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Carcinomatosis is a medical condition in which multiple carcinomas will develop from a primary source, often simultaneously.

They are forms of metastasis and whether they spread in a general manner or in a defined pattern, they will still be a form of disseminated cancer. In medical circles, the term Carcinomatosis refers a situation in which multiple sites will have multiple secondaries. It was, strictly speaking, used to refer to carcinomas which are a type of epithelial cancers and not lymphomas, cancer of the lymphatic system or sarcomas either. The term has been diluted to mean all those cancers that have a characteristic spread. There are various types of Carcinomatosis: Types of Carcinomatosis It is important to note that Carcinomatosis by far and large refer to cancer tumors that originate from epithelial surfaces called adenocarcinomas while if the cancer cells are of mesenchymal origin or sarcomas then their dissemination is called sarcomatosis. The classification of Carcinomatosis is based on whether they affect the lungs or the other body cavities. 1. Lung Carcinomatosis There are two kinds of Carcinomatosis for the lungs;

Lymphangitic carcinomatosis This Carcinomatosis is formed when a cancerous tumor metastasizes into the lung. In the lungs, the tumors will form nodules that are not only distinct but will be spread by the lymph vessels that are found in the lungs. Up to seven percent of these cells will be spread. The effect is impairment of breathing mechanism which in addition will make the lungs to become stiffer and compression of the blood vessels that are located alongside the lymph vessels that have been distended.

Miliary carcinosis Miliary carcinosis is similar to military tuberculosis in terms of their radiographic appearance. They are characterized by a pattern of metastases which are not only modular but also small in sizes.

2. The other types of Carcinomatosis are found in the body cavities and which manifest by the growth of tumor cells along the surfaces of the organs lining the body cavities include:

Peritoneal Carcinomatosis

This Carcinomatosis affect the lining of the abdominal cavity with the most common being ovarian carcinomas. The cells produce fluids which can generate ascites. This process is very typical in carcinomatosis but is a rare occurrence in peritoneal sarcomatosis. The fluid produced can take two form, mucinous as is the case in pseudomyxoma peritonei or just plain serous as is the case in peritoneal carcinoma.

Pleural carcinosis

This is a type of Carcinomatosis that though it grows in body cavities, it is associated with poor prognosis in addition to the obvious malignant pleural effusion.

Leptomeningeal Carcinomatosis

This is a form of Carcinomatosis in which tumor grows central nervous system especially its meningeal covering. Carcinomatosis manifests itself differently according to the part of the body which it affects. The shortening of breath and haemoptysis, for, example is a symptom if it occur in the lungs. Others include: Jaundice if Carcinomatosis affects the live Neurological features and incessant headaches similar to migraines if the brain is affected. Pathological fractures and pain if the bones are involved.

http://carcinomatosis.net/

Carcinomatosis is a type of cancer which leads to development of carcinomas in multiple forms and simultaneously from the primary source of the cancerous cells. Though similar to

the other metastatic diseases, it is different in that it does not only involve the spread of the carcinomas from their sources to regional nodes because multiple sites will have multiple secondaries. There are many types of cancers whose carcinomas spread from source. Though some medical experts refer to all these forms as types of Carcinomatosis, the term should not include sarcomas or lymphomas and strictly speaking should only include carcinomas or epithelial cancers. Carcinomatosis is not easy to identify from the myriad of cancers and worse, treatment options are not completely curative but a control mechanism How Carcinomatosis is diagnosed The biggest challenge in the diagnosis of Carcinomatosis is that it presents itself with symptoms or diseases that are associated with other medical conditions. For example Carcinomatosis of the liver manifests as jaundice which can also result from other causes such as liver cirrhosis or gallstones. The latter diseases have to be tested for and ruled out before the jaundice is ruled out to be specifically as a result of Carcinomatosis. Of course there are other properties of cancerous cells that will be used to tell if the disease is as a result of Carcinomatosis or not. When the presence of carcinomas or cancerous cells can be identified with in a n organ to gether with the following diseases, then it is a strong indication of Carcinomatosis: Haemoptysis in the lungs Neurological diseases of the brain Pathological fracture of bones When Carcinomatosis is the biggest suspect, the doctors will seek to find a primary tumor which, to complicate the diagnosis process, may lack in up to six percent of the patients. Recent improvement in investigative technology is providing the much needed breakthrough for the process with histology being the leading breakthrough so far though it can be anaplastic and result in no convincing diagnosis at all. How Carcinomatosis is managed

Aforementioned, there is no realistic means of curing Carcinomatosis. Available common treatments for several types of cancers, like chemotherapy and radiotherapy produce a minimal effect, often palliative. Surgery is often performed on the carcinomas. Such surgery debaulks the tumour so that chemotherapy thoroughly destroys the infected epithelial tissues. For the liver cases, the metastases that are secondary to colorectal cancer may be resectioned leading to limited number of successes in different patients. On the flip side there are patients who have had considerable success with the mainstream chemotherapy and radiotherapy in combination to other treatment forms. For example, patients who have leptomeningeal metastases that are a secondary of cancer of the breast have been to improve to the point of recovery with a combination of intravenous chemotherapy, intrathecal chemotherapy, radiotherapy applied to spinal leptomeninges and radiotherapy for the whole brain. For the patients who have no hope of imptovement pain control and dyspnoea in palliative care or dying at home management techniques can still be used. Carcinomatosis is described as a condition in which multiple carcinomas develop simultaneously, usually after dissemination from a primary source. It implies more than spread to regional nodes and even more than just metastatic disease. The term is usually taken to mean that there are multiple secondaries in multiple sites. Strictly, it should be used only for epithelial cancers or carcinomas and not sarcomas or lymphomas but has been extended to include all types of cancer which have spread. The word is now used to describe conditions with more limited spread, as in:

Leptomeningeal carcinomatosis - involvement of leptomeninges through seeding via the cerebrospinal fluid, which occurs either by direct spread or via the bloodstream. Any cancer can cause this but adenocarcinomas are most commonly involved.[1] Lymphangitic carcinomatosis - diffuse infiltration of the lungs with obstruction of the lymphatic channels.[2] Peritoneal carcinomatosis - spread of metastases into the peritoneum, usually from ovarian and colorectal cancers.[3]

Presentation
Carcinomatosis may be a progression of known disease. It may be the presentation of recurrence or it may be the primary presenting feature. Presentation will depend upon where is affected.

In the lungs it may present as shortness of breath and haemoptysis.[2] In the liver it often presents as jaundice.[4] In the brain there may be headaches, vomiting and neurological features.[5] In bones there may be pain or pathological fracture.[6]

Differential diagnosis
When these features present, the question is whether this is part of the known disease or something else. For example, is jaundice due to metastatic carcinoma in the liver or gallstones? When carcinomatosis is the presenting feature it is usual to seek a primary tumour. In 5% of patients, none is found.[7] Histology may be anaplastic and give no help, although improvements in investigative technology are helping to narrow the differential diagnosis (see below).[8]

Investigations
The purpose of investigations are to confirm the nature of the disease and to assess its severity and extent.

In cases of unknown primary, FBC may show iron deficiency suggestive of gastrointestinal malignancy, microscopic haematuria may reveal occult genitourinary malignancy and occult blood may point to a colorectal cause. In cases where the primary is known, FBC, U&E, creatinine and liver function tests may indicate severity. Modern imaging techniques such as ultrasound, CT and MRI scanning as well as older investigations, such as CXR, provide very good information and an exploratory laparotomy is rarely required nowadays. It may be desirable to obtain tissue for histology. Techniques now employed to assist with differential diagnosis include:[8] o Light microscopy. o Immunohistochemistry - perioxidase-labelled antigen is used to identify specific tumour markers (eg prostate specific antigen). o Electron microscopy. o Chromosome studies - these are occasionally helpful (eg DNA amplification of Epstein Barr virus in suspected occult nasopharyngeal carcinoma). Tumour markers for leptomeningeal metastases have been identified.[9]

Management
Usually there is no realistic hope of curative therapy although chemotherapy and radiotherapy may have a palliative effect. Surgery may be palliative and 'debulking' of the tumour before chemotherapy may be helpful. Resection of liver metastases secondary to colorectal cancer has had some success in limited disease.[4] There are some subgroups of patients who do relatively well with treatment:

Multi-modality treatment (intrathecal chemotherapy, intravenous chemotherapy, whole brain radiotherapy and radiotherapy to the spinal leptomeninges) has been seen to improve survival rates in patients with leptomeningeal metastases secondary to breast cancer.[10] Lymphatic carcinomatosis can sometimes be stabilised, or at least the progression reduced, by chemotherapy.[11] This may be systemic or via infusion into the cerebrospinal fluid. Radiotherapy may be required if the tumour tissue is bulky or causing symptoms.[12] Peritoneal carcinomatosis can occasionally be treated with intraperitoneal and/or intravenous chemotherapy. Treatment can be started postoperatively or chemotherapy

drugs can even be instilled in the abdominal cavity during surgery. These approaches have resulted in demonstrable improvements in survival rates.[3] Transcatheter arterial chemoembolisation (TACE) has resulted in a successful outcome, particularly in patients with neuroendocrine tumours and colorectal metastases. A microcatheter is inserted into the hepatic blood supply and a combination of chemotherapeutic agents and embolic agents are injected.[4] Various ablative techniques have been used to destroy liver metastases, including freezing, microwaves, lasers and the use of alternating current within the radiofrequency range.[4] The surgical treatment of bone metastases can improve life expectancy and the quality of life.[13]

For patients who are incurable, a frank and honest discussion must take place. This may require more than one session and the skills for breaking bad news are required. Other considerations may be dying at home and dyspnoea in palliative care. Pain control in terminal care and nausea and vomiting in palliative care may also warrant attention. Palliative care should not be seen as a failure. It is a very demanding and very rewarding aspect of medical practice.[14]
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Further reading & references


1. Grossman SA, Krabak MJ; Leptomeningeal carcinomatosis. Cancer Treat Rev. 1999 Apr;25(2):103-19. 2. Khan AN; Lymphangitic Carcinomatosis, eMedicine, Feb 2008 3. Specialty Section for the Treatment of Peritoneal Carcinomatosis; Surgicaloncology.com 2008 4. Khan AN; Liver Metastases, eMedicine, Feb 2009 5. Weil RJ, Palmieri DC, Bronder JL, et al; Breast cancer metastasis to the central nervous system. Am J Pathol. 2005 Oct;167(4):913-20. 6. Mercadante S; Malignant bone pain: pathophysiology and treatment. Pain. 1997 Jan;69(12):1-18. 7. Hospital Guidelines - Palliative Care; Calderdale Royal Hospital 2005 8. Tan WW; Metastatic Cancer, Unknown Primary Site, eMedicine, Mar 2009 9. Walbert T, Groves MD; Known and emerging biomarkers of leptomeningeal metastasis and its response to Future Oncol. 2010 Feb;6(2):287-97. 10. Rudnicka H, Niwinska A, Murawska M; Breast cancer leptomeningeal metastasis--the role of multimodality treatment. J Neurooncol. 2007 Aug;84(1):57-62. Epub 2007 Feb 20. 11. Ikezoe J, Godwin JD, Hunt KJ, et al; Pulmonary lymphangitic carcinomatosis: chronicity of radiographic findings in long-term survivors. AJR Am J Roentgenol. 1995 Jul;165(1):49-52. 12. Chamberlain MC; Leptomeningeal metastasis. Semin Neurol. 2010 Jul;30(3):236-44. Epub 2010 Jun 24. 13. Cappuccio M, Bandiera S, Babbi L, et al; Management of bone metastases. Eur Rev Med Pharmacol Sci. 2010 Apr;14(4):407-14. 14. Vejlgaard T, Addington-Hall JM; Attitudes of Danish doctors and nurses to palliative and terminal care. Palliat Med. 2005 Mar;19(2):119-27.

http://www.patient.co.uk/doctor/Carcinomatosis.htm

Peritoneal carcinomatosis is a type of secondary cancer that affects the lining of the abdominal cavity, called the peritoneum. It occurs when cancer metastasizes from another part of the body and implants into the lining. Peritoneal carcinomatosis most commonly follows severe or untreated pancreas, ovarian, stomach, and colon cancer. Symptoms can vary, but many people experience extreme fatigue and abdominal pain. Quick, aggressive treatment in the form of medications and surgery is vital in preventing fatal complications. Some cancers are more likely to metastasize quickly to the peritoneum than others. Not surprisingly, tumors in organs that are inside or adjacent to the abdominal cavity present the highest risk of developing peritoneal carcinomatosis. People who have widespread cancer in their stomachs and intestines are at an especially high risk, especially if they cause ulcers and ruptures. Ovarian, liver, and pancreatic cancer are notorious for spreading quickly as well. Occasionally, a tumor far from the abdomen or a bone cancer can result in peritoneal carcinomatosis after cancerous cells invade lymph nodes and the bloodstream. The most common symptoms of peritoneal carcinomatosis include acute or chronic aches, cramps, bloating, and full-body fatigue. Many symptoms are caused when excess fluid accumulates in the abdominal cavity, a direct consequence of nearby tumor activity. Other problems such as breathing difficulties, digestive problems, and chest pains may be present as well, depending on the extent and location of the original cancer. In most cases, doctors are already aware that patients have primary cancers before they develop peritoneal carcinomatosis. Most people are already receiving some form of cancer treatment for the metastasis. Ultrasounds and computerized tomography scans are taken to look for signs of tumors, fluid accumulation, and damaged tissue in the abdominal cavity. If something suspicious is found, a tissue biopsy may be necessary to confirm it is cancer. Treatment decisions are made right away to provide patients with the best possible chances of recovery. Surgery is the treatment of choice when tumors are small and isolated in the peritoneum. If an entire tumor can be removed and the primary cancer treated effectively, the patient has a good prognosis. Cancers in their later stages are typically treated with a combination of surgery, chemotherapy, and radiation. Even when it appears that treatment is successful, the condition can become a recurring problem. Regular checkups and exams are vital elements of follow-up care to make sure future problems are kept to a minimum.
Diagnosis

Diagnosis of LC is made with positive CSF cytologic results, subarachnoid metastases identified on radiologic studies, or a history and physical examination suggestive of LC along with abnormal CSF findings. Lumbar puncture

The standard diagnostic procedure Positive CSF cytology is found on the initial lumbar puncture in 50-70% and in nearly all cases after 3 attempts Increased CSF pressure and elevated CSF protein are also commonly found.

Imaging studies

Gadolinium-enhanced multiplanar MRI is the preferred imaging modality over CT because of its sensitivity and specificity MRI findings considered diagnostic of LC include leptomeningeal enhancement of the brain, spinal cord, cauda equina, or subependymal areas, which extend into the sulci of the cerebrum or folia of the cerebellum MRI of the spinal cord can show nerve-root thickening, cord enlargement, intraparenchymal and subarachnoid nodules, or epidural compression

See Workup for more detail.


Management

Leptomeningeal carcinomatosis is incurable and difficult to treat. Treatment goals include improvement or stabilization of the patient's neurologic status, prolongation of survival, and palliation. Most patients require a combination of surgery, radiation, and chemotherapy. The standard therapies are (1) radiation therapy to symptomatic sites and regions where imaging has demonstrated bulk disease and (2) intrathecal chemotherapy. Radiation palliates local symptoms, relieves CSF flow obstruction, and treats areas such as nerve-root sleeves, Virchow-Robin spaces, and the interior of bulky lesions that chemotherapy does not reach. Intrathecal chemotherapy treats subclinical leptomeningeal deposits and tumor cells floating in the CSF, preventing further seeding.[2] Cytarabine (Ara-C), methotrexate (MTX), and thiotepa are 3 agents routinely administered. Supportive care for patients includes analgesia with opioids, anticonvulsants for seizures, antidepressants, and anxiolytics. Attention problems and somnolence from whole-brain radiation can be treated with psychostimulants or modafinil

Pathophysiology
Metastatic seeding of the leptomeninges may be explained by the following 6 postulated mechanisms: (1) hematogenous spread to choroid plexus and then to leptomeninges, (2) primary hematogenous metastases through the leptomeningeal vessels, (3) metastasis via the Batson venous plexus, (4) retrograde dissemination along perineural lymphatics and sheaths, (5) centripetal extension along perivascular and perineural lymphatics from axial lymphatic nodes and vessels through the intervertebral and possibly from the cranial foramina to the leptomeninges, and (6) direct extension from contiguous tumor deposits. CSF flow then seeds the tumor cells widely, with infiltration greatest at the basilar cisterns and dorsal surface of the spinal cord, particularly the cauda equina. Signs and symptoms are usually attributable to obstruction of CSF flow by tumor adhesions that leads to one of the following:

Increased intracranial pressure (ICP) or hydrocephalus Local tumor infiltration in the brain or spinal cord that causes cranial-nerve palsies or radiculopathies

Alterations in the metabolism of nervous tissue that cause seizures, encephalopathy, or focal deficits Occlusion of blood vessels as they cross the subarachnoid, leading to infarcts

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