Sexcord/ stromal ovarian tumors overview

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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

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

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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

Ovarian sex cord-stromal tumors are a diverse group of both benign and malignant neoplasms that develop from the dividing cell population which would normally give rise to cells that surrounds the oocytes, including the cells that produce ovarian hormones (the nongerm cell and nonepithelial components of the gonads).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. 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. 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.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.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. CT and MRI are very helpful in the diagnosis of these tumors. Surgery and chemotherapy are the mainstay of treatment for these tumors.

Historical Perspective

There is limited information about the historical perspective of sexcord/ stromal ovarian tumors

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

The cause of sexcord/ stromal ovarian tumors has not been identified.Mutations mainly involving FOXL2, DICER1, STK11 are involved.

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

Biopsy is the gold standard test for the diagnosis of sexcord/ stromal ovarian tumors. There is no single diagnostic study of choice for the diagnosis of sexcord/ stromal ovarian tumors, but these can be diagnosed based on CT and MRI findings

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

Patients with sexcord/ stromal ovarian tumors usually appear normal except few abdominal or pelvic and genitourinary findings on examination. Abdominal findings include Abdominal distension, increased abdominal girth, abdominal tenderness in the right/left lower abdominal quadrant, a palpable abdominal mass in the right/left lower abdominal quadrant, guarding, ascites, hemoperitoneum.

Laboratory Findings

Laboratory findings consistent with the diagnosis of sexcord/ stromal ovarian tumors include identifying the presence or absence of tumor markers like AMH: anti-Müllerian hormone; AFP: alpha-fetoprotein; E2: estradiol; hCG: human chorionic gonadotropin; LDH: lactate dehydrogenase; testost: testosterone; andro: androstenedione; DHEA: dehydroepiandrostenedione;

Electrocardiogram

There are no ECG findings associated with the diagnosis of sexcord-stromal ovarian tumors.

X-ray

There are no x-ray findings associated with sexcord/ stromal ovarian tumors.

Echocardiography and Ultrasound

There are no echocardiography findings associated with sexcord/ stromal ovarian tumors. Ultrasound may be helpful in the diagnosis of sexcord/ stromal ovarian tumors. Findings on an ultrasound suggestive of sexcord/ stromal ovarian tumors include adnexal hypoechoic masses with clear border and acoustic attenuation as well as minimal doppler flow signals.

CT scan

Pelvic ct scan may be helpful in the diagnosis of sexcord/ stromal ovarian tumors. Findings on CT scan suggestive of sexcord/ stromal ovarian tumors include multicystic masses with solid components and either irregularly thickened or thin septations for both adult and juvenile granulosa cell tumors, Fibromas on delayed contrast-enhanced computed tomography usually shows a solid, well-defined, homogeneous ovarian mass which is isodense to the uterus with very sparse contrast uptake. Sclerosing stromal tumor will show peripheral contrast uptake, reflecting prominent vasculature in the cellular areas, with centripetal progression on late images. Leydig tumors tend to be small and hypoattenuating.

MRI

MRI may be helpful in the diagnosis of sexcord/ stromal ovarian tumors. Findings on MRI suggestive of sexcord/ stromal ovarian tumors depends on the tumor subtypes. Granulosa cell tumors show heterogeneous signal intensity on both T1WI and T2WI and high signal intensity on DWI images. Fibroma, Fibrothecoma, and Thecoma appear as hypointense masses on T1-weighted MRI with very low signal intensity on T2-weighted imaging. Sclerosing stromal tumor of ovary show hyperintense cystic components or a heterogeneous solid mass of intermediate to high signal intensity on T2-weighted MRI.

Other Imaging Findings

PET-CT may be helpful in the diagnosis of sexcord/ stromal ovarian tumors. On fluorodeoxyglucose-positron emission tomography/computed tomography malignant ovarian tumors generally have intense uptake, whereas tumors with a small solid content show decreased uptake.

Other Diagnostic Studies

There are no other diagnosticstudies associated with sexcord/ stromal ovarian tumors

Treatment

Surgery

Surgery is the mainstay of treatment for sexcord/ stromal ovarian tumors. The feasibility of surgery depends on the stage of the tumor at diagnosis. Both benign and malignant ovarian sex cord-stromal tumors are managed surgically. Treatment in all postmenopausal and pre-menopausal women with bilateral involvement of ovaries includes total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH-BSO).Unilateral salpingo-oophorectomy (USO) with preservation of the contralateral ovary and the uterus is considered to be adequate surgical treatment for the majority of pre-menopausal patients with granulosa cell tumors.BEP(bleomycin, etoposide, cisplatin) is the most accepted chemotherapy regimen even for recurrent disease that is refractory to hormone therapy. Hormone treatment is usually added for advanced granulosa cell tumors(GrCTs), given their frequent association with oestrogen dependence and usually indolent course.

Primary Prevention

There are no established measures for the primary prevention of sexcord/ stromal ovarian tumors

Secondary Prevention

There are no established measures for the secondary prevention of sexcord/ stromal ovarian tumors

References


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