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In general, epithelioid sarcoma is a slow-growing and relatively painless tumor, often resulting in a lengthy period of time between presentation and diagnosis. Due to its ambiguity, it is often misdiagnosed, mistaken as a persistent wart or cyst. It most commonly presents itself in the distal limbs (fingers, hands, forearms, or feet) as a small, soft mass or a series of bumps. It is most often described as a firm-to-hard palpable mass, either in the deep soft tissue or in the dermis. Often, superficial lesions will ulcerate causing a mistaken diagnosis of a poorly healing traumatic wound or wart. About 13% of patients will present with multifocal tumors, and about 13% of patients will present with metastatic disease.
Epithelioid sarcoma is a rare soft tissue sarcoma arising from Mesenchyme tissue and characterized by epithelioid-like features. It accounts for less than 1% of all soft tissue sarcomas. It was first clearly characterized by F.M. Enzinger in 1970. It commonly presents itself in the distal limbs (fingers, hands, forearms, or feet) of young adults as a small, soft mass or a series of bumps. A proximal version has also been described, frequently occurring in the upper extremities. Rare cases have been reported in the pelvis, vulva, penis, and spine.
Histologically, epithelioid sarcoma forms nodules with central necrosis surrounded by bland, polygonal cells with eosinophilic cytoplasm and peripheral spindling. Epithelioid sarcomas typically express vimentin, cytokeratins, epithelial membrane antigen, and CD34, whereas they are usually negative for S100, desmin, and FLI-1. They typically stain positive for CA125.
Epithelioid sarcoma most commonly strikes young adults, yet no age group is immune. The disease has a tendency to develop local recurrences and metastasis thereafter to regional lymph nodes, lung, bone, brain, and other locations, including the scalp. Generally speaking, epithelioid sarcoma has a high rate of relapse after initial treatment and tends to recur locally (at or near the original tumor site). Epithelioid sarcoma also demonstrates lymphatic spread (in 22-48% of cases), and metastasis (in 21-63% of cases). These events, as well as advanced stage (progression) and grade (aggressiveness), are predictive of an overall worse outcome. The overall five-year survival rate for epithelioid sarcoma is anywhere from 25 to 78%. Importantly, the 10-year and 15-year survival rate drops off significantly. Associated with a more positive outcome are younger age, female vs. male sex, distal vs. proximal location, smaller tumor size, and negative margins upon tumor resection.
There are few early warning signs that a patient has a DSRCT. Patients are often young and healthy as the tumors grow and spread uninhibited within the abdominal cavity. These are rare tumors and symptoms are often misdiagnosed by physicians. The abdominal masses can grow to enormous size before being noticed by the patient. The tumors can be felt as hard, round masses by palpating the abdomen.
First symptoms of the disease often include abdominal distention, abdominal mass, abdominal or back pain, gastrointestinal obstruction, lack of appetite, ascites, anemia, and/or cachexia.
Other reported symptoms include unknown lumps, thyroid conditions, hormonal conditions, blood clotting, kidney or urological problems, testicle, breast, uterine, vaginal, or ovarian masses.
It presents as a slow growing mass that especially affects tendons and aponeuroses and it is deeply situated. Patients often perceive it as a lump or hard mass. It causes either pain or tenderness but only until it becomes large enough. This kind of tumor is commonly found in the extremities especially around the knee, feet and ankle. Patients diagnosed with clear cell sarcoma are usually between the ages of 20 and 40.
Dermatofibrosarcoma protuberans can begin as a minor firm area of skin most commonly about to 1 to 5 cm in diameter. It can resemble a bruise, birthmark, or pimple. It is a slow growing tumor and is usually found on the torso but can also be found on the arms, legs, head and neck. About 90% of DFSPs are low grade sarcomas. About 10% are mixed; they contain a high-grade sarcomatous component (DFSP-FS); therefore, they are considered to be intermediate-grade sarcomas. DFSPs rarely lead to a metastasis (fewer than 5% do metastasise), but DFSPs can recur locally. DFSPs most often arise in patients who are in their thirties, but sometimes have been described in children or the elderly.
Dermatofibrosarcoma protuberans (DFSP)
is a very rare tumor. It is a rare neoplasm of the dermis layer of the skin, and is classified as a sarcoma. There is only about one case per million per year. DFSP is a fibrosarcoma, more precisely a cutaneous soft tissue sarcoma. In many respects, the disease behaves as a benign tumor, but in 2–5% of cases it can metastasize, so it should be considered to have malignant potential. It occurs most often in adults in their thirties; it has been described congenitally, in children, and the elderly. It accounts for approximately 2–6% of soft tissue sarcoma cancers.
Symptoms may include:
- Swelling in the extremities (arms or legs), also called peripheral edema; the swelling often is painless.
- Difficulty in moving the extremity that has the tumor, including a limp.
- Soreness localized to the area of the tumor or in the extremity.
- Neurological symptoms.
- Pain or discomfort: numbness, burning, or "pins and needles."
- Dizzyness and/or loss of balance.
When the tumor is large and there is presence of necrosis and local recurrence, the prognosis is poor. Presence of metastasis occurs in more than 50% cases and the common places of its occurrence are the bone, lymph node and lungs. Five-year survival rates, which are reported to be between 50-65%, can be misleading because the disease is prone to late metastasis or recurrence. Ten and twenty-year survival rates are 33% and 10%, respectively.
Desmoplastic small-round-cell tumor is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Reported sites of metastatic spread include the liver, lungs, lymph nodes, brain, skull, and bones.
The tumor is classified as a soft tissue sarcoma. It is considered a childhood cancer that predominantly strikes boys and young adults. The disease rarely occurs in females, but when it does the tumors can be mistaken for ovarian cancer.
In dogs, mast cell tumors are the most frequent round cell tumor.
ASPS may exist in the patient’s body for a long time before being diagnosed. It can grow large and push aside surrounding tissues for a long time before causing any discomfort. Therefore, ASPS symptoms may either be a painless swelling, or a soreness caused by compressed nerves or muscles, affecting the range of motion in the area.
Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the soft tissue that surrounds nerves. They are a type of sarcoma. Most malignant peripheral nerve sheath tumors arise from the nerve plexuses that distribute nerves into the limbs—the brachial and lumbar plexuses—or from nerves as they arise from the trunk.
Synovial sarcoma usually presents with an otherwise asymptomatic swelling or mass, although general symptoms related to malignancies can be reported such as fatigue.
Perivascular epithelioid cell tumour, also known as PEComa or PEC tumour, is a family of mesenchymal tumours consisting of perivascular epithelioid cells (PECs). These are rare tumours that can occur in any part of the human body.
The cell type from which these tumours originate remains unknown. Normally, no perivascular epitheloid cells exist; the name refers to the characteristics of the tumour when examined under the microscope.
Establishing the malignant potential of these tumours remains challenging although criteria have been suggested; some PEComas display malignant features whereas others can cautiously be labeled as having 'uncertain malignant potential'. The most common tumours in the PEComa family are renal angiomyolipoma and pulmonary lymphangioleiomyomatosis, both of which are more common in patients with tuberous sclerosis complex. The genes responsible for this multi-system genetic disease have also been implicated in other PEComas.
Many PEComa types shows a female predominance in the sex ratio.
Ewing's sarcoma is more common in males (1.6 male:1 female) and usually presents in childhood or early adulthood, with a peak between 10 and 20 years of age. It can occur anywhere in the body, but most commonly in the pelvis and proximal long tubular bones, especially around the growth plates. The diaphyses of the femur are the most common sites, followed by the tibia and the humerus. Thirty percent are overtly metastatic at presentation. Patients usually experience extreme bone pain.
Signs and symptoms include: intermittent fevers, anemia, leukocytosis, increased sedimentation rate, and other symptoms of inflammatory systemic illness. Also, depending on the type, progression, and location of the tumor, great pain may occur.
According to the Bone Cancer Research Trust (BCRT), the most common symptoms are: localized pain, swelling, and sporadic bone pain with variable intensity. The swelling is most likely to be visible if the sarcoma is located on a bone near the surface of the body, but when it occurs in other places deeper in the body, like on the pelvis, it may not be visible.
UPS occurs most commonly in the extremities and retroperitoneum, but has been reported in other sites. Metastasis occurs most frequently in the lungs (90%), bones (8%), and liver (1%).
In the extremities, it presents itself as a painless enlarging soft tissue mass.
Undifferentiated pleomorphic sarcoma (UPS), also undifferentiated pleomorphic sarcoma (PUS) and previously malignant fibrous histiocytoma (abbreviated MFH), is a type of cancer and soft tissue sarcoma.
It is considered a diagnosis of exclusion for sarcomas that cannot be more precisely categorized. Other sarcomas are cancers that form in bone and soft tissues, including muscle, fat, blood vessels, lymph vessels, and fibrous tissue (such as tendons and ligaments).
Spindle cell sarcoma is a type of connective tissue cancer in which the cells are spindle-shaped when examined under a microscope. The tumors generally begin in layers of connective tissue such as that under the skin, between muscles, and surrounding organs, and will generally start as a small lump with inflammation that grows. At first the lump will be self-contained as the tumor exists in its stage 1 state, and will not necessarily expand beyond its encapsulated form. However, it may develop cancerous processes that can only be detected through microscopic examination. As such, at this level the tumor is usually treated by excision that includes wide margins of healthy-looking tissue, followed by thorough biopsy and additional excision if necessary. The prognosis for a stage 1 tumor excision is usually fairly positive, but if the tumors progress to levels 2 and 3, prognosis is worse because tumor cells have likely spread to other locations. These locations can either be nearby tissues or system-wide locations that include the lungs, kidneys, and liver. In these cases prognosis is grim and chemotherapy and radiation are the only methods of controlling the cancer.
Spindle cell sarcoma can develop for a variety of reasons, including genetic predisposition but it also may be caused by a combination of other factors including injury and inflammation in patients that are already thought to be predisposed to such tumors. Spindle cells are a naturally occurring part of the body's response to injury. In response to an injury, infection, or other immune response the connective tissues will begin dividing to heal the affected area, and if the tissue is predisposed to spindle cell cancer the high cellular turnover may result in a few becoming cancerous and forming a tumor.
Although ASPS displays a relatively indolent course, the ultimate prognosis is poor and is often characterized by late metastases.
The prognosis for rhabdomyosarcoma has improved greatly in recent decades, with over 70% of patients surviving for five years after diagnosis.
The precursor cell of PEComas is currently unknown; there is no normal counterpart "perivascular epitheloid cell". Genetically, PECs are linked to the tuberous sclerosis genes TSC1 and TSC2, although this link is stronger for angiomyolipoma and lymphangioleiomyomatosis than for other members of the PEComa family.
ERMS is the more common of two major subtypes of rhabdomyosarcoma, the other being alveolar rhabdomyosarcoma.
It has been informally classified as a "small round blue cell tumor" because of the characteristic microscopic appearance of its cells after histological staining with hematoxylin and eosin.
Leiomyosarcoma, also referred to as LMS, is a malignant (cancerous) smooth muscle tumor. A benign tumor originating from the same tissue is termed leiomyoma. It is also important to note that while it has been believed that leiomyosarcomas do not arise from leiomyomas, there are leiomyoma variants for which classification is evolving.
About 1 person in 100,000 gets diagnosed with LMS each year. Leiomyosarcoma is one of the more common types of soft-tissue sarcoma, representing 10 percent to 20 percent of new cases. (Leiomyosarcoma of the bone is more rare.) Sarcoma is rare, consisting of only 1 percent of cancer cases in adults. Leiomyosarcomas can be very unpredictable. They can remain dormant for long periods of time and recur after years. It is a resistant cancer, meaning generally not very responsive to chemotherapy or radiation. The best outcomes occur when it can be removed surgically with wide margins early, while small and still in situ.
A synovial sarcoma (also known as: malignant synovioma) is a rare form of cancer which occurs primarily in the extremities of the arms or legs, often in close proximity to joint capsules and tendon sheaths. As one of the soft tissue sarcomas, it is one of the rarest forms of soft tissue cancer.
The name "synovial sarcoma" was coined early in the 20th century, as some researchers thought that the microscopic similarity of some tumors to synovium, and its propensity to arise adjacent to joints, indicated a synovial origin; however, the actual cells from which the tumor develops are unknown and not necessarily synovial.
Primary synovial sarcomas are most common in the soft tissue near the large joints of the arm and leg but have been documented in most human tissues and organs, including the brain, prostate, and heart.
Synovial sarcoma occurs most commonly in the young, representing
about 8% of all soft tissue sarcomas but about 15–20% of cases occur in adolescents and young adults. The peak of incidence is in the third decade of life, with males being affected more often than females (ratio around 1.2:1).
Ewing sarcoma or Ewing's sarcoma () is a malignant small, round, blue cell tumor. It is a rare disease in which cancer cells are found in the bone or in soft tissue. The most common areas in which it occurs are the pelvis, the femur, the humerus, the ribs and clavicle (collar bone).
Since a common genetic locus is responsible for a large percentage of Ewing sarcoma and primitive neuroectodermal tumors, these are sometimes grouped together in a category known as the Ewing family of tumors.
Ewing sarcoma occurs most frequently in teenagers and young adults, with a male/female ratio of 1.6:1.
Although usually classified as a bone tumor, Ewing's sarcoma can have characteristics of both mesodermal and ectodermal origin, making it difficult to classify.
James Ewing (1866–1943) first described the tumour, establishing that the disease was separate from lymphoma and other types of cancer known at that time.
In their early stages, soft-tissue sarcomas usually do not cause symptoms. Because soft tissue is relatively elastic, tumors can grow rather large, pushing aside normal tissue, before they are felt or cause any problems. The first noticeable symptom is usually a painless lump or swelling. As the tumor grows, it may cause other symptoms, such as pain or soreness, as it presses against nearby nerves and muscles. If in the abdomen it can cause abdominal pains commonly mistaken for menstrual cramps, indigestion, or cause constipation.