Rare presentation of a right retroperitoneal accessory spleen: A case report

Abstract

Abstract. An accessory spleen is a congenital malformation, which is defined as ectopic splenic parenchyma. Here, an extremely rare case of a right retroperitoneal accessory spleen, mimicking a retroperitoneal neoplasm, is reported. A 40-year-old woman was referred following the incidental detection of a retroperitoneal neoplasm. Computed tomography and magnetic resonance imaging scans confirmed the presence of a retroperitoneal neoplasm at the hepatorenal recess. Retroperitoneoscopic excision was conducted, with excellent results. Pathological examination of the resected specimen revealed splenic tissue. In conjunction with a review of the literature and a discussion of the salient radiological features, the present case highlights the requirement for accurate preoperative diagnosis of an accessory spleen in the right retroperitoneal space, in order to avoid unnecessary surgical intervention. IntroductionAn accessory spleen is a congenital defect, defined as ectopic splenic parenchyma separated from the main body of the spleen. Although the majority are benign and do not usually require treatment, they may be mistaken for enlarged lymph nodes or neoplasms. Primary retroperitoneal neoplasms are a group of rare but diverse neoplasms that arise within the retroperitoneal space, which account for 0.1-0.2% of all malignancies in the body. Notably, 80-90% of all primary retroperitoneal tumors are malignant (1,2). Primary retroperitoneal neoplasms are classified as solid or cystic masses. Solid neoplasms are divided into three main categories according to the tissue of origin:Mesodermal tumors, neurogenic tumors and extragonadal germ cell tumors (3). Computed tomography (CT) and magnetic resonance imaging (MRI) are essential for the characterization of primary retroperitoneal neoplasms, evaluating the extent of local invasion, identification of metastases, and determination of the optimal treatment response for such neoplasms (4). Therefore accurate preoperative diagnosis is important in order to avoid unnecessary surgical intervention. The current report discusses the case of a patient with a right retroperitoneal accessory spleen, mimicking a retroperitoneal neoplasm. Written informed consent was obtained from the patient's family. Case reportA 40-year-old woman without any abdominal discomfort was referred to Zhejiang Hospital (Zhejiang, China) for surgical excision, following the incidental detection by B ultrasound of a right retroperitoneal neoplasm at Zhejiang Hospital. Routine physical examination and laboratory data, including full blood count, blood glucose, C-reactive protein, tumor markers and liver function tests, were all unremarkable. Her medical history was notable for a splenectomy performed in 2002, for splenic rupture as a result of upper abdominal trauma.Ultrasonography demonstrated a space-occupying neoplasm, with a less abundant vascular supply than the surrounding normal tissue, located in the right retroperitoneal space (Fig. 1). CT imaging revealed a well-marginated ovoid neoplasm, of ~38.0x25...

Right sided spleen laying retro-duodenal: A case report and review of the literature

“…This case is that of a patient with a right sided accessory spleen directly anterior to the kidney and completely separate from the supra-renal gland. To the best of our knowledge, this is the first reported right accessory spleen laying retro-duodenal and second largest reported [5] , [6] , [7] , [8] ( Table 1 ).…”

Spleno-adrenal fusion mimicking an adrenal metastasis of a renal cell carcinoma: A case report and embryological background

“…Accessory spleens may represent a differential diagnostic challenge, and several cases of an accessory spleen being mistaken for an adrenal tumor have been published [ 15 , 16 , 17 ]. The very rare accessory spleens mimicking a retroperitoneal tumor on the right side represent an even more challenging situation [ 18 , 19 , 20 ]. If a developmentally separate heterotopic splenic nodule is directly connected to another organ, the term “spleno-visceral fusion” should be used instead of “accessory spleen;” however, there is inconsistency in the literature and the term “accessory spleen” is also used in this context [ 21 ].…”

“…Congenital connections of heterotopic splenic tissue and the adrenal gland have been theoretically assumed, but we were unable to find any documented case of spleno-adrenal fusion. There are several case reports of accessory spleens in the right suprarenal space misinterpreted as retroperitoneal neoplasms, but there was no direct connection to the right adrenal gland [ 18 , 19 , 20 ]. As already mentioned, an adrenal myelolipoma should be considered and ruled out if an adrenal tissue sample contains hematopoietic elements.…”

“…We therefore considered our finding to be a congenital heterotopy. Accessory spleens in the right suprarenal space misinterpreted and removed as retroperitoneal neoplasms have been published, but these cases do not mention a direct relationship of splenic tissue to the adrenal gland [ 18 , 19 , 20 ]. We have not found any previous mention of spleno-adrenal fusion in the literature.…”

Challenging diagnostic of an accessory spleen mimicking a retroperitoneal tumor: A case report and review of the literature

“…The retroperitoneal AS is a rare entity. It is usually misdiagnosed, and mistaken for a retroperitoneal neoplasm (5). Imaging ndings are useful to characterize primary retroperitoneal tumors and to help the diagnosis of accessory spleen.…”