Diagnostic Ultrasound - Abdomen and Pelvis

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Mucinous Ovarian Cystadenoma/Carcinoma TERMINOLOGY Abbreviations • Benign or malignant mucinous tumor Definitions • Mucinous epithelial neoplasm, which can be benign (mucinous cystadenoma), borderline (low malignant potential), or malignant (mucinous cystadenocarcinoma) IMAGING General Features • Best diagnostic clue ○ Multilocular cystic mass with low-level internal echoes • Location ○ Bilateral in 5% of benign and 20% of malignant tumors • Size ○ Variable in size, but often large, filling entire pelvis and extending into upper abdomen ○ Some of the largest tumors ever reported are mucinous cystadenomas – Massive size alone can suggest mucinous etiology Ultrasonographic Findings • Grayscale ultrasound ○ Typically multiloculated with thin septations ○ Papillary projections much less common than in serous tumors ○ Mucin creates low-level echoes within loculi – Typically have multiple loculi of varying echogenicity – Echogenicity variable depending on concentration of mucin ○ Solid components increase suspicion for malignancy ○ Pseudomyxoma peritonei: Potential form of peritoneal spread with amorphous, mucoid material insinuating itself around mesentery, bowel, and solid organs – More echogenic than simple ascites – Has mass effect with scalloping along solid organs (especially liver) and bowel matted posteriorly (rather than free floating) – May have subtle septations • Color Doppler ○ Vascularity seen within solid components CT Findings • Variable attenuation of loculi, depending on concentration of mucin • Peritoneal metastases often low attenuation ○ May be difficult to differentiate from fluid-filled bowel • Enhancement of solid portions with contrast MR Findings • Signal intensity varies depending on concentration of mucin • Loculi with high concentration of mucin will be higher signal on T1WI and lower signal on T2WI ○ Creates a stained glass appearance DIFFERENTIAL DIAGNOSIS Endometriomas • Also contain low-level echoes • MRI helpful: Blood high signal on T1WI with T2 shading Serous Cystadenoma/Carcinoma • More often unilocular • Cyst contents not as echogenic • Papillary projections common Germ Cell Tumors • Can have low-level echoes similar to mucin • Typically more complicated with calcifications, fluid-fluid levels, etc. Hemorrhagic Cyst • Smaller and unilocular • Resolves on follow-up scan Mucocele • Dilated appendix filled with mucinous material PATHOLOGY General Features • Etiology ○ Not completely understood ○ 1 theory is "incessant ovulation": Repeated microtrauma with cellular repair to surface epithelium – Increased risk: Nulliparity, early menarche, late menopause (more ovulatory cycles) – Reduced risk: Multiparity, late menarche, early menopause, oral contraceptive use (fewer ovulatory cycles) • Genetics ○ Hereditary causes in 5-10% of ovarian cancers (mutations in BRCA1 and BRCA2 tumor suppressor genes) • Associated abnormalities ○ May occasionally be hormonally active, producing estrogen • Ovarian neoplasms ○ Epithelial tumors: 60-70% of all tumors; 85-90% of malignancies ○ Germ cell tumors: 15-20% of all tumors; 3-5% of malignancies ○ Sex cord-stromal tumors: 5-10% of all tumors; 2-3% of malignancies ○ Metastases and lymphoma: 5-10% of all tumors; 5-10% of malignancies • Benign epithelial tumors ○ Serous cystadenoma: 20-25% ○ Mucinous cystadenoma: 20-25% • Malignant epithelial tumors ○ Serous cystadenocarcinoma: 40-50% ○ Endometrioid carcinoma: 20-25% ○ Mucinous cystadenocarcinoma: 5-10% ○ Clear cell carcinoma: 5-10% ○ Brenner tumor: 1-2% ○ Undifferentiated carcinoma: 4-5% • Method of spread ○ Intraperitoneal dissemination most common – Greater omentum, right subphrenic region, and pouch of Douglas most common sites found at surgery ○ Direct extension to surrounding organs ○ Lymphatic spread to paraaortic and pelvic nodes ○ Hematogenous spread least common Diagnoses: Female Pelvis 817

Mucinous Ovarian Cystadenoma/Carcinoma Diagnoses: Female Pelvis – Liver and lung most common sites Staging, Grading, & Classification • FIGO staging system of ovarian carcinoma ○ Stage I: Tumor limited to ovaries – IA: Unilateral, no malignant ascites – IB: Bilateral, no malignant ascites – IC: Tumor limited to 1 or both ovaries with any of the following □ IC1: Surgical spill intraoperatively □ IC2: Capsule rupture before surgery, or tumor on ovarian/fallopian tube surface □ IC3: Malignant cells present in ascites or peritoneal washings ○ Stage II: Tumor involves 1 or both ovaries with pelvic extension – IIA: Extension to uterus or fallopian tubes, no malignant ascites – IIB: Extension to other pelvic tissues, no malignant ascites ○ Stage III: Peritoneal implants outside pelvis &/or retroperitoneal nodal metastases – IIIA1: Positive retroperitoneal lymph nodes only – IIIA2: Microscopic metastasis outside pelvis &/or positive retroperitoneal lymph nodes – IIIB: Macroscopic extrapelvic implants ≤ 2 cm ± positive nodes – IIIC: Macroscopic extrapelvic implants > 2 cm or ± positive nodes ○ Stage IV: Distant metastases (excluding peritoneal implants) – IVA: Pleural effusion with positive cytology – IVB: Hepatic &/or splenic parenchymal metastasis, or metastasis to extra-abdominal organs (including inguinal nodes and nodes outside of abdomen) Microscopic Features • Ovarian origin of pseudomyxoma peritonei called into question ○ Most cases now thought to be appendiceal with metastases to ovary ○ Appendix should be thoroughly examined with special tissue staining in every case CLINICAL ISSUES Presentation • Most common signs/symptoms ○ Incidental mass discovered on exam ○ Pelvic discomfort/pain from large tumors – Massive tumors can actually cause weight gain and distended abdomen ○ Symptoms from metastatic disease • CA-125 not useful for mucinous tumors: False-negative in 30% Demographics • Age ○ Mucinous cystadenoma 3rd-5th decade ○ Mucinous cystadenocarcinoma in peri- and postmenopausal age group • Epidemiology ○ Mucinous tumors 2nd most common epithelial neoplasm (serous most common) ○ Malignant: 10%; borderline: (Low malignant potential) 10%; benign 80% ○ Bilateral in 5% of benign tumors, 10% of borderline tumors and 20% of malignant tumors Natural History & Prognosis • 95% 5-year survival for low malignant potential tumors ○ If metastatic, prognosis is similar to those with frankly malignant histology • 5-year survival for malignant epithelial tumors ○ Stage I: 90% ○ Stage II: 70% ○ Stage III: 39% ○ Stage IV: 17% Treatment • Primary treatment is surgery ○ Complete staging laparotomy and tumor debulking (cytoreduction) – Staging laparotomy includes hysterectomy with bilateral salpingo-oophorectomy, pelvic and paraaortic node biopsies, omentectomy, peritoneal biopsies and washings – More conservative surgery may be done for women with stage I disease in reproductive age group – Care taken to avoid intraoperative rupture □ May increase potential for recurrence ○ Gelatinous, insinuating nature of pseudomyxoma peritonei makes complete resection difficult – Recurrence common and multiple laparotomies required ○ Chemotherapy after cytoreductive surgery ○ Neoadjuvant chemotherapy before cytoreductive surgery in patients with unresectable disease – Includes bulky disease in difficult to reach areas (porta hepatis, lesser sac, root of mesentery), extensive surrounding organ or sidewall invasion, or stage IV disease DIAGNOSTIC CHECKLIST Image Interpretation Pearls • Mucinous tumors are less commonly malignant than serous tumors SELECTED REFERENCES 1. Sayasneh A et al: The characteristic ultrasound features of specific types of ovarian pathology (Review). Int J Oncol. 46(2):445-58, 2015 2. Ledermann JA et al: Gynecologic Cancer InterGroup (GCIG) consensus review for mucinous ovarian carcinoma. Int J Gynecol Cancer. 24(9 Suppl 3):S14-9, 2014 3. Alcázar JL et al: Clinical and ultrasound features of type I and type II epithelial ovarian cancer. Int J Gynecol Cancer. 23(4):680-4, 2013 4. Lalwani N et al: Histologic, molecular, and cytogenetic features of ovarian cancers: implications for diagnosis and treatment. Radiographics. 31(3):625- 46, 2011 818

Mucinous Ovarian Cystadenoma/Carcinoma<br />

TERMINOLOGY<br />

Abbreviations<br />

• Benign or malignant mucinous tumor<br />

Definitions<br />

• Mucinous epithelial neoplasm, which can be benign<br />

(mucinous cystadenoma), borderline (low malignant<br />

potential), or malignant (mucinous cystadenocarcinoma)<br />

IMAGING<br />

General Features<br />

• Best diagnostic clue<br />

○ Multilocular cystic mass with low-level internal echoes<br />

• Location<br />

○ Bilateral in 5% of benign <strong>and</strong> 20% of malignant tumors<br />

• Size<br />

○ Variable in size, but often large, filling entire pelvis <strong>and</strong><br />

extending into upper abdomen<br />

○ Some of the largest tumors ever reported are mucinous<br />

cystadenomas<br />

– Massive size alone can suggest mucinous etiology<br />

Ultrasonographic Findings<br />

• Grayscale ultrasound<br />

○ Typically multiloculated with thin septations<br />

○ Papillary projections much less common than in serous<br />

tumors<br />

○ Mucin creates low-level echoes within loculi<br />

– Typically have multiple loculi of varying echogenicity<br />

– Echogenicity variable depending on concentration of<br />

mucin<br />

○ Solid components increase suspicion for malignancy<br />

○ Pseudomyxoma peritonei: Potential form of peritoneal<br />

spread with amorphous, mucoid material insinuating<br />

itself around mesentery, bowel, <strong>and</strong> solid organs<br />

– More echogenic than simple ascites<br />

– Has mass effect with scalloping along solid organs<br />

(especially liver) <strong>and</strong> bowel matted posteriorly (rather<br />

than free floating)<br />

– May have subtle septations<br />

• Color Doppler<br />

○ Vascularity seen within solid components<br />

CT Findings<br />

• Variable attenuation of loculi, depending on concentration<br />

of mucin<br />

• Peritoneal metastases often low attenuation<br />

○ May be difficult to differentiate from fluid-filled bowel<br />

• Enhancement of solid portions with contrast<br />

MR Findings<br />

• Signal intensity varies depending on concentration of mucin<br />

• Loculi with high concentration of mucin will be higher signal<br />

on T1WI <strong>and</strong> lower signal on T2WI<br />

○ Creates a stained glass appearance<br />

DIFFERENTIAL DIAGNOSIS<br />

Endometriomas<br />

• Also contain low-level echoes<br />

• MRI helpful: Blood high signal on T1WI with T2 shading<br />

Serous Cystadenoma/Carcinoma<br />

• More often unilocular<br />

• Cyst contents not as echogenic<br />

• Papillary projections common<br />

Germ Cell Tumors<br />

• Can have low-level echoes similar to mucin<br />

• Typically more complicated with calcifications, fluid-fluid<br />

levels, etc.<br />

Hemorrhagic Cyst<br />

• Smaller <strong>and</strong> unilocular<br />

• Resolves on follow-up scan<br />

Mucocele<br />

• Dilated appendix filled with mucinous material<br />

PATHOLOGY<br />

General Features<br />

• Etiology<br />

○ Not completely understood<br />

○ 1 theory is "incessant ovulation": Repeated microtrauma<br />

with cellular repair to surface epithelium<br />

– Increased risk: Nulliparity, early menarche, late<br />

menopause (more ovulatory cycles)<br />

– Reduced risk: Multiparity, late menarche, early<br />

menopause, oral contraceptive use (fewer ovulatory<br />

cycles)<br />

• Genetics<br />

○ Hereditary causes in 5-10% of ovarian cancers (mutations<br />

in BRCA1 <strong>and</strong> BRCA2 tumor suppressor genes)<br />

• Associated abnormalities<br />

○ May occasionally be hormonally active, producing<br />

estrogen<br />

• Ovarian neoplasms<br />

○ Epithelial tumors: 60-70% of all tumors; 85-90% of<br />

malignancies<br />

○ Germ cell tumors: 15-20% of all tumors; 3-5% of<br />

malignancies<br />

○ Sex cord-stromal tumors: 5-10% of all tumors; 2-3% of<br />

malignancies<br />

○ Metastases <strong>and</strong> lymphoma: 5-10% of all tumors; 5-10%<br />

of malignancies<br />

• Benign epithelial tumors<br />

○ Serous cystadenoma: 20-25%<br />

○ Mucinous cystadenoma: 20-25%<br />

• Malignant epithelial tumors<br />

○ Serous cystadenocarcinoma: 40-50%<br />

○ Endometrioid carcinoma: 20-25%<br />

○ Mucinous cystadenocarcinoma: 5-10%<br />

○ Clear cell carcinoma: 5-10%<br />

○ Brenner tumor: 1-2%<br />

○ Undifferentiated carcinoma: 4-5%<br />

• Method of spread<br />

○ Intraperitoneal dissemination most common<br />

– Greater omentum, right subphrenic region, <strong>and</strong> pouch<br />

of Douglas most common sites found at surgery<br />

○ Direct extension to surrounding organs<br />

○ Lymphatic spread to paraaortic <strong>and</strong> pelvic nodes<br />

○ Hematogenous spread least common<br />

Diagnoses: Female <strong>Pelvis</strong><br />

817

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