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Int J Surg Case Rep
Int J Surg Case Rep
International Journal of Surgery Case Reports
2210-2612
Elsevier

S2210-2612(24)00908-8
10.1016/j.ijscr.2024.110127
110127
Case Report
Sudden rupture of a giant solid pseudopapillary tumour of the pancreas during caesarean delivery in a pregnant woman with severe preeclampsia: A case report and literature review
Xu Ruiqing littleqing1977@126.com
⁎
Fa Hongwen
Tianjin NanKai Hospital, Tianiin Hospital of Integrated Chinese and Western Medicine, No. 6 Changjiang Road, Nankai District, Tianjin 300100, China
⁎ Corresponding author. littleqing1977@126.com
08 8 2024
9 2024
08 8 2024
122 11012715 5 2024
30 7 2024
6 8 2024
© 2024 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Limited.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction and importance

Nonspecific presentations during pregnancy can mask early signs and symptoms of upper abdominal tumours, making the preoperative diagnosis of upper abdominal tumours difficult. Solid pseudopapillary neoplasm of the pancreas (SPN) is a rare exocrine tumour of the pancreas, and SPN in combination with preeclampsia during pregnancy is even rarer.

Case presentation

In this paper, we report a case of SPN combined with preeclampsia during pregnancy and sudden rupture of a giant retroperitoneal SPN during a caesarean section, which resulted in life-threatening intra-abdominal haemorrhage. After exclusion of obstetric factors, a rapid response team was activated, multidisciplinary treatment (MDT) was carried out, and the patient was treated promptly and appropriately by resection of the giant retroperitoneal tumour, partial resection of the body and tail of the pancreas, and abdominal drainage.

Clinical discussion

To our knowledge, this is the first reported case of SPN combined with preeclampsia during pregnancy, and a rapid and timely MDT could have ensured the patient's life.

Conclusion

When dealing with a pregnant woman with an acute abdomen, the obstetrician should communicate fully with the woman to ensure that the most likely diagnosis is obtained. In the event of an unexpected accident during a caesarean section, it is important to remain calm, activate the Rapid Response Team and seek an MDT to ensure the life of the mother.

Highlights

• Solid pseudopapillary neoplasm of the pancreas was in combination with preeclampsia during pregnancy.

• A multidisciplinary treatment was carried out, and the patient was treated promptly and appropriately.

• Nonspecific presentations during pregnancy can mask early signs and symptoms of upper abdominal tumours.

Keywords

Preeclampsia
Caesarean section
Solid pseudopapillary neoplasm of the pancreas
Rupture
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pmc1 Introduction

SPN is a rare exocrine tumour of the pancreas that is pathologically classified as a low-grade malignant tumour of epithelial origin, with a low incidence and an unknown pathogenesis. SPN is particularly prevalent among young women in their twenties and thirties. The clinical manifestations of SPNs are often nonspecific, with no clinical symptoms when the tumour is small. SPN is rare, and the combination of SPN and pregnancy is even rarer and is often difficult to diagnose preoperatively. This paper is the first to report an extremely rare case of SPN during pregnancy combined with sudden rupture of a large retroperitoneal pancreatic tumour encountered during a caesarean section, resulting in life-threatening intra-abdominal haemorrhage. This case suggests that the diagnosis of epigastric tumours during pregnancy is difficult and that their nonspecific presentation can mask early signs and symptoms. The presence of clinical signs and symptoms in pregnant women who cannot be explained by pregnancy alone should be taken seriously by clinicians. It is also hoped that the report of this rare case will serve as a wake-up call to all clinicians to improve perinatal care, strengthen relevant investigations and detect abnormalities as early as possible. In the case of sudden intraoperative abdominal haemorrhage, the possibility of a ruptured surgical tumour should be considered after excluding obstetric factors. It is important to be calm, activate a rapid response team and seek multidisciplinary treatment (MDT) to ensure the life of the patient. This study was approved by the protocol. This work has been reported in line with the SCARE criteria [1].

2 Case presentation

A 22-year-old woman was admitted to the hospital as an emergency due to “gravidity 2, parity 0, 38 weeks of pregnancy, oedema for half a month and sudden increase in blood pressure for one day”.

The patient had a regular menstrual period, a urine human chorionic gonadotropin (HCG) (+) test >30 days after menopause, and no early pregnancy reaction. She denied having a history of birth control or viral infection. Her initial blood pressure during pregnancy was 120/80 mmHg, and her nuchal translucency (NT) at 12 weeks gestation was 1.2 mm. There was no abnormal NT, and there was spontaneous foetal movement at 4+ months of pregnancy and normal prenatal examinations. Noninvasive prenatal examinations revealed a low-risk pregnancy, and 4D ultrasound showed no abnormalities. Her oral glucose tolerance test (OGTT) result was 4.26–9.1–8.62 mmol/L, and she was diagnosed with gestational diabetes mellitus, which was controlled by diet and exercise. A routine pregnancy blood test revealed an HGB level of 94 g/L. The patient was treated with ferrous succinate oral solution (40 mg, BID) to correct anaemia, and the patient's haemoglobin (HGB) level in her last pregnancy blood test was 102 g/L. At 14 weeks of pregnancy, the patient presented with bilateral foot swelling, which gradually progressed to bilateral lower-limb oedema. Tests revealed that the patient's blood pressure was within the normal range, her urine protein was negative, and she had no other discomfort.

On the day of admission, the patient had a regular pregnancy check-up, a blood pressure of 151/94 mmHg, a urine protein result of 3+ and bilateral lower-limb oedema (++) and was referred to our hospital for treatment due to limited access to local hospitals. The patient's blood pressure was 167/109 mmHg, with no dizziness or headache, no blurred vision, no nausea or vomiting, and no significant upper abdominal discomfort, and the amniotic fluid sac had not broken. The patient had an abortion 1 year prior with clearance of 1 foetus. The patient had a history of being physically fit and denied having a specific medical history or family history of hereditary disease. Relevant tests were performed on admission. Mild nausea and vomiting occurred in early pregnancy and were relieved after 12 weeks without obvious epigastric discomfort. At admission, her T was 36.5 °C, her BP was 156/111 mmHg, her P was 98 beats/min, her R was 20 beats/min, and no abnormalities in her heart or lungs were detected. Her abdominal pattern of pregnancy or oedema of both lower limbs was also observed (++). The results of routine blood testing were 3.51 × 1012/L↓ (3.8–5.1 × 1012/L) for RBCs, 102 g/L↓ (115–150 g/L) for HGB, and 114 × 109/L↓ (125–350 × 109/L) for PLTs. The results of routine urine testing were urine protein 3+ (negative). The results of coagulation testing were 128.4 % PT-%↑ (80–120 %) and 7.030 mg/L D-dimer↑ (0–0.55 mg/L). The results of liver function testing were ALT 10 U/L (7–40 U/L), AST 21 U/L (7–45 U/L), and ALP 173 U/L↑ (35–100 U/L). The results of renal function testing were 57.9 μmol/L CREA (41–73 mmol/L), 335 μmol/L UA (154–357 mmol/L), and 3.73 mmol/L BUN (2.6–7.5 mmol/L). The glucose and electrolyte levels were 3.92 mmol/L↓ (3.9–6.1 mmol/L) for GLU and <21.00 ng/mL↓ (28–72 ng/mL) for MYO and 895.90 pg/mL↑ (0–100 pg/mL) for NT-proB-type natriuretic peptide. The calcium, phosphorus and magnesium concentrations were 2.10 mmol/L↓ (2.25–2.75 mmol/L, respectively. Electrocardiography (ECG) revealed sinus tachycardia, and ST showed a 0.5-mm decrease in V4–V6. Obstetric ultrasound revealed the following: intrauterine pregnancy, singleton pregnancy, cephalic position, and hyponatremia (amniotic fluid index (AFI) 49 mm).

The admission diagnosis was as follows: 1. gravida 2, parity 0, 38 weeks of pregnancy, left occipitotransverse (LOT); 2. hypertensive disorders of pregnancy, namely, severe preeclampsia; 3. hypohydramnios; 4. combined anaemia of pregnancy; 5. umbilical cord encirclement; and 6. diabetes mellitus in pregnancy.

Severe preeclampsia at 38 weeks gestation must be terminated as soon as possible to ensure the safety of the mother and child; this should be the first consideration in regard to vaginal labour; however, if the patient cannot deliver vaginally in a short period and the condition progresses rapidly, the indication for caesarean section can be relaxed. Therefore, after admission to the hospital, the patient's condition was actively controlled with antispasmodic antihypertensive agents, and the mother and child were closely monitored with intravenous magnesium sulfate antispasmodic: 5 g of magnesium sulfate dissolved in 100 mL of 5 % dextrose solution, 20 min of rapid drip, and then 10 g of magnesium sulfate dissolved in 500 mL of 5 % dextrose solution for static maintenance (25 drops/min), while oral labetalol (100 mg, TID) was used as an antihypertensive agent. Therefore, intravenous magnesium sulfate was used to relieve spasm, and oral labetalol (100 mg, TID) was given to lower blood pressure while presurgical preparation was carried out. On the night after admission, the patient reported decreased foetal movement, a blood pressure of 172/105 mmHg, dizziness and breath-holding discomfort. Considering that the patient's blood pressure was not well controlled and her condition worsened within a short period, the night shift doctor decided to terminate the pregnancy by immediate caesarean section to ensure the safety of the mother and baby. The patient was admitted to the operating room that evening, and combined lumbar and epidural anaesthesia was administered successfully. Intraoperative access to the abdominal cavity was achieved, and approximately 500 mL of dark bloody ascites was observed on exploration. Due to the decision of emergency surgery, the operator neglected the importance of preoperative surgical ultrasound and rushed the surgery without perfecting the surgical ultrasound. Therefore, in regard to the sudden and unusual dark bloody ascites observed during surgery, the operator first considered that this might be due to rupture of the subperitoneal haematoma of the liver complicated by severe preeclampsia, but rupture of the uterus or rupture of other pelvic and abdominal organs or tumours was not excluded based on clinical experience. The woman rapidly delivered a live female infant with no postnatal asphyxia and a 1-min Apgar score of 9 (skin colour-1). The membranes and placenta were delivered intact, the uterine cavity was rapidly cleared, and the myometrium was closed with continuous full-layer sutures. During suturing, persistent active bleeding from the pelvis was noted. On immediate exploration, the uterus and bilateral adnexa were normal in appearance, and there was no bleeding from other parts of the pelvis. The patient had a history of severe preeclampsia, and serious preeclampsia complications with subliver capsule haematoma formation and possible liver rupture could not be excluded. At this point, the woman's blood pressure suddenly dropped, and her heart rate increased to 130–140 beats/min. Therefore, the critical maternal resuscitation process was started immediately, general anaesthesia was requested, subclavian vein catheterization and tracheal intubation were established, the blood bank was urgently contacted, a green blood supply channel was opened, the abdominal cavity was filled with multiple blood pads to stop the bleeding. The gastrointestinal surgeon was immediately contacted for consultation on the stage. The surgeon extended the incision from the caesarean section upward to remove the old blood clot and blood from the upper abdomen by approximately 1500 mL. On exploration, the surface of the greater omentum, liver and spleen was smooth with no rupture or bleeding. A large tumour, approximately 15 × 12 cm in diameter, with a surface rupture of approximately 4–5 cm and still exhibiting active bleeding, was visible after opening the gastrocolic ligament. The tumour was large, and the spleen was torn during intraoperative dissection, so parallel splenectomy was performed (Fig. 1). After superficial bleeding was controlled, the tumour was separated from the surrounding tissues, and finally, retroperitoneal giant tumour resection, partial resection of the body and tail of the pancreas, and intraperitoneal drainage were performed. The postoperative tumour specimen was brittle and contained many blood clots and some fish meat-like substances, which were sent for pathology. The total volume of intraoperative bleeding was approximately 3500 mL, with 1200 mL of blood and 800 mL of plasma transfused. After the operation, the patient was transferred to the intensive care unit (ICU) to continue transfusion of blood and plasma as well as coagulation factors to replenish blood volume and improve blood clotting, received respiratory and circulatory support, and received correction of electrolyte disorders and acid–base imbalance until stabilization of the patient's condition. A total of 2400 mL of blood, 1200 mL of plasma, 300 mL of platelets and 10 U of cold precipitate were transfused. The patient was awake two days after the surgery and was successfully extubated. She was transferred to the general obstetric ward three days after surgery; continued anti-inflammatory, hypotensive, rehydration support and thromboprophylaxis treatment; she was discharged from the hospital two weeks after surgery.Fig. 1 (a) Large retroperitoneal tumour detected intraoperatively; (b) postoperative excised tumour specimen and spleen.

Fig. 1

Postoperative pathology revealed the following: 1. (retroperitoneal) solid pseudopapillary neoplasm of the pancreas, with immunohistochemical staining showing AACT (+), β-catenin (nuclear pulp +), Syn (+), CD10 (+), Vimentin (+), CyclinD1 (weak +), CD56 (partial +), PR (−), CgA (−), CKpan (−), E-cadherin (−), and Ki67 (approx. 2 % +); 2. a spleen consistent with mild splenic stasis, 4 periportal lymph nodes with reactive hyperplasia and pancreatic tissue; and 3. in part of the tail of the body of the pancreas + large omentum, the examination material was consistent with omental tissue, with 3 lymph nodes exhibiting reactive hyperplasia.

The patient was instructed to undergo follow-up every 3 months for 1 year, every 6 months after 1 year, and once per year after 2 years and thereafter. At the present six-month follow-up, no abnormalities were found on ultrasound or computed tomography (CT) of the whole abdomen. Patients are satisfied with the results of their treatment (Fig. 2).Fig. 2 Postoperative pathological images. (a) SPN tumour tissue; the upper left is the tumour area, the lower right is the normal pancreas, and the boundary between the tumour and the normal pancreas is still clear. (b) Tumour cells with unclear boundaries, eosinophilic cytoplasm, some cells with slightly clear boundaries, foamy or transparent cytoplasm, round or oval nuclei located in the centre, and some nuclear grooves were observed, and nuclear schizophrenia was rare. (c) The cystic area is often a collection of foamy tissue cells, and the cyst wall is fibrotic. (d) Pseudopapillary area with branching papillae, papillae covered with several layers of tumour cells, with abundant blood vessels or blood sinuses.

Fig. 2

3 Discussion

SPNs of the pancreas are rare exocrine tumours of the pancreas, also known as papillary and solid epithelial tumours of the pancreas, cystic solid tumours of the pancreas, papillary cystic epithelial tumours, solid papillary epithelial tumours of the pancreas, and Frantz tumours [2]. According to the latest pathological classification, SPNs of the pancreas are categorized as low-grade malignant tumour of epithelial origin [3], with a low incidence of 0.17 % to 3 % [4,5]. Its pathogenesis is still unclear. Most scholars believe that SPN originates from pancreatic tissue, but with the in-depth study of its immunohistochemistry and other studies, it was found that the tumour has multiple components, namely, endocrine, exocrine and focal epithelial components of the pancreas, suggesting that SPN most likely originates from pancreatic embryonic pluripotent stem cells [6]. SPN has a clear sex- and age-specific tendency, occurring mostly in young women, with over 90 % of pancreatic pseudopapillary tumours occurring in young women in their twenties and thirties [7]. The clinical presentation is mostly nonspecific, and there are no clinical symptoms when the tumour volume is small. Preoperative diagnosis is therefore difficult, and patients are easily misdiagnosed [8]. Some patients present with vague pain or discomfort in the upper abdomen, which is not clearly distinguishable from hepatobiliary disease. The tumour is usually found accidentally by ultrasound during physical examination, and the diameter of the SPN tumour is usually large. Some patients show symptoms of tumour compression, such as vague pain and discomfort in the upper abdomen, abdominal distension, nausea, and vomiting. In severe cases, the tumour may manifest as intestinal obstruction. Cases of acute pancreatitis and tumour rupture leading to acute abdomen have also been reported [9]. This patient experienced nausea and vomiting in early and mid-pregnancy, but this was considered a normal reaction to pregnancy and was not taken seriously. If the tumour ruptures and bleeds, she may even have symptoms such as gastrointestinal bleeding and anaemia. Pseudopapillary tumours of the pancreas are more commonly found in the caudal part of the body of the pancreas but can also occur in the head of the pancreas; they rarely cause dilatation of the pancreaticobiliary ducts or jaundice, as they tend to grow exophytically. Imaging is the main diagnostic tool for this condition. Imaging methods include ultrasound, abdominal CT and magnetic resonance imaging (MRI). Ultrasound of the abdomen is simple and inexpensive and can reveal the location, size and morphology of tumours. Colour Doppler (CDFI) can detect the internal blood flow of a tumour, but in practice, abdominal ultrasound is highly susceptible to intra-abdominal gas interference, has relatively limited value for definitive diagnosis, and can be used as a primary screening tool. CT and MRI are more advantageous in showing the components of the lesion and can improve the accuracy of the diagnosis. Most of the laboratory tests for SPNs are normal, and CA199, CA125, AFP, CEA and other tumour indicators are also mostly within the normal range, so they have no significant value for diagnosis. SPNs are rarely diagnosed preoperatively and must be differentiated from nonfunctioning islet cell tumours, pancreatic adenocarcinoma and pancreatic cystadenoma. The main treatment for SPNs is surgical resection [10]. Surgical resection provides a good prognosis with a cure rate of 95 % [11], a five-year survival rate of 95–98 % and a 10-year survival rate of 93 % [12], but close follow-up is still required [13].

SPNs are rare, and no clinical cases of SPNs during pregnancy have been reported. This paper is the first report of an extremely rare case of SPN during pregnancy combined with sudden intraoperative rupture of a large pancreatic tumour, resulting in life-threatening intra-abdominal haemorrhage. The tumour was found in time, and the multidisciplinary effort eventually resolved the problem. Many lessons were learned from the management of this case.

Usually, if there are no special clinical symptoms, it is difficult to perform targeted ultrasound of the upper abdomen and more diagnostic CT and MRI, so it is difficult to detect abdominal tumours by means of conventional pregnancy tests, and it is difficult to achieve early diagnosis and treatment. This patient was a young woman who had no conscious discomfort except for nausea or vomiting in the early and mid-pregnancy periods, so the doctor in charge did not improve the surgical ultrasound and other examinations before the emergency surgery, which led to a passive situation during the operation.

Intraoperative tumour rupture may be related to the following factors: the most basic pathophysiological changes associated with hypertensive disorders during pregnancy are spasm of small blood vessels and endothelial damage throughout the body, increased vascular permeability, a large pancreatic tumour that is densely vascularized and rich in blood transport, and rapid changes in blood pressure before surgery, leading to spontaneous rupture of tumour blood vessels and intratumor haemorrhage.

This case is a wake-up call for clinicians and emphasizes the importance of perinatal care, strengthening investigations, paying attention to the complaints of pregnant women, and drawing sufficient attention from clinicians when pregnant women present with clinical symptoms and signs that cannot be explained by pregnancy alone. In pregnant women with acute abdomen combined with pregnancy, if ultrasound examination fails to reveal positive findings, CT or MRI of the abdomen should be considered for further diagnosis [14]. The American College of Obstetricians and Gynaecologists (ACOG) states that, at present, radiation doses from diagnostic tests do not affect the developing embryo or foetus (but should be avoided at 8–15 weeks of gestation) [15].

4 Conclusion

When treating a pregnant woman with an acute abdomen, the obstetrician should fully communicate with her and recommend imaging to further clarify the diagnosis and to try to detect the lesion as early as possible to reduce complications for the mother and child and to obtain the best possible prognosis. Due to the unpredictable nature of obstetrics, if sudden intra-abdominal bleeding occurs intraoperatively, the possibility of surgical tumour rupture should be considered at this point after obstetric factors have been ruled out. It is important to remain calm, activate a rapid response team and seek MDT to ensure the preservation of maternal life.

Patient consent section

The authors obtained written and signed consent to publish a case report from the patient prior to submission.

Declaration of competing interest

The authors declare no conflicts of interest.

Data availability

The data are available upon request from the authors.
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