Giant splenic cyst and solid pseudopapillary tumour of the pancreas managed with distal pancreatectomy and splenectomy

Abstract

Solid pseudopapillary tumours of the pancreas and giant splenic cysts are very rare entities, and their coexistence in a young female patient has not been previously reported in the literature. We present the case of a 27-year-old woman who presented with abdominal pain and two masses on abdominal imaging. A mass located in the right upper quadrant was biopsied, and histological and immunohistochemical analysis showed a solid pseudopapillary tumour of the pancreas. A giant cystic splenic lesion was also noted. The patient underwent a distal pancreatectomy and splenectomy in our referral centre. Margins were negative on histopathological examination. Negative surgical margins were achieved with distal pancreatectomy and splenectomy despite the large size of the pancreatic tumour. The management of solid pseudopapillary tumours of the pancreas is often challenging and the concomitant presence of a giant splenic cyst poses additional challenges to the surgical management of such tumours.

Solid Pseudopapillary Neoplasms Are Rare, Indolent Pancreatic Tumors in Young Women

“…Patients usually present with nonspecific clinical manifestations related to intra-abdominal mass such as pain, early fullness, dyspepsia, nausea, and vomiting; although most patients are asymptomatic, they are diagnosed incidentally on examination or abdominal imaging. However, in our case, the sharp and itchy acute abdomen forced the patient to refer to the emergency ward a couple of times which distinguished our patient from other reported cases [ 2 , 9 , 12 , 13 ]. Although obstructive jaundice is a common symptom in the head pancreatic tumor, its presence is so rare in SPN patients [ 14 ] that guides us to discriminate between SPN and pancreatic cancer [ 13 ].…”

“…Nevertheless, the incidence of metastatic lesions and recurrence is 10-15% [ 3 , 4 ]. A high ki67 index may exhibit the probability of malignant course and metastases [ 2 , 6 , 12 ]. SPN can affect predominantly young females in their second to fourth decade [6, 11].…”

“…On the other hand, the results of a systematic review showed the absence of any significant differences in postoperative morbidity and postoperative fistula rates between open and minimally invasive pancreatectomy [ 10 ]. The concomitant incidence of SPNs with splenic cyst is not frequently reported, and no associations have been found between them as Hajjar et al mentioned [ 2 ].…”

“…Solid pseudopapillary neoplasm is a rare tumor with low malignant potential, accounting for 1-2% of exocrine pancreatic neoplastic lesions [ 1 ]. In advance of WHO redefining it as a solid pseudopapillary tumor of the pancreas in 1996, it was named Frantz Tumor owing to Virginia Frantz, the pathologist who initially described it [ 2 ]. It mostly affects young women during their reproductive age [ 3 – 5 ].…”

“…As SPNs have an excellent prognosis, and the complete resection is a curative treatment, accurate and prompt diagnosis by imaging modalities and pathological examinations are critical. Complete resection is a choice of treatment upon the site of the tumor [ 1 , 2 ], but also, other authors argued about the priority of minimally invasive pancreatectomy may be more effective [ 9 , 10 ]. In the present case, we aim to elucidate the critical aspects of this rare pancreatic tumor with accompanying splenic cyst.…”