cellulose (MESH:D002482), cyclophosphamide (MESH:D003520), Hemangiol (MESH:D011433), Tc-99m (MESH:D013667), talc (MESH:D013627), blood glucose (MESH:D001786), minocycline (MESH:D008911), acetylsalicylic acid (MESH:D001241), low-molecular-weight heparin (MESH:D006495), monoethanolamine oleate (MESH:C431086), oxycodone (MESH:D010098), cyanoacrylate (MESH:D003487), povidone-iodine (MESH:D011206), doxycycline (MESH:D004318), sisomicin (MESH:D012853), metronidazole (MESH:D008795), Medium-chain triglyceride (MESH:C000709826), miransertib (MESH:C000608559), dipyridamole (MESH:D004176), Sudan III (MESH:C033006), polyurethane (MESH:D011140), prednisolone (MESH:D011239), bleomycin (MESH:D001761), n-butyl-2-cyanoacrylate (MESH:D004659), lipid (MESH:D008055), fat (MESH:D005223), potassium (MESH:D011188), Timolol (MESH:D013999), ibuprofen (MESH:D007052), triglyceride (MESH:D014280), pingyangmycin (MESH:C025703), Vincristine (MESH:D014750), acetic acid (MESH:D019342), sodium fusidate (MESH:D005672), sildenafil (MESH:D000068677), heparin (MESH:D006493), Imiquimod (MESH:D000077271), alcohol (MESH:D000438), ticlopidine (MESH:D013988), potassium titanyl phosphate (MESH:C064806), Octreotide (MESH:D015282), catecholamine (MESH:D002395), imatinib (MESH:D000068877), ethanol (MESH:D000431), VICRYL (MESH:D011098), alexandrite (MESH:C112654), polyvinyl alcohol (MESH:D011142)
- **Species:** Homo sapiens (human, species) [taxon 9606]
- **Mutations:** start/stop
- **Cell lines:** CQ37 — Mus musculus (Mouse), Hybridoma (CVCL_XH92)

## Full text

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## Figures

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## References

809 references — full list in the complete paper: https://tomesphere.com/paper/PMC13017083/full.md

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Source: https://tomesphere.com/paper/PMC13017083