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Expression of transcription factors in MEN1-associated pancreatic neuroendocrine tumors

Summary

MEN1-associated pancreatic neuroendocrine tumors (pNETs) may potentially express distinct hormones, but the mechanism has not been elucidated. Transcription factors such as MafA and Pdx1 have been identified to lead to beta cell differentiation, while Arx and Brn4 to alpha cell differentiation in developing pancreas. We hypothesized those transcription factors are important to produce specific hormones in pNETs, similarly to developing pancreas, and examined the expression of transcription factors in a case of MEN1 who showed immunohistological coexistence of several hormone-producing pNETs including insulinoma. A 70-year-old woman was found to manifest hypoglycemia with non-suppressed insulinemia and hypercalcemia with elevated PTH level. She was diagnosed as MEN1 based on the manifestation of primary hyperparathyroidism, pituitary adenoma and insulinoma, with genetic variation of MEN1 gene. She had pylorus-preserving pancreaticoduodenectomy because CT scan and SACI test indicated that insulinoma was localized in the head of the pancreas. Histopathological finding was MEN1-associated NET, G1. Interestingly, immunohistological examination of the resected pancreas revealed that two insulinomas, a glucagon-positive NET and a multiple hormone-positive NET coexisted. Hence, we examined the expression of transcription factors immunohistochemically to elucidate the role of the transcription factors in MEN1-associated hormone-producing pNETs. We observed homogeneous expressions of MafA and Pdx1 in insulinomas and Arx in glucagon-positive NET, respectively. Moreover, multiple hormone-positive NETs expressed several transcription factors heterogeneously. Collectively, our results suggested that transcription factors could play important roles in the production of specific hormones in MEN1-associated pNETs, similar to islet differentiation.

Learning points:

To date, it has been shown that different hormone-producing tumors coexist in MEN1-associated pNETs; however, the underlying mechanism of the hormone production in MEN1-associated pNETs has not been well elucidated.

Although this case presented symptomatic hypoglycemia, several hormone-producing pNETs other than insulinoma also coexisted in the pancreas.

Immunohistochemical analysis showed MafA and Pdx1 expressions distinctly in insulinoma, and Arx expression particularly in a glucagon-positive NET, while a multiple hormone-positive NET expressed MafA, Pdx1 and Arx.

Collectively, clinicians should consider that several hormone-producing pNETs may coexist in a MEN1 case and examine both endocrinological and histopathological analysis of pNETs, regardless of whether symptoms related to the excess of hormones are observed or not.

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Sclerosing thymoma-like thymic amyloidoma with nephrotic syndrome: a case report

Primary localized amyloidosis presenting as an isolated mediastinal mass is extremely rare, especially in the thymus. Sclerosing thymoma is also an extremely rare anterior mediastinal tumor, pathologically cha...

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Sudden sensorineural hearing loss in children and adolescents: Clinical characteristics and age-related prognosis

Publication date: Available online 6 September 2017
Source:Auris Nasus Larynx
Author(s): Jin Youp Kim, Jae Joon Han, Woong Sang Sunwoo, Ja-Won Koo, Seung-Ha Oh, Min-Hyun Park, Young Ho Kim
ObjectiveAlthough many studies have investigated sudden sensorineural hearing loss (SSNHL) in adults, there were few studies on SSNHL in the pediatric population; especially research on treatment and prognosis of pediatric SSNHL was limited. The aim of this study was to evaluate clinical characteristics, treatment outcomes and prognostic factors in children and adolescents with SSNHL.MethodsA retrospective review of medical records of 67 pediatric patients (67 ears) who had diagnosed with SSNHL at our hospitals was performed to analyze patients' clinical manifestations and audiograms. All patients were treated with high-dose systemic prednisolone (1mg/kg), and 17 of them underwent intratympanic steroid injection therapy. Audiological evaluation was carried out before and after treatment, and hearing recovery was defined as complete recovery and partial recovery according to Siegel's criteria. Patients were divided into two groups: childhood group (ages between 4 and 12 years old) and adolescence group (age>12years), and clinical characteristics and treatment outcomes were investigated. In addition, patients were divided into two groups according to degree of hearing recovery, and evaluation was made regarding possible prognostic factors.ResultsThe recovery rate in total 67 patients was 55.2%. The recovery rate of the childhood group was significantly lower than that of the adolescence group (p=0.038). While the presence of vertigo did not significantly correlate with prognosis (p=0.219), the presence of tinnitus was significantly associated with hearing recovery (p=0.005). Audiological assessment revealed that a low initial hearing threshold, high speech discrimination score, and descending type of audiogram were positively associated with hearing recovery (p=0.002, p=0.003, and p=0.029, respectively).ConclusionThe childhood group had worse treatment outcomes than the adolescence group. High initial hearing threshold and absence of tinnitus were poor prognostic factors of hearing recovery. Active treatment is required for patients with these poor prognostic factors and childhood patients with SSNHL.



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Response to the letter to the editor of Lambros et al.

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