Neuroendocrine Tumors: Case Study on Diagnosis, Treatment Strategies & Patient Management

Abstract

NETs are a relatively new and very rare category of neoplasms that represent neuroendocrine cells, which exhibit characteristics of both nerve and endocrine cells. NETs arise in many organs, although they occur most commonly in the pancreas, gastrointestinal tract, and lungs. As such, these tumors pose a difficult diagnosis due to the variety in their presentation and the generally nonspecific symptoms. This case study review will discuss the clinical implications of NETs as viewed by a patient diagnosed with a metastatic pancreatic neuroendocrine tumor. It helps to elucidate some of the inherent difficulties in attaining an accurate diagnosis, the diversity of multiplicity in treatment approaches initiated, and management. A deeper understanding of complexity in NET enables healthcare providers to address diagnostic deficiencies and weaknesses in treatment that subsequently improve the outcome for the patient.

Introduction

Neuroendocrine tumors are a heterogeneous group of malignancies derived from neuroendocrine cells dispersed throughout the body and play a critical role in hormonal and neurotransmitter regulation. Classifications of NETs can be performed based on their anatomic site of origin, functional status, or differentiation.

There has been an increasing awareness of NETs in clinical practices, which brings with it an appreciation of the potential necessity for better diagnostic modalities and therapeutic strategies. A case study is presented here that underscores the complexities of diagnosis and management of a patient who had a pancreatic neuroendocrine tumor by focusing on challenges encountered and approaches used as treatment.

Case Presentation

Patient Background

A 55-year-old woman presented to an oncology clinic with complaints of recurring abdominal pain, unintentional weight loss, and episodes of flushing. Her medical history also included hypertension and cholecystitis for which she had undergone cholecystectomy two years prior. She reported no known family history of cancer.

During physical examination, this patient presented with some diffuse tender areas of the abdomen and had no masses felt on palpation. Some of the laboratory work revealed elevated serum levels of chromogranin A, which warranted further investigation.

Diagnostic Challenges

The workup for the diagnosis started with imaging studies. A contrast-enhanced abdominal computed tomography (CT) scan showed a mass in the tail of the pancreas measuring 3 cm in diameter with multiple hepatic lesions. These findings were suspicious of the possibility of a neuroendocrine tumor, especially based on the markedly elevated chromogranin A level. However, the initial biopsy of the pancreatic lesion proved inconclusive and complicated further diagnosis.

Due to the unclear pathology, a gallium-68 DOTATATE PET scan was ordered as a radiotracer for somatostatin receptors that are commonly overexpressed in NET. Sure enough, the mass on the pancreas was confirmed with additional avid uptake in the liver lesions on PET suggesting metastatic pancreatic neuroendocrine tumor.

Treatment Approaches

Multidisciplinary Team Involvement

Due to the complexity of NETs, it was very important to approach the patient multidisciplinary for optimum management. In this case, the oncology team was comprised of medical oncologists, surgical oncologists, radiologists, and endocrinologists. A consensus was reached on initiating treatment that the main role was symptom management and tumor control.

Surgical Intervention

Although metastases were present, the primary pancreatic tumor was found to be resectable. The entire tumor was adequately removed using a distal pancreatectomy with splenectomy. The postoperative pathology report confirmed a well-differentiated grade 2 neuroendocrine tumor with lymphovascular invasion.

Adjuvant Therapy

Following surgery, the patient was started on a regimen of somatostatin analogs (octreotide) to manage symptoms and inhibit tumor growth. The decision to initiate adjuvant therapy was guided by the tumor's histopathological features, including the Ki-67 index and mitotic count.

Ongoing Management and Follow-Up

Regular follow-up appointments were established to monitor the patient’s clinical status, including serum chromogranin A levels and imaging studies to assess for recurrence or progression of disease.

Managing Side Effects and Complications

While on octreotide therapy, the patient experienced some side effects, including diarrhea and abdominal discomfort, which were managed with supportive care. Education regarding the potential side effects of therapy was provided, and adjustments to the dosage were made as necessary to optimize her quality of life.

Discussion

Diagnostic Challenges in Neuroendocrine Tumors

Due to their frequently insidious onset and variable clinical presentation, the diagnosis of NETs presents special challenges. Symptoms are usually not so typical and nonspecific that delays in diagnosis are unavoidable unless there is a high index of suspicion in patients presenting with gastrointestinal complaints and hormonal syndromes.

Advanced imaging and biomarkers are required to make the heterogeneous histological features of NETs correctly diagnosed and staged. Gallium-68 DOTATATE PET scans have fundamentally changed the face of diagnostics with greater characterization of NETs and better localization of metastases.

Treatment Strategies for Neuroendocrine Tumors

This should be tailored to the grade and stage of the tumor or by patient preference for each NET. The most important associations that deliver the best results are surgical resection, medical therapy, and surveillance.

Somatostatin analogs remain the cornerstone of therapy for functionally active NETs. In addition to alleviating symptoms, this treatment exerts some antitumor effects. The decision to use therapy must always be made in view of current clinical conditions, including the patient's general condition, tumor burden, and response to prior therapies.

Conclusion

This case report well represents the controversial aspects of the diagnosis and management of neuroendocrine tumors, especially in the case of a metastatic pancreatic neuroendocrine tumor. Advanced imaging techniques combined with multidisciplinary collaboration are crucial for managing such cases.

But as we learn more about NETs, research will continue; we will make better tools for diagnosis and treatments that, ultimately, will help a patient a lot better. Education and awareness among healthcare providers promote navigating the intricacies of NETs, ensuring timely diagnosis and effective treatment strategies to best serve the patient's wishes.

References

1. Modlin, I. M., & Kidd, M. (2015). Neuroendocrine tumors: A comprehensive review of the epidemiology, diagnosis, and management. The American Journal of Medicine, 128(12), 1205-1212.

2. Kulke, M. H., & Meyer, T. (2019). Management of neuroendocrine tumors: An evolving landscape. Nature Reviews Clinical Oncology, 16(2), 77-91.

3. Strosberg, J. R., & Cheema, F. H. (2018). Treatment of neuroendocrine tumors: Current perspectives. Therapeutic Advances in Medical Oncology, 10, 1758835918757112.

4. Krenning, E. P., & Bakker, W. H. (2007). Somatostatin receptor imaging in neuroendocrine tumors: Applications in clinical practice. Journal of Nuclear Medicine, 48(5), 727-732.

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