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Durable and deep response to CVD chemotherapy in SDHB-mutated metastatic paraganglioma: case report.

Front Endocrinol (Lausanne)

January 2025

Division of Abdominal Tumor, Department of Medical Oncology, Cancer Center and State Key Laboratory of Biological Therapy, West China Hospital, Sichuan University, Chengdu, Sichuan, China.

Introduction: Succinate dehydrogenase subunit B (SDHB)-mutated paragangliomas (PGLs) are rare neuroendocrine tumors characterized by increased malignancy, readily metastasizing, and poorer prognosis. Here we report a case of SDHB-mutated metastatic PGL, wherein the patient showed significant tumor shrinkage and complete symptom remission following chemotherapy. We aim to contribute additional evidence to the existing knowledge associated with SDHB-mutated PGLs.

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Purpose: Determining the primary origin of non-organ-confined neuroendocrine tumors (NETs) for accurate diagnosis and management. Neuroendocrine tumors are rare neoplasms with diverse clinical behaviors. Determining their primary origin remains challenging in cases of non-organ-confined NETs.

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Hypoglycemia in non-diabetic individuals is a rare but critical condition that often signals an underlying pathology. Insulinoma, a rare neuroendocrine tumor of the pancreas, is a key differential diagnosis. As the most common functional pancreatic neuroendocrine tumors, insulinomas originate from pancreatic islet cells and are predominantly benign.

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Paragangliomas originating from blood vessels are exceptionally rare, presenting diagnostic challenges due to their histological resemblance to other vascular neoplasms. We present a case study of a 60-year-old woman with underlying hypertension and dyslipidemia with obesity, initially diagnosed with angiosarcoma based on imaging and histological characteristics viewed via CT-guided biopsy. Intraoperative exploration revealed a lobulated tumor located between the inferior vena cava (IVC) and aorta measuring 7 cm × 8 cm, during which the patient developed transient hemodynamic instabilities.

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Introduction: Castleman's disease (CD) represents a rare polyclonal lymphoproliferative disorder characterized by atypical lymph node hyperplasia, the precise etiology of which remains undefined. Pancreatic involvement of CD is particularly uncommon and often misdiagnosed due to its nonspecific clinical features, making it difficult to distinguish from tumors with abundant blood supply such as solid pseudopapillary tumors and neuroendocrine tumors. Multimodal imaging plays a crucial role in diagnosing pancreatic CD and determining the extent of lymph node involvement.

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