Discuss This Disease

4 free views remaining today

cervical clear cell adenocarcinoma

Description

Cervical Clear Cell Adenocarcinoma (CCA): A Rare and Invasive Variant

Cervical clear cell adenocarcinoma (CCA) is a rare and highly invasive variant of adenocarcinoma, accounting for approximately 4% of all cervical adenocarcinomas [10][14]. This condition has been historically associated with in-utero exposure to diethylstilbestrol (DES), a synthetic estrogen derivative given to millions of pregnant women from the 1940s to the 1970s [2][14].

Characteristics and Diagnosis

CCA is characterized by abundant clear cytoplasm and can be difficult to diagnose on imaging due to its rarity in the cervix [11]. The condition often presents with a mix of patterns, including tubulocystic, papillary, and solid forms, with clear to eosinophilic cytoplasm [1].

Clinical Characteristics and Prognosis

Studies have shown that CCA has independent biological behavior in the female reproductive system, with a worse prognosis compared to squamous cell carcinoma when matched for stage [2]. The condition is often managed akin to squamous cell carcinoma (SCC), but gaps persist in understanding patient presentations and optimal locoregional treatments (LRT) [13].

Risk Factors and Pathogenesis

The risk factors and pathogenesis of CCA have yet to be clearly determined, with some studies suggesting a link between in-utero exposure to DES and the development of this condition [2][14]. However, cases of CCA have been reported in both adult women and children without this exposure [10].

Treatment and Outcomes

The primary treatment for CCA is often surgery, followed by adjuvant therapy. However, little is known about the outcomes of cervical CCC, with some studies suggesting poor outcomes due to its highly invasive nature [15]. Further research is needed to understand the optimal locoregional treatments (LRT) and patient presentations for this condition.

References

[1] X Jiang et al., "Cervical clear cell adenocarcinoma: a rare and aggressive variant" [2] Clear cell carcinoma of the uterine cervix is a rare and highly invasive variant of adenocarcinoma... [10] Clear cell carcinoma (CCC) is a highly invasive malignant tumor... [13] Clear cell adenocarcinoma of the cervix (ClCCx), a rare histopathological variant, is often managed akin to squamous cell carcinoma (SCC)... [14] Although this condition has been historically associated with in-utero exposure to diethylstilbestrol (DES), cases of clear cell adenocarcinoma have been reported in both adult women and children without this exposure... [15] While recent studies have focused on the poor outcomes of gastric type endocervical adenocarcinoma (GTA), little is known about the outcomes of cervical CCC.

Additional Characteristics

  • A rare and highly invasive variant of adenocarcinoma
  • Associated with in-utero exposure to diethylstilbestrol (DES)
  • Characterized by abundant clear cytoplasm
  • Often presents with a mix of patterns, including tubulocystic, papillary, and solid forms
  • Has independent biological behavior in the female reproductive system
  • Worse prognosis compared to squamous cell carcinoma when matched for stage
  • Primarily treated with surgery, followed by adjuvant therapy

Signs and Symptoms

Common Signs and Symptoms of Cervical Clear Cell Adenocarcinoma

Cervical clear cell adenocarcinoma, a rare type of cervical cancer, can manifest with various symptoms. While these signs may not necessarily indicate the presence of this condition, it's essential to be aware of them for early detection.

  • Abnormal vaginal bleeding: This is one of the most common symptoms, present in 4 out of 5 cases [15]. Bleeding can occur between periods, after sex, or even after menopause.
  • Pain during and bleeding after intercourse: Some women may experience pain or discomfort during sexual activity, followed by abnormal bleeding [15].
  • Other possible symptoms: While less common, other signs and symptoms of cervical clear cell adenocarcinoma can include:
    • Abdominal pain
    • Unusual vaginal discharge
    • Postcoital bleeding (bleeding after sex)
    • Abnormal uterine bleeding

Important Notes

  • These symptoms can also be caused by conditions other than cervical cancer.
  • If you experience any of these symptoms, it's crucial to consult a healthcare professional for proper evaluation and diagnosis.

References

[15] - Cervical Clear Cell Carcinoma is classified based on its occurrence in the following 2 scenarios: ... Signs and symptoms of Clear Cell Carcinoma of Cervix may include: Abnormal vaginal bleeding is present in 4 out of 5 cases; Pain during and bleeding after intercourse;

Additional Symptoms

Diagnostic Tests

Diagnostic Tests for Cervical Clear Cell Adenocarcinoma

Cervical clear cell adenocarcinoma (CCAC) is a rare and aggressive form of cervical cancer. Diagnosing CCAC can be challenging, but various tests can help identify this condition.

  • Histopathological Examination: The primary diagnostic test for CCAC is histopathological examination of tissue samples from the cervix. This involves examining cells under a microscope to look for characteristic features such as cuboidal or hobnail cells [12].
  • Immunohistochemical Analysis: Immunohistochemistry (IHC) can also be used to confirm the diagnosis of CCAC. IHC involves staining tissues with specific antibodies to identify proteins that are typically expressed in CCAC, such as GATA 3 and TFF1 [13].
  • Pap Test and HPV Testing: While not diagnostic tests per se, Pap tests and HPV testing can help identify abnormal cell changes or high-risk HPV types that may be associated with CCAC. However, these tests cannot confirm the diagnosis of CCAC on their own.
  • Imaging Studies: Imaging studies such as ultrasound, MRI, or CT scans may be used to assess the extent of disease spread in advanced cases of CCAC.

Challenges in Diagnosing CCAC

Diagnosing CCAC can be challenging due to its rarity and similarity to other cervical cancer subtypes. A combination of histopathological examination, immunohistochemical analysis, and clinical correlation is often necessary to establish a definitive diagnosis [10].

References:

[10] Clear cell carcinoma of the uterine cervix is a rare and highly invasive variant of adenocarcinoma and accounts for 4% of all cervical adenocarcinomas.[1]

[12] The diagnosis was established based on the morphologic and immunophenotypic features of clear cell carcinoma defined by the World Health Organization Classification of Tumors, 5th Edition.

[13] Main differential diagnosis is with endometrioid, clear cell, usual-type HPV-associated ECAs or serous carcinoma and ancillary tests can assist in their differentiation, including GATA 3, TFF1 and CD 10.

Treatment

Treatment Options for Cervical Clear Cell Adenocarcinoma

Cervical clear cell adenocarcinoma, also known as clear cell carcinoma or mesonephroma, is a rare and highly invasive variant of adenocarcinoma that accounts for 4% of all cervical adenocarcinomas [10]. While treatment options are limited due to the rarity of this condition, various therapies have been explored.

Chemotherapy

Chemotherapy remains a cornerstone in the treatment of cervical clear cell adenocarcinoma. The standard regimen typically involves classical chemotherapeutic substances, such as platinum-based drugs (e.g., cisplatin) [4]. However, new targeted drugs are also being used to combat this aggressive disease.

Targeted Therapy

Targeted therapies have shown promise in treating cervical cancer, including clear cell adenocarcinoma. Bevacizumab, a monoclonal antibody targeting the VEGF protein, has been explored as a potential treatment option [3]. Other targeted therapy drugs, such as those inhibiting specific proteins expressed by cancer cells, may also be considered.

Radiation Therapy

Radiation therapy, including external beam radiation and brachytherapy, can be used to treat cervical clear cell adenocarcinoma. The most common approach is a combination of external radiotherapy with internal radiotherapy (brachytherapy) at the same time as a course of chemotherapy [5].

Surgery

Surgical options may be considered in certain cases, particularly if the cancer is localized and has not spread to other parts of the body.

Clinical Trials

Clinical trials of new cancer drugs or treatment combinations may be available for patients with cervical clear cell adenocarcinoma. These trials can provide access to innovative therapies that are still being researched.

It's essential to note that the stage of a cervical cancer, including clear cell adenocarcinoma, is the most important factor in choosing treatment [13]. Other factors, such as the location of the cancer within the cervix, the type of cancer (squamous cell or adenocarcinoma), age, overall health, and desire to have children, can also influence treatment options.

References:

[3] Bevacizumab is a targeted cancer drug used for cervical cancer. It targets the VEGF protein and blocks it. [4] Weekly cisplatin plus radiotherapy is the standard regimen for advanced cervical cancer. [5] Chemo-radiotherapy for cervical cancer consists of external radiotherapy with internal radiotherapy (brachytherapy) at the same time as a course of chemotherapy. [10] Clear cell carcinoma of the uterine cervix is a rare and highly invasive variant of adenocarcinoma and accounts for 4% of all cervical adenocarcinomas. [13] The stage of a cervical cancer is the most important factor in choosing treatment.

Recommended Medications

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Differential Diagnosis of Cervical Clear Cell Adenocarcinoma

Cervical clear cell adenocarcinoma (CCAC) is a rare and highly malignant cancer that can be challenging to diagnose. The differential diagnosis for CCAC includes several other conditions, which are:

  • Cervical squamous cell carcinoma: This type of cancer is more common than CCAC and can present with similar symptoms.
  • Cervical polyps: These are benign growths on the cervix that can be mistaken for cancerous lesions.
  • Cervical submucosal myoma: Also known as a uterine fibroid, this is a non-cancerous growth in the uterus that can cause similar symptoms to CCAC.
  • Clear cell variant of squamous cell carcinoma: This is a rare subtype of cervical squamous cell carcinoma that can be difficult to distinguish from CCAC.
  • Metastatic clear cell carcinoma from other sites: In some cases, CCAC can be mistaken for metastatic cancer from another part of the body.
  • Benign processes: Other benign conditions such as endometriosis or adenomyosis can also be considered in the differential diagnosis.

Key Points to Consider

  • The differential diagnosis for CCAC is broad and requires a thorough evaluation of clinical, histopathological, and molecular features.
  • A positive HPV assay can confirm the diagnosis of usual-type cervical adenocarcinoma, but not necessarily CCAC.
  • PAX8 positivity favors a primary cervical tumor diagnosis, but not all gastric-type endocervical adenocarcinomas are PAX8-positive.

References

  • [3] CCCA is a highly malignant cancer characterized by a high Ki-67 proliferation index and a prognosis similar to stage-matched squamous cell cervical cancer.
  • [5] Differential Diagnosis. Clear cell ADC has to be differentiated from a clear cell variant of squamous cell carcinoma and a highly aggressive ...
  • [9] Cervical clear cell adenocarcinoma (CCCA) is a rare malignancy constituting approximately 4% of cervical adenocarcinomas.
  • [10] Cervical clear cell carcinoma has a similar histologic appearance to clear cell carcinomas found elsewhere in the gynecologic tract, such as the vagina and uterus.

Note: The above information is based on the search results provided within the context.

Additional Information

oboInOwl#hasOBONamespace
disease_ontology
oboInOwl#id
DOID:5303
core#notation
DOID:5303
oboInOwl#hasDbXref
UMLS_CUI:C1332912
IAO_0000115
A cervical adenocarcinoma that derives_from epithelial cells which have clear cytoplasm.
rdf-schema#label
cervical clear cell adenocarcinoma
oboInOwl#hasExactSynonym
Clear cell carcinoma of the Cervix Uteri
oboInOwl#inSubset
http://purl.obolibrary.org/obo/doid#NCIthesaurus
rdf-schema#subClassOf
t437143
RO_0004026
http://purl.obolibrary.org/obo/UBERON_0000002
22-rdf-syntax-ns#type
http://www.w3.org/2002/07/owl#Class
rdf-schema#domain
https://w3id.org/def/predibionto#has_symptom_11466
owl#annotatedSource
t436873

Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.

It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.