Paraneoplastic Syndrome

People with this inherited disorder are likely to develop several kinds of tumors, including, in some cases, renal cell carcinoma.

As mentioned before, an increase of estrogen in men can help nurture this type of cancer. As breast cells grow and divide quicker in response to the hormone. Genetics also play a small role, as in if a child inherits a corrupted gene from one of his parents. Although, the real cause has yet to be pinpointed by scientists.

This syndrome is from a mutation of a gene on chromosome 15 and this causes problems in the production of fibrillin-1 which is a protein that is an important part of connective tissue. The name for the gene is FBN1. Basically, it is the “glue” that helps to support the tissues in the human body. A child born to a parent with this syndrome has a 50% of having it. However, in the remaining 25%, neither parent has the disease which gives them a 1 in 10,000 chance of having a child with this disorder. When a child of two unaffected parents is born with it then the genetic mutation occurs in either the egg or sperm cell at the time of conception.

Paragangliomas are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, small organs consisting mainly of neuroendocrine cells that are derived from the embryonic neural crest and have the ability to secrete catecholamines (1). They are closely related to pheochromocytomas, which are sometimes referred to as intra-adrenal paragangliomas (2). The majority appear to be sporadic, but approximately one-third to one-half are associated with an inherited syndrome (3, 4). The four genetic syndromes that are associated are: multiple endocrine neoplasia 2A and 2B, neurofibromatosis type 1, von Hippel Lindau, and the Carney-Stratakis dyad. The male-to-female ratio is approximately equal among patients with hereditary paraganglioma,

A permanent change in a gene that can be passed on to children. The rare, early-onset familial

It is an inherited genetic disease. It is passed down in families in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show any symptoms. Around 1 person in every 150 people are thought to be carriers. There are two types of Krabbe disease the infantile and the late on-set. The infantile form, which affects 85 to 90% of people with

This abnormality is caused by a gene translocation

Paraneoplastic syndromes are rare disorders triggered by a person's immune system in response to a cancerous tumor. Paraneoplastic syndromes can affect many areas of the body systems, including the nervous system, hormone (endocrine) system, skin (dermatologic) system, blood (hematologic) system, and joints (rheumatologic) system.

Propelled malignancy is growth that has spread to far off parts of your body. This can incorporate your liver, brain or bones. Treatment relies on upon your general wellbeing and how best in class the growth is. It might be conceivable to hold the tumor under control by surgery or chemotherapy. Radiotherapy and chemotherapy can both help control side effects by contracting a disease with the goal that it doesn't bring about weight and torment and moderates its development. This is called palliative treatment.

Exact cause remains unidentified, however it is believed that the patient’s genes and environment may be contributing factors.

In rare cases, the disease may be due to a gene mutation that happens spontaneously.

Oncologic Emergencies are both life threatening and detrimental to a patient’s health. As stated by (Tan), “An oncologic emergency is a clinical condition resulting from a structural or metabolic change caused by cancer or its treatment that requires immediate medical intervention to prevent loss of life or quality of life.” Oncologic emergencies can be further classified as metabolic, hematologic, neurologic, or cardiovascular. Superior vena cava syndrome and spinal cord compression can be considered oncologic emergencies. Superior vena cava syndrome is classified as cardiovascular while spinal cord compression is classified as neurologic and/or structural. Treatment options vary depending on the severity of the disease. When

Paraneoplastic neurologic syndromes (PNS) are syndromes that result from dysfunction of the nervous system, caused by a benign or malignant tumor via mechanisms other than direct tumor cell infiltration, metastasis, coagulopathy, infection or any treatment side effects [1]. All parts of the central and peripheral nervous system may be affected by PNS and, as a result, signs and symptoms are diverse. Most PNS of the central nervous system have in common a subacute course leaving the patients severely disabled in weeks to months and inflammatory changes in the CSF, including moderate lymphocytic pleocytosis, increased protein levels and IgG index and presence of CSF-specific oligoclonal bands[2]. Early recognition may be difficult

Once a paraneoplastic etiology is strongly suspected, a careful search for the underlying neoplasm is mandatory. If detailed history taking, thorough physical examination and high resolution computed tomography (CT) of chest, abdomen and pelvis do not show a primary tumor, whole body positron-emission tomography (PET) or PET/CT may be the best screening method for locating the occult cancer(Patel et al. 2008). Besides, the type of antibody and paraneoplastic syndrome may suggest a specific underlying tumor and indicate more directed diagnostic tests, such as mammography (or MRI) when breast cancer is suspected.

Paraganglioma is a rare neoplasm of neuroendocrine origin which may develop at many different anatomical sites including head and neck, thorax, and abdomen. The occurrence of paraganglioma in lower spine is rare, but well established as one of the differential. The investigation of choice pre-operatively is magnetic resonance imaging4, which usually shows a very well circumscribed partially cystic mass which is iso- or hypointense to spinal cord on T1-weighted images, and hyperintense on T2-weighted images4. These MRI features are similar to other cauda equine tumors such as ependymoma2. However sometimes MRI may show serpentine congested ectatic blood vessels with “cap sign” (a low intensity signal rim) on T2-weighted images which are considered diagnostic for paragangliomas2,7.