• Journal of Digestive Cancer Research
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• v.3 no.1
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• pp.21-25
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• 2015

Gastric neuroendocrine tumors (GNETs, also known as gastric carcinoids) are rare form of hormone-secreting neoplasms that present with varied clinical syndromes. There are four types of GNETs based on size, proliferation, localization, differentiation, and hormone production. Type I GNET is related to autoimmune atrophic gastritis and hypergastrinemia. Type II GNETs are related to multiple endocrine neoplasia (MEN)-1, Zollinger-Ellison syndrome and hypergastrinemia. Type 3 GNETs are not associated with any background pathology, and type 4 GNETs are poorly differentiated tumors. The most useful diagnostic and prognostic marker for gastrointestinal NETs is plasma chromogranin A (CgA) levels. Endoscopic ultrasound is the method of choice to determine tumor size and depth of infiltration. For optimal management, the type, biology, and stage of the tumor must be considered. Here, we provide a comprehensive and up-to-date review of GNETs.

• The Korean Journal of Nuclear Medicine
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• v.25 no.1
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• pp.122-128
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• 1991

Multiple endocrine neoplasia type 2 (MEN type 2, Sipple's syndrome) is a rare disorder characterized by the association of medullary carcinoma of the thyroid, parathyroid hyperplasia and can be diagonsed in early stage of the disease by meticulous screening tests of the family. This case report describes the location and categorization of tumors using $^{99m}Tc-pertechnetate,\;^{131}I-NaI,\;^{99m}Tc-pentavalent(V)$, DMSA $^{131}I-MIBG$ scans in two cases of MEN type 2 occurred in a 32-year old women and her 29-year old brother. In MEN type 2, we think, combined use of $^{99m}Tc-(V)-DMSA,\;^{99m}Tc-pertechnetate\;and\;^{131}I-MIBG$ may be useful for the categorization of tumor mass lesions and planning appropriate therapy.

• Journal of Chest Surgery
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• v.52 no.6
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• pp.420-424
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• 2019

Atypical thymic carcinoid is an extremely rare tumor with a poor prognosis. In addition to its known association with multiple endocrine neoplasia type 1, its hallmark characteristics include local invasion and early distant metastasis. In this report, we share our experience treating atypical thymic carcinoid in a patient with Zollinger-Ellison syndrome.

• Journal of Yeungnam Medical Science
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• v.31 no.2
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• pp.131-134
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• 2014

Familial isolated primary hyperparathyroidism(FIPH) is associated with multiple endocrine neoplasia type 1 (MEN1) syndrome, primary hyperparathyroidism accompanied by jaw-tumor syndrome, and familial hypocalciuric hypercalcemia. FIPH may be an early stage of MEN1 or an allelic variant of MEN1. Thymic carcinoid tumor is a rare tumor in MEN1 syndrome. Here, the authors report the case of a 40-year-old man diagnosed with recurrent thymic carcinoid tumor and FIPH. Both the patient and his elder sister had been previously diagnosed to have FIPH with a novel frameshift mutation in the MEN1 gene. Initially, the patient underwent thymectomy because of an incidental finding of a mediastinal mass in his chest X-ray, and had remained asymptomatic over the following 4 years. Pancreas computed tomography conducted to evaluate MEN1 syndrome revealed anterior and middle mediastinal masses, and resultantly, massive mass excision was performed. Histological findings disclosed atypical carcinoids with infiltrative margins. In view of the thymic carcinoid tumor relapse that occurred in this patient, the authors recommend that regular pancreas and pituitary imaging studies be conducted for FIPH associated with a MEN1 gene mutation.

• The Korean Journal of Nuclear Medicine
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• v.29 no.4
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• pp.460-464
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• 1995

[ $^{201}T1/^{99m}Tc$ ] pertechnetate subtraction scintigraphy of the parathyroid gland was performed in a study of 8 patients with clinical and biochemical evidence of hyperparathyroidism for diagnosis and Localization of the lesion prior to sugery. 6 cases of parathyroid adenomas were accurately localized and 2 cases of diffuse hyperplasia associated with chronic renal failure(CRF) and multiple endocrine neoplasia (MEN) type I. $^{201}T1/^{99m}Tc$ pertechnetate scan was useful for patients with hyperparathyroidism, especially in definite localization preoperatively.

• Molecules and Cells
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• v.45 no.4
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• pp.202-215
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• 2022

The androgen receptor (AR) is an important therapeutic target for treating prostate cancer (PCa). Moreover, there is an increasing need for understanding the AR-independent progression of tumor cells such as neuroendocrine prostate cancer (NEPC). Menin, which is encoded by multiple endocrine neoplasia type 1 (MEN1), serves as a direct link between AR and the mixed-lineage leukemia (MLL) complex in PCa development by activating AR target genes through histone H3 lysine 4 methylation. Although menin is a critical component of AR signaling, its tumorigenic role in AR-independent PCa cells remains unknown. Here, we compared the role of menin in AR-positive and AR-negative PCa cells via RNAi-mediated or pharmacological inhibition of menin. We demonstrated that menin was involved in tumor cell growth and metastasis in PCa cells with low or deficient levels of AR. The inhibition of menin significantly diminished the growth of PCa cells and induced apoptosis, regardless of the presence of AR. Additionally, transcriptome analysis showed that the expression of many metastasis-associated genes was perturbed by menin inhibition in AR-negative DU145 cells. Furthermore, wound-healing assay results showed that menin promoted cell migration in AR-independent cellular contexts. Overall, these findings suggest a critical function of menin in tumorigenesis and provide a rationale for drug development against menin toward targeting high-risk metastatic PCa, especially those independent of AR.

• The Korean Journal of Nuclear Medicine
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• v.29 no.3
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• pp.319-327
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• 1995

$^{131}I$-metaiodobenzylguanidine(MIBG) has been used for the diagnosis and treatment of neural crest tumors. We report our experience with this agent in 8 patients[1 multiple endocrine neoplasia(MEN) type IIb; 2 malignant pheochromocytoma; 5 medullary thyroid carcinoma(MTC)]. The therapeutic procedure consisted of 30-200 mCi of $^{131}I-MIBG$ administered by slow I.V. infusion, given at 3-6 months intervals. Commutative activity ranged from 150 mCi to 410 mCi, in 1 to 4 courses. $^{131}I-MIBG$ therapy resulted in significant disease free interval in 1 malignant pheochromocytoma(no measurable lesion) after surgery; complete hormonal and tumoral response in 2 MTC(1 MEN IIb): stable disease in 1 recurred pheochromocytoma(MEN IIb): stable disease but symptomatic improvement in 1 MTC, progressive disease in 1 malignant pheochromocytoma and 2 MTC. The patients who showed progression appeared to have large inoperable tumors or postoperative remnant tumors.