Intrapancreatic accessory spleen： A case report and review of the literature

Here, we report a case of intrapancreatic accessory spleen confirmed by pathologic diagnosis and discuss its differential diagnosis and surgical management with a review of the literature.     

Enlarged accessory spleen presenting stomach submucosal tumor

A 62-year-old man presented with upper abdominal discomfort underwent upper gastrointestinal endoscopy. Gastroscopy and endoscopic ultrasonography revealed a submucosal tumor (SMT) with homogenous echogenicity originated from extragastric organs. An abdominal contrast-enhanced computed tomography (CT) showed that the well-marginated ovoid mass, approximately 6 cm in diameter, enhanced homogenously to a similar degree as splenic parenchyma. 99mTechnetium sulfur colloid scintigraphy revealed the splenic nature of the mass. A diagnosis of accessory spleen mimicking gastric SMT was made. Subsequent follow-up was uneventful without performing splenectomy.     

Thrombocytopenia in association with a wandering spleen

An ectopic, so-called wandering spleen is an uncommon occurrence. We present the case of a young woman who presented with abdominal pain and was found to have an enlarged spleen, located in the lower abdomen and pelvis. The possibility of lymphoma was entertained because of concomitant findings of thrombocytopenia and a possible mesenteric mass. The mass was subsequently found at laparotomy to be the tail of the malpositioned pancreas, and the thrombocytopenia resolved with splenectomy. Review of the literature indicates that lymphoma is an uncommon finding in wandering spleens, that wandering spleens are enlarged in most cases, and that thrombocytopenia, while uncommon, can be seen, in particular when associated with torsion of an elongated splenic pedicle. © 1996 Wiley-Liss, Inc.     

Intrapancreatic accessory spleen: a diagnostic dilemma

Background

As intrapancreatic accessory spleen (IPAS) is rarely encountered during clinical practice, the aim of this review was to summarize the epidemiologic features, the diagnosis and treatment of IPAS.

Intrapancreatic accessory spleen. A rare cause of a pancreatic mass.

CONCLUSION: The clinical significance of intrapancreatic accessory spleens resides in the mimicry of pancreatic cancer. Radionuclide tests (Octreotide scan and Tc99m sulfur colloid scan) should be undertaken to distinguish these lesions from neuroendocrine tumors, hypervascular metastases and pancreatic carcinoma. If the tests are equivocal, diagnostic laparotomy or laparoscopy is recommended. BACKGROUND: Despite its relatively common occurrence, intrapancreatic ectopic splenic tissue is rarely detected owing to its asymptomatic nature. METHODS: We report a case of a clinically asymptomatic patient in which abdominal computed tomography (CT) scans revealed a mass of 1.5 cm in diameter in the distal pancreas. The tumor markers CA 19-9 and carcinoembryonic antigen (CEA) were slightly elevated, and pancreatic neoplasm was suspected. RESULTS: Left pancreatic resection and splenectomy were performed. The removed specimen disclosed the presence of an accessory spleen within the pancreatic tail.     

胰腺内副脾误诊为神经内分泌肿瘤病例特点及误诊分析（附 10例报道）

目的 探讨胰腺内副脾患者的病例特点及误诊原因，以提高对该病的认识，减少误诊及不必要的手术。方法 回顾性收集术前诊断为胰腺神经内分泌肿瘤，经术后病理确诊为胰腺内副脾的患者10例(共12 个病灶)，分析其CT、MRI、Ga68-dotatatePET-CT图像、病理特点及临床资料。结果 10例患者中9例为单发，1例有3个病灶；12个病灶均位于胰尾、均与周围组织边界清晰、均为富血供结节；病灶最大径0.3~3 cm, 平均1.43 cm。MRI表现：T1W1均呈低信号，T2W1 8例呈高信号（80%），DWI 7例呈高信号（70%）；增强扫描动脉期6例呈均匀强化，只有 1例呈 “花斑样”不均匀强化。3例行Ga68-dotatatePET-CT检查的患者病灶均呈高代谢，SUVmax约为脾脏SUV的一半。结论 IPAS一般位于胰尾，病灶小于3cm，边界清晰；在MRI T1W1序列上呈低信号，T2W1及DWI多呈高信号，增强扫描动脉期很少见到典型的“花斑样”不均匀强化，静脉期及延迟期多为均匀强化，68Ga标记生长抑素受体PET/CT检查时可能会出现高代谢。     

Giant epithelial cyst of the accessory spleen

Splenic cysts are uncommon, and cysts derived from the accessory spleen are rare. We report a case of a huge splenic cyst derived from the accessory spleen in the omentum, concomitant with multiple epithelial cysts of the primary spleen. Both serum and cystic fluid concentrations of carbohydrate antigen (CA)19-9 were markedly elevated. A huge monolocular cyst occupied the entire abdomen. A total of 7?l of aspirated cystic fluid was turbid and yellowish-brown. The cyst wall, which consisted of CA19-9-positive squamous epithelium, contained approximately 2 × 3 × 1?cm of splenic tissue. There was a separate multicystic 16 × 8-cm spleen in the normal position. The CA19-9 serum level returned to normal postoperatively.     

Epidermoid cyst in an intrapancreatic accessory spleen: a case report

Ectopic splenic tissue in the abdominal cavity is a common entity, with a reported incidence of 10% in the general population. However, an intrapancreatic accessory spleen is a rare disease, and moreover cyst formation in it is exceedingly rare. A 58-year-old woman with a 25-mm multilocular cyst in the tail of the pancreas detected incidentally by ultrasonography was admitted for further evaluation. Because malignancy could not be ruled out, a spleen-preserving distal pancreatectomy was performed. The cut surface of the surgical specimen showed a multilocular cyst surrounded by brown solid tissue resembling normal spleen. Pathological examination revealed it was stratified squamous epithelium and was surrounded by splenic tissue. The final pathological diagnosis was epidermoid cyst in an accessory spleen in the pancreas. This cyst has no characteristic features on diagnostic imaging. Consequently, it is not possible to make a definite preoperative diagnosis in most cases. Epidermoid cyst in intrapancreatic splenic tissue is another lesion to be considered in the differential diagnosis of pancreatic tail tumors.     

Intrahepatic accessory spleen: imaging features.

The authors present a case report of a 60-year-old man with a hepatic unknown mass. For diagnosis, they used ECO, CT (with and without contrast), MR (with and without contrast) and an ultrasound-assisted percutaneous lesion biopsy. Thus the mass-lesion in the liver appeared to be an intrahepatic accessory spleen in a patient afflicted with chronic hepatitis.     

胰腺异位副脾的MDCT特征

目的 总结胰腺异位副脾(IPAS)的多层螺旋CT(MDCT)征象,提高对该病的认识和诊断正确率.方法 收集术前行MDCT检查并经手术和病理确诊的7例IPAS患者资料,回顾性分析IPAS的MDCT特征性表现,包括病变的位置、大小、形态、边界、密度、强化特点.结果 本组男性4例,女性3例,平均年龄49岁.所有病灶均位于胰腺尾部被膜下胰腺实质内偏背侧.病灶呈类圆形3例,椭圆形4例,边界清晰.病变均为实性结构,内无坏死区,未见钙化灶.病灶最大直径0.9～1.8 cm,平均为1.4 cm.与胰腺实质相比,CT平扫时肿块实质密度均较均匀,动脉期呈不均匀稍高密度3例,均匀稍高密度4例,门脉期均呈均匀稍高密度.平扫时肿块CT值范围50～ 61 HU,平均55 HU;动脉期CT值范围80～110 HU,平均97 HU;门脉期CT值99～ 120 HU,平均102 HU.行血管成像的3例患者,不管是动脉还是静脉均未见受压、被侵犯征象,未见有血管与病灶相连.结论 IPAS具有特征性的MDCT影像表现,对于胰腺尾部较小实性病灶,其密度及强化方式与脾脏相似时应考虑IPAS的可能.     

Epidermoid cyst originating from an intrapancreatic accessory spleen

We report a rare case of an epidermoid cyst originating from an intrapancreatic accessory spleen, in a 40-year-old Japanese man with no clinical symptoms. A cystic tumor in the pancreatic tail was detected incidentally by abdominal ultrasonography. The patient was referred to the KKR Tachikawa Hospital for further examination of the tumor. Preoperative imaging findings suggested that the tumor was an epidermoid cyst originating from an intrapancreatic accessory spleen. On both pre-and post-contrast computed tomography and magnetic resonance images, the solid compartment of the tumor had the same X-ray attenuation and intensity as the spleen. Upon surgical excision, the mass consisted of solid and cystic components that were macroscopically evident on the preoperative images. Microscopic analysis revealed that the solid component was an accessory spleen in the pancreatic tail, whereas the cystic component was lined with stratified epithelium representative of an epidermoid cyst. This is the thirteenth report (in English) of an epidermoid cyst originating from an intrapancreatic accessory spleen, and the first case to be diagnosed prior to surgery.     

Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera

Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%, respectively, thrombocytosis of 501 000/μL, and leukocytosis of 20 410/μL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally.     

Ectopic Pancreas: A Diagnostic Dilemma

An ectopic pancreas is defined as pancreatic tissue lacking vascular or anatomic communication with the normal body of the pancreas. It is rarely symptomatic as it is found incidentally at laparotomy most of the time. Despite advances in diagnostic modalities, it still remains a challenge to the clinician to differentiate it from a neoplasm. It is prudent to differentiate it from neoplastic etiologies, as simple surgical excision can potentially be curative. We discuss the presentation, diagnosis, and treatment of an interesting case of ectopic pancreas presenting as a gastric antral tumor.