Intravenous leiomyomatosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
Synonyms and keywords: Nesidioblastoma, IVLM
Overview
Intravenous leiomyomatosis is also refered as [IVL]. Intravenous leiomyomatosis is characterized by the extension into venous channels of histologically benign smooth muscle tumor arising from either the wall of a vessel or from a uterine leiomyoma. The etiology of intravenous leiomyomatosis is unclear. Intravenous leiomyomatosis must be differentiated from other diseases such as renal malignancies and sarcoma. The median age is 45 years, with patients ranging from 26 to 70 years old. Only females may develop intravenous leiomyomatosis. Surgery is the mainstay of therapy for intravenous leiomyomatosis. It can grow in to lymphatics /veins.
Historical Perspective
- Intravenous leiomyomatosis first described in 1896 by German Pathologist Birch-Hirschfeld.[1]
- First case of leiomyo-matosis of pelvic origin with intravascular extension to cardiac cavities was described in 1907 in Germany by Dürck and Hörmann.[2]
Classification
- There is no established system for the classification of intravenous leiomyomatosis.
Pathophysiology
- Intravenous leiomyomatosis is characterized by the extension into venous channels of histologically benign smooth muscle tumor arising from either the wall of a vessel or from a uterine leiomyoma.[3]
- Approximately 40% of leiomyomata have cytogenetic abnormalities.
- They are benign tumors of uterus that extend to veins system but do not invade the surrounding tissues [4]
- They contains receptors to Estrogen and progesterone and hence response to these hormones [5]
- It is also referred as quasi-malignant behavior due to its speedy spreading behavior. [6]
- Patients are exclusively female, and the majority are white, premenopausal, and multiparous.[7]
- Intravenous leiomyomatosis should be considered in young women with cardiac symptoms who have a right atrial mass as well as a pelvic mass or who have previously undergone hysterectomy for leiomyoma uterus with intravenous involvement.[8]
- On microscopy it is seen as benign, well-differentiated tumor with smooth muscle growing within veins as worm-like projections.
- Immunohistochemical studies show presence of desminand smooth muscle actin, confirming their smooth muscle nature.
- Tumor cells showed bizarre nuclear morphology with hyperchromatic multilobated nuclei, the mitotic activity was low, with mitotic index of less than 1 per 50 high-power fields
- It has been divided in subtitles based on microscopic appearance as namely, cellular, myxoid and bizarre,however it is not commonly used.
Causes
- The etiology of intravenous leiomyomatosis is unclear.[9]
- Only two cytogenetic reports in IVL and both exhibited a karyotype with a der(14)t(12;14)(q15;q24) and two normal copies of chromosome 12 which has close association to uterine leiomyoma genetics
Differentiating Intravenous Leiomyomatosis from other Diseases
- Intravenous leiomyomatosis must be differentiated from other conditions such as intravenous thrombus, leiomyosarcoma, right atrial myxoma and tumor thrombosis with malignant carcinoma, for example, renal carcinoma, hepatocellular carcinoma, adrenal cortical carcinoma.[10]
Epidemiology and Demographics
- The median age at the time of diagnosis is 45 years, with patients ranging from 26 to 70 years old. [11]
- Female are exclusively affected with intravenous leiomyomatosis.
- Patients affected are of reproductive age group mostly around fifth decade of life.
- Age from 28 to 76 years (median, 42 years).
- It is diagnosis only of female [12]
- They are seen more in white female.[13]
Risk Factors
- Common risk factors in the development of intravenous leiomyomatosis are age, cytogenetics , and prior history of this disorder.
Screening
- There is insufficient evidence to recommend routine screening for intravenous leiomyomatosis.
Natural History, Complications and Prognosis
- If left untreated, patients with intravenous leiomyomatosis may progress to develop congestive heart failure, venous obstruction, and sudden death.[14]
- Prognosis is generally excellent.
Diagnosis
Diagnostic Criteria
- There is no specific diagnostic Criteria for intravenous leiomyomatosis.
- Diagnosis may be challenging. However, a history of A history of hysterectomy or the presence of uterine leiomyomas may be helpful.[15]
History and Symptoms
- Patients may be asymptomatic or have symptoms of uterine leiomyomas, syncopal episodes, and dyspnea on exertion.[16] [17] [18]
- Symptoms of intravenous Leiomyomatosis may include dyspnoea, loss of consciousness, lower extremity edema, chest pain, abdominal pain, and fatigue.
Physical Examination
- Patients with intravenous Leiomyomatosis usually depends on location and extension which may include jugular venous distention, increased pulse rate, fluid shift in abdominal exam, and lower extremity edema.
Laboratory Findings
- There are no specific laboratory findings associated with intravenous Leiomyomatosis.
Electrocardiogram
- There are no ECG findings associated with intravenous Leiomyomatosis.
X-ray
- There are no x-ray findings associated with intravenous Leiomyomatosis.
Echocardiography or Ultrasound
- Echocardiography/ultrasound may be helpful in the diagnosis of intravenous Leiomyomatosis.
- Findings on an echocardiography/ultrasound suggestive of/diagnostic of intravenous Leiomyomatosis include [finding 1], [finding 2], and [finding 3].
CT scan
- Pelvic CT scan may be helpful in the diagnosis of intravenous leiomyomatosis.[19]
- Findings on CT scan suggestive of/diagnostic of intravenous leiomyomatosis include an irregular mass exhibiting heterogeneous enhancement with extension into adjacent veins.
MRI
- Pelvic MRI may be helpful in the diagnosis of intravenous leiomyomatosis.[15]
- Findings on MRI suggestive of/diagnostic of intravenous leiomyomatosis include a leiomyomatous lesion or a distorted uterus with projections into vasculature.
- The tumor usually shows low signal intensity similar to that of smooth muscle on T2-weighted images.
Treatment
Medical Therapy
- There is no treatment for intravenous leiomyomatosis; the mainstay of therapy is surgery.
Surgery
- Surgery is the treatment of choice, and complete removal of the tumor is mandatory.
- Recurrences up to 15 years after the primary occurrence in the patients with Incomplete resection of the tumor.[20]
- Bilateral oophorectomy causes shutdown of hormonal stimulation hence helps preventing recurrence.[21]
- Surgical removal can be done in single or staged procedures,[22]
- If staged procedure is chosen than it is done with abdominopelvic and intrathoracic components in two separate operations within a short time interval. [23]
- In literature it is seen the Complete excision with one-stage operation was used in 19 (27.9%) and Complete excision with two-stage operation in 29 (42.7%)
Primary Prevention
- There are no primary preventive measures available for [IVL].
Secondary Prevention
- There are no secondary preventive measures available for [IVL].
References
- ↑ Quade BJ, Dal Cin P, Neskey DM, Weremowicz S, Morton CC (2002). "Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case". Mod Pathol. 15 (3): 351–6. doi:10.1038/modpathol.3880529. PMID 11904348.
- ↑ https://www.ncbi.nlm.nih.gov/pubmed/23563052
- ↑ Mariyappa N, Manikyam UK, Krishnamurthy D, Preeti K, Agarwal Y, Prakar U (2012). "Intravenous leiomyomatosis". Niger J Surg. 18 (2): 105–6. doi:10.4103/1117-6806.103122. PMC 3762011. PMID 24027407.
- ↑ Dal Cin P, Quade BJ, Neskey DM, Kleinman MS, Weremowicz S, Morton CC (2003). "Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24)". Genes Chromosomes Cancer. 36 (2): 205–6. doi:10.1002/gcc.10159. PMID 12508249.
- ↑ Dal Cin P, Quade BJ, Neskey DM, Kleinman MS, Weremowicz S, Morton CC (2003). "Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24)". Genes Chromosomes Cancer. 36 (2): 205–6. doi:10.1002/gcc.10159. PMID 12508249.
- ↑ Quade BJ, Dal Cin P, Neskey DM, Weremowicz S, Morton CC (2002). "Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case". Mod Pathol. 15 (3): 351–6. doi:10.1038/modpathol.3880529. PMID 11904348.
- ↑ Quade BJ, Weremowicz S, Neskey DM, Vanni R, Ladd C, Dal Cin P; et al. (2003). "Fusion transcripts involving HMGA2 are not a common molecular mechanism in uterine leiomyomata with rearrangements in 12q15". Cancer Res. 63 (6): 1351–8. PMID 12649198.
- ↑ Dal Cin P, Quade BJ, Neskey DM, Kleinman MS, Weremowicz S, Morton CC (2003). "Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24)". Genes Chromosomes Cancer. 36 (2): 205–6. doi:10.1002/gcc.10159. PMID 12508249.
- ↑ "Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations1". line feed character in
|title=at position 54 (help) - ↑ Lee, Sak; Kim, Do-Kyun; Narm, Kyoung Shik; Cho, Sang-Ho (2011). "Pulmonary Artery Embolization of Intravenous Leiomyomatosis Extending into the Right Atrium". The Korean Journal of Thoracic and Cardiovascular Surgery. 44 (3): 243–246. doi:10.5090/kjtcs.2011.44.3.243. ISSN 2233-601X.
- ↑ Canzonieri V, D'Amore ES, Bartoloni G, Piazza M, Blandamura S, Carbone A (1994). "Leiomyomatosis with vascular invasion. A unified pathogenesis regarding leiomyoma with vascular microinvasion, benign metastasizing leiomyoma and intravenous leiomyomatosis". Virchows Arch. 425 (5): 541–5. PMID 7850080.
- ↑ Poliquin V, Victory R, Vilos GA (2008). "Epidemiology, presentation, and management of retroperitoneal leiomyomata: systematic literature review and case report". J Minim Invasive Gynecol. 15 (2): 152–60. doi:10.1016/j.jmig.2007.12.009. PMID 18312983.
- ↑ Poliquin V, Victory R, Vilos GA (2008). "Epidemiology, presentation, and management of retroperitoneal leiomyomata: systematic literature review and case report". J Minim Invasive Gynecol. 15 (2): 152–60. doi:10.1016/j.jmig.2007.12.009. PMID 18312983.
- ↑ Schäfer HM, Isaak A, Gürke L (2017). "Case report of an intracaval leiomyomatosis 10 months after complete hysterectomy". Int J Surg Case Rep. 35: 1–3. doi:10.1016/j.ijscr.2017.03.031. PMC 5394212. PMID 28414995.
- ↑ 15.0 15.1 Fasih, Najla; Prasad Shanbhogue, Alampady K.; Macdonald, David B.; Fraser-Hill, Margaret A.; Papadatos, Demetrios; Kielar, Ania Z.; Doherty, Geoffrey P.; Walsh, Cynthia; McInnes, Matthew; Atri, Mostafa (2008). "Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations". RadioGraphics. 28 (7): 1931–1948. doi:10.1148/rg.287085095. ISSN 0271-5333.
- ↑ Nakayama Y, Kitamura S, Kawachi K, Kawata T, Fukutomi M, Hasegawa J; et al. (1994). "Intravenous leiomyomatosis extending into the right atrium". Cardiovasc Surg. 2 (5): 642–5. PMID 7820530.
- ↑ Moorjani N, Kuo J, Ashley S, Hughes G (2005). "Intravenous uterine leiomyosarcomatosis with intracardial extension". J Card Surg. 20 (4): 382–5. doi:10.1111/j.1540-8191.2005.200476.x. PMID 15985146.
- ↑ Poliquin V, Victory R, Vilos GA (2008). "Epidemiology, presentation, and management of retroperitoneal leiomyomata: systematic literature review and case report". J Minim Invasive Gynecol. 15 (2): 152–60. doi:10.1016/j.jmig.2007.12.009. PMID 18312983.
- ↑ Xu, Zhi-Feng (2013). "Uterine intravenous leiomyomatosis with cardiac extension: Imaging characteristics and literature review". World Journal of Clinical Oncology. 4 (1): 25. doi:10.5306/wjco.v4.i1.25. ISSN 2218-4333.
- ↑ Yanagiya A, Yamada O, Nanbu T, Hamada H, Takada J, Matsuura M; et al. (2015). "[One-stage resection of intravenous leiomyomatosis extending into the right atrium]". Kyobu Geka. 68 (3): 188–91. PMID 25743551.
- ↑ Castelli P, Caronno R, Piffaretti G, Tozzi M (2006). "Intravenous uterine leiomyomatosis with right heart extension: successful two-stage surgical removal". Ann Vasc Surg. 20 (3): 405–7. doi:10.1007/s10016-006-9024-0. PMID 16583249.
- ↑ Nam MS, Jeon MJ, Kim YT, Kim JW, Park KH, Hong YS (2003). "Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literature". Gynecol Oncol. 89 (1): 175–80. PMID 12694674.
- ↑ Tielliu IF, Otterman ML, Meuzelaar JJ, Zeebregts CJ, Peeters PM (2010). "Intravenous leiomyomatosis: report of two cases and strategy for surgical resection". Minerva Chir. 65 (4): 489–93. PMID 20802437.