Sclerosing angiomatoid nodular transformation presenting with abdominal hemorrhage: First report in infancy

Gloria Pelizzo, Vincenzo Villanacci, Luisa Lorenzi, Orietta Doria, Anna Maria Caruso, Vincenza Girgenti, Elettra Unti, Laura Putignano, Gabrio Bassotti, Valeria Calcaterra

Research output: Contribution to journalArticle

Abstract

A limited number of sclerosing Angiomatoid Nodular Transformation (SANT) have been reported in pediatric age. We describe the first case of SANT occurring in a nine-week-old female infant that was admitted to our unit for severe abdominal distension and rectal bleeding. Enlarged spleen was detected on physical examination. Laboratory investigations revealed severe anemia and coagulation abnormalities. Abdominal ultrasound and computed tomography revealed ascites and splenomegaly with a large mass at the lower medial splenic pole. A diagnosis of intra-abdominal hemorrhage was presumed and an exploratory laparotomy was performed. A complete transformation of the giant splenomegaly to bossellated masses and multiple bleeding capsular ruptures without subcapsular hematoma were found and an urgent splenectomy was performed. At histology, a SANT was diagnosed (CD34, CD31, CD8 positivity). The postoperative follow up was uneventful. SANT may also occur in infancy with a potentially life-threatening presentation. Splenectomy may represent the only treatment in severe cases.

Original languageEnglish
Pages (from-to)31-34
Number of pages4
JournalPediatric Reports
Volume11
Issue number2
DOIs
Publication statusPublished - Jan 1 2019

Keywords

  • Infant
  • Sclerosing Angiomatoid Nodular Transformation
  • Spleen

ASJC Scopus subject areas

  • Pediatrics

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