Iranian Journal of Pathology

Sclerosing Angiomatoid Nodular Transformation of Spleen (SANTS); Case Report of a 12-Year-Old Patient

Document Type : Case Reports

Authors

Farzaneh Ramezani 1
Sare Kamali 2
Ramin Mashoufi 3
Seyed Ali Ebrahimi 4
Maryam Soltan 5

1 Department of Pathology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

2 Department of Pathology, Faculty of Medicine, Hormozgan University of Medical Sciences, Bandar Abbas, Iran

3 Department of Radiology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

4 Department of Medical Sciences, Islamic Azad University, Mashhad, Iran.

5 Department of Pathology, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran

https://doi.org/10.30699/ijp.2025.2034980.3332
Abstract
Background: Sclerosing angiomatoid nodular transformation of the spleen (SANT) is a rare, benign vascular lesion predominantly described in adults. Pediatric cases are exceptionally uncommon and present a diagnostic challenge due to nonspecific clinical presentations and imaging findings.
Case Presentation: We report the case of a 12-year-old boy presenting with recurrent abdominal pain localized around the umbilicus, accompanied by intermittent nausea over a three-month period. Physical examination revealed mild tenderness without guarding. Laboratory findings were unremarkable. Abdominal ultrasound demonstrated a hypoechoic splenic lesion, further evaluated by multidetector computed tomography (MDCT), which revealed a heterogeneous hypodense mass in the spleen. The patient underwent partial laparoscopic splenectomy. Histopathological examination showed a nodular architecture with fibrous bands, capillary-like vascular channels lined by endothelial cells, and a lymphoplasmacytic infiltrate. Immunohistochemical staining was positive for CD31, CD34, and CD8, supporting the diagnosis of SANT.
Conclusion: Although benign, SANT can mimic more aggressive splenic pathologies. This case underscores the importance of considering SANT in the differential diagnosis of splenic masses in pediatric patients and highlights the role of histopathology and immunohistochemistry in achieving a definitive diagnosis.

Keywords

Splenic neoplasms
Sclerosing angiomatoid nodular transformation
Pediatric spleen lesion
Case report

Subjects

  1. Martel M, Cheuk W, Lombardi L, Lifschitz-Mercer B, Chan JK, Rosai J. Sclerosing angiomatoid nodular transformation (SANT): report of 25 cases of a distinctive benign splenic lesion. Am J Surg Pathol. 2004;28(10):1268-79. [DOI:10.1097/01.pas.0000138004.54274.d3] [PMID]
  2. Wang H, Hu B, Chen W, Ren J, Liu P, Shi H. Clinicopathological features of sclerosing angiomatoid nodular transformation of the spleen. Pathol Res Pract. 2021;224:153490. [DOI:10.1016/j.prp.2021.153490] [PMID]
  3. Yoshii H, Izumi H, Nomi M, Tajiri T, Mukai M, Nomura E, et al. Sclerosing Angiomatoid Nodular Transformation of the Spleen: A Case Report. Tokai J Exp Clin Med. 2020;45(4):236-42.
  4. Weinreb I, Bailey D, Battaglia D, Kennedy M, Perez-Ordonez B. CD30 and Epstein-Barr virus RNA expression in sclerosing angiomatoid nodular transformation of spleen. Virchows Arch. 2007;451(1): 73-9. [DOI:10.1007/s00428-007-0422-7] [PMID]
  5. Nagai Y, Hayama N, Kishimoto T, Furuya M, Takahashi Y, Otsuka M, et al. Predominance of IgG4+ plasma cells and CD68 positivity in sclerosing angiomatoid nodular transformation (SANT). Histopathology. 2008;53(4):495-8. [DOI:10.1111/j.1365-2559.2008.03118.x] [PMID]
  6. Trigui A, Toumi N, Bouzid A, Saumtally MS, Akrout A, Trabelsi J, et al. Sclerosing Angiomatoid Nodular Transformation of the Spleen: A Systematic Review. Current Surgery Reports. 2024:1-12. [DOI:10.1007/s40137-024-00402-2]
  7. Ong BS, Thomas R. Sclerosing Angiomatoid Nodular Transformation (SANT): A Rare Splenic Tumor and Unusual Cause of Anemia. Am J Case Rep. 2021; 22:e933598. [DOI:10.12659/AJCR.933598]
  8. He C, Yang W. Multifocal Sclerosing Angiomatoid Nodular Transformation of the Spleen With Splenomegaly and Thrombocytopenia. Balkan Med J. 2022;39(1):73-5. [DOI:10.4274/balkanmedj.galenos.2021.2021-8-14] [PMID] [PMCID]
  9. Liao J, Wang Z, Li Q, Gou Z, Bai X, Kang H, et al. CT and MRI features of sclerosing angiomatoid nodular transformation of the spleen: A report of 18 patients with pathologic correlation. Diagn Interv Imaging. 2021;102(6):389-96. [DOI:10.1016/j.diii.2021.01.003] [PMID]
  10. Raja F, Kumar V, Moll E, Hammad A, Ayub S. Sclerosing Angiomatoid Nodular Transformation of the Spleen: A Report of Rare Case and Literature Review. Cureus. 2023;15(9):e45422. [DOI:10.7759/cureus.45422]
  11. Cosme A, Tejada A, Bujanda L, Vaquero M, Elorza JL, Ojeda E, et al. Littoral-cell angioma of the spleen: a case report. World J Gastroenterol. 2007;13(48):6603-4. [DOI:10.3748/wjg.v13.i48.6603] [PMID] [PMCID]
  12. Giovagnoni A, Giorgi C, Goteri G. Tumours of the spleen. Cancer Imaging. 2005;5(1):73-7. [PMCID] [DOI:10.1102/1470-7330.2005.0002] [PMID]
  13. Lee H, Maeda K. Hamartoma of the spleen. Arch Pathol Lab Med. 2009;133(1):147-51. [DOI:10.5858/133.1.147] [PMID]
  14. Van den Eede S, Van de Voorde N, Vanhoenacker F, Op de Beeck B. Sclerosing Angiomatoid Nodular Transformation of the Spleen: A Diagnostic Conundrum. J Belg Soc Radiol. 2022;106(1):12. [DOI:10.5334/jbsr.2689] [PMID] [PMCID]
  15. Jin Y, Hu H, Regmi P, Li F, Cheng N. Treatment options for sclerosing angiomatoid nodular transformation of spleen. HPB (Oxford). 2020;22(11):1577-82. [DOI:10.1016/j.hpb.2020.01.014] [PMID]
Iranian Journal of Pathology
Volume 20, Issue 2
Spring 2025
Pages 239-243

  • Receive Date 24 January 2024
  • Revise Date 16 April 2024
  • Accept Date 16 September 2024

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