Solid Pseudopapillary Tumor of the Pancreas with Node Invasion: What to do? A Case Report and Literature Review

Case Report

Austin J Surg. 2022; 9(2): 1290.

Solid Pseudopapillary Tumor of the Pancreas with Node Invasion: What to do? A Case Report and Literature Review

Sekkat H1,2*, Moufid A1,2, Nadouri J1,2, Mesbahi OE4, Rimani M3, Bakali Y1,2, Alaoui MM1,2, Sabbah F1,2, Hrora A1,2 and Raiss M1,2

1Digestive Surgical Department C, Ibn Sina University Hospital, Morocco

2Faculty of Medicine and Pharmacy, Mohammed V University in Rabat, Morocco

3Hassan Pathology Center, Rabat, Morocco

4Nakhil Oncology Center, Rabat, Morocco

*Corresponding author: Hamza Sekkat, Digestive Surgical Department C, Ibn Sina University Hospital. Rabat, Morocco

Received: August 17, 2022; Accepted: September 13, 2022; Published: September 20, 2022

Abstract

Solid Pseudopapillary Neoplasms (SPN) of the pancreas are rare neoplasms representing 1-2% of all pancreatic tumors, and considered as low-grade malignancies. This pathology mainly affects young women. Its prognosis is usually excellent when the tumor is limited to the pancreas, with a cure rate greater than 95% after a complete surgical resection. Preoperative diagnosis is always difficult. SPNs can be metastatic. Hepatic and lymph node localizations are the most reported in the literature. The recurrence rate after surgical resection is 3-9%. We report the case of a 36-year-old patient who was complaining of abdominal pain for 2 months. An abdominal contrast-enhanced Computed Tomography (CT) scan showed a solido-cystic mass of the tail of the pancreas.

The patient underwent laparoscopic radical antegrade modular pancreatosplenectomy. The diagnosis of SPN with lymph node metastasis was confirmed by histopathology and immunohistochemistry, requiring adjuvant chemotherapy.

Keywords: Solid pseudopapillary tumor; Laparoscopy; Neoplasm; Node invasion; Recurrence; Pancreas

Introduction

The pseudopapillary tumor of the pancreas is an extremely rare exocrine tumor [1], representing only 1 to 2% of exocrine tumors of the pancreas, and 5% of cystic pancreatic tumors [2]. It was first described by Frantz in 1959 [3], then classified by the World Health Organization (WHO) in 1996 as a borderline tumor, called SPN (Solid pseudopapillary neoplasms) [43]. It generally affects young women, [5]. The body and tail of the pancreas are the most common sites of SPN [6]. The majority of SPN have a low-grade malignancy, with a good prognosis [7]. Most patients have localized tumors, and only 9-15% are being locally advanced or metastatic [3]. Due to the rarity of the tumor, there are no well-defined recommendations regarding the extent of tumor resection, lymph node dissection or management of metastasis [2].

We report a case of young woman with a Frantz tumor of the pancreatic tail, treated laparoscopically with lymph node metastasis in the pathological study. We discuss the appropriate therapeutic strategy and we report a literature review.

Case Report

A thirty six years old woman appeared in consultation for atypical pain in the left upper quadrant for 2 months. No not other associated digestive or extra-digestive signs were reported . The patient had no medical history otherwise and the physical examination was normal.

Abdominal ultrasound and an injected CT scan showed a tissue nodular formation adhering to the tail of the pancreas measuring 41 x 45mm, that respects the spleen and the left kidney, suspecting a SPN in the first place. No locoregional lymphadenopathy or ascites were identified (Figure 1). Tumor markers were normal.

Figure 1: Abdomen CT scans showing a well-defined; tissue nodular formation adhered to the pancreas tail measuring 41X45 mm.

    

    
    

    

    Figure 1: Abdomen CT scans showing a well-defined; tissue nodular formation adhered to the pancreas tail measuring 41X45 mm.

The patient was operated on laparoscopically. Exploration found a mass measuring 4 cm, developed on the inferior border of the pancreas tail with contact to splenic hilum (Figure 2).

Figure 2: Intraoperative pictures. A: A preoperative picture of the tumor after releasing the pancreas tail. B: A preoperative picture showing the tumor adhering to the spleen.

    

    
    

    

    Figure 2: Intraoperative pictures. A: A preoperative picture of the tumor after releasing the pancreas tail. B: A preoperative picture showing the tumor adhering to
the spleen.

In order to preserve the spleen, a splenic vessels liberation of the the pancreas was carried out, but suspicion of splenic invasion forced us to perform an associated splenectomy.

The specimen was extracted into a bag through a small pfannenstiel-type incision.

The postoperative period passed smoothly and the patient was discharged after four days.

The diagnosis of a solid tumor pseudopapillary pancreas was confirmed by histological study. One nine nodes of the splenic hilum dissection was metastatic. The tumor was classified pT3N1 (Figure 3).

Figure 3: Histopathological examination of the surgical specimen. A, B: Hematoxylin Eosin Safran (HES Coloration): Solid and cystic tumor. C: Papillary and pseudopapillary architecture. D, E: Metastatic lymph node. F: Immunohistochemistry: Progesterone (+): Intense and diffuse nuclear expression.

    

    
    

    

    Figure 3: Histopathological examination of the surgical specimen. A, B: Hematoxylin Eosin Safran (HES Coloration): Solid and cystic tumor. C: Papillary and
pseudopapillary architecture. D, E: Metastatic lymph node. F: Immunohistochemistry: Progesterone (+): Intense and diffuse nuclear expression.

Adjuvant chemotherapy was decided in a multidisciplinary meeting. The patient received 6 chemotherapy sessions, based on 5-fluoro-uracile, Irinotecan et Oxaliplatin. A Close follow-up was initiated for this patient. At 40 months after surgery, the patient is still alive, and has not shown any recurrence.

Discussion

The solid pseudopapillary tumor of the pancreas is a rare tumor that accounts for less than 2% of exocrine tumors of the pancreas [1]. It more frequently affects females between 20 and 30 years old. The rapid progression of the disease during pregnancy prompted the idea to study the relationship between SPN and women sex hormones [8]. It generally has a low malignant potential, but in males, the course is fatal and the prognosis is worse [9].

SPN went unrecognized for a long time. Up to the year 1999, only 334 cases were reported in the literature [10]. The more frequent use of CT and MR imaging, and the better knowledge of the pathology during the last two decades, has allowed the continuous diagnosis of this kind of tumor [11]. However, and despite this development, and technological revolution, the diagnosis of SPN is not always obvious. The non-specificity of the symptoms, [3] the similarity of imaging results between the cystic lesions [12], and the non-existence of specific markers of this tumor [13] always poses a problem of preoperative diagnosis. Some teams propose a preoperative radioguided biopsy for histological proof before surgical procedure, but this attitude is avoided by several others because of possible propagation risk of the tumor [14].

Of all laparoscopic pancreatic resections, distal pancreatectomy is the most commonly performed [10]. Radical Antegrade Modular Pancreatosplenectomy (RAMPS) is the procedure of choice for body and tail of the pancreas tumor resections. Although this technique has not shown superiority over the Retrograde Pancreatosplenectomy standard in terms of survival rate and recurrence-free survival, it allows to achieve high rates of a negative margin and resected metastatic lymph nodes [15,16]. Laparoscopic pancreatectomies for SPN frequency remains low, even if the procedure is considered relatively simple given that it does not require complex reconstructions of the digestive tract or anastomosis [3,17,18]. A series of studies have demonstrated the benefit of laparoscopic distal pancreatectomy over laparotomy. There are no significant differences in postoperative morbidity and mortality. Resection margins, lymph node yield and long-term survival are also the same [12]. However, laparoscopy allows postoperative pain reduction and therefore a lower need for analgesics, a shorter hospital stay and a faster return to normal activity. It also causes less wound complications, blood loss, and shorten operative time [19,20]. Furthermore, better aesthetic results are observed [21].

SPN exceptionally gives rise to metastases. The most common metastatic sites are liver and lymph nodes [12]. Several studies with the number of patients varying between 03 and 115 patients reported no cases of lymph node invasion after resection of a papillary pseudo solid tumor of the pancreas [2,6,8,10,22,23]. Larger series or literature reviews have reported lymphatic invasion rates varying between 0.5 and 15 percent [1,3,24-28] Yao et al in his study which contained 2450 cases cited in English and Chinese literature before 2020, found a percentage of lymph node metastases of 0.4% (12 patients) [29].

Lymph node metastases are considered as a risk factor for recurrence, and are one of the criteria for malignancy of SPNs [25,26].

However, the need for lymphadenectomy remains a controversial problem. Several authors consider it unnecessary to perform lymph node dissection associated with curative resection for a pseudo-solid tumor of the pancreas [2,23,30], while others strongly recommend it, as patients who have had a lymph dissection have a better prognosis [25,31].

There is no consensus neither on the adjuvant treatment protocol, in case of a solid pseudopapillary tumor of the pancreas with a high malignancy potential. The role of chemotherapy or radiotherapy remains vague and their effectiveness is poorly understood [32]. Several chemotherapy protocols have been used on different patients, with some good results [33]. Chemotherapy based on 5-fluorouracil and gemcitabine is the most used for a solid pseudopapillary malignant tumor [4,34]. This same treatment was a failure for Tajima in India, and the progression of liver metastases under chemotherapy prompted the team to propose Hepatic Arterial Infusion (HAI) chemotherapy, which was more effective [35]. Shimizu et al also reported a case that was sensitive to gemcitabine, docetaxel, paclitaxel, epirubicin, and mitomycin C [36], and it is quite the opposite of what Matsuda described in his case report, or this protocol was not very useful [37]. Shunrong Ji’s patient received 2 cycles of floxuridine and oxaliplatin after liver metastasis [33]. Other molecules like tamoxifen, cisplatin, ifosfamide, etoposide, and vincristine have been described by other teams [34,38,39], as well as several therapeutic methods, such as radiotherapy, transcatheter arterial chemoembolization, selective internal radiotherapy or even liver transplantation [4] In our case, the use of 5-fluoro-uracile, Irinotecan et Oxaliplatine, had a very good result, and our patient showed no recurrence 3 years and half after the surgery.

Conclusion

Solid pseudopapillary tumors of the pancreas are rare tumors with low potential for malignancy. Metastasis appearance, hepatic or lymph node in the majority of cases, impairs the prognosis. Surgery remains the treatment of choice for this type of tumor, but the lack of consensus on what to do in metastatic cases, should push us to carry out multicenter studies on an international scale in order to find the best way to take charge of these patients especially since it is a pathology that affects young healthy subjects.

Data Availability Statement

The data that support the findings of this article are available from the corresponding author upon reasonable request.

Competing Interests

The authors have no conflicts of interest and source of funding. The subject of the article had no commercial interest, no financial or material support.

Ethics Statement

Drs Hamza Sekkat, Abdellah Moufid, Jaouad Nadour, Omar El Mesbahi, Mouna Rimani, Younes Bakali , Mouna Mhamdi Alaoui, Farid Sabbah, Abdelmalek Hrora , Mohammed Raiss declare no conflict of interest.

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Citation: Sekkat H, Moufid A, Nadouri J, Mesbahi OE, Rimani M, Bakali Y, et al. Solid Pseudopapillary Tumor of the Pancreas with Node Invasion: What to do? A Case Report and Literature Review. Austin J Surg. 2022; 9(2): 1290.