{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T01:36:03Z","timestamp":1760060163887,"version":"build-2065373602"},"reference-count":26,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2025,8,5]],"date-time":"2025-08-05T00:00:00Z","timestamp":1754352000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Pusan National University Yangsan Hospital"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Introduction: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a very rare subtype of cutaneous T-cell lymphoma. It is characterized by the neoplastic infiltration of subcutaneous adipose tissue. Its clinical presentation, including subcutaneous nodules, fever, and systemic symptoms, often mimics inflammatory panniculitis, making diagnosis difficult. Case Presentation: This case report describes a 14-year-old female presenting with fever, limb pain, swelling, and subcutaneous nodules, who was ultimately diagnosed with SPTCL via punch biopsy and BIOMED-2 clonality assays, confirming positive T-cell receptor-\u03b3 chain gene rearrangement. Positron emission tomography\u2013computed tomography revealed diffuse subcutaneous involvement across multiple body regions. Methylprednisolone and cyclosporine A treatment rapidly resolved her symptoms, with laboratory parameters, including ferritin and inflammatory markers, showing significant improvement. Next-generation sequencing identified a heterozygous C9 gene mutation (c.346C&gt;T, p.Arg116Ter), adding a novel genetic dimension to the case. Following a tapered discontinuation of immunosuppressive therapy, the patient achieved sustained remission without relapse for over 1 year. Conclusions: We report a case of adolescent SPTCL treated with immunosuppressive therapy and suggest that immunosuppressive therapy should be considered before chemotherapy in pediatric patients with SPTCL but without HLH.<\/jats:p>","DOI":"10.3390\/children12081029","type":"journal-article","created":{"date-parts":[[2025,8,5]],"date-time":"2025-08-05T08:46:55Z","timestamp":1754383615000},"page":"1029","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A Case of Pediatric Subcutaneous Panniculitis-like T-Cell Lymphoma Successfully Treated with Immunosuppressive Therapy"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2798-863X","authenticated-orcid":false,"given":"Min Chong","family":"Kim","sequence":"first","affiliation":[{"name":"Department of Pathology, Yeungnam University Medical Center, Daegu 42415, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dong Hoon","family":"Shin","sequence":"additional","affiliation":[{"name":"Department of Dermatology, College of Medicine, Yeungnam University, Daegu 42415, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6822-1051","authenticated-orcid":false,"given":"Jae Min","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pusan National University School of Medicine, Pusan National University Children\u2019s Hospital, Yangsan 50612, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,8,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1186\/s13023-022-02575-4","article-title":"A retrospective study of 18 children with subcutaneous panniculitis-like T-cell lymphoma: Multidrug combination chemotherapy or immunomodulatory therapy?","volume":"17","author":"Duan","year":"2022","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"5064","DOI":"10.1182\/blood-2008-10-184168","article-title":"Cutaneous lymphoma incidence patterns in the United States: A population-based study of 3884 cases","volume":"113","author":"Bradford","year":"2009","journal-title":"Blood"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1097\/00000478-199101000-00002","article-title":"T-cell lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemophagocytic syndrome","volume":"15","author":"Gonzalez","year":"1991","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1703","DOI":"10.1182\/blood-2018-11-881268","article-title":"The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas","volume":"133","author":"Willemze","year":"2019","journal-title":"Blood"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"3768","DOI":"10.1182\/blood-2004-09-3502","article-title":"WHO-EORTC classification for cutaneous lymphomas","volume":"105","author":"Willemze","year":"2005","journal-title":"Blood"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"4575","DOI":"10.1007\/s10238-023-01210-1","article-title":"Subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic lymphohistiocytosis: A systematic review of 63 patients reported in the literature","volume":"23","author":"Ou","year":"2023","journal-title":"Clin. Exp. Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1097\/MPH.0000000000001434","article-title":"Pediatric Subcutaneous Panniculitis-like T-Cell Lymphoma with Hemophagocytosis Showing Complete Resolution with the BFM90 Protocol: Case Report and Review of Literature","volume":"41","author":"Singh","year":"2019","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1916","DOI":"10.1002\/pbc.24638","article-title":"Pediatric subcutaneous panniculitis-like T-cell lymphoma with features of hemophagocytic syndrome","volume":"60","author":"Merritt","year":"2013","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"5741","DOI":"10.1007\/s00277-024-05953-z","article-title":"Treatment outcomes between cyclosporin and chemotherapy in adult subcutaneous panniculitis-like T-cell lymphoma: A report from nation-wide Thai lymphoma study group registry","volume":"103","author":"Tirachotikul","year":"2024","journal-title":"Ann. Hematol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1165","DOI":"10.1002\/pbc.24462","article-title":"Subcutaneous panniculitis-like T-cell lymphoma in the pediatric age group: A lymphoma of low malignant potential","volume":"60","author":"Huppmann","year":"2013","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2025","DOI":"10.1002\/pbc.25626","article-title":"Subcutaneous panniculitis-like T-cell lymphoma: Pediatric case series demonstrating heterogeneous presentation and option for watchful waiting","volume":"62","author":"Johnston","year":"2015","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"526","DOI":"10.1111\/pde.12452","article-title":"Subcutaneous Panniculitis-Like T-Cell Lymphoma in Children: A Review of the Literature","volume":"32","author":"Hu","year":"2015","journal-title":"Pediatr. Dermatol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"528","DOI":"10.3109\/08880018.2014.896062","article-title":"Pediatric subcutaneous panniculitis-like T-cell lymphoma with favorable result by immunosuppressive therapy: A report of two cases","volume":"31","author":"Nagai","year":"2014","journal-title":"Pediatr. Hematol. Oncol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"e576","DOI":"10.1111\/ajd.13684","article-title":"Subcutaneous panniculitis-like T-cell lymphoma in a 14-year-old female homozygous for HAVCR2 mutation","volume":"62","author":"Frederiks","year":"2021","journal-title":"Australas. J. Dermatol."},{"key":"ref_15","first-page":"1058","article-title":"HAVCR2 mutations are associated with severe hemophagocytic syndrome in subcutaneous panniculitis-like T-cell lymphoma","volume":"135","author":"Sonigo","year":"2020","journal-title":"Blood"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1182\/blood-2007-04-087288","article-title":"Subcutaneous panniculitis-like T-cell lymphoma: Definition, classification, and prognostic factors: An EORTC Cutaneous Lymphoma Group Study of 83 cases","volume":"111","author":"Willemze","year":"2008","journal-title":"Blood"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"100512","DOI":"10.1016\/j.modpat.2024.100512","article-title":"Fifth Edition of the World Health Organization Classification of Tumors of the Hematopoietic and Lymphoid Tissues: Mature T-Cell, NK-Cell, and Stroma-Derived Neoplasms of Lymphoid Tissues","volume":"37","author":"Miranda","year":"2024","journal-title":"Mod. Pathol."},{"doi-asserted-by":"crossref","unstructured":"Zhou, H., Xie, F., Zhou, L., and Liu, L. (2025). Pediatric subcutaneous panniculitis-like T-cell lymphoma: A case report and review of the literature. Pediatr. Radiol., advance online publication.","key":"ref_18","DOI":"10.1007\/s00247-025-06283-6"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"312","DOI":"10.4143\/crt.2014.46.3.312","article-title":"Cyclosporine A as a Primary Treatment for Panniculitis-like T Cell Lymphoma: A Case with a Long-Term Remission","volume":"46","author":"Lee","year":"2014","journal-title":"Cancer Res. Treat. Off. J. Korean Cancer Assoc."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"358","DOI":"10.2340\/00015555-2543","article-title":"Subcutaneous Panniculitis-like T-cell Lymphoma: Immunosuppressive Drugs Induce Better Response than Polychemotherapy","volume":"97","author":"Michonneau","year":"2017","journal-title":"Acta Derm. Venereol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1111\/ajco.13787","article-title":"The pathophysiology and current treatments for the subcutaneous panniculitis-like T cell lymphoma: An updated review","volume":"19","author":"Lin","year":"2023","journal-title":"Asia Pac. J. Clin. Oncol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1007\/s00439-002-0870-8","article-title":"Founder effect of the C9 R95X mutation in Orientals","volume":"112","author":"Khajoee","year":"2003","journal-title":"Hum. Genet."},{"key":"ref_23","first-page":"144","article-title":"High Incidence of Complement C9 Deficiency in Koreans","volume":"35","author":"Kang","year":"2005","journal-title":"Ann. Clin. Lab. Sci."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"2395","DOI":"10.1001\/archinte.1990.00390220127027","article-title":"Recurrent meningitis in a patient with congenital deficiency of the C9 component of complement. First case of C9 deficiency in Europe","volume":"150","author":"Zoppi","year":"1990","journal-title":"Arch. Intern. Med."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"276","DOI":"10.3109\/s101650070017","article-title":"Beh\u00e7et\u2019s disease associated with complement component 9 (C9) deficiency","volume":"10","author":"Horiuchi","year":"2000","journal-title":"Mod. Rheumatol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1136\/ard.46.2.153","article-title":"SLE-like and sicca symptoms in late component (C9) complement deficiency","volume":"46","author":"Sugimoto","year":"1987","journal-title":"Ann. Rheum. Dis."}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/12\/8\/1029\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T18:23:19Z","timestamp":1760034199000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/12\/8\/1029"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,8,5]]},"references-count":26,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2025,8]]}},"alternative-id":["children12081029"],"URL":"https:\/\/doi.org\/10.3390\/children12081029","relation":{},"ISSN":["2227-9067"],"issn-type":[{"type":"electronic","value":"2227-9067"}],"subject":[],"published":{"date-parts":[[2025,8,5]]}}}