Combined two-stage total pancreatoduodenectomy and esophagectomy for synchronous malignancy of the pancreatic corpus and the esophagus: A surgical case report

Abstract

Introduction

Synchronous visceral malignancy is rare especially for esophagogastric junction adenocarcinoma combined with malignancy in the pancreas. So far only 7 cases of combined partial pancreatoduodenectomy and esophagectomy for synchronous malignancy have been described in the literature and none for combined total pancreatectomy and esophagectomy.

Presentation of case

We report the case of a 67-year-old male patient, who underwent multi-modality treatment including two-stage total pancreatoduodenectomy and subsequent Ivor-Lewis esophagectomy for synchronous adenocarcinoma of the distal esophagus and pancreatic multilocal metastases of a renal cell carcinoma after nephrectomy 17 years ago. Pathology revealed R0 resections for both malignancies and there were no postoperative complications. A 12 months follow-up showed no signs of recurrence and a good quality of life.

Conclusion

Curative-intent, combined oncological two-stage open total pancreatoduodenectomy and esophagectomy with several days interval is safe and feasible in selected cases when performed by an experienced interdisciplinary team in a high-volume surgical center.

Highlights

• •Synchronous visceral malignancy in esophagus and pancreas is extremely rare.
• •These cases will often be deemed irresectable and receive only palliative radiochemotherapy.
• •Here we present a combined two-stage total pancreatoduodenectomy and esophagectomy.
• •To our knowledge, this is the first procedure of its kind ever reported in literature.
• •There were no complications and no recurrence at 1-year follow-up with a good quality-of-life.

1. Introduction

Multiple cancer disease refers to cases in which more than one malignancy is diagnosed in the same patient, either simultaneously or sequentially. Metachronous multiple cancer disease is characterized by two or more malignancies, while synchronous multiple cancer disease is characterized by two or more malignancies diagnosed within six months [1]. The incidence of multiple cancer disease is low. However, due to the increasing patient age and improved access to healthcare, the prevalence of two independent malignancies simultaneously existing within one patient is rising. Usually in cases of multiple cancer disease, there is metachronous malignancy with involvement of endemic widespread cancer diseases or derived metastases. Examples of these endemic widespread cancer diseases include hematologic cancer, prostate cancer or breast cancer and oncological outcome has significantly improved for these over the last decades [2].

Synchronous malignancies are still unusual [3]. Especially the case of synchronous esophageal carcinoma and multiple pancreatic metastases from a former renal cell carcinoma (RCC) has not been described in the literature so far and guidelines or even evidence-based concepts are missing.

Curative treatment for the combination of these two entities can only be radical surgical removal. However, standard procedures of one-stage total pancreatoduodenectomy and esophagectomy applying standard gastric conduit reconstruction are not compatible. Consequently, an adapted surgical strategy for this highly individual approach had to be derived preoperatively. A solution can be total pancreatoduodenectomy and esophagectomy with colonic interposition. Pancreatoduodenectomy is a relatively high-risk surgical procedure with a morbidity rate of 20.8–68.2 % and a mortality rate of 0–7.1 % [4]^, [5]. Esophagectomy with colonic interposition is also a high-risk surgical procedure with a high rate of morbidity (26.5–75 %) and mortality rate (0–16.7 %) [6].

A one-stage operation including these surgical procedures would be an extremely invasive operation with potentially combined risk of intraoperative as well as postoperative complications and even death. Therefore, a two-stage approach was chosen, which was originally introduced for high-risk patients with esophageal cancer [7]^, [8].

Patient treatment and management was performed at a high-volume academic surgical center in Germany and with an interdisciplinary team including surgical staff, radiologists, oncologists and experienced intensive care physicians.

This case report is supposed to provide inspiration and guidance for future comparable cases and has been reported in line with the CARE criteria [9] and SCARE criteria [10].

2. Case presentation

The patient is a 67-year-old retired Caucasian male with a height of 180 cm, a weight of 82 kg, a body surface area of 1.98 m² (Du Bois) and a body mass index of 26.7 kg/m². At in-domo presentation the patient had already received 4 cycles of FLOT chemotherapy (5-fluorouracil, folic acid, oxaliplatin & docetaxel) and was in relatively good athletic shape (Eastern Co-operative Oncology Group (ECOG) Performance Status 1). There were no suspicious clinical findings in the extensive physical examination and no complaints or symptoms. There was no history of drug abuse and no suspicious family or psychosocial history. The patient was a former smoker (50 pack years). External documentation showed the following diagnoses:

Primary diagnoses:

• - 1.
  Esophagogastric junction (EGJ) adenocarcinoma Type I (histologically confirmed diagnosis)

  • o
    Initially: cT2 cN0 cM1 (LYM) (presumably one involved right hilar lymph node)
  • o
    After neoadjuvant treatment: ypT1b ypN0(0/18) M1 (LYM) L0 V0 Pn0 G1
• - 2.4 pancreatic lesions which, due to their contrast agent behavior, would be compatible with either metastases of a RCC or less likely primary neuroendocrine tumors of the pancreas

Secondary diagnoses:

• ‐Right nephrectomy and adrenalectomy for RCC (R0 resection) in 2005
• ‐Thyroidectomy for benign thyroid tumor in 2014
• ‐Arterial hypertension & hypercholesterinemia
• ‐Segmental pulmonary artery embolism in July 2021
• ‐Idiopathic polyneuropathy

Preoperative medication:

• ‐Apixaban 5 mg 1-0-1 (due to pulmonary artery embolism diagnosed in July 2021)
• ‐Bisoprolol 2,5 mg 1-0-0
• ‐Simvastatin 20 mg 0-0-1
• ‐Pantoprazole 40 mg 1-0-1
• ‐L-Thyroxine 175 μg 1-0-0

3. Oncological timeline

The oncological timeline is provided in days before and after surgery with the day of the first surgery as reference day 0 (Fig. 1). The in-domo presentation of the patient was 18 days prior to surgery in order to obtain a second opinion on a surgical strategy for his case. He had already received external diagnostics including computed tomography (CT), magnetic resonance imaging (MRI), pancreatic biopsy and transbronchial biopsy and was put on chemotherapy with FLOT. The complete timeline of this oncological treatment is provided below:

• ‐Day-188: Initial hospitalization and thoracic CT due to shortness of breath with detection of segmental pulmonary artery embolism and a right hilar lymph node enlargement of up to 19 mm.
• ‐Day-184: Abdominal CT with detection of hard to delimit, but tumor-suspect pancreatic lesions (head: 29 mm, tail: 18 mm and 26 mm).
• ‐Day-176: Esophagogastroduodenoscopy with bioptic evidence of EGJ adenocarcinoma 42–45 cm from dental arch; carcinoembryonic antigen (CEA) at 9.3 ng/ml.
• ‐Day-175: MRI of the pancreas with detection of 4 pancreatic metastases presumably of the former RCC (head: 29 mm, corpus: 19 mm, tail: 16 mm & 21 mm, EGJ adenocarcinoma cannot be distinguished).
• ‐Day-166 to -76: 4 cycles of neoadjuvant chemotherapy with FLOT.
• ‐Day-70: Endosonographic fine needle aspiration of pancreatic lesions without successful histopathological identification of tissue entity.
• ‐Day-30: Bronchoscopy & transbronchial needle aspiration of right hilar lymph node without detection of malignant cells.
• ‐Day-18: First in-domo case presentation with decision for surgery.
• ‐Day-18: Abdominal & thoracic CT with constant oncological findings with a tendency of increased pancreatic tumor burden (head: 27 mm, corpus: 18 mm, tail: 21 mm & 26 mm) and a suspicious right hilar lymph node with 22 mm.
• ‐Day 0: First surgery.
• ‐Day 5: Second surgery.
• ‐Day 0 to 14: Treatment on intermediate care unit.
• ‐Day 18: Hospital discharge.
• ‐Day 137: Adjuvant ablative 6-MV-photon-radiation-therapy with right-hilar suspicious lymph node as target volume with 5 Gray single dose and 60 Gray cumulative dose.
• ‐Day 360: 1-year follow-up abdominal & thoracic CT with no sign for recurrence.

Fig. 1.

Oncological timeline. Timeline with all relevant numbers of days. The day of first surgery is used as reference. Visualization is not true to scale and only for illustrative purposes.

4. Diagnostic assessment

The patient had already received external diagnostics including CT, MRI, pancreatic biopsy and transbronchial biopsy when presenting at our outpatient clinic for oncological consultation. He received final preoperative thoracic and abdominal CT imaging.

4.1. Gastroscopy

Bioptic evidence of EGJ adenocarcinoma 42–45 cm from dental arch.

4.2. MRI

Detection of 4 pancreatic lesions (presumably metastases) of the former RCC (head: 29 mm, corpus: 19 mm, tail: 16 mm & 21 mm, EGJ adenocarcinoma cannot be distinguished).

4.3. Endosonographic fine needle aspiration of the pancreatic lesions

Tissue neither representative nor sufficient for histopathological evaluation.

4.4. Bronchoscopy & transbronchial needle aspiration of right hilar lymph node

No detection of malignant cells.

4.5. Preoperative abdominal & thoracic CT

• ‐Cancer location 1 (esophagus): CT-morphologically no tumor-specific wall thickening of the gastroesophageal junction can be delineated.
• ‐Cancer location 2 (pancreas): minimally increased pancreatic tumor burden (head: 27 mm, corpus: 18 mm, tail: 21 mm & 26 mm)
• ‐Lung and pleura: 6 mm nodulus in right upper lobe, identical compared to previous CT; no other new noduli; not suspicious regarding morphology.
• ‐Thoracic lymph nodes: metastasis in right hilum increasing in size (23 mm; previously 20 mm).
• ‐Liver, peritoneum, abdominal lymph nodes and bone: free from metastasis.
• ‐Evaluation: Oncological findings are largely constant with a tendency of increased tumor burden compared to the previous CT. No signs of remnant pulmonary arterial embolism (Fig. 2).

Fig. 2.

CT images of esophageal cancer and clear cell RCC pancreatic metastasis after neoadjuvant treatment. a, EGJ I carcinoma (not clearly identifiable in CT). b, metastases 1 and 2 in pancreatic corpus and tail. c, metastasis 3 in pancreatic tail. d, metastasis 4 in pancreatic tail. e, depiction of pancreatic metastases with frontal view. f, 3D-visualization with metastases in green color. Orange arrows indicate location of malignancy. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)

5. Therapeutic intervention

Due to the invasiveness of the procedure, surgery was planned as a two-stage approach with several days in between assumingly with the necessity for intermediate care treatment.

5.1. First surgery

• 1.Total pancreato-duodenectomy, splenectomy, partial omentectomy and cholecystectomy with end-to-side hepaticojejunostomy and with blind closure of the stomach plus creation of a percutaneous gastric outlet fistula.
• 2.Complete mobilization of the colonic frame, dissection of the median colonic vessels, right hemicolectomy due to extensive scarring and subsequent deserosations during preparation and terminal ileostomy as well as the mobilization of the transverse mesocolon and preparation of a transverse colonic interposition pediculated to the left colonic vessels for subsequent esophageal replacement. The patient was transfused with 4 fresh frozen plasma and lost about 1800 ml of blood. Detailed information on surgical aspects of the procedure can be found in Fig. 3 and Supplement Text 1.

Fig. 3.

Schematic drawing of anatomical situations. a-c, gastrointestinal anatomy. d-f, vascular colonic anatomy. a, d, preoperative situation. b, e, anatomic situation after first procedure. c, f, anatomic situation after second procedure. Orange indicates malignancy, red indicates arteries, green indicates gastric fistula, black circle indicates stoma. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)

5.2. First intermediate care treatment (for 5 days)

The Patient was extubated and awake with only minor oxygen demand of 2 l per minute for a saturation >95 %. Diuresis was regular. Drainage fluid was unsuspicious and the amount constantly decreasing. Hemostasis was physiological and hemoglobin was stable. Regular control of blood sugar levels was performed and the patient was started on insulin therapy.

5.3. Second surgery (5 days later)

• 1.Reexploration, abdomino-thoracic esophageal resection and reconstruction through isoperistaltic elevation of the transverse colon pediculated to the left colonic vessels with end-to-side intrathoracic esophago-transversostomy and end-to-side descendo-jejunostomy as interposition.
• 2.Side-to-side jejuno-jejunostomy as a Braun's entero-anastomosis.
• 3.Terminal ileostomy closure and side-to-side ileo-descendostomy.

Detailed information on surgical aspects of the procedure can be found in Fig. 3 and Supplement Text 2.

5.4. Second intermediate care treatment (for 9 days)

Oral intake was started gradually on postoperative day 5 (day 10 after initial surgery) with clear fluid. The thoracic drainage could be removed on postoperative day 8 (day 13 after initial surgery). The gastric tube could be removed on postoperative day 9 (day 14 after initial surgery). Gastrointestinal passage was intact and there were no subjective complaints. The patient could be transferred to regular ward on postoperative day 9 (day 14 after initial surgery), where he was trained on insulin therapy and discharged from hospital on postoperative day 13 (day 18 after initial surgery). There were no postoperative complications (Table 1).

Table 1.

Laboratory values over postoperative course.

In-hospital day	Postoperative day	Leukocytes [×1012/L]	CRP [mg/L]	Kreatinine [mg/dL]	Sodium [mmol/L]	Potassium [mmol/L]	Calcium [mmol/L]	Hemoglobin [g/dL]	Thrombocytes [/nL]
1	-1	6.03	2.3	1.01	142	4.38	2.3	–	199
2	0	10.24	–	1.08	142	5.02	2.27	11	149
3	1	11.17	62.8	1.13	139	4.68	2.15	10.7	160
4	2	11.48	74.1	1.08	140	4.70	1.94	9.6	161
5	3	8.78	67.0	1.06	140	4.32	2.17	9.1	142
6	4	9.77	45.1	0.98	141	4.25	2.09	9.2	168
7	(0) 5	8.58	44.3	1.12	141	4.08	2.09	9	217
8	(1) 6	11.39	113.4	1.49	143	4.26	1.94	9.6	210
9	(2) 7	7.41	185.8	1.32	141	4.47	1.74	8.5	256
10	(3) 8	4.33	141.4	1.13	142	4.63	1.97	8	317
11	(4) 9	5.20	96.5	1.01	141	3.93	1.97	7.7	361
12	(5) 10	7.33	75.3	0.98	136	4.26	2.03	8.4	415
13	(6) 11	8.43	56.2	0.81	130	3.85	1.98	8.8	536
15	(8) 13	10.07	39.4	1.25	132	4.77	1.99	9.9	833
16	(9) 14	9.54	40.9	1.17	133	4.78	1.92	9.7	826
19	(12) 17	10.30	17.7	0.98	134	4.67	1.86	9.9	1109
–	(37) 42	5.74	2.0	1.21	135	4.65	2.14	10.7	417

Postoperative medication:

• ‐Bisoprolol 2,5 mg 1-0-0
• ‐Simvastatin 20 mg 0-0-1
• ‐Pantoprazole 40 mg 1-0-1
• ‐L-Thyroxine 175 μg 1-0-0
• ‐ASS 100 mg 1-0-0 in case of thrombocytosis over 1000/nL
• ‐Kreon 25.000 IE before oral intake and adjusted according to demand
• ‐Intensified insulin therapy (combined short- and long-acting)

6. Follow-up and outcomes

6.1. Histopathological evaluation of the esophageal specimen

Gastroesophageal specimen with a well-differentiated adenocarcinoma of the esophago-gastric transition, with infiltration of the submucosa of the esophagus, apparently originating on the floor of a chronic reflux-related inflammation with Barret's mucosa. Tumor-free esophageal margin of specimen and tumor-free gastral resection margin. The resection was performed locally in healthy tissue. No evidence of lymphatic, vascular, or perineural sheath invasion. 18 tumor-free perigastric lymph nodes with sinus histiocytosis and multiple, non-necrotic granulomas, compatible with a sarcoid-like reaction.

6.2. Final tumor classification

TNM (8. Version): pT1b; pN0 (0/18), L0, V0, Pn0; Grading: G1; R-classification (locally): R0.

Degree of tumor regression (Becker-scheme): Grade 2 (>10 and <50 % of vital residual tumor).

6.3. Histopathological evaluation of the pancreatic specimen

Total pancreatectomy specimen with formations of a clear cell carcinoma, according to the conventional morphologic and immunohistochemical findings, well probably to be a metastatic lesion of the former clear cell RCC (Fig. 4). Pancreatic parenchyma with pancreatic intraductal neoplasia, with low-grade epithelial dysplasia (PanIN low-grade). Peritumoral chronic pancreatitis, with fibrosis and atrophy. Bile duct dissection margin tumor-free. Oral and aboral small bowel dissection margins tumor- and dysplasia-free, with chronic inflammatory infiltrates at the level of the mucosa. Tumor-free spleen. 43 tumor-free peripancreatic lymph nodes, with numerous granulomas, consistent with a sarcoid-like reaction.

Fig. 4.

Pathohistological samples. a, Axial section of the pancreatic head. A solid, yellow and well-defined tumor with a 2 cm diameter could be identified (orange arrow). b, Hematoxylin-Eosin-stained histologic section of the pancreatic head showed a non-capsulated, solid carcinoma, composed of large clear cells. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)

Microscopically, nodular configured epithelial tumors are seen in the pancreatic parenchyma, with a predominantly solid and nest-like growth pattern. The tumor cells are polygonal, with clear, broad cytoplasm and a distinct cell membrane. Nuclei are ovally-round and relatively monomorphic. Hyaline tumor stroma with dense capillary network, fresh hemorrhage, and signs of older hemorrhage. Evidence of blood vessel invasion. Adjacent dilated lateral ducts lined by partly flat, partly papillary epithelium. Epithelial cells cuboidal, with pale to eosinophilic cytoplasm and monomorphic, round, basally arranged nuclei. Peritumorally, there is periductal, inter- and intralobular connective tissue fiber proliferation of the pancreatic parenchyma and atrophy of the acini. In the immunohistochemical staining, the tumor cells showed nuclear positivity for PAX8, with negativity for synaptophysin and CK7 (Fig. 5).

Fig. 5.

Immunohistological stainings. a, axial section of the pancreatic tail. A second solid, yellow and well-defined tumor with a 1,5 cm diameter was identified. b, Histologic section of the pancreatic tail showed a non-capsulated, solid carcinoma, composed of large clear cells. c, PAX8 showed a nuclear expression in tumor cells, as expected for a renal cell origin. d-e, the tumor was negative for cytokeratin 7 and Chromogranin A, therefore a potential neuroendocrine tumor with clear cell change could be excluded. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)

Investigation of the inked resection borders depict infiltration of the peripancreatic fat with a fine layer of intact fibrous tissue (R0) (Fig. 6a). Vascular invasion is typical for metastases of clear cell RCC and can often be seen in the peripancreatic fat tissue, but was completely resectable in this case (Fig. 6b).

Fig. 6.

Pathohistological samples. a, Tumor foci showed infiltration of the peripancreatic fat. In this section the dorsal margin of the pancreatic tail was inked. In the inset a fine layer of fibrous tissue separates the invasive front from the inked surface, corresponding to a complete surgical resection (R0). b, Vascular (venous) invasion is the most frequent metastatic pathway in clear cell RCC. Vascular invasion is observed very often in the vessels of peripancreatic fat tissue. Intravascular foci can reach significant volume and originate multiple nodules dispersed in the peripancreatic tissue, simulating lymph node metastases.

The 12-month follow-up provided no signs of clinical recurrence. The patient was in good general condition with sufficient diabetes control and no complaints or symptoms other than having to eat several small meals a day in order to not feel nauseous. He had a full score on the Katz scale for activities of daily living and was independent and mobile. Radiological imaging showed no signs of recurrence. The anastomoses continue to be sufficient. There was a peritruncal soft tissue accumulation with no signs of malignancy, a known avital lymph node metastasis in right hilum with decreasing size, a constant nodulus in the right upper pulmonary lobe as well as a hyperdensity in right lower pulmonary lobe constant in size (Fig. 7).

Fig. 7.

CT images of the 1-year follow-up. a-b, depiction of transverse-colon-interposition (red arrow indicates anastomosis and transverse colon). c, peritruncal reactive soft tissue of postoperative origin without signs of malignancy. d, known avital lymph node metastasis in right hilum with decreasing size. e, constant nodulus in the right upper pulmonary lobe. f, constant perifissural, fusiform hyperdensity in right lower pulmonary lobe. No radiological signs of recurrence. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)

The patient is scheduled for annual oncological follow-up visits including radiology and laboratory values with no further adjuvant therapies planned.

7. Discussion

Even in large-scale reviews, a synchronous pancreatic lesion diagnosed in patients presenting with esophageal carcinoma is rare with approximately 0.07 to 0.1 % of patients affected [11]^, [12].

Regarding the two independent, synchronously identified malignancies in this patient, there are some interesting considerations worth pointing out. While EGJ adenocarcinoma is already a common cancer entity, its incidence has still been increasing significantly over recent years especially in Western societies [13]. Yet, more interesting are the pancreatic metastases as an expression of recurrence of a clear cell RCC decades after initial curative nephrectomy. According to the literature this seems not to be an uncommon behavior [14]^, [15]^, [16]^, [17]. Although metachronous pancreatic lesions represent a particular group correlating with a rather indolent course of RCC, the occurrence of pancreatic metastases decades after nephrectomy is associated with a more preferable prognosis compared to short-term metastases or metastases in other locations [18]^, [19]. Unlike patients with short-term lymphatic or hepatic metastases from other cancer entities, patients with resected long-term pancreatic metastasis from RCC present an explicitly high survival rate of up to 88 % at 5 years [18]. This might be due to the fact that usually there is no involvement of peripancreatic lymph nodes. Radical complex peripancreatic lymphadenectomy is therefore not necessary and R0 resection can be achieved more effectively, which explains the excellent long-term survival achieved with surgery.

Despite immense developments in adjuvant therapy, surgical resection is usually the only option for cure in both entities. With the complexity of these procedures in mind, data has demonstrated reduced operative mortality at high volume centers [20]^, [21]. Despite the poor quality of available evidence and the great interstudy heterogeneity, combined procedures may be feasible with acceptable safety and satisfactory oncologic outcomes on individual basis [22]. However, these procedures have to be seen as salvage therapies with relevant intraoperative and perioperative morbidity and mortality risk for the patient. Patients have to understand these risks during consent and the procedures have to be reserved for the most experienced surgeons at renowned high-volume centers with tight affiliations to experienced intensive care physicians.

Specifically for simultaneous esophageal and pancreatic resection, there are 7 cases reported for synchronous malignancies [23]^, [24]^, [25]^, [26]^, [27]^, [28]^, [29] and 1 case reported for single esophageal malignancy with chronic pancreatitis [30]. All of these cases were partial pancreatic resections with pancreato-jejunostomy for reconstruction (Table 2). The combination of Ivor-Lewis esophagectomy and total pancreatectomy has never been reported in the literature before.

Table 2.

Other case reports with esophagectomy and pancreatectomy (pancreatic head resection or total resection) for synchronous malignancies. pr is pylorus-resecting. pp. is pylorus-preserving. PD is pancreatoduodenectomy. TTE is transthoracic esophagectomy. THE is transhiatal esophagectomy.

Author, year	Title	Diagnosis	Resection	Reconstruction	Complications & oncological result
de la Vega et al. 2021 [23]	Combined one-stage esophagectomy and duodeno-pancreatectomy for synchronous cancers of the esophagus and pancreatic ampulla in an elderly patient https://pubmed.ncbi.nlm.nih.gov/31230557/	‐Adenocarcinoma of the distal esophagus ‐Adenocarcinoma of the ampulla of Vater	TTE, total gastrectomy, pr-partial PD (one-stage)	Right hemicolon interposition	Comp: jejunal perforation (erosion by jejunostomy catheter) requiring relaparot-omy for over-sewing on POD 10 (Dindo-Clavien IIIB); anemia with transfusion of 2 red blood cell concentrates Onc: R0, but 2 biliary liver metastasis at 12 months
Fukaya et al. 2014 [24]	Two-stage operation for synchronous triple primary cancer of the esophagus, stomach, and ampulla of Vater: report of a case https://pubmed.ncbi.nlm.nih.gov/23504004/	‐Ampullary cancer ‐Middle thoracic esophageal cancer ‐Early gastric cancer on anterior wall of lower gastric body	1. Right transthoracic subtotal, external neck esophagostomy, gastrostomy 2. Total gastrectomy, TTE, pr-partial PD (two-stage; 35 days later)	Ileocolonic interposition via the percutaneous route without vascular anastomosis	Comp: Grade B pancreatic fistula with drainage (Dindo-Clavien IIIA) Onc: hepatic metastases 5 months postop; death 12 months postop
Kim et al., 2011 [25]	Combined esophagectomy and pancreatoduodenectomy: expanded indication for supercharged jejunal interposition https://pubmed.ncbi.nlm.nih.gov/21541769/	‐Pancreatic cancer ‐Esophageal cancer	THE, pr-partial PD, splenectomy (one-stage)	Roux-Y esophago-jejunostomy (supercharged jejunoplasty) [31]	Comp: atrial fibrillation (Dindo-Clavien II) Onc: R0
Gyorki et al. 2011 [26]	Management of synchronous tumors of the esophagus and pancreatic head: a novel approach https://pubmed.ncbi.nlm.nih.gov/21929904/	‐Adenocarcinoma of the distal esophagus ‐Neuroendocrine tumor of pancreatic head	1. TTE 2. pp-partial PD (two-stage; 6 months later)	Colon interposition	Comp: delayed gastric emptying (Dindo-Clavien II) Onc: -
Jayaprakash et al. 2008 [27]	Management of synchronous adenocarcinoma of the esophago-gastric junction and ampulla of Vater: case report of a surgically challenging condition https://pubmed.ncbi.nlm.nih.gov/19785744/	‐EGJ II adenocarcinoma ‐Ampullary adenocarcinoma	Pr-partial PD, gastrectomy, limited TTE (one-stage)	Roux-Y esophago-jejunostomy	Comp: no Onc: R0
Kurosaki et al. 2000 [28]	Thoracic esophagectomy combined with pylorus-preserving pancreatoduodenectomy in a one-stage procedure: report of a case https://pubmed.ncbi.nlm.nih.gov/10664342/	‐Esophageal squamous cell carcinoma ‐Intraductal polypoid tumor in the pancreatic head	THE, pp-partial PD (preserving gastroduodenal artery and right gastroepiploic vein) (one-stage)	Retrosternal gastric tube with cervical anastomosis	Comp: no Onc: recurrence at anastomotic site
Mafune et al. 1995 [29]	Synchronous cancers of the esophagus and the ampulla of Vater after distal gastrectomy: successful removal of the esophagus, gastric remnant, duodenum, and pancreatic head https://pubmed.ncbi.nlm.nih.gov/8551739/	‐Squamous cell esophageal carcinoma ‐Adenocarcinoma of the ampulla of Vater	Subtotal TTE, total residual gastrectomy, pr-partial PD (one-stage)	Colon interposition	Comp: anastomotic leakage Onc: -

While these other cases sometimes presented with postoperative complications or unsatisfactory oncological outcomes, the case presented in this report neither had any postoperative complications nor recurrence within 1 year after surgery. Therefore, this case report is the first element of proof for the feasibility of simultaneous two-stage oncological total pancreatectomy and Ivor-Lewis esophagectomy. Beneficial to the oncological outcome in this case - but also a limitation for its transferability to primary pancreatic malignancy - is the fact that although formally the pancreatectomy is performed radically, the lymphadenectomy in theory does not have to be as extensive. This is due to the fact that the pancreatic lesions were not primary cancer, but long-term metastases and therefore do not migrate to peripancreatic lymph nodes as regularly.

8. Patient perspective

“One year after this surgery there have been no complications and no relapse of the disease. I am extremely grateful to have been treated by your team. You have helped me a lot. It was the best decision I could have made. I would be very happy to stay under your medical supervision.”

(Translated from German)

9. Conclusion

Synchronous visceral malignancy of the esophagus and pancreas is extremely rare and typical resection strategies such as oncological pancreatectomy and Ivor-Lewis esophagectomy with gastric conduit reconstruction might not be compatible. Specifically, integrity of the right gastroepiploic and consequently also gastroduodenal artery is essential for gastric conduit perfusion. However, these arteries are often involved in malignancy of the pancreatic head rendering them necessary to resect. Therefore, only alternative resection and reconstruction strategies such as colonic interposition can be applied. With this case report we depict the feasibility of combined two-stage total pancreatoduodenectomy and esophagectomy as the first case to be ever reported in literature. There were neither postoperative complications nor recurrence at the 1-year follow-up with a good quality-of-life which illustrates the potential of this strategy as a curative approach.

Patient consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

The patient provided his written informed consent to be subject of this case report, including the publication of any potentially identifiable images or data included in this article. The ethical committee of the hospital gave the agreement to report this case.

Funding

There has been no funding for this case report.

Author contribution

Conception/design: ASF, FN, MS. Provision of study materials and patient: ASF, MS. Collection and assembly of data: ASF, VH, SR, GAS, FN, MS. Analysis and interpretation: ASF, FN, MS. Manuscript writing: ASF, FN, MS. All authors contributed to the article and approved the submitted version.

Guarantor

Alexander Studier-Fischer, Martin Schneider.

Research registration number

Not applicable.

Declaration of competing interest

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Appendix A. Supplementary data

Supplementary material

Data availability statement

The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding authors.