Krukenberg tumor

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.

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.

Pathophysiology

• The majority of Krukenberg’s tumors are bilateral.
• Stomach is the primary site in the majority of Krukenberg tumor cases (70%).
• The pathogenesis of krukenberg tumors is from metastasis of tumor cells from the stomach, appendix or colon to the ovaries.
  • 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.
• 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%).

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:
  • Lymphatic spread; or
  • Direct seeding across the abdominal cavity

Differentiating [disease name] from other Diseases

• Krukenberg's tumor must be differentiated from the following:

• 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.

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:
  • 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.
• Early clinical features include abdominal pain and distension(from the large, bilateral ovarian masses).
• If left untreated, patients with krukenberg tumors may progress to develop virilization from the excessive hormone production from the ovarian stroma.
• Common complications of [disease name] include ascites, virilization, and pseudo-Meig syndrome.
  • 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. The median survival of patients is between 7 to 14 months.

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.
• Symptoms of krukenberg tumors may include the following:

• 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:

• Abdominal or pelvic mass
• Ascites
• Hirsutism

Laboratory Findings

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

Imaging Findings

X Ray

• 
  X ray showing prominent small bowel loops filled with gas, no air-fluid level, no radiographic signs of intestinal obstruction

Ultrasound

• On pelvic ultrasound, krukenberg tumor is characterized by the following:</ref>
  • 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

• 
  Large, solid, well defined bilateral ovarian masses
• 
  Large, solid, well defined bilateral ovarian masses
• 
  Large, solid, well defined bilateral ovarian masses
• 
  Large, solid, well defined bilateral ovarian masses

CT

• On CT, krukenberg tumor is characterized by the following:
  • Large, lobulated, and multicystic masses with soft-tissue components which are indistinguishable from primary ovarian cancers
  • Presence of a concurrent gastric or colic mural lesion

• 
  Krukenberg tumour of the right ovary from colon cancer

MRI

• On pelvic MRI, krukenberg tumor is characterized by the following:
  • Bilateral complex masses with hypo-intense solid components (dense stromal reaction)
  • Internal hyperintensity (mucin) on T1 and T2 weighted MR images
  • Strong contrast enhancement is usually seen in the solid component or the wall of the intratumoral cyst

• 
  Coronal T2 image
• 
  Axial T1 image
• 
  Sagittal T2 image
• 
  Axial T2 image
• 
  Axial T1 C+ fat sat dynamic contrast study image

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:
  • 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

• Management of krukenberg tumors is driven by the identification and treatment of the primary cancer.
• Chemotherapy/radiation has no significant effect on the prognosis of patients with Krukenberg tumors.

Surgery

• Bilateral oophorectomy may only be performed for patients where the metastasis is localized to the ovaries.
• The significance of early detection of ovarian metastasis and the importance of monitoring serum CA 125 level may improve the prognosis.
• Surgical resection may not be an option if the tumor has already metastasized to other sites.

Prevention

• There are no preventive measures available for krukenberg tumors.

Case Studies

Case #1