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The prognosis varies depending on the site of origin, the type of cancer cell, the tumor size, the depth, and proximity to lymph nodes. Well-differentiated liposarcomas treated with surgery, intra-operative distilled water lavage and radiation have a low recurrence rate (about 10%) and rarely metastasize.
Five-year survival rates vary from 100% to 56% based on histological subtype.
The diagnosis is established by histologic examination of the tissue, i.e., biopsy or excision. Lipoblasts are often present; these are cells with an abundant clear multi-vacuolated cytoplasm and an eccentric darkly staining nucleus that is indented by the vacuoles.
Sarcomas are given a number of different names based on the type of tissue that they most closely resemble. For example, osteosarcoma resembles bone, chondrosarcoma resembles cartilage, liposarcoma resembles fat, and leiomyosarcoma resembles smooth muscle.
In addition to being named based on the tissue of origin, sarcomas are also assigned a grade (low, intermediate, or high) based on the presence and frequency of certain cellular and subcellular characteristics associated with malignant biological behavior. Low grade sarcomas are usually treated surgically, although sometimes radiation therapy or chemotherapy are used. Intermediate and high grade sarcomas are more frequently treated with a combination of surgery, chemotherapy and/or radiation therapy. Since higher grade tumors are more likely to undergo metastasis (invasion and spread to locoregional and distant sites), they are treated more aggressively. The recognition that many sarcomas are sensitive to chemotherapy has dramatically improved the survival of patients. For example, in the era before chemotherapy, long-term survival for patients with localized osteosarcoma was only approximately 20%, but now has risen to 60–70%.
The 5-year disease-free survival for age >5 years is 50-60%. Another report found a similar 5-year survival at about 65% with 51% progression-free survival. The 10-year disease-free survival is 40-50%. Younger ages showed lower 5 and 10-year survival rates. A 2006 study that observed 133 patients found 31 (23.3%) had a recurrence of the disease within a five-year period.
hTERT and yH2AX are crucial markers for prognosis and response to therapy. High hTERT and low yH2AX expression is associated with poor response to therapy. Patients with both high or low expression of these markers make up the moderate response groups.
MEM comprises a heterogeneous group of neoplasms believed to originate from the neural crest. First hints to this type of tumor were probably from Shuangshoti and Nestky (1971) and from Holimon and Rosenblum (1971) (2-3). Additional contributions were provided thereafter by Naka et al. (1975), Karcioglu et al. (1977), Cozzutto et al. (1982) and Kawamoto et al. (1987).
Kosem et al. collected 44 cases of MEM in a 2004 review and examined management data finding out that resection with pre- or post-surgery chemotherapy yielded the best results with one death only in 13. In the five cases reported by Mouton et al. an aggressive chemotherapy and adequate surgical excision granted a disease-free interval for 7 to 50 months. The attainability of radical surgical
ablation seems the most important prognostic factor (10).
The main features of this tumor is to comprise either ectodermal derivatives (neuroblasts and ganglion cells) or mesenchymal components mostly represented by plump, elongated cells in interlacing bundles often showing rhabdomyoblastic differentiation, including strap-like and racket-shaped cells (2-6). A myofibril-like structure and cross striations can be identified. Liposarcoma-like and chondroid foci can be an additional finding. Fibrosarcoma-like and fibrous histiocytoma-like areas can be observed as well as neurofibromatous and neuroblastic components with rosette formation. Ganglion cells can appear immature and atypical, they can be bi- or multinucleated and showing evidence of Nissl substance (2-6).
Rhabdomyoblasts and poorly differentiated small cells display positivity for desmin and myosin while neural areas are variably sensitive to S-100. Ganglion cells are strongly positive for NSE. It is important to point out that the ectodermal component may be sometimes scanty and can be overlooked whereas in specimens after chemotherapy the ganglioneuroma component is increased and even overwhelming.
Differential diagnosis should consider rhabdomyosarcoma, Triton tumor, teratoma, Wilms tumor and benign, mature ectomesenchymoma (ectomesenchymal hamartoma).
Complete surgical excision is the treatment of choice, associated with an excellent long term clinical outcome.
It is important to separate hiberoma from adult rhabdomyoma, a granular cell tumor and a true liposarcoma.
Adult survivors of childhood cancer have some physical, psychological, and social difficulties.
Premature heart disease is a major long-term complication in adult survivors of childhood cancer. Adult survivors are eight times more likely to die of heart disease than other people, and more than half of children treated for cancer develop some type of cardiac abnormality, although this may be asymptomatic or too mild to qualify for a clinical diagnosis of heart disease.
Children with cancer are at risk for developing various cognitive or learning problems. These difficulties may be related to brain injury stemming from the cancer itself, such as a brain tumor or central nervous system metastasis or from side effects of cancer treatments such as chemotherapy and radiation therapy. Studies have shown that chemo and radiation therapies may damage brain white matter and disrupt brain activity.
A myxoid liposarcoma is a malignant adipose tissue neoplasm of myxoid appearance histologically.
Myxoid liposarcomas are the second most common type of liposarcoma, representing 30–40% of all liposarcomas in the limbs; occurring most commonly in the legs, particularly the thigh, followed by the buttocks, retroperitoneum, trunk, ankle, proximal limb girdle, head and neck, and wrist. They occur in the intermuscular fascial planes or deep-seated areas. They present as a large, slow-growing, painless mass.
They are associated with a fusion between DDIT3 or "CHOP" (at 12q13.1-q13.2) and FUS or "TLS" (at 16p11.2) or EWS (at 22q12.2).
The specific translocation of FUS-DDIT3 is t(12;16)(q13;p11).
Embryonal tumor is a mass of rapidly growing cells. It is believed that it begins in embryonic (fetal) tissue. Embryonal tumors may be benign or malignant, and include neuroblastomas and Wilms tumors. Also called embryoma. Embryomas have been defined as: "Adult neoplasms expressing one or more embryo-exclusive genes", in: "Embryoma Gene Networks", http://www.embryomas.net
Embryomas can appear in the lungs.
It is not a precise term, and it is not commonly used in modern medical literature. Embryomas have been defined as: "Adult neoplasms expressing one or more embryo-exclusive genes".
Leiomyoma is the most common benign mesenchymal tumor of esophagus and second most common benign tumor of the small bowel (with gastrointestinal stromal tumor as most common). Although leiomyoma is the most common benign esophageal tumor, malignant carcinoma is still 50 times more likely. Approximately 50% of cases are found in the jejunum, followed by the ileum in 31% of cases. Almost one half of all lesions are less than 5 centimeters.
Usually, treatment of a lipoma is not necessary, unless the tumor becomes painful or restricts movement. They are usually removed for cosmetic reasons, if they grow very large, or for histopathology to check that they are not a more dangerous type of tumor such as a liposarcoma. This last point can be important as the characteristics of a "bump" are not known until after it is removed and medically examined.
Lipomas are normally removed by simple excision. The removal can often be done under local anaesthetic, and takes less than 30 minutes. This cures the great majority of cases, with about 1–2% of lipomas recurring after excision. Liposuction is another option if the lipoma is soft and has a small connective tissue component. Liposuction typically results in less scarring; however, with large lipomas it may fail to remove the entire tumor, which can lead to regrowth.
New methods under development are supposed to remove the lipomas without scarring. One is removal by injecting compounds that trigger lipolysis, such as steroids or phosphatidylcholine.
A leiomyoma, also known as fibroids, is a benign smooth muscle tumor that very rarely becomes cancer (0.1%). They can occur in any organ, but the most common forms occur in the uterus, small bowel, and the esophagus. Polycythemia may occur due to increased erythropoietin production as part of a paraneoplastic syndrome.
The word is from "" + "" + "", "smooth-muscle tumor".
Benign lipoblastomatosis (also known as an "embryonic lipoma") is a tumor frequently confused with a liposarcoma, affecting exclusively infants and young children, with approximately 90% occurring before 3 years of age.
Myxoid lipoblastoma is a cutaneous condition characterized by excess mucin. It resembles myxoid liposarcoma.
Little is known in terms of effective means of prevention. Due to the low likelihood of transmission even from an infected mother, it is not recommended to expose the mother and child to the additional risks of caesarean section to prevent the transmission of this disease during vaginal childbirth. Opting for a caesarean section does not guarantee that transmission will not still occur.
Lipomatosis is believed to be a hereditary condition in which multiple lipomas are present on the body.
Adiposis dolorosa (Dercum disease) is a rare condition involving multiple painful lipomas, swelling, and fatigue. Early studies mentioned prevalence in obese postmenopausal women. However, current literature demonstrates that Dercum disease is present in more women than men of all body types; the average age for diagnosis is 35 years.
Benign symmetric lipomatosis (Madelung disease) is another condition involving lipomatosis. It nearly always appears in middle-aged males after many years of alcoholism. But, non-alcoholics and females can also be affected.
Laryngeal papillomatosis can be diagnosed through visualization of the lesions using one of several indirect laryngoscopy procedures. In indirect laryngoscopy, the tongue is pulled forward and a laryngeal mirror or a rigid scope is passed through the mouth to examine the larynx. Another variation of indirect laryngoscopy involves passing a flexible scope, known as a fiberscope or endoscope, through the nose and into the throat to visualize the larynx from above. This procedure is also called flexible fiberoptic laryngoscopy.
The appearance of papillomas has been described as multiple or rarely, single, white growths with a lumpy texture similar to cauliflower. Papillomas usually present in the larynx, especially on the vocal folds and in the space above the vocal folds called the ventricles. They can spread to other parts of the larynx and throughout the aerodigestive tract, from the mouth to the lower respiratory tract. Spread to regions beyond the larynx is more common in children then adults. Growths tend to be located at normal junctions in squamous and ciliated epithelium or at tissue junctions arising from injury.
A confirmatory diagnosis of laryngeal papillomatosis can only be obtained through a biopsy, involving microscopic examination and HPV testing of a sample of the growth. Biopsy samples are collected under general anesthesia, either through direct laryngoscopy or fiberoptic bronchoscopy.
After removal, the testicle is fixed with Bouin's solution because it better conserves some morphological details such as nuclear conformation. Then the testicular tumor is staged by a pathologist according to the TNM Classification of Malignant Tumors as published in the AJCC Cancer Staging Manual. Testicular cancer is categorized as being in one of three stages (which have subclassifications). The size of the tumor in the testis is irrelevant to staging. In broad terms, testicular cancer is staged as follows:
- Stage I: the cancer remains localized to the testis.
- Stage II: the cancer involves the testis and metastasis to retroperitoneal and/or paraaortic lymph nodes (lymph nodes below the diaphragm).
- Stage III: the cancer involves the testis and metastasis beyond the retroperitoneal and paraaortic lymph nodes. Stage 3 is further subdivided into non-bulky stage 3 and bulky stage 3.
Further information on the detailed staging system is available on the website of the American Cancer Society.
Although testicular cancer can be derived from any cell type found in the testicles, more than 95% of testicular cancers are germ cell tumors (GCTs). Most of the remaining 5% are sex cord-gonadal stromal tumours derived from Leydig cells or Sertoli cells. Correct diagnosis is necessary to ensure the most effective and appropriate treatment. To some extent, this can be done via blood tests for tumor markers, but definitive diagnosis requires examination of the histology of a specimen by a pathologist.
Most pathologists use the World Health Organization classification system for testicular tumors:
- Germ cell tumors
- "Precursor lesions"
- Intratubular germ cell neoplasia
- Unclassified type (carcinoma in situ)
- Specified types
- "Tumors of one histologic type (pure forms)"
- Seminoma
- Variant - Seminoma with syncytiotrophoblastic cells
- Spermatocytic seminoma
- Variant - spermatocytic seminoma with sarcoma
- Embryonal carcinoma
- Yolk sac tumor
- Trophoblastic tumors
- Choriocarcinoma
- Variant - monophasic choriocarcinoma
- Placental site trophoblastic tumour
- Cystic trophoblastic tumor
- Teratoma
- Variant - Dermoid cyst
- Variant - Epidermoid cyst
- Variant - Monodermal teratoma (Carcinoid, Primitive neuroectodermal tumor (PNET), Nephroblastoma-like tumor, others.
- Variant - Teratomic with somatic-type malignancy
- "Tumours of more than one histologic type (mixed forms)"
- Embryonal carcinoma and teratoma
- Teratoma and seminoma
- Choriocarcinoma and teratoma.embryonal carcinoma
- Others
- Sex cord/Gonadal stromal tumors
- Leydig cell tumor
- Sertoli cell tumor
- Lipid rich variant
- Scleriosing variant
- Large cell calcifying variant
- Intratubular sertoli cell neoplasia in Peutz-Jeghers syndrome
- Granulosa cell tumor
- Adult type
- Juvenile type
- Thecoma Fibroma Group
- Thecoma
- Fibroma
- Sex cord/gonadal stromal tumor - incompletely differentiated
- Sex cord/gonadal stromal tumor - mixed types
- Mixed Germ Cell and Sex Cord/Gonadal Stromal Tumors
- Gonadoblastoma
- Germ cell-sex cord/gonadal stromal tumor, unclassified
- Miscellaneous tumours of the testis
- Carcinoid
- Tumors of ovarian epithelial types
- Serous tumor of borderline malignancy
- Serous carcinoma
- Well differentiated endometrioid tumor
- Mucinous cystadenoma
- Mucinous cystadenocarcinoma
- Brenner tumor
- Nephroblastoma
- Paraganglioma
- Haematopoietic tumors
- Tumours of collecting ducts and rete
- Adenoma
- Carcinoma
- Tumors of the paratesticular structures
- Adenomatoid tumor
- Malignant and Benign Mesothelioma
- Adenocarcinoma of the epididymis
- Papillary cystadenoma of the epididymis
- Melanotic neuroectodermal tumor
- Desmoplastic small round cell tumor
- Mesenchymal tumors of the spermatic cord and testicular adnexae
- Lipoma
- Liposarcoma
- Rhabdomyosarcoma
- Aggressive angiomyxoma
- Angiomyofibroblastoma-like tumor (see Myxoma)
- Fibromatosis
- Fibroma
- Solitary fibrous tumor
- Others
- Secondary tumors of the testis
Acrospiroma (also known as hidradenoma) is a cutaneous condition, primarily occurring in adult women, that is a form of benign adnexal neoplasm closely related to poroma.