With the widespread introduction of advanced endoscopic techniques in medical settings, the incidence of major duodenal papilla (MDP) neoplasms has been compared to the previous decade. [1-3, 13]. Regardless of the presence of the clinical manifestations, according to the majority of authors, MDP adenomas must be removed due to the potential risk of malignant transformation. [3-5].

With the development of surgical endoscopy, approaches to the treatment of MDP neoplasms have undergone remarkable changes towards greater use of minimally invasive intraluminal endoscopic techniques [8,9,14].

The spectrum of intraluminal surgical interventions for adenomas of MDP includes tumor removal using MDP resection and papillectomy, which, in the case of lateral spread of the tumor to the walls of the duodenum, can be supplemented by endoscopic mucosal resection (EMR) or submucosal dissection (ESD).

In the case of intraductal spread, high-frequency electrosurgical or radiofrequency ablation (RFA), as well as argon plasma destruction of the intraductal component of the tumor, can be used. [14]

Despite the remaining risks of postoperative complications, minimally invasive surgical procedures have increasingly gained recognition among researchers as the optimal method for the treatment of patients with neoplasms of the MDP due to the short hospital stays and rapid postoperative rehabilitation [8,9,14]. At the same time, the major surgical interventions, such as pancreaticoduodenectomy (also known as the Whipple procedure) and transduodenal papillectomy, used for the treatment of MDP tumors, showed an association with a high level of postoperative complications and mortality, reaching 25-45% and 8-10%, respectively [6,7,15,16]. Such aggressive therapeutic strategies should not be the primary therapeutic method for patients with benign tumors of the papillary region. That approach can be considered in the case of histologically confirmed malignancy of the MDP neoplasm [9].

For more than 20 years, the Department of Surgery of the National Research Center named after A.V. Vishnevsky has gained significant experience in the treatment and long-term follow-up of patients with major duodenal papillary adenomas. Based on our expertise in endoscopic diagnosis and treatment for patients, we have developed an endoscopic classification for the neoplasms of MDP, which allows us to choose the most optimal method of surgical intervention. This classification provides an accurate topical description of the tumor, taking into account its size, the pattern of growth, the extent of spread into the walls of the duodenum or the distal part of the common bile duct (CBD), and the main pancreatic duct (the duct of Wirsung).

The endoscopic classification of the neoplasms of MDP, which we have pioneered, relies on the data acquired through duodenoscopy and endosonography, in addition to routine radiological diagnostic methods such as CT and MRI. During preoperative duodenoscopy and endosonography, the presence and extent of spread of the extrapapillary and intraductal components of the tumor are assessed. The main proposition of our classification of MDP tumors is to clarify the indications for performing one or another endoscopic intervention and to define the limits of endoscopic and surgical methods of tumor removal.

From the year 2000 to 2022, 149 patients were diagnosed and treated with Major Duodenal Papilla (MDP) tumors in the Surgical Endoscopic Department at the National Medical Research Center of Surgery named after A.V. Vishnevsky. In the preoperative stage, in addition to computer tomography (CT) and magnetic resonance imaging (MRI), we performed a duodenoscopy with concurrent biopsy and endosonography. In order to assess the duodenal papilla region. This thorough assessment aimed to clarify the tumor size, the nature of its growth, the degree of spread into the walls of the duodenum or the terminal sections of the common bile duct (CBD) and MPD (main pancreatic duct), and the involvement of the CBD and the duct of Wirsung. A total of 134 endoscopic procedures were performed, with morphology reports confirming the presence of MDP adenomas in 126 cases.  In 61 patients, the neoplasms of the major duodenal papilla were represented by adenomas localized within the MDP without spreading to the walls of the duodenum.

In these cases, neoplasms were removed by resection of the MDP in a single block (en bloc resection) or in fragments (piecemeal resection). In another 30 cases, lateral spread of the adenoma to the walls of the duodenum was noted. For these patients, resection of the MDP was supplemented with loop endoscopic mucosal resection (EMR), and in some cases, endoscopic submucosal dissection (ESD) with preliminary submucosal lifting, as the optimal technique for removing the tumor with minimal risks of complications.

Among the remaining 35 cases, endosonography revealed invasion of adenoma to the ampulla of the MDP and the terminal sections of the CBD and/or main pancreatic duct, while only 7 patients in this group had intraductal adenomatous growth. In the majority of such observations, a combination of intra-ampullary or intraductal tumor growth with an extrapapillary component was noted.

These patients underwent endoscopic papillectomy, supplemented by electrosurgical ablation of the intraductal component, and in cases of extensive intraductal tumor spread of more than 10 mm, we used the intraductal RFA technique.

In 8 cases, the morphological study of extracted specimens was removed. Besides the parts of adenomas, there were also foci of adenocarcinoma identified, and therefore these patients were referred for resection interventions.

Another 15 patients with confirmed MDP adenomas were not operated on because of the small size of the tumors and the presence of aggravating concomitant diseases.

These patients are still under dynamic observation.

It is important to mention that the patients diagnosed in the preoperative stage showed signs of infiltrative tumor growth involving the muscular layer of the duodenal wall, and in the cases of morphologically confirmed adenocarcinoma, the endoscopic treatment was not chosen as a method of treatment.

Those patients were referred for the primary surgical investigation and were not included in the study.

Classification

Based on the analysis of endoscopic and EUS pictures of 149 patients, we developed an endoscopic classification of MDP neoplasms.

In this classification, we have identified 4 types of MDP neoplasms (presented in Table 1) depending on their size, growth patterns, and spread to the walls of the duodenum and the terminal section of the CBD and MPD.

Table 1: Distribution of the MDP Neoplasms by Growth Pattern and Spread to the Walls of the Duodenum and the Terminal Sections of CBD and MPD.

I Type (Extra Papillary): Extrapapillary growth pattern of the tumor (fig. 1). This type of neoplasm is localized within the MDP, with no signs of spread to the duodenum or the terminal segments of the common bile duct (CBD) or the main pancreatic duct (MPD). The choice of surgical intervention for this type depends on the size of the adenoma.

Figure 1: Type I Neoplasm (Scheme): Tumor with Extrapapillary Type of Growth, Localized Within the MDP, Without Any Signs of Spreading to the Duodenum or the MPD and CBD.

The optimal endoscopic intervention for adenoma sizes less than 2 cm is “en bloc” resection of the MPD. If the size of the tumor exceeds 2 cm, it is advisable to remove it using “piece-meal” (fragmentary) resection.

II Type (Extra Papillary + Duodenum): Tumor with extra papillary growth pattern and spread along the walls of the duodenum (Fig. 2) into the walls of the duodenum (Fig. 2). Depending on the direction of growth of the tumor spreading laterally on the walls of the duodenum, 4 subtypes are distinguished: SP (Supra Papillary)—spreading proximally to MPD, IP (Infra Papillary)—spreading distally to MDP, LPR (LateroPapillary Right)—lateropapillary spreading to the right of MDP, LPL (LateroPapillary Left)—lateropapillary spreading to the left.

Figure 2: Type II Neoplasm (Scheme): Tumor with Extrapapillary Growth Pattern with Spreading to the Walls of the Duodenum.

There are also 2 additional subtypes of type II: PD (Peri/Parapapillary Diverticulum)—when the adenoma spreads into the walls of the para- or peripapillary diverticulum, and IM (Invasion Muscle)—when there is a tumor invasion into the muscular layer of the duodenal wall (Fig. 3). For type II tumors, in addition to resection of the MDP, it is necessary to remove the laterally spreading component of the tumor by resection of the duodenal mucosa or dissection in the submucosal layer. If the length of the laterally spreading component of the tumor is more than 5 mm, the tumor should be removed in fragments in order to reduce the risk of complications.

Figure 3: Type II Tumor (Scheme): The Extrapapillary Component of the Tumor with Invasion into the Muscular Layer of the Duodenum.

A confirmed IM (invasion muscle) subtype, with EUS signs of invasion into the muscular layer of the duodenal wall, is a severe limitation for endoscopic treatment, since it indicates the malignant nature of the neoplasm.

III Type (Intra Ductal): Tumor with intraductal spread. Depending on the extent of involvement of ductal structures in the tumor, type III neoplasms are classified into 4 subtypes:

CBD (Common Bile Duct)—the tumor spreads into the terminal part of the CBD (fig. 4); MPD (Main Pancreatic Duct)—indicating neoplastic proliferation confined to the terminal region of the main pancreatic duct (fig. 5); IA (Intraampullary)—the tumor is represented exclusively by the intraampullary component (fig. 6); subtype CBD+MDP—the tumor spreads simultaneously to the terminal sections of both ducts (fig. 7).

Figure 4: Type III Neoplasm (Scheme): Spreading of the Tumor into the Common Bile Duct.

Figure 5: Type III Tumor (Scheme): Spreading of the Tumor into the Main Pancreatic Duct.

Figure 6: Type III Tumor (Scheme): Intraductal Tumor with Spreading into the Ampulla of MDP.

Figure 7: Type III Tumor (Scheme): Spreading of the Tumor into Both Ducts (CBD and MPD).

Type III tumors can be removed using both endoscopic and surgical techniques.

The optimal surgical procedure for the type IA neoplasm is endoscopic papillectomy.

When removing adenomas of CBD and MPD subtypes with tumor spread to ducts less than 10 mm, the optimal combination is papillectomy followed by destruction of the intraductal component of the tumor by performing high-frequency electrosurgical ablation or RFA.

If the intraductal proliferation of the adenoma is more than 10 mm, endoscopic intervention without the possibility of performing intraductal RFA in a medical center is not advisable due to the high risk of recurrence of the adenoma. In such cases, resection surgery should be considered. If the medical center has the technical proficiency to perform intraductal radiofrequency ablation, it is possible to carry out destruction of the intraductal component of the tumor, including the extended part, more than 10 mm. [10-12].

IV type (Extra Papillary + Intra Ductal): Tumor with mixed extrapapillary and intraductal growth pattern. Neoplasms of this type are generally a combination of types I and III (Fig. 8) or types II and III (Fig. 9). Removal of type IV tumors is possible according to the same principles that are used when removing type I, II, and III MPD adenomas, but taking into account their combination. For example, an MPD adenoma with an extrapapillary component less than 2 cm, without spreading to the walls of the duodenum, but involving the terminal part of the CBD for less than 10 mm, according to endoscopic typing, is related to mixed type IV (type I (EP) + type III (ID), subtype CBD). For such a tumor, the optimal choice is to perform en bloc, followed by high-frequency electrosurgical or radiofrequency ablation of the intraductal component of the adenoma.

Figure 8: Type IV Tumor (Scheme): Tumor with Mixed Extrapapillary and Intraductal Growth Pattern (Combination of Type I and III).

A -The Tumor is localized within the MDP with Spreading into the Common Billie Duct; B – The Tumor iLocalized within the MDP with Spreading the Main Pancreatic Duct; C – The Tumor is localized within the MDP with Spreading into the Both Ducts (CBD and MPD).

Figure 9: Type IV Tumor (Scheme): Tumor with Mixed Extrapapillary and Intraductal Growth Pattern (Combination of the Types II and III).

A- The tumor is spreading into the duodenum and the common bile duct; B – the tumor is spreading into the duodenum and the main pancreatic duct; C – the tumor is spreading into the duodenum and the both ducts (CBD and MPD).

In accordance with advanced research, the anatomical and physiological characteristics of the major duodenal papilla zone are a predisposing factor to the formation of various tumors, including adenomas. The main reason behind the tendency for proliferative processes is the direct contact of different types of epithelium in the zone of terminal segments of the common biliary duct and the main pancreatic duct when they flow into the duodenal passage [1]. Because of the high risk of malignancy, all MDP adenomas must be removed regardless of their histopathological structure. The main problem in choosing the types of operation for MDP adenomas is the challenge of determining the borders of the tumor, which limits the use of endoscopic methodologies. Up to date, the absence of endoscopic classification of MDP neoplasms has not made it possible to clearly outline systematized standards for choosing one or another kind of endoscopic or surgical investigation. The outcome of a prolonged miscellaneous approach to the selection of endoscopic intervention types in the absence of a standardized classification system has been a high level of complications during the endoscopic excision of major duodenal papilla tumors. According to the publications, such difficulties as post-manipulation pancreatitis, pancreatic necrosis, hemorrhaging, proliferation of the duodenal wall, and combinations of these complications result in a high rate of frequency when endoscopic procedures are performed [8,9,14]. However, taking into account a number of recent research studies, endoscopic investigations are recognized as the first-rate method of treating patients with MDP tumors due to their minimal invasiveness and short rehabilitation period [1,8,9,14]. The proficiency of the endoscopic classification system for tumors of the major duodenal papilla that we determined has demonstrated the clinical importance of its grading. The primary advantage of our classification is the possibility of choosing a preoperative stage on an individualized basis, optimal upon the specific growth pattern of the tumor. This approach allows specialists to clearly determine the tactics for managing patients with MDP tumors and minimize the risk of complications.

The endoscopic classification presented by us makes it possible to categorize MDP tumors depending on the characteristics of growth as well as their topographical and anatomical attributes. This classification allows for standardized criteria by choosing the optimal selection of surgical resection of these tumors. The implementation of this classification into clinical practice allows us, based on preoperative endoscopic examination of patients, to select the optimal volume of endoscopic excision of MDP tumors with the most conservative resection within healthy tissue and minimize the risk for postoperative complications.

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