Glomangiosarcoma is a low grade

tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

of the

soft tissue Soft tissue connective tissue, connects and surrounds or supports internal organs and bones, and includes muscle, tendons, ligaments, Adipose tissue, fat, fibrous tissue, Lymphatic vessel, lymph and blood vessels, fasciae, and synovial membranes.� ...

. They rarely

metastasize Metastasis is a pathogenic agent's spreading from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, ...

, but metastases are possible. It is also known as malignant

glomus tumor :''Glomus tumor was also the name formerly (and incorrectly) used for a tumor now called a paraganglioma.'' A glomus tumor (also known as a "solitary glomus tumor") is a rare neoplasm arising from the glomus body and mainly found under the nail, ...

. Positive staining for

vimentin Vimentin is a structural protein that in humans is encoded by the ''VIM'' gene. Its name comes from the Latin ''vimentum'' which refers to an array of flexible rods. Vimentin is a Intermediate filament#Type III, type III intermediate filamen ...

has been reported.

Signs and symptoms

Glomangiosarcoma usually manifest as painful blue-red dermal

nodules Nodule may refer to: *Nodule (geology), a small rock or mineral cluster *Manganese nodule, a metallic concretion found on the seafloor *Nodule (medicine), a small aggregation of cells *Root nodule Root nodules are found on the roots of plants, p ...

that range from 0.5-2cm in size.

Causes

Glomangiosarcomas can arise de novo or from a preexisting benign glomus tumour.

Diagnosis

Typically, a definitive diagnosis cannot be made based solely on radiologic findings and clinical symptoms. To get a conclusive diagnosis of glomangiosarcoma,

immunohistochemical staining Immunohistochemistry is a form of immunostaining. It involves the process of selectively identifying antigens in cells and tissue, by exploiting the principle of antibodies binding specifically to antigens in biological tissues. Albert Hewett ...

in addition to hematoxylin and eosin staining is needed. Histologically, glomangiosarcomas differ from benign glomus tumors in that they include sheets of uniform, round to oval cells with eosinophilic

cytoplasm The cytoplasm describes all the material within a eukaryotic or prokaryotic cell, enclosed by the cell membrane, including the organelles and excluding the nucleus in eukaryotic cells. The material inside the nucleus of a eukaryotic cell a ...

, many vascular spaces, and cellular pleomorphism linked to frequent mitotic figures. Glomangiosarcomas display many of the same

antigens In immunology, an antigen (Ag) is a molecule, moiety, foreign particulate matter, or an allergen, such as pollen, that can bind to a specific antibody or T-cell receptor. The presence of antigens in the body may trigger an immune response. An ...

as their benign counterparts on

immunohistochemical Immunohistochemistry is a form of immunostaining. It involves the process of selectively identifying antigens in cells and tissue, by exploiting the principle of antibodies binding specifically to antigens in biological tissues. Albert Hewett ...

labeling; these

include

smooth muscle Smooth muscle is one of the three major types of vertebrate muscle tissue, the others being skeletal and cardiac muscle. It can also be found in invertebrates and is controlled by the autonomic nervous system. It is non- striated, so-called bec ...

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 ...

, and muscle-specific

. On the other hand, benign tumors stain more highly for

and

myosin Myosins () are a Protein family, family of motor proteins (though most often protein complexes) best known for their roles in muscle contraction and in a wide range of other motility processes in eukaryotes. They are adenosine triphosphate, ATP- ...

, and malignant tumors stain more intensely for

. Studies conducted in the past have also revealed changes in the expression of

Bcl-2 Bcl-2, encoded in humans by the ''BCL2'' gene, is the founding member of the Bcl-2 family of regulator proteins. BCL2 blocks programmed cell death (apoptosis) while other BCL2 family members can either inhibit or induce it. It was the first a ...

and

p53 p53, also known as tumor protein p53, cellular tumor antigen p53 (UniProt name), or transformation-related protein 53 (TRP53) is a regulatory transcription factor protein that is often mutated in human cancers. The p53 proteins (originally thou ...

in comparison to benign glomus tumors. These lesions in soft tissue and skin need to be distinguished from other cutaneous round cell malignancies such hemangiopericytoma,

leiomyosarcoma A leiomyosarcoma (LMS) is a rare malignant (cancerous) smooth muscle tumor. The word is . The stomach, bladder, uterus, blood vessels, and Gastrointestinal tract, intestines are examples of hollow organs made up of smooth muscles where LMS can be ...

melanoma Melanoma is the most dangerous type of skin cancer; it develops from the melanin-producing cells known as melanocytes. It typically occurs in the skin, but may rarely occur in the mouth, intestines, or eye (uveal melanoma). In very rare case ...

, and

Merkel cell carcinoma Merkel cell carcinoma (MCC) is a rare and aggressive skin cancer occurring in about three people per million members of the population. It is also known as cutaneous APUDoma, Primary tumor, primary neuroendocrine tumor, neuroendocrine carcinoma ...

Treatment

Although

Mohs micrographic surgery Mohs surgery, developed in 1938 by general surgeon Frederic E. Mohs, is microscopically controlled surgery used to treat both common and rare types of skin cancer. During the surgery, after each removal of tissue and while the patient waits, the ...

has been previously described, the standard of care for malignant glomus tumors still involves wide local excision with negative surgical margins. It is not currently advised to use adjuvant

chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...

for primary glomangiosarcoma.

References

External links

{{Medical resources , ICD11 = {{ICD11, XH05Y1 , ICD10 = {{ICD10, D18.0 , ICD10CM = , ICD9 = , ICDO = 8710/3 , OMIM = , MeshID = , DiseasesDB = , SNOMED CT = 13875003 , Curlie = , MedlinePlus = , eMedicineSubj = , eMedicineTopic = , PatientUK = , NCI = , GeneReviewsNBK = , GeneReviewsName = , NORD = , GARDNum = , GARDName = , RP = , AO = , WO = , OrthoInfo = , Orphanet = , Scholia = Q5571213 , OB = Dermal and subcutaneous growths Types of neoplasia