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These lesions rarely require surgery unless they are symptomatic or the diagnosis is in question. Since these lesions do not have malignant potential, long-term observation is unnecessary. Surgery can include the removal of the head of the pancreas (a pancreaticoduodenectomy), removal of the body and tail of the pancreas (a distal pancreatectomy), or rarely removal of the entire pancreas (a total pancreatectomy). In selected cases the surgery can be performed using minimally invasive techniques such as laparoscopy.
Wide excision is the treatment of choice, although attempting to preserve hearing. Based on the anatomic site, it is difficult to completely remove, and so while there is a good prognosis, recurrences or persistence may be seen. There is no metastatic potential. Patients who succumb to the disease, usually do so because of other tumors within the von Hippel-Lindau complex rather than from this tumor.
Most of these tumors are treated with surgical removal. It is non recurrent.
Treatment may include the following:
- Surgery with or without radiation
- Radiotherapy
Fast neutron therapy has been used successfully to treat salivary gland tumors, and has shown to be significantly more effective than photons in studies treating unresectable salivary gland tumors.
- Chemotherapy
The standard of care for mucinous adenocarcinoma with clinical condition PMP involves cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC), by surgical oncologists who specialize in treating PMP. Some surgeons also apply early post-operative intraperitonial chemotherapy (EPIC), adjunct to surgical cytoreduction and HIPEC. In situations where surgery is not required immediately, patients can be monitored via CT scans, tumor marker laboratory tests, and physical symptoms, to determine when, and if, surgery is warranted. Although some surgical procedures may be rather extensive, patients can and do recover from surgery, and the majority of these patients can and do live productive lives.
In debulking, the surgeon attempts to remove as much tumor as possible. CRS or cytoreductive surgery involves surgical removal of the peritoneum and any adjacent organs which appear to have tumor seeding. Since the mucus tends to pool at the bottom of the abdominal cavity, it is common to remove the ovaries, fallopian tubes, uterus, and parts of the large intestine. Depending upon the spread of the tumor, other organs might be removed, including but not limited to the gallbladder, spleen, and portions of the small intestine and/or stomach. For organs that cannot be removed safely (like the liver), the surgeon strips off the tumor from the surface.
Treatment is variable, both due to its rarity and to its frequently slow-growing nature. Treatment ranges from watchful waiting to debulking and hyperthermic intraperitoneal chemotherapy (HIPEC, also called intraperitoneal hyperthermic chemotherapy, IPHC) with cytoreductive surgery.
Uterine fibroids can be treated with the same methods like sporadic uterine fibroids including antihormonal treatment, surgery or embolisation. Substantially elevated risk of progression to or independent development of uterine leiomyosarcoma has been reported which may influence treatment methods.
The predisposition to renal cell cancer calls for screening and, if necessary, urological management.
The skin lesions may be difficult to treat as they tend to recur after excision or destructive treatment. Drugs which affect smooth muscle contraction, such as doxazosin, nitroglycerine, nifedipine and phenoxybenzamine, may provide pain relief.
Topical lidocaine patches have been reported to decrease in severity and frequency of pain cutaneous leiomyomas.
There is no way to reverse VHL mutations, but early recognition and treatment of specific manifestations of VHL can substantially decrease complications and improve quality of life. For this reason, individuals with VHL disease are usually screened routinely for retinal angiomas, CNS hemangioblastomas, clear-cell renal carcinomas and pheochromocytomas. CNS hemangioblastomas are usually surgically removed if they are symptomatic. Photocoagulation and cryotherapy are usually used for the treatment of symptomatic retinal angiomas, although anti-angiogenic treatments may also be an option. Renal tumours may be removed by a partial nephrectomy or other techniques such as radiofrequency ablation.
Cystadenoma (or "cystoma") is a type of cystic adenoma.
When malignant, it is called cystadenocarcinoma.
Mucinous cystadenoma is a benign cystic tumor lined by a mucinous epithelium. It is a type of cystic adenoma (cystadenoma).
Mucinous cystadenoma may arise in a number of locations; however, mucinous cystadenoma at different locations are not generally considered to be related to one another.
Serous cystadenoma may refer to:
- Ovarian serous cystadenoma, a very common benign tumour of the ovary.
- Pancreatic serous cystadenoma, also known as "serous microcystic adenoma".
Pancreatic serous cystadenoma, also known as serous cystadenoma of the pancreas and serous microcystic adenoma, a benign tumour of pancreas. It is usually found in the head of the pancreas, and may be associated with von Hippel-Lindau syndrome.
In contrast to some of the other cyst-forming tumors of the pancreas (such as the intraductal papillary mucinous neoplasm and the mucinous cystic neoplasm), serous cystic neoplasms are almost always entirely benign. There are some exceptions; rare case reports have described isolated malignant serous cystadenocarcinomas. In addition, serous cystic neoplasms slowly grow, and if they grow large enough they can press on adjacent organs and cause symptoms.
An endolymphatic sac tumor is a very uncommon papillary epithelial neoplasm arising within the endolymphatic sac or endolymphatic duct. This tumor shows a very high association with von Hippel-Lindau syndrome (VHL).
When not otherwise specified, the ICD-O coding is 8440/0. However, the following classifications also exist:
A physician's response to detecting an adenoma in a patient will vary according to the type and location of the adenoma among other factors. Different adenomas will grow at different rates, but typically physicians can anticipate the rates of growth because some types of common adenomas progress similarly in most patients. Two common responses are removing the adenoma with surgery and then monitoring the patient according to established guidelines.
One common example of treatment is the response recommended by specialty professional organizations upon removing adenomatous polyps from a patient. In the common case of removing one or two of these polyps from the colon from a patient with no particular risk factors for cancer, thereafter the best practice is to resume surveillance colonoscopy after 5–10 years rather than repeating it more frequently than the standard recommendation.
Ovarian cystadenoma is a cystic benign tumor of the ovary. Two types are recognized: serous and mucinous.
Ovarian serous cystadenoma, also (less precisely) known as serous cystadenoma, is the most common ovarian neoplasm, representing 20% of ovarian neoplasms, and is benign.Human Reproduction. University of Utah Medpath http://library.med.utah.edu/kw/human_reprod/seminars/seminar4B2.html.
It has a very superficial resemblance to the most common type of ovarian cancer (serous carcinoma of the ovary) under the microscope; however, (1) it is virtually impossible to mix-up with its malignant counterpart (serous carcinoma), and (2) does not share genetic traits of indeterminate serous tumours, also called "serous borderline tumours", that may transform into serous carcinoma.
Serous cystadenomas (of the ovary) are not related to serous cystadenomas of the pancreas, i.e. the presence of an ovarian "or" pancreatic one does "not" suggest an increased risk for the other one.
Pain associated with ovarian cysts may be treated in several ways:
- Pain relievers such as acetaminophen, nonsteroidal anti-inflammatory drugs, or opioids.
- While hormonal birth control prevents the development of new cysts in those who frequently get them, it is not useful for the treatment of current cysts.
Warthin's tumor, also known as papillary cystadenoma lymphomatosum, is a benign cystic tumor of the salivary glands containing abundant lymphocytes and germinal centers (lymph node-like stroma). It is named for pathologist Aldred Scott Warthin, who described two cases in 1929.
Hidradenoma (also known as acrospiroma, from "akral ""peripheral"" + spiroma ""epithelial tumor of sweat gland") refers to a benign adnexal tumor of the apical sweat gland. Another name for Hidradenoma is Cystadenoma and Hydrocystadenomas. These are 1–3 cm translucent blue cystic nodules.It usually presents as a single, small skin-colored lesion, and is considered distinct from the closely related poroma. Hidradenomas are often sub-classified based on subtle histologic differences, for example:
- clear-cell hidradenoma or acrospiroma
- nodular hidradenoma or acrospiroma
- solid-cystic hidradenoma
Discussion of sweat gland tumors can be difficult and confusing due to the complex classification and redundant terminology used to describe the same tumors. For example, acrospiroma and hidradenoma are synonymous, and sometimes the term "acrospiroma" is used to generally describe benign sweat gland tumors. In addition, a single lesion may contain a mixture of cell-types. There has also been a change in understanding about how tumors that were previously believed to strictly derive from specific sweat gland types may, in fact, derive from both eccrine "or" apocrine glands.
Hidradenomas are by definition benign, with malignant transformation very rare. When tumors show malignant characteristics, they are known as hidradenocarcinoma. Surgical excision is usually curative and local recurrences are rare, although malignant tumors may metastasize.
The three basic types of treatment are surgery, radiation therapy, and chemotherapy.
Surgery is performed by urologists; radiation therapy is administered by radiation oncologists; and chemotherapy is the work of medical oncologists. In most patients with testicular cancer, the disease is cured readily with minimal long-term morbidity. While treatment success depends on the stage, the average survival rate after five years is around 95%, and stage 1 cancers cases, if monitored properly, have essentially a 100% survival rate.
Salivary gland tumours or neoplasms are tumours that form in the tissues of salivary glands. The salivary glands are classified as major or minor. The major salivary glands consist of the parotid, submandibular, and sublingual glands. The minor salivary glands consist of 800-1000 small mucus-secreting glands located throughout the lining of the oral cavity.
The initial treatment for testicular cancer is surgery to remove the affected testicle (orchiectomy). While it may be possible, in some cases, to remove testicular cancer tumors from a testis while leaving the testis functional, this is almost never done, as the affected testicle usually contains pre-cancerous cells spread throughout the entire testicle. Thus removing the tumor alone without additional treatment greatly increases the risk that another cancer will form in that testicle.
Since only one testis is typically required to maintain fertility, hormone production, and other male functions, the afflicted testis is almost always removed completely in a procedure called inguinal orchiectomy. (The testicle is almost never removed through the scrotum; an incision is made beneath the belt line in the inguinal area.) In the UK, the procedure is known as a radical orchidectomy.
Mucinous cystadenomas may be found in the:
- Ovary - ovarian mucinous cystadenoma
- Pancreas - pancreatic mucinous cystadenoma
- Peritoneum - peritoneal mucinous cystadenoma
- Liver - mucinous cystadenoma of the liver
- Vermiform appendix - appendiceal mucinous cystadenoma (see cystadenoma)
Serous cystadenocarcinoma is a type of tumor in the cystadenocarcinoma grouping.
Most commonly the primary site of serous cystadenocarcinoma is the ovary. Rare occurrence in the pancreas has been reported, although this is not typical, with the majority of microcystic pancreatic masses representing alternate disease processes such as the more benign serous cystadenoma.