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Angiomyolipoma of Nasal Cavity

Articleangiomyolipomanasalcavity
Ear, Nose, & Throat (ENT)
Diseases & Conditions
+2
Contributed byKrish Tangella MD, MBAMay 24, 2021

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

  • AML of Nasal Cavity
  • Nasal Angiomyolipoma
  • Nasal Cavity Angiomyolipoma

What is Angiomyolipoma of Nasal Cavity? (Definition/Background Information)

  • An angiomyolipoma (AML) is a benign tumor that is a mixture of blood vessels (angio-), smooth muscles (myo-), and fat (or lipoma). The tumor is seen among a wide age range of adults and can occur at various locations in the body. But, the most common location is the kidney (renal AML)
  • Angiomyolipoma of Nasal Cavity is a very uncommon extrarenal AML that may be present in the nasal cavity in adult men and women. These tumors are termed as mucocutaneous angiomyolipomas
  • Angiomyolipoma can be associated with a syndrome (tuberous sclerosis) in some cases, termed syndromic angiomyolipoma; or in a majority of cases, it may not be associated with a genetic disorder, when it is termed non-syndromic angiomyolipoma. Angiomyolipoma of Nasal Cavity may be associated with tuberous sclerosis
  • The signs and symptoms depend upon the size of the tumors. Large tumors may appear as a polypoid nasal mass. It can cause bleeding from the nose, nasal congestion, and even snoring
  • Typically, a surgical excision and removal of Angiomyolipoma of Nasal Cavity may be undertaken. Recently, newer therapy involving FDA-approved medications may also be considered
  • The prognosis of Angiomyolipoma of Nasal Cavity is generally excellent with its complete removal, since it is a benign tumor. However, the prognosis also depends upon a set of factors that includes the severity of the signs and symptoms, age and overall health of the individual, response to treatment, and its association with tuberous sclerosis

Who gets Angiomyolipoma of Nasal Cavity? (Age and Sex Distribution)

No specific demographics for Angiomyolipoma of Nasal Cavity are evident, since it is an extremely rare tumor. On examination of the few cases (around 10), the tumor is seen to mostly affect middle-aged and older men. Nevertheless, women may also be present with the tumor. The age range is noted to be between 40-90 years.

Generally, in the case of angiomyolipoma, the following may be observed:

  • The sporadic (non-syndromic) cases of angiomyolipoma are usually seen in the age group 45-55 years
  • When in association with tuberous sclerosis complex (syndromic cases), the age group in which the tumor is often manifested is 25-35 years
  • No ethnic or racial preference is seen

What are the Risk Factors for Angiomyolipoma of Nasal Cavity? (Predisposing Factors)

Currently, no specific risk factors have been noted for Angiomyolipoma of Nasal Cavity. The risk factors for angiomyolipoma may include the following:

  • Tuberous sclerosis (TS) is a risk factor for angiomyolipoma, and hence, a family history of TS can increase the risk. Tuberous sclerosis complex is an inherited genetic disorder that can cause the formation of other tumor types, such as astrocytomas, rhabdomyomas, phakomas, oncocytomas, and angiofibromas, at various body locations
  • In general, AML can also be associated with other syndromes/disorders including:
    • von Recklinghausen disease (neurofibromatosis type I)
    • von Hippel-Lindau disease
    • Sturge-Weber syndrome
    • Autosomal dominant polycystic kidney disease
  • Some reports indicate that events causing an hormonal imbalance in the body, such as puberty and pregnancy in women, may be a risk factor

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 Angiomyolipoma of Nasal Cavity? (Etiology)

The exact cause and mechanism of Angiomyolipoma of Nasal Cavity formation, in a majority of cases, is unknown. Solitary tumors are believed to be the result of sporadic mutations on TSC1 or TSC2 genes, implying that they do not have a preceding family history of the condition.

  • Research has shown that the tumors arise from cells called perivascular epithelioid cells (PEC), which surrounds the blood vessels. Hence, angiomyolipoma (AML) is a type of a tumor known as a PEComa
  • Multiple angiomyolipomas are known to occur in a background of tuberous sclerosis (TS), which is a genetic condition. It may also occur when there is a positive family history of the condition
  • Tuberous sclerosis complex is caused by genetic alterations involving the TSC1 and TSC2 gene. In non-TS associated AML, the loss of heterozygosity on TSC1/TSC2 gene has been noted. Also, some reports indicate that mutations on RHEB gene are observed in sporadic (non-syndromic) cases
  • In general, syndromic AML is also associated with other genetic disorders such as von Recklinghausen disease, von Hippel-Lindau disease, Sturge-Weber syndrome, and autosomal dominant polycystic kidney disease
  • Some research indicates that hormonal influence may play a role in AML formation for the following reasons:
    • The tumor is more often seen in women
    • It grows larger during pregnancy
    • The onset can be after puberty
    • And high progesterone receptor activity is present on microscopic examination

What are the Signs and Symptoms of Angiomyolipoma of Nasal Cavity?

The signs and symptoms of Angiomyolipoma of Nasal Cavity may include the following:

  • Most tumors are small-sized and may not exhibit any signs and symptoms
  • The tumor may appear like a polyp
  • Large tumors (size over 4 cm) can compress the surrounding structures or organs; but, large sizes have not been observed so far
  • Tumors tend to grow larger in size during pregnancy
  • Depending on the location of the tumor in the nose or nasal cavity, it may cause the following:
    • Snoring
    • Headaches
    • Inflammation of the nasal cavity; obstruction
    • Nosebleeds
    • Sinus congestion
    • Dry or stuffy nose
    • Difficulty in hearing
  • Bleeding can occur within large tumors; hemorrhage within the tumors can lead to tissue death (or infarction)

In general, the following may be observed about angiomyolipoma:

  • In sporadic cases (when not associated with tuberous sclerosis), angiomyolipoma is usually single. Often with sporadic tumors, they tend to be larger and so pain may be observed
  • With tuberous sclerosis (TS), multiple tumors may be seen around the body or in the same organ. But with TS, tumors are known to be more asymptomatic and are often discovered incidentally

How is Angiomyolipoma of Nasal Cavity Diagnosed?

A diagnosis of Angiomyolipoma of Nasal Cavity may involve the following tests and procedures:

  • Complete physical exam with evaluation of medical history
  • ENT examination
  • Plain X-ray of the head
  • CT or CAT scan with contrast of the head and neck region usually shows a well-defined mass, which may have calcifications. This radiological procedure creates detailed 3-dimensional images of structures inside the body
  • MRI scans of head and neck: 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
  • Vascular angiographic studies of the tumor

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, because 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
  • The tumors may have varying proportions of blood vessels, smooth muscle, and fat cells, when examined by a pathologist under a microscope
  • 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

Note: A differential diagnosis, to eliminate other tumor types is considered, before arriving at a conclusion.

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 Angiomyolipoma of Nasal Cavity?

The complications of Angiomyolipoma of Nasal Cavity are dependent upon the size of the tumor and may include:

  • Large tumor masses may get secondarily infected with bacteria or fungus
  • Stress and anxiety due to fear of cancer or cosmetic concerns due to facial disfigurement
  • Damage to the muscles, vital nerves, and blood vessels, during surgery
  • Post-surgical infection at the wound site is a potential complication
  • Research has not conclusively proven that angiomyolipoma can turn malignant. Although, some cases of sarcoma developing from AML (not associated with tuberous sclerosis) have been noted

How is Angiomyolipoma of Nasal Cavity Treated?

The treatment measures for Angiomyolipoma of Nasal Cavity may include the following:

  • Majority of asymptomatic tumors are not surgically removed: The healthcare provider may recommend a ‘wait and watch’ approach for small-sized tumors presenting mild signs and symptoms, after a diagnosis of angiomyolipoma is established
  • Pain medications for AML causing pain
  • Surgical intervention with complete excision can result in a complete cure. It can also help reduce the chances of tumor recurrence. Surgery may be performed if the tumor presents swallowing difficulties or cosmetic issues
  • Tumor embolization is a possible treatment option. Here the blood supply to the tumor is blocked resulting in its shrinkage or death
  • Newer medical treatments; recently FDA-approved medications may also be used
  • Treatment of the underlying or associated conditions, if any
  • Post-operative care is important: A minimum activity level is ensured, until the surgical wound heals
  • Follow-up care with regular screening may be recommended by the healthcare provider

How can Angiomyolipoma of Nasal Cavity be Prevented?

Current medical research has not established a method of preventing both syndromic and non-syndromic forms of Angiomyolipoma of Nasal Cavity. However, in case it is associated with genetic disorders, such as tuberous sclerosis, then the following may be considered:

  • Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
  • If there is a family history of the condition, then genetic counseling will help assess risks before planning for a child
  • Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders such as tuberous sclerosis
  • Regular medical screening at periodic intervals with tests and physical examinations are strongly recommended

What is the Prognosis of Angiomyolipoma of Nasal Cavity? (Outcomes/Resolutions)

  • The prognosis of Angiomyolipoma of Nasal Cavity depends upon the severity of the signs and symptoms. It also depends upon the overall health of the individual, association with tuberous sclerosis or other genetic conditions, and response to therapy
  • Typically, individuals with small-sized tumors have a better prognosis than those with larger-sized tumors (over 4 cm in size). Also, those with non-syndromic (sporadic) tumors have a comparatively better prognosis than individuals with tumors that are seen in association with a genetic disorder (syndromic)
  • In most cases, the prognosis of small-sized solitary tumors is excellent with surgical intervention or appropriate treatment, since these are benign tumors

Additional and Relevant Useful Information for Angiomyolipoma of Nasal Cavity:

Angiomyolipoma can occur at various locations in the body, such as the soft tissue, skin, uterus, fallopian tube, spermatic cord, penis, liver, and lung.

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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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