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==Epidemiology and Demographics==
==Epidemiology and Demographics==
===Incidence===
===Incidence===
*The incidence of [disease name] is estimated to be approximately 0.16 per 100,000 individuals.<ref name="pmid23024922">{{cite journal| author=Tellier F, Steibel J, Chabrier R, Blé FX, Tubaldo H, Rasata R et al.| title=Sentinel lymph nodes fluorescence detection and imaging using Patent Blue V bound to human serum albumin. | journal=Biomed Opt Express | year= 2012 | volume= 3 | issue= 9 | pages= 2306-16 | pmid=23024922 | doi=10.1364/BOE.3.002306 | pmc=PMC3447570 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23024922  }} </ref><ref>Krukenberg tumour. Radiopedia. Accessed on March 23, 2016.</ref>
*The incidence of [disease name] is estimated to be approximately 0.16 per 100,000 individuals.<ref name="pmid23024922">{{cite journal| author=Tellier F, Steibel J, Chabrier R, Blé FX, Tubaldo H, Rasata R et al.| title=Sentinel lymph nodes fluorescence detection and imaging using Patent Blue V bound to human serum albumin. | journal=Biomed Opt Express | year= 2012 | volume= 3 | issue= 9 | pages= 2306-16 | pmid=23024922 | doi=10.1364/BOE.3.002306 | pmc=PMC3447570 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23024922  }} </ref><ref name=ok>Krukenberg tumour. Radiopedia. Accessed on March 23, 2016.</ref>
   
   
===Age===
===Age===
Line 109: Line 109:


===Laboratory Findings===
===Laboratory Findings===
*There are no specific laboratory findings associated with [disease name].
*There are no specific laboratory findings associated with krukenberg tumors.


*A  [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
===Imaging Findings===
===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].
====Ultrasound====
*On pelvic ultrasound, krukenberg tumor is characterized by the following:<ref name="pmid23024922">{{cite journal| author=Tellier F, Steibel J, Chabrier R, Blé FX, Tubaldo H, Rasata R et al.| title=Sentinel lymph nodes fluorescence detection and imaging using Patent Blue V bound to human serum albumin. | journal=Biomed Opt Express | year= 2012 | volume= 3 | issue= 9 | pages= 2306-16 | pmid=23024922 | doi=10.1364/BOE.3.002306 | pmc=PMC3447570 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23024922  }} </ref></ref><ref name=ok>Krukenberg tumour. Radiopedia. Accessed on March 23, 2016.</ref>
*[Imaging study 1] is the imaging modality of choice for [disease name].
**Bilateral, solid ovarian masses with clear, well defined margins
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
**An irregular hyper-echoic solid pattern and moth eaten like cyst formation is also considered a characteristic feature
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
 
====Gallery====
<Gallery>
Image:Krukenberg-tumour-3.JPG|Large, solid, well defined bilateral ovarian masses<ref name=op>Image courtesy of  Dr Prashant Mudgal. [http://www.radiopaedia.org Radiopaedia] (original file [http://radiopaedia.org/cases/krukenberg-tumour-3 ‘’here’’]). [http://radiopaedia.org/licence Creative Commons BY-SA-NC]</ref>
 
Image:Krukenberg-tumour-3_(2).JPG|Large, solid, well defined bilateral ovarian masses<ref name=op>Image courtesy of  Dr Prashant Mudgal. [http://www.radiopaedia.org Radiopaedia] (original file [http://radiopaedia.org/cases/krukenberg-tumour-3 ‘’here’’]). [http://radiopaedia.org/licence Creative Commons BY-SA-NC]</ref>
 
Image:Krukenberg-tumour-3_(3).JPG|Large, solid, well defined bilateral ovarian masses<ref name=op>Image courtesy of  Dr Prashant Mudgal. [http://www.radiopaedia.org Radiopaedia] (original file [http://radiopaedia.org/cases/krukenberg-tumour-3 ‘’here’’]). [http://radiopaedia.org/licence Creative Commons BY-SA-NC]</ref>
 
Image:Krukenberg-tumour-3_(4).JPG|Large, solid, well defined bilateral ovarian masses<ref name=op>Image courtesy of  Dr Prashant Mudgal. [http://www.radiopaedia.org Radiopaedia] (original file [http://radiopaedia.org/cases/krukenberg-tumour-3 ‘’here’’]). [http://radiopaedia.org/licence Creative Commons BY-SA-NC]</ref>
   
   
=== Other Diagnostic Studies ===
=== Other Diagnostic Studies ===

Revision as of 15:42, 22 March 2016

Krukenberg tumor
Krukenberg tumor
ICD-10 C56
ICD-9 183
ICD-O: 8490/6
DiseasesDB 30081
MeSH C04.557.470.200.025.415.410

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]

Synonyms and keywords: carcinoma mucocellulare; Synonym 2; Synonym 3

Overview

Krukenberg's tumor is a rare metastatic signet ring cell adenocarcinoma of the ovary.[1]

Historical Perspective

  • Krukenberg's tumor was first described as a new type of primary ovarian malignancy by Friedrich Ernst Krukenberg (1871–1946), a German gynecologist and pathologist, in 1896 which was later confirmed to be of metastatic gastrointestinal tract origin.[1]

Pathophysiology

  • The majority of Krukenberg’s tumors are bilateral.[1]
  • Stomach is the primary site in the majority of Krukenberg tumor cases (70%).[1]
  • The pathogenesis of krukenberg tumors is from metastasis of tumor cells from the stomach, appendix or colon to the ovaries.[2]
    • Metastasis is more likely via the lymphatic spread. However, direct seeding across the abdominal cavity may also occur.
  • On gross pathology, asymmetrically enlarged ovaries with a bosselated contour, usually solid, yellow or white cross sectioned surfaces, and the absence of adhesions or peritoneal deposits are characteristic findings of krukenberg tumors.[1]
  • On microscopic histopathological analysis, krukenberg tumors are characterized by the following features:
    • Tumor composed of two components:
      • Epithelial
        • Mucin-secreting signet ring cells with eccentric hyperchromatic nuclei
        • Cytoplasm may be eosinophilic and granular, pale and vacuolated, or a bull's eye (targetoid) appearance with a large vacuole with a central to paracentral eosinophilic body composed of a droplet of mucin
        • Signet ring cells may be single, clustered, nested, or arranged in tubules, acini, trabeculae, or cords
      • Stromal
        • Plump and spindle-shaped cells with minimal cytologic atypia or mitotic activity
        • Focal or diffuse stromal edema which may form pseudo cysts
        • Desmoplastic reaction may be present
  • Stomach is the primary site in the majority of Krukenberg tumor cases (70%).[1]

Causes

  • Krukenberg's tumor may be caused by metastasis of the tumor cells from a primary cancer in the stomach, appendix, or the colon via:[2]
    • Lymphatic spread; or
    • Direct seeding across the abdominal cavity

Differentiating [disease name] from other Diseases

  • Krukenberg's tumor must be differentiated from the following:[1]
  • Primary mucinous carcinoma
  • Mucinous carcinoid tumor
  • Signet ring stromal tumor
  • Sclerosing stromal cell tumor
  • Clear cell adenocarcinoma of the ovary
  • Sertoli-Leydig cell tumor

Epidemiology and Demographics

Incidence

  • The incidence of [disease name] is estimated to be approximately 0.16 per 100,000 individuals.[3][4]

Age

  • Patients of all age groups may develop krukenberg tumors.
  • Krukenberg's tumor is more commonly observed among women in their fifth decade of lives, with anaverage around 45 years of age.

Risk Factors

  • There are no known direct causes for krukenberg tumors. Common risk factors for ovarian cancer may include:[5]
    • Nulliparity
    • Early menarche
    • Late menopause
    • Hormone therapy
    • Fertility medications

Natural History, Complications and Prognosis

  • The majority of patients with krukenberg tumor remain asymptomatic or have non-specific gastrointestinal symptoms.[1]
  • Early clinical features include abdominal pain and distension(from the large, bilateral ovarian masses).[1]
  • If left untreated, patients with krukenberg tumors may progress to develop virilization from the excessive hormone production from the ovarian stroma.[6]
  • Common complications of [disease name] include ascites, virilization, and pseudo-Meig syndrome.[6][1]
    • Pseudo-Meig syndrome is defined as a hydrothorax with ascites, but with the absence of tumor cells.
  • Prognosis is generally poor, and the 5 ­year survival rate of patients with krukenberg tumor is lower in patients in with a preoperative serum CA 125 levels greater than 75 U/mL when compared with patients with CA 125 levels less than 75 U/mL.[7] The median survival of patients is between 7 to 14 months.[6]

Diagnosis

Diagnostic Criteria

  • The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • Krukenberg's tumor is usually asymptomatic.[1]
  • Symptoms of krukenberg tumors may include the following:[1][2]
  • Non-specific gastrointestinal symptoms
  • Abdominal or pelvic pain
  • Abdominal distension
  • Bloating
  • Dyspareunia
  • Vaginal bleeding
  • Menstrual irregularities
  • Hirsutism

Physical Examination

  • Patients with krukenberg tumor usually appear well in the early stages.
  • Physical examination may be remarkable for:[2][1]
  • Abdominal or pelvic mass
  • Ascites
  • Hirsutism

Laboratory Findings

  • There are no specific laboratory findings associated with krukenberg tumors.

Imaging Findings

Ultrasound

  • On pelvic ultrasound, krukenberg tumor is characterized by the following:[3]</ref>[4]
    • Bilateral, solid ovarian masses with clear, well defined margins
    • An irregular hyper-echoic solid pattern and moth eaten like cyst formation is also considered a characteristic feature

Gallery

<Gallery> Image:Krukenberg-tumour-3.JPG|Large, solid, well defined bilateral ovarian masses[8]

Image:Krukenberg-tumour-3_(2).JPG|Large, solid, well defined bilateral ovarian masses[8]

Image:Krukenberg-tumour-3_(3).JPG|Large, solid, well defined bilateral ovarian masses[8]

Image:Krukenberg-tumour-3_(4).JPG|Large, solid, well defined bilateral ovarian masses[8]

Other Diagnostic Studies

Immunohistochemistry

  • Krukenberg's tumor may also be diagnosed using immunohistochemistry.
  • Findings on immunohistochemistry include:
    • Cytokeratins (AE1/AE3) positive
    • Epithelial membrane antigen positive
    • Vimentin negative
    • Inhibin negative

=Serum CA-125

  • Serum concentrations of CA 125 may be helpful for:[1]
    • Post-operative follow-up of patients for evaluation of complete resection of the tumor
    • Follow-up of patients with a history of primary adenocarcinomas (particularly gastrointestinal) for early detection of ovarian metastasis

Treatment

Medical Therapy

  • There is no treatment for [disease name]; the mainstay of therapy is supportive care.
  • The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
  • [Medical therapy 1] acts by [mechanism of action1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

  • Surgery is the mainstay of therapy for [disease name].
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
  • [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

  • There are no primary preventive measures available for [disease name].
  • Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
  • Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

Case Studies

Case #1

References

  1. ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 Al-Agha OM, Nicastri AD (2006). "An in-depth look at Krukenberg tumor: an overview". Arch Pathol Lab Med. 130 (11): 1725–30. doi:10.1043/1543-2165(2006)130[1725:AILAKT]2.0.CO;2. PMID 17076540.
  2. ↑ 2.0 2.1 2.2 2.3 Krukenberg tumor. Wikipedia. https://en.wikipedia.org/wiki/Krukenberg_tumor#cite_note-Young2006-4 Accessed on March 21, 2016.
  3. ↑ 3.0 3.1 Tellier F, Steibel J, Chabrier R, Blé FX, Tubaldo H, Rasata R; et al. (2012). "Sentinel lymph nodes fluorescence detection and imaging using Patent Blue V bound to human serum albumin". Biomed Opt Express. 3 (9): 2306–16. doi:10.1364/BOE.3.002306. PMC 3447570. PMID 23024922.
  4. ↑ 4.0 4.1 Krukenberg tumour. Radiopedia. Accessed on March 23, 2016.
  5. ↑ Ovarian cancer. Wikipedia. https://en.wikipedia.org/wiki/Ovarian_cancer#Genetics Accessed on March 23, 2016.
  6. ↑ 6.0 6.1 6.2 Hornung M, Vogel P, Schubert T, Schlitt HJ, Bolder U (2008). "A case of virilization induced by a Krukenberg tumor from gastric cancer". World J Surg Oncol. 6: 19. doi:10.1186/1477-7819-6-19. PMC 2275731. PMID 18279511.
  7. ↑ Khan M, Bhatti RP, Mukherjee S, Ali AM, Gilman AD, Mirrakhimov AE; et al. (2015). "A 26-year-old female with metastatic primary gastrointestinal malignancy presenting as menorrhagia". J Gastrointest Oncol. 6 (2): E21–5. doi:10.3978/j.issn.2078-6891.2014.080. PMC 4311099. PMID 25830046.
  8. ↑ 8.0 8.1 8.2 8.3 Image courtesy of Dr Prashant Mudgal. Radiopaedia (original file ‘’here’’). Creative Commons BY-SA-NC
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