Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres

Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres

Article
Digestive Health
Diseases & Conditions
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Contributed byMaulik P. Purohit MD MPHAug 14, 2018

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

  • Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres
  • Hepatic Clear Cell Sugar Tumor
  • PEComa of Falciform Ligament/Ligamentum Teres

What is Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres? (Definition/Background Information)

  • Clear Cell Myomelanocytic Tumor (CCMT) of Falciform Ligament/Ligamentum Teres is an extremely rare type of PEComa. As the name suggests, these tumors arise from the falciform ligament and ligament teres present in the right side of the liver. Therefore, it is also known as CCMT of Liver
  • PEComa is a group of soft tissue tumors that show perivascular epithelioid-cell (PEC) differentiation. PEComa tumors may be benign, malignant, or of uncertain malignant potential
  • Clear Cell Myomelanocytic Tumors of Falciform Ligament/Ligamentum Teres are mostly benign and are rarely known to be malignant (with metastatic tendency). These tumors can be associated with a syndrome (tuberous sclerosis) in some cases
  • Small-sized tumors may not present any significant signs and symptoms, while large tumors may compress the surrounding organs and structures causing pain and mass effect. Since the liver is involved, it can result in abdominal pain, weight loss, and easy bleeding (due to liver injury). Typically, a surgical excision of the tumor with its entire removal may be undertaken
  • The prognosis of benign Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres is generally excellent with its complete removal, in many cases. The prognosis of malignant tumors may depend upon several factors, such as tumor stage and health status of the individual

Who gets Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres? (Age and Sex Distribution)

  • Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres usually occurs in young adults and children (up to 3 years of age recorded)
  • Both male and female genders are affected; though a female predominance is noted
  • No ethnic or racial preference is seen

What are the Risk Factors for Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres? (Predisposing Factors)

  • Currently, no risk factors have been noted for sporadic Clear Cell Myomelanocytic Tumors of Falciform Ligament/Ligamentum Teres
  • The familial form of CCMT of Falciform Ligament/Ligamentum Teres is associated with tuberous sclerosis complex, which is an inherited genetic disorder that can cause the formation of other tumor types

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s 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 Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres? (Etiology)

The cause of formation of Clear Cell Myomelanocytic Tumors of Falciform Ligament/Ligamentum Teres may be due to genetic factors. It is a type of PEComa tumor and the following abnormalities have been noted for PEComas:

  • Currently, studies indicate defects in the following genes: TSC2 and TFE3
  • Additionally, the following chromosomal aberration is noted: Mutations in chromosome 16 due to loss of 16p (short arm)
  • One specific case of CCMT of Liver showed translocation t(p3;10)

What are the Signs and Symptoms of Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres?

The signs and symptoms of Clear Cell Myomelanocytic Tumors of Falciform Ligament/Ligamentum Teres may include the following:

  • Most tumors are small-sized and may not exhibit any significant signs and symptoms
  • Large tumors may show pain and compress the surrounding structures
  • The size of the tumor may range from 5-20 cm
  • The right side of the liver is involved causing associated signs and symptoms including:
    • Nausea and vomiting
    • Weight loss
    • Abdominal pain
    • Easy bruising or bleeding that occurs because of liver failure
  • CCMT of Liver is a type of sugar tumor. All “sugar” tumors are usually benign, while some may be malignant and metastasize/spread to other regions
  • Large tumors can affect the functioning of the liver
  • With tuberous sclerosis (TS), multiple tumors may be seen around the body or in the same organ. Tumors present with TS are generally known to be more asymptomatic and are often discovered incidentally

How is Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres Diagnosed?

A diagnosis of Clear Cell Myomelanocytic Tumors of Falciform Ligament/Ligamentum Teres may involve the following tests and procedures:

  • Complete physical exam with evaluation of medical history: If the tumor is associated with tuberous sclerosis; then, the signs and symptoms of tuberous sclerosis may be noted. This can also help the healthcare provider to suspect the condition
  • Plain X-ray of the abdomen
  • Ultrasound scan of the abdomen
  • CT or CAT scan with contrast of the abdomen may show a well-defined mass, which may have calcifications. This radiological procedure creates detailed 3-dimensional images of structures inside the body
  • MRI scans of the abdomen: 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
  • Liver function tests
  • Vascular angiographic studies of the tumor
  • MRI scans and PET scans may help differentiate benign and malignant tumors

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

A differential diagnosis, to eliminate the following tumor types is considered, before arriving at a definite diagnosis:

  • Adipocytic tumor
  • Clear cell sarcoma
  • Gastrointestinal stromal tumor (GIST)
  • Melanoma
  • Smooth muscle tumor

Note: In a majority of cases, CCMT of Falciform Ligament/Ligamentum Teres is diagnosed incidentally.

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 Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres?

The complications of Clear Cell Myomelanocytic Tumors of Falciform Ligament/Ligamentum Teres may include:

  • Severe liver failure due to damage to the liver; massive, large-sized tumors can lead to severe symptoms that may damage the liver
  • Portal hypertension if the tumor is large and compresses the portal veins
  • Recurrence of CCMT of Liver following surgery
  • Malignant transformation resulting in metastasis to various regions, in rare instances
  • Damage to the muscles, vital nerves, and blood vessels, during surgery
  • Post-surgical infection at the wound site is a potential complication
  • Complications due to underlying tuberous sclerosis genetic disorder

How is Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres Treated?

The treatment measures for Clear Cell Myomelanocytic Tumors of Falciform Ligament/Ligamentum Teres may include the following:

  • Surgical intervention with complete excision can result in a complete cure. The surgical procedure used is known as hemi-hepatectomy and cholecystectomy; which involves a removal of the right half of the liver and gall bladder (and bile duct)
  • Tumor embolization (performed on a case-by-case basis) is a possible treatment option. Here the blood supply to the tumor is blocked resulting in its shrinkage or death
  • 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 Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres be Prevented?

Current medical research has not established a method of preventing Clear Cell Myomelanocytic Tumors of Falciform Ligament/Ligamentum Teres. 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 (long-term) with tests and physical examinations are recommended

What is the Prognosis of Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres? (Outcomes/Resolutions)

  • The prognosis of Clear Cell Myomelanocytic Tumors of Falciform Ligament/Ligamentum Teres depends upon the severity of the signs and symptoms. It also depends upon whether the tumor is benign or malignant, the overall health of the individual, association with tuberous sclerosis, and response to therapy (including successful surgical removal of the tumor)
  • The prognosis of benign tumor is generally excellent with treatment in many cases. The prognosis of tumors undergoing malignant transformation (in rare cases) may depend upon several factors including the stage of the tumor and overall health of the individual

Additional and Relevant Useful Information for Clear Cell Myomelanocytic Tumor of Falciform Ligament/Ligamentum Teres:

When clear cell ‘sugar’ tumor occurs in the kidney, it is known as a renal capsuloma.

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Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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