Metastasized pancreatic neuroendocrine tumor in a teenage girl: a case report

Tremmel, T.; Holland‐Cunz, Stefan; Günther, Patrick

doi:10.1186/s13256-015-0708-3

Cited by 7 publications

(6 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…[12,13] Other gastrointestinal sites can be rarely involved, including the liver, pancreas, duodenum, and small intestine. [14] In some cases, it is not possible to determine the site of origin of the tumor. [12] Duodenal NETs are rare; they are usually found in the proximal duodenum, as seen in the case described here, and they present as intraluminal polyps or mural masses.…”

Section: Discussionmentioning

confidence: 99%

Pediatric gastroenteropancreatic neuroendocrine tumor

et al. 2019

View full text Add to dashboard Cite

Introduction:Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are a heterogeneous group of epithelial neoplasms originating from the diffuse neuroendocrine cell system of the gastrointestinal tract and pancreas. They are very rare, especially in pediatric age, and vary widely in terms of clinical presentation, malignant potential, and prognosis.Patient concerns:A 9 years’ old, white female child presented with abdominal pain and diarrhea mixed with bright red blood lasting 2 days followed by hematemesis.Diagnosis:Routine laboratory tests revealed microcytic anemia. Upper endoscopy showed a 20-mm polypoid lesion in the posterior wall of the duodenal bulb. Biopsy specimens were taken and histologic analysis showed a well-differentiated neuroendocrine tumor G1, with a ki-67 index <2%, an expression of chromogranine A (CgA), synaptophysin and somatostatin receptor type 2A (SSTR2A). Endoscopic ultrasound showed a 21-mm hypoechoic, hypervascular lesion involving the mucosal, submucosal, and muscular layers and a 15-mm hypoechoic round periduodenal lymph node. Gallium-68-somatostatin receptor positron emission tomography (PET with Ga-DOTATOC) showed one area of tracer uptake in the duodenum and other one near the duodenum compatible with the primary tumor site and a lymph node respectively. All the tests confirmed the diagnosis of a GEP-NET of the duodenal bulb, with a single lymph-node metastasis.Interventions:The patient underwent an open duodenal wedge resection.Outcomes:The follow-up at 6, 24, and 36 months and then yearly after surgery for a total of 42 months showed no evidence of recurrence.Conclusion:Duodenal neuroendocrine tumors represent 1% to 3% of all GEP-NETs. They are rare in adults and extremely rare in children. Therefore, the diagnostic and therapeutic approach should be multidisciplinary, including laboratory, endoscopic, and specific imaging tests and strictly follows guidelines, to avoid misdiagnosis and inadequate treatments. Although the prognosis is benign in most cases, they can present with metastases. Therefore, a careful follow-up is extremely important.

show abstract

Section: Discussionmentioning

confidence: 99%

Pediatric gastroenteropancreatic neuroendocrine tumor

et al. 2019

View full text Add to dashboard Cite

show abstract

“…Among GEP-NETs, appendiceal neuroendocrine tumors (aNETs) are the most common, occurring in 78% to 79% of cases [6,7] . Other gastrointestinal sites, including the liver, pancreas, duodenum, and small intestine, may rarely be affected [8] . The liver is the most common site for metastatic NETs [9] ; however, primary hepatic NETs are rare and only account for 0.3% of all NETs [10] .…”

Section: Introductionmentioning

confidence: 99%

“…[6,7] Other gastrointestinal sites, including the liver, pancreas, duodenum, and small intestine, may rarely be affected. [8] The liver is the most common site for metastatic NETs [9] ; however, primary hepatic NETs are rare and only account for 0.3% of all NETs. [10] In some cases, it is not possible to determine the origin of the tumor.…”

Section: Introductionmentioning

confidence: 99%

Childhood neuroendocrine tumors of the digestive system

Kartal¹

2022

Medicine

View full text Add to dashboard Cite

The prevalence and incidence of neuroendocrine tumors (NETs) are increasing in the pediatric population. This increase can be associated with improved diagnostics and increased detection rates of the disease. We aimed to discuss the clinical and pathological characteristics of patients with this rare disease who were followed and treated at our center.The medical records of children (aged 0–18 years) with NETs of the digestive system, followed up and treated between 2007 and 2020 at Ondokuz Mayis University Faculty of Medicine, were reviewed.Overall, 16 patients (8 girls and 8 boys) were analyzed. Fifteen patients had NETs in the appendix; 14 of these had grade I NETs, and 1 had grade II NETs. No additional surgery was performed except for appendectomy. All patients were in complete remission at the last follow-up (median 38 months). The other patient, a 12-year-old girl, had a primary hepatic neuroendocrine carcinoma (grade III NET). Three cycles of neoadjuvant and adjuvant platinum-based chemotherapy were administered, and right hepatectomy was performed to remove the mass. The patient is being followed-up for approximately 3 years without disease recurrence.Most NETs are observed in adults, and most studies have focused on this population. Unlike adults, increasing awareness of the disease in the pediatric population (especially in cases of acute appendicitis), discovering therapeutic treatments, and sharing experiences are crucial for developing an optimal therapeutic approach for pediatric NETs.

show abstract

“…In the period from 1994 to 2009, the incidence of NETs increased from 1.09 cases to 5.25 cases in 10 5 persons per year . This kind of tumor usually appears in adults, but can also appear in younger individuals including children . One of the possible therapeutic options for these tumors is peptide receptor radionuclide therapy (PRRT), which is based on somatostatin analogs labelled with energetic beta emitters, such as 90 Y or 177 Lu .…”

Section: Introductionmentioning

confidence: 99%

A five‐compartment biokinetic model for ⁹⁰Y‐DOTATOC therapy

et al. 2018

View full text Add to dashboard Cite

Purpose Neuroendocrine tumors (NETs) are now routinely treated by radiopeptide targeted therapy using somatostatin receptor‐binding peptides such as 90Y‐ and 177Lu‐DOTATOC. The objective of this work was to develop a biokinetics model of 90Y labelled DOTATOC, which is applied in the therapy of NETs to estimate doses in kidney and tumor. Methods A multi‐compartment model described by two sets of differential equations, one set for the actual 30‐min infusion and the other set for the post‐infusion period was developed and activities were measured by liquid scintillation counting in blood (compartment 1) and the urine (compartment 3). The inter‐compartment transfer coefficients, λij, were varied to yield the best fit of the calculated to the measured time‐activity data and the 90Y‐DOTATOC time‐activity data in the five‐compartments comprising the human body were thus determined. The resulting time‐activity curves were integrated over the interval from 0 to 72 h post administration to obtain the number of radioactive decays in each compartment and, in case of the kidneys and tumor, then multiplied by the self‐dose 90Y beta particle absorbed fraction, determined by Monte Carlo (MC) simulation, the kidney and tumor absorbed doses. Results Transfer coefficients λij, were determined for five‐compartments for all patients. Time‐ activity curves of 90Y‐DOTATOC in 14 patients were determined, and two typical ones are shown graphically. Absorbed doses in the tumor and kidneys, obtained by the developed method, were determined. The mean absorbed dose in a kidney per unit of administered activity is 1.43 mGy/MBq (range 0.73–2.42 mGy/MBq). The tumor dose was determined as 30.94 mGy/MBq (range 20.05–42.31 mGy/MBq). Conclusion Analytical solution of a biokinetic model for 90Y‐DOTATOC therapy enabled determination of the transfer coefficients and derivation of time‐activity curves and kidney and tumor absorbed doses for 14 treated patients. The model can be applied to other radionuclides where elimination is predominantly through urine, which is often the case in radiopharmaceuticals.

show abstract

Metastasized pancreatic neuroendocrine tumor in a teenage girl: a case report

Cited by 7 publications

References 11 publications

Pediatric gastroenteropancreatic neuroendocrine tumor

Pediatric gastroenteropancreatic neuroendocrine tumor

Childhood neuroendocrine tumors of the digestive system

A five‐compartment biokinetic model for ⁹⁰Y‐DOTATOC therapy

Contact Info

Product

Resources

About

Metastasized pancreatic neuroendocrine tumor in a teenage girl: a case report

Cited by 7 publications

References 11 publications

Pediatric gastroenteropancreatic neuroendocrine tumor

Pediatric gastroenteropancreatic neuroendocrine tumor

Childhood neuroendocrine tumors of the digestive system

A five‐compartment biokinetic model for 90Y‐DOTATOC therapy

Contact Info

Product

Resources

About

A five‐compartment biokinetic model for ⁹⁰Y‐DOTATOC therapy