Cushing Syndrome Secondary to A Thymic Carcinoid Tumor Due to Multiple Endocrine Neoplasia Type 1

Ghazi, Ali A.; Dezfooli, Azizollah Abbasi; Mohamadi, Farzaneh; Yousefi, Seyed Vahid; Amirbaigloo, Alireza; Ghazi, Siavash; Pourafkari, Marina; Berney, Daniel M.; Ellard, Sian; Grossman, Ashley

doi:10.4158/ep11038.cr

Cited by 27 publications

(20 citation statements)

References 20 publications

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“…All patients with ECS due to NET had a typical clinical picture of CS with a 'moon face', truncal obesity, weight gain, muscle weakness, fatigability and purple striae, while patients with SCLC did not show these classic manifestations of Cushing's syndrome. Patient 12 was a non-classic case of MEN I syndrome with Cushing's syndrome due to a thymic neuroendocrine tumour and hyperparathyroidism who underwent thoracotomy twice because of tumour recurrence, as reported earlier [18]. All patients except four (cases 6, 11, 13 and 14) had hypertension, with systolic blood pressures ranging from 120 to 190 mm Hg and diastolic blood pressures ranging from 80 to 100 mmHg.…”

Section: Sclc Patients (Patients 1 10 11 14)mentioning

confidence: 74%

“…Histopathologic evaluations including immunohistochemistry (IHC) studies were interpreted by three expert academic pathologists. One patient had MEN1 syndrome and has been reported earlier [18]. The protocol was approved by the ethics committee of the Research Institute for Endocrine Sciences affiliated to Shahid Beheshti University of Medical Sciences, Tehran, Iran.…”

Section: Prace Oryginalnementioning

confidence: 99%

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Ektopowy zespół Cushinga u pacjentów z nowotworem śródpiersia lub płuc — doniesienie z ośrodka trzeciego stopnia referencyjności w Iranie

Ghazi

Dezfooli

Amirbaigloo

et al. 2015

Endokrynologia Polska

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Introduction: Ectopic Cushing`s syndrome (ECS) secondary to neuroendocrine tumours (NETs) of the lung and mediastinum are rarely encountered. In this study, we present our experience in Iran on 15 patients with ECS secondary to lung and mediastinal tumours over a period of 27 years. Material and methods: Since 1985, 15 patients with ECS secondary to lung and mediastinal tumours have been diagnosed and prospectively followed by the endocrinology team of Taleghani Hospital, Tehran, Iran. The clinical signs and symptoms, laboratory findings, radiological features, immunohistochemical characteristics, management strategies and outcome data are here presented. Results: There were six women and nine men, aged 26-70 years, all presenting with typical features of Cushing's syndrome. Based on histopathologic evaluations, four patients had small cell lung cancer (SCLC) and seven patients had pulmonary NETs, one patient had a thymic NET, and one case was diagnosed as a lung tumourlet. The mean ± SD serum cortisol, 24h urine free cortisol and plasma ACTH were 47.2 ± 20.5 µg/dL, 2,702 ± 5,439 µg/day, and 220 ± 147 pg/mL, respectively. Pulmonary lesions ranged in diameter from 1.1 to 4 cm (mean 1.9 ± 1.1 cm). One patient had a 10 cm mediastinal mass. The duration of follow up in these cases was between one month and seven years (mean 29.9 ± 27.5 months). The four patients with SCLC died within three months of diagnosis. Conclusion: Our data demonstrates the protean clinical and laboratory manifestations of ECS secondary to lung and mediastinal tumours, the problems encountered in diagnosis, and the need for a multidisciplinary approach. This study confirms other series from Western Europe and North America that, unlike the SCLC patients who show a poor outlook, ECS secondary to lung carcinoids has a more favourable prognosis. (Endokrynol Pol 2015; 66 (1): 2-9)

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Section: Sclc Patients (Patients 1 10 11 14)mentioning

confidence: 74%

Section: Prace Oryginalnementioning

confidence: 99%

Ektopowy zespół Cushinga u pacjentów z nowotworem śródpiersia lub płuc — doniesienie z ośrodka trzeciego stopnia referencyjności w Iranie

Ghazi

Dezfooli

Amirbaigloo

et al. 2015

Endokrynologia Polska

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“…These values were relatively low compared to other malignancies (17,18). In thymic malignancies, Ghazi et al recently reported that Ki67 labeling indices changed 5% in a thymic typical carcinoid at the first surgery to 30% in the invasive recurrent lesions at the second surgery (19). Since Ki67 is a marker of cell proliferation, the result seems to be reasonable in thymomas with slow growth.…”

Section: Discussionmentioning

confidence: 96%

Clinicopathological analysis of small-sized thymoma with podoplanin and Ki 67 expression analysis

Yokota

Tateyama

Yano

et al. 2012

Molecular and Clinical Oncology

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Abstract. Thymoma is the most common tumor of the anterior mediastinum for which surgical resection is currently the primary form of treatment. An increase in the incidence of a small-sized (≤3 cm) thymoma (SST) has recently been noted. Clinicopathological factors and prognosis of SST have not been reported previously. In this study, the clinicopathological data of 21 SST patients were reviewed and podoplanin and Ki67 immunohistochemistry were assessed to determine the biological behavior of SSTs. Pathological diagnosis of SSTs revealed the following types: A (n=1), AB (n=8), B1 (n=5), B2 (n=6) and B3 (n=1). The Masaoka-Koga stages of 21 thymoma patients were I (n=16), II (n=3), III (n=1) and IVb (n=1). In the case of the stage IVb thymoma, phrenic nerve, mediastinal pleura invasion and anterior mediastinal lymph node metastasis were observed. The Ki67 labeling index of this stage IVb was found to be 3.2. This case was also positive for podoplanin and was one of the only 2 cases that were positive for podoplanin. This patient succumbed to thymoma. Advanced stage thymomas are possibly included in SSTs although the majority of SSTs are classified into early stages of disease. Findings of this study suggest that podoplanin analyzed by immunohistochemistry may be useful to determine the malignant behavior of SSTs.

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“…However, there are numerous case reports showing that MEN-1 patients with thymic carcinoids could also produce ectopic ACTH [18,[42][43][44] . Thus, while rare, the possibility of MEN-1 should be considered in all patients with thymic carcinoids.…”

Section: Thymic Carcinoids With Csmentioning

confidence: 99%

Thymic Neuroendocrine Neoplasms: Biological Behaviour and Therapy

Jia

Šulentić

et al. 2017

Neuroendocrinology

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Thymic neuroendocrine neoplasms are rare tumours, but their management can often be highly problematic. While previously assumed to be essentially variants of bronchopulmonary (lung) carcinoids, they are generally more aggressive and more difficult to treat. Some 25% are associated with multiple endocrine neoplasia-1, while a higher proportion are associated with the ectopic ACTH syndrome, and occasionally both. We discuss the classification of these tumours, their biology as far as is known, and their clinical, biochemical and imaging features. We also review possible management options and suggest stratagems to optimise their treatment, which even today is far from optimal.

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Cushing Syndrome Secondary to A Thymic Carcinoid Tumor Due to Multiple Endocrine Neoplasia Type 1

Cited by 27 publications

References 20 publications

Ektopowy zespół Cushinga u pacjentów z nowotworem śródpiersia lub płuc — doniesienie z ośrodka trzeciego stopnia referencyjności w Iranie

Ektopowy zespół Cushinga u pacjentów z nowotworem śródpiersia lub płuc — doniesienie z ośrodka trzeciego stopnia referencyjności w Iranie

Clinicopathological analysis of small-sized thymoma with podoplanin and Ki 67 expression analysis

Thymic Neuroendocrine Neoplasms: Biological Behaviour and Therapy

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