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Carcinomatosis is described as a condition in which multiple carcinomas develop simultaneously, usually after dissemination (distributed over a considerable area) 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.
- Carcinosis, or carcinomatosis, is disseminated cancer, forms of metastasis, whether used generally or in specific patterns of spread.
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 any widespread dissemination of cancer in the body, and also conditions with specific anatomical spread, as in:
- Leptomeningeal carcinomatosis
- Involvement of leptomeninges through seeding via the cerebrospinal fluid, which occurs either by direct spread or via the bloodstream.
- It is an uncommon and usually late complication of cancer.
- Any cancer can cause this but adenocarcinomas are most commonly involved. Breast cancer is one of the most common causes.
- Presentation is very variable, and may be nonspecific (for example, headache or confusion) or with focal or multifocal neurological deficits (for example, cranial nerve palsies).
- Diagnosis is based on careful history and neurological examination, cerebrospinal fluid cytology and neuroimaging (preferably gadolinium-enhanced MRI).
- Pulmonary lymphangitic carcinomatosis
- Diffuse infiltration of the lungs with obstruction of the lymphatic channels.
- May occur with a variety of different cancers, most commonly breast, stomach, pancreas, lung, and prostate.
- Lymphangitic carcinomatosis can also affect the kidneys and usually presents with acute kidney injury (AKI)[4].
- Peritoneal carcinomatosis
- Spread of metastases into the peritoneum, usually from gynecological (usually ovarian) and gastrointestinal cancers.
- The occurrence of peritoneal carcinomatosis has been shown to significantly decrease overall survival in patients with liver and/or extraperitoneal metastases from gastrointestinal cancer.
Symptoms
- Will depend upon what area(s) is affected.
Diagnosis
Testing is to confirm the nature of the disease and to assess its severity and extent.
- Blood tests: in cases of unknown primary, FBC may show iron deficiency suggestive of gastrointestinal malignancy, microscopic hematuria 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 LFTs may indicate severity.
- Imaging: newer imaging techniques such as ultrasound, CT and MRI scanning as well as older investigations, such as CXR, provide very good information although an exploratory laparotomy may sometimes be required for peritoneal carcinomatosis. As above, gadolinium-enhanced MRI is the preferred first-choice investigation for suspected leptomeningeal involvement.
- Biopsy: it may be desirable to obtain tissue for histology. Transbronchial biopsy provides definitive diagnosis for pulmonary lymphangitic carcinomatosis[3]. Histological analysis helps guide management choice and is one of the factors to consider in deciding on whether aggressive treatment with surgery and/or chemotherapy is appropriate. The evolution of molecular pathology has allowed it to contribute significantly to management decisions.
Treatment
- As this condition may occur from numerous etiologies, specific management strategy options are also numerous and may be targeted towards the specific cancer. In many cases 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 tumor before chemotherapy may be helpful. Resection of liver metastases secondary to colorectal cancer has had some success in limited disease. There are some subgroups of patients who do relatively well with treatment.
- Multimodality treatment (palliation with surgery, radiation, and/or chemotherapy, administered either systemically or directly into the cerebral spinal fluid) may be used in patients with leptomeningeal metastases secondary to breast cancer although prognosis remains poor. A multimodality approach (of cytoreduction surgery followed by hyperthermic intraperitoneal chemotherapy) is also used in selected people with peritoneal carcinomatosis and may improve prognosis significantly in some cases.