Cervical cancer forms in the tissues of the cervix. It is usually slow-growing and caused by the human papillomavirus (HPV), but it rarely produces symptoms. Approximately 100 of the cases diagnosed in the United States each year are small cell cervical cancer (SCCC), an aggressive variant.
Is SCCC rare?
Despite the rarity of the disease, Michael Frumovitz, M.D.
, associate professor in the Department of Gynecologic Oncology and Reproductive Medicine
at MD Anderson, has worked to recruit patients with SCCC to participate in research at the institution.
He is also involved in fundraising efforts for the cause and plans to use the funding to help build a worldwide tumor registry to make patient information more readily accessible to all doctors interested in studying SCCC.What is small cell cervical cancer?
Small cell cervical cancer is a rare subtype of cervical cancer. It is a high-grade variant of a larger group of tumors called neuroendocrine cancers. These are cancers that form in the hormone-producing cells of the body's neuroendocrine system, which is composed of cells that are a cross between traditional endocrine cells (or hormone-producing cells) and nerve cells.
In the United States, there will be about 11,000 new cases of cervical cancer this year. A rough estimate is that only about 100 of these will be small cell. Most women with cervical cancer will have either squamous cancer (80% to 85%) or adenocarcinoma (15% to 20%).
These two subtypes are HPV-related. However, for neuroendocrine cervical tumors, there is no known HPV link.
In the cervix, small cell is the most common type of neuroendocrine tumor, but it still accounts for less than 1% of all cervical cancers.
What causes SCCC?
Because this tumor is so rare, we have very little understanding of the cause. We do not know if it is HPV-related (likely not) or if other factors are in play.What are the symptoms?
While many people with SCCC experience no warning signs, some symptoms may include:
How is SCCC diagnosed?
- Vaginal bleeding
- Vaginal discharge
- Postcoital bleeding
Many tumors are found on abnormal Pap smears, but many women have normal Pap smears up until the tumor is found. Pap smears are designed to detect dysplasia or premalignant disease.
As small cell does not have a known premalignant state, Pap tests often do not detect them. Most cases of SCCC are diagnosed because of symptoms, or a gynecologist notices an abnormal-appearing cervix on an exam and biopsies it.
What are the main treatments for SCCC?
Because this cancer is so aggressive, we have adopted an equally aggressive treatment regimen. For small tumors limited to the cervix (less than 4 cm), we typically perform a radical hysterectomy and lymph node dissection followed by combined chemotherapy
, and possibly more chemotherapy. For larger tumors or metastatic disease, we have been using combined chemotherapy and radiation, plus additional chemotherapy.
The chemotherapy we typically use is cisplatinum and etoposide. This regimen is based upon data gained through studying neuroendocrine lung tumors because no studies have been done in neuroendocrine cervical tumors. Only small case studies are covered in the literature.What research, if any, related to this disease is being conducted at MD Anderson?
As for research
, we were the only institution to ever open a prospective Phase II study with weekly doses of Taxol® and Avastin® for these patients. Due to the rarity of the disease, we were only able to accrue three patients onto the study in 13 months, so it closed.
This amazing group of women with small cell and large cell disease, their friends and families have found one another on Facebook. They offer continuous support and camaraderie to one another.
They have also begun to raise funds for research into this rare disease (more than $30,000 to date). When we have enough funding, our plan is to start a worldwide tumor registry for SCCC to see if we can collect enough data on treatments and outcomes to look for what might, and what might not be, at work in this disease.Update: The Neuroendocrine Cervical Tumor Registry (NeCTuR) is open for enrollment. Please email email@example.com for more information.Related storyWoman Beats Rare Form of Cervical Cancer With Help of Faith and Family