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Primary Vaginal Adenocarcinoma - An Uncommon Tumor of the Female Genital System

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Primary vaginal adenocarcinoma is an uncommon cancer, making up one percent to two percent of all gynecologic cancers. Continue reading to know more about it.

Medically reviewed byDr. Rajesh Gulati

Published At June 25, 2024
Reviewed AtJune 25, 2024

What Is Adenocarcinoma?

Adenocarcinoma develops in glandular epithelial cells that produce mucus, digestive juices, and other fluids. Adenocarcinoma can spread to other areas of the body, called metastatic adenocarcinoma. A variety of environmental and lifestyle risk factors have been linked to the development of cancer. Different locations have different carcinogens and risk factors. Tobacco smoking appears to have a significant impact on most of them.

The clinical appearance of patients with adenocarcinoma varies depending on the tumor's initial location of involvement and the extent of other organ involvement. Obtaining the patient's family history is important because some malignancies are inherited. Patients may be asymptomatic or exhibit nonspecific symptoms, such as unintentional weight loss, along with additional symptoms based on the organ involved with metastasis. The bones, lungs, liver, and lymphatic systems are the most often affected locations, accounting for roughly 60 % of all cases.

What Is Vaginal Adenocarcinoma?

Vaginal cancer or carcinoma is sporadic, accounting for approximately one to two percent of all gynecological malignancies. They develop as primary squamous cell malignancies or as a result of cervical or vulval expansion. Primary mucinous vaginal adenocarcinoma of the intestinal type is a very rare malignancy with uncertain histogenesis that presents a diagnostic challenge for clinicians and histopathologists.

Vaginal cancer is the development of cells that begin in the vagina. The cells multiply rapidly, allowing them to penetrate and destroy healthy human tissue. The vagina is a component of the female reproductive system. It is a muscular tube that links the uterus to the external genitalia. The vagina can also be referred to as the birth canal. Cancer that originates in the vagina is uncommon. The vast majority of vaginal cancers begin elsewhere and spread to the vagina.

Vaginal cancer might not show any signs at first. As it progresses, vaginal cancer can cause signs and symptoms like:

  • Unusual vaginal bleeding, such as after menopause or after sexual activity.

  • Vaginal discharge.

  • A bump or lump in the vagina.

  • Urination is painful.

  • Urinate frequently.

  • Constipation.

  • Pelvic discomfort.

Intestinal-type adenocarcinoma of the vagina remains a problem for histopathologists since the tumor appearance and immunohistochemical staining closely match those of gastrointestinal cancer. Primary vaginal adenocarcinoma of the intestinal type should be thoroughly investigated to rule out a primary adenocarcinoma of another region, such as the rectum, colon, breasts, ovary, uterus, or cervix. Vaginal cancer develops when cells in the vagina change their DNA (deoxyribonucleic acid).

A cell's DNA stores the instructions that teach it what to do. In healthy cells, DNA directs growth and multiplication at a predetermined rate. The instructions command the cells to die at a specific moment. In cancer cells, DNA mutations provide distinct instructions. The modifications instruct cancer cells to produce many more cells quickly. Cancer cells can live even when healthy cells perish, resulting in an abnormally large number of cells.

The primary adenocarcinoma of the vagina accounts for 8 % to 10 % of cases. Herbst et al.44 reported a link between diethylstilbestrol (DES) and the development of clear cell adenocarcinoma in females who had exposure through the uterus during the first 16 weeks of pregnancy. Most of these instances were diagnosed between the ages of 14 and 22. A 2017 retrospective study of 420 cases indicated that most were in younger women, with a modest second peak at 42 years. Intrauterine DES has been prohibited since the 1970s, and it is believed that clear cell adenocarcinomas caused by intrauterine DES exposure will diminish in the coming decades.

What Are the Different Types of Vaginal Cancer?

Vaginal cancer is a very rare malignancy in which cells in the vagina suffer aberrant alterations and divide rapidly. There are various forms of vaginal cancer, which are classified according to the type of cell in which the cancer develops.

They are:

  • Squamous Cell Carcinoma: Squamous cell cancer develops in the flat cells located in the vagina, known as squamous cells. Squamous cell carcinoma is among the most prevalent kinds of vaginal cancer. It accounts for roughly 90 percent of all cases.

  • Melanoma: Melanoma develops in the cells that give the vagina its color (melanocytes). Vaginal melanomas are exceptionally rare.

  • Sarcoma: Sarcoma originates in the connective and muscle tissue that comprise the vaginal wall. Vaginal sarcomas, like vaginal melanomas, are exceedingly rare. There are several forms of sarcoma. The most frequent type is rhabdomyosarcoma, which primarily affects young people. The most common age group for leiomyosarcoma is 50 and older.

  • Vaginal Adenocarcinoma: Vaginal adenocarcinoma starts in gland cells in the vagina. It is more common among adults over the age of 50. The exception is clear cell adenocarcinoma, which frequently affects people under the age of 50 who were exposed to the chemical diethylstilbestrol (DES) when growing in the uterus.

What Are the Pathological and Radiological Findings of Vaginal Adenocarcinoma?

Lesions typically develop on the posterior wall of the upper portion of the vagina. The common patterns of illness include an ulcerating or fungating tumor or an annular constricting lesion.

Imaging characteristics will vary depending on the histological type of cancer.

General signal characteristics have been reported:

  • T1: Is isointense to muscle.

  • T2: Higher signal intensity relative to muscle.

Reported signal features for squamous cell carcinoma include the following:

  • T1: Indicates low signal intensity.

  • T2: Indicates intermediate signal intensity.

What Is the Differential Diagnosis for Vaginal Adenocarcinoma?

Malignant vaginal involvement from metastatic spread is far more common, and, except for a few instances of metastases from extragenital malignancies, the most prevalent form of metastatic disease involves direct local invasion through the female genital system. According to some studies, primary vaginal carcinoma should only be identified when other gynecologic cancers have been ruled out.

What Is the Treatment for Vaginal Adenocarcinoma?

The prognosis varies substantially depending on the stage. Cancer in situ and extremely early-stage invasive cancer are frequently treated with surgery. However, radiation therapy is the conventional treatment for women with vaginal cancer. Advanced stages are commonly treated with chemotherapy and radiotherapy (such as Cisplatin).

Conclusion:

These tumors are uncommon. Thus, therapy depends on reports of cases and series compiled throughout time. There is no level 1 evidence to suggest the optimal therapy alternatives. It is advised that a multidisciplinary team be included in treatment decisions, mainly when dealing with children and adolescents. Ideally, these patients should be directed to centers of excellence with prior experience in similar circumstances.

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