primary ovarian carcinoid syndrome
Introduction
Introduction to primary ovarian carcinoid syndrome Primary ovarian carcinoid syndrome refers to the site of primary carcinoid tumor in the ovary. It is a special tumor originating from neuroendocrine cells, also known as argyrophiloma. Because of some endocrine substances, there are serotonin. (5-HT), histamine, dopamine, kinin, prostaglandin E, P, F substances, somatostatin, islet, endorphin, enkephalin, gastrin, and rare adrenal gland Corticosteroids (ACTH), melanocyte stimulating hormone (MSH), growth hormone, insulin, gastric peptides, etc., act on different organs of the body, so it can cause skin flushing, diarrhea, bronchospasm, telangiectasia, heart damage, etc. Group syndrome, called POCS. basic knowledge The proportion of illness: the incidence rate is 0.003% - 0.005% Susceptible people: women Mode of infection: non-infectious Complications: tricuspid atresia, heart failure
Cause
The cause of primary ovarian carcinoid syndrome
(1) Causes of the disease
May be formed by benign teratoma derived from neuroendocrine cells in the respiratory and gastrointestinal epithelial components, so 85% to 90% of ovarian carcinoids are components of teratomas.
(two) pathogenesis
The general view of ovarian carcinoid tumors has yellow solid nodules in the teratoma, with a diameter of 3 to 15 cm, an average of 9 cm. The tumor cells are uniform in size, and the cancerous cells from the midgut are arranged in an island or nest shape. The carcinoid cancer cells in the intestine and hindgut are arranged in trabecular or striate bands. Under the electron microscope, the neurosecretory granules of the island-like carcinoid cancer cells are irregularly shaped like dumbbells, while the neurosecretory granules of the trabecular banded carcinoid are round. Shape or oval.
Primary ovarian cancer of the ovary forms a solid mass or nodule in the wall of a teratoma or mucinous tumor. There are two kinds of goblet cells that secrete mucus and neuroendocrine cells that are positive for argyrophilic reaction. The stained CEA, chromogranin A and synaptophysin can distinguish the above two kinds of cells. Under the microscope, the tumor cells are arranged in a nest, a cord or acinar, and a mucus pool is often formed.
Goiter-type tumor cells are arranged in trabeculae, island or mixed type, carcinoid and thyroid tissue are mixed, and carcinoid cells are pro-silver and argyrophilic, immunohistochemical staining for thyroglobulin and neuroendocrine cell markers. Positive, under the electron microscope, round or oval neuroendocrine granules can be found, with a diameter of 100-400 nm.
The histology of ovarian carcinoid tumors can be divided into the following types:
1. Island shape (intestinal derivation).
2. Island-like carcinoids associated with mature cystic teratoma.
3. Trabecular (derivative of the foregut and hindgut).
4. Trabecular carcinoid associated with cystic teratoma.
5. Goiter (derived from endoderm with thyroid and c-cell differentiation).
6. Goiter associated with mature cystic teratoma.
7. Mucinous carcinoid (cardiomyocyte type or glandular carcinoma).
8. Mixed type (combination of 2 or more simple types).
Prevention
Primary ovarian carcinoid syndrome prevention
Patients with primary ovarian carcinoid should be diagnosed early, timely surgery, and follow-up.
Complication
Primary ovarian carcinoid syndrome complications Complications tricuspid atresia heart failure
Common tricuspid atresia or pulmonary stenosis, murmurs in the corresponding auscultation area, and finally lead to right heart failure, two-dimensional echocardiography can confirm the diagnosis.
Symptom
Symptoms of primary ovarian carcinoid syndrome Common symptoms Follicle stimulating hormone (F... urgency, heavy diarrhea, pelvic mass, right heart failure, goiter, bronchospasm, pulmonary stenosis
More than 90% of primary ovarian carcinoid syndrome is limited to one ovary, and generally does not metastasize. The average ovarian involvement is 8cm, and the solid carcinoid component accounts for 5% to 95% of the tumor volume.
About one-third of patients with island-like carcinoids develop carcinoid syndrome, and patients with trabecular banded carcinoid and goiter have rarely develop carcinoid syndrome.
Carcinoid syndrome is difficult to recognize in the early stage, and most of them are recognized after histological diagnosis, and sometimes diagnosed after the occurrence of metastasis in several years of diagnosis.
Facial flushing is a common early symptom of carcinoid syndrome. It is mainly based on medical history and determination of 5-hydroxyindoleacetic acid (5-HIAA) in urine. Clinically, the content of 5-HIAA in urine is determined in 24 hours. The normal value is 2-8 mg. /24h, more than 30mg/24h is positive, 5-HIAA elevation may be a marker of tumor secretion activity.
About 60% of patients with carcinoid syndrome have paroxysmal watery diarrhea, which is often urgency and often occurs in the morning, and diarrhea often occurs during facial flushing.
Cardiac involvement in carcinoid patients is common in the late stage of the disease, the incidence rate is 69% to 72%, mainly affecting the heart valve, common tricuspid atresia or pulmonary stenosis, hearing noise in the corresponding auscultation area, and finally leading to right heart failure, two-dimensional ultrasound A cardiac exam can confirm the diagnosis.
In recent years, it has been reported that patients with carcinoid tumors have severe pain and pressure of bowel movements. This may be due to the tumor producing gastrointestinal hormone peptide YY, which inhibits intestinal peristalsis.
Primary ovarian carcinoid and its syndrome are not easy to diagnose before surgery, and it is easy to cause misdiagnosis. Most of them must be recognized after histological diagnosis.
Examine
Examination of primary ovarian carcinoid syndrome
1. It was determined that the 5-HIAA content in the 24h urine was more than 30mg/24h.
2. Tumor marker examination.
3. Laparoscopy, histopathological examination.
Diagnosis
Diagnosis and diagnosis of primary ovarian carcinoid syndrome
Clinical findings of pelvic mass, suspected teratoma, and some endocrine substances and their skin flushing, diarrhea, bronchospasm, telangiectasia, heart damage syndrome, should be highly suspicious of this syndrome, laboratory determination 24h The 5-HIAA content in the urine exceeds 30mg/24h, and the syndrome should be considered. The pathological diagnosis of the mass after the mass is confirmed as carcinoid.
Intestinal manifestations should be differentiated from gastrointestinal infectious diseases; those with changes in heart valves should be differentiated from cardiovascular diseases such as rheumatic heart disease.
