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Laparoscopic splenectomy for tumor in children: Report of an exceptional etiology. Do you know SANT?

Christian Pioplat¹, Yohann Robert¹, Ionella Nechifor¹, Hervé Testard¹, Blandine Fabre¹, Marie Parrens², Jérôme Duret¹, Corinne Alla¹, Dominique Plamtaz¹
¹Hôpital Couple Enfant, CHU de Grenoble, France, ²Groupe Hospitalier Haut Lévêque, CHU de Bordeaux, France

 

Abstract 

Aims: Solid tumors of the spleen are extremely rare in children. Sclerosing angiomatoid nodular transformation of the spleen (SANT) is an exceptional etiology.
Case report: A 14-years old girl experienced asthenia and was treated for urinary tract infection. She was addressed following sonographic finding of a 70 mm in diameter, heterogeneous, vascularized splenic solid tumor.
Biology has found anemia. CT and MRI confirmed enhancement of the central portion after injection. Several hypotheses have been advanced (vascular malformation? hamartoma?) but malignancy could not be ruled out. Laparoscopic total splenectomy was undertaken, the spleen was then placed in a retrieval bag and extracted without fragmentation by a suprapubic incision. Follow-up was uneventful. Histology has suggested the diagnosis of sclerosing angiomatoïd nodular transformation of the spleen (SANT). The decline since the intervention is 9 months.
Discussion: Described for the first time by Martel in 2004, SANT is exceptional. 97 cases have been detailed by Falk in 2012 and only 3 cases involved children. The lesion is often asymptomatic and discovered incidentally by imaging, more rarely symptomatic. Some MRI characteristics have been described. Diagnostic biopsy may allow avoiding surgery, but exposes to complications (intraperitoneal bleeding, swarming if malignancy). Laparoscopic splenectomy is not contrindicated but it seems preferable, to not fragment the spleen and to extract it via a cosmetic incision in an extraction bag. In case of non-central tumors partial splenectomy is possible without taking a risk of tumor rupture. Differential diagnoses are vascular lesions of the spleen: hemangioma, coastal cell angioma, hemangioendothelioma and inflammatory myofibroblastic tumor. Immuno-histo-chemical characteristics are helpful.
Conclusion: SANT should be kept in mind in children in cases of solid vascular splenic tumors. Imaging may be suggestive but is rarely sufficient to avoid splenectomy. Laparoscopic splenectomy, respecting the rules of oncological surgery, led to the diagnosis and brings healing.