Giant Angiomatoid Cellular Blue Nevus of Vagina

Giant Angiomatoid Cellular Blue Nevus of Vagina

Article
Sexual Health
Women's Health
+3
Contributed byKrish Tangella MD, MBASep 16, 2018

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

  • Angiomatoid Giant Cellular Blue Naevus of Vaginal Wall
  • Giant Angiomatoid Cellular Blue Naevus of Vagina
  • Vaginal Angiomatoid Giant Cellular Blue Nevus

What is Giant Angiomatoid Cellular Blue Nevus of Vagina? (Definition/Background Information)

  • A nevus is a mole on the skin that can occur on any part of the body. A blue nevus is a benign proliferation of melanocytes (cell that make melanin pigment) in the dermis or skin
  • A rare variant of blue nevus of vagina called Giant Angiomatoid Cellular Blue Nevus of Vagina has been described in the medical literature. In some cases, the tumor is associated with pregnancy and may present as a slow-growing soft tissue mass
  • The tumor is described as ‘angiomatoid’, since the nevus cells are mixed with numerous blood vessels. The angiomatoid giant cellular blue nevus has also been described in non-genital areas too, such as in the chest wall
  • There are no clearly established risk factors for Giant Angiomatoid Cellular Blue Nevus of Vagina and the cause of tumor formation is unknown. The tumor is extremely rare and very few cases have been reported
  • Giant Angiomatoid Cellular Blue Nevi of Vagina are generally asymptomatic in nature and no significant signs and symptoms or complications are present. However, they may grow to larger sizes during pregnancy and result in associated complications
  • The treatment of Giant Angiomatoid Cellular Blue Nevus of Vagina may be considered on a case-by-case basis and surgery may be considered to remove the tumor. The prognosis is generally good with appropriate treatment, since these tumors are benign

Who gets Giant Angiomatoid Cellular Blue Nevus of Vagina? (Age and Sex Distribution)

  • Giant Angiomatoid Cellular Blue Nevus of Vagina is an extremely rare tumor that is sometimes seen in pregnant women
  • All racial and ethnic groups may be at risk for the tumor

What are the Risk Factors for Giant Angiomatoid Cellular Blue Nevus of Vagina? (Predisposing Factors)

  • No specific risk factors have been identified for Giant Angiomatoid Cellular Blue Nevus of Vagina
  • However, the tumor is known to be associated with pregnancy, in some cases

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 Giant Angiomatoid Cellular Blue Nevus of Vagina? (Etiology)

  • The cause of Giant Angiomatoid Cellular Blue Nevus of Vagina formation is unknown. It is described as a variant of vaginal blue nevus
  • Blue nevus is believed to form from abnormally located melanocytes (or pigmented cells)

What are the Signs and Symptoms of Giant Angiomatoid Cellular Blue Nevus of Vagina?

Giant Angiomatoid Cellular Blue Nevus of Vagina may not present any signs and symptoms, when the blue nevus is small. The general features of the condition include:

  • The giant angiomatoid cellular blue nevus may be as large as 6 cm
  • The tumor is present on the vaginal wall and large sizes may obstruct the uterine cervix
  • During pregnancy, an increase in size of the tumor is noted. The tumor can also form into a cyst-like mass
  • In general, a blue nevus is usually slow-growing and pigmented. The color may vary from blue to blue black. The moles are flattened lesions with a maximum size of 2 cm. The borders of the nevus may not be well-defined

Note: Even though an increase in tumor size is noted during pregnancy, it is not conclusively proven that the growth during pregnancy is because of hormonal influence.

How is Giant Angiomatoid Cellular Blue Nevus of Vagina Diagnosed?

In most cases, a Giant Angiomatoid Cellular Blue Nevus of Vagina may be diagnosed incidentally. The following diagnostic tools may be employed by the healthcare provider:

  • Complete physical (pelvic) examination with evaluation of medical history
  • A colposcopic examination may show a deeply pigmented area on the surface of the vaginal wall

If the tumor is large in size, then the following diagnostic modalities may be considered:

  • Ultrasound scan of the abdomen
  • Transvaginal ultrasound: Transvaginal ultrasound inserts an ultrasound probe into the vagina designed to take pictures of the insides of the uterus
  • CT or CAT scan with contrast of the abdomen and pelvis may show a well-defined mass. This radiological procedure creates detailed 3-dimensional images of structures inside the body
  • MRI scans of the abdomen and pelvis: Magnetic resonance imaging (MRI) uses a magnetic field to create high-quality pictures of certain parts of the body, such as tissues, muscles, nerves, and bones. These high-quality pictures may reveal the presence of the tumor

Note:

  • Ultrasound scans are typically performed during pregnancy, but radiological imaging studies, such as CT scans and MRI scans, are not recommended. However, if the healthcare provider assesses that the benefits of a CT or MRI scan outweighs the risk for pregnancy, such imaging procedures may be considered
  • In majority of the cases, no biopsy is necessary. But they may be performed to rule out other conditions presenting similar signs and symptoms, such as a melanoma

Although the above modalities can be used to make an initial diagnosis, a tissue biopsy of the tumor is necessary to make a definitive diagnosis to begin treatment. The tissue for diagnosis can be procured in multiple different ways which include:

  • Fine needle aspiration (FNA) biopsy of the tumor: A FNA biopsy may not be helpful, since the tumor is rare; also one may not be able to visualize the different morphological areas of the tumor. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
  • Core biopsy of the tumor
  • Open biopsy of the tumor

Tissue biopsy:

  • A tissue biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist 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. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the 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 Giant Angiomatoid Cellular Blue Nevus of Vagina?

There are frequently no complications that arise from a Giant Angiomatoid Cellular Blue Nevus of Vagina. However, the following may be noted in some cases:

  • Emotional stress and concern due to the presence of a vaginal tumor
  • Since the tumor is vascular, it can result in excess bleeding on ulceration or when subjected to trauma
  • The presence of a large tumor may necessitate a cesarean section (c-section) delivery of the baby

Very rarely, blue nevus may be seen along with malignant melanoma, which may need to be investigated by a healthcare provider. However, typically a blue nevus does not transform into a melanoma.

How is Giant Angiomatoid Cellular Blue Nevus of Vagina Treated?

The following treatment measures for Vaginal Angiomatoid Giant Cellular Blue Nevus may be considered:

  • Surgical excision and removal of the tumor/mole may be undertaken using the following options:
    • Myolysis of 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
  • In pregnant women the following considerations may be taken:
    • Once the condition is diagnosed, the pregnant mother is closely monitored
    • The healthcare provider may recommend an increased frequency of prenatal appointments to monitor progress of the baby’s growth
    • A cesarean section (C-section) delivery of the baby may be necessary, in some cases (if the tumor is large). The tumor mass can also be surgically removed during the C-section
  • Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
  • A complete excision of the tumor can result in a cure

How can Giant Angiomatoid Cellular Blue Nevus of Vagina be Prevented?

Current medical research has not established a method of preventing the occurrence of Giant Angiomatoid Cellular Blue Nevus of Vagina.

What is the Prognosis of Giant Angiomatoid Cellular Blue Nevus of Vagina? (Outcomes/Resolutions)

  • In a majority of cases, the prognosis of Giant Angiomatoid Cellular Blue Nevus of Vagina is excellent, since it is a benign tumor
  • The tumor is vascular; hence, it can bleed excessively if the tumor is ulcerated or traumatized. An excessive loss of blood can adversely affect the prognosis

Additional and Relevant Useful Information for Giant Angiomatoid Cellular Blue Nevus of Vagina:

  • A blue nevus is a benign proliferation of melanocytes in the dermis/skin

The following link can help you understand blue nevus:

http://www.dovemed.com/diseases-conditions/blue-nevus/

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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