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Synchronous pancreatic neuroendocrine tumors and renal cell cancer are extremely rare. Von-Hipple-Landau syndrome is a major association. A 43-year-old male patient with left upper quadrant pain and significant weight loss was diagnosed with a synchronous pancreatic tail neuroendocrine tumor with solitary splenic metastasis and a clear-cell renal cell carcinoma of the left kidney. Sonography and a computed tomography scan of the abdomen showed a complex exophytic left renal mass and a necrotic lesion limited to the spleen. Although not apparent on preoperative imaging, distal pancreatic mass was also discovered intraoperatively. Subsequently, left radical nephrectomy, splenectomy, and distal pancreatectomy were performed, and the synchronous primaries and splenic metastasis were confirmed histopathologically. This case is unique in that it demonstrates multiple extremely rare events occurring simultaneously, namely pancreatic and kidney primaries, as well as solitary splenic metastasis., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Introduction and Importance: Teratomas are nonseminomatous germ cell tumor. Retroperitoneal teratoma is uncommon, especially in adults. Surgical excision the main stay of therapy for mature teratoma and also it the way for defnitive diagnosis., Case Presentation: This case report discuss a 20 year old female patient who presented with long standing abdominal pain. On abdominal examination there was firm, immobile huge mass irregular mass involving left lower and upper abdomen crossing the midline. The ultrasound and CT scan was suggestive of retroperitoneal teratoma. Preoperative CA19.9 was elevated, which drop significantly postoperatively. Patient was explored and complete tumor resection is done. The biopsy confirms mature teratoma. The postoperative follow up was unremarkable., Clinical Discussion: Retroperitoneal teratomas are very rare in adults and only few cases have been reported in literature. Primary retroperitoneal teratomas (PRT) are rare, with incidence of 1 %-11 %. Imaging has vital role for diagnosis of retroperitoneal. Tumor markers can be elevated in PRT. Most mature teratomas are benign in nature. Surgical excision is necessary for definitive diagnosis and it is main stay of management for retroperitoneal teratoma., Conclusion: Preoperative Imaging is important not only for diagnosis but also for preoperative planning. Surgical resection is the main stay of management for mature retroperitoneal teratoma. Despite its benign nature mature retroperitoneal teratoma need close follow up, because of its low risk of malignant transformation., Competing Interests: Conflict of interest statement All authors declare that they have no conflict of interest., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)