Mature Cystic Teratoma of the Ovary

Cancer / By

Mature Cystic Teratoma of the Ovary

Description

  •  most common benign ovarian neoplasm arising from germ cell layer of ovary(2,3,4)

Also Called

  •  dermoid cyst
  •  dermoid cyst of ovary
  •  dermoid ovarian cyst
  •  benign cystic teratoma
  •  mature teratoma
  •  mature cystic ovarian teratoma
  •  ovarian mature cystic teratoma

Types

  • types characterized histologically by predominant tissue type, with mature tissue derived from 3 germ cell layers(2,3)
    •  ectoderm (skin or neural)
    •  mesoderm (muscle, bone, cartilage, or fat)
    •  endoderm (mucinous or ciliated epithelium)

Epidemiology

Who Is Most Affected

  • younger women, particularly those 30-40 years old (reported mean age 30 years old), but may occur at any age(2,3,4)

Incidence/Prevalence

  • teratomas are the most common germ cell tumor of the ovary, and are reported to account for about 10-20% of all ovarian tumors and 60% of benign ovarian tumors(1,2,3,4)
  • most common benign ovarian neoplasm in women age < 20 years(1,4)
  • reported to account for about 50% of pediatric ovarian tumors(1)
  • reported incidence about 1.2-14.2 cases per 100,000 persons annually(4)
  • typically occur unilaterally, with 10%-17% of cases reported to occur bilaterally and 72.2% of unilateral cases reported to occur on the right side(1,2,4)
  • mature cystic teratoma of the ovary in 30% of 140 women age < 21 years with noninflammatory ovarian masses in case series (Obstet Gynecol 2000 Aug;96(2):229)

Risk Factors

  • having first-degree relative with mature cystic ovarian teratoma may be associated with increased risk for mature cystic teratoma
    •  based on case-control study
    •  285 women with mature cystic ovarian teratoma and 378 controls (primarily postpartum women) without mature cystic ovarian teratoma and with normal ovaries on ultrasound exam were interviewed to determine presence of ovarian cyst in first-degree relatives
    •  mature cystic ovarian teratoma found in ≥ 1 first-degree relative in 9.8% of families of women with mature cystic ovarian teratoma vs. 2% of families of controls (adjusted odds ratio 5.6, 95% CI 2.24-14.2)
    •  Reference – Gynecol Obstet Invest 2003;56(4):203

Etiology and Pathogenesis

Causes

  •  abnormal growth originating from germ cell layer of ovary(2,3)

Pathogenesis

  • mature ovarian teratomas typically have the following characteristics
  •  malignant transformation of mature cystic teratoma of ovary may occur after development of the teratoma(2,4)

History and Physical

History

Chief Concern (CC)

  •  usually asymptomatic and often incidental finding during routine pelvic exam or during imaging for another indication(1,2,4)
  • most common symptoms, if present, may include(4)
    • abdominal pain
    • abdominal distention
    • constipation
    • increased urinary frequency
    • palpable abdominal or pelvic mass
  • other symptoms might include(1)
    •  abnormal vaginal bleeding, including menstrual disturbances
    •  hirsutism
  • about 30% of patients having treatment for ovarian teratoma are symptomatic, and abdominal pain most common symptom, particularly in younger patients
    •  based on retrospective cohort study
    • 517 patients aged 10-90 years with mature cystic teratoma had 573 tumors removed
      •  29.4% were symptomatic
      • among symptomatic patients
        •  abdominal or pelvic pain in 77.6%
        •  dysmenorrhea in 6.6%
        •  pressure in 5.9%
        •  back pain in 4.6%
        •  dyspareunia in 1.3%
        •  mass identified by patient in 1.3%
    • Reference – Obstet Gynecol 1994 Jul;84(1):22

History of Present Illness (HPI)

  •  patients may remain asymptomatic(2,4)
  • consider ovarian torsion (most common complication) in women presenting with
    • pelvic or abdominal pain, which typically has acute onset but may also be characterized as
      • constant or intermittent (due to twisting and untwisting of ovary over time)
      • having onset with sudden change in position or activity
      • lasting for several days or months
      • having variable intensity
      • radiating to flank or groin (reported in about 50% of patients)
    • nonspecific symptoms such as nausea and/or vomiting or abdominal bloating
  •  spontaneous rupture is rare complication (reported < 1% of cases)(1)
  • ask about vaginal bleeding, urinary symptoms (frequency or retention) constipation, diarrhea, weight loss, cachexia, and fever, which are reported to occur in 1%-6% of patients with squamous cell carcinoma of mature cystic teratoma of the ovary(4)

Physical

Abdomen

  •  palpate for abdominal tenderness or mass(2,4)

Pelvic

  • perform bimanual exam to evaluate for palpable adnexal mass
    •  palpable mass identified in 13% of girls < 20 years old and 3% of women > 20 years old in case series of 545 women and girls having surgery for mature ovarian teratoma (Am J Obstet Gynecol 2011 Jul;205(1):32.e1)
    •  may be bilateral, reported in 10%-17% of cases(1,4)

Diagnosis

Making the Diagnosis

  •  often incidental finding at routine pelvic examination(2)
  • most mature cystic teratomas of the ovary can be diagnosed by ultrasound but appearance of the teratoma may vary depending on presence of bones, teeth, hair, and echogenic fat material(1,2,3)
  • other imaging modalities including computed tomography and magnetic resonance imaging may be considered when ultrasound is nondiagnostic as both demonstrate intratumoral fat, which is specific to ovarian cystic teratoma(2)
  • histopathology can confirm diagnosis and exclude malignant transformation or component of immature teratoma within mature cystic teratoma(2)

Differential Diagnosis

  • immature teratoma
  • acute hemorrhage in a pelvic mass such as an ovarian cyst or an endometrioma may be echogenic in the acute stage and may resemble a dermoid plug
    • ultrasound appearance of hemorrhage within a pelvic mass depends on the interval between the bleed and imaging
    • posterior sound enhancement seen with acute hemorrhage can help distinguish hemorrhage from dermoid plug
    • Reference – AJR Am J Roentgenol 1996 Nov;167(5):1127PDF
  • pedunculated uterine leiomyoma, particularly if leiomyoma projects laterally or posteriorly into the uterus, or other varieties such as calcified leiomyoma, lipoleiomyoma, or myolipoma (AJR Am J Roentgenol 1996 Nov;167(5):1127PDF)
  • ovarian pathologies with echogenic mural module that is typical of cystic teratomas, including
  • computed tomography or magnetic resonance imaging may be more accurate in distinguishing dermoid from some ovarian pathologies, such as
    • endometriosis
    •  mature cystic teratoma without fatty component
    •  unusual variants of myoma (e.g., lipoleiomyoma)
    • Reference – Eur J Radiol 2009 Dec;72(3):454

Testing Overview

  • several imaging modalities can be considered for diagnosis of mature cystic teratoma, which has a wide spectrum of appearances in different imaging modalities; each imaging feature reflects different combinations of histological components within the teratoma
  • serum cancer antigen 19-9 (CA19-9) and cancer antigen 125 (CA-125) may be useful in discriminating between benign lesions of the ovary and malignant neoplasm (level 2 [mid-level] evidence)
  • histopathology can confirm diagnosis and exclude malignant transformation or component of immature teratoma within mature cystic teratoma, and typically shows mature tissues of ectoderm (skin or brain), mesoderm (muscle or fat), and endoderm (mucinous or ciliated epithelium)

Blood Tests

  • cancer antigen (CA) 125 (CA-125) and CA19-9 are tumor markers commonly used in evaluation of adnexal masses
    • CA19-9
      •  normal level < 35 units/mL
      •  CA19-9 reported to be elevated in 37.4% of 139 patients with histologically proven mature cystic teratoma
      •  Reference – Reprod Sci 2014 Oct;21(10):1307
    • CA-125
      •  any elevation (CA-125 > 35 units/mL) in postmenopausal patient with adnexal mass suggests malignancy
      •  consider referral to gynecologic oncologist for CA-125 > 200 units/mL in premenopausal woman
      •  elevated CA-125 in 80% of patients with advanced ovarian cancer, but only 50% with stage I disease
      •  Reference – Obstet Gynecol 2007 Jul;110(1):201
  •  higher CA19-9 and CA-125 levels may be useful in discriminating malignancy from benign mature cystic teratoma of the ovary (level 2 [mid-level] evidence)
    •  based on retrospective cohort study
    •  322 women with pathologically-confirmed mature cystic teratoma of ovary (239 women) or ovarian cancer (83 women) were included
    • mean CA19-9 level 508.58 units/mL in women with ovarian cancer vs. 114.66 units/mL in women with mature cystic teratoma (p = 0.013)
    •  simultaneous elevation of CA-125 and CA19-9 associated with increased risk of malignant neoplasm compared to single elevation of CA19-9 (odds ratio 23.7, 95% CI 8.86-63.58)
    • Reference – Med Sci Monit 2013 Mar 29;19:230full-text

Imaging Studies

Ultrasound

  • Dermoid cyst on pelvic ultrasound – Dermoid cyst (mature cystic teratomas).Copyright© 2014, EBSCO Information Services.
  •  ultrasound is most commonly used imaging modality for assessment of pelvic organs and is primarily used to confirm the presence of a mass and to determine organ of origin(2,3)
  •  most mature cystic teratomas of the ovary can be diagnosed by ultrasound; appearance of the teratoma may vary depending on presence of bones, teeth, hair, and echogenic fat material(2,3)
  • most common findings for mature cystic teratoma on ultrasound(2,3)
    • shadowing echodensity (Rokitansky nodule or dermoid plug)
      • appears as densely echogenic protuberance projecting into cystic lumen
      • may show acoustic shadowing due to teeth, hair, and fat content
    • intratumoral fat, which appears as diffuse or regional high-amplitude echoes
    • tuft of hair, which appears as diffuse or regional high amplitude echoes
    • dot-dash sign, appears as hyperechoic lines and dots from hairs in different orientations in the imaging plane
    • tooth/calcification, which appears as regional high amplitude echoes with shadowing
  • other possible ultrasound findings(2,3)
    • comet tail appearance, or hypoechoic hair balls with posterior acoustic shadowing
    • anechoic sebum above hyperechoic layer of aqueous fluid and debris or supernatant hyperechoic sebum layer above hypoechoic aqueous fluid (fat-fluid/fluid-fluid level)
    • tip of the iceberg sign, which appears as echogenic focus with acoustic shadowing due to fatty fluid, hair, and cellular debris
    • rarely, floating balls sign, which appears as intracystic floating hyperechoic globules that move as the patient moves
  • ultrasound findings suggestive of malignancy
    •  isoechoic branching structures
    •  solid areas with flow at Doppler ultrasound of invasion into adjoining organs
    •  central flow on Doppler ultrasound
    •  Reference – Radiology 2010 Sep;256(3):943full-text
  • transvaginal ultrasound may not always identify mature cystic teratomas, but may help distinguish mature cystic teratomas from other ovarian cysts (level 2 [mid-level] evidence)
    •  based on diagnostic cohort study with blinding of reference standard not stated
    •  376 premenopausal women had transvaginal ultrasound (TVUS) 1 week prior to surgical intervention for adnexal mass, chronic pelvic pain, uterine fibroids, or infertility
    •  ultrasound findings compared with surgical and pathological findings (reference standard)
    •  prevalence of cystic teratomas by histopathology 4.8%
    •  81 of 740 ovaries (both ovaries in 35 women) were not visualized by TVUS
    •  among 659 ovaries visualized with TVUS for screening evaluation, 13 cystic teratomas diagnosed by transvaginal ultrasound and 11 confirmed by pathology
    • for detecting cystic teratoma during screening evaluation, transvaginal ultrasound had
      •  sensitivity 57.9%
      •  specificity 99.7%
    • among 123 ovarian cysts identified by TVUS, prevalence of mature cystic teratoma
      •  14.7% if functional cysts included
      •  26.4% if functional cysts excluded
    • for differentiating cystic teratoma from other ovarian masses, transvaginal ultrasound had
      •  sensitivity 84.6%
      •  specificity 98.2%
      •  positive predictive value 84.6%
      •  negative predictive value 98.2%
    • Reference – Obstet Gynecol 1995 Jan;85(1):48
  • ultrasound appears to distinguish mature cystic teratomas from other types of adnexal mass (level 2 [mid-level] evidence)
    •  based on diagnostic cohort study with blinding of reference standard and test under investigation not stated
    •  943 women with 1,035 adnexal masses scheduled for surgical removal had ultrasound exam 1 week prior to surgery
    •  in most cases, ultrasound performed transvaginally
    • mature cystic teratoma in 15.6% by histopathology
    •  mean diameter of teratoma 8 cm (range 1.5-18 cm)
    • for diagnosing mature cystic teratoma, ultrasound had
      •  sensitivity 94%
      •  specificity 99%
      •  positive predictive value 94%
      •  negative predictive value 98%
    • Reference – Zentralbl Gynakol 1996;118(3):136

Computed Tomography (CT)

  • CT is reported to have excellent sensitivity for detection of mature cystic teratoma of the ovary due to its ability to detect fat and subtle calcifications(2)
  • findings on CT may include(2)
    • intratumoral fat, which may appear as
      • component with density of -144 to -20 HU in Rokitansky nodule or cyst wall
      • layering component
      • floating mass mixed with hair
    • Rokitansky nodule (also called dermoid plug)
      • may appear as
        • round structure protruding into cystic lumen
        • mural thickening
        • bridge across cyst
        • a tooth
    • tooth/calcification
    • supernatant fatty layer, may have lower attenuation and dependent fluid layer with higher attenuation, or fat-fluid/fluid-fluid level
    • floating balls sign
      • relatively rare
      • floating globules in cyst fluid or interface of fat-fluid levels
  •  nodular formation and enhancement in soft tissue components and obtuse angle between soft tissue and inner wall of cyst on CT may be more often associated with malignant teratomas than benign teratomas
    •  based on retrospective cohort study
    • 1,325 women with teratomas were included
      •  12 women had malignant transformation of mature cystic ovarian teratoma
      •  1,313 women had benign mature cystic ovarian teratoma
    •  CT findings from 8 malignant and 15 benign teratomas were evaluated
    • comparing benign vs. malignant teratomas
      • soft tissue components in 33% vs. 75%
        •  nodular formation in soft tissue components in 0% vs. 100% (p < 0.05)
        •  enhancement in soft tissue components in 0% vs. 83% (p < 0.05)
        •  obtuse angle between soft tissue and inner wall of cyst in 20% vs. 83% (p < 0.05)
        •  transmural growth in 5% vs. 33% (not significant)
        •  lymphadenopathy in 0% vs. 13% (not significant)
        •  disseminated disease in 5% vs. 38% (not significant)
    •  Reference – Clin Imaging 2011 Jul-Aug;35(4):294

Magnetic Resonance Imaging (MRI)

  • findings on MRI may include(2)
    • intratumoral fat, which may appear as
      • component with high signal intensity on T1-weighted images
      • signal drop on fat-saturated T1-weighted images
    • chemical shift artefact, which may appear as
      • foci or areas of very high signal intensity on T2-weighted images
      • boundary artefact with high and low signal intensity bands on opposite sides of tumor
    • Rokitansky nodule (also called dermoid plug), which appears as a rounded structure protruding into cystic lumen, mural thickening, bridge across cyst, or cystic structure
    • intratumoral keratinoid material, which appears as a component with low signal intensity on T1-weighted and high signal intensity on T2-weighted images and diffusion restriction
    • tufts of hair, which appears as a component with chemical shift artefact in gravity-dependent part of cyst
    • palm tree-like protrusion, which appears as a polypoid mass protruding into cyst cavity with internal pattern resembling a palm tree
    • fat-fluid/fluid-fluid level, which appears as high signal intensity of supernatant fatty layer on T1-weighted images and low signal intensity on fat-suppressed T1-weighted images
    • floating balls sign, which appears as floating globules in gravity-independent position in cyst fluid or interface of fat-fluid level (relatively rare)

Biopsy and Pathology

  •  histopathology can confirm diagnosis and exclude malignant transformation or component of immature teratoma within mature cystic teratomas(2,3,4)
  • mature teratomas typically contain mature tissues of all germ layers including(2,3,4)
    • ectoderm (skin or brain), most predominant
    • mesoderm (muscle or fat)
    • endoderm (mucinous or ciliated epithelium)
  •  monodermal teratomas consist exclusively of endodermal or ectodermal tissue and include struma ovarii, carcinoid tumors, neuroectodermal tumors, sebaceous tumors(2,3)
  • mature cystic teratoma usually appears as an unilocular cystic cavity upon macroscopic examination but may also include septa dividing the cyst into several compartments(2)
  • tumor compartment is filled with sebaceous material that is liquid at body temperature and semisolid at room temperature(2)
  • a raised protuberance (Rokitansky protuberance) projects into the cyst cavity(2)
    • bone and teeth, when present, tend to locate in this protuberance
    • hair, when present, typically arises from this nodule and may also be floating with keratin and sebum in the lumen
  •  mature cystic teratoma of ovary with well-differentiated cerebellum tissue in 14-year-old female in case report (Pediatr Dev Pathol 2005 Mar-Apr;8(2):247)

Management

Management Overview

  • surgical management
    • surgical intervention for suspected mature ovarian teratomas is warranted for any of the following (ACOG Level C)
      • large, symptomatic masses
      • masses that are growing in size on serial imaging
      • suspected malignancy
    • cystectomy is considered usual management for symptomatic mature cystic teratoma of the ovary in nonpregnant premenopausal women
      • in pregnant patients, surgical removal of persistent adnexal masses in second trimester is common despite lack of evidence to support routine need
      • laparoscopy has fewer complications and shorter recovery time than laparotomy for functional cysts and benign ovarian tumors (including mature teratoma) (level 1 [likely reliable] evidence)
      •  laparoscopy for large adnexal cysts may be associated with increased risk for intraperitoneal spillage compared to laparotomy (level 3 [lacking direct] evidence)
  • expectant management
    • generally suggested for pregnant women with asymptomatic cysts or when the risk of surgery is greater than the risk of adnexal torsion
    • may be considered as an alternative to surgery for treatment of asymptomatic mature cystic teratoma of the ovary without suspected malignancy in nonpregnant patients, particularly in women concerned about effect of any ovarian cystectomy on ovarian reserve and function
  •  if teratoma not surgically removed, follow with ultrasound every 6 months to 1 year, regardless of patient’s age to ensure that cyst is not changing in size or internal architecture

Surgery and Procedures

General Information

  • surgical intervention for suspected mature ovarian teratomas is warranted for any of the following (ACOG Level C)(5)
    • large, symptomatic masses
    • masses that are growing in size on serial imaging
    • suspected malignancy
  • other considerations for surgical removal of teratomas include(1)
    •  age of patient
    •  desire for future fertility
    •  cyst size
  • simple cystectomy is preferred treatment of symptomatic mature cystic teratoma of the ovary, particularly in(1)
    •  premenopausal women who may wish to preserve fertility
    •  children and adolescents
  •  consider oophorectomy in women who do not wish to preserve their fertility(1)
  • laparoscopic removal may be associated with increased risk of intraoperative cyst rupture; however, risk of chemical peritonitis due to intraoperative cyst rupture appears low(1)
    • spillage of cyst contents should be avoided, and surgical removal can be performed with endoscopic retrieval bag(4)
    • if spillage does occur, accurate peritoneal washing with copious warmed fluid may be required(4)
    • higher risk of spillage reported with laparoscopic surgery to manage malignant transformation(3)
  • considerations for surgery in pregnant patients
    • in pregnant patients, surgical removal of persistent adnexal masses in second trimester is common despite lack of evidence to support routine need (Am Fam Physician 2009 Oct 15;80(8):815full-text)
    • if intervention during pregnancy is warranted based on symptoms, laparoscopic approaches and laparotomy may be options(5)

Adults

  • laparoscopy vs. laparotomy
    • laparoscopy has fewer complications and shorter recovery time than laparotomy for functional cysts and benign ovarian tumors (including endometrioma and mature teratomas) (level 1 [likely reliable] evidence)
      •  based on Cochrane review
      •  systematic review of 9 randomized trials comparing laparoscopy vs. laparotomy in 482 women with benign ovarian tumors
      •  compared to laparotomy, laparoscopic surgery associated with
        •  fewer adverse events (surgical injury or postoperative complications) in analysis of 9 trials with 482 women
          •  odds ratio (OR) 0.29 (95% CI 0.17-0.51)
          •  NNT 6-11 with 21.7% of women with laparotomy experiencing adverse events
        •  less postoperative pain (visual analog scale [VAS] score weighted mean difference [WMD] -2.36, 95% CI -2.7 to -2) in analysis of 3 trials with 186 women
        •  greater likelihood of being pain free after 2 days in analysis of 6 trials with 356 women
          •  OR 7.42 (95% CI 4.86-11.33)
          •  NNT 2-3 with 16.3% of women with laparotomy pain-free 2 days after surgery
        •  fewer days in hospital (WMD -2.88 days, 95% CI -3.11 to -2.66) in analysis of 8 trials with 442 women
      •  compared to minilaparotomy, laparoscopic surgery associated with
        •  fewer adverse events (surgical injury or postoperative complications) in analysis of 2 trials with 227 women
          •  OR 0.1 (95% CI 0.01-0.77)
          •  NNT 13-59 with 7.9% of women with minilaparotomy experiencing adverse events
        •  fewer women experiencing pain after 2 days in 1 trial with 127 women (33.3% vs. 82.8%, p < 0.0001, NNT 2)
      •  Reference – Cochrane Database Syst Rev 2009 Jan 21;(1):CD004751
    • laparoscopy for large adnexal cysts associated with greater risk of intraperitoneal spillage than laparotomy (level 3 [lacking direct] evidence)
      •  based on nonclinical outcomes of 1 randomized trial and 1 retrospective cohort study
      • randomized trial of 60 women aged 18-45 years with adnexal cyst 7-18 cm and no ultrasound suspicion of endometriosis or malignancy had diagnostic open laparoscopy then randomized to operative laparoscopy vs. laparoscopically guided minilaparotomy
        • comparing laparoscopy vs. minilaparotomy
          •  intraperitoneal spillage in 80% vs. 33% (p < 0.001, NNH 2)
          •  median operative time 85 minutes vs. 48 minutes (p < 0.001)
          •  mean scar size 2.6 cm vs. 4 cm (p < 0.001)
        • Reference – Obstet Gynecol 2007 Aug;110(2 Pt 1):241
      • retrospective cohort study of 330 patients with teratoma of diameter > 15 cm
        • mean tumor size 24.9 cm
        • symptoms reported in 69.1%
        • intraperitoneal spillage in 31.5% with laparoscopy vs. 19.6% with laparotomy (p < 0.05)
        • Reference – Oncol Lett 2012 Oct;4(4):672full-text
    • no randomized trials identified comparing laparoscopic surgery vs. laparotomy for benign ovarian tumor during pregnancy
  •  case report of successful laparoscopy for removal of dermoid cyst > 15 cm in diameter with torsion in 18-year-old woman at 18 weeks gestation (J Reprod Med 2013 May-Jun;58(5-6):271)
  • cystectomy via mesial incision of the ovarian cyst may decrease spillage rate and operative time compared to cystectomy with typical anti-mesenteric incision in women with mature cystic ovarian teratomas (level 3 [lacking direct] evidence)
    •  based on randomized trial without clinical outcomes
    •  67 women with mature cystic ovarian teratomas were randomized to cystectomy by mesial incision vs. typical anti-mesenteric incision
    • comparing mesial incision vs. anti-mesenteric incision
      •  spillage of intracystic content in 3% vs. 20.6% (p < 0.05)
      •  operative time 48 minutes vs. 76 minutes (p < 0.001)
    •  Reference – Fertil Steril 2012 Nov;98(5):1336
  • accidental cyst rupture in 28% and intraperitoneal spillage in 0% of patients with routine use of endoscopic retrieval bag during surgical management of mature cystic teratoma
    •  based on retrospective cohort study
    • 314 patients (mean age 37 years) having surgery for histologically diagnosed mature cystic teratoma of the ovary over 20-year period were included
    • routine use of endoscopic retrieval bag in 44.6%
    • mean cyst diameter 62.7 mm (range 15-350 mm)
    • accidental cyst rupture in 28% with routine use of endoscopic retrieval bag vs. 15% without routine use of endoscopic retrieval bag (p = 0.005)
    • intraperitoneal spillage in 0% with routine use of endoscopic retrieval bag because rupture occurred within bag
    • Reference – BJOG 2010 Jul;117(8):1027full-text
  • cyst rupture in 100% but intraperitoneal spillage in 0% of 45 patients (mean age 29 years) with mature cystic teratoma of the ovary (median size 55 mm) managed with laparoscopic controlled drilling in bag, excision of cysts, and removal of cysts in bag reported in case series (J Laparoendosc Adv Surg Tech A 2015 Feb;25(2):143

Children and Adolescents

  • laparoscopic surgery for benign ovarian cysts using conventional instruments for adults associated with similar operative time and postoperative length of hospital stay when used in children and adolescents < 20 years old
    •  based on retrospective cohort study
    • 106 children and adolescents < 20 years old (mean age 17 years) having laparoscopic surgery were included
      • dermoid cyst in 30.2%
      • simple cyst in 28.3%
      • endometrioma in 14.2%
      • tuboovarian abscess in 14.2%
      • mucinous cystadenoma in 5.7%
      • serous cystadenoma in 3.8%
      • other pathology results in 5.7%
    • laparoscopic procedures included
      • cystectomy in 48.1%
      • salpingo-oophorectomy in 15.1%
      • oophorectomy in 12.3%
      • salpingectomy in 10.4%
      • aspiration in 5.7%
      • fulguration in 4.7%
      • detorsion in 3.8%
    • comparing children and adolescents < 20 years old in current study vs. 433 women aged 20-50 years in previous study who had laparoscopic adnexal surgery in the same hospital from 2006 through 2012
      •  mean operative time 64.9 minutes vs. 77.3 minutes (p < 0.0001)
      •  mean postoperative length of hospital stay 4.91 days vs. 4.45 days (p = 0.037)
      •  no significant difference in estimated blood loss or postoperative hemoglobin levels
    •  Reference – JSLS 2015 Jan-Mar;19(1):e2014.00253full-text

Other Management

  • most adnexal masses in pregnancy appear to have a low risk of malignancy or acute complications and may be managed expectantly (ACOG Level C)(5)
  • expectant management suggested for most pregnant patients if
  • expectant management may be considered in nonpregnant patients, particularly in women concerned about effect of any ovarian cystectomy on ovarian reserve and function
    • for small (< 5-7 cm), asymptomatic, typical teratomas without suspected malignancy(1)
    • in whom surgical intervention is not indicated due to very advanced age or multiple comorbidities(5)
  • bilateral cysts, multiparity, 1 mm increase of maximum cyst diameter at final ultrasound, presenting for initial scan with transient abdominal pain, and younger age associated with failed expectant management in women with mature cystic teratoma of the ovary (level 2 [mid-level] evidence)
    •  based on retrospective cohort study
    • 289 women with 323 symptomatic or asymptomatic mature cystic teratomas of the ovary diagnosed with transvaginal ultrasound were included
    • 93 women (32.2%, mean age 33 years) with asymptomatic 105 mature cystic ovarian teratomas (32.5%) were followed with expectant management for > 3 months and included in analyses
      • asymptomatic in 69.9%
      • transient mild abdominal pain in 19.4%
      • clinical diagnosis of pelvic mass or swelling in 10.8%
    • median follow-up 12.6 months
    • maximum diameter of mature cystic ovarian teratoma at initial scan 29 mm in women with successful expectant management vs. 57 mm in women with failed expectant management (p < 0.001)
    • mean growth rate during follow-up 1.67 mm/year
    • no torsions, rupture, or infection occurred during follow-up period
    • surgical intervention after period of expectant management in 25.8%, with reasons including
      • patient choice in 50%
      • development of pain or distension in 37.5%
      • incidental to unrelated surgery in 12.5%
    • factors associated with
      • increased risk of failed expectant management
        • bilateral cysts compared to unilateral left ovarian dermoid cyst (adjusted odds ratio [OR] 18.39, 95% CI 1.75-193.38)
        • multiparity compared to nulliparity (adjusted OR 13.12, 95% CI 1.86-92.53)
        • 1 mm increase in cyst diameter at final ultrasound (adjusted OR 1.05, 95% CI 1.01-1.09)
      • decreased risk of failed expectant management
        • presenting for initial scan with prior history of cyst compared to presenting for initial scan with transient abdominal pain (adjusted OR 0.1, 95% CI 0.01-0.91)
        • 10-year increase in age (adjusted OR 0.24, 95% CI 0.09-0.65)
    • Reference – Ultrasound Obstet Gynecol 2010 Aug;36(2):235full-text
  • conservative management with ultrasound surveillance of mature cystic ovarian teratoma < 6 cm may delay surgical treatment and allow for pregnancy
    •  based on prospective cohort study
    • 86 women with ultrasound diagnosis of mature cystic ovarian teratoma < 6 cm were evaluated
      • 83.7% premenopausal (mean age 32 years)
      • 16.3% postmenopausal (mean age 61 years)
    • ultrasound follow-up at 3 months and 9 months, then annually
      • mean duration of follow-up
        •  34.5 months for premenopausal women
        •  35.3 months for postmenopausal women
    •  mean initial cyst size 3.7 cm in premenopausal women and 4.1 cm in postmenopausal women
    •  bilateral cysts in 10 women (11.6%)
    •  annual growth rate 1.77 mm in premenopausal women vs. -1.59 mm in postmenopausal women (p < 0.001)
    • 24 cysts removed (27.9%), all of which were benign mature cystic ovarian teratomas confirmed by histopathology
      •  20 premenopausal and 4 postmenopausal women
      •  21 met study criteria for removal when growth > 2 cm/year
      •  3 removed at cesarean section for obstetrical reasons
    • 28 women with dermoid cysts in situ delivered 35 healthy infants without complication attributable to mature cystic ovarian teratoma
      •  5 women conceived twice, 1 woman conceived 3 times
      •  23 spontaneous conceptions, 5 with assisted reproductive technology
    •  Reference – Fertil Steril 1997 Sep;68(3):501

Follow-up

  • use ultrasound for follow-up monitoring if surgical excision is not performed
    • repeat ultrasound imaging recommended whenever diagnosis is uncertain or when cancer remains in the differential diagnosis(5)
    •  ideal interval and duration for ultrasound follow-up has not been defined(5)
    • follow-up every 6 months to 1 year may be considered regardless of patient’s age to evaluate cyst for changes in size or internal architecture (Radiology 2010 Sep;256(3):943)
    •  suggestions for duration for follow-up include up to 1 year for masses without solid components and up to 2 years for masses with solid components(5)
    • for adnexal masses, including mature cystic teratomas, in which surgery is not performed due to risk of perioperative morbidity and mortality, appropriate frequency of repeat imaging has not been established(5)

Complications and Prognosis

Complications

Adnexal Torsion

  •  adnexal torsion is a surgical emergency that occurs due to twisting of the ovary and/or fallopian tube on the axis created between the infundibulopelvic ligament and the utero-ovarian ligament causing venous compression, edema, and ultimately ovarian ischemia and necrosis if untreated
  • most common in women of reproductive age, including pregnant women
  • risk factors include
    • prior adnexal torsion
    • ovarian cysts
    • ovulation induction (especially with hyperstimulation syndrome)
    • history of tubal ligation
    • pregnancy
  • typically presents with acute-onset unilateral pelvic or abdominal pain, but pain may also be chronic, intermittent, positional, variable in intensity, and may radiate to the flank or groin
  • physical exam often demonstrates lower abdominal tenderness and palpable, enlarged, tender ovary on bimanual exam, as well as fever as a later sign resulting from necrotic tissue
  • alternative diagnoses include appendicitis, ruptured ovarian cyst, pelvic inflammatory disease, ectopic pregnancy, and nephrolithiasis
  • preoperative CA19-9 level > 37 units/mL associated with increased ovarian torsion in women with mature cystic teratoma of the ovary (level 2 [mid-level] evidence)
    •  based on systematic review of observational studies
    • systematic review of 7 cohort studies evaluating preoperative CA19-9 levels in 995 women with mature cystic teratoma of the ovary
      • mean age ranged from 31 to 41 years (not reported for all)
      • tumor size ranged from 7.1 to 10.1 cm
      • elevated CA19-9 levels defined as > 37 units/mL
    • 63% had normal CA19-9 levels, 37% had elevated CA19-9 levels
    • comparing women with elevated CA19-9 levels to women with normal CA19-9 levels, elevated CA19-9 levels associated with
      • increased ovarian torsion (odds ratio 2.12, 95% CI 1.02-4.39) in analysis of 2 studies with 273 women
      • increased tumor size (mean difference 1.34 cm, 95% CI 0.35-2.32 cm) in analysis of 5 studies with 808 women
      • nonsignificant increase in risk of bilateral teratoma (odds ratio 1.61, 95% CI 0.91-2.85) in analysis of 7 studies with 995 women
    • Reference – Cureus 2019 Dec 10;11(12):e6342full-text

Spontaneous Rupture

  • spontaneous rupture of mature cystic teratoma reported to occur in 1%-4% of cases(2,3)
  • chronic rupture is more common than acute rupture(3)
  • may cause sebaceous fluid or particles to leak into peritoneal cavity, which may result in peritonitis(1,3)
  • findings on computed tomography or magnetic resonance imaging may include(3)
    • wall discontinuity
    • ascites
    • distorted shape
    • localized or diffuse peritoneal stranding
    • mass-like infiltration
  • may be difficult to distinguish from tuberculous peritonitis or peritoneal carcinomatosis(3)
    • integrity of cyst wall should be thoroughly examined and observation of fat fluid level of ascitic fluid suggests ovarian teratoma
    • necrotic lymph nodes associated with other granulomatous features suggests tuberculosis peritonitis while solid enhancing peritoneal mass with primary site of cancer may indicate peritoneal carcinomatosis

Malignant Transformation

  • malignant transformation of mature cystic teratoma of the ovary reported to occur in 0.8%-5.5% of cases(1,2,4)
  • reported to occur more frequently in women age ≥ 50 years(4)
  •  larger tumors (> 10 cm) may be associated with increased risk of malignant transformation (Int J Gynecol Cancer 2011 Apr;21(3):466)
  • squamous cell carcinoma reported to be most common form of malignant transformation(1,4)
    • typically larger than benign mature cystic teratoma with a reported diameter range 9.7 cm to 15.6 cm
    • clinical presentation
      • clinical manifestation as acute abdomen is rare, especially in postmenopausal women
      • most common symptoms include
        • abdominal pain, reported in 50%-58%
        • abdominal palpable mass or swelling, reported in 31%-71%
      • less common symptoms (reported in 1%-6% of cases) include
        • urinary frequency or retention
        • vaginal bleeding
        • constipation or diarrhea
        • weight loss
        • cachexia
        • fever
    • on gross examination, squamous cell carcinoma developed from mature cystic teratoma may appear as polypoidal lesion, intramural nodule, or mural plaque with hemorrhage and necrosis in cystic mass that includes pultaceous material and hair
    • standard treatment includes
      • bilateral salpingooophorectomy, total hysterectomy, and comprehensive surgical staging (peritoneal washing, omentectomy, appendectomy, peritoneal biopsies, and pelvic plus para-aortic lymphadenectomy) for early disease
      • optimal cytoreductive surgery for advanced disease
      • standard paclitaxel/carboplatin-based chemotherapy as adjuvant treatment
      • more aggressive regimens such as dose-dose paclitaxel/carboplatin-based chemotherapy or three-drug combination of paclitaxel, ifosfamide, and cisplatin (TIP regimen) may be considered for patients with high tumor burden or recurrent disease
    • 5-year overall survival reported to range from 28% to 66%
    • 75% of women with malignant transformation of mature cystic teratoma of ovary develop squamous cell carcinoma
      •  based on retrospective cohort study
      •  2,956 women from 1 institution in Korea from 1989 to 2007 were included
      •  2,019 women had mature cystic teratoma of ovary
      • 16 women had malignant transformation of mature cystic teratoma
        •  squamous cell carcinoma in 12 women (75%)
        •  adenocarcinoma in 3 women (20%)
        •  mucodermoid carcinoma in 1 patient (5%)
        •  median age of patient 50 years (range 29-75 years)
        •  mean tumor size 14.5 cm (range 5-26 cm)
      • Reference – Eur J Obstet Gynecol Reprod Biol 2008 Dec;141(2):173
  • other less frequent malignant transformations associated with mature cystic teratoma of the ovary include(4)
    • mucinous carcinoma
    • adenocarcinoma from respiratory ciliated epithelium
    • melanoma
    • carcinoid
    • thyroid carcinoma
    • sebaceous carcinoma
    • oligodendroglioma
    • sarcoma
  •  reported characteristics of mature cystic teratoma of the ovary having undergone malignant transformation
    •  based on 2 retrospective cohort studies
    • cohort of 16 patients (median age 50 years) with malignant transformation of mature cystic teratoma of the ovary
    • cohort of 11 patients (median age 47 years) with malignant transformation of mature cystic teratoma of the ovary
      •  carcinoid tumor in 6 patients (54.5%)
      •  squamous cell carcinoma in 4 patients (36%)
      •  transitional cell carcinoma in 1 patient (9%)
      • stage at initial presentation
        • stage I or stage II in 8 patients (73%)
        •  stage III or stage IV in 3 patients (27%)
      •  Reference – Saudi Med J 2009 Apr;30(4):524

Rare Complications

Prognosis

  •  typically slow growing (about 1.8 mm/year)(2)
  • benign mature cystic teratomas of the ovary may not be associated with adverse perinatal or neonatal outcomes
    •  based on population-based retrospective cohort study
    •  212,144 women with deliveries at 1 hospital in Israel from 1988 to 2007 were included
    • benign ovarian cysts occurred in 93 deliveries, with 36.7% diagnosed as mature cystic teratomas
    •  mean diameter of mature cystic teratoma 6.09 cm (range 3-20 cm)
    •  no significant association between benign mature cystic teratomas of the ovary and birth weight, congenital malformations, Apgar scores, or perinatal mortality
    •  Reference – Arch Gynecol Obstet 2010 May;281(5):811
  • recurrence after surgical removal
    • intraoperative spillage associated with increased risk for benign recurrence in female patients having surgical removal of benign ovarian cysts (level 2 [mid-level] evidence)
      • based on systematic review of observational studies
      • systematic review of 3 randomized trials and 25 observational studies evaluating short- and long-term complications in female patients with intraoperative rupture of benign ovarian cysts
      • 12 studies contributed to meta-analysis
      • comparing intraoperative spillage vs. no intraoperative spillage
        • intraoperative spillage associated with increased risk for benign recurrence (risk ratio 3.1, 95% CI 1.05-9.14) in analysis of 4 studies with 498 female patients
        • no significant differences in
          • ovarian cyst diameter in analysis of 4 studies
          • chemical peritonitis, reoperation, infertility, transient fever, or readmission in analysis of 1-4 studies
      • Reference – J Minim Invasive Gynecol 2021 May;28(5):957
    • recurrence of mature cystic teratoma of the ovary reported in 4.5% of women after surgery
      •  based on retrospective cohort study
      • 178 women (mean age 29 years) who had surgical management of mature cystic teratoma of the ovary were included
        • laparoscopy in 79.2%
        • laparotomy in 20.8%
      • follow-up ≥ 34 months
      • recurrence in 4.5% within 26 months of first surgery (range 6-26 months)
      • comparing recurrence vs. no recurrence
        • mean age 27.2 vs. 35.5 years (p = 0.02)
        • median gravidity 0 vs. 2 (p = 0.04)
        • median parity 0 vs. 1 (p = 0.04)
        • mean serum level of alpha-fetoprotein (AFP) tumor marker 1.2 vs. 2.1 (p = 0.04)
      • no significant associations between type of surgery, cyst location, or serum levels of tumor markers (other than AFP) and risk of recurrence
      • Reference – J Obstet Gynaecol 2016;36(3):289
    • recurrent or persistent mature cystic teratoma of ovary reported in 10.6% of adolescent/pediatric patients after cystectomy
      •  based on retrospective cohort study
      •  66 adolescent and pediatric patients < 18 years old (mean age 13 years) treated with ovarian cystectomy of mature teratoma of ovary by either laparoscopy or laparotomy were included
      • follow-up
        • annual ultrasound for up to 5 years in 53%
        • single ultrasound in 9%
        • no follow-up in 38%
      •  10.6% had recurrent or persistent teratoma of which 3% required repeat surgery
      •  Reference – J Pediatr Adolesc Gynecol 2014 Aug;27(4):222

Prevention and Screening

  •  not applicable

Guidelines and Resources

Guidelines

United States Guidelines

  •  American College of Obstetrics and Gynecology (ACOG) Practice Bulletin 174 on evaluation and management of adnexal masses can be found in Obstet Gynecol 2016 Nov;128(5):e210
  •  American College of Radiology (ACR) Appropriateness Criteria for clinically suspected adnexal mass, no acute symptoms can be found at ACR 2023 PDF

United Kingdom Guidelines

  • Royal College of Obstetricians and Gynaecologists/British Society of Gynaecological Endoscopy (RCOG/BSGE) guideline on ovarian masses in premenopausal women can be found at RCOG/BSGE 2011 Nov PDF

Canadian Guidelines

European Guidelines

Review Articles

  •  to search MEDLINE for (Mature cystic teratoma of the ovary) with targeted search (Clinical Queries), click therapy, diagnosis, or prognosis

Patient Information

References

General References Used

The references listed below are used in this DynaMed topic primarily to support background information and for guidance where evidence summaries are not felt to be necessary. Most references are incorporated within the text along with the evidence summaries.

  1. Multani J, Kives S. Dermoid cysts in adolescents. Curr Opin Obstet Gynecol. 2015 Oct;27(5):315-9.
  2. Sahin H, Abdullazade S, Sanci M. Mature cystic teratoma of the ovary: a cutting edge overview on imaging features. Insights Imaging. 2017 Apr;8(2):227-241full-text.
  3. Srisajjakul S, Prapaisilp P, Bangchokdee S. Imaging features of unusual lesions and complications associated with ovarian mature cystic teratoma. Clin Imaging. 2019 May 28;57:115-123.
  4. Gadducci A, Guerrieri ME, Cosio S. Squamous cell carcinoma arising from mature cystic teratoma of the ovary: A challenging question for gynecologic oncologists. Crit Rev Oncol Hematol. 2019 Jan;133:92-98.
  5. American College of Obstetricians and Gynecologists’ Committee on Practice Bulletins—Gynecology. Practice Bulletin No. 174: Evaluation and Management of Adnexal Masses. Obstet Gynecol. 2016 Nov;128(5):e210-e226.

Recommendation Grading Systems Used

  • American College of Obstetricians and Gynecologists (ACOG) grading system for recommendations
    • grades of evidence
      • Grade I – evidence obtained from ≥ 1 properly designed randomized controlled trial
      • Grade II-1 – evidence obtained from well-designed controlled trials without randomization
      • Grade II-2 – evidence obtained from well-designed cohort or case control studies, preferably from more than 1 center or research center
      • Grade II-3 – evidence obtained from multiple time series with or without intervention, or dramatic results in uncontrolled experiments
      • Grade III – opinions of respected authorities, based on clinical experience, descriptive studies, or reports of expert committees
    • levels of recommendation
      • Level A – recommendations based on good and consistent scientific evidence
      • Level B – recommendations based on limited or inconsistent scientific evidence
      • Level C – recommendations based primarily on consensus and expert opinion
    • Reference – ACOG Practice Bulletin 174 on evaluation and management of adnexal masses (Obstet Gynecol 2016 Nov;128(5):e210)

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