Sexcord/ stromal ovarian tumors overview

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Sexcord/ stromal ovarian tumors Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Sexcord/ Stromal Ovarian Tumors from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: ; Maneesha Nandimandalam, M.B.B.S.[2]

Overview

Historical Perspective

Classification

Sexcord/ stromal ovarian tumors may be classified according to WHO into 3 subtypes: Pure stromal tumors, pure sexcord tumors, mixed stromal and sexcord tumors. Histological classification of sexcord-stromal ovarian tumors includes granulosa stromal cell tumors, sertoli leydig cell tumors, gynandroblastoma, and unclassified.

Pathophysiology

The exact pathogenesis of sexcord/ stromal ovarian tumors is not fully understood. Mutations mainly involving FOXL2, DICER1, STK11 are involved. They are associated with ollier disease and maffucci syndrome.The microscopic pathology varies with the individual subtype of sexcord stromal ovarian tumors.

Causes

Differentiating sexcord/ stromal ovarian tumors from Other Diseases

On the basis of age of onset, vaginal discharge, and constitutional symptoms, ovarian cancer must be differentiated from tubo-ovarian abscess, ectopic pregnancy, hydrosalpinx, salpingitis, fallopian tube carcinoma, uterine leiomyoma, choriocarcinoma, leiomyosarcoma, pregnancy, appendiceal abscess, appendiceal neoplasm, diverticular abscess, colorectal cancer, pelvic kidney, advanced bladder cancer, and retroperitoneal sarcoma.

Epidemiology and Demographics

The yearly adjusted incidence rate is approximately 2 per 1,000,000 women for sexcord-stromal ovarian tumors(SCSTs). The mortality rate has gradually been declining from1976 (10.0 per 100,000) and 2015 (6.7 per 100,000) by 33%. The age at presentation varies depending on the subtypes of sexcord-stromal ovarian tumors. Sexcord-stromal ovarian tumors(SCSTs) have more predilection in women of Caucasian background. Rates are highest among Whites, intermediate for Hispanics, and lowest among Blacks, and Asian people. Intrestingly there has been increases in incidence and mortality rates in less developed countries with recent economic growth and lifestyle changes.

Risk Factors

Common risk factors in the development of sexcord/ stromal ovarian tumors include preterm birth, high gonadotrophin levels, increasing age at first pregnancy, obese and non-white women

Screening

There is insufficient evidence to recommend routine screening for sexcord/ stromal ovarian tumors. According to the US Preventive Services Task Force(USPSTF) , screening for sexcord/ stromal ovarian tumors is not recommended in asymptomatic women

Natural History, Complications, and Prognosis

The symtoms of sexcord/ stromal ovarian tumors include Adnexal mass, Abdominal & pelvic symptoms, Bloating, Urinary urgency or frequency, [[Dysphagia](difficulty)eating) or feeling full quickly, Pelvic or abdominal pain. Common complications of sexcord/ stromal ovarian tumors include malignant pleural effusion,bowel obstruction,ascites. The prognosis varies with the subtypes of tumor. Most sexcord-stromal ovarian tumors present at a low tumor stage and also prognosis in these patients is excellent.

Diagnosis

Diagnostic Study of Choice

History and Symptoms

The most common symptoms of sexcord/ stromal ovarian tumors include adnexal mass, bloating, urinary urgency or frequency, dysphagia(difficulty eating) or feeling full quickly, pelvic or abdominal pain. Less common symptoms of sexcord/ stromal ovarian tumors include lymphadenopathy, postmenopausal bleeding, typical features of bowel obstruction like nausea, vomiting, and distention. Specific symptoms pertinent to sexcord/ stromal ovarian tumors include hirsutism(excessive hairgrowth), virilization, menstrual changes like abnormal uterine bleeding, precocious puberty in children.

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Interventions

Surgery

Primary Prevention

Secondary Prevention

References

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