Small cell carcinoma of the cervix sccc was first described in 1957 and represents 1%3% of all cervical malignancies. For the prevention and early detection of cervical cancer. Small cell neuroendocrine carcinoma of the cervix is a rare disease, accounting for only up to 2 percent of all invasive cervical cancers. In this study, the authors report 25 chinese cases of sccc, with a particular focus on their clinical and pathological characteristics. Smallcell carcinoma of the cervix at 23 weeks gestation. Primary small cell carcinoma of the cervix sccc is recognised as a rare and aggressive malignant tumour with poor prognosis. Neuroendocrine tumors consist of a spectrum of malignancies that arise from the diffuse neuroendocrine cell system. Small cell carcinoma of the cervix of neuroendocrine origin causing obstructed labour perrin 1996 australian and new zealand journal of. Small and large cell cervical cancer md anderson cancer center.
The cervix is the tube that connects the uterus to the vagina. To date, no effective treatment protocol has been determined. In fact, small cell and large cell subtypes behave and are therefore treated similarly, and will be grouped together in the following statements. For small tumors of 4 centimeters or less, limited to the cervix, a radical hysterectomy and lymph node dissection are performed, followed by combined chemotherapy and radiation.
The incidence of bone metastasis of uterine cervical cancer is generally low, and in all cervical cancers, this incidence is only a few percent. Neuroendocrine small cell cervical carcinoma is an aggressive, but rare form of cervical cancer with an incidence of less than 3% of all cervical cancers. Dec 28, 2018 small cell cancer scc of the female genital tract fgt occurs most frequently in the cervix, but can also occur in the endometrium, ovary, fallopian tube, vagina, and vulva. Small cell carcinoma can consist of heterologous tumor cells because some cells are of epithelial origin and others are of neuroendocrine origin. Casereport small cell neuroendocrine carcinoma of the cervix in pregnancy. The clinical trials on this list are for small cell lung cancer treatment. It is an extremely aggressive cancer with a biological behavior.
Lymph nodes are beansized collections of immune system cells, to which cancers often spread first. Small cell cervical carcinoma sccc is a neuroendocrine tumor with a poor prognosis. However, rarer subtypes, such as small cell carcinoma hold a poorer prognosis, as in nonpregnant women. M indicates if the cancer has spread metastasized to distant sites, such as other organs or lymph nodes that are not near the cervix. Small cell carcinoma of the uterine cervix in pregnancy. Cytologically similar to counterparts in lung and other sites small tumor cells arranged as single cells or in groups and cords with barely visible cytoplasm, darkly staining nuclei with finely stippled chromatin with characteristic molding and inconspicuous nucleoli textacta cytol 1998. We treated a patient with progressive small cell carcinoma of the uterine cervix that metastasized to the cervical spine. Clinical trials look at new ways to prevent, detect, or treat disease.
Unlike squamous cell and adenocarcinoma, scnec is more likely to have lymphovascular space invasion and lymph node involvement at the time of diagnosis 1, 2. It is the 2nd or 3rd most common cancer in women worldwide, and over half a million cases are diagnosed each year. Women with earlystage clear cell carcinoma of cervix have better outcomes compared to those with more advanced cancer conditions almost all women diagnosed and treated at stage 0 cervical cancer survive for 5 years postdiagnosis 5year survival rate of 99100%. Most gynecologic oncology centers have little experience with this tumor, and only small series have been published.
A clinicopathological aspect of primary smallcell carcinoma. Neuroendocrine cancer of the uterine cervix is a rare and aggressive disease. Knowing the cancer s stage helps your doctor decide what treatment is best for you. The prognosis of women with necc is poor and there is no standardized therapy for this type of malignancy based on controlled trials. Stage 1 is the earliest stage when the cancer is still within the cervix. Cervix small cell neuroendocrine undifferentiated carcinoma. Prognostic factors in neuroendocrine small cell cervical. May 28, 2011 hi my name is pearlie and i have been diagnosed with small cell neuroendocrine carcinoma of the cerviz on january 4, 2011. In a series from the surveillance, epidemiology and end results seer database, the mean annual incidence in the united states from 1977 to 2003 was 0. Patients tend to develop distant metastasis early and thus are potential candidates for systemic therapy. Case report simultaneous small cell carcinoma of the. Highly aggressive smallcell neuroendocrine carcinoma cervix. Small cell carcinomas small ccs of the uterine cervix are rare and highly malignant neoplasms.
Sccc is a highgrade neuroendocrine tumor that shares numerous characteristics of small cell lung carcinoma sclc and has often been considered an extrapulmonary small cell. Objective small cell carcinoma of the uterine cervix is associated with a poor prognosis with a median overall survival that is quite low. These cancers occur in the hormoneproducing cells of the bodys neuroendocrine system, which is composed of cells that are a cross between. Small cell carcinoma is one of the most aggressive, poorly differentiated, and highly malignant neuroendocrine tumors and includes small cell lung cancer and. This is the first case in which the tumour is reported as causing obstructed labour. All trials on the list are supported by nci ncis basic information about clinical trials explains the types and phases of trials and how they are carried out.
The occurrence of cervical cancer during pregnancy is extremely rare, particularly small cell carcinoma. Small cell neuroendocrine carcinoma of the uterine cervix scnec is a rare malignancy, representing less than 5% of all cases of cervical cancer. Pdf diagnosis of small cell neuroendocrine carcinoma of. Cervical cancer stages how to stage cervical cancer. Small cell cervical cancer information jos cervical cancer. Small cell carcinoma of the uterine cervix is a rare histological entity that has a poor prognosis. Small cell cervical carcinoma sccc is a neuroendocrine tumor with great aggravation.
Small cell neuroendocrine histology and gastric type adenocarcinoma also. Although sccc has a worse prognosis than other histological types of uterine cervical cancer such as squamous cell carcinoma or adenocarcinoma, standard therapy for sccc remains to be. Neuroendocrine small cell carcinoma of the uterine cervix nesccc is an entity with very aggressive behaviour. Cervical cancer is a malignancy of the cervix, which is the lower part of the uteruswomb. Was yours just called small cell cervical cancer or did it go by another name. Three of the 10 scnecs were pure, whereas the other seven were mixed with adenocarcinoma andor squamous cell carcinoma or cervical intraepithelial neoplasia. It is characterized by frequent and early nodal and distant metastases, resulting in a relatively poor prognosis.
Small cell cervical cancer can also be found in combination with other more common forms of cervical cancer, such as squamous cell carcinoma and adenocarcinoma. Updates and management algorithm for neuroendocrine tumors of. Five of the 10 tumors were composed mainly of small oat cells and 5 of mainly larger intermediate cells. I am curious because i recently lost my 29 year old daughter to something similar. Small cell carcinoma of the cervix sccc is a rare neuroendocrine subtype of cervical cancer comprising 1% to 5% of all cervical cancer cases. Nivolumab is an immune checkpoint inhibitor specific for the programmed death 1 pd1 receptor that has led to clinical responses in many.
The classification of histological subtypes is based on the appearance of cells when observed under a microscope by a pathologist. Like smallcell cancer in the lungs, the lymph nodes play a major role in spreading the cancer throughout the body. A populationbased study evaluating standardized provincial treatment protocols. Thirtyfour patients with sccc were seen and treated at the british columbia cancer agency between may 1988 and november 2002. Clinicopathological aspects of small cell neuroendocrine. But small cell cervical cancer is faster growing and more aggressive than the other types of cervical cancer 3. The majority of both subtypes showed an insular pattern. For small cell lung cancer, a 2stage system is most often used. Histologically, scnec is indistinguishable from small cell carcinoma in other sites.
The optimal initial therapeutic approach to this rare disease has not yet been clearly defined. Simultaneous small cell carcinoma of the cervix and adenocarcinoma of the ovary. There are several differences between neuroendocrine and other types of cervical cancer, in prognosis, diagnosis and treatment. We performed a systematic literature search of the databases pubmed and cochrane central register of controlled trials to identify clinical trials describing the management and. It spreads much more quickly than non small cell lung cancer. Small cell lung cancer sclc is a fastgrowing type of lung cancer. Even secondhand tobacco smoke is a risk factor for lung cancer. Small cell lung cancer is slightly more common in men than women. Small cell neuroendocrine carcinoma of the cervix scnec is a rare and aggressive histology. We report the case of a patient with small cell carcinoma of the uterine cervix who underwent a radical hysterectomy during pregnancy. Between 1982 and 1993, eight cases of this disease were diagnosed at the queensland centre for gynaecological cancer among 1586 cervical cancers. The nccn guidelines panel for cervical cancer screening endorses the following guidelines for the prevention and early detection of cervical cancer.
Surgery, radiotherapy, and chemotherapy have been used either alone or in combination. Small cell carcinoma of the uterine cervix sccc is a rare subtype of cervical cancer with an aggressive behavior. Compared to non small cell carcinoma, small cell carcinoma has a shorter doubling time, higher growth fraction, and earlier development of metastases. Small cell carcinoma of the cervix at 32week gestation. As an aggressive tumor, sccc usually leads to a fatal clinical outcome. Nov 10, 2000 small cell carcinomas smccs, which form part of the spectrum of neuroendocrine tumors of the uterine cervix and account for approximately 5% of invasive cervical carcinomas, 1 have distinct clinical and histologic features. Patients with small cell neuroendocrine cervical cancer have a poor prognosis. Hi my name is pearlie and i have been diagnosed with small cell neuroendocrine carcinoma of the cerviz on january 4, 2011. Small and largecell neuroendocrine tumors of the cervix are exceedingly rare and exceedingly aggressive. Small cell cancer of the cervix is a very rare type of cervical cancer. However, smallcell lung cancer is more strongly linked to smoking than non small cell lung cancer. Vaginal bleeding, postcoital spotting, lower abdominal pain j clin diagn res 2014. Neuroendocrine small cell cervical carcinoma is an aggressive, but rare form of cervical cancer with an in cidence of less than 3% of all cervical cancers. Small cell neuroendocrine carcinoma, uterine cervix, treatment, prognosis, multimodality introduction cervical malignant tumor is one of the most common carcinomas in the female genital tract seriously endangers female health.
These are cancers that develop in the hormone producing cells of the neuroendocrine system. Most women diagnosed with small cell cancer of the cervix have early stage disease. Methods a total of 93 patients were involved in this retrospective study. Treatment clinical trials for small cell lung cancer. Small cell carcinoma of cervix smcc is a relatively rare subtype in the spectrum of cervical malignancies tavassoli and devilee, 2003. Metastatic small cell neuroendocrine carcinoma of the. Clinical trials are research studies that involve people. Jan 17, 2003 unlike patients with small cell lung carcinoma, women with small cell cervical carcinoma appear to have a significantly better cure rate, particularly those patients with early.
It was called small cell neuroendocrine carcinoma of the cervix that started with a tumor in her cervix. Aims small cell carcinoma is a variant of poorly differentiated neuroendocrine carcinoma. It accounts for approximately 25% of all cervical malignancies miller et al. The aim of this study was to determine the clinicopathologic characteristics that have an impact on survival in patients with small cell carcinoma of the uterine cervix. The nccn guidelines panel for cervical cancer screening endorses the following guidelines. Small cell neuroendocrine carcinomas of the uterine cervix. Smallcell carcinoma of the uterine cervix discovered with. Small cell carcinoma is a type of highly malignant cancer that most commonly arises within the lung, although it can occasionally arise in other body sites, such as the cervix, prostate, and gastrointestinal tract. Small cell neuroendocrine carcinoma of the uterine cervix. Thus, it remains to be determined whether this approach will have any survival. Small cell carcinoma of the cervix of neuroendocrine origin. Because nec of the cervix is uncommon, the etiology and predisposing risk factors are poorly understood. Metastatic small cell carcinoma to the masseter muscle.
Small cell neuroendocrine undifferentiated carcinoma. Smallcell cancer scc of the female genital tract fgt occurs most frequently in the cervix, but can also occur in the endometrium, ovary, fallopian tube, vagina, and vulva. It is a type of neuroendocrine tumor and is similar to pulmonary small cell carcinoma contains neuroendocrine granules. Small cell carcinoma of the cervix is a rare, poorly differentiated neuroendocrine tumor of cervix showing a high propensity to spread to lymph nodes as well as distant metastasis.
Small cell carcinoma is classified as a neuroendocrine tumor in the currently used world health organization histological classification of tumors of the uterine cervix 2003. Almost all cases of sclc are due to cigarette smoking. A 33yearold japanese woman with genital bleeding was referred at 15 weeks gestation. The patient, a 73yearold woman, presented with the symptom of numbness in her limbs. They are aggressive forms of a larger group of tumors called neuroendocrine cancers. Conclusion small cell carcinoma of cervix is a rare malignancy. Small cell carcinoma of the cervix sccc is a rare neuroendocrine malignancy comprising less than 35% of cervical carcinomas 1. Neuroendocrine carcinoma of the cervix necc is an aggressive histological variant of cervical cancer accounting for about 11. Pdf simultaneous small cell carcinoma of the cervix and.
Small cell carcinoma of the uterine cervix is a rare neuroendo crine tumor of the uterus about which little is known. Summary small cell carcinoma of the cervix complicating pregnancy is rare, only 4 cases having been reported. Small cell neuroendocrine carcinomas occur rarely in the cervix, vulva and vagina. Small cell carcinoma of the uterine cervix accounts for % of all cervix cancers. Metastatic small cell neuroendocrine carcinoma of the cervix treated with the pd1 inhibitor, nivolumab. Small cell neuroendocrine carcinoma, uterine cervix, treatment, prognosis, multimodality. Small cell cervical cancer sccc and large cell cervical cancer lccc make up a rare subtype of cervical cancer.
Pap smear screening for small cell carcinoma of the. Because of the high rates of nodal involvement even with earlystage disease, multimodality treatment with radiotherapy and. Clear cell carcinoma of cervix is a rare histological variant of cervical adenocarcinoma. Around 3 out of 100 cervical cancers 3% are this type. American cancer society, american society for colposcopy and cervical pathology, and american society for clinical pathology screening guidelines for the prevention and early detection of cervical cancer. Small cell carcinoma of the cervix is an uncommon aggressive variety of cervical cancer. Original article small cell neuroendocrine carcinoma of. N indicates any cancer spread to lymph nodes near the cervix. Small cell neuroendocrine carcinoma of the cervix cervical. Because small and large cell cervical cancers are aggressive, treatment usually involves a combination of surgery, chemotherapy andor radiation. Small cell carcinoma of the cervix is a rare and aggressive tumor.
Facts about neuroendocrine carcinoma of the cervix. Limited stage means that the cancer is only in one lung and maybe in lymph nodes on the same side of the chest. It resembles smallcell cancer of the lungs and accounts for less than 3% of all cervical cancers. When generating a differential diagnosis of masses in the head and neck, one should include metastatic disease from primary tumors below the clavicle. Small and large cell cervical cancer md anderson cancer. The tumor was extremely cellular, consisting of small. To determine the efficacy and toxicity of a combinedmodality regimen of irradiation with platinumbased combination chemotherapy in small cell carcinoma of the cervix sccc. Small cell carcinoma of the cervix was once considered to be a rare, highly aggressive subtype of squamous cell carcinoma, but evidence has accumulated that most of the tumours express one or more markers of neuroendocrine differentiation. Small cell carcinoma of the uterine cervix zhou 1998. The inner part of this tube is lined by tall cells that secrete mucus. Neuroendocrine carcinoma of the cervix necc is a rare variant of cervical cancer. Small cell nec is the most common type of necc, whereas welldifferentiated nets, especially net g1 typical carcinoid and net g2 atypical.
Herein, we describe the first cytological case of adenocarcinoma in situ ais admixed with scnec. I just turned 32 in march and i started going to the doctor in august 2010, took me 6 months to find out what was going on with me. Small cell neuroendocrine carcinoma scnc has been described in many extrapulmonary sites including breast, larynx, gastrointestinal tract, prostate, bladder, ovary, and cervix. For newly diagnosed patients, we employ a multimodality approach utilizing surgery, radiation, and chemotherapy for those with earlystage disease, and radiation and chemotherapy for those with advancedstage disease.
Rarely, nens may also occur in other organs such as the female genital tract. Small cell carcinoma of the uterine cervix smcc, which comprises about 2% to 5% of most cervical malignancies, tends to be progressive at an early stage, with metastasis to distant organs and lymph nodes, and is known to have a worse prognosis than squamous cell carcinoma or adenocarcinoma of the uterine cervix. Metastatic small cell neuroendocrine carcinoma of the cervix. Stage 4 means the cancer has spread to the bladder or back passage rectum, or further away such as to the liver or lungs. Small cell carcinoma of the cervix of neuroendocrine.
Small cell lung cancers are staged as limited stage and extensive stage. Compared to non small cell carcinoma, small cell carcinoma has a shorter doubling time, higher growth fraction, and earlier. The most common of these is small cell cancer of the cervix, the mean annual incidence of which is 0. Their course is frequently characterized by the development of widespread. Small cell carcinoma of the uterine cervix during pregnancy. Primary cervical small cell neuroendocrine carcinoma scnec is a rare and aggressive tumor. Neuroendocrine small cell carcinoma of the uterine cervix. Small cell and large cell cervical cancer treatment. Small cell cancer scc of the female genital tract fgt occurs most frequently in the cervix, but can also occur in the endometrium, ovary, fallopian tube, vagina, and vulva. Scc begins in the inner part of the cervix and is very hard to diagnose. Small cell carcinoma of the cervix radiology reference. Hoskins pj1, swenerton kd, pike ja, lim p, aquinoparsons c, wong f, lee n.
The nccn guidelines for cervical cancer include the management of squamous cell carcinoma. Two protocols were used, smcc may 1988 to december 1995 and. Small cell neuroendocrine carcinoma of the cervix in. Small cell cancer of the cervix cancer research uk.