Rare Chest Tumor Causes Ectopic Cushing’s Syndrome | The patient’s symptoms subside after surgery to remove the cancer tumor

A 33-year-old man with ectopic Cushing’s syndrome has seen his symptoms ease after undergoing surgery to remove a rare, slow-growing cancerous tumor from his chest.

The carcinoid tumor, a type of neuroendocrine tumor, was the cause of the man’s condition, researchers said.

“The patient had complete resolution of symptoms after surgery and no recurrences in 2 years of follow-up. Furthermore, with early diagnosis and timely surgical intervention, the patient survived without sequelae. [other resulting conditions] … 2 years after surgery,” the team wrote.

His case was described in the report, “Cushing’s syndrome due to atypical mediastinal carcinoid”, published in the magazine Radiology Case Reports.

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No symptoms for the patient 2 years after surgery

Cushing’s syndrome is characterized by symptoms associated with hypercortisolism, or high levels of cortisol. Cushing’s disease, a common form of the syndrome, is usually caused by benign tumors in the brain’s pituitary gland that lead to excessive production of adrenocorticotropic hormone (ACTH). In turn, excessive ACTH levels signal the adrenal glands, located above the kidneys, to produce too much cortisol.

In rare cases, known as ectopic Cushing’s syndrome, the condition is caused by ACTH-producing tumors found outside the pituitary or adrenal glands. Lung tumors represent about 50% of these cases.

Now, scientists in Vietnam have described the rare case of a young man with ectopic Cushing’s syndrome caused by a tumor in the mediastinum – the central compartment in the chest cavity that contains the heart, esophagus, trachea, thymus and other important structures.

The man was admitted to the hospital complaining of pain in his right thigh that had lasted for several days. He had recently been diagnosed with type 2 diabetes, for which he was being treated with metformin.

Physical examination and further tests revealed that he had an abscess on his right thigh. The abscess and resulting infection were controlled after incision and antibiotic therapy.

Laboratory tests showed that he had elevated levels of blood sugar, or glucose (11.8 millimoles per liter (mmol/L); normal range: 4.1–5.8 mmol/L). The percentage of glycated hemoglobin, that is, the portion of hemoglobin containing sugar in the blood, was 7.6% (normal range: 4.8–5.9%), indicative of diabetes. He was also diagnosed with hypokalemia, or low levels of potassium in his blood.

Blood sugar levels remained unstable despite insulin treatment, and potassium levels remained low regardless of potassium replacement treatment. Other laboratory tests to diagnose the cause of hypokalemia, including 24-hour urinary potassium, suggested renal potassium wasting.

Random serum cortisol was elevated in 1664 nanomol per liter (nmol/L; normal range: 120–620 nmol/L). ACTH was also high at 854 picograms per milliliter (pg/mL; normal range: 7.2–63 pg/mL).

Low- and high-dose dexamethasone suppression tests, which are used to screen for hypercortisolism and determine the source of excess cortisol, were ineffective in reducing cortisol levels, suggesting the presence of an ectopic ACTH-producing tumor.

MRI scans confirmed that he had no pituitary tumor, and further CT scans identified a mediastinal mass. There were no signs of lymph node involvement or tumor spread. The mass was completely surgically removed and was considered an intermediate grade tumor.

The tumor cells were positive for synaptophysin, CD56 and chromogranin, which are markers of neuroendocrine tumors (NETs) – rare tumors that affect cells that release hormones into the bloodstream (neuroendocrine cells).

The patient had complete resolution of symptoms after surgery and no recurrences in 2 years of follow-up.

One day after surgery, ACTH and cortisol levels returned to normal, as did potassium levels, without further treatment. Two months after surgery, glucose levels were normal without insulin or oral diabetic medication.

The effectiveness of the surgery in the treatment of Cushing’s was confirmed in two years of follow-up, with the patient presenting complete resolution of the disease, with normal levels of ACTH, cortisol, potassium and glucose, without signs of tumor recurrence.

“We present the case of a young man who presented with non-specific Cushing symptoms but had multiple complications. [caused] due to excess glucocorticoids. Due to early recognition and complete resection of malignancies, this patient had a better outcome and survival,” the researchers concluded.

Rare Chest Tumor Causes Ectopic Cushing’s Syndrome | The patient’s symptoms subside after surgery to remove the cancer tumor

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