Recurrent Ectopic Adrenocorticotropic Hormone Producing Thymic Carcinoid Detected With Octreotide Imaging

Silva, Frieda; Vázquez-Sellés, José; Aguiló, F; Vázquez, Gilberto; Flores, Charlie

doi:10.1097/00003072-199902000-00007

Cited by 17 publications

(4 citation statements)

References 4 publications

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“…In the presented patient, imagery diagnostic initially failed to visualize the thymic tumor. There are only few reports on non-diagnostic chest CT or MRI evaluation in patients with EAS NETTs ( 21 – 23 ); however, in the presented cases, NETTs were found on SRS, which is contrary to our report. The first 18F-FDG-PET-CT localized an area of 2.1 SUVmax in the anterior mediastinum, initially considered a residual thymus because of the physiological FDG uptake.…”

Section: Discussioncontrasting

confidence: 99%

Case report: A challenging case of severe Cushing’s syndrome in the course of metastatic thymic neuroendocrine carcinoma with a synchronous adrenal tumor

Dzialach,

Wojciechowska-Luzniak,

Maksymowicz

et al. 2024

Front. Endocrinol.

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Ectopic ACTH syndrome (EAS) remains one of the most demanding diagnostic and therapeutic challenges for endocrinologists. Thymic neuroendocrine tumors account for 5%–10% of all EAS cases. We report a unique case of a 31-year-old woman with severe EAS caused by primary metastatic combined large-cell neuroendocrine carcinoma and atypical carcinoid of the thymus. The patient presented with severe hypercortisolemia, which was successfully controlled with continuous etomidate infusion. Complex imaging initially failed to detect thymic lesion; however, it revealed a large, inhomogeneous, metabolically active left adrenal mass infiltrating the diaphragm, suspected of primary disease origin. The patient underwent unilateral adrenalectomy, which resulted in hypercortisolemia resolve. The pathology report showed an adenoma with adrenal infarction and necrosis. The thymic tumor was eventually revealed a few weeks later on follow-up imaging studies. Due to local invasion and rapid progression, only partial resection of the thymic tumor was possible, and the patient was started on radio- and chemotherapy.

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Section: Discussioncontrasting

confidence: 99%

Case report: A challenging case of severe Cushing’s syndrome in the course of metastatic thymic neuroendocrine carcinoma with a synchronous adrenal tumor

Dzialach,

Wojciechowska-Luzniak,

Maksymowicz

et al. 2024

Front. Endocrinol.

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“…While standard 111 In-octreotide scintigraphy (Octreoscan) may occasionally be useful on its own it generally has not shown a source undetectable on CT and MRI, although it does add supportive functional data [12,13,45]. However, even if the data do not support the superiority of the Octreoscan over CT scanning, as shown in a direct comparison [7,9,45], it has been proved useful to enhance their resolution for findings not seen on first assessment [9,15], and there are isolated cases that report its superiority [46,47]. It has been recently suggested that its performance is useful as complementary modality to CT and MRI in specific cases [15,48,49] and should be considered when other imaging modalities fail to localise the ACTH-secreting tumour [18,49,50].…”

Section: ≤5mentioning

confidence: 99%

The ectopic ACTH syndrome

Alexandraki

Grossman

2010

Rev Endocr Metab Disord

188

131

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Ectopic Cushing's syndrome usually relates to the ectopic ACTH syndrome (EAS) and represents ∼20% of ACTH-dependent and ∼10% of all types of Cushing's syndrome (CS). Nearly any neuroendocrine or non-endocrine tumours may be associated with EAS, but the more prevalent tumours are bronchial carcinoids, small cell lung carcinomas, pancreatic carcinoids, thymic carcinoids, medullary carcinomas of the thyroid, and phaeochromocytomas. Occult tumours are highly represented in all the series (12-38%) and constitute the more challenging cases of EAS, requiring long term follow-up. The lack of any completely reliable diagnostic test procedure and imaging to clearly reveal the source of EAS suggests that we should adopt a step-by-step multidisciplinary approach for their diagnosis and therapeutic management. Clinical features are often similar in ACTH-dependent CS, but the rapid onset and progress may suggest an ectopic source. A combination of biochemical tests and imaging studies seems the most appropriate approach for the prompt identification of EAS, even if there are several pitfalls to be avoided along the way. The most appropriate management for cure of EAS, when its source is identified, is surgical excision after controlling the hypercortisolaemia by inhibitors of cortisol secretion and other newer modalities alone or in combination; bilateral adrenalectomy remains an alternative option. Tumour histology, the presence of metastases and the effective control of hypercortisolaemia affect mortality and morbidity. If a source repeatedly fails to be found, the prognosis is often favourable but the identification of a malignant tumour should still be sought during life-long follow-up to avoid the calamity of misdiagnosis.

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“…Considering the low prevalence of patients with CS secondary to ectopic tumors, an ectopic source of ACTH hypersecretion seems to be more frequent in patients with biochemical episode of cycling [14]. Neuroendocrine tumors of the thymus are the most frequently represented, followed by neuroendocrine bronchial tumors [4, 19–26]. Other tumors reported as responsible of cyclic ectopic CS are pancreatic, renal and gastric NET [27–29], epithelial thymoma [30], phaeochromocytoma [31], carotid glomus tumor [32] and ectopic pituitary adenoma [33].…”

Section: Discussionmentioning

confidence: 99%

A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome

Albani¹,

Berr²,

Beuschlein

et al. 2019

BMC Endocr Disord

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BackgroundClinical care of patients with cyclic Cushing’s syndrome (CS) is challenging. Classical pitfalls include incorrect subtyping, unnecessary surgical procedures and delayed definite treatment.Case presentationA 43-year-old female suffered from a rapidly cycling ectopic CS. She experienced six cycles of severe hypercortisolism within a 2 year period (maximum plasma cortisol 5316 nmol/L, normal range 124.2–662.4 nmol/L; maximum urinary free cortisol 79,469 nmol/24 h, normal range < 414 nmol/24 h) lasting 2–9 weeks. The episodes were associated with pronounced hypokalemia (lowest K+ value recorded 2.4 mmol/l) and progressive signs and symptoms of CS. A bilateral inferior petrosal sinus sampling (BIPSS) performed during a trough phase was false positive for pituitary ACTH overproduction resulting in unnecessary transsphenoidal surgery while a second BIPSS performed during an active phase was indicative for ectopic CS. The 18F-DOPA PET/CT showed a pancreatic lesion, which was subsequently partially removed. Surprisingly, the histopathology was conclusive for ACTH-positive lymph node metastasis located in the retro-duodenal tissue of an occult neuroendocrine tumor WHO grade II. The primary tumor has not been identified so far and, because of the persistent hypercortisolism, the patient underwent bilateral adrenalectomy. Two years later, ACTH levels started to increase progressively. Percutaneous biopsy of a newly identified suspected lesion in the fifth thoracic vertebra revealed a metastasis with positive staining for ACTH, synaptophysin and chromogranin A. Therapy with carboplatin and etoposide was started and, since then, the patient underwent 12 cycles of chemotherapy.ConclusionsWe report the challenging case of a rapidly cycling CS secondary to ACTH-secreting neuroendocrine intestinal tumor of unknown primary. We highlight the importance of performing diagnostic tests only during the phases of active cortisol secretion and as soon as first symptoms appear to avoid pitfalls.

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Recurrent Ectopic Adrenocorticotropic Hormone Producing Thymic Carcinoid Detected With Octreotide Imaging

Cited by 17 publications

References 4 publications

Case report: A challenging case of severe Cushing’s syndrome in the course of metastatic thymic neuroendocrine carcinoma with a synchronous adrenal tumor

Case report: A challenging case of severe Cushing’s syndrome in the course of metastatic thymic neuroendocrine carcinoma with a synchronous adrenal tumor

The ectopic ACTH syndrome

A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome

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