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Rare Pseudopapillary Neoplasm of the Pancreas: A 10-Year Experience

Rare Pseudopapillary Neoplasm of the Pancreas: A 10-Year Experience Hindawi Surgery Research and Practice Volume 2021, Article ID 7377991, 8 pages https://doi.org/10.1155/2021/7377991 Research Article Rare Pseudopapillary Neoplasm of the Pancreas: A 10-Year Experience Suvendu Sekhar Jena , Samrat Ray , Sri Aurobindo Prasad Das , Naimish N Mehta , Amitabh Yadav , and Samiran Nundy Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, Old Rajinder Nagar, New Delhi 110060, India Correspondence should be addressed to Suvendu Sekhar Jena; suvu1078@gmail.com Received 3 June 2021; Revised 6 September 2021; Accepted 8 September 2021; Published 17 September 2021 Academic Editor: Cosimo Sperti Copyright © 2021 Suvendu Sekhar Jena et al. 'is is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Introduction. 'e solid pseudopapillary epithelial neoplasm (SPN) is a rare form of pancreatic neoplasm with an incidence of 2-3% of all pancreatic tumours. 'e recent increase in incidence is attributed to the increasing use of imaging techniques for nonspecific abdominal complaints. We report our institutional experience in the management of this tumour over the last decade. Method. We retrospectively analyzed from a prospectively maintained database of patients from January 2011 to December 2020 who were operated upon for SPN. All the patients were followed till date. Results. Of 479 patients operated on for various types of pancreatic tumours during this period, 15 (3.1%) had SPN. 'e mean age of presentation was 28 years with a female preponderance (12/15, 80%). 'e most common location was the body and tail of the pancreas (66%), and the mean size was 6.4 cm (2–15 cm). 'e tumour extent was defined as ‘borderline resectable’ in 20% of cases. Distal pancreatectomy was done in 11 patients with spleen preservation in 3. R0, R1, and R2 resection were done in 12, 2, and 1 patient(s), respectively. 'e operative mortality was 6.7%. All the patients are doing well on follow-up. Conclusion. SPN is a low-grade malignant tumour with a strong female predilection. Clinical manifestations have no specificity, imaging examination only contributes tumour location, and the final diagnosis rests on pathology. Surgery is the main modality of treatment and carries a good prognosis. complaints [3]. Cubilla and Fitzgerald reported an incidence 1. Introduction of SPN of 0.17% in 1979, Morohoshi et al. reported an Solid pseudopapillary neoplasms (SPNs) of the pancreas are incidence of 2.7% in 1987, and Koshmal et al. reported an defined by the new 5th edition of the World Health Or- incidence of 6.1% of all pancreatic tumours in 2004 [4–6]. ganization (WHO) Classification of Digestive System Tu- Lichtenstein was the first to report this entity, but Frantz in mors as low-grade malignant tumours composed of poorly 1959 described its pathology and Hamoudi et al. described cohesive epithelial cells which form solid and pseudopa- its electron microscopy features in 1970 [7–9]. Before being defined as a tumour of uncertain differentiation or solid pillary structures and lack a specific line of pancreatic ep- ithelial differentiation [1]. SPN incidence is around 2-3% of pseudopapillary neoplasm in 1996 by the World Health all pancreatic neoplasms. It has a female preponderance and Organization, it was also known as a solid cystic tumour, also malignant potential [2]. A total of 8334 cases have been papillary epithelial neoplasm, or papillary epithelial tumour reported in the English literature till 2018 with an increasing [10]. It is a rare tumour and has an estimated incidence of 2- incidence in recent times. 'is is attributed to the extended 3% of all pancreatic tumours and 6–12% of all pancreatic use of imaging techniques for nonspecific abdominal cystic neoplasms [3]. It usually affects women below the age complaints and their better availability since most of the of 40 years (2–85 years), with a male to female ratio of 7–11 : tumours are indolent and patients have nonspecific 1. In the paediatric age group, the incidence is 8–12.5%. 'e 2 Surgery Research and Practice usual location is the body and tail of the pancreas (55–60%) patient had hypertension as an associated comorbid illness followed by the head and neck of the gland (35–40%). It is with a mean Charlson comorbidity index of 8.7. usually associated with a favourable prognosis with a long- 'e majority of the patients presented with a mild, dull term disease-free survival of 95% [11]. SPNs are slow- aching type of pain (66.7%), while one patient was inci- growing tumours and have a low malignant potential, and dentally diagnosed while being evaluated for unexplained the usual symptoms are due to local manifestations of the anaemia when she was found to have a SPN along with a tumour. It may occasionally present with tumour rupture or uterine fibroid. One patient presented with severe abdom- metastasis. 'e origin of the disease is unknown with various inal pain and on evaluation was found to have a ruptured authors claiming different points of origin. Surgery is the SPN. 'ree patients presented with a lump in the abdomen. mainstay of treatmentwhich depends upon the location of 'e median size of the tumour was 6 cm (2–15 cm). 'e the tumour and ranges from distal pancreatectomy and location was the body and tail of the pancreas in 5, tail in 5, Whipple’s pancreaticoduodenectomy, to debulking. Ra- and body in 3, 1 each in head and uncinate process (Table 1). diotherapy and chemotherapy have very little or no role in Preoperative tumour markers like the CA 19-9 and its management [12]. We had previously published a case CEA, found to rule out primary pancreatic adenocarci- report of a young girl presented with ruptured SPN in 2016 noma or metastatic disease, were normal in all cases. 'e and highlighted various aspects of management [13]. 'e imaging modalities used were ultrasound (USG) abdomen present study was done to report our institutional experience and CECT abdomen. USG abdomen was done in 12 pa- in the management of this rare tumour over the last decade. tients, among whom the tumour was hypoechoic in 5, heteroechoic in 5, and isoechoic in 2 patients. CECT ab- domen revealed a borderline resectable lesion in 3 patients 2. Patients and Methods and resectable lesion in 12 patients. 'e tumour was solid We retrospectively analyzed, from a prospectively main- only in 8 patients, cystic only in 1 patient, and mixed solid tained database, all patients with a diagnosis of solid and cystic in 6 patients. 'e cystic areas probably indicated pseudopapillary epithelial neoplasm operated at the De- haemorrhage into the tumour. In 14 cases, the tumours partment of Surgical Gastroenterology and Liver Trans- were encapsulated. Calcification was seen in 12 patients plantation at Sir Ganga Ram Hospital, New Delhi, over a (Figure 1). Endoscopic ultrasound (EUS) was done as a part of the diagnostic procedure in 6 patients (Figure 2). 2 period of 10 years from January 2011 to December 2020. 'eir demographic data, associated comorbidities, pre- patients had a preoperative diagnosis other than SPN on senting complaints, preoperative blood parameters, and EUS, like islet cell tumour in one patient and pancreatic preoperative tumour markers like carcinoembryonic antigen cystadenoma in the other. A preoperative biopsy was done (CEA) and carbohydrate antigen 19-9 (CA 19-9) were an- in 8 patients, among whom 6 had fine-needle aspiration alyzed. 'e imaging results, i.e., the ultrasound and contrast- cytology via EUS and 2 underwent exploration and biopsy enhanced computed tomography (CECT) of the abdomen, somewhere else for a lump in the abdomen before coming were also analyzed. 'e type of surgery performed and its here (Table 2). complications along with the histopathology reports were All 15 patients underwent curative resection. 'e patient who presented with a tumour rupture underwent emergency also recorded, and the complications were graded according to the Clavien–Dindo classification (CDC) and compre- laparotomy due to haemodynamic instability and altered hensive complication index (CCI) [14, 15]. 'e postopera- sensorium. Upon exploration, around 1400 ml of blood was tive pancreatic fistula (POPF) was defined as per the found in the peritoneal cavity. 'e mass was found to be definition of an international study group of pancreatic adherent to the splenic flexure of the colon, spleen, and fistula. 'e patients were followed up till December 2020. transverse mesocolon with complete obliteration of the 'e pancreatic exocrine functions were assessed from the lesser sac. Because of her unstable condition, peritoneal symptoms and need for pancreatic enzyme supplementa- lavage and biopsy for the tumour were performed. She then tion, while the endocrine functions were assessed by the received four cycles of chemotherapy using vincristine need for insulin or any increase in the requirement of insulin (1.5 mg/m ), actinomycin D (0.045 mg/kg), and cyclophos- if already on insulin. 'e data were compared with those phamide (1200 mg/m ) to reduce the size of the tumour. 'e published from western countries as well as from India. tumour response was assessed with the USG abdomen. She Statistical analysis was performed using SPSS version 22.0 subsequently underwent distal pancreaticosplenectomy. software. Continuous variables were reported as mean- 'is procedure was performed in a total of 11 patients with ± standard deviation (±SD). Informed consent was taken spleen-preserving distal pancreatectomy in 3 patients. from the patients for the use of their data. Whipple’s pancreaticodudoenectomy was performed in 2 patients. One patient had a central pancreatectomy with Roux-en-Y reconstruction (Figure 3). None of the patients 3. Results underwent vascular resection or reconstruction. Of 11 pa- Of 479 patients operated for various types of pancreatic tients who underwent distal pancreatectomy with or without tumours during this period, 15 (3.1%) were operated for splenectomy, 6 patients underwent laparoscopic resection SPN. 'ey had a mean age of 28.13 (±11.2) years (11–47). Of while 5 underwent open resection. Multivisceral resection 15 patients, 12 were female and 3 were male. 'eir mean was not required in any of the patients. 'e average blood body mass index (BMI) was 22.37 (±3.85) kg/meter . Only 1 loss was 228 ml. A total of 4 patients received blood Surgery Research and Practice 3 Table 1: Demographic variables. 4. Discussion Variable n � 15 SPN is a rare pancreatic tumour, accounting for 1-2% of all Age in years (mean + SD) 28.13± 11.20 pancreatic neoplasms [16]. Unlike pancreatic adenocarci- Sex noma, these tumours are indolent and benign with a risk of Male 3 malignant transformation in around 10–15% [17]. 'eir cell Female 12 of origin is unknown. One theory suggests it originates from BMI (kg/m ) 22.37± 3.85 multipotent primordial germ cells lacking a definite dif- Presentation ferentiation of the exocrine or endocrine cell [18]. Various Symptomatic 14 authors have also described various sources of origin like the Incidentally 1 acinar cell and endocrine cell. Another theory suggests its Presenting symptoms origin from the incorporation of primitive ovarian cells Pain 113 within the pancreatic parenchyma during the seventh week Lump abdomen 1 of embryogenesis, but this does not explain its occurrence in Incidental males [19–22]. Tumour size in cm (median) 6.4 (2–15) It is frequently seen in young females in their second to Location of tumour fourth decade of life with a female preponderance of 80% Body 3 and mean age of 28.13± 11.2 years. Law et al. in their Tail 5 systematic review of 2744 patients revealed 87.8% incidence Body and tail 5 in females with an average age of 28.5± 13.7 years [23]. Head 1 Obesity had no relation with SPN in our study which was Uncinate process 1 also confirmed by Song et al. with an average BMI of SD: standard deviation; BMI: body mass index; n: number of patients. 23.7± 2.4 kg/m [8]. 'e average BMI in our study was 22.37± 3.85 kg/m (14.3–27.8). 'ere are some reports of an association between hepatitis B virus and SPN due to transfusion. 'e average duration of surgery was overexpression of beta-catenin in tumour cells, but none of 268.3± 145.8 min (Table 3). our patients had hepatitis B [24]. R0 resection was performed in 12 patients, while R1 'e symptoms are mostly nonspecific, and abdominal resection was performed in 2 patients. 'e patient, who pain caused by the mass is the most common manifestation presented with tumour rupture, initially underwent damage with nausea and vomiting being other symptoms [3]. 'e control surgery (R2) followed by tumour downsizing with subtle nature of their presentation leads to a delay in di- chemotherapy and complete resection. None of the patients agnosis, resulting in large tumours found at that time. had lymphovascular invasion, while 1 of them showed However, the size of the lesion does not affect resectability perineural invasion. On immunohistochemical (IHC) [25]. SPNs are usually bulky tumours (median tumour size staining, cells were stained positive for vimentin in 13 pa- 6 cm in our study). Despite their large size, they usually do tients and synaptophysin and beta-catenin in 11. Chro- not invade the surrounding structures but only displace mogranin was negative in all 15 patients (Table 4) (Figure 4). them, so symptoms like obstructive jaundice and pancrea- 'e Ki-67 proliferation index was less than 1% in all cases. titis are rare. Abdominal pain was the most common mode 'e average number of lymph nodes retrieved were 10 ( of presentation seen in 66.7% of our cases followed by a lump 2–24), and none of the nodes showed metastatic deposits. in 20%. Incidental detection was seen in one. Usually, it is 'ere was 1 patient with operative mortality. 'e patient restricted to the pancreas in approximately 85% of cases, underwent exploratory laparotomy, cholecystectomy, gas- while 10–15% of cases present with metastases [23]. 'e trojejunostomy, and biopsy of intra-abdominal mass else- most common sites of metastasis are the liver, regional where following which there was a bile leak followed by lymph nodes, omentum, and peritoneum [26]. None of our endoscopic stenting of the common bile duct. He then re- patients had metastasis during their presentation. ceived 3 cycles of chemotherapy and underwent distal Although rare, rupture of the tumour can also be a mode pancreaticosplenectomy in our hospital. He had a POPF that of presentation that can either be spontaneous or following was managed conservatively, and he was discharged with a blunt trauma to the abdomen. 'e reported incidence of drain in situ. Gradually, the drain output reduced and it was rupture in the literature is 2.7% with blunt trauma to the removed. He was readmitted with hematemesis and hem- abdomen being the most common cause [27]. 'e incidence orrhagic shock on postoperative day (POD) 45. On evalu- of spontaneous rupture is 1% of all SPN and thought to be ation, there was a pseudoaneurysm of the gastroduodenal due to sudden massive haemorrhage causing a rise in artery which was managed by angioembolization. He sub- pressure inside. One 11-year-old young girl presented to us sequently developed septicaemia and died on POD 88. with a sudden onset of abdominal pain with haemodynamic All the patients were followed up. At a median follow-up instability and underwent debulking because of bleeding and of 59 months (2–109), there was no recurrence. Except for infiltration of the surrounding structures. the patient who underwent damage control surgery, no 'e most common location of the tumour was the body patient received adjuvant chemotherapy. 'ere was no and tail of the pancreas in 80% of cases. Law et al. in their endocrine or exocrine insufficiency in any of the patients. meta-analysis showed 59.3% located in the body and tail of 4 Surgery Research and Practice (a) (b) Figure 1: (a) CECT abdomen showing an exophytic growth arising from the body and neck of the pancreas (yellow arrow) displacing the stomach with a peripheral curvilinear calcification (blue arrow). (b) Exophytic growth arising from the head of the pancreas (white arrow). Table 2: Diagnostic modalities and findings. Diagnostic modalities Incidence (n) Ultrasound (n �12) Hypoechoic 5 Heteroechoic 5 Isoechoic 2 CECT abdomen (n �15) Borderline resectable 3 Resectable 12 Encapsulated 14 Calcification 12 EUS Performed 6 Preoperative suspicion SPN 13 Others 2 Figure 2: A 3 × 4 cm lesion with a small cystic component arising Preoperative biopsy from the uncinate process of the pancreas. Performed 8 EUS guided 6 Exploratory laparotomy 2 the pancreas [23], and one of the largest meta-analyses Not performed 7 conducted by Yao et al. in 2450 Chinese patients also showed the most common location to be the body and tail of the pancreas. In contrast, Panieri et al. showed an equal spatial distribution of the tumour [28, 29]. 'e size of the defined in microcystic adenomas [32]. Ultrasonography, tumour or its location did not predict malignancy as re- CT scan, and MRI typically demonstrate the same char- ported by Yu et al. [30]. Ectopic locations have also been acteristics with an encapsulated tumour composed of solid described in the retro peritoneum, mesentery, and left and cystic elements, often with capsule rim-like calcifica- adrenal gland [28]. None of our patients had an ectopic tions as well as intraparenchymal calcifications. 'e lesion location. has well-defined margins often without pancreatic duct No additional information in our series was provided dilation. Ultrasonography demonstrates hypoechoic, iso- echoic, or heteroechoic components, while CT shows solid by the haematological investigations, while hyper- amylasaemia, elevated hepatic enzymes, and leukocytosis or cystic components with calcification. MRI and positron were occasionally identified [24]. In tumour markers like emission tomography can also be used. MRI is better than carbohydrate antigen 19-9 and carcinoembryonic antigen CT in various aspects like demonstration of a capsule and did not contribute to the diagnosis. In preoperative as- solid or cystic degeneration [33]. None of the radiological sessment, radiological studies are the most important. findings are specific for SPN, and similar findings can also Abdominal roentgenograms can demonstrate displace- be seen in other cystic neoplasms and pancreatoblastoma. ment of the stomach, colon, or spleen by an extrinsic mass Endoscopic ultrasound and fine-needle aspiration were [31]. Calcification is seldom encountered within the large used in 40% of cases. Preoperative tissue diagnosis is not mass. However, calcifications are peripheral and curvi- always necessary but can be used in cases of uncertain linear when present, as compared to the pattern of sunburst diagnosis [12]. Surgery Research and Practice 5 (a) (b) Figure 3: (a) Exophytic growth arising from the body and neck of the pancreas with reflected stomach anteriorly (white arrow) and normal pancreas (green arrow). (b) Whipple’s pancreaticodudoenectomy specimen; exophytic growth arising from the head of the pancreas with cystic areas (white arrow). Table 4: Immunohistochemical markers. Table 3: Operative parameters. IHC marker Positive (n) Negative (n) Variables n � 15 Vimentin 13 2 Surgery performed Beta-catenin 11 4 Distal pancreatectomy 11 Synaptophysin 11 4 Splenectomy 8 CD 10 9 6 Spleen preservation 3 PR 8 7 Whipple PD 2 Cytokeratin 6 9 Central pancreatectomy 1 NSE 5 10 Debulking 1 CEA 4 11 Laparoscopic resection 6 Chromogranin 0 15 Open resection 9 Blood loss (in ml) 228 PR: progesterone receptor; NSE: neuron-specific enolase; CEA: carci- Duration of surgery (min) 268.3± 145.8 noembryonic antigen. Blood transfusion 4 Hospital stay (days) 6 (3–16) Complication showed lymphovascular invasion in our study, and all the Clavien–Dindo grading 10 resected nodes were negative for tumour deposits. Meta- I 5 statectomy also improves long-term outcomes [12]. II 5 Debulking of a locally advanced tumour also has a role as III 0 shown by a previous case report from our institution [13]. IV 0 Enucleation is also another option for moving small tumours Comprehensive complication index 8.7 (0–36.2) Postoperative pancreatic fistula 8 away from the duct but carries a risk of fistulae and tumour Grade A 6 dissemination. Complete R0 resection was performed in Grade B 1 80% of patients, while 13.3% had an R1 resection. It is Grade C 1 difficult to differentiate between the malignant and benign Mortality 1 varieties as the criteria for malignancy is not clear cut. As per the WHO classification, the clear-cut criteria for malignant varieties are lymphovascular or perineural invasion and liver Distal pancreatectomy with or without splenectomy was or lymph node metastases which are known as pseudopa- performed in 11 (73.3%) cases, and Whipple’s pan- pillary carcinoma. A size of more than 5 cm, capsular in- creaticoduodenectomy was performed in 2 (13.3%) patients. vasion, high Ki-67 proliferation index, and growth into Although no patients in our study required vascular re- peripancreatic tissue are also linked with malignancy by a section, various studies have shown excellent long-term few authors [36]. Nishihara et al. proposed venous invasion, survival after vascular or multivisceral resection [34, 35]. high nuclear grade, and prominent necrobiotic nests as Extensive lymphadenectomy is not done always due to the indicators of malignancy [37]. Only one patient had a low incidence of lymph node metastases [12]. No patient perineural invasion in our study and is surviving 5 years after 6 Surgery Research and Practice (a) (b) (c) (d) (e) Figure 4: (a) Tumour cells arranged as solid nests and pseudopapillae with intervening hyalinised vascular channels (normal pancreas) (20x). (b) Tumour appears fairly delineated, however, in places it is intermixed with pancreatic acini at the periphery. 'e tumour is abutting the superficial fibres of the muscularis propria of the duodenum. (c) 'e tumour cells are polyhedral with mildly anisomorphic nuclei having longitudinal grooves, inconspicuous nucleoli, and moderate to scant amount of eosinophilic cytoplasm (40x). (d) Beta-catenin +. (e) Synaptophysin +. surgery without any recurrence. All our patients had a low foamy macrophages, and nuclear grooving in the absence of Ki-67 proliferation index of <1%. salt-pepper chromatin on histopathology are characteristics 'e most common complication following surgery was of SPN. Immunohistochemical markers are also peculiar to postoperative pancreatic fistula, probably due to the soft SPN and can be used to differentiate it from various con- nature of the pancreas, which was seen in 8 (53.3%) patients ditions like cystic neoplasms of the pancreas, pancreato- of which grade A fistula, that is a biochemical leak, was seen blastoma, acinic cell tumour, and neuroendocrine tumours [36]. Beta-catenin and Wnt signaling pathway have been in 6 patients while grades B and C were seen in one patient each. 'ere was one patient with mortality having grade C found to play an important role in the tumorigenesis and are fistula, following distal pancreaticosplenectomy due to sepsis consistently positive in 90% of cases of SPN, which is and haemorrhage from a gastroduodenal artery pseudoa- consistent with our study where beta-catenin was found to neurysm on POD 88. be positive in 86.6%. 'e other consistently positive markers On histopathology, SPN is a cellular neoplasm with cells were vimentin, neuron-specific enolase, and α1-antitrypsin. arranged in several layers around a fibrovascular stalk giving 'e neuroendocrine markers, except chromogranin A which a pseudopapillary appearance. 'e presence of a pseudo- is consistently negative in SPN, like synaptophysin, neuron- papillary structure, hyaline globules, cholesterol clefts, specific enolase, and CD 56 also showed variable expression Surgery Research and Practice 7 [37, 38]. 'e positive expression of the progesterone re- References ceptor was also seen in 53.35% of patients suggesting an [1] G. Kloppel, O. Basturk, D. S. Klimstra, L. Ak, and origin from ovarian cells. K. Notohara, “Solid pseudo-papillary neoplasm of the pan- 'e literature regarding the use of adjuvant chemotherapy creas,” in Tumors of Pancreas. 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Rare Pseudopapillary Neoplasm of the Pancreas: A 10-Year Experience

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Hindawi Surgery Research and Practice Volume 2021, Article ID 7377991, 8 pages https://doi.org/10.1155/2021/7377991 Research Article Rare Pseudopapillary Neoplasm of the Pancreas: A 10-Year Experience Suvendu Sekhar Jena , Samrat Ray , Sri Aurobindo Prasad Das , Naimish N Mehta , Amitabh Yadav , and Samiran Nundy Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, Old Rajinder Nagar, New Delhi 110060, India Correspondence should be addressed to Suvendu Sekhar Jena; suvu1078@gmail.com Received 3 June 2021; Revised 6 September 2021; Accepted 8 September 2021; Published 17 September 2021 Academic Editor: Cosimo Sperti Copyright © 2021 Suvendu Sekhar Jena et al. 'is is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Introduction. 'e solid pseudopapillary epithelial neoplasm (SPN) is a rare form of pancreatic neoplasm with an incidence of 2-3% of all pancreatic tumours. 'e recent increase in incidence is attributed to the increasing use of imaging techniques for nonspecific abdominal complaints. We report our institutional experience in the management of this tumour over the last decade. Method. We retrospectively analyzed from a prospectively maintained database of patients from January 2011 to December 2020 who were operated upon for SPN. All the patients were followed till date. Results. Of 479 patients operated on for various types of pancreatic tumours during this period, 15 (3.1%) had SPN. 'e mean age of presentation was 28 years with a female preponderance (12/15, 80%). 'e most common location was the body and tail of the pancreas (66%), and the mean size was 6.4 cm (2–15 cm). 'e tumour extent was defined as ‘borderline resectable’ in 20% of cases. Distal pancreatectomy was done in 11 patients with spleen preservation in 3. R0, R1, and R2 resection were done in 12, 2, and 1 patient(s), respectively. 'e operative mortality was 6.7%. All the patients are doing well on follow-up. Conclusion. SPN is a low-grade malignant tumour with a strong female predilection. Clinical manifestations have no specificity, imaging examination only contributes tumour location, and the final diagnosis rests on pathology. Surgery is the main modality of treatment and carries a good prognosis. complaints [3]. Cubilla and Fitzgerald reported an incidence 1. Introduction of SPN of 0.17% in 1979, Morohoshi et al. reported an Solid pseudopapillary neoplasms (SPNs) of the pancreas are incidence of 2.7% in 1987, and Koshmal et al. reported an defined by the new 5th edition of the World Health Or- incidence of 6.1% of all pancreatic tumours in 2004 [4–6]. ganization (WHO) Classification of Digestive System Tu- Lichtenstein was the first to report this entity, but Frantz in mors as low-grade malignant tumours composed of poorly 1959 described its pathology and Hamoudi et al. described cohesive epithelial cells which form solid and pseudopa- its electron microscopy features in 1970 [7–9]. Before being defined as a tumour of uncertain differentiation or solid pillary structures and lack a specific line of pancreatic ep- ithelial differentiation [1]. SPN incidence is around 2-3% of pseudopapillary neoplasm in 1996 by the World Health all pancreatic neoplasms. It has a female preponderance and Organization, it was also known as a solid cystic tumour, also malignant potential [2]. A total of 8334 cases have been papillary epithelial neoplasm, or papillary epithelial tumour reported in the English literature till 2018 with an increasing [10]. It is a rare tumour and has an estimated incidence of 2- incidence in recent times. 'is is attributed to the extended 3% of all pancreatic tumours and 6–12% of all pancreatic use of imaging techniques for nonspecific abdominal cystic neoplasms [3]. It usually affects women below the age complaints and their better availability since most of the of 40 years (2–85 years), with a male to female ratio of 7–11 : tumours are indolent and patients have nonspecific 1. In the paediatric age group, the incidence is 8–12.5%. 'e 2 Surgery Research and Practice usual location is the body and tail of the pancreas (55–60%) patient had hypertension as an associated comorbid illness followed by the head and neck of the gland (35–40%). It is with a mean Charlson comorbidity index of 8.7. usually associated with a favourable prognosis with a long- 'e majority of the patients presented with a mild, dull term disease-free survival of 95% [11]. SPNs are slow- aching type of pain (66.7%), while one patient was inci- growing tumours and have a low malignant potential, and dentally diagnosed while being evaluated for unexplained the usual symptoms are due to local manifestations of the anaemia when she was found to have a SPN along with a tumour. It may occasionally present with tumour rupture or uterine fibroid. One patient presented with severe abdom- metastasis. 'e origin of the disease is unknown with various inal pain and on evaluation was found to have a ruptured authors claiming different points of origin. Surgery is the SPN. 'ree patients presented with a lump in the abdomen. mainstay of treatmentwhich depends upon the location of 'e median size of the tumour was 6 cm (2–15 cm). 'e the tumour and ranges from distal pancreatectomy and location was the body and tail of the pancreas in 5, tail in 5, Whipple’s pancreaticoduodenectomy, to debulking. Ra- and body in 3, 1 each in head and uncinate process (Table 1). diotherapy and chemotherapy have very little or no role in Preoperative tumour markers like the CA 19-9 and its management [12]. We had previously published a case CEA, found to rule out primary pancreatic adenocarci- report of a young girl presented with ruptured SPN in 2016 noma or metastatic disease, were normal in all cases. 'e and highlighted various aspects of management [13]. 'e imaging modalities used were ultrasound (USG) abdomen present study was done to report our institutional experience and CECT abdomen. USG abdomen was done in 12 pa- in the management of this rare tumour over the last decade. tients, among whom the tumour was hypoechoic in 5, heteroechoic in 5, and isoechoic in 2 patients. CECT ab- domen revealed a borderline resectable lesion in 3 patients 2. Patients and Methods and resectable lesion in 12 patients. 'e tumour was solid We retrospectively analyzed, from a prospectively main- only in 8 patients, cystic only in 1 patient, and mixed solid tained database, all patients with a diagnosis of solid and cystic in 6 patients. 'e cystic areas probably indicated pseudopapillary epithelial neoplasm operated at the De- haemorrhage into the tumour. In 14 cases, the tumours partment of Surgical Gastroenterology and Liver Trans- were encapsulated. Calcification was seen in 12 patients plantation at Sir Ganga Ram Hospital, New Delhi, over a (Figure 1). Endoscopic ultrasound (EUS) was done as a part of the diagnostic procedure in 6 patients (Figure 2). 2 period of 10 years from January 2011 to December 2020. 'eir demographic data, associated comorbidities, pre- patients had a preoperative diagnosis other than SPN on senting complaints, preoperative blood parameters, and EUS, like islet cell tumour in one patient and pancreatic preoperative tumour markers like carcinoembryonic antigen cystadenoma in the other. A preoperative biopsy was done (CEA) and carbohydrate antigen 19-9 (CA 19-9) were an- in 8 patients, among whom 6 had fine-needle aspiration alyzed. 'e imaging results, i.e., the ultrasound and contrast- cytology via EUS and 2 underwent exploration and biopsy enhanced computed tomography (CECT) of the abdomen, somewhere else for a lump in the abdomen before coming were also analyzed. 'e type of surgery performed and its here (Table 2). complications along with the histopathology reports were All 15 patients underwent curative resection. 'e patient who presented with a tumour rupture underwent emergency also recorded, and the complications were graded according to the Clavien–Dindo classification (CDC) and compre- laparotomy due to haemodynamic instability and altered hensive complication index (CCI) [14, 15]. 'e postopera- sensorium. Upon exploration, around 1400 ml of blood was tive pancreatic fistula (POPF) was defined as per the found in the peritoneal cavity. 'e mass was found to be definition of an international study group of pancreatic adherent to the splenic flexure of the colon, spleen, and fistula. 'e patients were followed up till December 2020. transverse mesocolon with complete obliteration of the 'e pancreatic exocrine functions were assessed from the lesser sac. Because of her unstable condition, peritoneal symptoms and need for pancreatic enzyme supplementa- lavage and biopsy for the tumour were performed. She then tion, while the endocrine functions were assessed by the received four cycles of chemotherapy using vincristine need for insulin or any increase in the requirement of insulin (1.5 mg/m ), actinomycin D (0.045 mg/kg), and cyclophos- if already on insulin. 'e data were compared with those phamide (1200 mg/m ) to reduce the size of the tumour. 'e published from western countries as well as from India. tumour response was assessed with the USG abdomen. She Statistical analysis was performed using SPSS version 22.0 subsequently underwent distal pancreaticosplenectomy. software. Continuous variables were reported as mean- 'is procedure was performed in a total of 11 patients with ± standard deviation (±SD). Informed consent was taken spleen-preserving distal pancreatectomy in 3 patients. from the patients for the use of their data. Whipple’s pancreaticodudoenectomy was performed in 2 patients. One patient had a central pancreatectomy with Roux-en-Y reconstruction (Figure 3). None of the patients 3. Results underwent vascular resection or reconstruction. Of 11 pa- Of 479 patients operated for various types of pancreatic tients who underwent distal pancreatectomy with or without tumours during this period, 15 (3.1%) were operated for splenectomy, 6 patients underwent laparoscopic resection SPN. 'ey had a mean age of 28.13 (±11.2) years (11–47). Of while 5 underwent open resection. Multivisceral resection 15 patients, 12 were female and 3 were male. 'eir mean was not required in any of the patients. 'e average blood body mass index (BMI) was 22.37 (±3.85) kg/meter . Only 1 loss was 228 ml. A total of 4 patients received blood Surgery Research and Practice 3 Table 1: Demographic variables. 4. Discussion Variable n � 15 SPN is a rare pancreatic tumour, accounting for 1-2% of all Age in years (mean + SD) 28.13± 11.20 pancreatic neoplasms [16]. Unlike pancreatic adenocarci- Sex noma, these tumours are indolent and benign with a risk of Male 3 malignant transformation in around 10–15% [17]. 'eir cell Female 12 of origin is unknown. One theory suggests it originates from BMI (kg/m ) 22.37± 3.85 multipotent primordial germ cells lacking a definite dif- Presentation ferentiation of the exocrine or endocrine cell [18]. Various Symptomatic 14 authors have also described various sources of origin like the Incidentally 1 acinar cell and endocrine cell. Another theory suggests its Presenting symptoms origin from the incorporation of primitive ovarian cells Pain 113 within the pancreatic parenchyma during the seventh week Lump abdomen 1 of embryogenesis, but this does not explain its occurrence in Incidental males [19–22]. Tumour size in cm (median) 6.4 (2–15) It is frequently seen in young females in their second to Location of tumour fourth decade of life with a female preponderance of 80% Body 3 and mean age of 28.13± 11.2 years. Law et al. in their Tail 5 systematic review of 2744 patients revealed 87.8% incidence Body and tail 5 in females with an average age of 28.5± 13.7 years [23]. Head 1 Obesity had no relation with SPN in our study which was Uncinate process 1 also confirmed by Song et al. with an average BMI of SD: standard deviation; BMI: body mass index; n: number of patients. 23.7± 2.4 kg/m [8]. 'e average BMI in our study was 22.37± 3.85 kg/m (14.3–27.8). 'ere are some reports of an association between hepatitis B virus and SPN due to transfusion. 'e average duration of surgery was overexpression of beta-catenin in tumour cells, but none of 268.3± 145.8 min (Table 3). our patients had hepatitis B [24]. R0 resection was performed in 12 patients, while R1 'e symptoms are mostly nonspecific, and abdominal resection was performed in 2 patients. 'e patient, who pain caused by the mass is the most common manifestation presented with tumour rupture, initially underwent damage with nausea and vomiting being other symptoms [3]. 'e control surgery (R2) followed by tumour downsizing with subtle nature of their presentation leads to a delay in di- chemotherapy and complete resection. None of the patients agnosis, resulting in large tumours found at that time. had lymphovascular invasion, while 1 of them showed However, the size of the lesion does not affect resectability perineural invasion. On immunohistochemical (IHC) [25]. SPNs are usually bulky tumours (median tumour size staining, cells were stained positive for vimentin in 13 pa- 6 cm in our study). Despite their large size, they usually do tients and synaptophysin and beta-catenin in 11. Chro- not invade the surrounding structures but only displace mogranin was negative in all 15 patients (Table 4) (Figure 4). them, so symptoms like obstructive jaundice and pancrea- 'e Ki-67 proliferation index was less than 1% in all cases. titis are rare. Abdominal pain was the most common mode 'e average number of lymph nodes retrieved were 10 ( of presentation seen in 66.7% of our cases followed by a lump 2–24), and none of the nodes showed metastatic deposits. in 20%. Incidental detection was seen in one. Usually, it is 'ere was 1 patient with operative mortality. 'e patient restricted to the pancreas in approximately 85% of cases, underwent exploratory laparotomy, cholecystectomy, gas- while 10–15% of cases present with metastases [23]. 'e trojejunostomy, and biopsy of intra-abdominal mass else- most common sites of metastasis are the liver, regional where following which there was a bile leak followed by lymph nodes, omentum, and peritoneum [26]. None of our endoscopic stenting of the common bile duct. He then re- patients had metastasis during their presentation. ceived 3 cycles of chemotherapy and underwent distal Although rare, rupture of the tumour can also be a mode pancreaticosplenectomy in our hospital. He had a POPF that of presentation that can either be spontaneous or following was managed conservatively, and he was discharged with a blunt trauma to the abdomen. 'e reported incidence of drain in situ. Gradually, the drain output reduced and it was rupture in the literature is 2.7% with blunt trauma to the removed. He was readmitted with hematemesis and hem- abdomen being the most common cause [27]. 'e incidence orrhagic shock on postoperative day (POD) 45. On evalu- of spontaneous rupture is 1% of all SPN and thought to be ation, there was a pseudoaneurysm of the gastroduodenal due to sudden massive haemorrhage causing a rise in artery which was managed by angioembolization. He sub- pressure inside. One 11-year-old young girl presented to us sequently developed septicaemia and died on POD 88. with a sudden onset of abdominal pain with haemodynamic All the patients were followed up. At a median follow-up instability and underwent debulking because of bleeding and of 59 months (2–109), there was no recurrence. Except for infiltration of the surrounding structures. the patient who underwent damage control surgery, no 'e most common location of the tumour was the body patient received adjuvant chemotherapy. 'ere was no and tail of the pancreas in 80% of cases. Law et al. in their endocrine or exocrine insufficiency in any of the patients. meta-analysis showed 59.3% located in the body and tail of 4 Surgery Research and Practice (a) (b) Figure 1: (a) CECT abdomen showing an exophytic growth arising from the body and neck of the pancreas (yellow arrow) displacing the stomach with a peripheral curvilinear calcification (blue arrow). (b) Exophytic growth arising from the head of the pancreas (white arrow). Table 2: Diagnostic modalities and findings. Diagnostic modalities Incidence (n) Ultrasound (n �12) Hypoechoic 5 Heteroechoic 5 Isoechoic 2 CECT abdomen (n �15) Borderline resectable 3 Resectable 12 Encapsulated 14 Calcification 12 EUS Performed 6 Preoperative suspicion SPN 13 Others 2 Figure 2: A 3 × 4 cm lesion with a small cystic component arising Preoperative biopsy from the uncinate process of the pancreas. Performed 8 EUS guided 6 Exploratory laparotomy 2 the pancreas [23], and one of the largest meta-analyses Not performed 7 conducted by Yao et al. in 2450 Chinese patients also showed the most common location to be the body and tail of the pancreas. In contrast, Panieri et al. showed an equal spatial distribution of the tumour [28, 29]. 'e size of the defined in microcystic adenomas [32]. Ultrasonography, tumour or its location did not predict malignancy as re- CT scan, and MRI typically demonstrate the same char- ported by Yu et al. [30]. Ectopic locations have also been acteristics with an encapsulated tumour composed of solid described in the retro peritoneum, mesentery, and left and cystic elements, often with capsule rim-like calcifica- adrenal gland [28]. None of our patients had an ectopic tions as well as intraparenchymal calcifications. 'e lesion location. has well-defined margins often without pancreatic duct No additional information in our series was provided dilation. Ultrasonography demonstrates hypoechoic, iso- echoic, or heteroechoic components, while CT shows solid by the haematological investigations, while hyper- amylasaemia, elevated hepatic enzymes, and leukocytosis or cystic components with calcification. MRI and positron were occasionally identified [24]. In tumour markers like emission tomography can also be used. MRI is better than carbohydrate antigen 19-9 and carcinoembryonic antigen CT in various aspects like demonstration of a capsule and did not contribute to the diagnosis. In preoperative as- solid or cystic degeneration [33]. None of the radiological sessment, radiological studies are the most important. findings are specific for SPN, and similar findings can also Abdominal roentgenograms can demonstrate displace- be seen in other cystic neoplasms and pancreatoblastoma. ment of the stomach, colon, or spleen by an extrinsic mass Endoscopic ultrasound and fine-needle aspiration were [31]. Calcification is seldom encountered within the large used in 40% of cases. Preoperative tissue diagnosis is not mass. However, calcifications are peripheral and curvi- always necessary but can be used in cases of uncertain linear when present, as compared to the pattern of sunburst diagnosis [12]. Surgery Research and Practice 5 (a) (b) Figure 3: (a) Exophytic growth arising from the body and neck of the pancreas with reflected stomach anteriorly (white arrow) and normal pancreas (green arrow). (b) Whipple’s pancreaticodudoenectomy specimen; exophytic growth arising from the head of the pancreas with cystic areas (white arrow). Table 4: Immunohistochemical markers. Table 3: Operative parameters. IHC marker Positive (n) Negative (n) Variables n � 15 Vimentin 13 2 Surgery performed Beta-catenin 11 4 Distal pancreatectomy 11 Synaptophysin 11 4 Splenectomy 8 CD 10 9 6 Spleen preservation 3 PR 8 7 Whipple PD 2 Cytokeratin 6 9 Central pancreatectomy 1 NSE 5 10 Debulking 1 CEA 4 11 Laparoscopic resection 6 Chromogranin 0 15 Open resection 9 Blood loss (in ml) 228 PR: progesterone receptor; NSE: neuron-specific enolase; CEA: carci- Duration of surgery (min) 268.3± 145.8 noembryonic antigen. Blood transfusion 4 Hospital stay (days) 6 (3–16) Complication showed lymphovascular invasion in our study, and all the Clavien–Dindo grading 10 resected nodes were negative for tumour deposits. Meta- I 5 statectomy also improves long-term outcomes [12]. II 5 Debulking of a locally advanced tumour also has a role as III 0 shown by a previous case report from our institution [13]. IV 0 Enucleation is also another option for moving small tumours Comprehensive complication index 8.7 (0–36.2) Postoperative pancreatic fistula 8 away from the duct but carries a risk of fistulae and tumour Grade A 6 dissemination. Complete R0 resection was performed in Grade B 1 80% of patients, while 13.3% had an R1 resection. It is Grade C 1 difficult to differentiate between the malignant and benign Mortality 1 varieties as the criteria for malignancy is not clear cut. As per the WHO classification, the clear-cut criteria for malignant varieties are lymphovascular or perineural invasion and liver Distal pancreatectomy with or without splenectomy was or lymph node metastases which are known as pseudopa- performed in 11 (73.3%) cases, and Whipple’s pan- pillary carcinoma. A size of more than 5 cm, capsular in- creaticoduodenectomy was performed in 2 (13.3%) patients. vasion, high Ki-67 proliferation index, and growth into Although no patients in our study required vascular re- peripancreatic tissue are also linked with malignancy by a section, various studies have shown excellent long-term few authors [36]. Nishihara et al. proposed venous invasion, survival after vascular or multivisceral resection [34, 35]. high nuclear grade, and prominent necrobiotic nests as Extensive lymphadenectomy is not done always due to the indicators of malignancy [37]. Only one patient had a low incidence of lymph node metastases [12]. No patient perineural invasion in our study and is surviving 5 years after 6 Surgery Research and Practice (a) (b) (c) (d) (e) Figure 4: (a) Tumour cells arranged as solid nests and pseudopapillae with intervening hyalinised vascular channels (normal pancreas) (20x). (b) Tumour appears fairly delineated, however, in places it is intermixed with pancreatic acini at the periphery. 'e tumour is abutting the superficial fibres of the muscularis propria of the duodenum. (c) 'e tumour cells are polyhedral with mildly anisomorphic nuclei having longitudinal grooves, inconspicuous nucleoli, and moderate to scant amount of eosinophilic cytoplasm (40x). (d) Beta-catenin +. (e) Synaptophysin +. surgery without any recurrence. All our patients had a low foamy macrophages, and nuclear grooving in the absence of Ki-67 proliferation index of <1%. salt-pepper chromatin on histopathology are characteristics 'e most common complication following surgery was of SPN. Immunohistochemical markers are also peculiar to postoperative pancreatic fistula, probably due to the soft SPN and can be used to differentiate it from various con- nature of the pancreas, which was seen in 8 (53.3%) patients ditions like cystic neoplasms of the pancreas, pancreato- of which grade A fistula, that is a biochemical leak, was seen blastoma, acinic cell tumour, and neuroendocrine tumours [36]. Beta-catenin and Wnt signaling pathway have been in 6 patients while grades B and C were seen in one patient each. 'ere was one patient with mortality having grade C found to play an important role in the tumorigenesis and are fistula, following distal pancreaticosplenectomy due to sepsis consistently positive in 90% of cases of SPN, which is and haemorrhage from a gastroduodenal artery pseudoa- consistent with our study where beta-catenin was found to neurysm on POD 88. be positive in 86.6%. 'e other consistently positive markers On histopathology, SPN is a cellular neoplasm with cells were vimentin, neuron-specific enolase, and α1-antitrypsin. arranged in several layers around a fibrovascular stalk giving 'e neuroendocrine markers, except chromogranin A which a pseudopapillary appearance. 'e presence of a pseudo- is consistently negative in SPN, like synaptophysin, neuron- papillary structure, hyaline globules, cholesterol clefts, specific enolase, and CD 56 also showed variable expression Surgery Research and Practice 7 [37, 38]. 'e positive expression of the progesterone re- References ceptor was also seen in 53.35% of patients suggesting an [1] G. Kloppel, O. Basturk, D. S. Klimstra, L. Ak, and origin from ovarian cells. K. Notohara, “Solid pseudo-papillary neoplasm of the pan- 'e literature regarding the use of adjuvant chemotherapy creas,” in Tumors of Pancreas. 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