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This is a very rare neoplasm accounting for approximately 0.0003% of all tumors and about 2.5% of all external ear neoplasms. There is a wide age range at initial presentation, although the mean age is about 50 years of age. Females are affected slightly more often (1.5:1).
The tumor is rare, affecting adults in the 4th decade most commonly. Patients are usually younger than those who present with a lipoma. There is a slight male predominance. Hibernoma are most commonly identified in the subcutaneous and muscle tissue of the head and neck region (shoulders, neck, scapular), followed by thigh, back, chest, abdomen, and arms. In rare cases hibernoma may arise in bone tissue, however it is an incidental finding.
A 2006 review stated that RS often leads renal cancer between ages 30-50. Renal cancer kills about 1 in 3 people, but 5-year survival rates improved between 1974-1976 and 1995-2000, from 52% to 64%.
Treatment consists of wide resection or amputation. Metastases are rare at presentation but may occur in up to 30% of patients during the disease course. Prognosis is excellent, with overall survival of 85% at 10 years, but is lower when wide surgical margins cannot be obtained. This tumor is insensitive to radiation so chemotherapy is not typically used unless the cancer has metastasized to the lungs or other organs.
Smooth muscle tumor of uncertain malignant potential, abbreviated STUMP, is an uncommon tumor of the uterine smooth muscle that may behave like a benign tumor or a cancerous tumor.
This tumor should not be confused with the prostatic stromal tumor of uncertain malignant potential which may be abbreviated the same way ("STUMP").
The Bell criteria were developed to help categorize them and differentiate them from their main differential diagnoses, leiomyosarcoma and uterine leiomyoma.
Ceruminous adenocarcinoma is a malignant neoplasm derived from ceruminous glands of the external auditory canal. This tumor is rare, with several names used in the past. Synonyms have included cylindroma, ceruminoma, ceruminous adenocarcinoma, not otherwise specified (NOS), ceruminous adenoid cystic carcinoma (ACC), and ceruminous mucoepidermoid carcinoma.
According to a Dutch source juvenile pilocytic astrocytoma occurs at a rate of 2 in 100,000 people. Most affected are children ages 5–14 years. According to the National Cancer Institute more than 80% of astrocytomas located in the cerebellum are low grade (pilocytic grade I) and often cystic; most of the remainder are diffuse grade II astrocytomas.
Tumors of the optic pathway account for 3.6-6% of pediatric brain tumors, 60% of which are juvenile pilocytic astrocytomas. Astrocytomas account for 50% of pediatric primary central nervous system tumors. About 80-85% of cerebellar astrocytomas are juvenile pilocytic astrocytomas.
Recent genetic studies of pilocytic astrocytomas show that some sporadic cases have gain in chromosome 7q34 involving the BRAF locus.
JCT often is described as benign, however one case of metastasis has been reported, so its malignant potential is uncertain. In most cases the tumor is encapsulated.
Papillary tumors of pineal region are extremely rare, constituting 0.4-1% of all central nervous system tumors. These tumors most commonly occur in adults with the mean age being 31.5. There have been cases reported for people between the ages 5 to 66 years. There is a slight predominance of females who have these tumors.
The treatment is simple excision and exclusion of a malignant neoplasm.
Sebaceous lymphadenoma is a tissue diagnosis, e.g. salivary gland biopsy.
It may be confused with a number of benign and malignant neoplasms, including Warthin tumour, mucoepidermoid carcinoma and sebaceous lymphadenocarcinoma.
The average age at time of EIN diagnosis is approximately 52 years, compared to approximately 61 years for carcinoma. The timeframe and likelihood of EIN progression to cancer, however, is not constant amongst all women. Some cases of EIN are first detected as residual premalignant disease in women who already have carcinoma, whereas other EIN lesions disappear entirely and never lead to cancer. For this reason, treatment benefits and risks must be individualized for each patient under the guidance of an experienced physician.
Risk factors for development of EIN and the endometrioid type of endometrial carcinoma include exposure to estrogens without opposing progestins, obesity, diabetes, and rare hereditary conditions such as hereditary nonpolyposis colorectal cancer. Protective factors include use of combined oral contraceptive pills (low dose estrogen and progestin), and prior use of a contraceptive intrauterine device.
Complete surgical excision is the treatment of choice, associated with an excellent long term clinical outcome.
Surgical excision is the preferred method of treatment for benign glomus tumors.
The most common adverse effect is pain, which is usually associated with solitary lesions. Multiple tumors are less likely to be painful.
In one report, a patient with more than 400 glomus tumors had thrombocytopenia as a result of platelet sequestration (i.e. Kasabach-Merritt syndrome).
Malignant glomus tumors, or glomangiosarcomas, are extremely rare and usually represent a locally infiltrative malignancy. However, metastases do occur and are usually fatal.
An odontogenic keratocyst is a rare and benign but locally aggressive developmental cyst. It most often affects the posterior mandible. It most commonly presents in the third decade of life.
In the WHO/IARC classification of head and neck pathology, this clinical entity had been known for years as the odontogenic keratocyst; it was reclassified as keratocystic odontogenic tumour (KCOT) from 2005 to 2017. In 2017 it reverted to the earlier name, as the new WHO/IARC classification reclassified OKC back into the cystic category. The WHO/IARC classification no longer considers it a neoplasm, because the evidence supporting that hypothesis (for example, clonality) is considered insufficient. However, this is an area of hot debate within the head and neck pathology community, and some pathologists still regard OKC as a neoplasm despite the reclassification.
Smooth muscle tumours show a smooth muscle differentiation. There are two main types of smooth muscle tumour: the benign leiomyoma and the malignant leiomyosarcoma.
Mesenchymal neoplasms of the gallbladder are rare and in particular leiomyomas of the gallbladder have been rarely reported, all of them in patients with immune system disorders. Although, recently, a case was reported in absence of associated immunodeficiency at Monash Hospital in Melbourne Australia in a healthy 39-year-old woman with no symptoms.
KCOTs are thought to arise from the dental lamina and are associated with impacted teeth. Multiple odontogenic keratocysts are a feature of nevoid basal cell carcinoma syndrome.
Odotogenic Keratocysts are derived from the remnants of the Dental Lamina.
Hamartomas, while generally benign, can cause problems due to their location. For example, when located on the skin, especially on the face or neck, they can be very disfiguring. Cases have been reported of hamartomas the size of a small orange. They may obstruct practically any organ in the body, such as the colon, eye, etc. They are particularly likely to cause major health issues when located in the hypothalamus, kidneys, lips, or spleen. They can be removed surgically if necessary, and are not likely to recur. Prognosis will depend upon the location and size of the lesion, as well as the overall health of the patient.
Other relatively rare conditions have been reported in association with this disease. It is not yet known if these associations are fortuitous or manifestations of the condition itself.
Cerebral cavernomas and massive, macronodular adrenocortical disease have also been reported in association with this syndrome. A case of cutis verticis gyrata, disseminated collagenoma and Charcot-Marie-Tooth disease in association with a mutation in the fumarate hydratase gene has also been reported. Two cases of ovarian mucinous cystadenoma have also been reported with this mutation.
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".
Adamantinoma (from the Greek word "adamantinos", meaning "very hard") is a rare bone cancer, making up less than 1% of all bone cancers. It almost always occurs in the bones of the lower leg and involves both epithelial and osteofibrous tissue.
The condition was first described by Fischer in 1913.
Metanephric adenoma (MA)is a rare, benign tumour of the kidney, that can have a microscopic appearance similar to a nephroblastoma (Wilms tumours), or a papillary renal cell carcinoma.
It should not be confused with the pathologically unrelated, yet similar sounding, "mesonephric adenoma".
Atypical polypoid adenomyoma, abbreviated APA, is a rare uncommon benign tumour of the uterus.