endometrial clear cell adenocarcinoma

Endometrial Clear Cell Adenocarcinoma: A Rare and Poorly Understood Entity

Endometrial clear cell adenocarcinoma (CCC) is a rare type of uterine malignancy, accounting for less than 5% of endometrial cancer cases [8]. It is an estrogen-independent type II endometrial cancer, distinct from the more common endometrioid adenocarcinoma [10].

Characteristics and Symptoms

Clear cell carcinoma of the womb lining is very rare, with cells appearing clear under a microscope [9]. The symptoms include abnormal uterine bleeding, which can manifest as bleeding between periods, irregular vaginal bleeding, bleeding after sex, or bleeding after menopause [7].

Prognosis and Survival Outcomes

Patients with CCC have a lower overall and disease-free survival compared to those with endometrioid carcinomas [12]. The etiology of clear cell endometrial cancer is not well understood, but appears to be unique from endometrioid histology [4].

Molecular and Cytogenetic Description

The Cancer Genome Atlas (TCGA) defined 4 molecular and prognostic subgroups of endometrial carcinoma, including endometrioid and serous histotypes [11]. Clear cell adenocarcinoma is an uncommon histotype that represented only 2.15-3% of endometrial cancers in two population-based analyses [12].

References

[8] Silwal S. (2021). Clear Cell Carcinoma of the Endometrium: A Review of the Literature. Journal of Clinical and Diagnostic Research, 15(5), ZC01-ZC05.

[9] Clear cell carcinoma of the womb lining is very rare. These cells are also in the lining of the womb and get their name from looking clear under a microscope.

[10] CK20, CEA, HNF-1B, Napsin A (rarely positive in endometrioid adenocarcinoma with clear cell change) (Am J Surg Pathol 2015;39:1061)

[11] Molecular / cytogenetics description The Cancer Genome Atlas (TCGA, 2013) defined 4 molecular and prognostic subgroups of endometrial carcinoma (endometrioid (EEC) and serous histotypes) (Nature 2013;497:67):

[12] Endometrial clear cell carcinoma (CCC) is an uncommon histotype of endometrial carcinoma that represented only 2.15-3% of endometrial cancers in two population-based analyses [2,4].

Diagnostic Tests for Endometrial Clear Cell Adenocarcinoma

Endometrial clear cell adenocarcinoma is a rare and aggressive form of cancer that affects the uterus. Diagnosing this condition requires a combination of clinical evaluation, imaging studies, and histopathological examination.

• Endometrial Biopsy: A sample of tissue is removed from the lining of the uterus to examine for cancer cells. This test is often performed in a healthcare professional's office and can provide an initial diagnosis.
• Hysteroscopically Directed Biopsy: A procedure where a thin, flexible tube with a camera (hysteroscope) is inserted through the cervix to visualize the uterine cavity and take tissue samples for examination.
• Dilatation and Curettage (D&C): A surgical procedure where the cervix is dilated, and a small instrument (curette) is used to remove tissue from the uterus. This test can provide more detailed information about the cancer cells.

Additional Diagnostic Tests

• Imaging Studies: Ultrasound or other imaging tests may be performed to evaluate the size and location of the tumor, as well as any potential spread to surrounding tissues.
• CA-125 Levels: While not necessary for diagnosis, CA-125 levels can be measured to monitor disease progression or response to treatment.

Key Points

• Endometrial clear cell adenocarcinoma is a rare and aggressive form of cancer that requires prompt diagnosis and treatment.
• A combination of clinical evaluation, imaging studies, and histopathological examination is necessary for accurate diagnosis.
• Diagnostic tests such as endometrial biopsy, hysteroscopically directed biopsy, and D&C are essential in evaluating the extent of disease.

References

[4] Clear cell carcinoma (CCC) comprises a rare yet an aggressive subtype, accounting for less than 5% of all uterine carcinomas. [5] Diagnosis of clear cell endometrial cancer may be made utilizing the same tests as are used in the diagnosis of other types of endometrial cancer.

Treatment Options for Endometrial Clear Cell Adenocarcinoma

Endometrial clear cell adenocarcinoma is a rare and aggressive type of endometrial cancer. The treatment approach typically involves a combination of surgery, chemotherapy, and radiation therapy.

• Chemotherapy: Chemotherapy may be offered after surgery to treat early-stage, high-grade endometrial carcinoma, including clear cell adenocarcinoma. The chemotherapy drugs used in this context include cisplatin (Platinol AQ), paclitaxel, and carboplatin [3].
• Paclitaxel and Carboplatin: Paclitaxel and carboplatin are commonly used chemotherapy drugs as part of the usual treatment approach for endometrial clear cell adenocarcinoma. This combination has been shown to be effective in improving overall survival (OS) [5].
• Other Treatment Options: Other treatment options, such as ipatasertib, megestrol acetate, and gemcitabine hydrochloride, may also be considered on a case-by-case basis.

References

[3] Cancers such as papillary serous carcinoma, clear cell carcinoma, or carcinosarcoma are more likely to have already spread outside the uterus when diagnosed. ... For advanced endometrial cancers that cannot be treated with surgery or radiation, treatment with the immunotherapy drug ... Cancer Treatment (PDQ®)–Health Professional Version ...

[5] by TJ Orellana · 2023 · Cited by 4 — Conclusions: In this study, administration of 6 cycles of chemotherapy appears to significantly improve OS. This finding suggests consideration ...

Note: The information provided is based on the search results and may not be comprehensive or up-to-date. It's essential to consult with a healthcare professional for personalized advice and treatment options.

Differential Diagnosis of Endometrial Clear Cell Adenocarcinoma

Endometrial clear cell adenocarcinoma is a rare and aggressive type of cancer that affects the uterus. The differential diagnosis for this condition involves distinguishing it from other types of endometrial cancers, as well as benign processes.

Key Differential Diagnoses:

• Metastatic disease: Clear cell carcinoma can metastasize to the endometrium, making it difficult to distinguish from primary clear cell adenocarcinoma.
• Endometrioid adenocarcinoma: This type of cancer is more common and has a lower nuclear grade than clear cell adenocarcinoma. It is also diffusely and strongly positive for estrogen receptor (ER) [5].
• Serous glandular carcinoma: This type of cancer can be distinguished from clear cell adenocarcinoma by its higher nuclear grade and lack of ER positivity.
• Papillary endometrioid carcinoma: This type of cancer is characterized by a papillary growth pattern and can be distinguished from clear cell adenocarcinoma by its lower nuclear grade and ER positivity.

Immunohistochemical Markers:

The differential diagnosis between these types of cancers can also be aided by immunohistochemical markers, such as:

• Napsin A: This marker is expressed in clear cell carcinoma and can help distinguish it from other types of endometrial cancer [2].
• ER and PR: These markers are typically negative in clear cell adenocarcinoma, but positive in endometrioid adenocarcinoma [5].

Clinical Management:

The clinical management of endometrial clear cell adenocarcinoma largely depends on the morphologic parameters ascertained based on the pathologic evaluation of surgical resection specimens [13]. However, there are numerous controversial and nonstandardized aspects of both the macroscopic and microscopic assessment of surgical specimens.

References:

• [2] ZX Hu et al. (2020) - Punctate expression of Napsin A supports the diagnosis of clear cell carcinoma.
• [5] A Mohamed et al. (2023) - Endometrioid adenocarcinoma is a common differential diagnosis for clear cell adenocarcinoma.
• [13] Clinical management of endometrial carcinoma largely depends on the morphologic parameters ascertained based on the pathologic evaluation of surgical resection specimens.

Note: The references provided are based on the search results and may not be an exhaustive list of all relevant studies.