Adrenal incidentaloma (AI) is rare and found in approximately 2-4% of abdominal computed tomography scans. Up to 10% of patients with AI have autonomous secretion of adrenal hormones. If not quickly diagnosed and adequately treated, the outcome may be devastating to the patient. On very rare occasions, a pheochromocytoma may, in addition to the production of catecholamine, produce adrenocorticotropic hormone causing Cushing disease. We present a case of a patient with pheochromocytoma and Cushing syndrome.

This report describes the case of a 63-year-old female with a metastatic neuroendocrine tumor (NET). Imaging studies revealed a primary hepatic NET (PHNET) originating in the porta hepatis and associated with extensive hepatic metastasis. This represents an extremely rare presentation of PHNET associated with ectopic adrenocorticotropic hormone (ACTH) production and hypercortisolism. As such, it is a unique presentation of an otherwise rare pathology and hence we believe it contributes to the literature on PHNETs by supplementing it with information on an uncommon variation of an infrequent pathology.

Often Only a Side Event, but Sometimes Groundbreaking - an Ectopic ACTH Secretion as an Example of a Paraneoplastic Syndrome Abstract. Various tumors can cause a paraneoplastic ACTH production, particularly small-cell lung cancers. Patients suffer from a secondary Cushing syndrome with hypokalemia, hypertension, hyperglycemia and edema. There are three therapeutic approaches: treatment of the underlying disease, hormonal inhibition and symptomatic treatment. The prognosis of an ACTH-producing lung cancer is poor due to numerous complications. In the presented case study, the patient refused treatment of the underlying disease, therefore a palliative symptomatic treatment became of great importance.
Zusammenfassung. Eine paraneoplastische ACTH-Produktion tritt bei diversen Tumorentitäten auf, vor allem aber bei kleinzelligen Bronchialkarzinomen. Die Patienten präsentieren sich mit einem sekundären Cushing-Syndrom mit Hypokaliämie, arterieller Hypertonie, Hyperglykämie und Ödemen. Folgende drei Therapiesäulen bestehen: Eine Behandlung der Grunderkrankung, eine medikamentöse antihormonelle und eine symptomatische Therapie. Die Prognose eines ACTH-produzierenden Bronchialkarzinoms ist aufgrund der hohen Komplikationsrate insgesamt infaust. In unserem Fall lehnte die Patientin die Behandlung der Grunderkrankung ab, weshalb die palliative Behandlung mit symptomatischer Therapie in den Vordergrund rückte.

Desmoplastic spindle cell tumors of liver are rare tumors of low malignant potential characterized by well-demarcated nests of spindle and epithelioid cells in a dense desmoplastic stroma. While surgery remains the definitive treatment, there have been reports of tumor recurrence locally and metastasis which respond poorly to chemotherapy. Hepatic transplant has been attempted in cases of recurrence or large size of primary tumor. Long-term follow-up and imaging surveillance are required as these tumors have shown a tendency for recurrence many years after initial therapy.

Cushing\'s syndrome (CS) due to ectopic adrenocorticotrophic hormone (ACTH) is associated with a variety of tumours most of which arise in the thorax or abdomen. Prostate carcinoma is a rare but important cause of rapidly progressive CS. To report a case of severe CS due to ACTH production from prostate neuroendocrine carcinoma and summarise previous published cases. A 71-year-old male presented with profound hypokalaemia, oedema and new onset hypertension. The patient reported two weeks of weight gain, muscle weakness, labile mood and insomnia. CS due to ectopic ACTH production was confirmed with failure to suppress cortisol levels following low- and high-dose dexamethasone suppression tests in the presence of a markedly elevated ACTH and a normal pituitary MRI. Computed tomography demonstrated an enlarged prostate with features of malignancy, confirmed by MRI. Subsequent prostatic biopsy confirmed neuroendocrine carcinoma of small cell type and conventional adenocarcinoma of the prostate. Adrenal steroidogenesis blockade was commenced using ketoconazole and metyrapone. Complete biochemical control of CS and evidence of disease regression on imaging occurred after four cycles of chemotherapy with carboplatin and etoposide. By the sixth cycle, the patient demonstrated radiological progression followed by recurrence of CS and died nine months after initial presentation. Prostate neuroendocrine carcinoma is a rare cause of CS that can be rapidly fatal, and early aggressive treatment of the CS is important. In CS where the cause of EAS is unable to be identified, a pelvic source should be considered and imaging of the pelvis carefully reviewed.

BACKGROUND: Ectopic adrenocorticotropic hormone-producing primary liver tumors are rare, especially in children. We report the case of an adolescent boy of mixed Dutch and Moroccan descent with an adrenocorticotropic hormone-producing calcifying nested stromal-epithelial tumor with long-term follow-up. Thus far, only two such cases have been reported.
METHODS: A 16-year-old boy of mixed Dutch and Moroccan descent presented with Cushing syndrome and a palpable abdominal mass. A calcifying nested stromal-epithelial tumor was diagnosed. Postoperatively, his plasma adrenocorticotropic hormone concentration normalized. He remains in complete remission 13 years after tumor resection.
CONCLUSIONS: Calcifying nested stromal-epithelial tumor should be in the differential diagnosis of liver tumors, especially if associated with Cushing syndrome as significant morbidity and mortality may be associated. Literature on the topics involved is comprehensively reviewed.