Duodenal adenocarcinoma in an 11-year-old girl: a case report

¹Department-Habib Bourguiba Hospital, General Surgery, Sfax, Tunisia, ²Department-Fattouma, General Surgery, Bourguiba Hospital, Monastir, Tunisia, ³Department of Anesthesiology-Habib Bourguiba Hospital, Sfax, Tunisia

^&Corresponding author
Wael Boujelbéne, Department-Fattouma, General Surgery, Bourguiba Hospital, Sfax, Tunisia

Introduction    

Duodenal adenocarcinoma is a rare malignant tumor that represents less than 1% of all gastrointestinal cancers [1]. It is extremely rare in the pediatric population and represents an unusual entity. Due to nonspecific symptoms, the diagnosis is often delayed. Thus, this tumor has a poor prognosis [2]. We report a case of primary duodenal adenocarcinoma in an 11-year-old girl, who was successfully treated with a cephalic duodenopancreatectomy.

Patient and observation     

Patient information: the patient is an 11-year-old girl with no significant personal or family medical history.

Clinical findings: on examination, the child was in good general condition. His weight was 30 kg. Abdominal palpation did not reveal any abnormality.

Timeline of current episode: she presented with paroxysmal pain localized to the right hypochondrium, progressively evolving over the past three months. The pain was associated with postprandial vomiting but not with anorexia or weight loss.

Diagnostic assessment: there was no biological inflammatory syndrome or disturbance in liver and pancreatic balance. The determination of tumor markers such as CA 19-9, ACE, and alpha-fetoprotein was negative. An ultrasound was performed showing dilatation of the bile ducts upstream of a mass in the pancreaticoduodenal region.  A complementary CT scan was performed that showed a hypodense pancreatic cephalic mass with low enhancement after injection, measuring 21x26x5.1 mm and well-defined, resulting in significant dilatation of the main bile duct (measured at 18 mm) and the intrahepatic bile ducts ( Figure 1). Given the terrain and appearance of the CT scan, malformation pathology was suspected. MRI shows a hypointense mass on T1, hyperintense on T2, and moderate enhancement after gadolinium injection ( Figure 2). A lateral duodenoscopy was ordered, showing normal major and accessory papillae with the presence of a deep, irregularly contoured ulcerated process with an elevated bulge of 22 mm in diameter located just below the major papilla and on the medial border of D2 ( Figure 3). A duodenal biopsy and a biopsy of the detected mass were performed.

Diagnosis: duodenal biopsies and immunohistochemical tests are in favor of a moderately differentiated adenocarcinoma of the second duodenum infiltrating the head of the pancreas.

Therapeutic intervention: in front of this duodenal tumor that invades the pancreas, the surgical indication was presented at multidisciplinary consultation meetings.  Peroperative investigation showed a poorly limited mass of the duodenum, measuring about 03 cm, that infiltrated the pancreatic head. A cephalic duodenopancreatectomy was performed under general anesthesia, removing the entire tumor in one piece ( Figure 4). Due to the complexity of the surgical procedure, the patient was closely monitored postoperatively in an intensive care unit for the first 24 hours, after which she was transferred to our surgical ward.

Follow-up and outcome of interventions: the postoperative course was simple with the correct glycemic cycles. Refeeding on day 2 after the operation was well tolerated. The child was discharged on day 5 after the operation. Pathological examination of the operative specimen concluded a 4 cm well-differentiated duodenum adenocarcinoma infiltrating the ampulla of Vater, extending deep into the underlying pancreas sparing the peripancreatic fat with no signs of peri-nervous engorgement or vascular invasion. All surgical borders are intact. The lymph node survey showed no lymph node metastasis.  The tumor was classified as T4N0Mx. The postoperative ultrasound was normal and the current follow-up is 3 months without complications.

Patient perspective: the patient and her parents expressed their gratitude and satisfaction with the care she received. They reported feeling reassured throughout the process, from diagnosis to surgery, and appreciated the clear explanations provided by the medical team. The attentive postoperative monitoring and support helped them feel safe and confident in her recovery following the pancreaticoduodenectomy for duodenal adenocarcinoma. They emphasized their overall satisfaction with the management and the outcome of the treatment, highlighting the professionalism and dedication of the healthcare team.

Informed consent: the patient is a minor and therefore did not provide consent herself. Her parents have read and understood the information provided in the consent statement. They have voluntarily granted permission for the publication of their daughter’s medical case report as described. They acknowledge that all personal identifiers will remain confidential and that they retain the right to withdraw their consent at any time prior to publication.

Discussion     

Malignant tumors of the gastrointestinal tract are rare in children; duodenal adenocarcinomas are even rarer and represent less than 1% [1, 2]. In our patient, the diagnosis was particularly unexpected given her young age (11 years old) and the complete absence of personal or family history suggestive of genetic predisposing syndromes such as Lynch syndrome or familial adenomatous polyposis (FAP). The diagnosis is often made at an advanced stage due to the nonspecificity of the clinical symptoms [3]. The clinical signs are diverse: abdominal pain, melena, weight loss, vomiting, jaundice, and in most cases, the discovery of the tumor is fortuitous [4-6]. Unlike typical adult presentations where weight loss and jaundice predominate, our patient presented with isolated right hypochondrium pain and postprandial vomiting without weight loss or altered general status, reflecting the diagnostic difficulty in pediatric cases. Esogastroduodenoscopy with biopsy is a key exam for the definitive diagnosis [2]. However, radiological findings are essential to define the grade and therapeutic strategy [3]. In our case, cross-sectional imaging initially pointed toward a benign pancreaticoduodenal malformation-a common diagnostic pitfall in children-before duodenoscopy revealed an ulcerated lesion below the major papilla.

Curative management is surgical. Cephalic duodenopancreatectomy with monobloc tumor removal remains the gold standard for D1 and D2 tumors. Although these tumors are not very sensitive to chemotherapy, the role of neoadjuvant chemotherapy is being evaluated for locally advanced tumors [2]. Despite the technical challenge and high complexity of a duodenopancreatectomy in an 11-year-old child, complete monobloc resection was successfully achieved without postoperative complications, allowing early discharge on postoperative day 5. The 5-year survival rate is estimated to be 60% for curatively resected adenocarcinomas. Duodenal tumors are rare [2, 4]. The diagnosis is often made at an advanced stage. Early diagnosis and curative surgery are often correlated with longer overall survival [2]. Our case highlights that despite a locally advanced T4 stage infiltrating the pancreas, early radical surgical management can achieve complete pathological clearance, yielding a favorable short-term outcome at a 3-month follow-up.

Conclusion     

Primary duodenal adenocarcinoma in an 11-year-old girl was successfully managed with complete R0 cephalic duodenopancreatectomy despite initial cross-sectional imaging misidentifying the mass as a congenital malformation. This case demonstrates that primary duodenal malignancies, although exceptional in pediatric patients, must be considered in the differential diagnosis of persistent upper gastrointestinal pain and vomiting. Maintaining a high index of clinical suspicion when faced with atypical pancreaticoduodenal masses is critical to avoiding diagnostic delays, as early radical surgical resection with clear margins remains the cornerstone for achieving long-term disease-free survival.

Competing interests     

The authors declare no competing interests.

Authors' contributions     

Patient management: Wael Boujelbène, Amina Chaka and Islem Bradai. Data collection: Wael Boujelbène, Amina Chaka, Amin Zouari and Amine Chaabouni. Manuscript drafting: Wael Boujelbène, Amina Chaka, Nizar Kardoun and Salma Ketata. Manuscript revision: Wael Boujelbène, Amina Chaka and Salah Boujelbène. All authors read and approved the final manuscript.

Figures     

Figure 1: the abdominal contrast-enhanced computed tomography scan reveals a well-limited, hypo-intense pancreatic cephalic mass (21x26x5.1) with low post-injection enhancement;this lesion is responsible for a significant dilatation of both the intrahepatic and extrahepatic bile ducts

Figure 2: the magnetic resonance imaging demonstrates a moderately enhancing, T2-weighted hyperintense mass located at the pancreaticobiliary junction, associated with a characteristic double-duct dilatation

Figure 3: the lateral duodenoscopy shows a deep, irregular, and ulcerated mucosal lesion located beneath the major papilla in the second part of the duodenum

Figure 4: surgical specimen obtained after a successful cephalic duodenopancreatectomy (Whipple procedure), demonstrating the complete, en bloc resection of the tumor

References