Pituitary carcinoma

What it is

From: Orphanet

A rare pituitary tumor characterized by the presence of a pituitary adenoma that has metastasized either within the central nervous system, or to distant sites. The vast majority of pituitary carcinomas are hormonally active, most frequently with ACTH or prolactin production. The most common clinical symptoms are diabetes insipidus, optic nerve dysfunction, anterior pituitary dysfunction, palsy of cranial nerves III, IV, or VI, and headaches, although patients may also be asymptomatic. The tumors behave aggressively, and prognosis is poor.

Reported clinical features and what the terms mean

The following findings are associated with this condition in Orphanet. They are not a checklist for diagnosing yourself, and they do not all occur in every affected person. Some are examination, imaging or laboratory findings that cannot be recognised at home.

The frequency labels describe how often a finding was reported among people with the condition in the source. They do not give the chance that a person with that symptom has the condition. Definitions below reproduce HPO terminology; they explain the term, not the likely severity in an individual.

Headache · Very frequent (99-80%)

Cephalgia, or pain sensed in various parts of the head, not confined to the area of distribution of any nerve.

Increased circulating ACTH level · Very frequent (99-80%)

An abnormal increased in the concentration of corticotropin, also known as adrenocorticotropic hormone (ACTH), in the blood.

Increased circulating prolactin concentration · Very frequent (99-80%)

The presence of abnormally increased levels of prolactin in the blood. Prolactin is a peptide hormone produced by the anterior pituitary gland that plays a role in breast development and lactation during pregnancy.

Malignant neoplasm of the central nervous system · Very frequent (99-80%)

An abnormal growth of cells within the brain or spinal cord that exhibits aggressive behavior, rapid cellular proliferation, and the capacity to invade surrounding healthy neural tissue.

Pituitary carcinoma · Obligate (100%)

A pituitary tumor with subarachnoid, brain, or systemic metastasis. The diagnosis of a pituitary carcinoma requires evidence of metastatic disease, either outside the central nervous system (CNS) or as separate noncontiguous foci within the CNS.

Pituitary corticotropic cell adenoma · Very frequent (99-80%)

A type of pituitary adenoma that produces adrenocorticotropic hormone (ACTH).

Pituitary prolactin cell adenoma · Very frequent (99-80%)

A type of pituitary adenoma originating in prolactin secreting cells. This kind of adenoma is characterized by overproduction of prolactin, and may cause loss of menstrual periods and breast milk production in women.

Enlarged pituitary gland · Frequent (79-30%)

An abnormally increased size of the pituitary gland.

Hemianopia · Frequent (79-30%)

Partial or complete loss of vision in one half of the visual field of one or both eyes.

Hypopituitarism · Frequent (79-30%)

Progressive visual field defects · Frequent (79-30%)

Reduced visual acuity · Frequent (79-30%)

Abnormality of central motor function · Occasional (29-5%)

An anomaly of the control or production of movement in the central nervous system.

Ataxia · Occasional (29-5%)

Ataxia refers to impaired coordination of voluntary muscle movement. Cerebellar ataxia refers to ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits including asynergy (lack of coordination between muscles, limbs and joints), dysmetria (lack of ability to judge distances that can lead to under- or overshoot in grasping movements), and dysdiadochokinesia (inability to perform rapid movements requiring antagonizing muscle groups to be switched on and off repeatedly).

Other findings in the same source

From: Orphanet

Additional reported features include Elevated circulating growth hormone concentration (Occasional (29-5%)); Hearing impairment (Occasional (29-5%)); Hyperpituitarism (Occasional (29-5%)); Pituitary growth hormone cell adenoma (Occasional (29-5%)); Spinal cord lesion (Occasional (29-5%)); Diabetes insipidus (Very rare (<4-1%)); Pituitary gonadotropic cell adenoma (Very rare (<4-1%)); Pituitary thyrotropic cell adenoma (Very rare (<4-1%)). This is a selected summary, not a complete description of the condition.

When it may begin

From: Orphanet

All ages

Inheritance in the source

From: Orphanet

Unknown

Frequency and the population described

From: Orphanet

Annual incidence: <1 / 1 000 000; Europe; Value and class. Lifetime Prevalence: 1-9 / 1 000 000; Europe; Value and class. Annual incidence: <1 / 1 000 000; Norway; Value and class. Annual incidence: <1 / 1 000 000; Portugal; Value and class.

Which doctor should you see?

The suggested department for discussing Pituitary carcinoma is Oncology, with a oncologist and relevant organ specialist as the relevant type of clinician. General physician / Family Medicine; paediatrician for children. Referral depends on symptoms.

Additional services that may be relevant, depending on the findings, include: Relevant organ specialist / Surgical Oncology as indicated.

This is an editorial referral starting point. The appropriate clinic depends on the person’s age, symptoms, previous diagnosis and local services. The first clinician can decide whether another specialty or a team is needed; a department label does not confirm the diagnosis.

How to prepare for an assessment

Bring a short timeline of the main symptoms: when they first appeared, whether they are constant or episodic, what seems to change them, and how they affect daily activities. Include previous reports, discharge summaries, current medicines and supplements, allergies, and any relevant family history. A dated record is more useful than trying to match every feature in an online article.

Ask the clinician what is already established and what remains uncertain. If a test is suggested, ask what question it answers, what its limitations are and how the result would change the next step. The information here is not an instruction to arrange every possible test. In children, bring growth, developmental and school information if it is relevant to the concern.

• Has the exact tumour type been confirmed, and is staging relevant?
• What is the goal of each proposed treatment option?
• How will side effects, daily function and supportive care be addressed?

Treatment discussions and follow-up

The material gathered for this draft does not provide a complete condition-specific treatment pathway for Pituitary carcinoma. That gap does not mean that treatment is unavailable. A clinician needs to establish the diagnosis and review current guidance before recommending medicines, procedures, rehabilitation or other support.

Before leaving the appointment, clarify the next review date, who will communicate results, and whom to contact if the situation changes. Discuss difficulties with sleep, work, school, mobility, eating or emotional wellbeing when these are relevant. Practical support may require coordination between the treating clinician and other services.

The collected references do not establish a complete prevention or long-term outlook section for this entry. Missing information should not be interpreted as proof that prevention is impossible or that a particular outcome is inevitable. Ask what is known for the exact subtype, stage and personal circumstances, and which uncertainties remain.

When to seek emergency help

Severe breathing difficulty, collapse, new stroke-like symptoms, a seizure that is prolonged or repeated without recovery, uncontrolled major bleeding, or an immediate risk of self-harm require emergency help. In India, call 112 or reach the nearest emergency department. This is a general, non-exhaustive warning list; it is not a condition-specific triage tool.