Sclerosing Angiomatoid Nodular Transformation Presend Nodulartransformation Presenting with Abdominal Hemorrhage: First Report in Infancy

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 intraabdominal 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 lifethreatening presentation. Splenectomy may represent the only treatment in severe cases.

Sclerosing Angiomatoid Nodular Transformation of the Spleen, a Rare Cause for Laparoscopic Splenectomy: a Case Report and Literature Review

“…However, occurrence of SANT shows a young age trends in recent report [6,7]. Youngest SANT patient was a 7-month-old infant with splenomegaly [8]. Most cases are found incidentally since most patients are asymptomatic.…”

Sclerosing angiomatoid nodular transformation presenting with thrombocytopenia after laparoscopic splenectomy - Case report and systematic review of 230 patients

“…Moreover, SANT may be indirectly associated with other cases of malignity that have comorbidities (16%, n = 37) (colorectal cancer 2.1%, gastric cancer 2.1% and lung cancer 1.3%) as SANT was detected incidentally during the follow-up of primary malignities [ [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] ]. The literature reports three cases of thrombocytopenia in patients with SANT [ 6 , 73 , 77 ]. Dieobold et al [ 6 ] reported a patient presenting with thrombocytopenia due to paramyxovirus 19.…”

“…Gooch et al [ 73 ] noted that the surgical oncology consultation agreed that a splenic mass was the most likely cause of thrombocytopenia in their patient. Finally, Pelizzo et al [ 77 ] reported a case of SANT occurring in a nine-week-old female infant who was admitted with severe abdominal distension and rectal bleeding, severe anemia (Hb 4.6 g/dL), thrombocytopenia (54 × 103/μL) and coagulation abnormalities resulting from persistent abdominal hemorrhaging from ruptures of the spleen capsule that were un-responsive to blood transfusions. In the present study, no infection such as Epstein-Barr virus (EBV) infection or IGg4 was detected, and we presumed SANT to be the cause of the thrombocytopenia as the patient had normal range platelet levels after laparoscopic splenectomy.…”

“…As spleen volume extends, the spleen can be palpated during physical examination or imaging. Although clear information about splenomegaly is not available in the literature for all cases, those spleen weights given were mostly normal (19 patients; 8.2%) [ 2 , 6 , [15] , [16] , [17] , 26 , 37 , 57 , 61 , 77 ].…”

“…The curative treatment for SANT is splenectomy [ 46 , [48] , [49] , [50] , [51] , [52] , [53] , [55] , [56] , [57] , [58] , [59] , [60] ]. Open splenectomy or minimally invasive splenectomy are both options for surgical treatment [ [62] , [63] , [64] , [65] , [66] , [67] , [68] , [70] , [71] , [72] , [73] , [74] , [75] , [76] , [77] ]. In recent years, minimally invasive surgery such as robotic or laparoscopic splenectomy has become more popular due to a shorter hospital stay, less postoperative abdominal pain, and an early return to normal activity [ 18 , [63] , [64] , [65] , 78 ].…”