Glomangiosarcoma | |
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Other names | Malignant glomus tumor |
Specialty | Dermatology |
Glomangiosarcoma is a low grade [1] tumor of the soft tissue. They rarely metastasize, [2] but metastases are possible. [3] It is also known as malignant glomus tumor. [4] Positive staining for vimentin has been reported. [5]
Glomangiosarcoma usually manifest as painful blue-red dermal nodules that range from 0.5-2cm in size. [4]
Glomangiosarcomas can arise de novo or from a preexisting benign glomus tumour. [6]
Typically, a definitive diagnosis cannot be made based solely on radiologic findings and clinical symptoms. [7] To get a conclusive diagnosis of glomangiosarcoma, immunohistochemical staining in addition to hematoxylin and eosin staining is needed. [8]
Histologically, glomangiosarcomas differ from benign glomus tumors in that they include sheets of uniform, round to oval cells with eosinophilic cytoplasm, many vascular spaces, and cellular pleomorphism linked to frequent mitotic figures. [6] [9]
Glomangiosarcomas display many of the same antigens as their benign counterparts on immunohistochemical labeling; these antigens include vimentin, smooth muscle actin, and muscle-specific actin. [10] On the other hand, benign tumors stain more highly for actin and myosin, and malignant tumors stain more intensely for vimentin. [11] Studies conducted in the past have also revealed changes in the expression of Bcl-2 and p53 in comparison to benign glomus tumors. [3] [12]
These lesions in soft tissue and skin need to be distinguished from other cutaneous round cell malignancies such hemangiopericytoma, leiomyosarcoma, melanoma, and Merkel cell carcinoma. [4] [13]
Although Mohs micrographic surgery has been previously described, the standard of care for malignant glomus tumors still involves wide local excision with negative surgical margins. [4] It is not currently advised to use adjuvant chemotherapy for primary glomangiosarcoma. [14]
Sex cord–gonadal stromal tumour is a group of tumours derived from the stromal component of the ovary and testis, which comprises the granulosa, thecal cells and fibrocytes. In contrast, the epithelial cells originate from the outer epithelial lining surrounding the gonad while the germ cell tumors arise from the precursor cells of the gametes, hence the name germ cell. In humans, this group accounts for 8% of ovarian cancers and under 5% of testicular cancers. Their diagnosis is histological: only a biopsy of the tumour can make an exact diagnosis. They are often suspected of being malignant prior to operation, being solid ovarian tumours that tend to occur most commonly in post menopausal women.
Dermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive malignant cutaneous soft-tissue sarcoma. DFSP develops in the connective tissue cells in the middle layer of the skin (dermis). Estimates of the overall occurrence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year. In the United States, DFSP accounts for between 1 and 6 percent of all soft-tissue sarcomas and 18 percent of all cutaneous soft-tissue sarcomas. In the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma.
A glomus tumor is a rare neoplasm arising from the glomus body and mainly found under the nail, on the fingertip or in the foot. They account for less than 2% of all soft tissue tumors. The majority of glomus tumors are benign, but they can also show malignant features. Glomus tumors were first described by Hoyer in 1877 while the first complete clinical description was given by Masson in 1924.
Nodular fasciitis (NF) is a benign, soft tissue tumor composed of myofibroblasts that typically occurs in subcutaneous tissue, fascia, and/or muscles. The literature sometimes titles rare NF variants according to their tissue locations. The most frequently used and important of these are cranial fasciitis and intravascular fasciitis. In 2020, the World Health Organization classified nodular fasciitis as in the category of benign fibroblastic/myofibroblastic tumors. NF is the most common of the benign fibroblastic proliferative tumors of soft tissue.
Amelanotic melanoma is a type of skin cancer in which the cells do not make any melanin. They can be pink, red, purple or of normal skin color, and are therefore difficult to diagnose correctly. They can occur anywhere on the body, just as a typical melanoma can.
Infantile digital fibromatosis (IDF), also termed inclusion body fibromatosis, Reye tumor, or Reye's tumor, usually occurs as a single, small, asymptomatic, nodule in the dermis on a finger or toe of infants and young children. IMF is a rare disorder with approximately 200 cases reported in the medical literature as of 2021. The World Health Organization in 2020 classified these nodules as a specific benign tumor type in the category of fibroblastic and myofibroblastic tumors. IDF was first described by the Australian pathologist, Douglas Reye, in 1965.
Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's hemangio-endotheliome vegetant intravasculaire, Masson's lesion, Masson's pseudoangiosarcoma, Masson's tumor, and papillary endothelial hyperplasia, is a rare, benign tumor. It may mimic an angiosarcoma, with lesions that are red or purplish 5-mm to 5-cm papules and deep nodules on the head, neck, or upper extremities.
Collagenous fibroma, also known as desmoplastic fibroblastoma, is a slow-growing, deep-set, benign fibrous tumor, usually located in the deep subcutis, fascia, aponeurosis, or skeletal muscle of the extremities, limb girdles, or head and neck regions. The World Health Organization in 2020 reclassified desmoplastic fibroblastoma/collagenous fibroma as a specific benign tumor type within the broad category of fibroblastic and myofibroblastic tumors.
Atypical fibroxanthoma (AFX) of the skin is a low-grade malignancy related to malignant fibrous histiocytoma, which it resembles histologically. Atypical fibroxanthoma manifests as a hard, pink or red papule or nodule that grows over the course of several months and may bleed or ulcerate. They typically occur on the head and neck. Atypical fibroxanthoma is usually asymptomatic.
Chondroid lipomas are deep-seated, firm, yellow tumors that characteristically occur on the legs of women. They exhibit a characteristic genetic translocation t(11;16) with a resulting C11orf95-MKL2 fusion oncogene.
A cutaneous myxoma, or superficial angiomyxoma, consists of a multilobulated myxoid mass containing stellate or spindled fibroblasts with pools of mucin forming cleft-like spaces. There is often a proliferation of blood vessels and an inflammatory infiltrate. Staining is positive for vimentin, negative for cytokeratin and desmin, and variable for CD34, Factor VIIIa, SMA, MSA and S-100.
Malignant acrospiroma, also known as nodular hidradenocarcinoma, malignant nodular clear-cell hidradenoma, mucoepidermoid hidradenocarcinoma, clear-cell hidradenocarcinoma, malignant clear-cell acrospiroma, and clear-cell eccrine carcinoma, is a rare and aggressive tumour of the eccrine sweat glands. It was first documented by Keasbey and Hadley in 1954.
A renal oncocytoma is a tumour of the kidney made up of oncocytes, epithelial cells with an excess amount of mitochondria.
Juxtaglomerular cell tumor is an extremely rare kidney tumour of the juxtaglomerular cells, with fewer than 100 cases reported in literature. This tumor typically secretes renin, hence the former name of reninoma. It often causes severe hypertension that is difficult to control, in adults and children, although among causes of secondary hypertension it is rare. It develops most commonly in young adults, but can be diagnosed much later in life. It is generally considered benign, but its malignant potential is uncertain.
An Apocrine nevus is an extremely rare cutaneous condition that is composed of hyperplastic mature apocrine glands. Apocrine nevi present as a nodular skin-coloured lump. They are typically asymptomatic. Apocrine nevi can be congenital and they have been associated with other disorders.
Aggressive digital papillary adenocarcinoma, also known as digital papillary adenocarcinoma and papillary adenoma is a cutaneous condition characterized by an aggressive malignancy involving the digit between the nailbed and the distal interphalangeal joint spaces. Genetic studies reveal that human papilloma virus HPV42 is the likely driving virus in this cancer.
Malignant pilomatricoma is a cutaneous condition characterized by a locally aggressive tumor composed of hair-matrix cells.
A solid pseudopapillary tumour is a low-grade malignant neoplasm of the pancreas of papillary architecture that typically afflicts young women.
Plexiform angiomyxoid myofibroblastic tumor (PAMT), also called plexiform angiomyxoma, plexiform angiomyxoid tumor, or myxofibroma, is an extremely rare benign mesenchymal myxoid tumor along the gastrointestinal tract. Most of PAMTs occur in the gastric antral region, but they can be situated anywhere in the stomach. There is one recorded case of PAMT located in duodenum.
Fibroblastic and myofibroblastic tumors (FMTs) develop from the mesenchymal stem cells which differentiate into fibroblasts and/or the myocytes/myoblasts that differentiate into muscle cells. FMTs are a heterogeneous group of soft tissue neoplasms. The World Health Organization (2020) defined tumors as being FMTs based on their morphology and, more importantly, newly discovered abnormalities in the expression levels of key gene products made by these tumors' neoplastic cells. Histopathologically, FMTs consist of neoplastic connective tissue cells which have differented into cells that have microscopic appearances resembling fibroblasts and/or myofibroblasts. The fibroblastic cells are characterized as spindle-shaped cells with inconspicuous nucleoli that express vimentin, an intracellular protein typically found in mesenchymal cells, and CD34, a cell surface membrane glycoprotein. Myofibroblastic cells are plumper with more abundant cytoplasm and more prominent nucleoli; they express smooth muscle marker proteins such as smooth muscle actins, desmin, and caldesmon. The World Health Organization further classified FMTs into four tumor forms based on their varying levels of aggressiveness: benign, intermediate, intermediate, and malignant.