Ovarian annular tubular sex cord tumor

Brief introduction of ovarian ring tubular tumor Sexcordtumorwithannulartubulesofovary was first named by Scully in 1970. So far, only 100 cases have been reported in domestic and foreign literatures. The clinical manifestations of this tumor are endocrine disorders, which are functional tumors that secrete both estrogen and progesterone, and are low-grade malignant tumors. The age of onset of the patient was 4 to 64 years old, mostly occurred in women of childbearing age between 20 and 30 years old, with an average age of 26.7 years. basic knowledge The proportion of illness: 0.001% Susceptible people: women Mode of infection: non-infectious Complications: abdominal pain

Ovarian ring tubular tumor tumor etiology

(1) Causes of the disease

Ovarian ring tubular tumors have a family genetic factor.

(two) pathogenesis

General examination

(1) clinical with black spot polyp syndrome: the tumor volume is mostly small, can be found under the microscope, generally diameter <3cm, unilateral or bilateral, of which about 2 / 3 on both sides, solid, cut gray Yellow, single or multiple nodular, can have cystic changes, calcification is obvious.

(2) clinical without black spot polyp syndrome: large tumor volume, maximum 20cm, average diameter > 5cm, unilateral, round, elliptical or nodular, most with envelope, cut surface gray or pink, most To be solid, there may be hemorrhage, necrosis, cystic changes, calcification is rare, and the presence of mucinous cystadenoma or germ cell tumor can be found in the ipsilateral or contralateral ovary of individual cases.

2. The most characteristic of the examination under the microscope is the annular tubule structure.

(1) Simple annular tubules: one row of tumor cells are arranged in a fence shape along the periphery of the small tube, and the other row of tumor cells are arranged in a fence shape along the periphery of the glass-like material in the small tube, and the tumor cells are cytoplasm between the two rows of tumor cells. Its most characteristic organizational form.

(2) Complex annular small tube: consists of a continuous ring-shaped small tube network, each of which has a glass-like substance.

There is a fibrous ovarian stroma between the annular tubules, in which luteinized cells or cells like Leydig cells are visible, calcified plaques, and glassy changes are common.

Ovarian ring tubular tumor prevention

Early diagnosis, timely treatment, good follow-up, for young unfertilized, normal exploration of the contralateral ovary during surgery, pelvic exploration after diagnosis of clinical stage I, feasible side attachment resection, but long-term follow-up after surgery.

Ovarian ring tubular tumor tumor complications Complications, abdominal pain

Torsion can have symptoms of acute abdominal pain, and some patients are associated with Peutz-Jeghers syndrome.

Ovarian ring tubular tumor symptoms common symptoms menopausal irregular bleeding hemorrhoids vaginal bleeding melasma precocious nodules pelvic mass polyp pigment spots

Clinical manifestation

(1) pelvic mass: Because the size of the tumor is very different, the small tumor can only be seen under the microscope. The larger one can reach 20cm in diameter, so the pelvic mass can only be touched in some patients, and the large mass is found. Most of them are solid, they can also have cystic, smooth surface and good activity. When the tumor has metastasis, most of them are located in the retroperitoneum and the perirenal, and the mass formed by the tumor is inactive and fixed.

(2) Endocrine changes: menstrual disorders in patients with ovarian ring tubular tumors are the most important symptoms in the clinic. Different degrees of irregular vaginal bleeding, amenorrhea, postmenopausal bleeding, teenage precocious puberty, etc., serum estrogen and pregnancy Ketone tests showed that both hormones increased significantly. In cases with endometrial pathology, not only partial estrogen stimulation, endometrial polyps, pathological changes of proliferative lesions, but also partial pregnancy Endometrial gland atrophy under the influence of hormones, pathological changes of interstitial decidual changes.

(3) Peutz-Jeghers syn-drome (PJS): About one-third of patients may have multiple pigmentation spots on the face, lips, oral mucosa, tongue, fingers, toe, etc. Gastrointestinal tract (more in the small intestine) with hamartomatous polyps, patients may cause bleeding due to intestinal polyps, intestinal obstruction, individual patient's polyps appear in the nasopharynx, bladder, tracheal mucosa, PJS is autosomal dominant hereditary disease.

It is worth noting that only a small number of patients with ring-shaped tubular tumors have PJS. Box et al. (1995) also reported that many tumors may also have hereditary intestinal polyp symptoms, such as some tumors from the breast, thyroid, osteoma, ampulla. Cancer and adenoma with Gardner syndrome, etc., so PJS is not a complication unique to ovarian ring tubular tumors.

(4) Cervical lesions: About 5.4% of patients with cervical malignant adenocarcinoma, patients with irregular vaginal bleeding, contact bleeding and other symptoms, the cervix can be papillary, nodular, but also has no obvious appearance Abnormalities, cervical cytology, no malignant cell detection, tumors are mucinous adenocarcinoma, glandular morphology is irregularly branched, cell atypia and nuclear division are rare, but its growth pattern has a high degree of malignant tendency, often appearing more widely Invasive growth, although the proportion of ring-shaped tubular tumors with cervical mucinous adenocarcinoma is not high, but careful examination of the patient's cervix and necessary biopsy is very important, because the poor prognosis of cervical cancer will be self-contained There is a great impact, srivatsa et al reported that patients with stage Ib survived for 4 to 5 years, and patients with stage IV survived only 3 months.

Examination of ovarian ring tubular tumors

First, hormone level detection

Both estrogen and progesterone in serum increased significantly.

Second, histopathological examination.

1. Cervical cytology examination:

For mucinous adenocarcinoma, the shape of the gland is irregularly branched.

Rarely, cell atypicality and nuclear division often lead to a wider range of invasive growth.

2, endometrial pathology:

Estrogen stimulation: Some have pathological changes of endometrial polyps and proliferative lesions.

Progesterone stimulation: Partial changes in endometrial gland atrophy and interstitial decidual changes.

Diagnosis and diagnosis of ovarian ring tubular tumor

diagnosis

1. Patients with PJS have typical mucoal pigmentary dark spots, multiple polyps in the digestive tract, solid mass in the appendage, menstrual disorders, and serological examination of estrogen and progesterone are elevated, the clinical diagnosis is not difficult.

2. Do not combine PJS Most patients are not accompanied by PJS, only according to the attachment of solid mass, menstrual disorders, at this time, the patient serum hormone determination may be helpful, because the tumor cells secrete a large number of female, progesterone is a tubular Interstitial tumors have prominent endocrine changes, while stromal cell tumors support the secretion of androgens, and granulosa cell tumors mainly secrete estrogen.

Differential diagnosis

Note that several tumors of the same ovarian cord stromal tumors, such as ovarian granulosa cell tumor, ovarian cytoplasmic cell tumor, ovarian support-stromal cell tumor, etc., which are difficult to identify, are identified.

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