What are the other Names for this Condition? (Also known as/Synonyms)

• Cervical Plexiform Neurofibroma
• Plexiform Neurofibroma of Cervix

What is Plexiform Neurofibroma of Uterine Cervix? (Definition/Background Information)

• A Plexiform Neurofibroma of Uterine Cervix is a very rare, benign tumor that is observed in the cervix. Neurofibromas are tumors that arises from the nerves underneath the skin surface, or subcutaneously
• The risk factors and cause of development of Plexiform Neurofibroma of Uterine Cervix is unknown. In some cases, the presence of multiple tumors may indicate the presence of neurofibromatosis type 1 (NF-1), while solitary tumors are not associated with this genetic disorder
• Plexiform Neurofibroma of Uterine Cervix may present with abnormal vaginal bleeding and pelvic pain. Large-sized tumors may cause urination difficulties
• A treatment of Plexiform Neurofibroma of Uterine Cervix may be undertaken, if they cause significant signs and symptoms. In such cases, a simple surgical excision and removal of the tumor is sufficient treatment
• The prognosis is excellent on removal of the Plexiform Neurofibroma of Uterine Cervix, since it is a benign tumor. However, the prognosis may also depend upon the presence of any associated NF-1 (if any present)

Who gets Plexiform Neurofibroma of Uterine Cervix? (Age and Sex Distribution)

• Plexiform Neurofibroma of Uterine Cervix may be observed in middle-aged women, although they may be seen in women of a wider age range
• The most common location for neurofibromas affecting the genital tract is the vulva (area around the external opening of the vagina); Cervical Plexiform Neurofibromas are extremely rare
• This condition is observed worldwide; there is no ethnic or racial predisposition

What are the Risk Factors for Plexiform Neurofibroma of Uterine Cervix? (Predisposing Factors)

• No definitive risk factors for Plexiform Neurofibroma of Uterine Cervix have been currently identified. A majority of the tumors may be solitary in nature and occur sporadically
• However, if more than one tumor is present, then an association with neurofibromatosis type 1 (NF-1) is observed. NF-1, or von Recklinghausen disease, is a genetic condition that is characterized by skin pigmentation and the formation of non-cancerous tumors that affect the central nervous system

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Plexiform Neurofibroma of Uterine Cervix? (Etiology)

• The exact cause and development of Plexiform Neurofibroma of Uterine Cervix is unknown
• The presence of multiple neurofibromas may indicate neurofibromatosis type 1, which is an autosomal dominant genetic disorder

What are the Signs and Symptoms of Plexiform Neurofibroma of Uterine Cervix?

In most cases, there are no significant signs and symptoms of Plexiform Neurofibroma of Uterine Cervix. However, in some cases, the following may be noted:

• The tumors are solid and firm to touch
• Pain in the pelvic region that may be chronic
• Abnormal and heavy bleeding during menstruation
• Some tumors may grow to large sizes and may cause a feeling of fullness in the abdomen; enlargement of lower abdomen
• Frequent urination due to compression/pressure of the tumor
• Lower back pain
• Pain during sexual intercourse

If multiple tumors are noted, then it may occur against a background of neurofibromatosis type 1. In such cases, the associated signs and symptoms of NF-1 may also be observed.

How is Plexiform Neurofibroma of Uterine Cervix Diagnosed?

A diagnosis of Plexiform Neurofibroma of Uterine Cervix may involve the following procedures and tests:

• Complete physical (pelvic) examination with thorough evaluation of medical history
• Ultrasound scan of the pelvis: It is a noninvasive procedure that uses high frequency sound waves to produce real-time images
• Abdominal and pelvic CT scan: It is a noninvasive procedure that provides more details of soft tissues, blood vessels, and internal organs
• Pelvic MRI scan: It is a noninvasive medical test that uses a powerful magnetic field to produce images of soft tissues, bones, organs, and all other internal structures of the abdomen and pelvis
• Hysteroscopy: This procedure involves placing a probe through the cervix to examine the cavity of the uterus. This exam is helpful in detecting submucosal leiomyomas
• Hysterosalpingography: It is usually performed in individuals with infertility. In this procedure, the structure of the uterus and fallopian tubes are studied by using a dye and X-ray images
• Needle biopsy of tumor: A needle biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a needle biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
• Open biopsy of tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
• Differential diagnosis, to eliminate other tumor types is considered, before arriving at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Plexiform Neurofibroma of Uterine Cervix?

No significant complications generally arise from Plexiform Neurofibroma of Uterine Cervix; however, in some cases the following may be noted:

• Stress and anxiety due to fear of cancer in the cervix
• Heavy and prolonged menstrual bleeding may result in anemia
• Fertility issues due to mechanical obstruction of the cervical opening, due to large-sized tumors
• Recurrence of the tumor due to its partial or incomplete surgical removal
• Post-surgical infection at the wound site is a potential complication
• Complications may arise due to neurofibromatosis type 1 (if present)

How is Plexiform Neurofibroma of Uterine Cervix Treated?

The treatment measures adopted for Plexiform Neurofibroma of Uterine Cervix include:

• If there are no symptoms, then the healthcare provider may advise a ‘wait and watch’ approach following a diagnosis of plexiform neurofibroma
• Pain medications, in case of tumors causing pain
• Treating iron-deficiency anemia, if any
• Surgical intervention with complete excision can result in a complete cure. It can also help reduce the chances of tumor recurrence
  • Myomectomy: Removal of the tumor/fibroid (also known as fibroidectomy)
  • Myolysis of the tumor: In this procedure, a needle is inserted into the tumor. After the insertion, the tumor is destroyed either by using an electric current, or by a freezing technique
  • Tumor embolization is a possible treatment option. Here the blood supply to the tumor is blocked resulting in tumor death
  • Radiofrequency ablation: In this technique, the tumors are destroyed using radio waves
• Radiation therapy and chemotherapy are not usually required
• Treatment of underlying neurofibromatosis type 1 (if present) may be necessary
• Post-operative care is important: Minimum activity level is to be ensured until the surgical wound heals
• Follow-up care with regular screening and check-ups are important

How can Plexiform Neurofibroma of Uterine Cervix be Prevented?

Current medical research has not established a definitive way to prevent the formation of Plexiform Neurofibroma of Uterine Cervix.

What is the Prognosis of Plexiform Neurofibroma of Uterine Cervix? (Outcomes/Resolutions)

• The prognosis of Plexiform Neurofibroma of Uterine Cervix is excellent on its complete excision and removal
• Since, these are benign tumors, the prognosis is excellent, even if no treatment is provided and only periodic observation is maintained
• The prognosis of neurofibromas associated with neurofibromatosis type 1, depends upon the severity of the signs and symptoms of the genetic disorder

Additional and Relevant Useful Information for Plexiform Neurofibroma of Uterine Cervix:

• Fibroid tumor removal (or myomectomy) is the surgical removal of fibroids from the uterus

The following link will help you understand fibroid tumor removal surgical procedure:

http://www.dovemed.com/common-procedures/procedures-surgical/fibroid-tumor-removal/

• Hysterectomy is a surgical procedure characterized by the surgical removal of the uterus

The following link will help you understand hysterectomy surgical procedure:

http://www.dovemed.com/common-procedures/procedures-surgical/hysterectomy-abdominal-with-salpingo-oophorectomy/