Intravenous leiomyomatosis

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Intravenous leiomyomatosis
IntravascularLeiomyoma.jpg
Intravenous Leiomyoma

Intravenous leiomyomatosis is a rare condition seen exclusively in women in which leiomyomata, benign smooth muscle tumors, are found in veins. The masses are benign-appearing but can spread throughout the venous system leaving the uterus and even cause death when growing into the heart from the IVC. [1] While the possibility that these arose de novo from the smooth muscle in the blood vessel wall was considered, chromosomal analysis suggests a uterine origin. [2] Intravenous leiomyomata are usually but not always associated with uterine fibroids, and tend to recur.

Smooth muscle

Smooth muscle is an involuntary non-striated muscle. It is divided into two subgroups; the single-unit (unitary) and multiunit smooth muscle. Within single-unit cells, the whole bundle or sheet contracts as a syncytium.

Uterus major female hormone-responsive reproductive sex organ of most mammals including humans

The uterus or womb is a major female hormone-responsive secondary sex organ of the reproductive system in humans and most other mammals. In the human, the lower end of the uterus, the cervix, opens into the vagina, while the upper end, the fundus, is connected to the fallopian tubes. It is within the uterus that the fetus develops during gestation. In the human embryo, the uterus develops from the paramesonephric ducts which fuse into the single organ known as a simplex uterus. The uterus has different forms in many other animals and in some it exists as two separate uteri known as a duplex uterus.

Contents

This condition is related to benign metastasizing leiomyoma, in which the masses appear in more distant locations such as the lung and lymph nodes. [3]

Lung essential respiration organ in many air-breathing animals

The lungs are the primary organs of the respiratory system in humans and many other animals including a few fish and some snails. In mammals and most other vertebrates, two lungs are located near the backbone on either side of the heart. Their function in the respiratory system is to extract oxygen from the atmosphere and transfer it into the bloodstream, and to release carbon dioxide from the bloodstream into the atmosphere, in a process of gas exchange. Respiration is driven by different muscular systems in different species. Mammals, reptiles and birds use their different muscles to support and foster breathing. In early tetrapods, air was driven into the lungs by the pharyngeal muscles via buccal pumping, a mechanism still seen in amphibians. In humans, the main muscle of respiration that drives breathing is the diaphragm. The lungs also provide airflow that makes vocal sounds including human speech possible.

See also

Uterine fibroids

Related Research Articles

Leiomyoma cell type benign neoplasm that is a benign tumor of smooth muscle cells

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.

Adenomyosis medical condition characterized by the presence of glandular tissue in muscle

Adenomyosis is a gynecologic medical condition characterized by the abnormal presence of endometrial tissue within the myometrium. When endometrial tissue is present abnormally entirely outside the uterus, it is considered to be a similar but distinct medical condition, endometriosis. The two conditions are found together in many cases, but often occur separately. Before being recognized as a distinct condition, adenomyosis was called endometriosis interna. The less-commonly-used term "adenomyometritis" is a more specific name for the condition, specifying involvement of the uterus.

Vaginal bleeding is any bleeding through the vagina, including bleeding from the vaginal wall itself, as well as bleeding from another location of the female reproductive system, often the uterus. Generally, it is either part of a normal menstrual cycle or is caused by hormonal or other problems of the reproductive system, such as abnormal uterine bleeding. Vaginal bleeding may occur at any age, but always needs investigation when encountered in children or in postmenopausal women. Vaginal bleeding during pregnancy may indicate a possible pregnancy complication that needs to be medically addressed.

Gynecologic ultrasonography

Gynecologic ultrasonography or gynecologic sonography refers to the application of medical ultrasonography to the female pelvic organs as well as the bladder, the adnexa, and the recto-uterine pouch. The procedure may lead to other medically relevant findings in the pelvis.

Uterine fibroid uterine benign neoplasm derived from the smooth muscle layer of the uterus

Uterine fibroids, also known as uterine leiomyomas or fibroids, are benign smooth muscle tumors of the uterus. Most women have no symptoms while others may have painful or heavy periods. If large enough, they may push on the bladder causing a frequent need to urinate. They may also cause pain during sex or lower back pain.A woman can have one uterine fibroid or many. Occasionally, fibroids may make it difficult to become pregnant, although this is uncommon.

Myometrium

The myometrium is the middle layer of the uterine wall, consisting mainly of uterine smooth muscle cells, but also of supporting stromal and vascular tissue. Its main function is to induce uterine contractions.

Uterine myomectomy myomectomy

Myomectomy, sometimes also fibroidectomy, refers to the surgical removal of uterine leiomyomas, also known as fibroids. In contrast to a hysterectomy the uterus remains preserved and the woman retains her reproductive potential.

Leiomyosarcoma malignant smooth muscle cancer that can arise almost anywhere in the body, but is most common in the uterus, abdomen, or pelvis

Leiomyosarcoma, also referred to as LMS, is a malignant (cancerous) smooth muscle tumor. A benign tumor originating from the same tissue is termed leiomyoma. While it has been believed that leiomyosarcomas do not arise from leiomyomas, there are leiomyoma variants for which classification is evolving.

Uterine artery embolization

Uterine artery embolization is a procedure where an interventional radiologist uses a catheter to deliver small particles that block the blood supply to the uterine body. The procedure is done for the treatment of uterine fibroids and adenomyosis. Given that this minimally invasive procedure is commonly used in the treatment of uterine fibroids it is also called uterine fibroid embolization (UFE).

Fumarase protein-coding gene in the species Homo sapiens

Fumarase is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to malate. Fumarase comes in two forms: mitochondrial and cytosolic. The mitochondrial isoenzyme is involved in the Krebs Cycle, and the cytosolic isoenzyme is involved in the metabolism of amino acids and fumarate. Subcellular localization is established by the presence of a signal sequence on the amino terminus in the mitochondrial form, while subcellular localization in the cytosolic form is established by the absence of the signal sequence found in the mitochondrial variety.

HMGA2 protein-coding gene in the species Homo sapiens

High-mobility group AT-hook 2, also known as HMGA2, is a protein that, in humans, is encoded by the HMGA2 gene.

WNT7A protein-coding gene in the species Homo sapiens

Protein Wnt-7a is a protein that in humans is encoded by the WNT7A gene.

Aggressive angiomyxoma

Angiomyxoma is a myxoid tumor involving the blood vessels.

Endometrial stromal sarcoma Human disease

Endometrial stromal sarcoma is a malignant subtype of endometrial stromal tumor arising from the stroma of the endometrium rather than the glands. There are three grades for endometrial stromal tumors, as follows. It was previously known as endolymphatic stromal myosis because of diffuse infiltration of myometrial tissue or the invasion of lymphatic channels.

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.

Hereditary leiomyomatosis and renal cell cancer syndrome Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a hereditary cancer syndrome characterized by a predisposition to cutaneous and uterine leiomyomas and, in some families, to renal cell cancer

Reed’s syndrome is a rare inherited condition characterised by multiple cutaneous leiomyomas and, in women, uterine leiomyomas. It predisposes for renal cell cancer, an association denominated hereditary leiomyomatosis and renal cell cancer, and it is also associated with increased risk of uterine leiomyosarcoma. The syndrome is caused by a mutation in the fumarate hydratase gene, which leads to an accumulation of fumarate. The inheritance pattern is autosomal dominant.

A morcellator is a surgical instrument used for division and removal of large masses of tissues during laparoscopic surgery. In laparoscopic hysterectomy the uterus is minced up, or morcellated, into smaller pieces inside the woman's abdominal cavity in order to extract from the abdomen. It can consist of a hollow cylinder that penetrates the abdominal wall, ending with sharp edges or cutting jaws, through which a grasper can be inserted to pull the mass into the cylinder to cut out an extractable piece.

Relugolix chemical compound

Relugolix, sold under the brand name Relumina, is a gonadotropin-releasing hormone antagonist medication which is used in the treatment of uterine fibroids in Japan. It is also under development for use in other countries and for the additional indications of endometriosis and prostate cancer. Unlike most other GnRH modulators, but similarly to elagolix, relugolix is a non-peptide and small-molecule compound and is orally active. As of February 2019, it is in phase III clinical trials for endometriosis and prostate cancer. The medication was approved for use for the treatment of uterine fibroids in Japan in January 2019. A New Drug Application of relugolix for uterine fibroids is expected to be submitted in the United States late 2019.

Serdar Bulun is a gynecologist, with a special interest in the common gynecologic diseases, endometriosis and uterine fibroids.

Benign metastasizing leiomyoma is a rare condition characterized by the growth of uterine leiomyoma in the other regions especially the lungs.

References

  1. Worley Jr, M.; Aelion, A.; Caputo, T.; Kent, K.; Salemi, A.; Krieger, K.; Goldstein, M.; Kuo, D.; Slomovitz, B. (2009). "Intravenous leiomyomatosis with intracardiac extension: a single-institution experience". American Journal of Obstetrics and Gynecology. 201 (6): 574.5e1–5. doi:10.1016/j.ajog.2009.06.037. PMC   4309800 . PMID   19729144.
  2. Dal Cin, P.; Quade, B.; Neskey, D.; Kleinman, M.; Weremowicz, S.; Morton, C. (2003). "Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24)". Genes, Chromosomes & Cancer. 36 (2): 205–206. doi:10.1002/gcc.10159. PMID   12508249.
  3. Patton, K.; Cheng, L.; Papavero, V.; Blum, M.; Yeldandi, A.; Adley, B.; Luan, C.; Diaz, L.; Hui, P.; Yang, X. J. (2006). "Benign metastasizing leiomyoma: clonality, telomere length and clinicopathologic analysis". Modern Pathology. 19 (1): 130–140. doi:10.1038/modpathol.3800504. PMID   16357844.