What is Carney complex syndrome?

What is Carney complex syndrome?

Carney complex is a rare genetic disorder characterized by multiple benign tumors (multiple neoplasia) most often affecting the heart, skin and endocrine system and abnormalities in skin coloring (pigment) resulting in a spotty appearance to the skin of affected areas.

What is Lamb Syndrome?

LAMB syndrome. Specialty. Oncology, cardiology. Carney complex and its subsets LAMB syndrome and NAME syndrome are autosomal dominant conditions comprising myxomas of the heart and skin, hyperpigmentation of the skin (lentiginosis), and endocrine overactivity.

How common are myxomas?

Myxomas are more common in women. About 1 in 10 myxomas are passed down through families (inherited). These tumors are called familial myxomas. They tend to occur in more than one part of the heart at a time, and often cause symptoms at a younger age.

What is cutaneous myxoma?

Cutaneous myxoma, also known as superficial myxoma or superficial angiomyxoma, is a rare myxoid tumor of the skin and subcutis. Cutaneous myxomas are histologically identical to the cutaneous myxoid tumors seen in patients with Carney’s syndrome, although most occur sporadically.

How common is Carney complex?

How common is Carney complex? Carney complex is rare. Several hundred cases have been reported worldwide. It is estimated that between 60% and 75% of cases of Carney complex run in families.

What are the symptoms of Li-Fraumeni syndrome?

People with Li-Fraumeni syndrome should also watch closely for symptoms that could signal cancer, such as:

  • Unexplained weight loss.
  • Loss of appetite.
  • Aches, pains, lumps or swellings that cannot be explained.
  • Headaches or changes in vision or nerve function that do not go away.

What causes Lamb Shaffer syndrome?

Genetics. This condition is caused by mutations in the SRY-related HMG-box (SOX5) gene. This gene encodes a protein in the family of transcription factors involved in the regulation of embryonic development and in the determination of the cell fate. The gene is located on the short arm of chromosome 12 (12p12).

Can myxomas come back?

Recurrence is usually seen during the first 3 to 4 years, although it can emerge within a few months to several years after surgical excision. The recurrence rate is 22% in patients with Carney complex (3), a complex of myxomas, spotty skin pigmentation, and endocrine overactivity.

Do myxomas grow back?

Because fibroids can grow back, it is best to try to conceive as soon after a myomectomy as is safely possible and your recovery from surgery is complete. When incisions have been made into the uterine wall to remove fibroids, future pregnancy may be affected.

What is a myxoid?

Myxoid is a word pathologists use to describe connective tissue that looks more blue or purple compared to normal connective tissue when examined under the microscope. The type of connective tissue that usually shows myxoid type change is called the stroma.

Where is myxoma found?

Most frequently, myxomas are found in the heart and are referred to as cardiac myxomas, which are the most common type of primary cardiac tumors in adults. Cardiac myxomas are usually located in the atria, which are the two upper chambers of the heart, and known as atrial myxomas.

What kind of cancer is associated with Carney complex?

Although people with Carney complex have an increased risk of cancer, most tumors are benign. Cancers associated with Carney complex include liver, ovarian, testicular, and pancreatic cancer s.

How does Carney complex affect the thyroid gland?

Some individuals with Carney complex may have multiple tumors (nodules) affecting the thyroid. The thyroid is a butterfly-shaped gland at the base of the neck that secretes hormones that help to regulate growth and development in the body.

Are there any syndromes associated with thyroid cancer?

Other syndromes associated with thyroid cancer 1 1) Familial Adenomatous Polyposis (FAP). 2 2) Gardner’s Syndrome. 3 3) Cowden’s Syndrome (PTEN-Hamartoma Tumor Syndrome). 4 4) Carney’s Complex. 5 5) Werner’s Syndrome. 6 (more items)

What do you need to know about Carney complex?

Some individuals with Carney complex may have multiple tumors (nodules) affecting the thyroid. The thyroid is a butterfly-shaped gland at the base of the neck that secretes hormones that help to regulate growth and development in the body. In most cases, these nodules are benign nonfunctioning adenomas.

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