Sarcoidosis mimicking metastatic progression of pancreatic neuroendocrine tumor

Abstract

Rationale:Pancreatic neuroendocrine tumors (PNETs) account for less than 5% of all pancreatic tumors. PNETs develop from pancreatic endocrine islet cells and have a variable range of malignant potential. These neoplasms tend to have a slower growth rate than exocrine tumors and may remain undetectable for years. Achieving a correct diagnosis and staging is of key importance for the optimal management of the disease and requires experience with the disease, an accurate clinical status evaluation and a critical interpretation of the radiological findings derived from morphological and functional imaging techniques as well as an integrated multidisciplinary approach. The possibility that some clinical data and radiological findings encountered during the diagnostic and staging procedures may not be related to PNETs but to concomitant clinical conditions should always be taken into consideration. This is mandatory as an incorrect stadiation may lead to patients’ mis-management.Patient concerns:We report the case of a 34-year-old female, with a past medical history of idiopathic acute pancreatitis, presenting with a severe upper abdominal pain, steady and radiating to the back.Diagnoses:Initial investigations incidentally detected a nonfunctioning pancreatic neuroendocrine tumor (NF-PNET) of intermediate grade G2. Subsequent investigations aimed at determining a correct tumor staging showed a negative indium-111- OctreoScan but an increased 18F-labeled fluorodesossiglucose (18F-FDG) uptake in multiple bilateral nodules in the lungs and in 1 nodular lesion located in the right gluteal subcutaneous tissue. An early tumor progression of a G2 NF-PNET that had to be treated with chemotherapy was suspected.Interventions:The histological examination of the gluteal subcutaneous nodule showed noncaseating granulomas, disproving the initial clinical suspect and allowing the diagnosis of active sarcoidosis in the G2 NF-PNET patient.Lessons:A misdiagnosis and a consequent therapeutic mismanagement were avoided with the support of an integrated multidisciplinary team.

Pancreatic sarcoidosis mimicking neoplasia: Case report

“…The authors suggested that the increased risk of cancer following sarcoidosis may be due to the fact that sarcoidosis causes chronic inflammation, which eventually gives rise to malignancy [ 7 ]. Moreover, similar (but not identical) combinations of tumors identified in this case, have been previously reported, such as pancreatic neuroendocrine tumors with extra-pancreatic sarcoid [ 8 , 9 ]. However, a definite link between sarcoidosis and malignancy is yet to be confirmed.…”

“…More ominous diagnoses such as adenocarcinoma must be ruled out since symptoms may be nonspecific and mimic cancer, and pancreatic sarcoidosis can present concomitantly with pancreatic cancer [ 10 ]. Furthermore, sarcoidosis that does not involve the pancreas can co-present with primary tumors of the pancreas, including neuroendocrine tumors [ 8 ] and pseudopapillary tumors [ 11 ].…”

Sarcoidosis and neuroendocrine tumours: case report and literature review

“…The association between sarcoidosis and different types of malignancies has been previously described [2–10]. Carcinoid or neuroendocrine tumours (NETs) are rare tumours and association with sarcoidosis has been reported in the literature [11–25]. NETs can arise from any organ but most commonly from lung and small intestine [26].…”