Man’s aggressive salivary gland cancer triggers Cushing’s syndrome: Report
Early identification and treatment may be lifesaving, researchers say
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Although acinic cell carcinoma — a cancer of the glands that produce saliva — is usually considered low risk, it can behave aggressively and cause Cushing’s syndrome, according to researchers who described such a case involving a 58-year-old man in Turkey.
They added that if not recognized and treated at an early stage, it can rapidly become life-threatening.
“The rarity of this entity poses a diagnostic challenge,” wrote the group of four researchers from a hospital in Turkey. “Awareness of this association is critical, as early identification and intervention may be lifesaving in selected patients.”
The case was described in a letter to the editor, titled “Paraneoplastic Cushing’s syndrome due to ACTH-secreting acinic cell carcinoma of the parotid gland: A rare case,” in the European Annals of Otorhinolaryngology, Head and Neck Diseases.
Man’s tumor produced ACTH, causing Cushing’s syndrome
Cushing’s syndrome occurs when the body is exposed to excess cortisol for a long time. Most cases are caused by a tumor in the pituitary gland, a small gland at the base of the brain, in which case it is called Cushing’s disease. Pituitary tumors release excess ACTH, a hormone that prompts the body to produce cortisol.
In rarer cases, ACTH is produced by a tumor outside the pituitary gland. This is known as ectopic Cushing’s syndrome. When the source of ACTH is a cancerous tumor, it is known as paraneoplastic Cushing’s syndrome. In rare cases, a cancer of the salivary glands is involved.
Acinic cell carcinoma is a rare, usually slow-growing cancer of the salivary glands that may originate from the parotid gland, a major salivary gland located near the ear. In this case, the man’s tumor produced ACTH, causing Cushing’s syndrome.
The man had been developing swelling on the right side of his neck for over six months. An MRI scan showed a solid mass deep within the parotid gland. Needle biopsies to remove a small piece of tissue for examination under a microscope suggested a malignant tumor, and surgery to remove the gland was planned.
Before surgery could take place, the man’s health worsened quickly. He developed severe body swelling, darkening of the skin, fluid around the lungs, and general physical decline. These changes suggested a hormonal abnormality rather than simple tumor growth, leading to hospital admission.
Salivary gland cancer can behave aggressively in rare cases
During hospitalization, swelling of the throat caused breathing failure. An emergency tracheotomy, which is a surgical opening in the neck to allow air into the lungs, was required. Despite this, the patient exhibited marked facial and neck swelling, as well as skin discoloration.
Further surgery and biopsy confirmed the diagnosis of acinic cell carcinoma. A PET/CT scan, which detects areas of high metabolic activity typical of cancer, showed intense uptake in the parotid gland. Blood tests revealed low sodium, low protein levels, and high blood sugar, classic signs of Cushing’s syndrome caused by excess ACTH.
Treatment focused on supportive care, including managing breathing, fluids, and metabolic imbalances. Definitive treatment would have required surgical removal of the tumor, but the man’s condition declined too rapidly. He died from cardiac arrest before surgery could be performed.
Although acinic cell carcinoma is usually considered a low-risk tumor, this case demonstrates that it can behave aggressively. Doctors should suspect a paraneoplastic syndrome when a parotid tumor is accompanied by unexplained systemic, or whole-body, symptoms. Early diagnosis and coordinated care may be lifesaving in those cases.
An ACTH-producing acinic cell carcinoma “represents a rare but highly aggressive clinical entity,” the researchers wrote. “Early recognition may allow stabilization of paraneoplastic manifestations and improve patient outcomes.”
