Primary Mucinous Cystadenocarcinoma of Spleen with Pseudomyxoma Peritonei: a Very Rare Entity

Abstract

Primary mucinous cystadenocarcinoma of the spleen is an extremely rare tumour. Only 7 cases have been reported in the literature. Mucinous cystadenocarcinoma of the spleen presenting as pseudomyxoma peritonei has been reported only once. Our patient was initially managed as a case of a hydatid cyst of the spleen. Following splenectomy, histopathology reported it as mucinous cystadenocarcinoma. In view of the fact that metastatic cystadenocarcinoma is more common, investigations were performed for the evaluation of a primary site of malignancy. A whole-body PET CT scan did not reveal any metabolically active lesion in any part of the body. Both upper gastrointestinal endoscopy and colonoscopy did not reveal any primary malignant lesion. Hence, it was being reported as a primary mucinous cystadenocarcinoma of the spleen. It is being reported on account of its extreme rarity .