Neuroendocrine neoplasm of appendix

What it is

From: Orphanet

A rare sporadic neoplasm of the appendix and the second most common type of digestive endocrine tumor, often with no specific clinical presentation. They are divided into either classic endocrine tumor of the appendix or the more aggressive goblet cell carcinoma (GCC).

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.

Intestinal carcinoid · Obligate (100%)

Abdominal colic · Frequent (79-30%)

A type of abdominal pain that comes and goes in waves, most often starting and ending suddenly and being of severe intensity.

Abnormal bowel sounds · Frequent (79-30%)

An anomaly of the amount or nature of abdominal sounds. Abdominal sounds (bowel sounds) are made by the movement of the intestines as they promote passage of abdominal contents by peristalsis.

Bowel urgency · Frequent (79-30%)

A sudden, irresistible need to have a bowel movement.

Episodic abdominal pain · Frequent (79-30%)

An intermittent form of abdominal pain.

Hypoactive bowel sounds · Frequent (79-30%)

An decreased amount of bowel sounds.

Nausea and vomiting · Frequent (79-30%)

Nausea is a commonly encountered symptom that has been defined as an unpleasant painless subjective feeling that one will imminently vomit. Vomiting has been defined as the forceful expulsion of the contents of the stomach, duodenum, or jejunum through the oral cavity. While nausea and vomiting are often thought to exist on a temporal continuum, this is not always the case. There are situations when severe nausea may be present without emesis and less frequently, when emesis may be present without preceding nausea.

Poor appetite · Frequent (79-30%)

A reduced desire to eat.

Functional intestinal obstruction · Frequent (79-30%)

Mechanical ileus · Frequent (79-30%)

Anorexia · Occasional (29-5%)

Lack of desire to eat (loss of appetite).

Chronic noninfectious lymphadenopathy · Occasional (29-5%)

A chronic form of lymphadenopathy that is not related to infection.

Constipation · Occasional (29-5%)

Infrequent or difficult evacuation of feces.

Elevated circulating hepatic transaminase concentration · Occasional (29-5%)

Elevations of the levels of SGOT and SGPT in the serum. SGOT (serum glutamic oxaloacetic transaminase) and SGPT (serum glutamic pyruvic transaminase) are transaminases primarily found in the liver and heart and are released into the bloodstream as the result of liver or heart damage. SGOT and SGPT are used clinically mainly as markers of liver damage.

Other findings in the same source

From: Orphanet

Additional reported features include Hepatomegaly (Occasional (29-5%)); Ileal adenocarcinoma (Occasional (29-5%)); Adenocarcinoma of the colon (Occasional (29-5%)); Elevated serum acid phosphatase (Occasional (29-5%)); Protracted diarrhea (Occasional (29-5%)); Adrenocorticotropic hormone excess (Very rare (<4-1%)); Appendiceal mucinous neoplasm (Very rare (<4-1%)); Asthma (Very rare (<4-1%)); Heart murmur (Very rare (<4-1%)); Hypotension (Very rare (<4-1%)). This is a selected summary, not a complete description of the condition.

When it may begin

From: Orphanet

Adult

Which doctor should you see?

The suggested department for discussing Neuroendocrine neoplasm of appendix 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 Neuroendocrine neoplasm of appendix. 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.