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

Abstract

Background: Sclerosing angiomatoid vascular transformation (SANT) is a rare vascular disease of the spleen, which is difficult to diagnose due to its pre-intervention appearance of malignancy. Case Report: An 85-year-old male was transferred to our clinic for thrombocytopenia and splenic mass. A contrast enhanced abdominal CT and MRI showed nodular lesions, the largest 50mm in diameter, and several areas of heterogeneous contrast field involvement in the spleen parenchyma. Laparoscopic splenectomy was performed with normal range of platelet level. The patient's postoperative course was uneventful and he was discharged on the 6th postoperative day. Histopathology revealed SANT. The patient is now in the 18 th month of remission with platelet levels within normal range and with no recurrence.

Results: Between 2004 and April 2020, a total of 230 SANT patients who underwent laparoscopic or open splenectomy or biopsy were reported in the literature. Most patients were female (52.1%), and the median age was 46 years (9 weeks-85 years). Most patients were asymptomatic (56%). Open splenectomy was performed on 166 patients (72.1%),laparoscopic splenectomy on 35 patients (15.2%) and laparoscopic partial splenectomy on 15 patients (6.5%). The median operation time and spleen weight were 143 minutes (88-213) and 260gr (68-2,720), respectively. Median follow-up time was 12 months (0-166). No recurrence was seen in patients undergoing total splenectomy.

Conclusion: SANT is an unusual disease of the spleen. In the light of this systematic review, a minimally invasive method for total or partial splenectomy,specifically laparoscopy, can be preferred as the treatment of choice.

Keywords: Laparoscopy; Sclerosing angiomatoid vascular transformation (SANT); Splenectomy.

Fig. 1

Flow chart of the study.

Fig. 2

Abdominal CT (A&B) and Abdominal MR (C&D) show SANT with red arrows. (For interpretation of the references to color in this figure legend, the reader is referred to the Web version of this article.)

Fig. 3

Dilated vessel sections and nodular structures with spleen tissue (A) (Hematoxylin & Eosin X 100) and small nodules structures around the connective tissue (B) (Hematoxylin & Eosin X200).

Fig. 4

Diffuse CD68 positivity on immunohistochemical staining (X200).

Fig. 5

CD31 positive capillaries and small veins (Immunohistochemical staining) (X200).

Fig. 6

CD34 positive capillaries (Immunohistochemical staining) (X200).