Angiomyxoma is a

myxoid tumor A myxoid tumor is a connective tissue tumor with a "myxoid" background, composed of clear, mucoid substance. This tumoral phenotype is shared by many tumoral entities: * Myxomas ** Atrial myxoma ** Odontogenic myxoma ** Cutaneous myxoma ** Intra ...

involving the

blood vessel The blood vessels are the components of the circulatory system that transport blood throughout the human body. These vessels transport blood cells, nutrients, and oxygen to the tissues of the body. They also take waste and carbon dioxide away ...

s. It can affect the

vulva The vulva (plural: vulvas or vulvae; derived from Latin for wrapper or covering) consists of the external sex organ, female sex organs. The vulva includes the mons pubis (or mons veneris), labia majora, labia minora, clitoris, bulb of vestibu ...

and other parts of the

pelvis The pelvis (plural pelves or pelvises) is the lower part of the trunk, between the abdomen and the thighs (sometimes also called pelvic region), together with its embedded skeleton (sometimes also called bony pelvis, or pelvic skeleton). The ...

. The characteristic feature of this tumor is its frequent local recurrence and it is currently regarded as a non-metastasizing benign tumor.

Genetics

* HMGA2 rearrangement ** by translocation t(12;21)(q15;q21.1) ** by translocation t(11;12)(q23;q15) ** by translocation t(8;12)(p12;q15) * t(5;8)(p15;q22)

Pathology

Microscopy

* Vascular appearance of tumor * Hypocellular mesenchymal lesion * Spindled and stellate cells with an ill-defined cytoplasm * Cells loosely scattered in a myxoid stroma * No evidence of nuclear atypia and mitosis * Numerous, thin-to-thick wall vessels of different sizes * Myxoid, hypocellular background * Bland cytological appearance of spindle cells

Microscopical views

File:Aggressive angiomyxoma.jpg File:Aggressive angiomyxoma 2.jpg File:Aggressive angiomyxoma 6.jpg File:Aggressive angiomyxoma humpath 8.jpg File:Aggressive angiomyxoma - high mag.jpg

Immunochemistry

Immunohistochemical studies show strong staining for

desmin Desmin is a protein that in humans is encoded by the ''DES'' gene. Desmin is a muscle-specific, type III intermediate filament that integrates the sarcolemma, Z disk, and nuclear membrane in sarcomeres and regulates sarcomere architecture. Str ...

, estrogen receptors, and

progesterone receptor The progesterone receptor (PR), also known as NR3C3 or nuclear receptor subfamily 3, group C, member 3, is a protein found inside cells. It is activated by the steroid hormone progesterone. In humans, PR is encoded by a single ''PGR'' gene resid ...

s. Staining for

actin Actin is a family of globular multi-functional proteins that form microfilaments in the cytoskeleton, and the thin filaments in muscle fibrils. It is found in essentially all eukaryotic cells, where it may be present at a concentration of over ...

, CD34 and

smooth muscle actin Smooth may refer to: Mathematics * Smooth function, a function that is infinitely differentiable; used in calculus and topology * Smooth manifold, a differentiable manifold for which all the transition maps are smooth functions * Smooth algebrai ...

are intermediate. Staining for S-100 protein is negative.

Diagnosis

Differential diagnosis

Myxoid tumor A myxoid tumor is a connective tissue tumor with a "myxoid" background, composed of clear, mucoid substance. This tumoral phenotype is shared by many tumoral entities: * Myxomas ** Atrial myxoma ** Odontogenic myxoma ** Cutaneous myxoma ** Intra ...

s **

Angiomyofibroblastoma Angiomyofibroblastoma is an uncommon benign mesenchymal tumor. It occurs mostly in the vulvovaginal area of women, but can also be observed in men. The World Health Organization, 2020, reclassified these tumors as a specific type of tumor in the ...

Treatment

Prognosis

Although it is a benign tumour and does not invade neighbouring tissues, it has a tendency to recur after surgical excision so it is termed "aggressive". Recurrence can occur as early as six months from initial resection. Patients frequently present at tertiary medical centers with a history of

labial The term ''labial'' originates from '' Labium'' (Latin for "lip"), and is the adjective that describes anything of or related to lips, such as lip-like structures. Thus, it may refer to: * the lips ** In linguistics, a labial consonant ** In zoolog ...

mass (sometimes misdiagnosed as Gartner's cyst), with multiple surgical excisions from several surgeons. There is no standard medical therapy; agents reported to be effective in case reports include systemic hormonal therapy with SERMs such as

tamoxifen Tamoxifen, sold under the brand name Nolvadex among others, is a selective estrogen receptor modulator used to prevent breast cancer in women and treat breast cancer in women and men. It is also being studied for other types of cancer. It has b ...

or LHRH agonists ( leuprolide), and

cytotoxic Cytotoxicity is the quality of being toxic to cells. Examples of toxic agents are an immune cell or some types of venom, e.g. from the puff adder (''Bitis arietans'') or brown recluse spider (''Loxosceles reclusa''). Cell physiology Treating cells ...

("traditional")

chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemotherap ...

, as well as

radiation therapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...

especially for recurrent disease.

History

Aggressive angiomyxoma was originally described in 1983, but the term "angiomyxoma" dates back to at least 1952.

References

External links

{{Soft tissue tumors and sarcomas Vascular diseases Benign neoplasms